Literature DB >> 19946434

Treatment of dwarfism with recombinant human insulin-like growth factor-1.

Michael B Ranke1, Joachim Wölfle, Dirk Schnabel, Markus Bettendorf.   

Abstract

BACKGROUND: The growth hormone-IGF (insulin-like growth factor) system plays a central role in hormonal growth regulation. Recombinant human (rh) growth hormone (GH) has been available since the late 1980s for replacement therapy in GH-deficient patients and for the stimulation of growth in patients with short stature of various causes. Growth promotion by GH occurs in part indirectly through the induction of IGF-1 synthesis. In primary disturbances of IGF-1 production, short stature can only be treated with recombinant human IGF-1 (rhIGF-1). rhIGF-1 was recently approved for this indication but can also be used to treat other conditions.
METHODS: Selective review of the literature on IGF-1 therapy, based on a PubMed search. RESULTS AND
CONCLUSION: In children with severe primary IGF-1 deficiency (a rare condition whose prevalence is less than 1:10,000), the prognosis for final height is very poor (ca. 130 cm), and IGF-1 therapy is the appropriate form of pathophysiologically based treatment. There is no alternative treatment at present. The subcutaneous administration of IGF-1 twice daily in doses of 80 to 120 microg/kg accelerates growth and increases final height by 12 to 15 cm, according to current data. There is, however, a risk of hypoglycemia, as IGF-1 has an insulin-like effect. As treatment with IGF-1 is complex, this new medication should only be prescribed, for the time being, by experienced pediatric endocrinologists and diabetologists.

Entities:  

Keywords:  developmental disorder; dwarfism; growth; hormonal therapy; pediatric disease

Mesh:

Substances:

Year:  2009        PMID: 19946434      PMCID: PMC2780013          DOI: 10.3238/arztebl.2009.0703

Source DB:  PubMed          Journal:  Dtsch Arztebl Int        ISSN: 1866-0452            Impact factor:   5.594


  46 in total

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