LATE-ONSET NEPHROPATHIC CYSTINOSIS WITH MASSIVE PROTEINURIA. A STUDY OF THE RENAL LESIONS AND TUBULAR FUNCTIONS
نویسندگان
چکیده
منابع مشابه
Late-onset nephropathic cystinosis: clinical presentation, outcome, and genotyping.
BACKGROUND AND OBJECTIVES Cystinosis is an autosomal recessive disease characterized by the intralysosomal accumulation of cystine, as a result of a defect in cystine transport across the lysosomal membrane. Three clinical forms have been described on the basis of severity of symptoms and age of onset: infantile cystinosis, characterized by renal proximal tubulopathy and progression to end-stag...
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The incidence of atubular glomeruli in nephropathic cystinosis renal biopsies.
Nephropathic cystinosis results from lysosomal cystine storage and, if untreated with cysteamine, results in end-stage renal disease by 10 years of age. The renal Fanconi syndrome occurs in the first year of life and is accompanied by a characteristic "swan neck" deformity of the proximal renal tubule. The linkage between cystine storage, Fanconi syndrome, and renal failure has not been underst...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1974
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-197404000-00716