Reduced longevity in untreated patients with isolated HGH human growth hormone deficiency.
Besson A - J Clin Endocrinol Metab - 01-AUG-2003; 88(8): 3664-7
From NIH/NLM MEDLINE
NLM Citation ID:
12915652 (PubMed)
Full Source Title:
Journal of Clinical Endocrinology and Metabolism
Publication Type:
Journal Article
Language:
English
Author Affiliation:
Department of Paediatrics, University Children's Hospital, Inselspital, CH-3010 Bern, Switzerland.
Authors:
Besson A; Salemi S; Gallati S; Jenal A; Horn R; Mullis PS; Mullis PE
Abstract:
Increased longevity of hypopituitary dwarf mice and HGH- resistant knockout mice appears to be in contrast with observations made in clinical practice. In humans, on one hand hypopituitarism and HGH deficiency (HGH-D) are believed to constitute risk factors for cardiovascular disease and, therefore, early death. But on the other hand, patients with a PROP-1 gene mutation, presenting with a combined pituitary-derived hormonal deficiency, can survive to a very advanced age, apparently longer than normal individuals in the same population. The aim of this study was to analyze the impact of untreated HGH-D on life span. Hereditary dwarfism was recognized in 11 subjects. Genetic analysis revealed an underlying 6.7-kb spanning deletion of genomic DNA encompassing the HGH-1 gene causing isolated GHD. These patients (five males and six females) were never treated for their hormonal deficiency and thus provide a unique opportunity to compare their life span and cause of death directly with their unaffected brothers and sisters (11 males and 14 females) as well as with the normal population (100 males and females). Although the cause of death did not vary between the two groups, median life span in the GH-deficient group was significantly shorter than that of unaffected brothers and sisters [males, 56 vs. 75 yr (P < 0.0001); females, 46 vs. 80 yr (P < 0.0001)]. Therefore, with the wealth of information regarding the beneficial effects of GH replacement and the dramatic findings of this study, GH treatment in adult patients suffering from either childhood- or adult-onset HGH-D is crucially important.
Major Subjects:
- Dwarfism / * genetics
- Human Growth Hormone / * deficiency / genetics
- Longevity
Additional Subjects:
- Adult
- Aged
- Aged, 80 and over
- Blotting, Southern
- DNA / genetics / isolation & purification
- Female
- Genetic Screening
- Humans
- Leukocytes / chemistry
- Male
- Middle Aged
- Pedigree
- Research Support, Non-U.S. Gov't
- Reverse Transcriptase Polymerase Chain Reaction
- Switzerland
Chemical Compound Name:
12629-01-5(Human Growth Hormone); 9007-49-2(DNA)