(Arch Dis Child 1994; 70: 260-263) Severe combined immunodeficiency syndrome (SCIDS) is a heterogeneous group of inherited disorders characterised by the failure of cellular and humoral immunity. Children present with recurrent or persistent infections and without treatment die within the first year of life from overwhelming infection.' The prognosis of children with SCIDS has been transformed by bone marrow transplantation and data from a European collaborative study of affected infants (in which Newcastle upon Tyne and London are the participating centres in the UK) have shown 97°/0 long term survival for recipients of HIA identical sibling marrow and 52% survival for recipients of HLA mismatched (haploidentical) marrow from a parent.2 The survival rate for infants transplanted after 6 months of age is only 45%/o, however, compared with 70°/ in younger children. Although facilities for bone marrow transplantation for the treatment of SCIDS are available within the UK, many affected children without a family history are older than 6 months of age at referral, or are terminally ill with overwhelming infection, making transplantation a risky procedure.

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SUMMARY Forty nine infants of HIV seropositive women were followed up for a median of 24 months, together with 24 controls. The infection status of 11 index children under 18 months of age was indeterminate; 34 were presumed uninfected while four showed clinical and laboratory evidence of HIV disease. Based on current definitions of HIV infection and excluding children under 18 months old as well as those who had not been studied from birth, two out of 28 children were infected. The estimated rate of maternofetal transmission was therefore 7-1%. In children with proved infection, sequential laboratory data showed that hypergammaglobulinaemia was noted as early as 6 months and often predated clinical signs. This observation, in the presence of non-specific clinical findings, was helpful in alerting the paediatrician to a diagnosis of HIV infection.

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A four year old boy with symptoms of HIV infection and serum IgG of 53.2 g/l had been treated for 16 months with regular infusions of intravenous immunoglobulin (IV IgG). During one such infusion he developed temporary neurological symptoms and signs suggestive of the hyperviscosity syndrome. Serum relative viscosity was raised at 5.0 (normal range 0.42-2.78). Subsequent IV IgG infusions given at a slower rate have been without adverse reactions. In a study of eight HIV infected children including the index case, and 20 children not infected with HIV, serum relative viscosity was significantly raised in the HIV infected children (p less than 0.01; students t-test). Viscosity correlated with total serum IgG, which was raised in all HIV infected children, and with serum IgM. In HIV infected children with very high levels of serum IgG a slow rate of IV IgG infusion should therefore be chosen due to the possibility of hyperviscosity.