Table 4.
Clinical–immunological findings and therapies of 110 patients with an initial diagnosis of unPAD, according to age at diagnosis (<4 and >4 years).
| unPAD (110 pts) | <4 Years (59 pts) |
>4 Years (51 pts) |
p Value | |
|---|---|---|---|---|
| CLINICAL MANIFESTATIONS | 104/110 (94%) | 55/59 (93%) | 49/51 (96%) | ns |
| Infections | 84/104 (80%) | 46/55 (83%) | 38/49 (77%) | ns |
| RRTI | 26/84 (31%) | 8/46 (17%) | 18/38 (47%) | 0.04 |
| Otitis | 28/84 (33%) | 16/46 (35%) | 12/38 (32%) | ns |
| Bronchiolitis | 17/84 (20%) | 14/46 (30%) | 3/38 (8%) | 0.01 |
| Bronchitis | 11/84 (13%) | 6/46 (13%) | 5/38 (13%) | ns |
| Sinusitis | 13/84 (15%) | 8/46 (17%) | 5/38 (13%) | ns |
| Pneumonia | 26/84 (31%) | 9/46 (19%) | 17/38 (45%) | 0.018 |
| Gastroenteritis | 14/84 (17%) | 11/46 (24%) | 3/38 (8%) | ns |
| Viral Hepatitis | 1/84 (1%) | 1/46 (2%) | 0/38 (0%) | ns |
| UTI | 7/84 (8%) | 6/46 (13%) | 1/38 (3%) | ns |
| Meningitis | 2/84 (2%) | 0/46 (0%) | 2/38 (5%) | ns |
| Skin Abscesses | 5/84 (6%) | 0/46 (0%) | 5/38 (13%) | 0.016 |
| Giardiasis | 2/84 (2%) | 2/46 (4%) | 0/38 (0%) | ns |
| Rheumatic disease | 2/84 (2%) | 2/46 (4%) | 0/38 (0%) | ns |
| Skin infections | 1/84 (1%) | 1/46 (2%) | 0/38 (0%) | ns |
| Allergy | 37/104 (36%) | 14/55 (25%) | 23/49 (47%) | 0.0257 |
| Food Allergy | 8/37 (22%) | 4/14 (29%) | 4/23 (17%) | ns |
| Atopic Dermatitis | 15/37 (40%) | 6/14 (43%) | 9/23 (39%) | ns |
| Rhinitis | 23/37 (62%) | 9/14 (64%) | 14/23 (61%) | ns |
| Urticaria | 5/37 (13%) | 2/14 (14%) | 3/23 (13%) | ns |
| Asthma | 12/37 (32%) | 3/14 (21%) | 9/23 (39%) | ns |
| Conjunctivitis | 12/37 (32%) | 4/14 (29%) | 8/23 (35%) | ns |
| Angioedema | 1/37 (3%) | 1/14 (7%) | 0/23 (0%) | ns |
| Vernal keratoconjunctivitis | 1/37 (3%) | 0/14 (0%) | 1/23 (4%) | ns |
| Autoimmunity | 9/104 (9%) | 2/55 (4%) | 7/49 (14%) | ns |
| Hashimoto Thyroiditis | 1/9 (11%) | 1/2 (50%) | 0/7 (0%) | ns |
| Type 1 Diabetes | 1/9 (11%) | 0/2 (0%) | 1/7 (14%) | ns |
| Psoriasis | 1/9 (11%) | 0/2 (0%) | 1/7 (14%) | ns |
| Celiac Disease | 3/9 (33%) | 0/2 (0%) | 3/7 (43%) | ns |
| Alopecia | 1/9 (11%) | 0/2 (0%) | 1/7 (14%) | ns |
| Nephrotic syndrome | 2/9 (22%) | 0/2 (0%) | 2/7 (29%) | ns |
| Glomerulonephritis | 1/9 (11%) | 1/2 (50%) | 0/7 (0%) | ns |
| Non-infective pulmonary diseases | 2/104 (2%) | 2/55 (4%) | 0/49 (0%) | ns |
| Bronchiectasis | 1/2 (50%) | 1/2 (50%) | - | - |
| Cystic Fibrosis | 1/2 (50%) | 1/2 (50%) | - | - |
| Benign lymphoproliferation | 4/104 (4%) | 2/55 (4%) | 2/49 (4%) | ns |
| Lymphadenopathies | 3/4 (75%) | 2/2 (100%) | 1/2 (50%) | ns |
| Splenomegaly | 1/4 (25%) | 0/2 (0%) | 1/2 (50%) | ns |
| Hepatomegaly | 0/4 (0%) | 0/2 (0%) | 0/2 (0%) | ns |
| Neoplasia | 0/104 (0%) | - | - | - |
| HOSPITALIZATION | 28/110 (25%) | 15/59 (25%) | 13/51 (25%) | ns |
| Pneumonia | 10/28 (36%) | 2/15 (13%) | 8/13 (61%) | 0.016 |
| Meningitis | 2/28 (7%) | 0/15 (0%) | 2/13 (15%) | ns |
| Bronchiolitis | 6/28 (21%) | 4/15 (27%) | 2/13 (15%) | ns |
| Gastroenteritis | 3/28 (11%) | 3/15 (20%) | 0/13 (0%) | ns |
| UTI | 2/28 (7%) | 1/15 (7%) | 1/13 (8%) | ns |
| RRTI | 7/28 (25%) | 2/15 (13%) | 5/13 (38%) | ns |
| Cellulitis | 1/28 (4%) | 0/15 (0%) | 1/13 (8%) | ns |
| Mononucleosis | 1/28 (4%) | 1/15 (7%) | 0/13 (0%) | ns |
| Rheumatic disease | 2/28 (7%) | 1/15 (7%) | 1/13 (8%) | ns |
| IMMUNOLOGICAL ABNORMALITIES | 110/110 (100%) | 59/59 (100%) | 51/51 (100%) | ns |
| Isolated or combined IgG defect | 81/110 (74%) | 45/59 (76%) | 36/51 (71%) | ns |
| Isolated or combined IgA defect | 52/110 (47%) | 37/59 (63%) | 15/51 (29%) | 0.0006 |
| Isolated or combined IgM defect | 60/110 (54%) | 34/59 (58%) | 26/51 (51%) | ns |
| IgG subclass deficiency | 59/84 (70%) | 33/43 (77%) | 26/41 (63%) | ns |
| Poor specific antibody response to tetanus | 8/54 (15%) | 4/35 (11%) | 4/19 (21%) | ns |
| Poor specific antibody response to pneumococcus | 13/43 (30%) | 10/27 (37%) | 3/16 (19%) | ns |
| Low switched memory B cells | 22/44 (50%) | 13/26 (50%) | 6/18 (33%) | ns |
| Low IgM memory B cells | 4/44 (9%) | 1/26 (4%) | 3/18 (17%) | ns |
| THERAPIES | ||||
| Ig Replacement therapy | 3/110 (3%) | 2/59 (3%) | 1/51 (2%) | ns |
| Antibiotic Prophylaxis | 7/110 (6%) | 3/59 (5%) | 4/51 (8%) | ns |