CoChat
Sign Up
Cited by 86Hemoglobinopathies and Related DisordersDevelopmental and Educational NeuropsychologyNeonatal Health and Biochemistry

The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.

Neuropsychological Aspects of Pediatric Sickle Cell Disease

Kral, Mary C., Brown, Ronald T., Hynd, George W.·2001·Neuropsychology Review
DOI 10.1023/a:1012901124088
Open this paper in CoChat — free account

Abstract

Sickle cell disease (SCD), a class of genetic disorders characterized by abnormal, sickled red blood cells, is a chronic illness that results in progressive cerebrovascular disease. Neurocognitive sequelae of clinically apparent cerebrovascular accidents in children with SCD are characterized by pervasive impairments, including decrements in general intellectual functioning, language and verbal abilities, visual-motor and visual-spatial processing, memory, academic achievement, and processing of subtle prosodic information. In contrast, subtle neurocognitive deficits in the areas of attention and concentration, executive function, and visual-motor speed and coordination appear to be associated with silent infarcts that are not necessarily detected on physical examination. Investigation of the disease course and associated neurocognitive sequelae suggest a disease-specific model of neuropsychological impairment. Recommendations are made for clinical and research efforts in the field of pediatric neuropsychology.

Overview

Integrity signals

Registry

Crossref · 10.1023/a:1012901124088

Cites

0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Verification

Claims the paper makes

We couldn’t retrieve this paper’s full text, so its claims and self-check weren’t run. The registry and citation checks still apply.

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?

