Table 2.
Clinical findings of CEAS patients (n = 46)
| Sex male/female | 13/33 |
| Age at diagnosis (years, median) | 40 (7–69) |
| Age at onset (years, median) | 16.5 (1–69) |
| Consanguinity | 13 (28%) |
| Family history | 10 (22%) |
| Past history of NSAIDs useb | 2 (4.5%) |
| NSAIDs use at diagnosisc | 0 (0%) |
| History of H. pylori infectiond | 5 (24%) |
| Symptoms | |
| Anemia | 45 (98%) |
| Abdominal pain | 18 (39%) |
| Edema | 11 (24%) |
| Diarrhea | 2 (4%) |
| Hematemesis | 1 (2%) |
| Hematochezia | 1 (2%) |
| Laboratory data at diagnosis | |
| Hemoglobin (g/dl, median) | 9.6 (2.3–13.7) |
| Serum protein (g/dl, median) | 5.2 (2.7–8.2) |
| CRP (mg/dl, median) | 0.20 (0–1.6) |
| Surgery | 29 (63%) |
| Extra-intestinal manifestations | |
| Digital clubbinga | 10 (22%) |
| Periostosisa,e | 11 (25%) |
| Acroosteolysise | 1 (2%) |
| Arthralgia of large joints | 7 (15%) |
| Knee-joint effusions | 4 (9%) |
| Hyperhidrosis | 4 (9%) |
| Pachydermiaa | 8 (17%) |
| Seborrhea | 3 (7%) |
| Acne | 7 (15%) |
| Flushing | 4 (9%) |
| Patent ductus arteriosus | 1 (2%) |
| Delayed cranial suture closure | 0 |
aThese manifestations are included in the major clinical criteria for PHO
Data are available for b 44, c 45, and d 21 patients, respectively
eData are available for 44 patients with X-ray evaluation