Table 1 Clinical data
From: X-linked Hyper IgM (HIGM1) in an African kindred: the first report from South Africa
Patient No. | Age at investigation | Immunoglobulin levels (initial) | CD40L status | Treatment and Outcome |
|---|---|---|---|---|
1 | 10 months | IgM ↑, IgG ↓, IgA normal | Not done | 4-weekly IVIG administered. Developed bronchiectasis. Died at age 17 months during an episode of pneumonia and acute-on-chronic respiratory failure |
2 | 24 months | IgM normal, IgG ↓, IgA ↓ | CD40L deficiency | 4-weekly IVIG and cotrimoxazole prophylaxis administered. Developed cryptococcal-associated ascending cholangitis / chronic liver disease. Died at the age of 7 years of an unknown cause |
3 | 9 months | IgM ↑, IgG ↓, IgA normal | CD40L deficiency | 1 infusion of IVIG administered. Died at age 10 months due to interstitial pneumonia and acute respiratory failure |