The DOI matches its Crossref record; 27 of the 32 references it cites were found in a registry or an index, and 4 are not checked yet; 1 of the 34 sources its claims cite were checked for support.
Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients
Open accessAbstract
OBJECTIVES: This study is aimed at identifying discrete severity classes among adults with hypermobile Ehlers-Danlos syndrome (hEDS)/hypermobility spectrum disorders (HSD). METHODS: Subjects were selected according to the old and new nomenclatures and all completed a set of questionnaires exploring pain, fatigue, dysautonomic symptoms, coordination and attention/concentration deficits and quality of life in general. Data were investigated by hierarchical clustering on principal components. Cluster comparisons were then performed by using the two-sample unpaired t test and the standardized mean difference was reported as a measure of effect size. Conditional classification tree analysis and multivariable logistic regression were carried out in order to identify the profiles that were at higher risk to belong to the more severe cluster. Weighted linear combination was used to identify a numerical score measuring this risk. RESULTS: A total of 105 patients were selected and distributed in two distinct severity groups. These groups were statistically separated on the basis of 47 of 59 items/characteristics. One group featured the worse values of most questionnaire items (complex/severe cluster) and the other was dominated by the better values (simplex/milder cluster). Only three items were able to stratify patients according to their risk to belong to the complex cluster. A severity score was then constructed on these three items. CONCLUSION: Adults with hEDS/HSD can be separated in two severity classes, which do not mirror either the old or new criteria for hEDS. The identified severity score could allow a bi-dimensional approach to adults with hEDS/HSD for optimal management planning.
Verified 2026-10-06 · 4 references not checked yet
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26 Not checked yet · 22 Own result · 7 Own method · 6 Own interpretation · 2 No basis given · 1 Partly supported · accuracy not yet measured
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In the paper
“As admitted by most researchers, the presence of musculoskeletal complaints (the unique symptomatic item included in the 2017 criteria, i.e. criterion 2C) and the coexistence of JH-related co-morbidities are probably the leading biological factors affecting QoL in adults with hEDS/HSD [3,4].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Partly supportedcites [3] Tinkle, 2017, Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history, Am J Med Genet C Semin Med Genet sourceExperimental — accuracy not yet measured
The Quality of Life section links musculoskeletal pain and gastrointestinal disorders to poorer QoL, including in adults with hEDS. It does not establish musculoskeletal complaints and comorbidities as the leading biological factors; the Symptomatic Joint Hypermobility section says distress, coping, and behavioral responses may predict impairment and QoL better than pain intensity. The passages do not identify criterion 2C as the unique symptomatic item in the 2017 criteria. Page numbers are unavailable in our copy. (LLM, conf=0.87)
- Not checked yetcites [4] Castori, 2017, A framework for the classification of joint hypermobility and related conditions, Am J Med Genet C Semin Med Genet source
hedgedconditional- Asserted: passes — The authors put it forward as true.
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- Partly supported
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In the paper
“These two clusters did not differ in terms of age and clinical characteristics (BS, prevalence of Villefranche criteria and 2017 criteria), while they showed statistically and clinically significant differences in almost all items from the six questionnaires.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 105- Asserted: passes — The authors put it forward as true.
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In the paper
“The classification tree analysis was carried out by selecting three of the four items that showed a SMD >1.6.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
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In the paper
“The full HCPC strategy identified two sharply separated clusters of patients (Fig. 1).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
boostedn = 105- Asserted: passes — The authors put it forward as true.
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In the paper
“These two clusters did not differ in terms of age and clinical characteristics (BS, prevalence of Villefranche criteria and 2017 criteria), while they showed statistically and clinically significant differences in almost all items from the six questionnaires.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 105- Asserted: passes — The authors put it forward as true.
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In the paper
“By retrospectively applying the 2017 classification on previously gathered data, 58 (55.2%) met the 2017 hEDS, while the others were classified as HSD (44.8%).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 58- Asserted: passes — The authors put it forward as true.
