PO-0372Autoimmune Hepatitis: Biochemical And Clinical Review Of A Series Of 4 Cases

• Published in: Archives of disease in childhood 2014-10, Vol.99 (Suppl 2), p.A367-A367
• Main Authors: Munoz-Garcia, M, Arevalo-Garrido, A M, Perez-Parras, MA, de la Cruz-Moreno, J
• Format: Article
• Language: English
• ISSN: 0003-9888
• DOI: 10.1136/archdischild-2014-307384.1019

The clinical spectrum of Autoinmune hepatitis (AH) is very wide, ranging from asymptomatic individuals with abnormal liver function to those with fulminant liver failure. The objective of this study was to analyse the clinical presentation, biochemical and histological diagnosis of a number of cases of AH.Methodsbetween January-2006 and November-2013, we found 4 patients who were diagnosed AH.ResultsThe median age was 11.9 years (2-13), predominantly female (75%). The level of GOT and GPT were elevated, with a median of 259 IU (128-2451) and 309 IU (117-1449) respectively. The mean serum albumin was 3.9 plus or minus 0.3 g/dl and the presence of IgG mean was 2391.5 plus or minus 628. In all cases, the liver biopsy was performed to establish diagnosis. The anti-liver Kidney microsome type-1 (anti-LKM1) antibodies were present in all patients, and only in two of patients were found positive anti-smooth muscle (ASMA) antibodies. In 50% were positive for antinuclear (ANA). Among the associated comorbidities found a case of hypothyroidism and vitiligo. Note that two of the four cases correspond to the same family, where molecular genetic study was carried out, with both sisters carrying HLA haplotypes DRB1 07. DRB1 14/DQB1 02. DQB105. In the majority of patients had non-specific symptoms, and only one patient presented jaundiced with abdominal pain. At follow-up, all patients are alive and there were not progression to cirrhosis or fulminant liver failureConclusionIn our series, the autoimmune hepatitis was characterised predominant in female, with non-specific symptoms, had levels of transaminases elevated and associated with other autoimmune pathologies.