Glucagon stimulation test to assess growth hormone status in Prader-Willi syndrome

J Endocrinol Invest. 2021 Mar;44(3):621-629. doi: 10.1007/s40618-020-01367-6. Epub 2020 Jul 27.

Abstract

Purpose: Growth hormone deficiency (GHD) must be confirmed before starting treatment in adults with Prader-Willi syndrome (PWS). Most studies use the growth-hormone-releasing hormone plus arginine (GHRH-arginine) test. No data are available on the glucagon stimulation test (GST) in PWS. We compared the utility of fixed-dose (1 mg) GST versus GHRH-arginine test in diagnosing GHD.

Methods: Adults and late adolescents with PWS underwent both tests on separate days. In the GHRH-arginine test, GHD was defined according to body mass index. In the GST, two cutoffs were analyzed: peak GH concentration < 3 ng/mL and < 1 ng/mL. For analyses, patients were divided into two groups according to body weight (≤ 90 kg and > 90 kg).

Results: We analyzed 34 patients: 22 weighing ≤ 90 kg and 12 weighing > 90 kg. In patients weighing ≤ 90 kg, the two tests were concordant in 16 (72.72%) patients (k = 0.476, p = 0.009 with GST cutoff < 3 ng/mL, and k = 0.450, p = 0.035 with GST cutoff < 1 ng/mL). In patients weighing > 90 kg, the two tests were not concordant with GST cutoff < 3 ng/mL, but were concordant in 11 (91.6%) patients (k = 0.833, p = 0.003) with GST cutoff < 1 ng/mL. GH peaks on the two tests correlated (r = 0.725, p = 0.008).

Conclusion: Fixed-dose (1 mg) GST using a peak GH cutoff of < 3 ng/mL or < 1 ng/mL promises to be useful for screening for GHD in adults and late adolescents with PWS. However, in those weighing > 90 kg, the < 1 ng/mL cutoff seems better. Larger studies are necessary to establish definitive glucagon doses and cutoffs, especially in extremely obese patients.

Keywords: GHRH-arginine test; Glucagon-stimulation test; Growth hormone deficiency; Prader–Willi.

MeSH terms

  • Adolescent
  • Adult
  • Arginine / administration & dosage*
  • Female
  • Follow-Up Studies
  • Glucagon / administration & dosage*
  • Growth Hormone-Releasing Hormone / administration & dosage*
  • Human Growth Hormone / drug effects
  • Human Growth Hormone / metabolism*
  • Humans
  • Male
  • Middle Aged
  • Prader-Willi Syndrome / diagnosis*
  • Prader-Willi Syndrome / metabolism
  • Prognosis
  • Young Adult

Substances

  • Human Growth Hormone
  • Glucagon
  • Growth Hormone-Releasing Hormone
  • Arginine