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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| Format: | Article |
| Language: | English |
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Zaporizhzhia State Medical and Pharmaceutical University
2021-11-01
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| Series: | Zaporožskij Medicinskij Žurnal |
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| Online Access: | http://zmj.zsmu.edu.ua/article/view/229102/241672 |
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| 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 |
| collection | DOAJ |
| 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 |
| id | doaj-art-12ba9a77486040aa9288a9e246c40b4e |
| institution | Kabale University |
| issn | 2306-4145 2310-1210 |
| language | English |
| publishDate | 2021-11-01 |
| publisher | Zaporizhzhia State Medical and Pharmaceutical University |
| record_format | Article |
| series | Zaporožskij Medicinskij Žurnal |
| spelling | doaj-art-12ba9a77486040aa9288a9e246c40b4e2025-08-20T03:54:20ZengZaporizhzhia State Medical and Pharmaceutical UniversityZaporožskij Medicinskij Žurnal2306-41452310-12102021-11-0123688288810.14739/2310-1210.2021.6.229102Congenital hepatic fibrosis (a case report)O. O. Popovych0https://orcid.org/0000-0001-6278-6094L. V. Moroz1https://orcid.org/0000-0001-6278-6094Yu. V. Avdosiev2https://orcid.org/0000-0002-2677-4464F. A. Chabanov3https://orcid.org/0000-0001-9307-3679O. O. Voinalovych4https://orcid.org/0000-0002-6242-3346Vinnytsia National Pirogov Memorial Medical University, UkraineVinnytsia National Pirogov Memorial Medical University, UkraineState Institution “Institute of General and Emergency Surgery named after V. T. Zaycev of the National Academy of Medical Sciences of Ukraine”, Kharkiv, UkraineVinnytsia City Clinical Hospital No. 1, UkraineVinnytsia National Pirogov Memorial Medical University, UkraineCongenital 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.http://zmj.zsmu.edu.ua/article/view/229102/241672congenital hepatic fibrosispregnancytransient elastographysplenic arteryembolization |
| spellingShingle | O. O. Popovych L. V. Moroz Yu. V. Avdosiev F. A. Chabanov O. O. Voinalovych Congenital hepatic fibrosis (a case report) Zaporožskij Medicinskij Žurnal congenital hepatic fibrosis pregnancy transient elastography splenic artery embolization |
| title | 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_short | Congenital hepatic fibrosis (a case report) |
| title_sort | congenital hepatic fibrosis a case report |
| topic | congenital hepatic fibrosis pregnancy transient elastography splenic artery embolization |
| url | http://zmj.zsmu.edu.ua/article/view/229102/241672 |
| work_keys_str_mv | AT oopopovych congenitalhepaticfibrosisacasereport AT lvmoroz congenitalhepaticfibrosisacasereport AT yuvavdosiev congenitalhepaticfibrosisacasereport AT fachabanov congenitalhepaticfibrosisacasereport AT oovoinalovych congenitalhepaticfibrosisacasereport |