The DOI matches its Crossref record; 64 of the 66 references it cites were found in a registry or an index; 40 of the 80 sources its claims cite were checked for support.
Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing
Open accessAbstract
Of 7028 disorders with suspected Mendelian inheritance, 1139 are recessive and have an established molecular basis. Although individually uncommon, Mendelian diseases collectively account for ~20% of infant mortality and ~10% of pediatric hospitalizations. Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis. However, extension of preconception screening to most severe disease genes has hitherto been impractical. Here, we report a preconception carrier screen for 448 severe recessive childhood diseases. Rather than costly, complete sequencing of the human genome, 7717 regions from 437 target genes were enriched by hybrid capture or microdroplet polymerase chain reaction, sequenced by next-generation sequencing (NGS) to a depth of up to 2.7 gigabases, and assessed with stringent bioinformatic filters. At a resultant 160x average target coverage, 93% of nucleotides had at least 20x coverage, and mutation detection/genotyping had ~95% sensitivity and ~100% specificity for substitution, insertion/deletion, splicing, and gross deletion mutations and single-nucleotide polymorphisms. In 104 unrelated DNA samples, the average genomic carrier burden for severe pediatric recessive mutations was 2.8 and ranged from 0 to 7. The distribution of mutations among sequenced samples appeared random. Twenty-seven percent of mutations cited in the literature were found to be common polymorphisms or misannotated, underscoring the need for better mutation databases as part of a comprehensive carrier testing strategy. Given the magnitude of carrier burden and the lower cost of testing compared to treating these conditions, carrier screening by NGS made available to the general population may be an economical way to reduce the incidence of and ameliorate suffering associated with severe recessive childhood disorders.
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Claims the paper makes
50 Own result · 34 Own interpretation · 19 No basis given · 12 Partly supported · 9 Own method · 7 No full text of the sources · 5 Not checked yet · 4 Couldn’t confirm · 4 Sources not found · 2 Not supported · 1 Supported · accuracy not yet measured
At least one cited source doesn’t support this claim or points to a different paper, and none confirmed it — see the verdicts below.
In the paper
“In addition, some studies of cystic fibrosis carrier screening for a few common alleles have shown decreased prevalence of tested alleles with time, rather than reduced disease incidence (24, 25).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
What the sources say
- Not checked — no full text of the sourcecites [24] source
- Disputedcites [25] sourceExperimental — accuracy not yet measured
In “Newborn Screening Showing Decreasing Incidence of Cystic Fibrosis” (page unavailable), the authors report fewer Massachusetts newborns with cystic fibrosis from 2003 to 2006, alongside fewer with the ΔF508/ΔF508 genotype. The claim’s contrast—decreased prevalence of a tested genotype rather than reduced disease incidence—conflicts with that report; the paper also studies newborns, not allele prevalence among screened carriers. (LLM, conf=0.88)
Checked by a reasoning model — may be wrong.
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Data demonstrating the sensitivity and specificity of genotyping of disease mutations, particularly polynucleotide indels, gross insertions and deletions, copy number variations (CNVs), and complex rearrangements, are very limited (20–22).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Partly supportedcites [20] sourceExperimental — accuracy not yet measured
The main text reports indel-detection sensitivity below 80% and describes indel underestimation in difficult sequences; the Supplementary Methods describe validation of indels and large deletions. These passages show limitations in this genome analysis, but do not establish that sensitivity and specificity data for disease-mutation genotyping are generally very limited, particularly for CNVs and complex rearrangements. The supplied passages have no page numbers. (LLM, conf=0.76)
- Partly supportedcites [21] sourceExperimental — accuracy not yet measured
The Main section reports that no inferred indel genotype differences were replicated by a second method and that longer reads and lower error rates are needed to assess repetitive sequences comprehensively. Those findings support a narrower concern about genotyping limitations. The paper does not establish the broader claim that sensitivity-and-specificity data for disease mutations, gross insertions or deletions, CNVs, and complex rearrangements are very limited. (LLM, conf=0.74)
- Could not verifycites [22] sourceExperimental — accuracy not yet measured
The passages on sequencing errors and CNV-related assembly problems (section labelled Abstract; page unavailable) address technical difficulties, but do not say that sensitivity and specificity data for genotyping disease mutations are very limited. Searches for indels, large insertions and deletions, CNVs, and complex rearrangements found no passage making that broader claim. (LLM, conf=0.73)
- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- Partly supported
The cited sources that could be checked couldn’t confirm or dispute this claim.
In the paper
“Unexpectedly, 14 of 113 literature-annotated disease mutations were either incorrect or incomplete (table S7) (35–39).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
What the sources say
- Not checked — no full text of the sourcecites [35] source
- Not checked — no full text of the sourcecites [36]
- Not checked — no full text of the sourcecites [37] source
- Not checked — no full text of the sourcecites [38]
- Could not verifycites [39] sourceExperimental — accuracy not yet measured
Structural Studies (pp. 5–6) identifies the Hb Potomac substitution as β101 Glu→Asp. Our copy does not assess literature-annotated mutations for errors or incompleteness, or report a count of 14 out of 113. (LLM, conf=0.78)
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
The cited sources that could be checked couldn’t confirm or dispute this claim.
In the paper
“Indeed, 286 severe childhood autosomal recessive diseases encompassed 19,640 known disease mutations (8, 27, 28).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
What the sources say
- Not matchedcites [8]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
- Could not verifycites [27] sourceExperimental — accuracy not yet measured
The Abstract (page unavailable) reports more than 85,000 lesions across 3,253 genes, but does not give a count for severe childhood autosomal recessive diseases. Searches for “286,” “19,640,” and “autosomal recessive childhood disease” found no passage in our copy establishing either claimed figure. (LLM, conf=0.72)
- Not matchedcites [28]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- Not matched
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Given that the Human Gene Mutation Database (HGMD) lists 102,433 disease mutations (27), a number that is steadily increasing, a fixed-content method appeared impractical.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
What the sources say
- Partly supportedcites [27] sourceExperimental — accuracy not yet measured
The Correspondence section reports that HGMD records disease-associated mutations and that new entries have been increasing, exceeding 9,000 per year. It does not give the cited total of 102,433. The judgment that a fixed-content method was impractical is the citing authors’ inference, not a conclusion attributed to this source. (LLM, conf=0.84)
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- Partly supported
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“In 104 unrelated DNA samples, the average genomic carrier burden for severe pediatric recessive mutations was 2.8 and ranged from 0 to 7.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
plainn = 104- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Four hundred and forty-eight X-linked recessive and autosomal recessive diseases, encompassing 437 genes, met these criteria (table S1).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“mutation detection/genotyping had ~95% sensitivity and ~100% specificity for substitution, insertion/deletion, splicing, and gross deletion mutations and single-nucleotide polymorphisms.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Twenty-seven percent of mutations cited in the literature were found to be common polymorphisms or misannotated, underscoring the need for better mutation databases as part of a comprehensive carrier testing strategy.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Most recessive disorders for which a large proportion of burden was attributable to a few disease mutations were limited to specific ethnic groups.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
- It may state two separate claims as one. 51
- Probably not a substantial claim. 39
- The quote only partly states this claim. 1
At least one cited source doesn’t support this claim or points to a different paper, and none confirmed it — see the verdicts below.
In the paper
“However, concomitant analysis of normalized coverage in batches of samples appears to circumvent the need for adjustment for GC content (32), allowing more accurate detection of segmental losses.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
What the sources say
- Disputedcites [32] sourceExperimental — accuracy not yet measured
The Results state that each sample received its own GC-dependent weighting before its coverage was divided by an average across samples. The Methods likewise describe normalizing a GC-corrected distribution by a multi-sample baseline. Thus, the paper does not present batch normalization as circumventing GC adjustment; it uses both steps. (LLM, conf=0.88)
Checked by a reasoning model — may be wrong.
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Disputed
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“In genome research, an average depth of sequence coverage of 30-fold has been accepted as sufficient for single-nucleotide polymorphism (SNP) and nucleotide insertion or deletion (indel) detection (15–22).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Partly supportedcites [15] sourceExperimental — accuracy not yet measured
The Results (Accuracy of SNP detection; page not provided) say SNP-detection sensitivity is mainly limited by sequence coverage. The paper does not identify 30-fold average coverage as a sufficient threshold, and the Methods (Computational methods; page not provided) state that insertions and deletions were not considered. (LLM, conf=0.84)
- Partly supportedcites [16] sourceExperimental — accuracy not yet measured
The Results and Discussion section on coverage uniformity reports that 25-fold average coverage gave at least five reads for about 90% of targeted bases, and the paper evaluates SNP calls. It does not state that 30-fold coverage is an accepted sufficiency threshold or establish that depth for indel detection. (LLM, conf=0.83)
- Partly supportedcites [17] sourceExperimental — accuracy not yet measured
The paper reports SNP and indel detection in exomes with average coverage of 51× (Abstract; page unavailable). Its Methods specify at least 8× coverage for variant calling, but our copy does not say that an average depth of 30× is an accepted sufficient threshold for detecting both SNPs and indels. (LLM, conf=0.84)
- Partly supportedcites [18] sourceExperimental — accuracy not yet measured
The Conclusion (page unavailable) says average coverage of at least 20× appears the safest strategy, supporting the general point that substantial coverage aids variant detection. The paper does not identify 30× as an accepted threshold or establish its sufficiency for both SNP and indel detection; its quantified detection results concern SNPs. (LLM, conf=0.82)
- Could not verifycites [19] sourceExperimental — accuracy not yet measured
The Methods (Read mapping and variant analysis; page unavailable) use a ≥8× coverage cutoff for sequence calls and describe coding-indel identification, but do not assess whether 30-fold average coverage is sufficient. The Discussion (page unavailable) notes that low coverage can cause missed variant calls; searches for 30-fold coverage and its sufficiency found no statement of the claimed standard. (LLM, conf=0.76)
- Partly supportedcites [20] sourceExperimental — accuracy not yet measured
Table 1 reports 27.83-fold coverage for the Korean genome, and the Methods Summary says SNPs and indels were identified. That supports detection at approximately 30-fold coverage in this study, but the paper does not establish that 30-fold is an accepted general threshold for sufficient detection; the main text also reports indel-detection sensitivity below 80%. (LLM, conf=0.83)
- Could not verifycites [21] sourceExperimental — accuracy not yet measured
The Online Methods section on SNP detection describes read-count and quality filters, while the RRBS alignment section describes indel mapping; neither says that an average coverage depth of 30-fold is sufficient for detecting both. Searches for 30-fold coverage and a sufficiency threshold found no passage establishing that claim in our copy. (LLM, conf=0.74)
- Partly supportedcites [22] sourceExperimental — accuracy not yet measured
The passage describing the family's sequencing (section labelled Abstract; page unavailable) reports an average coverage of 40× and identification of single-nucleotide variants. It does not establish that 30× coverage is accepted as sufficient for detecting SNPs and indels, or report indel detection. (LLM, conf=0.83)
- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Partly supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Criteria included test accuracy and cost, disease severity, highly penetrant recessive inheritance, and whether an intervention was available for those identified.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Partly supportedcites [14] sourceExperimental — accuracy not yet measured
In “The screening test: availability and characteristics” (page unavailable), the report includes test sensitivity, specificity, and cost among its criteria. “Clinical characteristics of the condition” addresses disease burden, and “Diagnosis, follow-up, treatment, and management” addresses treatment availability. The cited criteria do not establish highly penetrant recessive inheritance as a requirement. (LLM, conf=0.88)
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may state two separate claims as one.
- Partly supported
The cited sources that could be checked couldn’t confirm or dispute this claim.
In the paper
“To be well suited for array-based screening, however, most carriers must be accounted for by a few mutations, and most disease mutations must be nucleotide substitutions (8, 27, 28).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
What the sources say
- Not matchedcites [8]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
- Could not verifycites [27] sourceExperimental — accuracy not yet measured
The Correspondence and Advanced search tools sections describe mutation types in HGMD, but neither discusses when array-based carrier screening is well suited. Searches for array screening and carrier frequency found no passage saying that a few mutations must account for most carriers or that most disease mutations must be nucleotide substitutions. (LLM, conf=0.72)
- Not matchedcites [28]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Not matched
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Target capture and next-generation sequencing (NGS) have shown efficacy and, recently, scalability for resequencing human genomes and exomes, providing an alternative potential paradigm for comprehensive carrier testing (15–22).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Partly supportedcites [15] sourceExperimental — accuracy not yet measured
The Results (Capturing and sequencing exon targets; Reproducibility, pages unavailable) demonstrate capture and next-generation sequencing of more than 15,000 human coding exons, with high-confidence genotypes across most of the targeted space. The introductory text reports that whole human genomes had been sequenced with next-generation instruments. The paper does not demonstrate target capture at whole-exome or whole-genome scale, so the scalability claim is supported for large targeted subsets rather than those complete targets. (LLM, conf=0.84)
- Partly supportedcites [16] sourceExperimental — accuracy not yet measured
The Abstract and scale-up Results report effective next-generation sequencing after targeted enrichment of human genomic regions, scaled to 1.49 Mb. The Discussion describes larger-scale exon capture reported by other studies. This paper does not demonstrate scalable capture and resequencing of whole human genomes or exomes; its own experiments cover subsets of them. (LLM, conf=0.87)
- Partly supportedcites [17] sourceExperimental — accuracy not yet measured
The paper’s Abstract reports targeted capture and massively parallel sequencing of 12 human exomes, with sensitive variant detection across more than 300 Mb of coding sequence. Its discussion describes the approach as reproducible and able to extend a given amount of sequencing across more samples, supporting efficacy and scalability for exomes. It does not demonstrate resequencing whole human genomes. (LLM, conf=0.84)
- Partly supportedcites [18] sourceExperimental — accuracy not yet measured
The Abstract and Conclusion report effective target capture and NGS for sequencing exomes from eight people. They do not demonstrate scalability to large cohorts or whole-genome resequencing; the Introduction says full-genome sequencing of many people remains prohibitively costly. Page numbers are unavailable in our copy. (LLM, conf=0.86)
- Supportedcites [19] sourceExperimental — accuracy not yet measured
The Methods section (page unavailable) describes targeted capture and next-generation sequencing of four human exomes. The paper’s introductory discussion (page unavailable) also describes whole-genome resequencing as increasingly practical and exome resequencing as a substantially less sequencing-intensive, high-throughput alternative, supporting the broad efficacy and scalability claim. The carrier-testing application is the citing authors’ proposed use, not a finding attributed to this paper. (LLM, conf=0.85)
- Partly supportedcites [20] sourceExperimental — accuracy not yet measured
The main text reports massively parallel sequencing of one human genome and high-depth sequencing of selected regions using BAC clones; the Methods Summary gives a six-week run time using three instruments. This supports the feasibility of next-generation whole-genome sequencing, but the paper does not demonstrate exome resequencing, target capture, or scalability across multiple genomes. (LLM, conf=0.85)
- Partly supportedcites [21] sourceExperimental — accuracy not yet measured
The Main section reports high-coverage, near-complete whole-genome sequencing of two human twins, supporting the efficacy of next-generation sequencing for human genomes. The paper does not establish target capture or exome resequencing, or demonstrate scalability beyond the genomes studied (searched: target capture, exome, scalability). (LLM, conf=0.84)
- Partly supportedcites [22] sourceExperimental — accuracy not yet measured
The paper’s sequencing-method and family-analysis passages (section labeled Abstract; page unavailable) describe short-read sequencing of four complete human genomes and its effectiveness in narrowing candidate genes for two disorders. They do not demonstrate target capture, exome resequencing, or scalability beyond this family quartet. (LLM, conf=0.86)
boosteddirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Partly supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Hitherto, important criteria precluding extension of preconception screening to most severe recessive mutations or the general population have been cost [defined in that report as an overall analytical cost requirement of <$1 per test per condition (14)] and the absence of accurate, sensitive, scalable technologies.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Partly supportedcites [14] sourceExperimental — accuracy not yet measured
In “The screening test: availability and characteristics” (page unavailable), the report values low-cost, high-throughput testing and sets a criterion of less than $1 per infant screened. It does not state an overall analytical cost requirement of less than $1 per test per condition; its criteria concern newborn screening, not preconception screening. (LLM, conf=0.87)
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Partly supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Given the need for highly accurate carrier detection, we required >10 uniquely aligned reads of quality score >20 and >14% of reads to call a variant (20, 21).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
What the sources say
- Partly supportedcites [20] sourceExperimental — accuracy not yet measured
The Methods Summary (page not available) says sequences and runs were used if their average Q scores were at least 20, and that SNPs and indels were identified using optimized filters. It does not specify more than 10 uniquely aligned reads or more than 14% variant-supporting reads; its average-quality criterion is also not the claimed per-read threshold. (LLM, conf=0.82)
- Partly supportedcites [21] sourceExperimental — accuracy not yet measured
The Methods Summary (page unavailable) reports 11–44× coverage and Q ≥ 20 for genotyping, while the Online Methods describe filters based on uniquely aligning reads and the percentage of reads calling a SNP. Our copy does not establish the claimed combined rule of >10 uniquely aligned reads, quality score >20, and >14% variant-supporting reads; the Methods Summary instead mentions ≥1 uniquely aligning read and refers to supplementary frequency cutoffs. (LLM, conf=0.85)
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Partly supported
Every source the paper cites for this claim was checked and supports it.
In the paper
“Notably, substitutions, indels, splicing mutations, and gross deletions account for the vast majority (96%) of annotated mutations (27).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
What the sources say
- Supportedcites [27] source
Table 1 (Correspondence; no page indicated) lists 48,343 missense or nonsense substitutions, 8,219 splicing mutations, 13,628 small deletions, 5,567 small insertions, 1,244 small indels, and 5,158 large deletions in HGMD Professional. Together these are 82,159 of its 85,558 entries, or approximately 96%, as of 1 December 2008. (LLM, conf=0.94)
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Preliminary experiments suggested that existing protocols for Agilent SureSelect hybrid capture (15) and RainDance microdroplet polymerase chain reaction (PCR) (16) but not Febit HybSelect microarray-based biochip capture (30) or Olink padlock probe ligation and PCR (31) yielded consistent target enrichment.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
What the sources say
- Supportedcites [15] source
Citation (15) is attached to Agilent hybrid capture, not to the RainDance, Febit, or Olink methods. The Results section (“Hybrid selection method”) describes capture using Agilent-synthesized oligonucleotides, and “Reproducibility” reports closely matching coverage across independent selections. The preliminary experiments are the citing authors’ own framing. (LLM, conf=0.87)
- Supportedcites [16] source
Citation 16 is attached to the RainDance microdroplet PCR protocol, not to the other named methods or the citing authors’ preliminary experiments. The Online Methods identify RainDance equipment in the microdroplet workflow, and the Discussion reports highly reproducible amplification and efficient enrichment of targeted sequences (page not available in our copy). (LLM, conf=0.92)
- Partly supportedcites [30] sourceExperimental — accuracy not yet measured
The Microarray design and synthesis section (p. 2) identifies a febit Geniom microfluidic array, and Figure 1 (p. 3) describes probes capturing PCR products for detection. The paper does not identify this method as HybSelect or assess whether it yielded consistent target enrichment in the citing authors’ preliminary experiments. (LLM, conf=0.82)
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Given that the Human Gene Mutation Database (HGMD) lists 102,433 disease mutations (27), a number that is steadily increasing, a fixed-content method appeared impractical.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
What the sources say
- Partly supportedcites [27] sourceExperimental — accuracy not yet measured
The Correspondence section says HGMD records disease-causing mutations and that its annual number of new entries has been steadily increasing. Our copy does not report the claimed total of 102,433 disease mutations; the exact figure is therefore unsupported by this citation. (LLM, conf=0.88)
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Partly supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Previous studies have identified CNVs on the basis of changes in regional coverage along a chromosome in an individual sample (20, 21).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
What the sources say
- Supportedcites [20] source
The CNV-detection discussion reports reduced sequencing coverage for deletions in the AK1 individual genome, and Figure 2 describes a coverage drop defining a deletion. It also reports that increased regional coverage confirmed copy-number gains. The paper used additional evidence alongside coverage, but the claim does not say coverage was the sole basis for detection. (LLM, conf=0.88)
- Partly supportedcites [21] sourceExperimental — accuracy not yet measured
The Online Methods section on array-based genotyping and CNV detection (page unavailable) confirms that the study identified CNVs in individual DNA samples. It says detection used changes in array-probe intensity and SNP allele ratios, not regional sequencing coverage along a chromosome; that specific method is not supported. (LLM, conf=0.83)
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“At high volume, the overall analytical cost of the hybrid enrichment-based test was $378, achieving the requirement of <$1 per test per condition and approximating that expended on treatment of severe recessive childhood disorders per U.S. live birth (14, 29).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
What the sources say
- Partly supportedcites [14] sourceExperimental — accuracy not yet measured
The screening-test criteria section specifies a cost criterion of less than $1 per infant screened, and the changing technological landscape section says multiplex testing can reduce cost per condition. Neither section establishes a less-than-$1-per-condition requirement or shows that a $378 hybrid enrichment-based test meets it. The copy also does not give the $378 figure or the claimed comparison with treatment expenditure per U.S. live birth. (LLM, conf=0.88)
- Not checked — no full text of the sourcecites [29] source
plain- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
- Partly supported
The cited sources support only part of this claim — read each source’s verdict below.
