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Signifance of Liver Ductal Plate Malformation in Differential Diagnosis of Autosomal Recessive Polycystic Kidney Disease: An Autopsy Case

Year 2015, Volume: 40 Number: Supplement 1, 18 - 21, 08.10.2015
https://doi.org/10.17826/cutf.24555

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.

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.

Otozomal Resesif Polikistik Böbrek Hastalığı ile Birlikte Görülen Karaciğer Duktal Alan Malformasyonun Ayırıcı Tanıdaki Önemi: Bir Otopsi Olgusu

Year 2015, Volume: 40 Number: Supplement 1, 18 - 21, 08.10.2015
https://doi.org/10.17826/cutf.24555

Abstract

Kistik böbrek hastalığı, yeni doğan ölümüne sebep olabileceği için uterin dönemde tanınması gereken bir grup hastalığı temsil eder. Ölümcül seyreden otozomal resesif geçişli polikistik böbrek hastalığı oldukça nadir görülen bir hastalık olup, karaciğer lezyonları da bu hastalığa eşlik edebilir. Sunulan otopsi olgusu 31+1 haftalık ölü doğmuş erkek fetus olup yapılan otopside kistik böbrek hastalığı saptanmıştır. Mikroskopik incelemede böbrekte medüller alanlarda görülen değişik boyutlardaki kistlerin yanı sıra karaciğerde portal alanlarda genişleme, duktuslarda uzama ve kıvrılma ile karakterize duktal lezyon görülmüştür. Morfolojik bulgularla olgu otozomal resesif geçişli polikistik böbrek hastalığına eşlik eden duktal alan malformasyonu olarak tanımlanmıştır. Bu hastalık oldukça nadir görülmekle birlikte genellikle yaşamla bağdaşmaz. Ayırıcı tanısında diğer böbrek kistleri yer almaktadır. Bu kistik hastalıkların bir kısmı yetişkin çağa kadar bulgu vermeyen iyi klinik gidiş gösterebilir. Bu olgu agresif klinik gidişi, kendine özgü morfolojik özellikleri ile diğer kistik böbrek hastalıklarından ayrılır.

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.
There are 9 citations in total.

Details

Primary Language English
Journal Section Case Report
Authors

Kıvılcım Erdoğan

Sevil Karabağ This is me

Şeyda Erdoğan This is me

Figen Doran This is me

Publication Date October 8, 2015
Published in Issue Year 2015 Volume: 40 Number: Supplement 1

Cite

MLA Erdoğan, Kıvılcım et al. “Signifance of Liver Ductal Plate Malformation in Differential Diagnosis of Autosomal Recessive Polycystic Kidney Disease: An Autopsy Case”. Cukurova Medical Journal, vol. 40, 2015, pp. 18-21, doi:10.17826/cutf.24555.