From: Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study
n | % | 95% CI | IR per 100,000 person yearsa | 95% CI | |
---|---|---|---|---|---|
I. Metabolic | 55 | 65.5 | 54.3–75.5 | 4.21 | 3.23–5.49 |
A. Subcellular organelles | 28 | 33.3 | 23.4–44.5 | 2.14 | 1.48–3.11 |
Lysosomal | 23 | 27.4 | 18.2–38.2 | 1.76 | 1.17–2.65 |
Mitochondrial | 3 | 3.6 | 0.7–10.1 | 0.23 | 0.07–0.71 |
Peroxisomal | 2 | 2.4 | 0.3–8.3 | 0.15 | 0.04–0.61 |
B. Intermediate metabolism | 27 | 32.1 | 22.4–43.2 | 2.07 | 1.42–3.02 |
Organic aciduria | 11 | 13.1 | 6.7–22.2 | 0.84 | 0.47–1.52 |
Fatty acid oxidation defect | 6 | 7.1 | 2.7–14.9 | 0.46 | 0.21–1.02 |
Urea cycle disorders | 4 | 4.8 | 1.3–11.7 | 0.31 | 0.12–0.82 |
Galactosemia | 4 | 4.8 | 1.3–11.7 | 0.31 | 0.12–0.82 |
Unspecified | 2 | 2.4 | 0.3–8.3 | 0.15 | 0.04–0.61 |
II. Neurodegenerative | 27 | 32.1 | 22.4–43.2 | 2.10 | 1.42–3.02 |
A. Specified | 10 | 11.9 | 5.9–20.8 | 0.77 | 0.41–1.42 |
B. Unspecified | 17 | 20.2 | 12.3–30.4 | 1.30 | 0.81–2.09 |
III. Infectious | 2 | 2.4 | 0.3–8.3 | 0.15 | 0.04–0.61 |
Total | 84 | 100 | - | 6.43 | 5.19–7.97 |