Inborn Errors of Metabolism in Pediatric Intensive Care Unit: Much More to Understand.

Abstract

We read with interest the recently published article titled “ Inborn Errors of Metabolism in a Tertiary Pediatric Intensive Care Unit ” by Lipari et al 1 and want to make few important comments. Authors enrolled 65 cases of inborn errors of metabolism (IEMs) with 88 admission to a pediatric intensive care unit (PICU) in Portugal over a period 11 years (2009 – 2019) accounting for 2% of PICU admissions. The children with intoxication disorders, energy metabolism defects, complex molecules, and other disorders accounted for 35.4% ( n ¼ 23), 32.3% ( n ¼ 21), 26.2% ( n ¼ 17), and 6.1% ( n ¼ 4), respectively. The median age at admission to PICU was 3 years (range: 3 days – 21 years) and 70.4% ( n ¼ 62) admissions were for metabolic decompensation and 29.5% ( n ¼ 26) were elective/scheduled surgery/procedure admissions. The reasons for decompensation included infections (55.4%, n ¼ 36) and metabolic stress during neonatal period (18.7%, n ¼ 12). The common clinical presentations were respiratory failure (34.1%, 30/88) and neurological deterioration (29.5%, 26/88). The treatment included mechanical ventilation ( n ¼ 30), continuous venovenous hemodia fi ltration (CVVHDF) ( n ¼ 16), speci fi c nutritional management, and supportive care. The median duration of PICU stay was 3.6 days (range: