Document Detail

Insulin-like growth factor-I treatment in two children with growth hormone gene deletions.
MedLine Citation:
PMID:  10417965     Owner:  NLM     Status:  MEDLINE    
Two unrelated Brazilian patients had homozygous 6.7 kb deletions in the GH-1 gene (girl and boy, 1.8 and 3.3 yr, heights -7.9 and -6.0 SDS, respectively). Desensitization using small amounts of exogenous GH (0.033 IU/kg body weight/week, divided into daily s.c. injections) was attempted, but anti-GH antibodies appeared. Replacement with usual doses of hGH induced only transient increase in growth. IGF-I therapy with increasing doses resulted in catch-up growth without side-effects. Growth velocity was 7.5 cm/yr in the first year and 8.4 cm/yr in the next 6 months in patient 1, and 6.7 cm/yr in the first year, 5.9 cm/yr in the second year and 7.9 cm/yr in the third year of IGF-I treatment in patient 2, when the daily dose of 240 micrograms/kg was divided into three injections. IGFBP-3 levels were low (0.55 and 0.40 mg/I) and did not increase after IGF-I treatment, suggesting that this GH effect is not mediated by IGF-I, and injected IGF-I had a rapid disappearance rate. We conclude that IGF-I promotes growth by endocrine mechanisms and constitutes an effective treatment for patients with GH insensitivity secondary to GH antibodies.
I J Arnhold; S B Oliveira; M G Osorio; B B Mendonca
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric endocrinology & metabolism : JPEM     Volume:  12     ISSN:  0334-018X     ISO Abbreviation:  J. Pediatr. Endocrinol. Metab.     Publication Date:    1999 Jul-Aug
Date Detail:
Created Date:  1999-08-26     Completed Date:  1999-08-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9508900     Medline TA:  J Pediatr Endocrinol Metab     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  499-506     Citation Subset:  IM    
Unidade de Endocrinologia do Desenvolvimento, Hospital das Clinicas, University of São Paulo School of Medicine, Brazil.
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MeSH Terms
Antibodies / blood
Body Height
Child, Preschool
Gene Deletion*
Growth Disorders / drug therapy,  genetics
Hormone Replacement Therapy
Human Growth Hormone / genetics*,  immunology,  therapeutic use
Insulin-Like Growth Factor Binding Protein 3 / blood
Insulin-Like Growth Factor I / administration & dosage,  therapeutic use*
Reg. No./Substance:
0/Antibodies; 0/Insulin-Like Growth Factor Binding Protein 3; 12629-01-5/Human Growth Hormone; 67763-96-6/Insulin-Like Growth Factor I

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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