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In the paper
“The analysis had an area under the ROC curve of 0.987 (95% CI 0.973, 1.000).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 1
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“These two clusters did not differ in terms of age and clinical characteristics (BS, prevalence of Villefranche criteria and 2017 criteria), while they showed statistically and clinically significant differences in almost all items from the six questionnaires.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plaincorrelationaln = 105 ·- Asserted: passes — The authors put it forward as true.
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In the paper
“There was also an excess of males in the simplex cluster (Table 1).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“our results confirmed the high impact of pain and fatigue on the QoL of adults with hEDS/HSD”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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In the paper
“failed to identify them as the more sensible factors modulating QoL variability within the phenotypic heterogeneity of hEDS/HSD”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“We also noted an excess of males in the simplex (milder) cluster (Table 1).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Among the 59 items used to separate these two clusters, 47 (79.7%) showed significant differences.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“Among the questionnaires used in this study, COMPASS-31 was the most reliable questionnaire for separating patients into the two identified severity classes.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“The assumption of a clinical continuity between hEDS and HSD emerges from the early observation of a nearly complete overlap between hEDS and joint hypermobility syndrome (JHS), an obsolete term previously used to define aspecific phenotypes of symptomatic/clinically relevant JH [5].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [5] Grahame, 2000, The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS), J Rheumatol
plaincorrelational- Asserted: passes — The authors put it forward as true.
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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
“Adults with hEDS/HSD can be separated in two severity classes, which do not mirror either the old or new criteria for hEDS.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Abstract
plain- Asserted: passes — The authors put it forward as true.
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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
“Adults with hEDS/HSD can be separated in two severity classes, which do not mirror either the old or new criteria for hEDS.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Abstract
plain- Asserted: passes — The authors put it forward as true.
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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 result also supports the concept that the distinction between hEDS and HSD according to the current nosology does not mirror any difference in severity.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
plain- Asserted: passes — The authors put it forward as true.
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None of the sources cited for this claim have been checked yet.
In the paper
“Inclusion of adult patients only did not allow us to explore the impact of the developmental trajectories proposed by others [11,12] on the resulting phenotype.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [11] Pacey, 2015, Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach, Arch Dis Child source
- Not checked yetcites [12] Scheper, 2017, The natural history of children with joint hypermobility syndrome and Ehlers-Danlos hypermobility type: a longitudinal cohort study, Rheumatology (Oxford) source
plain- Asserted: passes — The authors put it forward as true.
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In the paper
“may reflect the natural influence of sexual dimorphism on the eventual phenotype [25].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [25] Castori, 2010, Ehlers-Danlos syndrome hypermobility type and the excess of affected females: possible mechanisms and perspectives, Am J Med Genet A source
hedgedconditional- Asserted: passes — The authors put it forward as true.
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In the paper
“Interestingly, cardiovascular dysautonomia and a range of other functional symptoms commonly reported in hEDS/HSD are also considered typical manifestations of small-fibre neuropathy [31].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [31] Sène, 2018, Small fiber neuropathy: diagnosis, causes, and treatment, Joint Bone Spine source
plaincorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“features, including dyspraxia, learning disorder and attention deficit hyperactivity disorder, were demonstrated to relevantly contribute to QoL in both children and adults with symptomatic JH and hEDS/HSD [6,8].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [6] Morlino, 2019, Italian validation of the functional difficulties questionnaire (FDQ-9) and its correlation with major determinants of quality of life in adults with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder, Am J Med Genet B Neuropsychiatr Genet source
- Not checked yetcites [8] Piedimonte, 2018, Exploring relationships between joint hypermobility and neurodevelopment in children (4–13 years) with hereditary connective tissue disorders and developmental coordination disorder, Am J Med Genet B Neuropsychiatr Genet source
boostedcorrelational- Asserted: passes — The authors put it forward as true.
