The long term treatment of childhood hypogammaglobulinaemia in Melbourne with intravenous gammaglobulin, 1972-1985.

Intravenous immunoglobulin has been used for hypogammaglobulinaemic conditions treated at the Royal Children's Hospital, Melbourne since 1972. Fifty-four children have been treated. Nineteen have been males with congenital hypogammaglobulinaemia (including 12 with sex-linked agammaglobulinaemia and 5 with hypogammaglobulinaemia with IgM); 8 have had common variable immunodeficiency, and 11 have had severe combined immune deficiency. Intravenous immunoglobulin has also been used for some patients with transient hypogammaglobulinaemia, isolated IgG deficiency, isolated IgA deficiency and isolated IgM deficiency. Infusions are given four weekly at a dose of 5-7.5 ml/kg of a 6% preparation (300-450 mg/kg). At diagnosis, a loading dose is given of 10-15 ml/kg (600-900 mg/kg). Previous studies have demonstrated a half-life of 25 days. The median preinfusion IgG concentration for the 22 children receiving monthly infusions currently is 68 IU/ml. Hospitalisation rates for infective illness have been reduced with the use of intravenous gammaglobulin. No patients are known to have developed hepatitis. Reactions to infusions are experienced by 60% of patients. These have not been reduced significantly by the addition of 10% maltose, but have been lessened considerably by using intravenous methylprednisolone (1 mg/kg) before the commencement of infusion.