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Published on: September 22, 2019
Small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis
Aleksandra Lisowska1, Natalia Kobelska-Dubiel1, Irena Jankowska2
1Department of Pediatric Gastroenterology and Metabolic Diseases, Poznań University of Medical Sciences, Poznań, Poland.
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.
Methods:
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.
Results:
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.
Conclusions:
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.
Insights
Small intestinal bacterial overgrowth (SIBO) is common in patients with progressive familial intrahepatic cholestasis (PFIC). This SIBO often persists or recurs, even with treatment, highlighting a significant comorbidity.
Area of Science:
- Gastroenterology
- Hepatology
- Microbiology
Background:
- Progressive familial intrahepatic cholestasis (PFIC) is a group of rare genetic liver diseases.
- The coexistence of small intestinal bacterial overgrowth (SIBO) in PFIC patients has not been previously studied.
- PFIC's cholestatic nature suggests a potential link with SIBO.
Purpose of the Study:
- To determine the incidence of small intestinal bacterial overgrowth (SIBO) in patients diagnosed with progressive familial intrahepatic cholestasis (PFIC).
- To investigate the prevalence of SIBO in a cohort of PFIC patients.
Main Methods:
- A cohort of 26 patients with PFIC (aged 8-25 years) was enrolled.
- Hydrogen-methane breath tests were used to diagnose SIBO.
- Genetic analysis of the ABCB11 gene and biochemical assessments were performed.
Main Results:
- Small intestinal bacterial overgrowth (SIBO) was confirmed in 35% of PFIC patients via initial breath tests.
- Borderline results were observed in 19% of patients.
- SIBO showed a tendency to persist or recur in follow-up breath tests, with no significant correlation to clinical or biochemical parameters.
Conclusions:
- Small intestinal bacterial overgrowth (SIBO) is a frequent comorbidity in patients with progressive familial intrahepatic cholestasis (PFIC).
- The persistent or recurrent nature of SIBO in PFIC patients warrants further investigation and potential therapeutic considerations.
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