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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 clustering reasonably reflects a dichotomic distribution of the multidimensional deterioration of QoL, examined by the use of six heterogeneous questionnaires.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
hedgedcorrelational- Asserted: passes — The authors put it forward as true.
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In the paper
“In a single study, small-fibre neuropathy occurred with a very high rate in adults with EDS, including JHS/hEDS [30].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [30] Cazzato, 2016, Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes, Neurology source
boostedcorrelational- Asserted: passes — The authors put it forward as true.
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None of the sources cited for this claim have been checked yet.
In the paper
“Although the first phase is observed in or self-described by most patients, not all individuals seem to have a disease course invariably progressing to the pain and stiffness phases.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [23] Castori, 2010, Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients, Am J Med Genet A source
- Not checked yetcites [24] Castori, 2013, Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type, Am J Med Genet A source
hedged- Asserted: passes — The authors put it forward as true.
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- It may state two separate claims as one. 16
- Probably not a substantial claim. 27
- The quote only partly states this claim. 1
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 the optimal cut-off of 12.7, we observed a sensitivity of 0.94 (95% CI 0.85, 0.98), specificity of 0.95 (95% CI 0.84, 0.99), positive predictive value of 0.97 (95% CI 0.89, 1.00), negative predictive value of 0.91 (95% CI 0.78, 0.97) and an overall accuracy of 0.94 (95% CI 0.88, 0.98) (Fig. 4).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 1
plaincorrelational- Asserted: passes — The authors put it forward as true.
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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
“Sixty-nine individuals met the Villefranche criteria (72.4%) and 36 met the Brighton criteria (27.6%).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 105- Asserted: passes — The authors put it forward as true.
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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
“Accordingly, the COMPASS-31 total score, BPI current intensity (of pain) and SF-36 physical fatigue were considered the more discriminating markers to predict inclusion in the complex (severe) cluster (Fig. 2).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plaincorrelational- Asserted: passes — The authors put it forward as true.
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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
“Sixty-three (60.0%) patients were classified as complex (i.e.severe) and 42 (40.0%)were classified as simplex (i.e.milder).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 105- Asserted: passes — The authors put it forward as true.
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In the paper
“A total of 105 patients were selected and distributed in two distinct severity groups.”
- Also stated in: § Results · plain
“A total of 105 patients were enrolled in this study.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Abstract
plainn = 105- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim.
- Also stated in: § Results · 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
“PCA analysis was performed on 48 continuous items (after scaling data to unit variance) from the six administrated questionnaires.Three main components accounting for 50% of the total amount of variability were identified.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
plainn = 105- Asserted: passes — The authors put it forward as true.
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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
“More specifically, the worse and better values from the different questionnaires aggregated together in cluster one (complex or severe) and cluster two (simplex or milder), respectively.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
plaincorrelational- Asserted: passes — The authors put it forward as true.
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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 work identified two distinct classes of adults with hEDS/EDS.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
plain- Asserted: passes — The authors put it forward as true.
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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
“These two groups were clearly distinguishable by the values of all items of the COMPASS-31, SF-36 and ASRS-V1.1,andmost items of the BPI, MFI-20 and FDQ-9 AUC: area under the curve.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
boostedcorrelational- Asserted: passes — The authors put it forward as true.
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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
“Conversely, no significant differences were noted for the clinical diagnosis according to the old and new nomenclatures (i.e.no excess of hEDS 2017 and Villefranche criteria in the two clusters) and for age.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
plain- Asserted: passes — The authors put it forward as true.
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It may state two separate claims as one.
In the paper
“Accordingly, the recent literature exploring the clinical ramifications of hEDS and HSD groups together these conditions in a single phenotype termed hEDS/HSD [69].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [69]
plain- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim.
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
“To minimize subjectivity in choosing a cluster solution, we serially performed hierarchical clustering followed by k-means portion clustering upon the regression-based factors directly derived from PCA as follows.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Statistical analysis
plaindirect- Asserted: passes — The authors put it forward as true.
