title: Focal segmental glomerulosclerosis in desquamative interstitial pneumonia.
abstract: Renal involvement in desquamative interstitial pneumonitis (DIP) manifesting as chronic renal failure has been reported only once. An uncommon disorder in children, DIP has been associated with a variety of systemic disorders and has an immune-mediated pathogenesis. A 16-year-old Black male was diagnosed to have DIP on lung biopsy at the age of 10 months. He was first noted to have proteinuria at age 5 which progressed to nephrotic syndrome by age 13 when the laboratory tests showed elevated IgG, normal serum complement, increased circulating immune complexes and absent anti-GBM antibodies. A percutaneous renal biopsy specimen performed at age 13 revealed focal segmental glomerulosclerosis. Despite prednisone treatment of 2 mg/kg/day for 12 weeks, renal failure progressed requiring hemodialysis. Pulmonary functions, although reduced, remained stable.
authors.affiliation: Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee.
authors.forename: K J
authors.initials: KJ
authors.lastname: Sheth
authors.forename: H E
authors.initials: HE
authors.lastname: Leichter
authors.forename: G
authors.initials: G
authors.lastname: Kishaba
authors.forename: A H
authors.initials: AH
authors.lastname: Cohen
mesh_terms: D000293:Adolescent; D001706:Biopsy; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology; D011658:Pulmonary Fibrosis / Q000150:complications*; D013997:Time Factors