scholarly journals Congenital Hepatic Fibrosis as a Cause of Recurrent Cholangitis: A Case Report and Review of the Literature

Livers ◽  
2021 ◽  
Vol 1 (3) ◽  
pp. 132-137
Author(s):  
Emanuele Palomba ◽  
Marco Maggioni ◽  
Giulia Viero ◽  
Davide Mangioni ◽  
Rosa Lombardi ◽  
...  
Keyword(s):  
Hepatic Fibrosis ◽  
Clinical Manifestations ◽  
Multidrug Resistant ◽  
Antimicrobial Treatment ◽  
Congenital Hepatic Fibrosis ◽  
Systemic Involvement ◽  
Ductal Plate ◽  
Ductal Plate Malformation ◽  
Caroli Syndrome ◽  
Suppressive Antibiotic Therapy

Rare liver diseases caused by ductal plate malformation, such as congenital hepatic fibrosis (CHF), Caroli syndrome, and polycystic liver disease, can have clinical manifestations such as recurrent cholangitis—frequently involving multidrug-resistant microorganisms—leading to difficulties in selecting the optimal antimicrobial treatment. Without prompt recognition, these infections severely hamper the patient’s quality of life and can develop into life-threatening complications. We report here the case of a 50-year-old woman with a history of recurring cholangitis with occasional systemic involvement leading to bloodstream infection, who ultimately received a diagnosis of CHF and was put on chronic suppressive antibiotic therapy while on the waiting list for a liver transplant. We also reviewed the literature collecting cases of recurrent infections occurring in patients with ductal plate malformation.

scholarly journals Overview of Congenital Hepatic Fibrosis in Pediatrics: A Review

2021 ◽  
pp. 470-477
Author(s):  
Ahmed Abdelsamie Fadl ◽  
Sarah jamal Almujil ◽  
Ahmed Saeed S. Banheem ◽  
Nasser Naif Alsuhaymi ◽  
Mansour Hemaid Alhelali ◽  
...  
Keyword(s):  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Treatment Method ◽  
Congenital Hepatic Fibrosis ◽  
Angiotensin Ii Receptor ◽  
Polycystic Kidney ◽  
Genetic Abnormalities ◽  
Receptor Blockers ◽  
Ductal Plate Malformation ◽  
Caroli Syndrome

Congenital hepatic fibrosis is a rare developmental illness caused by a ductal plate malformation, often known as ciliopathy or fibrocystic liver disease. Hepatosplenomegaly and portal hypertension are two symptoms. The disease affects 1/10000–20000 people. frequently associated with a variety of illnesses caused by genetic abnormalities, such as autosomal recessive polycystic kidney disease (ARPKD) and Caroli syndrome. There hasn't been a way to stop or reverse the progression of congenital hepatic fibrosis until now. Clinical trials of anti-fibrotic medicines such as colchicine, interferon gamma, angiotensin II receptor blockers, pirfenidone, and ursodeoxycholic acid found no significant benefit. The only known cure for CHF is liver transplantation, which is recommended when the condition has progressed to the point when symptoms of liver failure have appeared. In this article we will be making overview of the disease. It’s symptoms and diagnosis, different treatment method, and we will compare some of the articles published about the disease.

scholarly journals Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Beidi Zhu ◽  
Zunguo Du ◽  
Zhengxin Wang ◽  
Yang Li ◽  
Jiming Zhang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Literature Review ◽  
Hepatic Fibrosis ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Case Series ◽  
Congenital Hepatic Fibrosis ◽  
Multidisciplinary Cooperation ◽  
Central Nervous Systems

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. 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. 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% vs. 21%, p=0.004). Malformation of kidneys and bile duct abnormalities were common, and multisystem involvement included eyes, other digestive organs, and genital and central nervous systems. 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.

