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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2014  |  Volume : 25  |  Issue : 4  |  Page : 840-843
Caroli's syndrome with autosomal recessive polycystic kidney disease


Division of Pediatric Nephrology, Department of Pediatrics, Lokmanya Tilak Municipal General Hospital and Medical College, Sion, Mumbai, India

Correspondence Address:
Prithi Shenoy
Division of Pediatric Nephrology, Department of Pediatrics, Lokmanya Tilak Municipal General Hospital and Medical College, Sion, Mumbai - 400 022
India
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DOI: 10.4103/1319-2442.135176

PMID: 24969198

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Caroli's syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management.


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