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Year : 2011  |  Volume : 21  |  Issue : 2  |  Page : 123-125

Giant cell transformation of podocytes: A unique histological feature associated with cystinosis

1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pediatric Nephrology, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
A K Dinda
Additional Professor, Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-4065.78067

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Fanconi's syndrome is an unusual cause of renal insufficiency in pediatric patients. Infantile cystinosis is one of the identifiable and treatable etiologies of Fanconi's syndrome. Early diagnosis of cystinosis permits institution of specific therapy with cysteamine. A 3-year-old girl presented with failure to thrive, polyuria, and polydipsia. She was found to have renal tubular defect with renal dysfunction and bilateral small contracted kidneys. A renal biopsy revealed extensive giant cell transformation of podocytes in the glomeruli with focal tubular atrophy and dilatation. However, no crystals were identified. Subsequent ophthalmoscopic examination revealed fine cystine crystals in the cornea and a diagnosis of cystinosis causing Fanconi's syndrome was made. Polykaryocytic transformation of visceral epithelial cells is an important diagnostic clue of nephropathic cystinosis and should be carefully looked for in renal biopsy from a child with Fanconi's syndrome and renal insufficiency.


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Indian Journal of Nephrology
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Online since 20th Sept '07