Rosaura Leis

Vitamin and mineral status in patients with hyperphenylalaninemia.

Natural sources of protein and some vitamins and minerals are limited in phenylketonuria (PKU) treated patients, who should receive optimal supplementation although this is not yet fully established. We conducted a cross-sectional observational multicenter study including 156 patients with hyperphenylalaninemia. Patients were stratified by age, phenotype, disease detection and type of treatment. Annual median blood phenylalanine (Phe) levels, Phe tolerance, anthropometric measurements, and biochemical parameters (total protein, prealbumin, electrolytes, selenium, zinc, B12, folic acid, ferritin, 25-OH vitamin D) were collected in all patients. 81.4% of patients had biochemical markers out of recommended range but no clinical symptoms. Total protein, calcium, phosphorus, B12, ferritin, and zinc levels were normal in most patients. Prealbumin was reduced in 34.6% of patients (74% with PKU phenotype and 94% below 18 years old), showing almost all (96.3%) an adequate adherence to diet. Selenium was diminished in 25% of patients (95% with PKU phenotype) and also 25-OHD in 14%. Surprisingly, folic acid levels were increased in 39% of patients, 66% with classic PKU. Phosphorus and B12 levels were found diminished in patients with low adherence to diet. Patients under BH4 therapy only showed significant lower levels of B12. This study shows a high percentage of prealbumin and selenium deficiencies as well as an increased level of folic acid in PKU treated patients, which should lead us to assess an adjustment for standards supplements formulated milks.

Metformin for Obesity in Prepubertal and Pubertal Children: A Randomized Controlled Trial

The present study is an evaluation of metformin’s effect on BMI z score, insulin sensitivity, and inflammation and cardiovascular risk factors in prepubertal and pubertal children who are obese. OBJECTIVES: Metformin has shown its effectiveness in treating obesity in adults. However, little research has been conducted in children, with a lack of attention on pubertal status. The objectives were to determine whether oral metformin treatment reduces BMI z score, cardiovascular risk, and inflammation biomarkers in children who are obese depending on pubertal stage and sex. METHODS: This was a randomized, prospective, double-blind, placebo-controlled, multicenter trial, stratified according to pubertal stage and sex, conducted at 4 Spanish clinical hospitals. Eighty prepubertal and 80 pubertal nondiabetic children who were obese aged 7 to 14 years with a BMI >95th percentiles were recruited. The intervention included 1 g/d of metformin versus placebo for 6 months. The primary outcome was a reduction in BMI z score. Secondary outcomes comprised insulin resistance, cardiovascular risk, and inflammation biomarkers. RESULTS: A total of 140 children completed the study (72 boys). Metformin decreased the BMI z score versus placebo in the prepubertal group (−0.8 and −0.6, respectively; difference, 0.2; P = .04). Significant increments were observed in prepubertal children treated with metformin versus placebo recipients in the quantitative insulin sensitivity check index (0.010 and −0.007; difference, 0.017; P = .01) and the adiponectin–leptin ratio (0.96 and 0.15; difference, 0.81; P = .01) and declines in interferon-γ (−5.6 and 0; difference, 5.6; P = .02) and total plasminogen activator inhibitor-1 (−1.7 and 2.4; difference, 4.1; P = .04). No serious adverse effects were reported. CONCLUSIONS: Metformin decreased the BMI z score and improved inflammatory and cardiovascular-related obesity parameters in prepubertal children but not in pubertal children. Hence, the differential response according to puberty might be related to the dose of metformin per kilogram of weight. Further investigations are necessary.

Prevalence of Functional Gastrointestinal Disorders in Children and Adolescents in the Mediterranean Region of Europe

BACKGROUND & AIMS: Little is known about the prevalence of functional gastrointestinal disorders (FGIDs) in children from the Mediterranean area of Europe. We aimed to assess the prevalence of FGIDs in children and adolescents in this region. METHODS: We collected data on 13,750 children (4–18 years old) enrolled in the Mediterranean–European Area Project, a school‐based health study performed in Croatia, Greece, Israel, Italy, Jordan, Lebanon, Macedonia, Serbia, and Spain. Data were collected from March to June and in September of 2016. We analyzed data from 6602 students 4 to 10 years old (group A; mean age, 7.7 ± 1.9 y), and 7148 subjects 11 to 18 years old (group B; mean age, 13.8 ± 2.1 y). Children with FGIDs were identified based on answers to questionnaires on pediatric gastrointestinal symptoms, selected based on Rome III criteria. RESULTS: In group A, the prevalence of FGIDs was 20.7%. The most frequent disorders were functional constipation (11.7%), irritable bowel syndrome (IBS, 4%), aerophagia (3.5%), and abdominal migraine (3.1%). The prevalence of abdominal migraine was significantly higher in girls than in boys (P = .007). In group B, the overall prevalence of FGIDs was 26.6%. The most frequent disorders were functional constipation (13.1%), abdominal migraine (7.8%), aerophagia (6.3%), and IBS (5.6%). In group B, FGIDs had a higher prevalence among girls than boys (P < .001). In both groups, we found significant differences in the prevalence of specific disorders among specific countries. CONCLUSIONS: In an analysis of data on children 4 to 18 years old from the Mediterranean–European Area Project, we found FGIDs to be more frequent in girls. Functional constipation, aerophagia, abdominal migraine, and IBS are the most common disorders. However, the prevalence of FGIDs varies significantly among countries.

Micronutrient in hyperphenylalaninemia.

The data presented here are the biochemical parameters of 156 patients with hyperphenylalaninemia. PKU patients, who, in order to maintain optimal serum Phe concentrations, receive dietary treatment consisting of a diet low in natural protein supplemented with special low protein foods and a Phe-free amino acid mixture, vitamins and minerals. The obtained data reflects a high percentage of patients with prealbumin and selenium deficiencies, as well as an increased level of folic acid. This data article is related to the research article entitled, “Vitamin and mineral status in patients with hyperphenylalaninemia”, by Crujeiras et al. [1].

El yogur y recomendaciones dietéticas en la intolerancia a la lactosa

Malabsorption to lactose is caused by the inability to digest sugar due to the decrease in the activity of intestinal lactase. Malabsorption may be due to a primary or secondary disorder. Adult type primary hypolactasia is an autosomal recessive disorder, characterized by the progressive loss of lactase after weaning. The secondary hypolactasia is a transitory disorder, which will be corrected after the cure of the basic pathology. For lactose malabsorption diagnosis, the hydrogen and methane exhaled tests after lactose overload stand out and, in the case of the primary adult type, the molecular test of the simple nucleotide polymorphism (SNP C / T-13910). However, the diagnosis of lactose intolerance requires the presence of symptoms after consumption. The treatment of primary adult-type hypolactasia consists in decreasing the lactose in the diet below the trigger dose. A significant percentage of individuals with malabsorption tolerate habitual amounts of consumption. Practically 99% of them tolerate yogurt or fermented dairy products, thus allowing to cover the daily recommendations of calcium and vitamin D intake. In addition, nutritional strategies that reduce the lactose load, gastric emptying time and / or intestinal transit time or increase lactic activity and colonic compensation, will allow a greater tolerance.