Neurocognitive functions and behavioral profiles in children with nephropathic cystinosis
المؤلفون المشاركون
Ali, Riham
Makar, Samuel
al-Bakr, Azizi
Sulayman, Nifin A.
المصدر
Saudi Journal of Kidney Diseases and Transplantation
العدد
المجلد 25، العدد 6 (31 ديسمبر/كانون الأول 2014)، ص ص. 1224-1231، 8ص.
الناشر
تاريخ النشر
2014-12-31
دولة النشر
السعودية
عدد الصفحات
8
التخصصات الرئيسية
الموضوعات
الملخص EN
Children with nephropathic cystinosis (NCTN) have evidence of defective intellectual functions and behavioral disorders.
This prospective study was performed to detect the cognitive dysfunctions in patients with this rare hereditary lysosomal storage disease, define their behavioral phenotypes, and study the findings on magnetic resonance imaging (MRI) of the brain.
Thirteen patients with confirmed diagnosis of cystinosis (mean age ± SD 5.9 ± 3.0, range 1.5 – 12 years) were subjected to the Stanford Binet test, Porteus Maze test, Child Behavior Checklist, and MRI brain.
Thirteen age- and sex-matched children served as the control subjects (mean age ± SD 5.9 ± 2.9, range 1.7 – 12 years).
The intelligence quotient (IQ) was significantly lower in patients with cystinosis (P < 0.001), with a significant defect in verbal (language, memory, and comprehension) and non-verbal abilities (visual perception and visiospatial and motor performance).
A discrepancy between both abilities was detected— the non-verbal ability being lower; however, it did not reach statistical significance.
Furthermore, analysis revealed the visiospatial ability to be significantly lower compared to the visual perception.
In comparison to healthy controls, children with NCTN had evidence of increased incidence of behavioral problems, mainly social (P = 0.023).
An MRI of the brain revealed varying degrees of atrophic changes in seven patients.
Patients with NCTN need a wider scope of attention and care, encompassing not only the metabolic multisystem derangement, but also the neuropsychological impairment in the context of multidisciplinary management.
This approach is crucial in formulating comprehensive plans for social and educational rehabilitation.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Ali, Riham& Makar, Samuel& al-Bakr, Azizi& Sulayman, Nifin A.. 2014. Neurocognitive functions and behavioral profiles in children with nephropathic cystinosis. Saudi Journal of Kidney Diseases and Transplantation،Vol. 25, no. 6, pp.1224-1231.
https://search.emarefa.net/detail/BIM-431438
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Ali, Riham…[et al.]. Neurocognitive functions and behavioral profiles in children with nephropathic cystinosis. Saudi Journal of Kidney Diseases and Transplantation Vol. 25, no. 6 (Dec. 2014), pp.1224-1231.
https://search.emarefa.net/detail/BIM-431438
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Ali, Riham& Makar, Samuel& al-Bakr, Azizi& Sulayman, Nifin A.. Neurocognitive functions and behavioral profiles in children with nephropathic cystinosis. Saudi Journal of Kidney Diseases and Transplantation. 2014. Vol. 25, no. 6, pp.1224-1231.
https://search.emarefa.net/detail/BIM-431438
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references : p. 1230-1231
رقم السجل
BIM-431438
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر