Table 1 Cases Analyzed
From: Acid ceramidase deficiency: Farber disease and SMA-PME
| Â | Cases mentioned in literature | Average age of onset | Average age of last documentation | Average age of death |
|---|---|---|---|---|
Classic & Severe FD | 102 | 5.8 ± 4.6 M (70) | 1.9 ± 1.8 Y (18) | 2.6 ± 6.0 Y (61) |
Mild & Intermediate FD | 40 | 1.5 ± 1.4 Y (34) | 14.9 ± 18.1 Y (26) | 14.3 ± 8.1 Y (8) |
FD (Unspecified) | 16 | – | – | – |
SMA-PME | 23 | 5.8 ± 4.2 Y (18) | 16.5 ± 5.7 Y (15) | 14.4 ± 3.0 Y (5) |
SMA-PME Like | 20 | 8.9 ± 7.38 (20) | 22.68 ± 17.8 Y (10) | 21.9 ± 17.2 Y (10) |
Total FD | 158 | Â | Â | Â |
Total SMA-PME | 43 | Â | Â | Â |