Metabolic studies in two boys with classical progeria

Two boys with classical progeria have been studied by a variety of laboratories in the Boston area. Evidence for unresponsiveness to growth hormone, relative insulin resistance, and highly cross-linked collagen is presented. The data support the concept that progeria represents a form of mesenchymal...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1969-02, Vol.43 (2), p.207-216
Main Authors: Villee, D B, Nichols, Jr, G, Talbot, N B
Format: Article
Language:English
Subjects:
Adult
Amino Acids - metabolism
Basal Metabolism
Bone and Bones - metabolism
Child
Collagen - analysis
Connective Tissue - metabolism
Endocrine Glands - physiopathology
Fatty Acids, Nonesterified - metabolism
Female
Glucose Tolerance Test
Glycerol - metabolism
Growth Hormone
Humans
Insulin - blood
Male
Mitochondria, Muscle - metabolism
Oxygen Consumption
Progeria - metabolism
Progeria - physiopathology
Skin - analysis
Skin - metabolism
Solubility
Tolbutamide
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Summary:Two boys with classical progeria have been studied by a variety of laboratories in the Boston area. Evidence for unresponsiveness to growth hormone, relative insulin resistance, and highly cross-linked collagen is presented. The data support the concept that progeria represents a form of mesenchymal dysplasia in which connective tissue cells are unresponsive to growth influences. It is hypothesized that this failure to grow may account for the "older" collagen which may in turn produce structural abnormalities in vessel walls predisposing to atherosclerosis.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.43.2.207