Highlights of the ESPEN‐ESPGHAN‐ECFS Guidelines on Nutrition Care for Infants and Children With Cystic Fibrosis

Abstract

T he European Society for Clinical Nutrition and Metabolism (ESPEN) launched a process of developing updated guidelines on nutrition care for infants, children, and adults with cystic fibrosis (CF). To that end, a group of experts systematically reviewed the medical literature to summarize current knowledge on epidemiology and pathophysiology, prevention, and treatment of CF-related undernutrition. The group included representatives from several European countries, physicians, dietitians, and educators, all experts in the field of CF, and the guidelines coordinator. The group recommended evidence-based guidelines on nutrition care for infants, children, and adults with CF. The experts followed the GRADE method, which was based on determinations of grade of evidence and strength of recommendation. The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESP-GHAN) and the European Society for Cystic Fibrosis (ECFS), who are partners of these guidelines, reviewed the final manuscript, with an external review for ESPGHAN. The reader of the Journal is kindly invited to refer to the original article published in the June 2016 issue of Clinical Nutrition (1). This summary will outline some of the recommendations on nutrition care for infants and children with CF.

Mortalidad en Fibrosis Quística. Análisis Retrospectivo De Cinco Años en Institución De Alta Complejidad. Medellín-Colombia

“…El estudio colombiano del 2004 reportó que el 29.7% de los pacientes presentaba desnutrición global (definida por un indicador Peso/Edad por debajo de -2 DE), sin embargo, no se pudo establecer el indicador Peso/Talla en el 60% de ellos, y muy posiblemente las cifras de desnutrición en esta población son mayores (3) . El beneficio de mantener un adecuado estado nutricional en estos pacientes obliga a las instituciones donde se realiza la atención y seguimiento, a brindar una adecuada asistencia en nutrición, acorde a las guías establecidas para la enfermedad (23,24) . En este estudio se evidenció, que ningún paciente tuvo un seguimiento ambulatorio con nutricionista por falta de autorización de sus aseguradoras y todos tenían compromiso nutricional.…”

Management of Exocrine Pancreatic Insufficiency in Children

“…This mandates that the clinician has a high index of suspicion for this condition and highlights the importance of early laboratory evaluation . Growth failure in EPI is often multifactorial and is attributed to the mismatch between reduced intake, intestinal malabsorption and excessive losses, and increased energy needs . Most of the inherited syndromes with EPI are multisystem disorders, and the presence of extra‐GI manifestations will also help direct the diagnosis (Table ).…”

“…The α‐tocopherol level is usually measured in conjunction with fats or cholesterol and expressed as a ratio . A plasma α‐tocopherol/cholesterol ratio of >5.4 mg/g is preferred in patients with CF . Vitamin E deficiency, once frequent in CF patients without supplementation, is now uncommon .…”

“…Poor bone health does negatively impact the post‐lung transplant status . Guidelines from ESPEN, ESPGHAN, and ECFS recommend bone‐density assessment using dual‐energy X‐ray absorptiometry (DEXA) to be performed from 8 to 10 years of life for children with CF and to be repeated every 1–5 years based on past measurements or the presence of risk factors such as steroid therapy …”

Nutritional characterization of children and adolescents with cystic fibrosis

“…Postulándose a la fecha, que el niño debe recibir una alimentación balanceada teniendo como parámetros de evaluación de requerimientos (2). energéticos, sus curvas de ganancia en peso y talla Si con un aporte energético común para un niño sano, el niño con FQ crece y se desarrolla normalmente, no ameritaría un aporte extra sino, una vigilancia cercana para detectar cualquier (2,4,40) necesidad de intervención oportuna .…”