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- W2036542904 abstract "A 4-year-old Caucasian boy was discovered to have proteinuria on routine examination. The protein excretion rate was 43 mg/kg/h (normal <4) and protein–creatinine ratio 2.3 (normal <0.2). There was no history of edema, fever, or infection. Family history was negative for renal disease. Height and weight were at 50th percentile and blood pressure was normal. Urinalysis showed specific gravity 1.025, pH 7, no blood or glucose, protein >300 mg%, no cells, casts, or crystals. Urine calcium–creatinine ratio was 0.03 and tubular reabsorption of phosphate was 95%. Renal biopsy revealed 20 normocellular glomeruli, many with multinucleated podocytes (Figure 1). Immunofluorescence and electron microscopy were unremarkable. Slit-lamp examination revealed symmetric corneal crystals. Peripheral blood polymorphonuclear leukocyte half-cystine level was 1.92 nmol per mg protein (control, 0.12±0.06), confirming the diagnosis of cystinosis. Following 16 months of oral cysteamine and cysteamine drops, the leukocyte cystine level had decreased to 0.34 nmol per mg protein and corneal cystine crystals had decreased. He continues to manifest low-molecular-weight proteinuria (urine protein/creatinine ratio 1.5), without Fanconi syndrome, and normal serum creatinine. Cystinosis is an autosomal recessive lysosomal storage disease caused by deficiency of cystinosin, a lysosomal membrane cystine transporter. This defect leads to crystallization of cystine within lysosomes of many tissues, most notably kidney and the cornea. The renal manifestations of cystinosis include Fanconi syndrome, mild proteinuria, and progressive renal failure. The renal pathological findings of cystinosis include chronic tubulointerstitial nephropathy, multinucleated podocytes, and intracellular crystals (or clefts) within interstitial histiocytes. Multinucleated podocytes have only rarely been described in other glomerular diseases.1.Nagata M. Yamaguchi Y. Komatsu Y. et al.Mitosis and the presence of binucleate cells among glomerular podocytes in diseased human kidneys.Nephron. 1995; 70: 68-71Crossref PubMed Scopus (54) Google Scholar Their pathogenesis is obscure, but possibly involves aberrant nuclear division without cytokinesis in injured podocytes, as has been described in some cases of focal segmental glomerulosclerosis. As illustrated here, multinucleated podocytes should suggest the possibility of nephropathic cystinosis, even in the absence of a history of Fanconi syndrome or chronic tubulo-interstitial nephropathy. Early diagnosis and treatment of cystinosis can prevent complications and maintain renal function, as illustrated by the patient in this report." @default.
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- W2036542904 date "2010-11-01" @default.
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- W2036542904 title "Multinucleated podocytes: a diagnostic clue to cystinosis" @default.
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- W2036542904 doi "https://doi.org/10.1038/ki.2010.341" @default.
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