Insulin Resistance in Laron Syndrome (Primary Insulin-like Growth Factor-I (IGF-I) Deficiency) and Effect of IGF-I Replacement Therapy

Author(s):  
Zvi Laron ◽  
Yaron Avitzur ◽  
Beatrice Klinger
2008 ◽  
Vol 48 ◽  
pp. S65-S66
Author(s):  
M. García-Fernández ◽  
J.E. Puche ◽  
G. Delgado ◽  
S. González-Barón ◽  
I. Castilla-Cortázar

1994 ◽  
Vol 3 (Supple5) ◽  
pp. 239-239
Author(s):  
Yoshihiko Takahashi ◽  
Hiroko Kadowaki ◽  
Yasuo Akanuma ◽  
Takashi Kadowaki ◽  
Yoshio Yazaki

1998 ◽  
pp. 176-180 ◽  
Author(s):  
Z Laron ◽  
B Klinger

Serum gonadotrophins. androgens, insulin and insulin-like growth factor-I (IGF-I) were determined before and during long-term treatment with recombinant IGF-I of seven males with Laron syndrome, and the changes correlated with changes in testicular volume and penile size. The subjects were four boys below the age of 5, two boys aged 10 and 14 but prepubertal and one 28-year-old fully sexually developed adult. IGF-I was administered by a once daily subcutaneous injection of 150 microg/kg per day to the boys and 120 microg/kg per day to the adult patient. In the very young boys no change in serum gonadotrophins, androgens, gonads or genitals was registered. In the two older boys and the adult patient, there was a progressive rise in luteinizing hormone, follicle-stimulating hormone and testosterone. Concomitantly, there was an increase in size of the testes and penile length. The two boys started puberty. As very high serum IGF-I levels were registered in the adult patient, the daily dose was progressively decreased to 70 microg/kg per day. Stopping the IGF-I administration in this patient, according to the protocol, led to a return to pretreatment serum levels and testicular and penile size. This report shows for the first time a direct effect of IGF-I on sex hormones and sex organs in the male.


1997 ◽  
Vol 136 (1) ◽  
pp. 92-95 ◽  
Author(s):  
Robert Bjerknes ◽  
Per Vesterhus ◽  
Dagfinn Aarskog

Abstract Insulin-like growth factor-I (IGF-I) is a growth hormone-dependent peptide with growth and immunoregulatory properties, and in Laron syndrome growth hormone insensitivity induces impaired production of IGF-I. In the present study we have determined the neutrophil expression of IGF-I receptors (IGF-I-Rs), as well as the IGF-I-induced priming of neutrophil functional capacity, in two children with Laron syndrome treated with recombinant human IGF-I, and in age-matched controls. Before treatment, the patient neutrophil expression of IGF-I-Rs was significantly increased. However, with replacement therapy the neutrophil IGF-I-R expression was downregulated to levels similar to those of the controls within one month. In the patients, the phagocytic capacity and oxidative burst of unprimed neutrophils were normal and similar to controls before the start of treatment. Moreover, IGF-I efficiently primed both patient and control neutrophils to increase their phagocytic capacity and oxidative burst in vitro. However, before therapy, the priming response to IGF-I was significantly stronger in the neutrophils in the patients than in the controls. The present data support earlier studies by us and others demonstrating that IGF-I is a potent regulator of mature neutrophil function, but also suggest that these leukocytes may function normally in the presence of very low levels of IGF-I in vivo. European Journal of Endocrinology 136 92–95


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