الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Paucity of the intrahepatic bile ducts (PIHBD) is a pathologic diagnosis characterized by loss of intrahepatic bile ducts in more than fifty percent of portal tracts in a specimen that contains a minimum of 10 portal tracts. Thus, the diagnosis of PIBD needs a pathologic examination of a liver biopsy.
Two groups of paucity of intrahepatic bile ducts (PIHBD) are recognized: syndromic and non-syndromic. The syndromic group is also termed Alagille syndrome or arteriohepatic dysplasia. Alagille syndrome may affect five body areas (liver, heart, skeleton, face, and eye). At least three of five major criteria are needed for the diagnosis: characteristic facies, chronic cholestasis, cardiac abnormalities, vertebral arch defects, and the presence of posterior embryotoxon.
The non-syndromic group has been associated with different abnormalities: metabolic or viral diseases (cytomegalovirus (CMV), rubella), chromosomal disorders (trisomy 18 and 21), altered bile acid metabolism and cystic fibrosis. Most cases are classified as primary or idiopathic.
The aim of the present study was to evaluate the history, clinical and biochemical characteristics, follow up and outcome of children with the diagnosis of paucity of the intrahepatic bile ducts attending the hepatology clinic at AUCH during the last five years period from (January 2012 – January 2017).
This retrospective study was conducted on the files of 25 infants and children with the diagnosis of intrahepatic bile duct paucity who attended the Hepatology Clinic of Alexandria University Children’s Hospital during the five years period from (January 2012 – January 2017).
The files were reviewed for the data of:
1. History and clinical examination
2. Laboratory investigation
3. Medical imaging evaluation
4. Liver biopsy
5. Follow up data and outcome.
The studied children were classified into two groups according to etiology: Syndromic group (Alagille syndrome) included 7 cases, and non syndromic group which included 18 cases. The most common etiology in non syndromic group was Idiopathic hepatitis (13 patients, 72.2%).
The majority of patients in the non syndromic group were male infants (12 patients, 66.7%), while females represented the majority in syndromic group (5 patients, 71.4%). The main age at onset was more earlier in syndromic group (16.6 9.1 days) than the non syndromic group (47.94 ± 29.05 days).
Almost all patients in both groups were born full term and delivered through cesarean section. Positive family history of similar conditions was found in (5 patients, 27.8%) of non syndromic group and (2 patients, 28.6%) of syndromic group. Positive consanguinity represented 44% of total cases. All patients presented with jaundice and abdominal distension. Clay colored stool as one of the presenting symptoms was found in about 66.7% of non syndromic group and 71.4% of syndromic group.