Effect of Acute Administration of Insulin-Like Growth Factor I in Patients With Laron-Type Dwarfism

Laron, Zvi; Erster, B.; Klinger, B.; Anin, S.

doi:10.1016/s0140-6736(88)90236-x

Cited by 96 publications

(46 citation statements)

References 6 publications

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“…LS is caused by deletions or mutations in the growth hormone receptor (GHR) gene resulting in dysfunction of the GHR [33]. LS patients exhibit growth retardation, such as small genitalia and gonads, and recombinant human insulin growth factor I (IGF-I) is administered as a therapy for LS patients because their IGF-I levels are severely depressed [34][35][36][37][38]. In a manner similar to LS disease, HNF-1α deficiency causes a reduction in IGF-I gene expression [14].…”

Section: Discussionmentioning

confidence: 99%

The Production of a Diabetic Mouse Using Constructs Encoding Porcine Insulin Promoter-Driven Mutant Human Hepatocyte Nuclear Factor-1.ALPHA.

Watanabe¹,

Umeyama²,

Kawano

et al. 2007

Journal of Reproduction and Development

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Abstract. A diabetic mouse model was produced using a mutant human hepatocyte nuclear factor-1α gene (HNF1αP291fsinsC) regulated by the porcine insulin promoter. The functionality of two different constructs containing HNF1αP291fsinsC, termed PD1 and PD2 (cytomegalovirus enhancer minus and plus), were examined in transgenic mice. The blood glucose levels and body weights of the PD1 transgenic mice did not differ from their non-transgenic littermates over the period from 3 to 8 weeks of age. Conversely, the PD2 transgenic mice exhibited hyperglycemia and decreased body weight. Western blot analysis demonstrated that mutant HNF-1α protein (HNF1αP291), derived from the PD2 transgene, was expressed in the PD2 mice. Morphometric studies of the pancreas of a PD2 mouse revealed that the number of pancreatic islets present was less than that in the nontransgenic mice, indicating disturbed islet neogenesis. These results suggest that impaired insulin secretion in disrupted islets causes hyperglycemia. In addition, the phenotype of PD2 transgenic mice similar to that of the HNF-1α gene-deficient mouse, which displays growth retardation and impaired viability. These results indicate that HNF1αP291 expression driven by the porcine insulin promoter, together with the cytomegalovirus enhancer, induces a diabetic phenotype in transgenic mice.

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Section: Discussionmentioning

confidence: 99%

The Production of a Diabetic Mouse Using Constructs Encoding Porcine Insulin Promoter-Driven Mutant Human Hepatocyte Nuclear Factor-1.ALPHA.

Watanabe¹,

Umeyama²,

Kawano

et al. 2007

Journal of Reproduction and Development

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“…However, it is structually similar to insulin and shares many of its biological properties (Rinderknecht and Humbel 1978a, 1978b, Froesch and Zapf 1985, Zapf et al, 1984. IGF-I is now produced by recombinant-DNA technology and thus available for laboratory and clinical use (Schalch et al, 1984, Niwa et al, 1986, Scheiwiller et al, 1986, Guler et al, 1987, Laron et al, 1988. Insulin-like growth factor elicits two types of biological effects, an insulin-like effect and a growth-promoting effect.…”

mentioning

confidence: 99%

Effects of sc Administration of Recombinant Human Insulin-Like Growth Factor I (IGF-I) on Normal Human Subjects.

Takano¹,

Hizuka²,

Asakawa³

et al. 1990

Endocrinol Japon

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“…This may reflect that either hypoglycaemia was missed in previous studies that used conventional finger-prick measurements [7,10] or that our two patients showed an unusual idiopathic response that is not representative of a classic patient with this condition. Previous studies [6] have demonstrated that the route of administration and the length of time on treatment affect the extent of hypoglycaemia. This makes it more difficult to interpret the outcomes of our study and emphasizes the need for further research in this area.…”

Section: Discussionmentioning

confidence: 99%

“…However, the extent of hypoglycaemia seems to depend on the route of administration and the length of time on treatment. Intravenous rIGF-1 administration causes a rapid lowering of blood glucose to a mean of 45% of basal levels [6]. Subcutaneous rIGF-1 administration was reported to cause no increase in hypoglycaemia during a randomized controlled trial [7] as evaluated by blood glucose measurements every 4 h; however, observational studies are not consistent with this trial [8,9].…”

Section: Introductionmentioning

confidence: 99%

Use of Continuous Glucose Monitoring to Identify Glucose Dysregulation in Growth Hormone Insensitivity Syndrome

et al. 2014

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Background/Aims: Monogenic forms of growth hormone insensitivity (GHI) and its treatment with recombinant insulin-like growth factor-1 (rIGF-1) are both associated with glucose dysregulation. We used the information provided by continuous glucose monitoring systems (CGMS) for the clinical management of two children with monogenic GHI prior to the commencement of therapy as well as during the years when they received rIGF-1 treatment and continued to do so after the cessation of therapy. Methods: We evaluated the extent of hyper- and hypoglycaemia with CGMS. Results: In one patient, before treatment CGMS identified self-limiting nocturnal hypoglycaemia. Initiation of rIGF-1 treatment resulted in severe and persistent hypoglycaemia with an absence of spontaneous recovery. Corrective dietary measures were instituted. In a second patient, who had a poor growth response to rIGF-1 therapy, CGMS identified significant fluctuations in daytime glucose levels whilst on treatment with evidence of postprandial hyperglycaemia and both rebound and nocturnal hypoglycaemia. Given the lack of improved growth and the documented glucose dysregulation, treatment was stopped and repeat measurements with CGMS 1 month afterwards showed complete resolution. Conclusions: We have demonstrated that CGMS is an effective tool to assess glucose dysregulation in patients with GHI and alters clinical management.

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Effect of Acute Administration of Insulin-Like Growth Factor I in Patients With Laron-Type Dwarfism

Cited by 96 publications

References 6 publications

The Production of a Diabetic Mouse Using Constructs Encoding Porcine Insulin Promoter-Driven Mutant Human Hepatocyte Nuclear Factor-1.ALPHA.

The Production of a Diabetic Mouse Using Constructs Encoding Porcine Insulin Promoter-Driven Mutant Human Hepatocyte Nuclear Factor-1.ALPHA.

Effects of sc Administration of Recombinant Human Insulin-Like Growth Factor I (IGF-I) on Normal Human Subjects.

Use of Continuous Glucose Monitoring to Identify Glucose Dysregulation in Growth Hormone Insensitivity Syndrome

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