Progressive tubulointerstitial renal disease in infancy with associated hepatic abnormalities
| Autor: | William E. Harmon, Levey Rh, Shanley P, Seymour Rosen, Carpenter To, Harris Hw |
|---|---|
| Rok vydání: | 1986 |
| Předmět: |
Male
Pathology medicine.medical_specialty Biopsy medicine.medical_treatment Hepatosplenomegaly Kidney Fibrosis medicine Humans Dialysis Kidney transplantation Hyperparathyroidism business.industry Liver Diseases Hepatobiliary disease Infant General Medicine medicine.disease Kidney Transplantation Liver Kidney Failure Chronic Kidney Diseases medicine.symptom Hepatic fibrosis Enlarged kidney business |
| Zdroj: | The American Journal of Medicine. 81:169-176 |
| ISSN: | 0002-9343 |
| DOI: | 10.1016/0002-9343(86)90205-6 |
| Popis: | Clinical, pathologic, and biochemical data are reported in two male infants who had rapidly progressive renal failure, enlarged kidneys, hepatosplenomegaly, and fat malabsorption. One infant, studied prior to the onset of significant renal insufficiency, manifested renal Fanconi syndrome, hyperparathyroidism, and marked hypocalcemia. After a brief period of dialysis, both received renal transplants. Neither has clinical evidence of reoccurrence of the renal disorder in the transplant, but both still have hepatic abnormalities. Morphologic features present in both patients include a renal lesion characterized by tubulointerstitial injury with a tubulocystic component and hepatic abnormalities with bile duct proliferation, portal fibrosis, and inflammation. These cases do not readily conform to any single published diagnostic category, including nephronophthisis-congenital hepatic fibrosis or infantile polycystic kidney disease, and appear to be unique. |
| Databáze: | OpenAIRE |
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