Long-term growth hormone therapy in mitochondrial cytopathy.

Abstract

OBJECTIVE:

To describe in a 5-year-old Caucasian male with mitochondrial cytopathy, a biochemical growth hormone (GH) deficiency associated with normal GH biological activity as evaluated by Nb2 cell bioassay and normal serum IGF-I and IGFBP3 values increasing slightly after GH administration.

METHOD:

Serum GH concentrations were measured with a commercial immunofluorometric assay and with a biological assay, which uses the Nb2 cell line. Serum IGF-I and IGFBP3 concentrations were measured with RIA.

RESULTS:

The GH-supplementary therapy was initially effective in terms of growth gain, but no therapeutic benefit was observed over a long period of time.

CONCLUSION:

In patients suffering from mitochondrial cytopathy, short stature seems to be attributed more to a disease-related inadequate protein substrate than to the non-classical GH deficiency.

• Authors: Salvatore Barberi, Elena Bozzola, Angela Berardinelli, Cristina Meazza, Mauro Bozzola
• Journal: Hormone research (Horm Res) Vol. 62 Issue 2 Pg. 103-6 ( 2004) ISSN: 0301-0163 [Print] Switzerland
• PMID: 15331853 (Publication Type: Case Reports, Journal Article)

Chemical References

• Human Growth Hormone

Topics

• Child, Preschool
• Growth Disorders (drug therapy, pathology)
• Hormone Replacement Therapy
• Human Growth Hormone (administration & dosage, therapeutic use)
• Humans
• Male
• Mitochondrial Diseases (drug therapy, pathology)