Table 1.
Demographic Characteristics and Major Clinical Findings in 32 Patients With Niemann-Pick Disease Type C
| Variable | |
|---|---|
| Sex, n (%) | |
| Male | 17 (53) |
| Female | 15 (47) |
| Age at diagnosis, y | |
| Median | 3.7 |
| Range | 0.25–33 |
| Age at first anesthetic, y | |
| Median | 6.9 |
| Range | 1.8–33 |
| Weight, kg | |
| Mean | 30.4 |
| Range | 9–142 |
| ASA physical status, n (%) | |
| II | 3 (9) |
| III | 29 (91) |
| Clinical and laboratory findings, n (%) | |
| Hepatosplenomegaly | 31 (97) |
| Elevated transaminases | 31 (97) |
| Thrombocytopenia | 16 (50) |
| Pulmonary disease | 10 (31) |
| Cognitive impairment | 20 (63) |
| Ataxia | 26 (81) |
| Vertical gaze palsy | 26 (81) |
| Hearing problem | 22 (69) |
| Speech delay | 27 (84) |
| Dysphagia | 20 (63) |
| Behavior problems | 13 (41) |
| Seizure | 12 (38) |
ASA, American Society of Anesthesiologists. Pulmonary disease included chronic cough and/or history of aspiration pneumonia.