Assessing the quality and value of metabolic chart data for capturing core outcomes for pediatric medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

Table 4 Age based rates of metabolic decompensation, estimated by one or two raters identifying a decompensation

Age Group	Number of potential events considered	No. events labelled as metabolic decompensations	Rate per 100 children per year (95% CI)^a
Overall
Definite	99	35	5.0 (3.6–6.9)
Definite + Possible		55	7.9 (6.0–10.2)
< 12 months
Definite		16	13.0 (7.8–21.0)
Definite + Possible	38	22	18.0 (11.7–27.1)
≥ 12 months
Definite		19	3.3 (2.1–5.1)
Definite + Possible	61	33	5.7 (4.0–8.0)

a) A single child may contribute to both age groups due to longitudinal follow-up
b) “Definite” = Chart-documented hypoglycemia or explicit documentation of metabolic decompensation, “Possible” = cases without chart-documented hypoglycemia prior to intravenous glucose administration
^a95% confidence intervals calculated using the exact Poisson distribution

ISSN: 1471-2431