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Egyptian Journal of Medical Human Genetics

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Hepatoadrenal syndrome in Egyptian children with liver cirrhosis with and without sepsis

AAM Elfaramawy

Abstract


The similarities between septic shock and liver failure led to the proposal of the term hepatoadrenal syndrome. Adrenal insufficiency has been demonstrated in patients with severe liver disease irrespective of the presence of sepsis. The objective of this study was to evaluate children with liver cirrhosis for the presence of adrenal insufficiency especially during critical illness. This pilot study was designed to evaluate adrenal function for 24 children with liver cirrhosis of various etiologies by measuring basal cortisol level and measuring the peak level after 30 min of short low dose ACTH stimulation test. They were categorized in two groups; group 1 included 12 patients with sepsis and group 2 included 12 patients without sepsis. It was found in this study that no one of patients of group1 or group 2 had absolute adrenal insufficiency; however 11 patients, 8 (66.6%) of group 1 and 3 patients (25%)of group 2 had relative adrenal insufficiency (RAI) as the increment detected in cortisol level after stimulation was<9µlgm/dL. There was significantly higher incidence of RAI in group 1 compared to group 2 (P= 0.05). Most of patients with RAI (72.7%)were categorized as having Child–PughCliver cirrhosis. The presence of ascites, high temperature, high C reactive protein, neutrophilia, high ALT, AST, high total bilirubin, prolonged INR and lower albumin were all risk factors associated with the occurrence of RAI. Survival rate in patients with normal adrenal function (92%) was significantly better than in patients with RAI (27%) (P =0.02). It was concluded from this study that a high incidence of relative adrenal insufficiency was found in children with liver cirrhosis. It was more common in the presence of sepsis, related to the degree of liver cirrhosis and carried a bad prognosis.

Keywords: Liver cirrhosis; Adrenal insufficiency; Hepatoadrenal syndrome; Sepsis




http://dx.doi.org/10.1016/j.ejmhg.2012.04.005
AJOL African Journals Online