Lisowska Aleksandra, Kobelska-Dubiel Natalia, Jankowska Irena, Pawłowska Joanna, Moczko Jerzy, Walkowiak Jarosław
Department of Pediatric Gastroenterology and Metabolic Diseases, Poznań University of Medical Sciences, Poznań, Poland.
Department of Gastroenterology, Hepatology and Nutrition, Children's Memorial Health Institute, Warszawa, Poland.
Acta Biochim Pol. 2014;61(1):103-7. Epub 2014 Mar 17.
BACKGROUND & AIMS: To date, no studies concerning the presence of small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis were published. Based upon characteristic of progressive familial intrahepatic cholestasis one can expect the coexistence of small intestinal bacterial overgrowth. The aim of the study was to assess the incidence of small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis.
26 patients aged 8 to 25 years with progressive familial intrahepatic cholestasis were included in the study. Molecular analysis of ABCB11 gene was performed in the vast majority of patients. In all patients Z-score for body weight and height, biochemical tests (bilirubin, bile acid concentration, fecal fat excretion) were assessed. In all patients hydrogen-methane breath test was performed.
On the basis of first hydrogen-methane breath test, diagnosis of small intestinal bacterial overgrowth was confirmed in 9 patients (35%), 5 patients (19%) had borderline results. The second breath test was performed in 10 patients: in 3 patients results were still positive and 2 patients had a borderline result. The third breath test was conducted in 2 patients and positive results were still observed. Statistical analysis did not reveal any significant correlations between clinical, biochemical and therapeutic parameters in patients with progressive familial intrahepatic cholestasis and coexistence of small intestinal bacterial overgrowth.
Our results suggest that small intestinal bacterial overgrowth is frequent in patients with progressive familial intrahepatic cholestasis. Moreover, it seems that this condition has the tendency to persist or recur, despite the treatment.
迄今为止，尚无关于进行性家族性肝内胆汁淤积症患者小肠细菌过度生长情况的研究发表。基于进行性家族性肝内胆汁淤积症的特点，可以预期小肠细菌过度生长与之并存。本研究的目的是评估进行性家族性肝内胆汁淤积症患者小肠细菌过度生长的发生率。
本研究纳入了26例年龄在8至25岁之间的进行性家族性肝内胆汁淤积症患者。绝大多数患者进行了ABCB11基因的分子分析。对所有患者评估了体重和身高的Z评分、生化检查（胆红素、胆汁酸浓度、粪便脂肪排泄）。对所有患者进行了氢-甲烷呼气试验。
基于首次氢-甲烷呼气试验，9例患者（35%）确诊为小肠细菌过度生长，5例患者（19%）结果处于临界值。10例患者进行了第二次呼气试验：3例患者结果仍为阳性，2例患者结果处于临界值。2例患者进行了第三次呼气试验，仍观察到阳性结果。统计学分析未显示进行性家族性肝内胆汁淤积症患者的临床、生化和治疗参数与小肠细菌过度生长并存之间存在任何显著相关性。
我们的结果表明，进行性家族性肝内胆汁淤积症患者小肠细菌过度生长较为常见。此外，尽管进行了治疗，这种情况似乎有持续或复发的倾向。
