Sheth K J, Leichter H E, Kishaba G, Cohen A H
Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee.
Child Nephrol Urol. 1992;12(1):43-6.
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.
仅有一例报告显示，脱屑性间质性肺炎（DIP）累及肾脏时表现为慢性肾衰竭。DIP在儿童中是一种罕见疾病，与多种全身性疾病相关，其发病机制为免疫介导。一名16岁黑人男性在10个月大时经肺活检被诊断为DIP。他5岁时首次被发现有蛋白尿，13岁时发展为肾病综合征，当时实验室检查显示IgG升高、血清补体正常、循环免疫复合物增加且抗肾小球基底膜（GBM）抗体阴性。13岁时进行的经皮肾活检标本显示局灶节段性肾小球硬化。尽管给予泼尼松2mg/（kg·天）治疗12周，但肾衰竭仍进展，需要进行血液透析。肺功能虽有所下降，但保持稳定。
