Effects on growth, weight and body composition after CFTR modulators in children with cystic fibrosis.

BACKGROUND: Cystic Fibrosis (CF) is associated with compromised nutrition status, which is responsible for morbidity and mortality along with lung function decline. This study was designed to examine changes in anthropometric markers and body composition parameters by bioelectrical impedance analysis after CFTR modulator (CFTRm) treatment. METHODS: We compared anthropometric parameters and body composition before and after 6 and 12 months of CFTRm treatment. Results are stratified into subgroups according to the modulator used with dual therapy with lumacaftor + ivacaftor or tezacaftor + ivacaftor (LUMA/TEZ + IVA) or triple therapy with elexacaftor + tezacaftor + ivacaftor (ELE + TEZ + IVA). Body composition data are available in patients treated with ELE + TEZ + IVA. RESULTS: Two hundred and thirty-four children (55.1% male) were recruited. The median age was 13.6 years (inter-quartile range [IQR] 10.7-16.1). We can observe a statistically significant increase in the weight Z score and BMI Z score after CFTRm. In terms of changes in body composition, we observe a significant increase in fat mass (FM) expressed both in kilograms and as a percentage at 6 months (p < .05; Wilcoxon-test), with no such differences found at 12 months. We also observe a statistically significant increase in fat-free-mass (FFM), expressed in kilograms at 6 and 12 months (p < .05; Wilcoxon-test). CONCLUSION: Weight status improved and changes in body composition occurred in children after CFTRm therapy, including an increase of fat mass. Further studies are needed to confirm these changes in body composition and their impact on disease progression.

[Prevalence of ankyloglossia in newborns in Asturias (Spain)]. / Prevalencia de anquiloglosia en recién nacidos en el Principado de Asturias.

INTRODUCTION: The prevalence of ankyloglossia has been estimated at around 4% of live births. Its prevalence at national level is unknown. MATERIAL AND METHODS: Multicenter, prospective observational study. Six hospitals in Asturias took part. All newborns were examined on Sundays, Tuesdays and Thursdays for 3 months. Coryllos and Hazelbaker criteria were used to diagnose ankyloglossia. RESULTS: The prevalence in the 667 newborns examined was 12.11% (95% CI: 9.58 to 14.64), of whom 62% were male. One in 4 children with ankyloglossia had a family history. According to Coryllos' classification, type II was the most common (54%). CONCLUSIONS: The prevalence of ankyloglossia in Asturias was 2 to t3 times higher than expected. The diagnostic criteria for ankyloglossia needs to be unified, and further studies are required to determine the association with breastfeeding difficulties and other health problems.

[Oral rush desensitization for cow milk allergy: Clinical and immunological follow-up]. / Eficacia y seguridad de una pauta rush de inducción de tolerancia oral en pacientes con alergia a proteínas de leche de vaca: evolución clínica e inmunológica.

OBJECTIVE: The aim of this study was to evaluate the safety and efficacy of oral rush desensitization in children with cow milk allergy. MATERIAL AND METHODS: Prospective study. We included IgE-mediated cow milk allergy children over 3 years in 3 Spanish hospitals. Increasing doses of cow milk for 5 days from 1 cc of 1% to 200 cc of pure milk were administered. Clinical follow-up was conducted and we compared specific IgE levels at onset, 6, 12 and 24 months after desensitization. RESULTS: We included 18 children (13 males) between 3 and 14 years (mean 5.96). A total of 271 doses were administered; there were 55 adverse reactions (84% mild). At the end of the protocol, 100% showed some degree of tolerance (39% total). Full tolerance was achieved in 72% of patients after two years. Two children failed to achieve tolerance. There was a significant decrease in the levels of specific IgE to cow milk and alpha-lactalbumin at 24 months, and to casein at 6, 12 and 24 months, compared to baseline. CONCLUSIONS: Oral rush desensitization is a safe and effective therapeutic option for patients with persistent cow milk allergy to medium term.

[Insulin resistance in overweight cystic fibrosis paediatric patients]. / Resistencia insulínica en pacientes pediátricos con fibrosis quística y sobrepeso.

AIM: To determine the prevalence of overweight in paediatric patients with cystic fibrosis (CF) and to analyse its role as diabetogenic insulin resistance factor and risk of hypertriglyceridaemia. PATIENTS AND METHODS: A total of 109 CF patients (47% males) between 5 and 18 years were divided into 3 groups according to body mass index (BMI): underweight, normal and overweight. Total cholesterol, triglycerides, C- reactive protein (CRP), glycosylated haemoglobin, HOMA-IR and QUICKI index were determined. Insulinogenic index, ISI composite and areas under the curve (AUC) for glucose and insulin were obtained by oral glucose tolerance test (OGTT). RESULTS: Six patients (5.5%) were overweight. All groups had similar distribution by age, sex and CFTR mutation, although the proportion of pancreatic sufficient (3/6, 50%) was higher in overweight patients (P=.003). The prevalence of glycaemic disorders was similar between groups. Baseline insulin, HOMA-IR, and insulin during the OGTT (peak and AUC) were higher in overweight patients. All patients had a delayed insulin response in OGTT. CONCLUSIONS: Overweight CF patients do not have a higher incidence of glycaemic disorders, but their hyperinsulinism and insulin resistance may be additional diabetogenic risk factors.

[Gastrointestinal disorders in children with cerebral palsy and neurodevelopmental disabilities]. / Patología gastrointestinal en niños con parálisis cerebral infantil y otras discapacidades neurológicas.

Recent data suggest that, contrary to initial expectations with improvements in perinatal medicine, the prevalence of cerebral palsy has not decreased over the last 20 years. Gastrointestinal disorders are a major chronic problem in most of children with cerebral palsy and in children with neurodevelopmental disabilities. A multidisciplinary approach, with input from neurologists, gastroenterologists, nurses, dieticians and other specialists, can make a major contribution to the medical wellbeing and quality of life of these children and their caregivers. This article focuses on diagnostic methods and therapeutic options available for major nutritional and gastrointestinal problems in patients with neurological disabilities: gastroesophageal reflux, constipation and swallowing disorders.