Clinical and Paraclinical Findings in Children With Congenital Hepatic Fibrosis: A Single Center 10-Year Study

Authors

  • Ali Bazi Faculty of Allied Medical Sciences, Zabol University of Medical Sciences, Zabol, Iran
  • Ali Derakhshan Pediatric Department, Shiraz University of Medical Sciences, Shiraz, Iran
  • Ali Jangjou Emergency Medicine Department, Shiraz University of Medical Sciences, Shiraz, Iran
  • Amir Saeidi Pediatric Department, Shiraz University of Medical Sciences, Shiraz, Iran
  • Farzaneh Nejati Pediatric Department, Shiraz University of Medical Sciences, Shiraz, Iran
  • Fatemeh Parooie Pediatric Digestive and Hepatic Diseases Research Center, Amir-Al-Momenin Hospital, Zabol University of Medical Sciences, Zabol, Iran
  • Iraj Shahramian Pediatric Digestive and Hepatic Diseases Research Center, Amir-Al-Momenin Hospital, Zabol University of Medical Sciences, Zabol, Iran
  • Morteza Salarzaei Pediatric Digestive and Hepatic Diseases Research Center, Amir-Al-Momenin Hospital, Zabol University of Medical Sciences, Zabol, Iran
Abstract:

Background: Congenital hepatic fibrosis (CHF) is an autosomal hereditary disorder affecting the porto-biliary system. It is a rare hereditary disorder often presenting in childhood or adolescence with hepatomegaly, splenomegaly, and gastrointestinal bleeding. A timely diagnosis of organomegalies by sonography can prevent esophageal varices. Liver transplantation is now the only cure for CHF. Objectives: The current study aimed to determine clinical and paraclinical findings in patients diagnosed with CHF from 2008 to 2017. Methods: This was a descriptive cross-sectional study of all children Results: Overall, 32 CHF patients were included during the study period. Of these, 12 (37.5%) and 20 (62.5%) were female and male, respectively. The most frequent clinical presentations at diagnosis were hepatomegaly (81%), splenomegaly (68%), gastrointestinal bleeding (43%), abdominal protrusion (40%), ascites (21%), and epistaxis (6%). Severely enlarged livers were observed in 2 patients. Only 5 patients showed a normal-sized spleen, and kidney sonographic findings were normal in 30 patients. Liver enzymes were not severely deviated from the normal range. There was a significant association between spleen size and esophageal varices (P = 0.01). Overall, 8 patients were liver transplanted due to decompensated cirrhosis. One patient developed bone marrow suppression secondary to the Epstein bar virus and ultimately succumbed to post-transplant lymphoproliferative disorder. In the study period, 2 girls and 2 boys died of disease complications. Conclusion: The results of the present study indicated that the most common clinical findings of CHF in pediatric patients are splenomegaly and hepatomegaly presenting as abdominal distention and gastrointestinal bleeding. Laboratory data can be normal in most cases, but ultrasonographic findings (in liver, spleen, and even kidneys) can be helpful. The present study also showed that patients with splenomegaly are at higher risk of esophageal varices.

Upgrade to premium to download articles

Sign up to access the full text

Already have an account?login

similar resources

Congenital hepatic fibrosis in an aborted calf

An aborted female Holstein foetus with marked generalized anasarca was referred to the Excellence Centre for Ruminant Abortion and Neonatal Mortality, Ferdowsi University of Mashhad. On postmortem examination, red-tinged ascites, pale and firm liver with extreme irregularity and numerous round to oval slightly raised foci on the capsular surface were seen. Histological examination revealed wide...

full text

a single center study of clinical and paraclinical aspects in iranian patients with allergic rhinitis

allergic rhinitis (ar) is a common form of allergic disorder in iran and rest of the world. this study was conducted to investigate the symptoms and paraclinical aspects of this allergy among iranian patients. a total of 206 patients (51.5%: male), with one of the specific symptoms of ar, from march 2005 to march 2007 were enrolled in the study. a written questionnaire was completed for each pa...

full text

A single center study of clinical and paraclinical aspects in Iranian patients with allergic rhinitis.

Allergic rhinitis (AR) is a common form of allergic disorder in Iran and rest of the world. This study was conducted to investigate the symptoms and paraclinical aspects of this allergy among Iranian patients. A total of 206 patients (51.5%: male), with one of the specific symptoms of AR, from March 2005 to March 2007 were enrolled in the study. A written questionnaire was completed for each pa...

full text

Ophthalmologic Findings in Children with Leukemia: A Single-Center Study

OBJECTIVES Ophthalmologic disease in patients with acute leukemia occurs due to primary leukemic infiltration (involvement), or secondary to the disease and its treatment. In recent years the life expectancy of acute leukemia patients has increased with the advent of modern therapies. The present study aimed to determine the incidence of ocular manifestations in children with acute leukemia. ...

full text

Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study

Background and Aim: Steroid-resistant nephrotic syndrome (SRNS) accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.Materials and Met...

full text

Deferasirox in Chelation Naive Children with Transfusional Iron Overload in Basra, Iraq: A Two-Year Single Center Study

Background: Effective management of iron overload in patients receiving long-term blood transfusion requires assessment and monitoring of both severity of iron overload and excessive iron chelation. We aimed to evaluate the efficacy and safety of Deferasirox (DFX) in chelation naive patients with transfusion dependent thalassemia and sickle cell disease. Methods: Chelation naive patients with ...

full text

My Resources

Save resource for easier access later

Save to my library Already added to my library

{@ msg_add @}


Journal title

volume 5  issue 2

pages  42- 46

publication date 2020-06-16

By following a journal you will be notified via email when a new issue of this journal is published.

Hosted on Doprax cloud platform doprax.com

copyright © 2015-2023