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Response to nutritional and growth hormone treatment in progeria

J E Abdenur1, W T Brown, S Friedman

  • 1Miami Children's Hospital, FL, USA.

Metabolism: Clinical and Experimental
|August 1, 1997
PubMed

Insights

Hutchinson-Gilford progeria syndrome (HGPS) is linked to elevated growth hormone (GH) and basal metabolic rate (BMR), causing failure to thrive. Treatments showed limited long-term benefits for growth and atherosclerosis.

Area of Science:

  • Endocrinology
  • Metabolic Disorders
  • Genetics

Background:

  • Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder with unknown molecular causes.
  • Patients exhibit failure to thrive (FTT), alopecia, scleroderma, joint stiffness, and severe atherosclerosis, with a median lifespan of 13 years.
  • Cardiovascular complications are the primary cause of mortality in HGPS.

Purpose of the Study:

  • To investigate the etiology of growth failure in HGPS through endocrine and metabolic studies.
  • To evaluate the response to nutritional therapy (NT) and growth hormone (GH) treatment in HGPS patients.

Main Methods:

  • Endocrine and metabolic assessments were conducted in five HGPS patients.
  • Nutritional therapy (NT) and GH treatment were administered to three patients.
  • Growth velocity (GV) and basal metabolic rate (BMR) were monitored.

Main Results:

  • Elevated GH levels and an increased BMR were characteristic findings in HGPS, potentially explaining FTT.
  • NT alone showed modest improvements in weight gain and GV.
  • Combined NT and GH treatment increased GV and growth factors but paradoxically decreased BMR, with diminishing responses over time.

Conclusions:

  • HGPS is associated with elevated GH and BMR, contributing to growth failure.
  • While NT and GH offer temporary benefits, they do not prevent the progression of atherosclerosis.
  • Further research is needed to understand the complex metabolic and endocrine aspects of HGPS.

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