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Probably not a substantial claim.
In the paper
“The 2017 classification also placed an emphasis on the various JH-related co-morbidities as the most likely determinants of quality of life (QoL) in hEDS/HSD [4].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [4] Castori, 2017, A framework for the classification of joint hypermobility and related conditions, Am J Med Genet C Semin Med Genet source
hedgeddirect- Asserted: passes — The authors put it forward as true.
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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
“Symptomatology and QoL were screened by using a set of validated questionnaires selected to explore a wide array of multisystemic involvement.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Patient selection and assessment
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.
In the paper
“For this reason, a continuous spectrum of phenotypes is proposed ranging from asymptomatic, non-syndromic JH to hEDS and passing through HSD [4].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [4] Castori, 2017, A framework for the classification of joint hypermobility and related conditions, Am J Med Genet C Semin Med Genet 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.
Probably not a substantial claim.
In the paper
“In contrast, the new hEDS criteria were conceived to simplistically recognize patients with more convincing structural systemic involvement and/or Mendelian transmission within a wider spectrum of JHrelated phenotypes [10].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [10] Castori, 2017, Contemporary approach to joint hypermobility and related disorders, Curr Opin Pediatr source
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.
In the paper
“To date, the underlying genetic defect is demonstrable in all EDS variants except hypermobile EDS (hEDS), which lacks a confirmatory laboratory test.Hence the diagnosis of hEDS remains based on physical examination, personal history and formal exclusion of other, partially overlapping disorders [3].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [3] Tinkle, 2017, Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history, Am J Med Genet C Semin Med Genet source
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
“Attention was focussed on joints included in the Beighton score (BS), which is, at the moment, considered the best tool for the assessment of generalized JH [16] (supplementary material, section Methods, available at Rheumatology online).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Patient selection and assessment
What the sources say
- Not checked yetcites [16] Juul-Kristensen, 2017, Measurement properties of clinical assessment methods for classifying generalized joint hypermobility—a systematic review, Am J Med Genet C Semin Med Genet source
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.
In the paper
“Fatigue, cardiovascular dysautonomia and functional gastrointestinal disorders are among the leading features that, although not included in the diagnostic criteria, affect the health status of adults with hEDS/HSD [2022].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [2022]
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.
In the paper
“An exploratory study on 89 JHS children identified five subphenotypes, namely joint affected, athletic, systemic, soft tissue affected and high BMI [11].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [11] Pacey, 2015, Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach, Arch Dis Child source
plainn = 89- 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.
None of the sources cited for this claim have been checked yet.
In the paper
“Heterogeneity in terms of disease expression was put forward years ago by our group, when we approached the natural history of what was previously called JHS/hEDS [23,24].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [23] Castori, 2010, Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients, Am J Med Genet A source
- Not checked yetcites [24] Castori, 2013, Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type, Am J Med Genet A source
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.
In the paper
“These categories were recognized by the prevalence of selected items, such as BMI by age and number of painful joints.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [11] Pacey, 2015, Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach, Arch Dis Child source
plaincorrelationaln = 89- 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.
In the paper
“the difference in disease expression between sexes is well known in hEDS/HSD”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [25] Castori, 2010, Ehlers-Danlos syndrome hypermobility type and the excess of affected females: possible mechanisms and perspectives, Am J Med Genet A source
boostedcorrelational- 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.
In the paper
“In this work, they found that JHS children with a higher incidence of multisystemic involvement and poor postural control more commonly present a deteriorating trajectory with increased functional impairment [12].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [12] Scheper, 2017, The natural history of children with joint hypermobility syndrome and Ehlers-Danlos hypermobility type: a longitudinal cohort study, Rheumatology (Oxford) source
plaincorrelationaln = 81- 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.
