Alagille's syndrome associated with cystic renal disease
| Autor: | Fernando Alvarez, Steven R. Martin, Garel L |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 1996 |
| Předmět: |
Cystic kidney
Male Pathology medicine.medical_specialty Polycystic Kidney Diseases Adolescent business.industry Infant Kidney Diseases Cystic medicine.disease Renal dysplasia Alagille Syndrome Cystic kidney disease Cholestasis Pediatrics Perinatology and Child Health Alagille syndrome Polycystic kidney disease medicine Humans Cyst Differential diagnosis business Research Article Ultrasonography |
| Popis: | Although renal abnormalities have been described in children with Alagille's syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille's syndrome are described, in two of whom a unilateral multicystic dysplastic kidney was detected by prenatal ultrasound; in the other, a solitary cortical cyst was found later in childhood. All have normal renal function, growth, and liver synthetic function but continue to have clinical and biochemical signs of cholestasis. These cases show that unilateral cystic kidney disease with or without renal dysplasia may be associated with Alagille's syndrome, that the clinical course is not necessarily unfavourable, and that Alagille's syndrome should be included in the differential diagnosis of cystic kidney disorders associated with cholestatic liver disease. Patients with Alagille's syndrome should be evaluated by renal ultrasound. |
| Databáze: | OpenAIRE |
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