Signifance of Liver Ductal Plate Malformation in Differential Diagnosis of Autosomal Recessive Polycystic Kidney Disease: An Autopsy Case
Abstract
Cystic renal diseases are a group of disorders that cause neonatal deaths. Autosomal recessive polycystic kidney disease (ARPKD) is a rare entity which may be associated with liver disorders. Presenting autopsy case was stillbirth in 31 + 1 gestational week. In postmortem autopsy,viewed through the capsule, the renal surface was filled by innumerable miniature cysts about 1 mm in diameter. istopathological examination revealed multiple cysts of variable size in the renal medulla and tortuosity, elongation of ductal plate of liver. The autopsy was evaluated as ARPKD and ductal plate malformation. This disorder is not usually compatible with life. Some of the other cystic kidney diseases have better clinical outcome and must be distinguished from ARPKD.
Keywords
Autosomal recessive polycystic kidney disease, ductal plate malformation, autopsy
References
- Potter EL. Normal and Abnormal Development of the Kidney. Chicago: Year Book Medical, 1972.
- Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics. 2003;111:1072–80.
- Srinath A, Shneider BL. Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. J Pediatr Gastroenterol Nutr. 2012;54:580–7.
- Shneider BL, Magid MS. Liver disease in autosomal recessive polycystic kidney disease. Pediatr Transplant. 2005;9:634-9.
- Shorbagi A, Bayraktar Y. Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol. 2010;16:683-90.
- Tahvanainen E, Tahvanainen P, Kaariainen H, Hockerstedt K. Polycystic liver and kidney diseases. Ann Med. 2005;37:546-55.
- Capisonda R, Phan V, Trabuci J, Daneman A, Balfe JW, Guay-Woodford LM. Autosomal recessive polycystic kidney disease: outcomes from a single- center experience. Pediatr Nephrol. 2003;18:119-26.
- Jörgensen MJ. The ductal plate malformation. Acta Pathol Microbiol Scand Suppl. 1977;257:1-88.
- Luoto TT, Pakarinen MP, Jahnukainen T, Jalanko H. Liver disease in autosomal recessive polycystic kidney disease: clinical characteristics and management in relation to renal failure. JPGN. 2014;59:190–196.
