ALAGILLES SYNDROME-ASSOCIATED WITH CYSTIC RENAL-DISEASE

Although renal abnormalities have been described in children with Alag ille's syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille's synd rome 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 func tion, growth, and Liver synthetic function but continue to have clinic al and biochemical signs of cholestasis. These cases show that unilate ral cystic kidney disease with or without renal dysplasia may be assoc iated with Alagille's syndrome, that the clinical course is not necess arily 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 b e evaluated by renal ultrasound.