Citations

Cites

Flagged first, then by citations

Research works printed but not linked: 75

  1. Adams, R. J., Aaslid, R., Gammal, T. E., Nichols, F. T., and McKie, V. (1988a). Detection of cerebral vasculopathy in sickle cell disease using transcranial doppler ultrasonography and magnetic resonance imaging: Case report. Stroke 19: 518–520. · DOI
  2. Adams, R. J., McKie, V. C., Carl, E. M., Nichols, F. T., Perry, R., Brock, K., McKie, K., Figueroa, R., Litaker, M., Weiner, S., and Brambilla, D. (1997). Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Annals of Neurology 42: 699–704. · DOI
  3. Adams, R. J., McKie, V. C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., Abboud, M., Gallagher, D., Kutler, A., Nichols, F. T., Bonds, D. R., and Brambilla, D. (1998). Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. New England Journal of Medicine 339: 5–11. · DOI · Paper page
  4. Adams, R., McKie, V., Nichols, F., Carl, E., Zhang, D.-L., McKie, K., Figueroa, R., Litaker, M., Thompson, W., and Hess, D. (1992). The use of transcranial ultrasonography to predict stroke in sickle cell disease. New England Journal of Medicine 326: 605–610. · DOI
  5. Adams, R. J., Nichols, F. T., Aaslid, R., McKie, V. C., McKie, K., Carl, E., Stephens, S., Thompson, W. O., Milner, P., and Figueroa, R. (1990). Cerebral vessel stenosis in sickle cell disease: Criteria for detection by transcranial Doppler. American Journal of Pediatric Hematology/Oncology 12: 277–282. · DOI
  6. Adams, R. J., Nichols, F. T., Figueroa, R., McKie, V., and Lott, T. (1992). Transcranial doppler correlation with cerebral angiography in sickle cell disease. Stroke 23: 1073–1077. · DOI
  7. Adams, R. J., Nichols, F. T., Stephens, S., Carl, E. M., McKie, V., Fischer, C., and Thompson, W. O. (1988c). Transcranial Doppler: The influence of age and hematocrit in normal children. Journal of Cardiovascular Ultrasound 7: 201–205.
  8. Armstrong, F. D., Thompson, R. J., Wang, W., Zimmerman, R., Pegelow, C. H., Miller, S., Moser, F., Bello, J., Hurtig, A., and Vass, K. (1996). Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease. Pediatrics 97: 864–870. · DOI
  9. Boni, L. A., Brown, R. T., Davis, P. C., Hsu, L., and Hopkins, K. (2001). Social information processing and magnetic resonance imaging in children with sickle cell disease. Journal of Pediatric Psychology 26: 309–319. · DOI
  10. Bonner, M. J., Gustafson, K. E., Schumacher, E., and Thompson, R. J. (1999). The impact of sickle cell disease on cognitive functioning and learning. School Psychology Review 28: 182–193. · DOI
  11. Brown, R. T., Armstrong, F. D., and Eckman, J. R. (1993a). Neurocognitive aspects of pediatric sickle cell disease. Journal of Learning Disabilities 26: 33–45. · DOI
  12. Brown, R. T., Buchanan, I., Doepke, K., Eckman, J. R., Baldwin, K., Goonan, B., and Schoenherr, S. (1993b). Cognitive and academic functioning in children with sickle-cell disease. Journal of Clinical Child Psychology 22: 207–218. · DOI
  13. Brown, R. T., Davis, P. C., Lambert, R., Hsu, L., Hopkins, K., and Eckman, J. (2000). Neurocognitive functioning and magnetic resonance imaging in children with sickle cell disease. Journal of Pediatric Psychology 25: 503–513. · DOI
  14. Brown, R. T., Mulhern, R., and Simonian, S. (in press). Diseases of the blood and blood forming organs. In Boll, T., Bennett-Johnson, S., Perry, N., and Rozensky, R. (eds.), Health and Behavior, vol. III, American Psychological Association, Washington, DC.
  15. Chapar, G. N., Doctors, S. R., Radel, E., and Coupey, S. M. (1986). Minimal neurological deficits in adolescents with sickle cell disease. Journal of Developmental and Behavioral Pediatrics 7: 205. · DOI
  16. Charache, S., Lubin, B., and Reid, C. D. (eds.) (1989). Management and Therapy of Sickle Cell Disease (NIH Publication No. 89–2117), National Institutes of Health, Washington, DC.
  17. Chodorkoff, J., and Whitten, C. F. (1963). Intellectual status of children with sickle cell anemia. Journal of Pediatrics 63: 30–35. · DOI
  18. Cohen, M. J., Branch, W. B., McKie, V. C., and Adams, R. J. (1994). Neuropsychological impairment in children with sickle cell anemia and cerebrovascular accidents. Clinical Pediatrics 33: 517–524. · DOI
