Original ArticleClinico-laboratory study on children with auto-immune hepatitis in Upper Egypt
Introduction
Auto-immune hepatitis (AIH) is a chronic disorder of the immune system targeting the liver, characterised by loss of immunological tolerance against the hepatocytes and including chronic inflammatory destruction of the liver parenchyma, cirrhosis and, eventually, liver failure [1], [2]. It has been described since the early 1950s, but its aetiology remains unknown [3] and is characterised serologically by high aminotransferase levels, elevated immunoglobulin G (IgG) and the presence of autoantibodies [4]. AIH is divided into two types according to the autoantibody profile: patients with type 1 are positive for antinuclear antibody (ANA) and/or anti-smooth muscle antibody (ASM), and patients with type 2 are positive for anti-liver–kidney–microsomal antibody type-1 (anti-LKM-1) [4], [5]. In keeping with other auto-immune disorders, AIH predominantly affects female patients at any age; the disease has been diagnosed as early as the age of 6 months, and its onset may range from asymptomatic to hyperacute with liver failure [5], [6]. Without treatment, AIH may develop into liver cirrhosis with potentially fatal outcomes. Early diagnosis and prompt intervention are thus important to save lives [7]. There is an increase in the yearly prevalence of AIH in childhood. The mode of presentation in childhood is variable, and the disease should be suspected and excluded in all children presenting with symptoms and signs of prolonged or severe liver disease [8].
This study aims to assess frequency, clinical manifestations, biochemical features and outcome of AIH in children attending Assuit University Hospitals in Upper Egypt with acute icteric hepatitis and seronegative viral markers (anti-hepatitis A virus (HAV) IgM, HbsAg, anti-hepatitis C virus antibody (anti-HCV Ab)).
Section snippets
Patients and methods
During the period from January 2005 to December 2009, 75 children with acute icteric hepatitis and seronegative viral markers (anti-HAV IgM, hepatitis B surface antigen, anti-HCV antibodies) were referred to the gastroenterology and hepatology unit of the Pediatric Department and the Department of Tropical Medicine of Assuit University Hospitals (a tertiary care referral hospital in Upper Egypt) for further diagnosis. AIH could be diagnosed in 34 children (45.3%). They were 24 females and 10
Statistical methods
Analysis was carried using Statistical Package for Social Sciences (SPSS) (version 16). The numerical data were represented as mean ± SD. For comparison of the two groups, Student’s t-test was used. Qualitative variables were compared using Fisher’s exact test. For all tests, the difference was considered significant if the probability (P) was <0.05.
Results
Out of 75 children with acute icteric hepatitis and seronegative viral marking (anti-HAV IgM, HbsAg and anti-HCV Ab), AIH could be diagnosed in 34 (45.3%).
Among 34 children diagnosed as AIH, 24 were females (70.5%) with a mean age of 8.7 ± 3.4 years and 10 were males (29.5%) with a mean age of 9.5 ± 2.8 years at time of presentation.
None of the patients included in this study had a previous history of blood transfusion, surgery or drug intake, and their first-degree relatives did not have history of
Discussion
In our study AIH could be detected in 34 out of 75 children (45.3%), based on the International Scoring Criteria of Autoi-mmune Hepatitis [9]. This strongly suggests that AIH should be included in the differential diagnosis of children with unexplained hepatitis-like manifestations. The designation of probable AIH by the international scoring systems is based on differences in clinical manifestations and does not reflect differences in the validity of the diagnosis or its treatment response;
Conflicts of interest
The authors declared that there was no conflict of interest.
References (28)
- et al.
Acute and chronic hepatitis
- et al.
Autoimmune hepatitis in Italy: the Bologna experience
J Hepatol
(2009) - et al.
International autoimmune hepatitis group report: review of criteria for diagnosis of autoimmune hepatitis
J Hepatol
(1999) Comparability of probable and definite autoimmune hepatitis by international diagnostic scoring criteria
Gastroenterology
(2011)- et al.
Clinical features and biochemical data of Caucasian children at diagnosis of autoimmune hepatitis
J Autoimmun
(2005) - et al.
Autoimmune hepatitis
J Hepatol
(2000) - et al.
Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five cases
Am J Gastroenterol
(1998) - et al.
Autoimmune hepatitis in children
Clin Liver Dis
(2002) Autoimmune hepatitis
N Engl J Med
(2006)- et al.
Autoimmune hepatitis, from mechanisms to therapy
Hepatology
(2006)
Aetiopathogenesis of autoimmune hepatitis
World J Gastroenterol
Autoimmune hepatitis: a childhood disease
Curr Pediatr Rev
Guidelines for therapy of autoimmune liver disease
Semin Liver Dis
Autoimmune paediatric liver disease
World J Gastroenterol
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