Summary:Severe combined immunodeficiency (SCID) is a rare primary immunodeficiency disorder with an estimated overall frequency of 1 in 75 000 live births. Bone marrow transplantation is the only curative treatment available. Using T cell-depleted HLA non-identical bone marrow requires preconditioning with a short course of cytotoxic chemotherapy. We report severe dental developmental anomalies in three such patients under longterm follow up. Bone Marrow Transplantation (2000) 25, 1007-1009. Keywords: SCID; BMT; dental developmental anomalies Severe combined immunodeficiency (SCID) is a rare primary immunodeficiency with an overall frequency estimated to be 1 in 75 000 live births. 1 SCID represents a group of diseases characterised by deficient T and B cell function with resulting susceptibility to infection and secondary failure of the patients to thrive. SCID syndromes are caused by multiple genetic defects and are fatal usually within the first year of life. Bone marrow transplantation (BMT) is the only curative treatment available. 2 BMT was introduced in the treatment of primary immunodeficiencies about 30 years ago. Since then BMT has made it possible to cure a substantial number of children who would otherwise not have survived. 2,3 In Europe, the overall 2-year survival for BMT for SCID is now over 60%. 3 At present, there are only two specialised centres in the United Kingdom for treating children with severe combined immunodeficiency. Both units, Great Ormond Street Hospital for Sick Children and Newcastle General Hospital are each transplanting about 15-20 children per year. Longterm follow-up post BMT of these children shows that the majority (85%) have normal life of good quality, normal growth and development and good overall immune function. 4 Survival however is not without costs as cytotoxic treatment does not discriminate between myeloid and Correspondence: BOI Cole,
Total IgE levels are nonspecific and these don't add in management of the patients.Guidelines to prescribe Epipen should be followed and every patient should be given Epipen when indicated.Guidelines for doing Specific IgE testing will be revised will be revised.Re audit will be done in due course to see if the updated guidelines are being implemented.
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