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

A. Haraldsson*, C. J.A.M. van der Burgt, C. M.R. Weemaes, B. Otten, J. A.J.M. Bakkeren, G. B.A. Stoelinga

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

13 Citations (Scopus)

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 anti-body 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.

Original languageEnglish
Pages (from-to)509-512
Number of pages4
JournalEuropean Journal of Pediatrics
Volume152
Issue number6
DOIs
Publication statusPublished - Jun 1993

Other keywords

  • Growth hormone deficiency
  • Immunodeficiency
  • Light chains
  • Mulibrey nanism

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