  19. Consensus Conference (1987). Newborn screening for sickle cell disease and other hemoglobinopathies. JAMA 258: 1205–1209. · DOI
  20. Craft, S., Schatz, J., Glauser, T. A., Lee, B., and DeBaun, M. R. (1993). Neuropsychologic effects of stroke in children with sickle cell anemia. Journal of Pediatrics 123: 712–717. · DOI
  21. Craft, S., Schatz, J., Glauser, T., Lee, B., and DeBaun, M. (1994). The effects of bifrontal stroke during childhood on visual attention: Evidence from children with sickle cell anemia. Developmental Neuropsychology 10: 285–297. · DOI
  22. Davis, H., Schoendorf, K. C., Gergen, P. J., and Moore, R. M. (1997). National trends in the mortality of children with sickle cell disease, 1968 through 1992. American Journal of Public Health 87: 1317–1322. · DOI
  23. DeBaun, M. R., Schatz, J., Siegel, M. J., Koby, M., Craft, S., Resar, L., Chu, J.-Y., Launius, G., Dadash-Zadeh, M., Lee, R. B., and Noetzel, M. (1998). Cognitive screening examinations for silent cerebral infarcts in sickle cell disease. Neurology 50: 1678–1682. · DOI
  24. Earley, C. J., Kittner, S. J., Feeser, B. R., Gardner, J., Epstein, A., Wozniak, M. A., Wityk, R., Stern, B. J., Price, T. R., Macko, R. F., Johnson, C., Sloan, M. A., and Buchholz, D. (1998). Stroke in children and sickle-cell disease: Baltimore–Washington Cooperative Young Stroke Study. Neurology 51: 169–176. · DOI · Paper page
  25. Embury, S. H., Hebbel, R. P., Mohandas, N., and Steinberg, M. H. (1994). Sickle Cell Disease: Basic Principles and Clinical Practice, Raven Press, New York.
  26. Flick, G. L., and Duncan, C. (1973). Perceptual motor dysfunction in children with sickle cell trait. Perceptual and Motor Skills 36: 234. · DOI
  27. Fowler, M. G., Whitt, J. K., Lallinger, R. R., Nash, K. B., Atkinson, S. S., Wells, R. J., and McMillan, C. (1988). Neuropsychologic and academic functioning of children with sickle cell anemia. Developmental and Behavioral Pediatrics 9: 213–220. · DOI
  28. Gammal, T. E., Adams, R. J., Nichols, F. T., McKie, V., Milner, P., McKie, K., and Brooks, B. S. (1986). MR and CT investigation of cerebrovascular disease in sickle cell patients. American Journal of Neuroradiology 7: 1043–1049.
  29. Gaston, M., Rosse, W. F., and The Cooperative Group (1982). The Cooperative Study of Sickle Cell Disease: Review of study design and objectives. The American Journal of Pediatric Hematology/Oncology 4: 197–201.
  30. Glauser, T. A., Siegel, M. J., Lee, B. C. P., and DeBaun, M. R. (1995). Accuracy of neurologic examination and history in detecting evidence of MRI-diagnosed cerebral infarctions in children with sickle cell hemoglobinopathy. Journal of Child Neurology 10: 88–92. · DOI
  31. Goonan, B. T., Goonan, L. J., Brown, R. T., Buchanan, I., and Eckman, J. R. (1994). Sustained attention and inhibitory control in children with sickle cell syndrome. Archives of Clinical Neuropsychology 9: 89–104. · DOI
  32. Hariman, L. M. D., Griffith, E. R., Hurtig, A. L., and Keehn, M. T. (1991). Functional outcomes of children with sickle-cell disease affected by stroke. Archives of Physical Medicine and Rehabilitation 72: 498–502.
  33. Hindmarsh, P. C., Brozovic, M., Brook, C. G. D., and Davies, S. C. (1987). Incidence of overt and covert neurological damage in children with sickle cell disease. Postgraduate Medical Journal 63: 751–753. · DOI
  34. Hughes, J. G., Diggs, L. W., and Gillespie, C. E. (1940). The involvement of the nervous system in sickle-cell anemia. Journal of Pediatrics 17: 166–184. · DOI
  35. Huttenlocher, P. R., Moohr, J. W., Johns, L., and Brown, F. D. (1984). Cerebral blood flow in sickle cell cerebrovascular disease. Pediatrics 73: 615–621. · DOI
  36. Kaul, D. K., Fabry, M. E., and Nagel, R. L. (1989). Erythrocytic and vascular factors influencing the microcirculatory behavior of blood in sickle cell anemia. Annals of the New York Academy of Sciences 565: 316–326. · DOI
  37. Knight, S., Singhal, A., Thomas, P., and Serjeant, G. (1995). Factors associated with lowered intelligence in homozygous sickle cell disease. Archives of Disease in Childhood 73: 316–320. · DOI
  38. Kogutt, M. S., Goldway, S. S., Gupta, K. L., Kaneko, K., and Humbert, J. R. (1994). Correlation of transcranial Doppler ultrasonography with MRI and MRA in the evaluation of sickle cell disease patients with prior stroke. Pediatric Radiology 24: 204–206. · DOI
