[Psychosocial intellectual development of children with infantile cystinosis and cerebral atrophy (author's transl)]. 1979

J H Ehrich, and G Wolff, and L Stoeppler, and R Heyer, and G Offner, and J Brodehl

The psychosocial and intellectual development of 4 boys with nephropathic cystinosis and brain atrophy documented by cranial computerized tomography was investigated by use of biographical data and psychological tests (HAWIK, Deutscher Rechtschreibtest). Inspite of the brain atrophy the patients showed low-normal intellectual capacities and mainly average school performance. There were no psychosocial abnormalities correlated to the primary metabolic disease. However, renal dwarfism led to mascotism requiring psychotherapy.

UI MeSH Term Description Entries
D008297 Male Males
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002653 Child Behavior Disorders Disturbances considered to be pathological based on age and stage appropriateness, e.g., conduct disturbances and anaclitic depression. This concept does not include psychoneuroses, psychoses, or personality disorders with fixed patterns. Childhood Behavior Disorders,Behavior Disorder, Child,Behavior Disorder, Childhood,Child Behavior Disorder,Childhood Behavior Disorder,Disorder, Childhood Behavior
D002657 Child Development The continuous sequential physiological and psychological maturing of an individual from birth up to but not including ADOLESCENCE. Infant Development,Development, Child,Development, Infant
D003554 Cystinosis A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME. Cystine Diathesis,Cystine Disease,Cystine Storage Disease,Cystinoses,Cystinosin, Defect of,Cystinosis, Nephropathic,Lysosomal Cystine Transport Protein, Defect Of,Nephropathic Cystinosis,Cystine Diatheses,Cystine Diseases,Cystine Storage Diseases,Cystinoses, Nephropathic,Defect of Cystinosin,Diatheses, Cystine,Diathesis, Cystine,Nephropathic Cystinoses,Storage Disease, Cystine,Storage Diseases, Cystine
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001284 Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Atrophies
D014888 Wechsler Scales Tests designed to measure intellectual functioning in children and adults. National Adult Reading Test,WAIS-R,WISC-IV,WISC-V,WMS-IV-NL,WPPSI,Wechsler Adult Intelligence Scale-Revised,Wechsler Intelligence Scale for Children,Wechsler Intelligence Scales,Wechsler Preschool and Primary Scale of Intelligence,Wechsler Test of Adult Reading,Intelligence Scales, Wechsler

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