In the paper
“Alternatively, microdroplet PCR should theoretically achieve a sensitivity of ~99%, albeit at higher cost (16, 47).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
What the sources say
- Partly supportedcites [16] sourceExperimental — accuracy not yet measured
The Discussion reports that more than 99.6% of amplicons were successfully amplified, which supports a roughly 99% figure for amplification success, not necessarily sensitivity. It separately reports a 94.5% variant-detection rate for microdroplet PCR. Our copy does not establish the claimed higher cost relative to an alternative. (LLM, conf=0.86)
- Partly supportedcites [47] sourceExperimental — accuracy not yet measured
The Discussion (page unavailable) reports correct variant calls at up to 99.5% of uniquely mappable positions in the targeted regions, supporting a near-99% figure for this method. It does not describe that figure as a theoretical sensitivity or state that microdroplet PCR costs more; searches for sensitivity and cost found no such comparison. (LLM, conf=0.86)
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
- Partly supported
The cited sources that could be checked couldn’t confirm or dispute this claim.
In the paper
“Twenty-six samples were well characterized, from “normal” individuals, and two had previously undergone genome sequencing (21).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > MATERIALS AND METHODS > DNA samples
What the sources say
- Could not verifycites [21] sourceExperimental — accuracy not yet measured
The Main section reports whole-genome sequencing of two twins in this study, but does not say they were previously genome-sequenced members of a 104-sample set. Searches for the 104 samples and 26 well-characterized normal individuals found no passage addressing those details in our copy. (LLM, conf=0.76)
plainn = 104- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
- Could not verify
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“The distribution of mutations among sequenced samples appeared random.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“At a resultant 160× average target coverage, 93% of nucleotides had at least 20× coverage”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
plain- Asserted: passes — The authors put it forward as true.
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- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“First, with the Illumina HiSeq SBS platform, we used the novel approach of read pair assembly before alignment (99% efficiency) to generate longer reads with high-quality scores (148.6 ± 3.8 nt combined read length and increase in nucleotides with quality score >30 from 75 to 83%).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
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- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“As sequence depth increased from 0.7 to 2.7 Gb, sensitivity increased from 93.9 to 95.6%, whereas PPV remained ~100% (Fig. 6A).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The aligned sequence coverage distribution was unimodal but flat (platykurtic) and right-skewed (Fig. 2B).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The requirement for ≥14% of reads to call a variant was highly effective for nucleotides with very high coverage and was derived from the genotype data discussed below.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The positive predictive value (PPV) of sequence-based SNP genotypes was 99.96% and negative predictive value (NPV) was 98.5%, as ascertained by array hybridization.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Targets with low (<3×) coverage were highly reproducible and had high GC content (table S6).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“This resulted in linear amplification of a subset of targets, ~5% of target nucleotides with zero coverage and a similar proportion of nucleotides on target to that obtained in the best hybrid capture experiments (~30%; Table 1).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“For known substitution, indel, splicing, gross deletion, and regulatory alleles in 76 samples, sensitivity was 100% (113 of 113 known alleles; table S7).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
plainn = 76- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Interference of bar code nucleotides with hybrid selection did not occur appreciably: The stoichiometry of multiplexed pools was essentially unchanged before and after hybrid selection.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“In practice, the coverage distribution was narrower than hybrid capture but with similar right skewing (Fig. 2D).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“For heterozygote detection, for example, this was equivalent to ~20× coverage, which was achieved in ~96% of exons with ~2.6 gigabases (Gb) of sequence (Fig. 2C).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“This was achieved with hybrid capture after one round of bait redesign for underrepresented exons and decreased bait representation in over-represented exons (Table 1).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one. The quote only partly states this claim.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“With these cutoffs and a requirement for 20× coverage and 10 reads of quality ≥20 to call a variant, the accuracy of sequence-based SNP genotyping was 98.8%, sensitivity was 94.9%, and specificity was 99.99%.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
In the paper
“NA02057, from a female with aspartylglucosaminuria (OMIM #208400), characterized as a compound heterozygote, was homozygous for two adjacent substitutions (AGA exon 4, c.482G>A, R161Q, chr4:178596918G>A and exon 4, c.488G>C, C163S, chr4:178596912G>C in 38 of 39 reads; Fig. 8), of which C163S had been shown to be the disease mutation (43).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
What the sources say
- Not checked — no full text of the sourcecites [43] source
plainSingle case- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“As expected, median coverage increased linearly with sequence depth.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The proportion of bases with greater than zero and >20× coverage increased toward asymptotes at ~99 and ~96%, respectively (Table 1 and Fig. 2C).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“However, whereas group sizes were small, no significant differences in total carrier burden were found between Caucasians and other ethnicities, between males and females, nor between affected and unaffected individuals (after correction for compound heterozygosity in those affected).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Carrier burden
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The requirement for at least 10 high-quality reads to substantiate a variant call resulted in a specificity of 99.96% for single-nucleotide substitutions (which is the limit of accuracy for the gold standard method used) and 100% for about 200 known mutations and new indels in our screening method.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Four hundred and thirty-seven genes, representing 507 recessive diseases, met these criteria, of which 448 diseases were severe (table S3).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > MATERIALS AND METHODS > Disease selection
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Specifically, current reference resources contain common polymorphisms that are annotated as disease mutations and erroneous disease mutations.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds. It may state two separate claims as one.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Seventy-four percent of these, however, were accounted for by 47 substitutions each with an incidence of ≥5%, of which 20 were homozygous in samples unaffected by the corresponding disease (table S8).”
- Also stated in: § DISCUSSION · plain
“Unexpectedly, we found that 74% of “disease mutation” calls were accounted for by substitutions with incidences of ≥5%, of which almost one-half were homozygous in samples unaffected by the corresponding disease.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Carrier burden
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
- Also stated in: § DISCUSSION · plain
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The burden of nonconservative, nonsynonymous, uncommon (<5% incidence) VUS was ~11 per sample.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
A result the paper reports from its own data — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Specificity was 99.96%, and a sensitivity of ~95% was attained with hybrid capture at a sequence depth of 2.5 Gb per sample.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Second, comprehensive mutation sets, allele frequencies in populations, and individual mutation genotype-phenotype relationships have been defined in very few recessive diseases.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds.
In the paper
“Of 7028 disorders with suspected Mendelian inheritance, 1139 are recessive and have an established molecular basis.”
- Also stated in: § INTRODUCTION · plain
“Over the past 25 years, 1139 genes that cause Mendelian recessive diseases have been identified (8).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
What the sources say
- Not matchedcites [8]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Also stated in: § INTRODUCTION · plain
A claim is checked only against the cited paper’s full text, never its abstract, and none is available for its sources.
In the paper
“Although individual Mendelian diseases are uncommon in general populations, collectively, they account for ~20% of infant mortality and ~10% of pediatric hospitalizations (6, 7).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
In the paper
“A recent workshop provided recommendations for qualification of new methodologies for broader population-based carrier screening (23). These were high analytical validity, concordance in many settings, high throughput, and cost-effectiveness (including sample acquisition and preparation).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
What the sources say
- Not matchedcites [23]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may state two separate claims as one.
- Not matched
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
boosteddirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Note that these genes, although a good representative set, require further assessment of clinical readiness before translation into clinical testing.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
plain- Asserted: passes — The authors put it forward as true.
