Pseudopituitary dwarfism due to resistance to somatomedin: A new syndrome

J. R. Bierich, H. Moeller, M. B. Ranke, R. G. Rosenfeld

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

Abstract

The case of an infant is described who at birth was already small and postnatally grew extremely slowly. At age 3 the girl's height was 65 cm, weight 5.6 kg, bone age 21 months. Basal plasma GH was 36-66 ng/ml, basal SM activity was rather high, being around 2.0 U/ml. RIA- and RRA-SM were also increased. Prolonged GH administration did not raise plasma SM. There was a tendency for hypoglycemic episodes in the presence of low insulin levels. Receptor studies with skin fibroblasts showed a diminution of the specific binding of SM-C by 50%. Apparently only the specific IGF-receptor is defective whereas the insulin receptor responds to the increased SM with hypoglycemia. The observation that the high plasma SM did not suppress the enhanced GH-secretion suggests that perhaps the hypothalamic IGF-receptor was also defective.

Original languageEnglish (US)
Pages (from-to)186-188
Number of pages3
JournalEuropean Journal of Pediatrics
Volume142
Issue number3
DOIs
StatePublished - Aug 1984

Keywords

  • Growth hormone
  • Pituitary dwarfism
  • Pseudopituitary dwarfism
  • Receptor defects
  • Somatomedin

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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