Table 1.
Characteristics of familial dysautonomia subjects with afferent autonomic failure and healthy control subjects
| Characteristic | FD subjects (n = 34) | Healthy control subjects (n = 34) | P value |
|---|---|---|---|
| Age, y | 23 (15, 28) | 19 (16, 25) | 0.32 |
| Female, % | 47 | 50 | 1.00 |
| Body surface area, m2 | 1.42 (1.13, 1.51) | 1.59 (1.49, 1.88) | 0.001 |
| Supine systolic blood pressure, mm Hg | 137 (128, 147) | 117 (110,123) | <0.001 |
| Supine diastolic blood pressure, mm Hg | 77 (66, 83) | 68 (64, 71) | <0.001 |
| Supine mean blood pressure, mm Hg | 99 (83, 104) | 85 (8, 86) | <0.001 |
| Blood urinary nitrogen, mg/dl | 24 (19, 30) | 13 (12, 16) | <0.001 |
| Serum creatinine, mg/dl | 0.8 (0.7, 1.0) | 0.8 (0.7, 0.9) | 0.66 |
| Estimated glomerular filtration rate, ml/min | 79 (69, 101) | 113 (97, 127) | <0.001 |
| Proteinuria, % | 9 | NA | — |
| Blood glucose, mg/dl | 82 (73, 100) | 81 (76, 86) | 0.79 |
| Hemoglobin, g/100 ml | 12.4 (11.6, 13.2) | 14.4 (13.3, 15.2) | <0.001 |
| Medications, % | |||
| Clonidine | 35 | ||
| Beta-blocker | 6 | ||
| Amlodipine | 9 | ||
| Midodrine | 41 | ||
| Fludrocortisone | 35 | ||
| Benzodiazepines | 47 |
Data presented as median value (25th, 75th percentile) for continuous variables or percentages for dichotomous variables. P value reported for comparison of familiar dysautonomia subjects with afferent autonomic failure vs. healthy control subjects.
Abbreviations: FD, familial dysautonomia; NA, not available.