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CASE REPORT
Year : 2011  |  Volume : 15  |  Issue : 4  |  Page : 331-333

Nephrogenic diabetes insipidus with idiopathic Fanconi's syndrome in a child who presented as vitamin D resistant rickets


Department of Pediatrics, Kalawati Saran Children's Hospital and Lady Hardinge Medical College, New Delhi, India

Correspondence Address:
Soumya Patra
Department of Pediatrics, Kalawati Saran Children's Hospital and Lady Hardinge Medical College, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-8210.85596

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Fanconi's syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycaemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium, and magnesium. Whereas diabetes insipidus is a disease of collecting tubules and child mainly presents with dehydration and hypernatremia. Though all the cases published till date were secondary to drugs, myeloma, hematological disorders, etc., we are reporting the first case of idiopathic Fanconi's syndrome along with nephrogenic diabetes insipidus in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi syndrome. We hypothesized that the NDI may be due to of severe hypokalemia induced tubular dysfunction.


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