title: Congenital hepatic fibrosis: clinical presentation, laboratory features and management at a tertiary care hospital of Lahore.
abstract: OBJECTIVE
  To describe the clinical presentations, laboratory features and management of congenital hepatic fibrosis patients at a tertiary care hospital.
  
  
  METHODS
  The case series was conducted at The Children Hospital and Institute of Child Health, Lahore, Pakistan, from July 2013 to June 2015, and comprised patients of congenital hepatic fibrosis diagnosed on the basis of liver biopsy. SPSS 20 was used for statistical analysis.
  
  
  RESULTS
  The mean age of 25 patients in the study was 8.5±2.74 years, and the male-to-female ratio was 3:1. Parents of 21(84%) patients had consanguineous marriage, and 14(56%) patients had family history of hematemesis and melena. Besides, 15(60%) patients presented with hematemesis, 12(48%) had abdominal distension, 5(20%) were picked up on screening of siblings and 5(20%) were referred from general practitioners on feeling of hepatosplenomegaly during routine examination. All had hepatomegaly with a mean size of 7.2 ±2.3cm palpable in midline. Splenomegaly was present in 24(96%). Overall, 15(60%) patients had oesophageal varices. Endoscopic band ligations were done in oesophageal variceal patients who were successfully managed, while 5(20%) patients required portosystemic shunt surgeries.
  
  
  CONCLUSIONS
  Congenital hepatic fibrosis was not uncommon in our population having high rate of consanguinity and most of them were familial cases.
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital & Institute of Child Health, Lahore.
authors.forename: Arit
authors.initials: A
authors.lastname: Parkash
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital & Institute of Child Health, Lahore.
authors.forename: Huma Arshad
authors.initials: HA
authors.lastname: Cheema
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital & Institute of Child Health, Lahore.
authors.forename: Hassan Suleman
authors.initials: HS
authors.lastname: Malik
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital & Institute of Child Health, Lahore.
authors.forename: Zafar
authors.initials: Z
authors.lastname: Fayyaz
mesh_terms: D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D016145:Endoscopy, Digestive System; D004932:Esophageal and Gastric Varices / Q000209:etiology* / Q000601:surgery; D005260:Female; D030342:Genetic Diseases, Inborn / Q000097:blood / Q000150:complications* / Q000473:pathology; D006396:Hematemesis / Q000209:etiology*; D006529:Hepatomegaly / Q000209:etiology*; D006801:Humans; D019934:International Normalized Ratio; D008026:Ligation; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000097:blood / Q000150:complications* / Q000473:pathology; D008297:Male; D010154:Pakistan; D010314:Partial Thromboplastin Time; D011170:Portasystemic Shunt, Surgical; D011517:Prothrombin Time; D013163:Splenomegaly / Q000209:etiology*; D062606:Tertiary Care Centers; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D005723:gamma-Glutamyltransferase; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase; D001663:Bilirubin
keywords: Congenital hepatic fibrosis, Hepatomegaly, Hematemesis.