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Introduction and Aim: Weekly home-based subcutaneous immunoglobulin (SCIg) therapy is an alternative to intravenous immunoglobulin (IVIg) in the treatment of patients with primary antibody deficiencies. The objective of this study was to investigate the efficacy, safety, related quality of life and cost effectiveness of SCIg in our area., Materials and Methods: Observational and descriptive study including paediatric patients with common variable immunodeficiency (CVID) receiving SCIg in our hospital (November 2006 to April 2008). Obtained data were compared with those from the last year with IVIg., Results: Eleven patients with CVID were included. Median age was 15 years. The median trough serum IgG level was 622 mg/dl with IVIg. In patients in whom the SCIg dose was maintained or reduced compared to IVIg, the median trough serum IgG level was 850 mg/dl (p < 0.0005). Annual rate of infection was 2.22 per patient-year, without significant differences to IVIg (p = 0.212). There were 58 treatment-related adverse events (AE) reported with SCIg (45 local AE and 13 systemic AE). The most frequent treatment-related adverse event was infusion-site reaction. Switching to home-based subcutaneous IgG treatment led to significant improvements in quality of life and substantial cost savings., Conclusions: We conclude that subcutaneous administration of 16% SCIg is a safe and cost-effective alternative to IVIg for replacement therapy of primary antibody deficiencies. Median trough serum IgG levels were higher with SCIg. Local AE were common but mild and the incidence decreased over time. Quality of life is significantly improved.

The Spanish Registry for Primary Immunodeficiency Diseases (REDIP) was organized in 1993. One thousand sixty-nine cases of primary immunodeficiency diseases (PID) were registered in patients diagnosed between January 1980 and December 1995. PID diagnosis was made according to the World Health Organization criteria. The most frequent disorders were IgA deficiency (n = 394) and common variable immunodeficiency (n = 213), followed by severe combined immunodeficiency (n = 61), C1 inhibitor deficiency (n = 52), X-linked agammaglobulinemia (n = 49), IgG subclass deficiency (n = 48), and chronic granulomatous disease (n = 32). A comparative study between REDIP and data recently obtained from the European registry (ESID Report, 1995) revealed important differences between phagocytic disorders and complement deficiencies reported in both registries, 4.9 vs 8.7 and 6.0 vs 3.6, while percentages of predominantly antibody deficiencies and T cell and combined deficiencies concurred with those reported in the European registry, 69.3 vs 64.7 and 14.7 vs 20.2, respectively. The heterogeneous nature of the geographical distribution of cases submitted may indicate underdiagnosis of PID in some country areas; surprisingly, the interval between the onset of clinical symptoms and diagnosis was significant, even in immunodeficiency diseases, such as IgA deficiency, which are easy to diagnose.

Serological follow-ups were performed in 19 newborns carrying HIV 1 antibodies over a period ranging between 9 and 62 months. Eight children developed AIDS ans 11 remained asymptomatic. Anti-HIV 1 antibodies were determined by ELISA and Western blot and p 24 antigen was detected by ELISA. Antibodies to HIV 1 disappeared in most children without AIDS symptoms between 10 and 12 months after birth, with antibodies against gp 41 being lost first. Children with AIDS remained positive during follow-up, although in 3 cases with positive was absent in all the asymptomatic children, while it was present in 75% of the patients with AIDS.

A seven years old boy with an hypogammaglobulinemia associated to a pauciarticular chronical juvenile arthritis, in which immunitary deficit diagnostic was made investigating his articular disease, is presented. Clinic, immunological screening, therapy and ulterior evolution with a two year substitutive immunoglobulin treatment are discussed. Authors also revise some aspects of these two diseases association.

Two hundred and ninety three cases of children born of HIV positive mothers have been observed between January 1984 and June 1988 in Catalonia and Balearic Islands, with a ration of 9.5/10,000 newborns. Sex was not significant in the infected group. Most of the cases (68%) have been detected since 1987 and represent a significant increase in HIV positive children and thus of those who will present with clinical manifestations of this infection.