- Checkable: fails — It may be too vague to check against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may be too vague to check against evidence. The paper’s conclusions may not depend on it. It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“However, extension of preconception screening to most severe disease genes has hitherto been impractical.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > Abstract
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“In particular, the accuracy of disease mutation genotypes derived from NGS of enriched targets has been uncertain.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“The proportion of targets with at least 20× coverage appeared to be useful for quality assessment.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
hedgedcorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Multiplexing of samples during hybrid selection and NGS had not previously been reported.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. The paper may give no reason why it holds.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“The number of mismatches tolerated for unique alignment of short-read sequences is highly constrained but increases with read length.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds. It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“However, comprehensive carrier testing also requires detection of polynucleotide indels, gross insertions, gross deletions, and complex rearrangements.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Other concerns with array-based screening for recessive disorders were type 1 errors in the absence of confirmatory testing and type 2 errors for disease mutations other than substitutions (complex rearrangements, indels, or gross deletions with uncertain boundaries).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Technology selection
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“The vast majority of disease mutations are single-nucleotide substitutions or small indels.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Third, gross deletions were detected both by perfect alignment to mutant junction reference sequences and by local decreases in normalized coverage (normalized to total sequence generated; C. H. Hu, personal communication).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Omission of gel purification was critical for scalability of library generation.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Unambiguous alignment of short-read sequences is typically confounded by repetitive sequences, but was not relevant for carrier testing, because targets overwhelmingly contained unique sequences.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
The paper states this without a source or data behind it here, so there is nothing to check it against. The quote shows it is in the paper.
In the paper
“Of these, the Illumina sequencing-by-synthesis (SBS) and SOLiD sequencing-by-ligation (SBL) platforms are widely disseminated and have throughput of at least 50 Gb per run and read lengths of at least 50 nt.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“This suggested that targets failing hybrid capture could be predicted and, perhaps, rescued by individual PCRs.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Analytic metrics
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
In the paper
“Nevertheless, a burden of 2.8 per genome agreed with theoretical estimates of reproductive lethal allele burden (56).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
What the sources say
- Not matchedcites [56]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Not matched
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“For example, comprehensive newborn screening for treatable or preventable Mendelian diseases would allow early diagnosis and institution of treatment while neonates are asymptomatic.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedgeddirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Third, many individuals in our cohort were affected by one of these diseases. Although a correction was made for compound heterozygote and homozygote alleles, the burden estimate did not correct for other potential selection biases.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
None of the sources cited for this claim have been checked yet.
In the paper
“Community-based carrier testing has had high uptake, without apparent stigma or discrimination and with substantial reductions in the frequencies of tested disorders (3, 48, 49, 51–54).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
What the sources say
- Not checked — no full text of the sourcecites [3]
- Not checked — no full text of the sourcecites [48] source
- Not checked — no full text of the sourcecites [49] source
- Not checked — no full text of the sourcecites [51]
- Not checked — no full text of the sourcecites [52] source
- Not matchedcites [53]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
- Not matchedcites [54]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: unclear — It is unclear whether the paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Combining this technology with genetic counseling could reduce the incidence of severe recessive pediatric diseases and may help to expedite diagnosis of these disorders in newborns.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper may give no reason why it holds. It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Finally, the technology platform described herein is agnostic with regard to target genes or clinical setting.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“We noted, however, that identification of larger polynucleotide indels was influenced in some sequences by the particular alignment seed, suggesting that additional refinement of alignment parameters is needed.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: fails — It may be too vague to check against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may be too vague to check against evidence. The paper’s conclusions may not depend on it.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Early treatment can have a profound impact on the clinical severity of conditions and could provide a framework for centralized assessment of investigational new treatments before organ failure.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. The paper may give no reason why it holds. It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Key advantages of NGS are clonal derivation (facilitating unambiguous detection of heterozygous and indel variants), maintenance of phase information (allowing haplotype derivation for adjacent variants), and highly redundant coverage (resulting in extremely low consensus error rates).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Although the analytical cost will decrease as the throughput of NGS improves, test interpretation, reporting, genetic counseling, and stewardship of mutation databases will confer considerable additional costs.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plaindirect- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“In some cases, such as Duarte variant galactosemia, molecular testing would be superior to conventional biochemical testing.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedged- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: fails — The paper may give no reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. The paper may give no reason why it holds.
In the paper
“However, the current study used DNA from Epstein-Barr virus (EBV)–transformed cell lines in which somatic hypermutation has been noted (45).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
What the sources say
- Not checked — no full text of the sourcecites [45] source
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“A variety of medical applications for this technology exist beyond use in preconception carrier screening.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“First, as discussed, an adequate compilation of pathogenic mutations does not currently exist, and strong evidence of pathogenicity was absent for some of the variants referred to as disease mutations.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it. It may state two separate claims as one.
In the paper
“As exemplified by cystic fibrosis, the carrier incidence and mutation spectrum of individual recessive disorders vary widely among populations (46).”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Carrier burden
What the sources say
- Not checked — no full text of the sourcecites [46] source
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
What the paper did — it cites no source for this, so there is none to check. The quote shows it is in the paper.
In the paper
“Adequacy of hierarchical clustering was attested to by samples from identical twins being nearest neighbors, as were two disease mutations in linkage disequilibrium.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Carrier burden
plaincorrelational- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: passes — The paper’s conclusions depend on it.
- Based: passes — The paper gives a reason why it holds.
It may state two separate claims as one.
A conclusion the paper draws from its own results — it cites no other source for it, so there is none to check. The quote shows it is in the paper.
In the paper
“Organ or symptom menu-based diagnostic testing, with masking of nonselected conditions, is anticipated to assist clinical geneticists and pediatric neurologists, because current practice often involves costly, sequential testing of numerous candidate genes.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
hedgedconditional- Asserted: passes — The authors put it forward as true.
- Checkable: fails — It may be too vague to check against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. It may be too vague to check against evidence. The paper’s conclusions may not depend on it.
In the paper
“Although technical standards and guidelines have been established for laboratory-developed genetic testing for rare disorders in accredited laboratories (26), there are several challenges in their adoption for NGS and bioinformatic-based testing of ~500 conditions.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
What the sources say
- Not matchedcites [26]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
plain- Asserted: passes — The authors put it forward as true.
- Checkable: passes — It can be checked against evidence.
- Load-bearing: fails — The paper’s conclusions may not depend on it.
- Based: passes — The paper gives a reason why it holds.
Probably not a substantial claim. The paper’s conclusions may not depend on it.
- Not matched
The authors state this as an assumption.
Cost-effectiveness is critical for adoption of carrier testing.
“First, cost-effectiveness was assumed to be critical for test adoption.”
The authors state this as an assumption.
The incremental cost of increasing the degree of multiplexing decreases toward an asymptote.
“The incremental cost associated with increasing the degree of multiplexing was assumed to decrease toward an asymptote.”
The authors state this as an assumption.
Very broad coverage of diseases offers optimal cost-benefit.
“Thus, very broad coverage of diseases was assumed to offer optimal cost-benefit.”
The authors state this as an assumption.
Physician, patient, and societal adoption of screening is optimal for the most severe and highly penetrant childhood diseases, before conception, and where the anticipated clinical validity and utility of testing are clear.
“Third, physician, patient, and societal adoption of screening was assumed to be optimal for the most severe and highly penetrant childhood diseases, before conception and where the anticipated clinical validity and clinical utility of testing was clear (26).”
The paper relies on this without support.
The 104 unrelated DNA samples are representative of the general population for genomic carrier burden of severe pediatric recessive mutations.
“In 104 unrelated DNA samples, the average genomic carrier burden for severe pediatric recessive mutations was 2.8 and ranged from 0 to 7.”
The paper relies on this without support.
The cost of carrier screening by next-generation sequencing is lower than the cost of treating severe recessive childhood conditions.
“Given the magnitude of carrier burden and the lower cost of testing compared to treating these conditions, carrier screening by NGS made available to the general population may be an economical way to reduce the incidence of and ameliorate suffering associated with severe recessive childhood disorders.”
The paper relies on this without support.
Therapies are available for many severe recessive diseases of childhood.
“Although TSD remains incurable, therapies are available for many severe recessive diseases of childhood.”
The paper relies on this without support.
Specific national standards for quality assurance, quality control, test accessioning and reporting, and proficiency evaluation do not currently exist.
“For example, specific national standards for quality assurance, quality control, test accessioning and reporting, and proficiency evaluation do not currently exist.”
The paper relies on this without support.
A two-stage approach with confirmatory testing of all positive results is currently used for preconception carrier screening of couples.
“Currently, a two-stage approach is used for preconception carrier screening of couples, with confirmatory testing of all positive results.”
The paper relies on this without support.
Severe, orphan disease mutations have an incidence below 1% and are not found in the homozygous state in unaffected individuals.
“Severe, orphan disease mutations should be uncommon (<1% incidence) and should not be found in the homozygous state in unaffected individuals.”
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
Each contradiction between two passages listed here was confirmed by an independent check.
Rated minor: it would not touch a result — a value no result rests on, a p-value that stays on the same side of the significance level, or a conflict that is clerical, resolved elsewhere in the paper, or outside its own findings.
In the paper
“The disease type was cardiac for 8, cutaneous for 45, developmental for 46, endocrine for 15, gastroenterological for 3, hematological for 15, hepatic for 3, immunological for 29, metabolic for 142, neurological for 122, ocular for 12, renal for 25, respiratory for 8, and skeletal for 28.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
Reported 448 · recomputed 501
Rated minor: it would not touch a result — a value no result rests on, a p-value that stays on the same side of the significance level, or a conflict that is clerical, resolved elsewhere in the paper, or outside its own findings.
In the paper
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
“Here, we report the development of a preconception carrier screen for 448 severe recessive childhood disease genes, based on target enrichment and NGS that meets most of these criteria, and use of the screen to assess carrier burden for severe recessive diseases of childhood.”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
“Four hundred and forty-eight X-linked recessive and autosomal recessive diseases, encompassing 437 genes, met these criteria (table S1).”
Rated minor: it would not touch a result — a value no result rests on, a p-value that stays on the same side of the significance level, or a conflict that is clerical, resolved elsewhere in the paper, or outside its own findings.
