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Table 1 Clinical characteristics of PAN and DADA2 patients at the time of diagnosis

From: Choroidal vascularity index as a biomarker of systemic inflammation in childhood Polyarteritis Nodosa and adenosine deaminase-2 deficiency

 PAN (n = 11)DADA2 (n = 4)
Female/Male6/51/3
Age at symptom onset, median (min-max)4 (2–10)3.5 (1.5–14)
Age at diagnosis median (min-max)6 (3–12)4 (3–15)
Age at the OCT visit median (min-max)8 (4–16)6 (5–16)
Constitutional symptoms, n (%)8 (73%)4 (100%)
Musculoskeletal symptoms, n (%)10 (90%)3 (75%)
Cutaneous involvement, n (%)8 (73%)3 (75%)
Hypertension, n (%)0 (0%)1 (25%)
Renal involvement, n (%)0 (0%)1 (25%)
Neurologic involvement, n (%)1 (9%)0 (0%)
Testis involvement, n (%)1 (9%)1 (25%)
Gastrointestinal system involvement, n (%)9 (82%)3 (75%)
Cardiovascular involvement, n (%)0 (0%)0 (0%)
Pulmonary involvement, n (%)0 (0%)0 (0%)
Medication n (%)
 Steroid11 (100%)4 (100%)
 Biologic agent1 (9%)4 (100%)
 DMARD8 (83%)0 (0%)