The Impact of congenital, severe, untreated human growth hormone HGH deficiency on bone size and density in young adults: insights from genetic HGH-releasing hormone receptor deficiency.
Maheshwari HG - J Clin Endocrinol Metab - 01-JUN-2003; 88(6): 2614-8
From NIH/NLM MEDLINE
NLM Citation ID:
12788864 (PubMed)
Full Source Title:
Journal of Clinical Endocrinology and Metabolism
Publication Type:
Journal Article
Language:
English
Author Affiliation:
Center for Endocrinology, Metabolism and Molecular Medicine, Department of Medicine, Northwestern University Medical School and Veterans Administration Chicago Health Care System, Lakeside Division, Chicago, Illinois 60611, USA.
Authors:
Maheshwari HG; Bouillon R; Nijs J; Oganov VS; Bakulin AV; Baumann G
Abstract:
HGH and IGF-I have well recognized effects on bone elongation during development, but their importance for bone mineralization and structure during the growth phase are less well understood. Because children with GH deficiency are generally treated with HGH, little detailed information exists in humans about the effects of long-term HGH deficiency on bone development. The recently described syndrome of genetic HGH-RH receptor deficiency in Pakistan (dwarfism of Sindh) affords a unique opportunity to examine the question of GH deficiency on bone development because the affected patients have congenital, severe, isolated GH deficiency, which had never been treated because of societal reasons. We performed dual energy x-ray absorptiometry scans in four adult males (age, 23-30 yr) to address the question of bone mineralization. Areal bone mineral density (BMD) was low (mean Z scores: -3.3, -2.1, -3.7, and -1.7) in the lumbar spine, femoral neck, forearm, and total skeleton, respectively. This low areal BMD is in part caused by the small bone size in these dwarfed patients. When corrected for size, volumetric BMD (bone mineral apparent density) was normal to near normal (mean Z scores: -1.2, +0.8, and +0.8 for lumbar spine, femoral neck and total skeleton, respectively). We conclude that GH/IGF-I deficiency has relatively little impact on bone mineralization during the bone accretion phase. This is in marked contrast to their effect on bone elongation and overall bone size.
Major Subjects:
- Bone Density
- Bone and Bones / metabolism / * pathology
- Human Growth Hormone / * deficiency
Additional Subjects:
- Adult
- Densitometry, X-Ray
- Femur Neck / metabolism
- Forearm
- Humans
- Lumbar Vertebrae / metabolism
- Male
- Metabolism, Inborn Errors / metabolism / pathology
- Receptors, Neuropeptide / deficiency
- Receptors, Pituitary Hormone-Regulating Hormone / deficiency
- Research Support, Non-U.S. Gov't
- Research Support, U.S. Gov't, Non-P.H.S.
- Research Support, U.S. Gov't, P.H.S.
Chemical Compound Name:
(Receptors, Neuropeptide); (Receptors, Pituitary Hormone-Regulating Hormone); (somatotropin releasing hormone receptor); 12629-01-5(Human Growth Hormone)