  39. Kramer, M. S., Rooks, Y., and Pearson, H. A. (1978). Growth and development in children with sickle-cell trait. New England Journal of Medicine 299: 686–689. · DOI
  40. Kugler, S., Anderson, B., Cross, D., Sharif, Z., Sano, M., Haggerty, R., Prohovnik, I., Hurlet-Jensen, A., Hilal, S., Mohr, J. P., and DeVivo, D. C. (1993). Abnormal cranial magnetic resonance imaging scans in sickle-cell disease: Neurological correlates and clinical implications. Archives of Neurology 50: 629–635. · DOI
  41. McCartney, J. P., Thomas-Lukes, K. M., and Gomez, C. R. (1997). Handbook of Transcranial Doppler, Springer-Verlag, New York. · DOI
  42. Moser, F. G., Miller, S. T., Bello, J. A., Pegelow, C. H., Zimmerman, R. A., Wang, W. C., Ohene-Frempong, K., Schwartz, A., Vichinsky, E. P., Gallagher, D., and Kinney, T. R. (1996). The spectrum of brain MR abnormalities in sickle-cell disease: A report from the Cooperative Study of Sickle Cell Disease. American Journal of Neuroradiology 17: 965–972.
  43. Nagel, R. L. (1994). Origins and dispersion of the sickle cell gene. In Embury, S. H., Hebbel, R. P., Mohandas, N., and Steinberg, M. H. (eds.), Sickle Cell Disease: Basic Principles and Clinical Practice, Raven Press, New York, pp. 353–380.
  44. Ohene-Frempong, K. (1991). Stroke in sickle cell disease: Demographic, clinical, and therapeutic considerations. Seminars in Hematology 28: 213–219.
  45. Ohene-Frempong, K., and Smith-Whitley, K. (1997). Use of hydroxyurea in children with sickle cell disease: What comes next? Seminars in Hematology 34: 30–41.
  46. Ohene-Frempong, K., Weiner, S. J., Sleeper, L. A., Miller, S. T., Embury, S., Moohr, J. W., Wethers, D. L., Pegelow, C. H., Gill, F. M., and the Cooperative Study of Sickle Cell Disease (1998). Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood 91: 288–294.
  47. Partington, M. D., Aronyk, K. E., and Byrd, S. E. (1994). Sickle cell trait and stroke in children. Pediatric Neurosurgery 20: 148–151. · DOI
  48. Pavlakis, S. G., Bello, J., Prohovnik, I., Sutton, M., Ince, C., Mohr, J. P., Piomelli, S., Hilal, S., and DeVivo, D. C. (1988). Brain infarction in sickle cell anemia: Magnetic resonance imaging correlates. Annals of Neurology 23: 125–130. · DOI
  49. Pavlakis, S. G., Prohovnik, I., Piomelli, S., and DeVivo, D. C. (1989). Neurologic complications of sickle cell disease. Advances in Pediatrics 36: 247–276. · DOI
  50. Pegelow, C. H., Adams, R. J., McKie, V., Abboud, M., Berman, B., Miller, S. T., Olivieri, N., Vichinsky, E., Wang, W., and Brambilla, D. (1995). Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. Journal of Pediatrics 126: 896–899. · DOI
  51. Powars, D., Wilson, B., Imbus, C., Pegelow, C., and Allen, J. (1978). The natural history of stroke in sickle cell disease. American Journal of Medicine 65: 461–471. · DOI · Paper page
  52. Prohovnik, I., Sano, M., DeVivo, D., Hurlet, A., Keilp, J., and Piomelli, S. (1995). Frontal lobe dysfunction in sickle-cell anemia. Journal of Cerebral Blood Flow and Metabolism 15: S800. · DOI
  53. Reing, A. B. (1975). Learning and behavioral correlates in learning disabled pupils prone to heterozygous thalassemia and sicklaemia. Journal of Genetic Psychology 127: 305–306. · DOI
  54. Rodgers, G. P., Clark, C. M., Larson, S. M., Rapoport, S. I., Nienhuis, A. W., and Schecter, A. N. (1988). Brain glusose metabolism in neurologically normal patients with sickle cell disease: Regional alterations. Archives of Neurology 45: 78–82. · DOI
  55. Rothman, S. M., Fulling, K. H., and Nelson, J. S. (1986). Sickle cell anemia and central nervous system infarction: A neuropathological study. Annals of Neurology 20: 684–690. · DOI · Paper page
  56. Russell, M. O., Goldberg, H. I., Reis, L., Friedman, S., Slater, R., Reivich, M., and Schwartz, E. (1976). Transfusion therapy for cerebrovascular abnormalities in sickle cell disease. Journal of Pediatrics 88: 382–387. · DOI
  57. Schatz, J., Brown, R. T., Pascual, J. M., Hsu, L., and DeBaun, M. R. (2001). Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease. Neurology 56: 1109–1111. · DOI
