Probiotic knowledge of adults with cystic fibrosis is limited but is associated with probiotic use: A cross-sectional survey study.

Background: Probiotics are used by people with cystic fibrosis (CF) and other chronic diseases to manage gastrointestinal symptoms. Aim: To describe probiotic knowledge; its relationship with probiotic use, probiotic information sources and factors influencing choice in adults with CF and a general population control group. Methods: A cross-sectional questionnaire study was conducted in adults with CF (n = 205) and Controls (n = 158). Probiotic knowledge was compared between CF and Controls using a knowledge score (maximum 5) based on predefined criteria: (1a) bacteria/microorganism; (1b) live; (2a) administered; (2b) adequate dose and (3) health benefit, using independent samples t-test. Two-way analysis of variance explored knowledge scores between CF and Control and between Ever User and Never User groups. Chi-square and Fisher's exact tests compared knowledge criterion, probiotic sources and influences on probiotic choice between groups. Thematic analysis of open-text responses explored probiotic-related knowledge and influences on probiotic decision making. Results: Knowledge scores (mean ± SD) did not differ between CF (1.70 ± 1.12) and Controls (1.89 ± 0.99), p = 0.13. Probiotic use was associated with knowledge score (p < 0.001). More CF Ever Users than Never Users correctly identified criteria 1a (65% vs. 38%), 1b (16% vs. 0%), 2a (45% vs. 22%) and 3 (73% vs. 42%) (all p < 0.005). CF participants considered 'dairy yoghurt' (69%), 'live cultures' (64%) and 'fermented foods' (37%) as 'all/mostly' probiotic sources. The internet was the commonest source of probiotic-related information. Conclusion: Probiotic knowledge and use were associated in adults with CF. Understanding of probiotic characteristics and sources were limited. Education is needed to help guide patient probiotic decision making.

Probiotic use in adults with cystic fibrosis is common and influenced by gastrointestinal health needs: A cross-sectional survey study.

BACKGROUND: Cystic fibrosis (CF) primarily affects the lung, however, gastrointestinal disorders and symptoms, including dysbiosis, also impact on morbidity and quality of life. There is interest in strategies to modulate the gastrointestinal microbiota, including probiotics, although the evidence remains inadequate to guide practice, and information on use is limited. The present study aimed to characterise probiotic use, beliefs and experiences of adults with CF. METHODS: A cross-sectional questionnaire study was conducted in adults with CF (n = 205) and a general population Control group (n = 158), recruited from Victoria, Australia. Participants were classified as probiotic 'Ever Users' or 'Never Users'. Outcomes included self-reported probiotic use and factors associated with probiotic use, which were analysed using logistic regression analysis. Open-ended questionnaire responses were thematically analysed. RESULTS: In total, 70% of adults with CF had ever used probiotics (supplements and/or foods), comparable to Controls (80%) (p = 0.03). Key reasons for CF probiotic use were gastrointestinal- and antibiotic-related (75%). Most CF Ever Users (73%) did not discuss probiotic use with CF clinicians and 33% were uncertain if probiotics had been helpful. Female gender (odds ratio [OR] = 2.82; 95% confidence interval [CI] = 1.36-5.87; p = 0.005), university-level education (OR = 2.73; 95% CI = 1.24-6.01; p = 0.01) and bloating on antibiotics (OR = 2.14; 95% CI = 1.04-4.40; p = 0.04) were independently associated with probiotic use in CF; as was female gender in Controls (OR = 2.84; 95% CI = 1.20-6.71; p = 0.02). CONCLUSIONS: Probiotics were used by adults with CF for gastrointestinal- and antibiotic-related reasons often without informing clinicians and despite uncertainty about perceived helpfulness. Further research investigating gastrointestinal outcomes of probiotics will inform practice recommendations guiding their use in CF and other chronic diseases.

Effect of probiotics on respiratory, gastrointestinal and nutritional outcomes in patients with cystic fibrosis: A systematic review.

BACKGROUND: An increasing body of research investigating the use of probiotics to improve health outcomes in patients with cystic fibrosis (CF) prompted the need to systematically assess and summarise the relevant literature. METHODS: An electronic search of five databases and three trial databases was conducted. Studies describing the administration of probiotics to patients with CF older than 2years, with a comparator group on respiratory, gastrointestinal and nutritional outcomes were included. RESULTS: Three pre-post studies and six randomised controlled trials met the inclusion criteria. Overall studies showed a positive effect of probiotics on reducing the number of pulmonary exacerbations and decreasing gastrointestinal inflammation. There was limited effect of probiotics on other outcomes and inadequate evidence for the effects of specific probiotic species and strains. CONCLUSION: The findings suggest that probiotics may improve respiratory and gastrointestinal outcomes in a stable CF clinic population with no reported evidence of harm. There is inadequate evidence at this time to recommend a specific species, strain or dose of probiotic as likely to be of significant benefit.

Neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1), previously known as von Recklinghausen disease, is a neurogenetic disorder distinct from neurofibromatosis type 2 (NF2). Approximately 1:2500 to 1:3500 individuals worldwide are affected, regardless of ethnicity or race. The classic manifestations of NF1 include café-au-lait macules, skinfold freckling, neurofibromas, brain tumors, iris hamartomas, and characteristic bony lesions. In addition, patients with NF1 are at increased risk for learning and intellectual disabilities, aqueductal stenosis, pheochromocytoma, vascular dysplasia, scoliosis, and cancer. In this chapter, we discuss the clinical and molecular features of NF1 as well as how insights into its underlying molecular pathophysiology have revealed new targets for therapeutic drug design.

Dietary intake of inulin-type fructans in active and inactive Crohn's disease and healthy controls: a case-control study.

BACKGROUND AND AIMS: Prebiotic inulin-type fructans are widely consumed in the diet and may have contrasting effects in Crohn's disease by stimulating gut microbiota and/or by generating functional gastrointestinal symptoms. The aim of this study was to measure fructan and oligofructose intakes in patients with active and inactive Crohn's disease compared with healthy controls. METHODS: Patients with active Crohn's disease (n = 98), inactive Crohn's (n = 99) and healthy controls (n = 106) were recruited to a case-control study. Dietary intake of inulin-type fructans was measured using a specific food frequency questionnaire and was compared between the three groups and between patients with different disease phenotypes (Montreal classification). Associations between intakes and disease activity (Harvey-Bradshaw Index, HBI) were also undertaken. RESULTS: Patients with active Crohn's disease had lower fructan intakes (median 2.9 g/d, interquartile range [IQR] 1.8) than those with inactive Crohn's (3.6 g/d, 2.1, p = 0.036) or controls (3.9 g/d, 2.1, p = 0.003) and lower oligofructose intakes (2.8 g/d, 1.8) than those with inactive Crohn's (3.5 g/d, 2.2, p = 0.048) or controls (3.8 g/d, 2.1, p = 0.003). There were no differences in intakes related to disease site or behaviour. There were negative correlations between HBI well-being score and fructan intake (ρ = -0.154, p = 0.03) and oligofructose intake (ρ = -0.156, p = 0.028) and for the HBI abdominal pain score and fructan (ρ = -0.164, p = 0.021) and oligofructose intake (ρ = -0.157, p = 0.027). CONCLUSIONS: Patients with active Crohn's disease consume lower quantities of fructans and oligofructose than their inactive counterparts and healthy controls. The impact of lower intakes of prebiotic fructans on gut microbiota is unknown and warrants further research.

Congenital lymphocytic choriomeningitis virus: when to consider the diagnosis.

Lymphocytic choriomeningitis virus is a rodent-borne arenavirus that can cause congenital infection affecting the developing central nervous system. When the infection occurs during pregnancy, the virus targets the fetal brain and retina, potentially causing ventriculomegaly, hydrocephalus, chorioretinitis, and neurodevelopmental abnormalities. It has been previously suggested that lymphocytic choriomeningitis virus be added to the list of congenital infections currently included in the TORCH acronym (toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis). We present 2 neonates with antenatally known ventriculomegaly that were diagnosed with congenital lymphocytic choriomeningitis virus infection after birth. In addition to ventriculomegaly, one had nonimmune hydrops fetalis and the other had intracranial hemorrhage. In view of the seroprevalence of lymphocytic choriomeningitis virus (4.7%-10%), our findings suggest that screening for congenital lymphocytic choriomeningitis virus infection should be considered in fetuses and newborns with ventriculomegaly as well as other abnormal neuroimaging findings such as intracranial hemorrhage.

Fermentable carbohydrate restriction reduces luminal bifidobacteria and gastrointestinal symptoms in patients with irritable bowel syndrome.

Preliminary studies indicate that dietary restriction of fermentable short-chain carbohydrates improves symptoms in irritable bowel syndrome (IBS). Prebiotic fructo-oligosaccharides and galacto-oligosaccharides stimulate colonic bifidobacteria. However, the effect of restricting fermentable short-chain carbohydrates on the gastrointestinal (GI) microbiota has never been examined. This randomized controlled trial aimed to investigate the effects of fermentable carbohydrate restriction on luminal microbiota, SCFA, and GI symptoms in patients with IBS. Patients with IBS were randomized to the intervention diet or habitual diet for 4 wk. The incidence and severity of symptoms and stool output were recorded for 7 d at baseline and follow-up. A stool sample was collected and analyzed for bacterial groups using fluorescent in situ hybridization. Of 41 patients randomized, 6 were withdrawn. At follow-up, there was lower intake of total short-chain fermentable carbohydrates in the intervention group compared with controls (P = 0.001). The total luminal bacteria at follow-up did not differ between groups; however, there were lower concentrations (P < 0.001) and proportions (P < 0.001) of bifidobacteria in the intervention group compared with controls when adjusted for baseline. In the intention-to-treat analysis, more patients in the intervention group reported adequate control of symptoms (13/19, 68%) compared with controls (5/22, 23%; P = 0.005). This randomized controlled trial demonstrated a reduction in concentration and proportion of luminal bifidobacteria after 4 wk of fermentable carbohydrate restriction. Although the intervention was effective in managing IBS symptoms, the implications of its effect on the GI microbiota are still to be determined.