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2020, Number 3

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Acta Pediatr Mex 2020; 41 (3)

Causes of hospitalizations in patients with inborn errors of intermediary metabolism: analyses of a series of cases in a third level hospital

Belmont-Martínez L, Ibarra-González IC, Vela-Amieva MB, Guillén-López S, López-Mejía L, Castillo-Razo IR, Francisco-Revilla-Estívil N, Cervantes-Bustamante R, Zárate-Mondragón F

Language: Spanish
References: 30
Page: 105-114
PDF size: 186.17 Kb.


ABSTRACT

Background: Inborn errors of metabolism (IEM) cause a significant morbidity and mortality suffered by pediatric hospital burden.
Objective: To analyze the quantity, type, cause, age at occurrence and evolution of the hospitalizations of patients with inborn errors of intermediate metabolism, in a third level pediatric care center.
Material and Methods: Retrospective analysis of the hospitalizations of a case series with IEM. Admission and discharge causes, clinical, demographic data, time and date hospitalizations and service that provided care were collected.
Results: In a 36 months period, 100 hospitalizations (88 urgent and 12 elective) occurred in 39 patients. Metabolic decompensations associated with gastrointestinal symptoms, infectious diseases and respiratory manifestations and propionate defects were the main causes of urgent hospitalizations (47.7%). 3 patients died (tyrosinemia, glutaric acidemia II and maple syrup urine disease). The main cause of elective hospitalization was for gastrostomy. Only 20.6% of patients had newborn screening for IEM. In our reference center, the patients with IEM contribute to 2.6% of emergency hospitalizations and 0.42% of total hospital admissions, with a mortality of 8.8%. We documented 2.6 hospitalizations of IEM patients per month, being most of them emergencies due to metabolic decompensation
Conclusions: Pediatricians must be trained to care for these patients and institutions must have human and economic resources to care for them.


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