  58. Schatz, J., Craft, S., Koby, M., Siegel, M., Resar, L., Lee, R., Chu, J.-Y., Launius, G., Dadash-Zadehm, M., and DeBaun, M. (1999). Neuropsychological deficits in children with sickle cell disease and cerebral infarction: Role of lesion site and location. Child Neuropsychology 5: 92–103. · DOI
  59. Schechter, A. N., and Noguchi, C. T. (1994). Sickle hemoglobin polymer: Structure–function correlates. In Embury, S. H., Hebbel, R. P., Mohandas, N., and Steinberg, M. H. (eds.), Sickle Cell Disease: Basic Principles and Clinical Practice, Raven Press, New York, pp. 33–51.
  60. Seibert, J. J., Glasier, C. M., Kirby, R. S., Allison, J. W., James, C. A., Becton, D. L., Kinder, D. L., Cox, K. S., Flick, E. L., Lairry, F., Jackson, J. F., and Graves, R. A. (1998). Transcranial Doppler, MRA, and MRI as a screening examination for cerebrovascular disease in patients with sickle cell anemia: An 8-year study. Pediatric Radiology 28: 138–142. · DOI
  61. Seibert, J. J., Miller, S. F., Kirby, R. S., Becton, D. L., James C. A., Glasier, C. M., Wilson, A. R., Kinder, D. L., and Berry, D. H. (1993). Cerebrovascular disease in symptomatic and asymptomatic patients with sickle cell anemia: Screening with duplex transcranial Doppler US-Correlation with MR imaging and MR angiography. Neuroradiology 189: 457–466.
  62. Serjeant, G. R. (1992). Sickle Cell Disease, 2nd edn. Oxford University Press, New York.
  63. Siegel, M. J., Luker, G. D., Glauser, T. A., and DeBaun, M. R. (1995). Cerebral infarction in sickle cell disease: Transcranial Doppler US versus neurologic examination. Radiology 197: 191–194. · DOI
  64. Spreen, O., Risser, A. T., and Edgell, D. (1995). Developmental Neuropsychology, Oxford University Press, New York. · DOI
  65. Steen, R. G., Reddick, W. E., Mulhern, R. K., Langston, J. W., Ogg, R. J., Bieberich, A. A., Kingsley, P. B., and Wang, W. C. (1998). Quantitative MRI of the brain in children with sickle cell disease reveals abnormalities unseen by conventional MRI. Journal of Magnetic Resonance Imaging 8: 535–543. · DOI
  66. Steen, R. G., Xiong, X., Mulhern, R. K., Langston, J. W., and Wang, W. C. (1999). Subtle brain abnormalities in children with sickle cell disease: Relationship to blood hematocrit. Annals of Neurology 45: 279–286. · DOI
  67. Stockman, J. A., Nigro, M. A., Mishkin, M. M., and Oski, F. A. (1972). Occlusion of large cerebral vessels in sickle-cell anemia. New England Journal of Medicine 287: 846–849. · DOI
  68. Swift, A. V., Cohen, M. J., Hynd, G. W., Wisenbaker, J. M., McKie, K. M., Makari, G., and McKie, V. C. (1989). Neuropsychologic impairment in children with sickle cell anemia. Pediatrics 84: 1077–1085. · DOI
  69. Verlac, S., Bernaudin, F., Tortrat, D., Brugieres, P., Mage, K., Gaston, A., and Reinert, P. (1995). Detection of cerebrovascular disease in patients with sickle cell disease using transcranial Doppler sonography: Correlation with MRI, MRA and conventional angiography. Pediatric Radiology 25: S14–S19. · DOI
  70. Wang, W. C., Langston, J. W., Steen, R. G., Wynn, L. W., Mulhern, R. K., Wilimas, J. A., Kim, F. M., and Figueroa, R. E. (1998). Abnormalities of the central nervous system in very young children with sickle cell anemia. Journal of Pediatrics 132: 994–998. · DOI
  71. Wasserman, A. L., Wilimas, J. A., Fairclough, D. L., Mulhern, R. K., and Wang, W. (1991). Subtle neuropsychological deficits in children with sickle cell disease. American Journal of Pediatric Hematology/Oncology 13: 14–20. · DOI
  72. Watkins, K. E., Hewes, D. K. M., Connelly, A., Kendall, B. E., Kingsley, D. P. E., Evans. J. E. P., Gadian, D. G., Vargha-Khadem, F., and Kirkham, F. J. (1998). Cognitive deficits associated with frontallobe infarction in children with sickle cell disease. Developmental Medicine and Child Neurology 40: 536–543. · DOI
  73. White, D. A., and DeBaun, M. (1998). Cognitive and behavioral function in children with sickle cell disease: A review and discussion of methodological issues. Journal of Pediatric Hematology/Oncology 20: 458–462. · DOI
  74. Wood, D. H. (1978). Cerebrovascular complications of sickle cell anemia. Stroke 9: 73–75. · DOI
  75. Zimmerman, R. A., Gill, F., Goldberg, H. E., Bilaniuk, L. T., Hackney, D. B., Johnson, M., Grossman, R. I., and HechtLeavitt, C. (1987). MRI of sickle cell cerebral infarction. Neuroradiology 29: 232–237. · DOI