In the paper
“The same group also traced the trajectories of functional impairment of a similar cohort of 81 patients in a subsequent 3 year longitudinal study [12].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [12] Scheper, 2017, The natural history of children with joint hypermobility syndrome and Ehlers-Danlos hypermobility type: a longitudinal cohort study, Rheumatology (Oxford) source
plainn = 81- 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
“Accordingly, the 2017 classification of EDS and related disorders admits the coexistence of relatively healthy individuals respecting the hEDS criteria and severely affected individuals with a diagnosis of HSD.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > 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.
None of the sources cited for this claim have been checked yet.
In the paper
“At that time, three possible different phases of disease progression were proposed: hypermobility (first phase), pain (second phase) and stiffness (third phase).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [23] Castori, 2010, Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients, Am J Med Genet A source
- Not checked yetcites [24] Castori, 2013, Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type, Am J Med Genet A source
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.
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
“Although the relatively small number of males may have negatively influenced the statistical power of the study”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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.
None of the sources cited for this claim have been checked yet.
In the paper
“More recently, cardiovascular autonomic abnormalities have been characterized in JHS/ hEDS with a variety of profiles, including postural orthostatic tachycardia syndrome, orthostatic intolerance and neuromediated hypotension [28,29].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [28] De Wandele, 2016, Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome, Rheumatology (Oxford) source
- Not checked yetcites [29] Celletti, 2017, Orthostatic intolerance and postural orthostatic tachycardia syndrome in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type: neurovegetative dysregulation or autonomic failure?, Biomed Res Int 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.
Probably not a substantial claim.
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
“We limited our investigation of the various QoL determinants to pain, fatigue, neurodevelopmental attributes and dysautonomic-related symptoms.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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
“Manifestations of psychological distress were not specifically included and therefore were limited to selected SF-36 items (e.g.psycho-emotional health).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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
“We already demonstrated that a positive FDQ-9 score is associated with different features affecting QoL in adults with hEDS/HSD [6].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Discussion
What the sources say
- Not checked yetcites [6] Morlino, 2019, Italian validation of the functional difficulties questionnaire (FDQ-9) and its correlation with major determinants of quality of life in adults with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder, Am J Med Genet B Neuropsychiatr Genet source
boostedcorrelational- 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
“The number of included subjects might affect the power of the study, whose results remain preliminary and considering the presumed rarity of the investigated phenotype.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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.
Probably not a substantial claim. It may state two separate claims as one.
- The authors state this as an assumption: The investigated phenotype is rare.
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
“Finally, this study was carried out on Italian patients and therefore we do not know if item values will distribute similarly in other populations.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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
“In other words, patient stratification by severity does not seem determined by the primary features of hEDS/HSD (e.g.pain), but rather by a presumably coexistent additional pathomechanism.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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.
In the paper
“Dysautonomia has been evoked as a clinically relevant manifestation of JHS [27].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [27] Hakim, 2004, Non-musculoskeletal symptoms in joint hypermobility syndrome. Indirect evidence for autonomic dysfunction?, Rheumatology (Oxford) source
hedgedcorrelational- 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.
In the paper
“Some correlations with selected QoL determinants also emerged, as patients with the high BMI subphenotype experienced more pain while those with the soft tissue affected subphenotype reported more fatigue [11].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Introduction
What the sources say
- Not checked yetcites [11] Pacey, 2015, Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach, Arch Dis Child source
plaincorrelationaln = 89- 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
“This is somewhat in contrast with the consolidated evidence that pain and fatigue are likely the most relevant determinants of QoL in EDS [26].”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
What the sources say
- Not checked yetcites [26] Voermans, 2011, Both pain and fatigue are important possible determinants of disability in patients with the Ehlers-Danlos syndrome hypermobility type, Disabil Rehabil source
hedgedcorrelational- 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
“This might suggest that the severity of hEDS/HSD is not significantly influenced by the 2017 criteria of hEDS and the aging process.”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
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.
The paper relies on this without support.
No similar study has been conducted in adults with hEDS/HSD, and existing data have not been explored to subclassify patients in clinical settings.