In the paper
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > INTRODUCTION
“Here, we report the development of a preconception carrier screen for 448 severe recessive childhood disease genes”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Disease inclusion
“Four hundred and forty-eight X-linked recessive and autosomal recessive diseases, encompassing 437 genes, met these criteria (table S1).”
Rated minor: it would not touch a result — a value no result rests on, a p-value that stays on the same side of the significance level, or a conflict that is clerical, resolved elsewhere in the paper, or outside its own findings.
In the paper
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > DISCUSSION
“The requirement for at least 10 high-quality reads to substantiate a variant call resulted in a specificity of 99.96% for single-nucleotide substitutions (which is the limit of accuracy for the gold standard method used)”
§ Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing > RESULTS > Clinical metrics
“With these cutoffs and a requirement for 20× coverage and 10 reads of quality ≥20 to call a variant, the accuracy of sequence-based SNP genotyping was 98.8%, sensitivity was 94.9%, and specificity was 99.99%. The positive predictive value (PPV) of sequence-based SNP genotypes was 99.96%”
Cited works that were retracted
Works this paper cites that were later retracted, from the retraction notices publishers deposit. Major when the notice came out before this paper was published.
A note, not an issue: for example, a cited work that was retracted only after this paper was published, which its authors could not have known.
Cited work 10.1097/00006250-200512000-00055 · retraction date not known · this paper published 2011-01-12
Citations
Cites
1 retracted
(2005) · Obstetrics and Gynecology · doi:10.1097/00006250-200512000-00055 · Cited by 120
RetractedTargeted capture and massively parallel sequencing of 12 human exomes
Ng, S. B., Turner, E. H., Robertson, P. D., Flygare, S. D., Bigham, A. W., Lee, C., Shaffer, T., Wong, M., Bhattacharjee, A., Eichler, E. E., et al. (2009) · Nature · doi:10.1038/nature08250 · Cited by 2,030
Self-citationExome sequencing identifies the cause of a mendelian disorder
Ng, S. B., Buckingham, K. J., Lee, C., Bigham, A. W., Tabor, H. K., Dent, K. M., Huff, C. D., Shannon, P. T., Jabs, E. W., Nickerson, D. A., et al. (2010) · Nature Genetics · doi:10.1038/ng.499 · Cited by 1,957
Self-citationPrescribing Personalized Nutrition for Cardiovascular Health: Are We Ready?
Juma, S., Imrhan, V., Vijayagopal, P., & Prasad, C. (2014) · Lifestyle Genomics · doi:10.1159/000370213 · Cited by 1,908
The complete genome of an individual by massively parallel DNA sequencing
Wheeler, D. A., Srinivasan, M., Egholm, M., Shen, Y., Chen, L., McGuire, A., He, W., Chen, Y.-J., Makhijani, V., Roth, G. T., et al. (2008) · Nature · doi:10.1038/nature06884 · Cited by 1,838 · cited 1×
“It also concurred with severe childhood recessive carrier burdens that we obtained by analyzing published individual genomes [2 substitution disease mutations in the Quake genome and a monozygotic twin pair (21, 57), 5 each in the YH and Watson genomes (58, 59), 4 in the NA07022 genome (60, 61), and 10 in the AK1 geno…”
MethodGnirke, A., Melnikov, A., Maguire, J., Rogov, P., LeProust, E. M., Brockman, W., Fennell, T., Giannoukos, G., Fisher, S., Russ, C., et al. (2009) · Nature Biotechnology · doi:10.1038/nbt.1523 · Cited by 1,433 · cited 2×
“SureSelect enrichment of 6-, 8-, or 12-plex pooled libraries was per Agilent protocols (15), with 100 ng of custom bait library, blocking oligonucleotides specific for paired-end sequencing libraries and 60-hour hybridization.”
BackgroundMethodHuman Genome Sequencing Using Unchained Base Reads on Self-Assembling DNA Nanoarrays
Drmanac, R., Sparks, A. B., Callow, M. J., Halpern, A. L., Burns, N. L., Kermani, B. G., Carnevali, P., Nazarenko, I., Nilsen, G. B., Yeung, G., et al. (2010) · Science · doi:10.1126/science.1181498 · Cited by 1,212
Analysis of Genetic Inheritance in a Family Quartet by Whole-Genome Sequencing
Roach, J. C., Glusman, G., Smit, A. F. A., Huff, C. D., Hubley, R., Shannon, P. T., Rowen, L., Pant, K. P., Goodman, N., Bamshad, M., et al. (2010) · Science · doi:10.1126/science.1186802 · Cited by 1,084
McDonald, C. M., Abresch, R. T., Carter, G. T., Fowler, W. M., Johnson, E. R., Kilmer, D. D., & Sigford, B. J. (1995) · American Journal of Physical Medicine & Rehabilitation · doi:10.1097/00002060-199509001-00003 · Cited by 1,070
Bobadilla, J. L., Macek, M., Fine, J. P., & Farrell, P. M. (2002) · Human Mutation · doi:10.1002/humu.10041 · Cited by 1,032 · cited 2×
“RainDance RDT1000 target enrichment was as described and used a custom primer library (16, 46): Genomic DNA samples were fragmented by nebulization to 2 to 4 kb and 1 mg mixed with all PCR reagents but primers.”
BackgroundMethod
Cited by
Sorted by citations
Items 201–300 of 693
The role of genetic testing in unexplained sudden death
Miles, C. J., & Behr, E. R. (2015) · Translational research · doi:10.1016/j.trsl.2015.06.007 · Cited by 49
Impacts of massively parallel sequencing for genetic diagnosis of neuromuscular disorders
Vasli, N., & Laporte, J. (2012) · Acta Neuropathologica · doi:10.1007/s00401-012-1072-7 · Cited by 46
“carriers of a heterozygous mutation[8].”
BackgroundSaunders, C., Moon, S. H., Liu, X., et al. (2014) · Human Mutation · doi:10.1002/humu.22743 · Cited by 45
“Alignment and variant calling were performed as previously reported [Bell et al., 2011; Saunders et al., 2012].”
MethodSelf-citationNext-generation carrier screening
Umbarger, M. A., Kennedy, C. J., Saunders, P., Breton, B., Chennagiri, N., Emhoff, J., Greger, V., Hallam, S., Maganzini, D., Perreault-Micale, C. L., et al. (2013) · Genetics in Medicine · doi:10.1038/gim.2013.83 · Cited by 45
“8,10 Finally, the workflow must be highly robust and reproducible, which can often be achieved through automation.”
Adults with septic shock and extreme hyperferritinemia exhibit pathogenic immune variation
Kernan, K. F., Ghaloul‐Gonzalez, L., Shakoory, B., Kellum, J. A., Angus, D. C., & Carcillo, J. A. (2018) · Genes and Immunity · doi:10.1038/s41435-018-0030-3 · Cited by 45
Nijmeijer, S. C. M., Conijn, T., Lakeman, P., Henneman, L., Wijburg, F. A., & Haverman, L. (2018) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2018.12.004 · Cited by 46
Expanded carrier screening in Chinese patients seeking the help of assisted reproductive technology
Xi, Y., Chen, G., Lei, C., Wu, J., Zhang, S., Xiao, M., Zhang, W., Zhang, Y., & Sun, X. (2020) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.1340 · Cited by 45
“Although there have been quite a few studies on ECS, ranging from panel design (Bell et al., 2011; Martin et al., 2015) to clinical implementation (Franasiak et al.”
BackgroundGreen, R., Rehm, H., & Kohane, I. (2013) · Elsevier eBooks · doi:10.1016/b978-0-12-382227-7.00009-4 · Cited by 55
“In a recent proof-of-concept study (Bell et al., 2011), carrier status for 448 severe recessive pediatric conditions was determined through targeted sequencing of 26 controls and 104 cases, including 76 people known to be carriers or affected by 1 of 37 diseases.”
BackgroundChen, C., Li, R., Sun, J., et al. (2021) · Genome Medicine · doi:10.1186/s13073-021-00836-8 · Cited by 44
Jones, M. A., Rhodenizer, D., da Silva, C., et al. (2013) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2013.05.012 · Cited by 43
You, Y., Sun, Y. V., Li, X., et al. (2014) · Genetics in Medicine · doi:10.1038/gim.2013.197 · Cited by 43
Heinrich, V., Stange, J., Dickhaus, T., Imkeller, P., Krüger, U., Bauer, S., Mundlos, S., Robinson, P. N., Hecht, J., & Krawitz, P. M. (2012) · Nucleic Acids Research · doi:10.1093/nar/gkr1073 · Cited by 43
“This heterozygous detection algorithm was shown to be highly sensitive for a coverage >20 (14).”
BackgroundBoardman, F., Young, P. J., & Griffiths, F. E. (2016) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.38031 · Cited by 43
“Autosomal recessive conditions, although individually rare, account for approximately 20% of infant mortality worldwide [Bell et al., 2011].”
BackgroundGradishar, W. J., Johnson, K., Brown, K. L., Mundt, E., & Manley, S. (2017) · The Oncologist · doi:10.1634/theoncologist.2016-0431 · Cited by 44
Jolfayi, A. G., Kohansal, E., Ghasemi, S., Naderi, N., Hesami, M., MozafaryBazargany, M., Moghadam, M. H., Fazelifar, A. F., Maleki, M., & Kalayinia, S. (2024) · Scientific Reports · doi:10.1038/s41598-024-56154-7 · Cited by 41
Noninvasive fetal genome sequencing: a primer
Snyder, M. W., Simmons, L. E., Kitzman, J. O., Santillan, D. A., Santillan, M. K., Gammill, H. S., & Shendure, J. (2013) · Prenatal Diagnosis · doi:10.1002/pd.4097 · Cited by 41
Larson, J. L., Silver, A. J., Chan, D., Borroto, C., Spurrier, B., & Silver, L. M. (2015) · BMC Medical Genetics · doi:10.1186/s12881-015-0246-2 · Cited by 40
“While existing qPCR approaches are accurate on a case-by-case basis, none of the current processes can be incorporated into cost-effective NGS screens for the simultaneous detection of carrier status at hundreds of genes [31].”
