RESUMEN
Cystic fibrosis is a multisystem disease with extremely variable onset, symptoms and course. One of the onset modality but also a complication of the disease is the pseudo-Bartter syndrome, characterized by hyponatremia, hypochloremic dehydration and metabolic alkalosis in absence of any renal disease. This syndrome occurs more frequently in the first year of life and has a peak in the summer. In this article, we describe two cases of cystic fibrosis associated with pseudo-Bartter syndrome in childhood. Excluding every possible cause of metabolic alkalosis associated with hyponatremia was crucial for our diagnostic pathway, and the experience gained with the first case helped a lot with the second one.
Asunto(s)
Síndrome de Bartter , Fibrosis Quística , Humanos , Fibrosis Quística/complicaciones , Fibrosis Quística/genética , Fibrosis Quística/diagnóstico , Síndrome de Bartter/complicaciones , Síndrome de Bartter/diagnóstico , Síndrome de Bartter/genética , Masculino , Femenino , Hiponatremia/etiología , Alcalosis/etiología , Preescolar , NiñoRESUMEN
Celiac Disease (CD) continues to require a strict lifetime gluten-free diet (GFD) to maintain healthy status. Many studies have assessed the GFD nutritional adequacy in their cohorts, but an overall picture in adults and children would offer a lifetime vision to identify actionable areas of change. We aimed at assessing the nutrient intakes of adult and pediatric CD patients following a GFD diet and identifying potential areas of improvement. Systematic review was carried out across PubMed, Scopus and Scholar up to October 2022, including full-text studies that assessed the nutrient intakes of CD patients on GFD, in terms of macro- and/or micronutrients (absolute or percentage daily average). Random-effect meta-analysis and univariable meta-regression were applied to obtain pooled estimates for proportions and influencing variables on the outcome, respectively. Thirty-eight studies with a total of 2114 patients were included. Overall, the daily energy intake was 1995 (CI 1884-2106) Kcal with 47.8% (CI 45.7-49.8%) from carbohydrates, 15.5% (CI 14.8-16.2%) from proteins, and 35.8% (CI 34.5-37.0%) from fats. Of total fats, 13.2% (CI 12.4-14.0%) were saturated fats. Teenagers had the highest consumption of fats (94.9, CI 54.8-134.9 g/day), and adults presented insufficient dietary fiber intake (18.9 g, CI 16.5-21.4 g). Calcium, magnesium, and iron intakes were particularly insufficient in adolescence, whereas vitamin D was insufficient in all age groups. In conclusion, GFD may expose CD patients to high fat and low essential micronutrient intakes. Given GFD is a lifelong therapy, to prevent the occurrence of diseases (e.g. cardiovascular or bone disorders) dietary intakes need to be assessed on long-term follow-ups.
Asunto(s)
Enfermedad Celíaca , Adolescente , Niño , Humanos , Adulto , Dieta Sin Gluten , Ingestión de Alimentos , Estado Nutricional , Grasas de la DietaRESUMEN
Obstructive sleep apnoea (OSA) is a very common disease with a prevalence that ranges from 1% to 6% in children. It is characterized by intermittent partial or complete occlusion of the upper airway during sleep, leading to recurrent arousals and disturbed sleep architecture, to neurocognitive disorders and alterations in homeostatic gas exchange. Cardiovascular complications may develop in children with OSA through various mechanisms including activation and dysregulation of the sympathetic nervous system, induction of pro-inflammatory and pro-oxidant status and increased risk of systemic hypertension. As the deleterious effects of OSA on the cardio-vascular system may start early in life, in this brief review we focused our attention both on early and late cardiological changes induced by apnoeic events in the paediatric population, by reviewing recent findings in the literature.