ANTIBODY DEFICIENCY AND ISOLATED GROWTH-HORMONE DEFICIENCY IN A GIRL WITH MULIBREY NANISM

A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral im munodeficiency consisted of subnormal concentration of serum IgG, in p articular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent. Antibody response in vivo was very low or absent and opsonization in vitro was defective. Total B-ce ll number was low. In addition, the serum kappa/lambda light chain rat ios within the immunoglobulin classes G, A, and M were abnormal. The d efective antibody response may be linked to the abnormal kappa/lambda light chain ratios. Endocrine functions were normal except for isolate d growth hormone deficiency. Therapy with human growth hormone resulte d in increased growth velocity but did not improve humoral immune func tions.