| Autor: |
Iris L. Rodenburg, Francjan J. van Spronsen, Esther van Dam, Charlotte M A Lubout, Marieke J Fokkert-Wilts, Diego Martinelli, Sarah C. Grünert, Tessa van Amerongen, Foekje de Boer, Frouke J. Weynschenk, Terry G J Derks, Irene J. Hoogeveen |
| Rok vydání: |
2020 |
| Předmět: |
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| Popis: |
Background - Patients with inborn errors of metabolism causing fasting intolerance are at risk of acute metabolic decompensations. Disease specific emergency protocols are widely available, but long-term data on safety and efficacy outcomes are lacking. We hypothesized that a generic emergency protocol can be safe and effective in patients with inborn errors of metabolism causing fasting intolerance. Results - We retrospectively evaluated our generic emergency protocol in 128 patients with a hepatic glycogen storage disease or fatty acid oxidation defect between February 1, 2014 and April 24, 2019. In total, 127 hospital admissions were documented in 54 out of 128 patients (42%). Hypoglycemia (glucose concentration |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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