Antibody deficiency and isolated growth hormone deficiency in a girl with Mulibrey nanism.

Abstract:

:A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular 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-cell number was low. In addition, the serum kappa/lambda light chain ratios within the immunoglobulin classes G, A, and M were abnormal. The defective antibody response may be linked to the abnormal kappa/lambda light chain ratios. Endocrine functions were normal except for isolated growth hormone deficiency. Therapy with human growth hormone resulted in increased growth velocity but did not improve humoral immune functions.

journal_name

Eur J Pediatr

authors

Haraldsson A,van der Burgt CJ,Weemaes CM,Otten B,Bakkeren JA,Stoelinga GB

doi

10.1007/BF01955061

subject

Has Abstract

pub_date

1993-06-01 00:00:00

pages

509-12

issue

6

eissn

0340-6199

issn

1432-1076

journal_volume

152

pub_type

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