BackgroundFunctional Genomics Applied to Cardiovascular Medicine
Cappola, T. P., & Margulies, K. B. (2011) · Circulation · doi:10.1161/circulationaha.111.027300 · Cited by 42
Thalassaemia screening and confirmation of carriers in parents
Barrett, A. N., Ramasamy, S., & Choolani, M. A. (2016) · Best Practice & Research Clinical Obstetrics & Gynaecology · doi:10.1016/j.bpobgyn.2016.10.015 · Cited by 43
Sivakumaran, T. A., Husami, A., Kissell, D., Zhang, W., Keddache, M. A., Black, A. P., Tinkle, B. T., Greinwald, J. H., & Zhang, K. (2013) · Otolaryngology · doi:10.1177/0194599813482294 · Cited by 39
The rapid evolution of molecular genetic diagnostics in neuromuscular diseases
Volk, A. E., & Kubisch, C. (2017) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000478 · Cited by 43
“common polymorphisms, sequencing errors, or do not shown any further evidence of pathogenicity [37].”
BackgroundIncidental Variants Are Critical for Genomics
Biesecker, L. G. (2013) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2013.04.001 · Cited by 40
Mathijssen, I. B., Henneman, L., van Eeten-Nijman, J. M. C., Lakeman, P., Ottenheim, C. P. E., Redeker, E. J. W., Ottenhof, W., Meijers-Heijboer, H. E. J., & van Maarle, M. C. (2015) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2015.01.004 · Cited by 40
Use of Targeted Exome Sequencing for Molecular Diagnosis of Skeletal Disorders
Polla, D. L., Cardoso, M. T., Silva, M. C. B., Cardoso, I. C. C., Medina, C. T. N., Araujo, R., Fernandes, C. C., Reis, A. M. M., de Andrade, R. V., Pereira, R. W., et al. (2015) · PLoS ONE · doi:10.1371/journal.pone.0138314 · Cited by 40
Pereira, N., Wood, M., Luong, E., Briggs, A., Galloway, M., Maxwell, R. A., & Lindheim, S. R. (2019) · Journal of Assisted Reproduction and Genetics · doi:10.1007/s10815-019-01414-z · Cited by 41
Bosdet, I., Docking, R., Butterfield, Y. S. N., Mungall, A. J. J., Zeng, T., Coope, R., Yorida, E., Chow, K., Bala, M., Young, S. S., et al. (2013) · Journal of Molecular Diagnostics · doi:10.1016/j.jmoldx.2013.07.004 · Cited by 39
Non‐invasive prenatal diagnosis for Down syndrome: the paradigm will shift, but slowly
BENN, P. A., Cuckle, H., & Pergament, E. (2012) · Ultrasound in Obstetrics and Gynecology · doi:10.1002/uog.11083 · Cited by 39
Genetics of movement disorders in the next‐generation sequencing era
Olgiati, S., Quadri, M., & Bonifati, V. (2016) · Movement Disorders · doi:10.1002/mds.26521 · Cited by 43
“field: up to 27% of the genetic variants in the scientific literature have been estimated to have a misleading designation.(96) Claims of the identification of a novel disease-causing gene should be considered with caution until confirmation is provided in independent data sets”
BackgroundNext-generation sequencing in neuromuscular diseases
Efthymiou, S., Manole, A., & Houlden, H. (2016) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000374 · Cited by 39
Sallevelt, S. C. E. H., Stegmann, A. P. A., de Koning, B., Velter, C., Steyls, A., van Esch, M., Lakeman, P., Yntema, H. G., Esteki, M. Z., de Die-Smulders, C. E. M., et al. (2021) · Genetics in Medicine · doi:10.1038/s41436-021-01116-x · Cited by 38
“Each individual is estimated to be heterozygous for up to seven AR pathogenic variants associated with severe disease.(1) When both partners of a couple carry a pathogenic variant in the same gene, they have a 25% risk of having affected offspring.”
BackgroundBowdin, S. C., Ray, P. N., Cohn, R. D., & Meyn, M. S. (2014) · Human Mutation · doi:10.1002/humu.22536 · Cited by 38
“Recent analyses indicate that disease-causing variants reported in the medical literature and in large-scale databases such as the Human Gene Mutation Database are frequently incorrect, with reported error frequencies of 4%–23% [Bell et al., 2011; Tong et al., 2011; Cassa et al., 2013].”
BackgroundUtility of Next Generation Sequencing in Clinical Primary Immunodeficiencies
Raje, N., Soden, S. E., Swanson, D. S., Ciaccio, C. E., Kingsmore, S. F., & Dinwiddie, D. L. (2014) · Current Allergy and Asthma Reports · doi:10.1007/s11882-014-0468-y · Cited by 40
Self-citationEthical quandaries around expanded carrier screening in third-party reproduction
Mertes, H., Lindheim, S. R., & Pennings, G. (2018) · Fertility and Sterility · doi:10.1016/j.fertnstert.2017.11.032 · Cited by 38
Ellard, S., Colclough, K., Patel, K., & Hattersley, A. T. (2019) · Journal of Clinical Investigation · doi:10.1172/jci133516 · Cited by 36
Molecular diagnosis of infantile onset inflammatory bowel disease by exome sequencing
Dinwiddie, D. L., Bracken, J. M., Bass, J., Christenson, K. M., Soden, S. E., Saunders, C., Miller, N., Singh, V., Zwick, D. L., Roberts, C. C., et al. (2013) · Genomics · doi:10.1016/j.ygeno.2013.08.008 · Cited by 38
“Successful enrichment was verified by qPCR of 4 targeted loci and 2 nontargeted loci of the sequencing library pre- and post-enrichment prior to sequencing [27].”
Self-citationSchuurmans, J., Birnie, E., Ranchor, A. V., et al. (2019) · European Journal of Human Genetics · doi:10.1038/s41431-019-0516-0 · Cited by 37
Reproductive options for families at risk of Osteogenesis Imperfecta: a review
Zhytnik, L., Simm, K., Salumets, A., Peters, M., Märtson, A., & Maasalu, K. (2020) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-020-01404-w · Cited by 37
Recent advances of genomic testing in perinatal medicine
Peters, D. G., Yatsenko, S. A., Surti, U., & Rajkovic, A. (2014) · Seminars in Perinatology · doi:10.1053/j.semperi.2014.10.009 · Cited by 36
Genetic Diseases That Predispose to Early Liver Cirrhosis
Scorza, M., Elce, A., Zarrilli, F., Liguori, R., Amato, F., & Castaldo, G. (2014) · International Journal of Hepatology · doi:10.1155/2014/713754 · Cited by 36
“Carrier and prenatal diagnosis can be offered if the disease-causingmutations have been identified in a family proband [95].”
BackgroundA Clinician’s perspective on clinical exome sequencing
O’Donnell‐Luria, A., & Miller, D. T. (2016) · Human Genetics · doi:10.1007/s00439-016-1662-x · Cited by 36
Beard, C., Amor, D. J., Pietro, L. D., et al. (2016) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.37697 · Cited by 36
“Recent developments in genetic testing technologies enable a move from screening for one genetic condition to screening for multiple conditions at one time point [Bell et al., 2011] providing the opportunity for cost effective and time efficient carrier screening.”
BackgroundThe current and future impact of genome-wide sequencing on fetal precision medicine
Sabbagh, R., & den Veyver, I. B. V. (2019) · Human Genetics · doi:10.1007/s00439-019-02088-4 · Cited by 38
Bailey, J. N., Patterson, C., de Nijs, L., Durón, R. M., Nguyen, V.-H. V., Tanaka, M., Medina, M. T., Jara‐Prado, A., Martínez-Juárez, I. E., Ochoa, A., et al. (2016) · Genetics in Medicine · doi:10.1038/gim.2016.86 · Cited by 33
A comprehensive strategy for exome-based preconception carrier screening
Sallevelt, S. C. E. H., de Koning, B., Szklarczyk, R., Paulussen, A. D. C., de Die-Smulders, C. E. M., & Smeets, H. J. M. (2016) · Genetics in Medicine · doi:10.1038/gim.2016.153 · Cited by 35
Targeted Next-Generation Sequencing for Clinical Diagnosis of 561 Mendelian Diseases
Liu, Y., Wei, X., Kong, X., et al. (2015) · PLoS ONE · doi:10.1371/journal.pone.0133636 · Cited by 35
Phenotyping: Targeting genotype's rich cousin for diagnosis
Baynam, G. S., Walters, M. J., Claes, P. D., Kung, S., Souëf, P. N. L., Dawkins, H. J. S., Bellgard, M., Gîrdea, M., Brudno, M., Robinson, P. N., et al. (2014) · Journal of Paediatrics and Child Health · doi:10.1111/jpc.12705 · Cited by 35
Wooderchak‐Donahue, W. L., O’Fallon, B. D., Furtado, L. V., Durtschi, J., Plant, P., Ridge, P. G., Rope, A. F., Yetman, A. T., & Bayrak‐Toydemir, P. (2012) · BMC Medical Genomics · doi:10.1186/1755-8794-5-50 · Cited by 35
Molster, C. M., Lister, K. J., Metternick-Jones, S. C., Baynam, G. S., Clarke, A. J., Straub, V. W., Dawkins, H. J. S., & Laing, N. G. (2017) · Frontiers in Public Health · doi:10.3389/fpubh.2017.00025 · Cited by 35
“This has been made feasible through advances in genotyping and genetic sequencing technologies, which enable the concurrent evaluation of genetic mutations for large numbers of recessive diseases, for relatively low additional cost (5).”