scholarly journals Diagnosis of Congenital Hepatic Fibrosis in Adulthood

2019 ◽  
Vol 153 (1) ◽  
pp. 119-125 ◽  
Author(s):  
Mohammed I Alsomali ◽  
Martha M Yearsley ◽  
Douglas M Levin ◽  
Wei Chen
Keyword(s):  
Hepatic Fibrosis ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Joubert Syndrome ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Single Institution ◽  
Caroli Disease ◽  
Radiologic Findings ◽  
Disease Associations

Abstract Objectives We studied clinicopathologic features of congenital hepatic fibrosis (CHF) that could aid the diagnosis of this relatively rare condition during adulthood. Methods Five consecutive adult CHF cases were identified in a single institution. Results Clinical manifestations of CHF varied from asymptomatic to requiring liver transplantation. Three of five cases had other disease associations, including Joubert syndrome, Caroli disease, polycystic kidney disease, and congenital anomaly of hepatic vasculature. No unique common radiologic findings were found. Histologically, all cases showed characteristic abnormal interlobular bile ducts embedded in fibrotic portal stroma, with varying degrees of liver fibrosis. Conclusions While other disease associations and characteristic liver histomorphology are helpful clues to suspect the diagnosis of CHF in adult patients, other differential diagnoses should be excluded clinically and radiologically. This study highlights the importance of a multidisciplinary diagnostic approach by pathologists, radiologists, and hepatologists for the accurate diagnosis of CHF during adulthood.

Noninvasive Diagnosis of Caroli Syndrome Associated With Congenital Hepatic Fibrosis Using Hepatobiliary Scintigraphy

1995 ◽  
Vol 20 (6) ◽  
pp. 512-514 ◽  
Author(s):  
JO??L DESROCHES ◽  
LAURENT SPAHR ◽  
FRANCOIS LEDUC ◽  
GILLES POMIER-LAYRARGUES ◽  
MICHEL PICARD ◽  
...  
Keyword(s):  
Hepatic Fibrosis ◽  
Noninvasive Diagnosis ◽  
Hepatobiliary Scintigraphy ◽  
Congenital Hepatic Fibrosis ◽  
Caroli Syndrome

scholarly journals Congenital hepatic fibrosis (a case report)

2021 ◽  
Vol 23 (6) ◽  
pp. 882-888
Author(s):  
O. O. Popovych ◽  
L. V. Moroz ◽  
Yu. V. Avdosiev ◽  
F. A. Chabanov ◽  
O. O. Voinalovych
Keyword(s):  
Hepatic Fibrosis ◽  
Transient Elastography ◽  
Clinical Manifestations ◽  
Diagnostic Algorithm ◽  
Diagnosis And Treatment ◽  
Type I ◽  
Liver Pathology ◽  
Congenital Hepatic Fibrosis ◽  
Splenic Artery Embolization

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.  

Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis

2010 ◽  
pp. 331-358 ◽  
Author(s):  
Ronen Arnon ◽  
Henrietta Kotlus Rosenberg ◽  
Frederick J. Suchy
Keyword(s):  
Hepatic Fibrosis ◽  
Congenital Hepatic Fibrosis ◽  
Caroli Disease ◽  
Caroli Syndrome

scholarly journals Caroli Syndrome in a Child: A Case Report

2019 ◽  
Author(s):  
Pantea Tajik ◽  
Amir Hossein Goudarzian
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Definitive Treatment ◽  
Congenital Hepatic Fibrosis ◽  
Portal Vein Pressure ◽  
Abdominal Sonography ◽  
Case Presentation ◽  
Intrahepatic Bile Ducts ◽  
Caroli Syndrome ◽  
Multiple Cysts

Introduction: Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis. Case Presentation: A 3-year-old boy with abdominal distention was referred to gastroentrology ward of Amiralmomenin hospital (Semnan, Iran) in summer 2018. In his abdominal sonography, multiple cysts were detected in the liver with hepatomegaly, and the portal vein pressure was 10 mm. Also, in liver biopsy, dilated portal bile ducts (trichrome stain) with inspissated bile and congenital hepatic fibrosis were reported. He was discharged after conservative therapy and followed up. Conclusion: Definitive treatment, i.e surgery, should be offered to prevent future complications.

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