Cited by

Sorted by citations

Metadata

Authors
Kral, M. C., Brown, R. T., & Hynd, G. W.
Affiliations
  • Medical University of South Carolina (US)
  • University of Georgia (US)
Corresponding author
Kral, Mary C.
ORCID
Venue
Neuropsychology Review
Publisher
Springer Science and Business Media LLC
Published
2001-12
Volume
11
Issue
4
Pages
179-196
ISSN
  • 1040-7308 (ISSN-L)
  • 1573-6660
DOI
10.1023/a:1012901124088
OpenAlex
W1502367468
Cited by
86
References
0
Citations by year
2026: 1 · 2025: 6 · 2023: 7 · 2022: 2 · 2021: 3 · 2020: 7 · 2019: 5 · 2018: 3 · 2017: 4 · 2016: 2 · 2015: 4 · 2014: 4 · 2013: 7 · 2012: 5
FWCI
0.52
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Neuropsychology Review (publishedVersion)
  • PubMed (publishedVersion)
  • Digital Scholarship - UNLV (University of Nevada Reno) (submittedVersion)
Topics
  • Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
  • Social Sciences › Psychology › Neuropsychology and Physiological Psychology › Developmental and Educational Neuropsychology
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Neonatal Health and Biochemistry
Keywords
sickle cell disease, pediatric sickle cell disease, neurocognitive impairment, cerebrovascular disease, silent cerebral infarction, intellectual functioning, memory, academic performance, prosodic processing, attention and concentration, executive function, visual motor speed, neuropsychological assessment
MeSH terms
Anemia, Sickle Cell, Brain, Brain Damage, Chronic, Cerebral Infarction, Child, Diagnostic Imaging, Humans, Neuropsychological Tests

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.

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 ›

Are you an author, or do you see a problem with this page? Request a review