Congenital hepatic fibrosis (a case report)

Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder of intrauterine morphogenesis of the portobiliary system, characterized by periportal fibrosis and defective remodeling of the bile ducts. The article describes the features of the clinical course, diagnosis and treatment of type I...

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Autores principales:	O. O. Popovych, L. V. Moroz, Yu. V. Avdosiev, F. A. Chabanov, O. O. Voinalovych
Formato:	article
Lenguaje:	EN RU UK
Publicado:	Zaporozhye State Medical University 2021
Materias:	congenital hepatic fibrosis pregnancy transient elastography splenic artery embolization Medicine R
Acceso en línea:	https://doaj.org/article/12ba9a77486040aa9288a9e246c40b4e
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spelling	oai:doaj.org-article:12ba9a77486040aa9288a9e246c40b4e2021-11-11T07:04:37ZCongenital hepatic fibrosis (a case report)10.14739/2310-1210.2021.6.2291022306-41452310-1210https://doaj.org/article/12ba9a77486040aa9288a9e246c40b4e2021-11-01T00:00:00Zhttp://zmj.zsmu.edu.ua/article/view/229102/241672https://doaj.org/toc/2306-4145https://doaj.org/toc/2310-1210Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder of intrauterine morphogenesis of the portobiliary system, characterized by periportal fibrosis and defective remodeling of the bile ducts. The article describes the features of the clinical course, diagnosis and treatment of type I CHF in the different terms of a patient’s life: from birth to her pregnancy, during pregnancy and after childbirth. The aim of this work is to familiarize specialists with the problem of diagnosis and treatment of type I CHF in a patient with a provisional misdiagnosis of cryptogenic liver cirrhosis. Results. The features of type I CHF that determine a wider diagnostic testing in long-term unspecified liver pathology cases were identified. First-time decompensation of portal hypertension (PH) occurred at 35 weeks of gestation with the development of ascitic-edematous syndrome, hypersplenism, preeclampsia without signs of hepatic encephalopathy (HE). Cesarean section was performed at 37 weeks’ gestation and healthy baby was born. One year and 9 months after delivery, hypersplenism compensation was achieved by performing splenic artery embolization (SAE). There were no clinical manifestations of HE throughout the follow-up period. Conclusions. The diagnostic algorithm for a long-term compensated unspecified liver pathology with PH should confirm or rule out CHF. Type I CHF patients can give birth to a healthy child. SAE successfully compensates for hypersplenism and PH in type I CHF. Transient elastography is an effective method for the assessment of liver fibrosis progression in patients with type I CHF. O. O. PopovychL. V. MorozYu. V. AvdosievF. A. ChabanovO. O. VoinalovychZaporozhye State Medical Universityarticlecongenital hepatic fibrosispregnancytransient elastographysplenic arteryembolizationMedicineRENRUUKZaporožskij Medicinskij Žurnal, Vol 23, Iss 6, Pp 882-888 (2021)
institution	DOAJ
collection	DOAJ
language	EN RU UK
topic	congenital hepatic fibrosis pregnancy transient elastography splenic artery embolization Medicine R
spellingShingle	congenital hepatic fibrosis pregnancy transient elastography splenic artery embolization Medicine R O. O. Popovych L. V. Moroz Yu. V. Avdosiev F. A. Chabanov O. O. Voinalovych Congenital hepatic fibrosis (a case report)
description	Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder of intrauterine morphogenesis of the portobiliary system, characterized by periportal fibrosis and defective remodeling of the bile ducts. The article describes the features of the clinical course, diagnosis and treatment of type I CHF in the different terms of a patient’s life: from birth to her pregnancy, during pregnancy and after childbirth. The aim of this work is to familiarize specialists with the problem of diagnosis and treatment of type I CHF in a patient with a provisional misdiagnosis of cryptogenic liver cirrhosis. Results. The features of type I CHF that determine a wider diagnostic testing in long-term unspecified liver pathology cases were identified. First-time decompensation of portal hypertension (PH) occurred at 35 weeks of gestation with the development of ascitic-edematous syndrome, hypersplenism, preeclampsia without signs of hepatic encephalopathy (HE). Cesarean section was performed at 37 weeks’ gestation and healthy baby was born. One year and 9 months after delivery, hypersplenism compensation was achieved by performing splenic artery embolization (SAE). There were no clinical manifestations of HE throughout the follow-up period. Conclusions. The diagnostic algorithm for a long-term compensated unspecified liver pathology with PH should confirm or rule out CHF. Type I CHF patients can give birth to a healthy child. SAE successfully compensates for hypersplenism and PH in type I CHF. Transient elastography is an effective method for the assessment of liver fibrosis progression in patients with type I CHF.
format	article
author	O. O. Popovych L. V. Moroz Yu. V. Avdosiev F. A. Chabanov O. O. Voinalovych
author_facet	O. O. Popovych L. V. Moroz Yu. V. Avdosiev F. A. Chabanov O. O. Voinalovych
author_sort	O. O. Popovych
title	Congenital hepatic fibrosis (a case report)
title_short	Congenital hepatic fibrosis (a case report)
title_full	Congenital hepatic fibrosis (a case report)
title_fullStr	Congenital hepatic fibrosis (a case report)
title_full_unstemmed	Congenital hepatic fibrosis (a case report)
title_sort	congenital hepatic fibrosis (a case report)
publisher	Zaporozhye State Medical University
publishDate	2021
url	https://doaj.org/article/12ba9a77486040aa9288a9e246c40b4e
work_keys_str_mv	AT oopopovych congenitalhepaticfibrosisacasereport AT lvmoroz congenitalhepaticfibrosisacasereport AT yuvavdosiev congenitalhepaticfibrosisacasereport AT fachabanov congenitalhepaticfibrosisacasereport AT oovoinalovych congenitalhepaticfibrosisacasereport
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Congenital hepatic fibrosis (a case report)

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