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Abstract
BackgroundPatients with isolated methylmalonic acidemia (MMA) usually experience recurrent episodes of acute metabolic decompensation or metabolic stroke, require frequent hospitalization, and have a relatively high mortality rate. The aim of our study was to assess factors predicting the in-hospital death of pediatric patients with isolated MMA. We performed a retrospective study using data from the Hospital Quality Monitoring System, a national inpatient database in China collected from 2013 to 2017. All patients under 18 years old with a diagnosis of isolated MMA were included. Demographic, hospital-related, and clinical features were collected. Poisson regression was performed to identify potential influencing variables associated with in-hospital death.ResultsFrom 2013 to 2017, among 2317 admissions for pediatric patients diagnosed with isolated MMA, 1.77% had the outcome of death. In the univariate analysis, patients aged under 1 year had a higher risk of death than did those aged 1 year or older (odds ratio [OR] = 2.63, 95% confidence interval [CI]: 1.36–5.07). There was a higher risk of in-hospital death for patients admitted through emergency departments or via referrals than for those admitted through other routes (OR = 3.76, 95% CI: 1.84–7.67). Deaths were higher in hospitals with volumes of less than 50 patients with isolated MMA during the five study years (OR = 2.92, 95% CI: 1.46–5.83). Moreover, the risk of in-hospital death gradually decreased over time (OR = 0.72, 95% CI: 0.57–0.90). In the multivariate analysis, the abovementioned associations with the risk of in-hospital death remained statistically significant. However, no significant associations were observed between specific clinical signs and in-hospital death in either the univariate or the multivariate analysis.ConclusionsYounger age, admission to hospitals with low patient volumes, and admission through emergency departments or referrals are associated with higher risk of in-hospital death. The co-existence of specific clinical signs appears to have no effect on in-hospital death.
Highlights
Methylmalonic acidemia (MMA) is a rare autosomal recessive disorder of propionic acid metabolism induced by the deficiency of methylmalonyl-CoA mutase or in the synthesis of its cofactor adenosylcobalamin [1]
Younger age, admission to hospitals with low patient volumes, and admission through emergency departments or referrals are associated with higher risk of in-hospital death
The co-existence of specific clinical signs appears to have no effect on in-hospital death
Summary
Methylmalonic acidemia (MMA) is a rare autosomal recessive disorder of propionic acid metabolism induced by the deficiency of methylmalonyl-CoA mutase or in the synthesis of its cofactor adenosylcobalamin [1]. This disease is characterized by an elevated concentration of methylmalonic acid in the patient’s blood and urine, which results from the failure to convert methylmalonylCoA into succinyl-CoA during propionyl-CoA metabolism. Patients with isolated methylmalonic acidemia (MMA) usually experience recurrent episodes of acute metabolic decompensation or metabolic stroke, require frequent hospitalization, and have a relatively high mortality rate. Poisson regression was performed to identify potential influencing variables associated with in-hospital death
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