BackgroundNoninvasive prenatal diagnosis by genome-wide haplotyping of cell-free plasma DNA
Che, H., Villela, D., Dimitriadou, E., Melotte, C., Brison, N., Neofytou, M. C., Bogaert, K. V. D., Tšuiko, O., Devriendt, K., Legius, E., et al. (2020) · Genetics in Medicine · doi:10.1038/s41436-019-0748-y · Cited by 35
The use of expanded carrier screening of gamete donors
Payne, M. R., Skytte, A., & Harper, J. C. (2021) · Human Reproduction · doi:10.1093/humrep/deab067 · Cited by 36
“Data suggest that everyone will carry at least one pathogenic variant that is associated with severe recessive childhood diseases; therefore, the more genes and associated diseases that are tested through ECS, the more carriers will be found (Bell et al. , 2011; Silver et al. , 2016).”
Annotating DNA Variants Is the Next Major Goal for Human Genetics
Cutting, G. R. (2014) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2013.12.008 · Cited by 36
The Genetics of Neuropsychiatric Diseases: Looking In and Beyond the Exome
Heinzen, E. L., Neale, B. M., Traynelis, S. F., Allen, A. S., & Goldstein, D. B. (2015) · Annual Review of Neuroscience · doi:10.1146/annurev-neuro-071714-034136 · Cited by 33
“This disconnect has directly contributed to a heavy burden of false-positive mutations in databases for Mendelian diseases (Bell et al. 2011).”
BackgroundGenomic and transcriptomic approaches to study immunology in cyprinids: What is next?
Petit, J., David, L., Dirks, R. P., & Wiegertjes, G. F. (2017) · Developmental & Comparative Immunology · doi:10.1016/j.dci.2017.02.022 · Cited by 37
“…and new research questions in multiple fields such 498 as ecology, population genetics, quantitative genetics, breeding and immunology could be addressed 499 with higher accuracy (Bell et al., 2011; Davey and Blaxter, 2011; Gjedrem and Robinson, 2014; Peterson 500 et al., 2012; Seeb et al., 2011).”
BackgroundThe Economics of Genomic Medicine
Feero, W. G., Wicklund, C. A. L., & Veenstra, D. L. (2013) · JAMA · doi:10.1001/jama.2013.113 · Cited by 34
Hu, H., Wienker, T. F., Musante, L., Kalscheuer, V. M., Kahrizi, K., Najmabadi, H., & Ropers, H. (2014) · Human Mutation · doi:10.1002/humu.22695 · Cited by 34
“…took MERAP only 17 hr to screen 195 samples for mutations in 520 genes implicated in severe related to recessive childhood disorders birth defects [Bell et al., 2011], and no more than 11 hr to analyze 60 individuals for mutations in a total of 1,222 disease genes, including most genes that have…”
BackgroundTranslational paradigms in pharmacology and drug discovery
Mullane, K., Winquist, R. J., & Williams, M. J. (2013) · Biochemical Pharmacology · doi:10.1016/j.bcp.2013.10.019 · Cited by 34
“ly found to be benign [158].”
Expanded carrier screening: a current survey of physician utilization and attitudes
Briggs, A., Nouri, P. K., Galloway, M., O’Leary, K., Pereira, N., & Lindheim, S. R. (2018) · Journal of Assisted Reproduction and Genetics · doi:10.1007/s10815-018-1272-8 · Cited by 34
New era in genetics of early-onset muscle disease: Breakthroughs and challenges
Ravenscroft, G., Davis, M. R., Lamont, P. J., Forrest, A. R. R., & Laing, N. G. (2016) · Seminars in Cell and Developmental Biology · doi:10.1016/j.semcdb.2016.08.002 · Cited by 31
Li, E. B., Truong, D., Hallett, S. A., et al. (2017) · PLoS Genetics · doi:10.1371/journal.pgen.1007009 · Cited by 33
“One focused resequencing study recently revealed that as many as 27% of previously published disease-causing variants were benign or lacked sufficient evidence for pathogenicity and therefore should be categorized as variants of unknown significance [9].”
BackgroundThiffault, I., Saunders, C., Jenkins, J. L., Raje, N., Canty, K. M., Sharma, M., Grote, L. E., Welsh, H. I., Farrow, E., Twist, G. P., et al. (2015) · BMC Medical Genetics · doi:10.1186/s12881-015-0177-y · Cited by 36
“Trio-exome sequencing was performed on CMH812 and her healthy parents (CMH813 & CMH814) following informed consent, and with methods as previously published [19-22].”
MethodSelf-citationElsayed, S. M., Phillips, J. B., Heller, R., Thoenes, M., Elsobky, E. S., Nürnberg, G., Nürnberg, P., Seland, S., Ebermann, I., Altmüller, J., et al. (2015) · Human Molecular Genetics · doi:10.1093/hmg/ddv022 · Cited by 34
Chokoshvili, D., Vears, D. F., & Borry, P. (2017) · Best Practice & Research Clinical Obstetrics & Gynaecology · doi:10.1016/j.bpobgyn.2017.02.006 · Cited by 33
Evolving minimum standards in responsible international sperm donor offspring quota
Janssens, P. M. W., Thorn, P., Castilla, J. A., et al. (2015) · Reproductive BioMedicine Online · doi:10.1016/j.rbmo.2015.01.018 · Cited by 32
Massively parallel sequencing: the new frontier of hematologic genomics
Johnsen, J. M., Nickerson, D. A., & Reiner, A. P. (2013) · Blood · doi:10.1182/blood-2013-07-460287 · Cited by 34
“Moreover, noninvasive prenatal diagnostic screening by genome sequencing is under way.(83) Detailed functional characterization of the consequences of sequence variation in genetic regulatory elements or protein function at single-nucleotide resolution are now possible through massively parallel reporter assay analysi…”
Drmanac, R. (2012) · Science · doi:10.1126/science.1221037 · Cited by 29
Genomic medicine in the military
Castro, M. J. D., Biesecker, L. G., Turner, C., Brenner, R., Witkop, C., Mehlman, M. J., Bradburne, C. E., & Green, R. C. (2016) · npj Genomic Medicine · doi:10.1038/npjgenmed.2015.8 · Cited by 32
Himes, P., Kauffman, T. L., Muessig, K. R., Amendola, L. M., Berg, J. S., Dorschner, M. O., Gilmore, M. J., Nickerson, D. A., Reiss, J. A., Richards, C. S., et al. (2017) · Genetics in Medicine · doi:10.1038/gim.2016.198 · Cited by 34
Attitudes of European Geneticists Regarding Expanded Carrier Screening
Janssens, S., Chokoshvili, D., Vears, D. F., Paepe, A. D., & Borry, P. (2016) · Journal of Obstetric, Gynecologic & Neonatal Nursing · doi:10.1016/j.jogn.2016.08.012 · Cited by 31
“Because individuals in the general population are estimated to carry several pathogenic mutations associated with severe monogenic disorders (Bell et al., 2011), it is reasonable to expect that any ECS test would identify a large proportion of couples as þ/ for at least one disorder.”
BackgroundInfluentialHanany, M., & Sharon, D. (2019) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2018-105971 · Cited by 31
Lew, R. M., Burnett, L., Proos, A., Barlow-Stewart, K. K., Delatycki, M. B., Bankier, A., Aizenberg, H., Field, M. J., Berman, Y. D., Fleischer, R., et al. (2014) · Journal of Paediatrics and Child Health · doi:10.1111/jpc.12632 · Cited by 31
Genomic intensive care: should we perform genome testing in critically ill newborns?
Wilkinson, D. J. C., Barnett, C., Savulescu, J., et al. (2015) · Archives of Disease in Childhood Fetal & Neonatal · doi:10.1136/archdischild-2015-308568 · Cited by 30
Swanson, A. L., Ramos, E., & Snyder, H. (2014) · Journal of Genetic Counseling · doi:10.1007/s10897-013-9684-1 · Cited by 30
“These panels include testing for disorders ranging from hereditary cardiomyopathies, deafness and cancer syndromes to large pan-ethnic carrier screens (Ambry Genetics 2013; Bell et al. 2011; Lombardi 2013; Smith 2013).”
BackgroundNext-generation sequencing still needs our generation's clinicians
Foley, A. R., Donkervoort, S. C., & Bönnemann, C. G. (2015) · Neurology Genetics · doi:10.1212/nxg.0000000000000019 · Cited by 30
“In fact, with the broad use of NGS, it has become apparent that pathogenicity has been assigned too liberally to variants that appear “damaging” by various algorithms but that are later found to also occur in healthy individuals.(7) Tian et al.”
