Carbohydrate metabolism in primary growth hormone resistance (Laron syndrome) before and during insulin-like growth factor-I treatment

• Published in: Metabolism, clinical and experimental Vol. 44; no. 10; pp. 113 - 118
• Main Authors: Laron, Z., Avitzur, Y., Klinger, B.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: New York, NY Elsevier Inc 01.10.1995; Elsevier
• Subjects: Biological and medical sciences; Blood Glucose - metabolism; Carbohydrate Metabolism; Endocrinopathies; Growth Disorders - drug therapy; Growth Disorders - metabolism; Growth Hormone - metabolism; Humans; Hypothalamus. Hypophysis. Epiphysis (diseases); Insulin Resistance; Insulin-Like Growth Factor I - deficiency; Insulin-Like Growth Factor I - therapeutic use; Medical sciences; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Obesity - complications; Target tissue resistance; Laron dwarfism; Pathophysiology; STH; Carbohydrate; Insulin like growth factor 1; Metabolism; Insulin
• ISSN: 0026-0495; 1532-8600
• DOI: 10.1016/0026-0495(95)90231-7

Among 43 patients with Laron syndrome followed in our clinic, we were able to study the carbohydrate metabolism from infancy into adult age in 30 patients. During infancy, fasting blood glucose levels were in the hypoglycemic range (mean ± SD, 3.5 ± 1.2 mmol/L) and increased at the end of a delayed puberty to 4.6 ± 0.6 mmol/L. Fasting plasma insulin was higher than expected for concomitant glucose levels, and several of the 20 patients who underwent an oral glucose tolerance test (OGTT) had glucose intolerance and relatively high insulin levels. In adult patients, insulinopenia developed and one 38-year-old patient developed non-insulin-dependent diabetes mellitus (NIDDM) with subsequent need for insulin therapy. Continuous insulin-like growth factor-I (IGF-I) treatment of a pubertal patient with glucose intolerance and hyperinsulinemia normalized both responses. In conclusion, long-term IGF-I deficiency leads to insulin resistance, which is reversed by exogenous IGF-I administration.