Growth hormone receptor deficiency in Ecuador: Clinical and biochemical phenotype in two populations

Jaime Guevara-Aguirre, Arlan L. Rosenbloom, Paul J. Fielder, Frank B. Diamond, Ronald (Ron) Rosenfeld

Research output: Contribution to journalArticle

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Abstract

We have identified 56 patients with GH receptor deficiency (Laron syndrome) from two provinces in southern Ecuador, one group of 26 (Loja province) with a 4:1 female predominance and 30 patients from neighboring El Oro province with a normal sex ratio. There were no significant differences between the Loja and El Oro populations in stature (-5.3 to -11.5 standard deviation score), other auxologic measures, or in biochemical measures. GH binding protein, the circulating extracellular domain of the GH receptor, was measured by ligand immunofunction assay and found to be comparably low in children and adults. Levels of insulin-like growth factor (IGF)-I and -II and the GH-dependent IGF binding protein-3 (measured by RIA) were significantly greater, and GH and IGF binding protein-2 levels significantly lower in adults than children. Levels of IGF-I (adults) and IGF binding protein-3 (children and adults) correlated inversely with statural deviation from normal (P <0.01). School performance was at an exceptionally high level, 41 out of 47 who had attended school being in the top 3 in classes of 15-50 persons.

Original languageEnglish (US)
Pages (from-to)417-423
Number of pages7
JournalJournal of Clinical Endocrinology and Metabolism
Volume76
Issue number2
StatePublished - Feb 1993
Externally publishedYes

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Laron Syndrome
Ecuador
Somatotropin Receptors
Insulin-Like Growth Factor Binding Protein 3
Insulin-Like Growth Factor I
Insulin-Like Growth Factor Binding Protein 2
Phenotype
Population
Assays
Insulin-Like Growth Factor II
Sex Ratio
Ligands

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Guevara-Aguirre, J., Rosenbloom, A. L., Fielder, P. J., Diamond, F. B., & Rosenfeld, R. R. (1993). Growth hormone receptor deficiency in Ecuador: Clinical and biochemical phenotype in two populations. Journal of Clinical Endocrinology and Metabolism, 76(2), 417-423.

Growth hormone receptor deficiency in Ecuador : Clinical and biochemical phenotype in two populations. / Guevara-Aguirre, Jaime; Rosenbloom, Arlan L.; Fielder, Paul J.; Diamond, Frank B.; Rosenfeld, Ronald (Ron).

In: Journal of Clinical Endocrinology and Metabolism, Vol. 76, No. 2, 02.1993, p. 417-423.

Research output: Contribution to journalArticle

Guevara-Aguirre, J, Rosenbloom, AL, Fielder, PJ, Diamond, FB & Rosenfeld, RR 1993, 'Growth hormone receptor deficiency in Ecuador: Clinical and biochemical phenotype in two populations', Journal of Clinical Endocrinology and Metabolism, vol. 76, no. 2, pp. 417-423.
Guevara-Aguirre J, Rosenbloom AL, Fielder PJ, Diamond FB, Rosenfeld RR. Growth hormone receptor deficiency in Ecuador: Clinical and biochemical phenotype in two populations. Journal of Clinical Endocrinology and Metabolism. 1993 Feb;76(2):417-423.
Guevara-Aguirre, Jaime ; Rosenbloom, Arlan L. ; Fielder, Paul J. ; Diamond, Frank B. ; Rosenfeld, Ronald (Ron). / Growth hormone receptor deficiency in Ecuador : Clinical and biochemical phenotype in two populations. In: Journal of Clinical Endocrinology and Metabolism. 1993 ; Vol. 76, No. 2. pp. 417-423.
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