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Background

Huntington-s disease HD is a severe inherited neurodegenerative disorder characterized, in addition to neurological impairment, by weight loss suggesting endocrine disturbances. The aims of this study were to look for neuroendocrine disturbances in patients with Huntington-s disease HD and to determine the relationship with weight loss seen in HD

Methods and Finding

We compared plasma levels of hormones from the five pituitary axes in 219 patients with genetically documented HD and in 71 sex- and age-matched controls. Relationships between hormone levels and disease severity, including weight-loss severity, were evaluated. Growth hormone GH and standard deviation score of insulin-like growth factor 1 SDS IGF-1 were significantly higher in patients than in controls 0.25 0.01–5.89 vs. 0.15 0.005–4.89 ng-ml, p = 0.013 and 0.16±1.02 vs. 0.06±0.91, p = 0.039; respectively. Cortisol was higher p = 0.002 in patients 399.14±160.5 nmol-L vs. 279.8±130.1 nmol-L, whereas no differences were found for other hormone axes. In patients, elevations in GH and IGF-1 and decreases in thyroid-stimulating hormone, free triiodothyronine and testosterone in men were associated with severity of impairments Independence scale, Functional score, Total Functional Capacity, Total Motor score, Behavioral score. Only GH was independently associated with body mass index β = −0.26, p = 0.001.

Conclusion

Our data suggest that the thyrotropic and in men gonadotropic axes are altered in HD according to the severity of the disease. The somatotropic axis is overactive even in patients with early disease, and could be related to the weight loss seen in HD patients.



Autor: Nadine Saleh, Stéphane Moutereau, Alexandra Durr, Pierre Krystkowiak, Jean-Philippe Azulay, Christine Tranchant, Emmanuel Brouss

Fuente: http://plos.srce.hr/



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