Response of Growth-Retarded Patients with Hand–Schüller–Christian Disease to Growth Hormone Therapy

Glenn D. Braunstein, Salvatore Raiti, James W. Hansen, Peter O. Kohler

Research output: Contribution to journalArticle

22 Scopus citations

Abstract

Five patients with growth retardation due to Hand–Schüller–Christian disease were treated for a maximum of two years with human growth hormone, which was administered intramuscularly, three times per week, in doses of 2 IU. All patients experienced a significant increment in growth rate in comparison to the year before the therapy. During the first year of therapy the mean growth rate was 7.5 cm—an increase of 5.2 cm over the pretreatment year (p< 0.01). The growth rate during the second year of treatment was less, with a mean increase of 2.8 cm per year (P<0.05). These results are similar to the response of patients with idiopathic growth hormone deficiency and add further evidence that the growth retardation associated with Hand–Schüller–Christian disease is due to an acquired growth hormone secretory deficiency. (N Engl J Med 292:332–333, 1975), Hand–Schüller–Christian disease is characterized by the infiltration of many areas of the body, including the hypothalamus, by foamy histiocytes.1 When the infiltration involves the hypothalamus, prepubertal growth retardation resembling that seen with idiopathic growth hormone deficiency and diabetes insipidus frequently occur.2 In an earlier study we demonstrated that most of our patients with Hand–Schüller–Christian disease and growth retardation had an inadequate growth hormone response to provocative tests although the remainder of the anterior pituitary hormones responded appropriately to a variety of stimuli.2 We postulated that the growth retardation was due to this isolated growth hormone secretory deficiency, and that the.

Original languageEnglish (US)
Pages (from-to)332-333
Number of pages2
JournalNew England Journal of Medicine
Volume292
Issue number7
DOIs
StatePublished - Feb 13 1975

ASJC Scopus subject areas

  • Medicine(all)

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