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J Inherit Metab Dis ; 44(5): 1124-1135, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-33844307

RESUMEN

Patients with inborn errors of metabolism causing fasting intolerance can experience acute metabolic decompensations. Long-term data on outcomes using emergency letters are lacking. This is a retrospective, observational, single-center study of the use of emergency letters based on a generic emergency protocol in patients with hepatic glycogen storage diseases (GSD) or fatty acid oxidation disorders (FAOD). Data on hospital admissions, initial laboratory results, and serious adverse events were collected. Subsequently, the website www.emergencyprotocol.net was generated in the context of the CONNECT MetabERN eHealth project following multiple meetings, protocol revisions, and translations. Representing 470 emergency protocol years, 127 hospital admissions were documented in 54/128 (42%) patients who made use of emergency letters generated based on the generic emergency protocol. Hypoglycemia (here defined as glucose concentration < 3.9 mmol/L) was reported in only 15% of hospital admissions and was uncommon in patients with ketotic GSD and patients with FAOD aged >5 years. Convulsions, coma, or death was not documented. By providing basic information, emergency letters for individual patients with hepatic GSD or the main FAOD can be generated at www.emergencyprotocol.net, in nine different languages. Generic emergency protocols are safe and easy for home management by the caregivers and the first hour in-hospital management to prevent metabolic emergencies in patients with hepatic GSD and medium-chain Acyl CoA dehydrogenase deficiency. The website www.emergencyprotocol.net is designed to support families and healthcare providers to generate personalized emergency letters for patients with hepatic GSD and the main FAOD.


Asunto(s)
Tratamiento de Urgencia/métodos , Enfermedad del Almacenamiento de Glucógeno Tipo I/metabolismo , Hipoglucemia/terapia , Errores Innatos del Metabolismo Lipídico/metabolismo , Telemedicina , Adolescente , Adulto , Niño , Preescolar , Ayuno , Ácidos Grasos/metabolismo , Femenino , Enfermedad del Almacenamiento de Glucógeno Tipo I/fisiopatología , Humanos , Hipoglucemia/etiología , Lactante , Recién Nacido , Errores Innatos del Metabolismo Lipídico/fisiopatología , Masculino , Persona de Mediana Edad , Oxidación-Reducción , Estudios Retrospectivos , Adulto Joven
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