Dietary practices in methylmalonic acidaemia: a European survey.

Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0-6 months; 7-12 months; 1-10 years; 11-16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice.

Perceived food intolerance and irritable bowel syndrome in a population 3 years after a giardiasis-outbreak: a historical cohort study.

BACKGROUND: Studies have shown an increased prevalence of irritable bowel syndrome (IBS) after acute gastroenteritis. Food as a precipitating and perpetuating factor in IBS has gained recent interest, but food intolerance following gastroenteritis is less investigated. The aims of this study were firstly, to compare perceived food intolerance in a group previously exposed to Giardia lamblia with a control group; secondly, to explore the relation with IBS status; and thirdly, to investigate associations with content of fermentable oligosaccharides, disaccharides, monosaccharides and polyols (FODMAP) in foods reported. METHODS: This is a historical cohort study with mailed questionnaire to 1252 Giardia exposed and a control cohort matched by gender and age. Differences between groups were investigated using bivariate and multivariate analyses. RESULTS: The questionnaire response rate in the exposed group was 65.3 % (817/1252) and in the control group 31.4 % (1128/3598). The adjusted odds ratio (OR) for perceived food intolerance for the exposed group was 2.00 with 95 % confidence interval (CI): 1.65 to 2.42, as compared with the control group. Perceived intolerance for dairy products was the most frequently reported intolerance, with an adjusted OR for the exposed of 1.95 (95 % CI: 1.51 to 2.51). Perceived intolerance for fatty foods, vegetables, fruit, cereals and alcohol was also significantly higher in the exposed group. The groups did not differ in perceived intolerance to spicy foods, coffee or soda. The association between exposure to Giardia infection and perceived food intolerance differed between the IBS group and the no-IBS group, but IBS was not a significant effect modifier for the association. Perceived intolerance for high FODMAP foods (adjusted OR 1.91) and low FODMAP foods (adjusted OR 1.55) was significantly associated with exposure status. CONCLUSION: Exposure to Giardia infection was associated with perceived food intolerance 3 years after giardiasis. IBS status did not alter the association between exposure status and perceived food intolerance. Perceived intolerance to high FODMAP foods and low FODMAP foods were both statistically significantly associated with exposure to Giardia infection.

Patients with subjective food hypersensitivity: the value of analyzing intestinal permeability and inflammation markers in gut lavage fluid.

BACKGROUND/AIM: Subjective food hypersensitivity is prevalent in the general population. The aim of this study was to seek objective evidence of food hypersensitivity by analyzing intestinal permeability and inflammation markers in gut lavage fluid. METHODS: Fifty-two patients with abdominal complaints self-attributed to food hypersensitivity were examined by skin prick test, serum IgE analysis, double-blind, placebo-controlled food challenge (DBPCFC), and intestinal lavage. The results were compared with those of 44 patients without food hypersensitivity. Neither the patients nor the controls had organic diseases that could explain their symptoms. Intestinal lavage was performed by administering 2 liters of isotonic polyethylene glycol (molecular weight 3,350 daltons) solution containing 50 microCi of [51Cr]EDTA through a nasoduodenal tube. The first clear fluid passed per rectum was collected and analyzed for histamine, eosinophilic cationic protein (ECP), tryptase, and calprotectin. The intestinal permeability was assessed by determining the 5-hour urinary excretion of [51Cr]EDTA. Calprotectin was also analyzed in native faecal samples. RESULTS: The ECP concentration in gut lavage fluid was significantly higher in the patients than in the controls (p = 0.007), but the overlap between groups was large. Food hypersensitivity was confirmed by positive DBPCFC in only 4 patients. On average, histamine and ECP concentrations were high in these patients. Tryptase, intestinal permeability, and faecal and lavage calprotectin levels were normal. CONCLUSIONS: Very few patients had objective evidence of food hypersensitivity. Analyzing intestinal permeability and inflammation markers in gut lavage fluid did not contribute to the diagnosis, but further studies on histamine and ECP are warranted.