BackgroundGall, T., Valkanas, E., Bello, C., Markello, T. C., Adams, C., Bone, W. P., Brandt, A. J., Brazill, J. M., Carmichael, L., Davids, M., et al. (2017) · Frontiers in Medicine · doi:10.3389/fmed.2017.00062 · Cited by 33
Bennett, T. D., Callahan, T. J., Feinstein, J. A., Ghosh, D., Lakhani, S. A., Spaeder, M. C., Szefler, S. J., & Kahn, M. G. (2019) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2018.12.041 · Cited by 31
Hussein, N. B., Weng, S. F., Kai, J., Kleijnen, J., & Qureshi, N. (2018) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd010849.pub3 · Cited by 30
The role of high-throughput technologies in clinical cancer genomics
Idris, S. F., Ahmad, S. S., Scott, M. A., Vassiliou, G. S., & Hadfield, J. (2013) · Expert Review of Molecular Diagnostics · doi:10.1586/erm.13.1 · Cited by 30
Population structure of indigenous inhabitants of Arabia
Mineta, K., Goto, K., Gojobori, T., & Alkuraya, F. S. (2021) · PLoS Genetics · doi:10.1371/journal.pgen.1009210 · Cited by 30
Combined linkage analysis and exome sequencing identifies novel genes for familial goiter
Yan, J., Takahashi, T., Ohura, T., Adachi, H., Takahashi, I., Ogawa, E., Okuda, H., Kobayashi, H., Hitomi, T., Liu, W., et al. (2013) · Journal of Human Genetics · doi:10.1038/jhg.2013.20 · Cited by 29
Silver, A. J., Larson, J. L., Silver, M. J., Lim, R. M., Borroto, C., Spurrier, B., Morriss, A., & Silver, L. M. (2016) · Genetic Testing and Molecular Biomarkers · doi:10.1089/gtmb.2016.0014 · Cited by 30
“A variety of experimental approaches have led researchers to the same conclusion: every human being is a carrier of multiple disease-causing mutations, most of which are rare and not clinically defined (Bell et al., 2011; Xue et al., 2012; Simons et al., 2014; Tabor et al., 2014; Henn et al., 2015; Schrodi et al., 201…”
Al-Murshedi, F. M., Meftah, D., & Scott, P. (2018) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2018.04.016 · Cited by 28
Prenatal detection of isolated bilateral hyperechogenic kidneys: Etiologies and outcomes
Shuster, S., Keunen, J., Shannon, P. T., Watkins, N., Chong, K., & Chitayat, D. A. (2019) · Prenatal Diagnosis · doi:10.1002/pd.5418 · Cited by 31
“Missense variants are difficult to classify, and studies have shown they are often mis‐annotated.(17,18) Therefore, current guidelines are conservative as clinical care often includes prenatal family planning decisions that have considerable impact.”
BackgroundSaldarriaga, J. F., López, J. E., Díaz-García, L., & Montoya-Ruíz, C. (2023) · Environmental Science and Pollution Research · doi:10.1007/s11356-023-25501-y · Cited by 27
“Also, deep sequencing of rRNA genes and functional regions has been shown to help in the development of new bioremediation strategies (Bell et al. 2011; Brennerova et al. 2009; Huang et al. 2022; Iwai et al. 2010; Malla et al. 2018).”
BackgroundWhen a "Disease-Causing Mutation" Is Not a Pathogenic Variant
Wang, J., & Shen, Y. (2013) · Clinical Chemistry · doi:10.1373/clinchem.2013.215947 · Cited by 27
Using Drosophila nephrocytes in genetic kidney disease
Helmstädter, M., & Simons, M. (2017) · Cell and Tissue Research · doi:10.1007/s00441-017-2606-z · Cited by 27
“In fact, a survey of 406 published severe disease gene mutations observed in 104 newly sequenced individuals revealed that 27% of these were either without clear pathogenetic evidence or were common polymorphisms (Bell et al. 2011).”
BackgroundLanglois, S., BENN, P. A., & Wilkins-Haug, L. E. (2014) · Prenatal Diagnosis · doi:10.1002/pd.4532 · Cited by 26
Estimating exome genotyping accuracy by comparing to data from large scale sequencing projects
Heinrich, V., Kamphans, T., Stange, J., Parkhomchuk, D., Hecht, J., Dickhaus, T., Robinson, P. N., & Krawitz, P. M. (2013) · Genome Medicine · doi:10.1186/gm473 · Cited by 26
Archibald, A. D., Hickerton, C. L., Wake, S., Jaques, A. M., Cohen, J., & Metcalfe, S. A. (2016) · Journal of Community Genetics · doi:10.1007/s12687-016-0262-8 · Cited by 25
Carrier Testing for Severe Childhood Recessive Diseases by Next-Generation Sequencing
Dungan, J. S. (2012) · Yearbook of Obstetrics Gynecology and Women s Health · doi:10.1016/j.yobg.2012.06.077 · Cited by 25
Nishio, S., & Usami, S. (2016) · Human Mutation · doi:10.1002/humu.23160 · Cited by 25
“Indeed, a not negligible portion of variants classified as “pathogenic variants” in public or commercial disease-specific databases are not truly pathogenic variants [Bell et al., 2011; Shearer et al., 2014; Wang and Shen, 2014], and re-classification of these variants is desired.”
BackgroundSun, Y., Man, J., Wan, Y., Pan, G., Du, L., Li, L., Yang, Y. S., Qiu, L., Gao, Q. C., Dan, H., et al. (2018) · Scientific Reports · doi:10.1038/s41598-018-30151-z · Cited by 25
Biotinidase deficiency: clinical and genetic studies of 38 Brazilian patients
Borsatto, T., Sperb‐Ludwig, F., Pinto, L. L. D. C., et al. (2014) · BMC Medical Genetics · doi:10.1186/s12881-014-0096-3 · Cited by 25
Pre-capture multiplexing improves efficiency and cost-effectiveness of targeted genomic enrichment
Shearer, A. E., Hildebrand, M. S., Ravi, H., et al. (2012) · BMC Genomics · doi:10.1186/1471-2164-13-618 · Cited by 25
Shim, H., Kim, J. H., Kim, C. Y., Hwang, S., Kim, H., Yang, S., Lee, J. E., & Lee, I. (2016) · Nucleic Acids Research · doi:10.1093/nar/gkw897 · Cited by 26
“Multiple large-scale studies have shown that a significant portion of reported diseaseassociated variants are either common polymorphisms (2) or are prevalent in healthy individuals (3,4).”
BackgroundMolecular Technologies Open New Clinical Genetic Vistas
Jackson, L., & Pyeritz, R. E. (2011) · Science Translational Medicine · doi:10.1126/scitranslmed.3002064 · Cited by 23
“Two recent studies published in Science Translational Medicine (Lo et al., 2010; Bell et al., 2011) demonstrate the potential of applying the latest genome-sequencing technologies to preconception carrier testing and noninvasive prenatal genetic diagnosis.”
BackgroundIwata, S., Ito, M., Nakata, T., et al. (2015) · Neuromuscular Disorders · doi:10.1016/j.nmd.2015.05.002 · Cited by 24
“A total of 31 mutations in HSPG2 in SJS have been reported to date: six missense (Table 1) [2,4,17], three nonsense [4,18], twelve splicing [2,4,18–20], and eleven indel mutations [4,19].”
BackgroundJerath, N. U., Mankodi, A. K., Crawford, T. O., Grunseich, C., Baloui, H., Nnamdi‐Emeratom, C., Schindler, A. B., Heiman-Patterson, T. D., Chrast, R., & Shy, M. E. (2017) · Muscle & Nerve · doi:10.1002/mus.25981 · Cited by 24
“fected individuals is required to differentiate diseasecausing mutations from common polymorphisms.(25) Family segregation analysis, clinical phenotyping,”
BackgroundA genome-wide case-only test for the detection of digenic inheritance in human exomes
Kerner, G., Bouaziz, M., Cobat, A., Bigio, B., Timberlake, A. T., Bustamante, J. C., Lifton, R. P., Casanova, J., & Abel, L. (2020) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1920650117 · Cited by 23
Morimoto, M., Nicoli, E., Kuptanon, C., et al. (2023) · Journal of Medical Genetics · doi:10.1136/jmg-2023-109420 · Cited by 23
The changing landscape of carrier screening: expanding technology and options?.
McGowan, M. L., Cho, D., & Sharp, R. R. (2013) · PubMed · Cited by 23
Metadata
- Authors
- Bell, C. J., Dinwiddie, D. L., Miller, N. A., Hateley, S. L., Ganusova, E. E., Mudge, J., Langley, R. J., Zhang, L., Lee, C. C., Schilkey, F. D., Sheth, V., Woodward, J. E., Peckham, H. E., Schroth, G. P., Kim, R. W., & Kingsmore, S. F.
- Affiliations
- National Center for Genome Resources (US)
- Illumina (United States) (US)
- Lifetech Scientific (China) (CN)
- Corresponding author
- Bell, Callum J.
- Dinwiddie, Darrell L.
- Kingsmore, Stephen F.
- ORCID
- Venue
- Science Translational Medicine
- Published
- 2011-01-12
- Volume
- 3
- Issue
- 65
- Pages
- 65ra4-65ra4
- ISSN
- 1946-6234 (ISSN-L)
- 1946-6242
- PMC
- PMC3740116
- OpenAlex
- W2064200890
- Cited by
- 706
- References
- 66
- Citations by year
- 2026: 16 · 2025: 18 · 2024: 28 · 2023: 18 · 2022: 30 · 2021: 46 · 2020: 32 · 2019: 38 · 2018: 45 · 2017: 45 · 2016: 48 · 2015: 78 · 2014: 69 · 2013: 83 · 2012: 60
- FWCI
- 51.97
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Science Translational Medicine (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- UNM’s Digital Repository (University of New Mexico) (submittedVersion)
- Funders
- National Institute of Allergy and Infectious Diseases (U01AI066569)
- National Center for Research Resources (P20RR016480, RR 016480)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (U19HD077693)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genomics and Rare Diseases
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Prenatal Screening and Diagnostics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- carrier screening, recessive genetic diseases, preconception screening, next-generation sequencing, targeted gene panel sequencing, hybridization capture, mutation detection, splicing mutation, large deletions, single nucleotide polymorphisms, mutation database, genetic counseling, Mendelian inheritance
- MeSH terms
- Base Sequence, Child, Female, Genes, Recessive, Genetic Testing, Heterozygote, Genetic Carrier Screening, Humans, Molecular Sequence Data, Mutation, Pregnancy, Prenatal Diagnosis, Genome, Human, Sequence Alignment, Sequence Analysis, DNA, Databases, Genetic
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
A language model drew the claims from the paper on 2026-09-28, under version 3 of the claim definition (prompt extract-v3).
A second model labelled each claim on 2026-09-28 (model jev-1.13-20260917, definition revision gold-guideline-v0.2).
Cited sources are checked with a language model’s help (gpt-6-sol).
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
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