Predictors of outcome in children with disorders of mitochondrial metabolism in the pediatric intensive care unit

Ehinger, Johannes K.; Karlsson, Michael; Sjövall, Fredrik; Leffler, Märta; McCormack, Shana E.; Kubis, Sherri E.; Åkesson, Anna; Falk, Marni J.; Kilbaugh, Todd J.

BACKGROUND: The aim of this study was to identify factors predicting outcome in patients with mitochondrial disease admitted to pediatric intensive care units (PICU). METHODS: Retrospective study of 2434 patients (age <21 years) admitted to a PICU from 1 January 2006 through 31 March 2016 and captured in the Virtual Pediatric Systems database with ICD9 diagnosis 277.87, disorders of mitochondrial metabolism. Factors influencing mortality and prolonged length of stay (≥14 days) were analyzed using logistic regression. RESULTS: Predictors independently affecting mortality (adjusted odds ratios and 95% confidence intervals, p < 0.05): age 1–23 months 3.4 (1.7–6.6) and mechanical ventilation 4.7 (2.6–8.6) were risk factors; post-operative 0.2 (0.1–0.6), readmission 0.5 (0.3–0.9), and neurologic reason for admittance 0.3 (0.1–0.9) were factors reducing risk. Predictors affecting prolonged length of stay: mechanical ventilation 7.4 (5.2–10.3) and infectious reason for admittance 2.0 (1.3–3.2) were risk factors, post-operative patients 0.3 (0.2–0.5) had lower risk. The utility of PRISM and PIM2 scores in this patient group was evaluated. CONCLUSIONS: The single most predictive factor for both mortality and prolonged length of stay is the presence of mechanical ventilation. Age 1–23 months is a risk factor for mortality, and infectious reason for admittance indicates risk for prolonged length of stay. IMPACT: Presence of mechanical ventilation is the factor most strongly associated with negative outcome in patients with mitochondrial disease in pediatric intensive care. Age 1–23 months is a risk factor for mortality, and infectious reason for admittance indicates risk for prolonged length of stay. PRISM3 and PIM2 are not as accurate in patients with mitochondrial disease as in a mixed patient population.