“No similar study has been carried out in adults with hEDS/HSD and accumulated data have not yet been explored with the aim to subclassify patients in a clinical setting.”
The authors state this as an assumption.
The 2017 hEDS criteria are useful for distinguishing patients with systemic connective tissue disease (structural anomalies) and/or families with Mendelian transmission within the spectrum.
“In the present study, we assumed that the 2017 hEDS criteria are useful to distinguish patients with a systemic connective tissue disease (in terms of structural anomalies) and/or families with Mendelian transmission of the disease within the spectrum, but they are probably inadequate to separate patients by severity, and hence medical needs.”
The authors state this as an assumption.
The 2017 hEDS criteria are probably inadequate for separating patients by severity and medical needs.
“In the present study, we assumed that the 2017 hEDS criteria are useful to distinguish patients with a systemic connective tissue disease (in terms of structural anomalies) and/or families with Mendelian transmission of the disease within the spectrum, but they are probably inadequate to separate patients by severity, and hence medical needs.”
The paper relies on this without support.
The hypermobility phase of JHS/hEDS is typically observed among children and adolescents.
“In line with the concept that the hypermobility phase is typically observed among children and adolescents”
The authors state this as an assumption.
The investigated phenotype is rare.
“considering the presumed rarity of the investigated phenotype”
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“Until now, no validated biological biomarkers have been identified for recognizing hEDS/HSD, which are dominated by extremely variable phenotypes and chronic disability affecting patients’ quality of life [111,116].”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [116]
In the citing paper
“Chronic pain represents a common complaint among hEDS/HSD patients affecting their quality of life [111,116], though specific molecular pathways or mediators of pain are still unknown.”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [116]
In the citing paper
“Unfortunately in recent cohort publications [16, 17], these new criteria seemed to not adequately identify the more severely affected patients”
From Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? (2021) · cites this paper as [17]
In the citing paper
“Reassuringly, the proportion of women [16, 17], mean age, prevalence of pain, and fatigue are similar to that in prior studies [16, 17, 40].”
From Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? (2021) · cites this paper as [17]
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
“Sixty-nine individuals met the Villefranche criteria (72.4%) and 36 met the Brighton criteria (27.6%).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
Reported 27.6 · recomputed 34.285714
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
“Sixty-nine individuals met the Villefranche criteria (72.4%) and 36 met the Brighton criteria (27.6%).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
Reported 72.4 · recomputed 65.714286
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
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > FIG. 5
“Translation into practice of the severity score might allow to place the patient in a low-intensity or high-intensity management programme according to the cluster (either complex/milder or complex/severe).”
§ Severity classes in adults with hypermobile EhlersDanlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients > Results
“Sixty-three (60.0%) patients were classified as complex (i.e.severe) and 42 (40.0%)were classified as simplex (i.e.milder).”
