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Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.

ABSTRACT: 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.

SUBMITTER: Zhu B

PROVIDER: S-EPMC7191434 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.

Zhu Beidi B Du Zunguo Z Wang Zhengxin Z Li Yang Y Zhang Jiming J Zhu Haoxiang H

Gastroenterology research and practice 20200421

<h4>Background</h4>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.<h4>Methods</h4>We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at t ...[more]

PMID: 32382272

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Project description:BackgroundPyrethroid-based mosquito repellents are widely used to control mosquito-borne diseases. Liquid mosquito-repellent vaporisers are effective modes of pyrethroid delivery but can also pose significant health risks if ingested or used improperly.ObjectiveThis systematic review was performed to assess the demographic distribution, clinical presentation, management strategies and outcomes in children resulting from accidental ingestion of liquid mosquito repellent vaporiser.MethodsThe study adheres to the reporting standards outlined in the PRISMA Statement for Systematic Reviews and was prospectively registered with PROSPERO (record # CRD42023413937) to enhance transparency and minimise reporting bias. A comprehensive search was conducted on PubMed, Scopus and Google Scholar using specific MeSH terms related to insecticides, mosquito repellents, pyrethroids, ingestion, poisoning, toxicity and prevention. The reference lists of the included studies were also reviewed for additional relevant articles. The inclusion criteria involved studies published in peer-reviewed journals between 2000 and 2023 that focused on children under 18 years old with a history of mosquito-repellent ingestion based on primary data.ResultsTwelve studies met the inclusion criteria; these were primarily case reports from India, China and the UAE. Male children were predominantly affected, and symptoms included vomiting, convulsions, cough and respiratory distress. Management primarily involved supportive and symptomatic measures, including atropine for salivation and antiepileptic drugs for seizures. Respiratory support was provided for respiratory complications.ConclusionDespite the known risks and diverse presentations of pyrethroid poisoning caused by liquid mosquito repellent vaporiser in children, the limited substantial evidence in the literature underscores the urgent need for comprehensive research to refine management approaches and enhance preventive measures.

Dataset Information

Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.

Publications

Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.

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