Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.
Journal
Gastroenterology research and practice
ISSN: 1687-6121
Titre abrégé: Gastroenterol Res Pract
Pays: Egypt
ID NLM: 101475557
Informations de publication
Date de publication:
2020
2020
Historique:
received:
30
06
2019
revised:
25
02
2020
accepted:
30
03
2020
entrez:
9
5
2020
pubmed:
10
5
2020
medline:
10
5
2020
Statut:
epublish
Résumé
Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults. We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients' age. A total of eight patients were diagnosed with congenital hepatic fibrosis in the study, including four children and four adults. The onset age of the children, who suffered from severe complications of portal hypertension and needed liver transplantation, ranged from 1 to 15 years old. The disorder developed in adults aged 26 to 60 years old. Three adults complained of recurrent abnormal liver function at the onset of illness, and they mainly received conservative treatments. The literature review included 30 children and 33 adults. In comparison, hepatomegaly was more common in children than in adults (57% Serious complications of portal hypertension developed in children requiring liver transplantation, while adults often had mild-to-moderate liver injuries upon onset. Adults with CHF varied a lot in clinical manifestations. Multiorgan involvement and unusual course are helpful to make a diagnosis. Timely histological assessment by liver biopsy and multidisciplinary cooperation are crucial for definitive diagnosis and early intervention.
Sections du résumé
BACKGROUND
BACKGROUND
Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults.
METHODS
METHODS
We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients' age.
RESULTS
RESULTS
A total of eight patients were diagnosed with congenital hepatic fibrosis in the study, including four children and four adults. The onset age of the children, who suffered from severe complications of portal hypertension and needed liver transplantation, ranged from 1 to 15 years old. The disorder developed in adults aged 26 to 60 years old. Three adults complained of recurrent abnormal liver function at the onset of illness, and they mainly received conservative treatments. The literature review included 30 children and 33 adults. In comparison, hepatomegaly was more common in children than in adults (57%
CONCLUSIONS
CONCLUSIONS
Serious complications of portal hypertension developed in children requiring liver transplantation, while adults often had mild-to-moderate liver injuries upon onset. Adults with CHF varied a lot in clinical manifestations. Multiorgan involvement and unusual course are helpful to make a diagnosis. Timely histological assessment by liver biopsy and multidisciplinary cooperation are crucial for definitive diagnosis and early intervention.
Identifiants
pubmed: 32382272
doi: 10.1155/2020/8284274
pmc: PMC7191434
doi:
Types de publication
Journal Article
Langues
eng
Pagination
8284274Informations de copyright
Copyright © 2020 Beidi Zhu et al.
Déclaration de conflit d'intérêts
The authors declare that there is no conflict of interests regarding the publication of this paper.
Références
Eur Rev Med Pharmacol Sci. 2015 Jun;19(12):2297-300
pubmed: 26166658
Gastroenterology. 2013 Jan;144(1):112-121.e2
pubmed: 23041322
Zhonghua Gan Zang Bing Za Zhi. 2019 Jun 20;27(6):463-465
pubmed: 31357765
Diagn Pathol. 2017 Aug 16;12(1):61
pubmed: 28814334
Pediatr Radiol. 2009 Feb;39(2):100-11
pubmed: 19089418
Clin Case Rep. 2017 May 23;5(7):1098-1102
pubmed: 28680603
Zhonghua Bing Li Xue Za Zhi. 2010 Jul;39(7):485-6
pubmed: 21055183
Zhonghua Gan Zang Bing Za Zhi. 2009 Aug;17(8):634-5
pubmed: 19719930
Clin Res Hepatol Gastroenterol. 2014 Dec;38(6):e115-6
pubmed: 24981780
World J Clin Cases. 2015 Oct 16;3(10):904-10
pubmed: 26488028
Medicine (Baltimore). 2017 Jan;96(3):e5843
pubmed: 28099341
J Pediatr Gastroenterol Nutr. 2013 Aug;57(2):161-6
pubmed: 23518487
Clin Res Hepatol Gastroenterol. 2014 Sep;38(4):407-14
pubmed: 24953524
Zhonghua Gan Zang Bing Za Zhi. 2008 Jun;16(6):477
pubmed: 18579008
Ann Saudi Med. 2014 Jan-Feb;34(1):81-3
pubmed: 24658561
Front Pediatr. 2017 Jun 07;5:114
pubmed: 28638817
Histopathology. 1992 Jun;20(6):465-77
pubmed: 1607148
Front Pediatr. 2017 May 29;5:124
pubmed: 28611971
Int J Surg Case Rep. 2017;34:134-138
pubmed: 28402911
Am J Clin Pathol. 2020 Jan 1;153(1):119-125
pubmed: 31584623
Zhonghua Gan Zang Bing Za Zhi. 2015 Jun;23(6):467-8
pubmed: 26419012
Front Pediatr. 2017 Apr 20;5:80
pubmed: 28473971
Clin J Gastroenterol. 2014 Feb;7(1):48-51
pubmed: 26183508
Clin Exp Hepatol. 2017 Sep;3(3):176-179
pubmed: 29062909
Clin Pract. 2015 Nov 05;5(4):792
pubmed: 26918098
World J Gastroenterol. 2010 Feb 14;16(6):683-90
pubmed: 20135715
Indian J Radiol Imaging. 2017 Jan-Mar;27(1):6-12
pubmed: 28515578
Saudi J Kidney Dis Transpl. 2014 Jul;25(4):840-3
pubmed: 24969198
Dig Liver Dis. 2014 Apr;46(4):295-301
pubmed: 24321359