Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure
Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affecte...
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| Format: | Article |
| Language: | English |
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Mashhad University of Medical Sciences
2014-04-01
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| Series: | International Journal of Pediatrics |
| Subjects: | |
| Online Access: | http://ijp.mums.ac.ir/pdf_2494_6248f4ac9a539275966ddb2ac651815a.html |
| _version_ | 1798034285119143936 |
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| author | A Azarfar MA Kiani Ag Keykhosravi Y Ravanshad |
| author_facet | A Azarfar MA Kiani Ag Keykhosravi Y Ravanshad |
| author_sort | A Azarfar |
| collection | DOAJ |
| description | Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an autosomal recessive polycystic kidney disease (ARPKD). Impaired renal function associated with CHF in adults is caused by an autosomal dominant polycystic kidney disease (ADPKD). Case presentation: We report the case of a 8-year-old Iranian girlwas admitted to our hospital for evaluation ofrenal failure. In patient hepatomegaly was noted incidentally on a routine physical examination and then kidney biopsy showed global sclerosis and A liver biopsy revealed proliferation of collagen fibres surrounding the portal area, a finding that was compatible with congenital hepatic fibrosisand our patient was scheduled for kidney and liver transplantation. Conclusion: The relationship of ARPKD to CHF is the subject of substantial controversy. Some clinicians suggest that the two conditions represent one disorder with a range of clinical/pathological presentations Key word: Congenital Hepatic Fibrosis Polycystic Kidney Disease, CRF. |
| first_indexed | 2024-04-11T20:41:17Z |
| format | Article |
| id | doaj.art-fb1b0b4d23ee4ecba47e13f7df922e58 |
| institution | Directory Open Access Journal |
| issn | 2345-5047 2345-5055 |
| language | English |
| last_indexed | 2024-04-11T20:41:17Z |
| publishDate | 2014-04-01 |
| publisher | Mashhad University of Medical Sciences |
| record_format | Article |
| series | International Journal of Pediatrics |
| spelling | doaj.art-fb1b0b4d23ee4ecba47e13f7df922e582022-12-22T04:04:12ZengMashhad University of Medical SciencesInternational Journal of Pediatrics2345-50472345-50552014-04-0122.140402494Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal FailureA Azarfar0MA Kiani1Ag Keykhosravi2Y Ravanshad3Assistant professor of Pediatric Nephrology, Mashhad University of Medical Sciences, Mashhad, Iran.Associate professor of Pediatric Gastroentrology, Mashhad University of Medical Sciences, Mashhad, Iran.Associate professor of Pediatric Nephrology, Mashhad University of Medical Sciences, Mashhad, Iran.4Mashhad University of Medical Sciences, Mashhad, Iran.Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an autosomal recessive polycystic kidney disease (ARPKD). Impaired renal function associated with CHF in adults is caused by an autosomal dominant polycystic kidney disease (ADPKD). Case presentation: We report the case of a 8-year-old Iranian girlwas admitted to our hospital for evaluation ofrenal failure. In patient hepatomegaly was noted incidentally on a routine physical examination and then kidney biopsy showed global sclerosis and A liver biopsy revealed proliferation of collagen fibres surrounding the portal area, a finding that was compatible with congenital hepatic fibrosisand our patient was scheduled for kidney and liver transplantation. Conclusion: The relationship of ARPKD to CHF is the subject of substantial controversy. Some clinicians suggest that the two conditions represent one disorder with a range of clinical/pathological presentations Key word: Congenital Hepatic Fibrosis Polycystic Kidney Disease, CRF.http://ijp.mums.ac.ir/pdf_2494_6248f4ac9a539275966ddb2ac651815a.htmlPoster PresentationN 21 |
| spellingShingle | A Azarfar MA Kiani Ag Keykhosravi Y Ravanshad Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure International Journal of Pediatrics Poster Presentation N 21 |
| title | Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure |
| title_full | Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure |
| title_fullStr | Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure |
| title_full_unstemmed | Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure |
| title_short | Congenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure |
| title_sort | congenital hepatic fibrosis an uncommon cause of chronic renal failure |
| topic | Poster Presentation N 21 |
| url | http://ijp.mums.ac.ir/pdf_2494_6248f4ac9a539275966ddb2ac651815a.html |
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