Citations
Cites
Unbiased Recursive Partitioning: A Conditional Inference Framework
Hothorn, T., Hornik, K., & Zeileis, A. (2006) · Journal of Computational and Graphical Statistics · doi:10.1198/106186006x133933 · Cited by 4,021
Strobl, C., Malley, J., & Tutz, G. (2009) · Psychological Methods · doi:10.1037/a0016973 · Cited by 2,583
The revised Ghent nosology for the Marfan syndrome: Table 1
Loeys, B., Dietz, H. C., Braverman, A. C., Callewaert, B. L., Backer, J. F. A. D., Devereux, R. B., Hilhorst‐Hofstee, Y., Jondeau, G., Faivre, L. O., Milewicz, D. M., et al. (2010) · Journal of Medical Genetics · doi:10.1136/jmg.2009.072785 · Cited by 2,245
The 2017 international classification of the Ehlers–Danlos syndromes
Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L., Bowen, J. M., Brady, A. F., Burrows, N. P., et al. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31552 · Cited by 1,936
Self-citationExploratory Multivariate Analysis by Example Using R
Husson, F., Lê, S., & Pagès, J. (2017) · Chapman & Hall/CRC eBooks · doi:10.1201/b21874 · Cited by 762
(2000) · PubMed · Cited by 738
A framework for the classification of joint hypermobility and related conditions
Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31539 · Cited by 602
Self-citationTinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31538 · Cited by 499
Self-citationJuul‐Kristensen, B., Schmedling, K., Rombaut, L., Lund, H., & Engelbert, R. H. H. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31540 · Cited by 229
Castori, M., Camerota, F., Celletti, C., Danese, C., Santilli, V., Saraceni, V. M., & Grammatico, P. (2010) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.33231 · Cited by 228
Self-citation
Cited by
Sorted by citations
Gensemer, C., Burks, R., Kautz, S. A., Judge, D. P., Lavallee, M. E., & Norris, R. A. (2020) · Developmental Dynamics · doi:10.1002/dvdy.220 · Cited by 163
The Relationship between Autism and Ehlers-Danlos Syndromes/Hypermobility Spectrum Disorders
Casanova, E. L., Baeza-Velasco, C., Buchanan, C. B., & Casanova, M. F. (2020) · Journal of Personalized Medicine · doi:10.3390/jpm10040260 · Cited by 109
Chiarelli, N., Ritelli, M., Zoppi, N., & Colombi, M. (2019) · Genes · doi:10.3390/genes10080609 · Cited by 82
Self-citationLam, C. Y., Palsson, O. S., Whitehead, W. E., Sperber, A. D., Tornblom, H., Simren, M., & Aziz, I. (2021) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2020.02.034 · Cited by 71
Aubry-Rozier, B., Schwitzguebel, A., Valerio, F., Tanniger, J., Paquier, C., Berna, C., Hügle, T., & Benaim, C. (2021) · Rheumatology International · doi:10.1007/s00296-021-04968-3 · Cited by 66
Metadata
- Authors
- Copetti, M., Morlino, S., Colombi, M., Grammatico, P., Fontana, A., & Castori, M.
- Affiliations
- Casa Sollievo della Sofferenza (IT)
- Azienda Ospedaliera San Camillo-Forlanini (IT)
- University of Brescia (IT)
- Corresponding author
- Castori, Marco
- ORCID
- Venue
- Rheumatology
- Publisher
- Oxford University Press (OUP)
- Published
- 2019-02-19
- Volume
- 58
- Issue
- 10
- Pages
- 1722-1730
- ISSN
- 1462-0324 (ISSN-L)
- 1462-0332
- OpenAlex
- W2916898328
- Cited by
- 55
- References
- 32
- Citations by year
- 2026: 3 · 2025: 6 · 2024: 6 · 2023: 8 · 2022: 7 · 2021: 11 · 2020: 9 · 2019: 4
- FWCI
- 4.67
- Type
- journal article
- Language
- en
- Open access
- green
- academic.oup.com
- Locations
- Lara D. Veeken (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion)
- Funders
- Ministero della Salute
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Health Professions › Physical Therapy, Sports Therapy and Rehabilitation › Musculoskeletal pain and rehabilitation
- Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
- Keywords
- hypermobile Ehlers-Danlos syndrome, hypermobility spectrum disorders, disease severity classification, severity scoring, pain, fatigue, coordination problems, quality of life
- MeSH terms
- Adult, Ehlers-Danlos Syndrome, Fatigue, Female, Humans, Italy, Joint Instability, Male, Middle Aged, Pilot Projects, Quality of Life, Surveys and Questionnaires, Severity of Illness Index, Syndrome, Linear Models, Arthralgia, Primary Dysautonomias
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 2026-10-06, under version 3 of the claim definition (model deepseek-v4.1-flash, prompt extract-v3).
A second model labelled each claim on 2026-10-06 (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.
What counts as a claim ›How we measure confidence ›How we check this ›
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