PHYSIOLOGICAL EVIDENCE OF HYPERMETABOLISM IN OSTEOGENESIS IMPERFECTA

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Abstract
Observations on 30 prepubertal patients with osteogenesis imperfecta have shown abnormally low bodyweights and elevations in metabolic rates, serum thyroxine levels, oral temperatures, heart and respiratory rates. The findings suggest that these patients are in a chronic hypermetabolic and hyperkinetic circulatory state, possibly related to abnormal thyroid function. Since excess thyroid hormone can uncouple oxidative phosphorylation, part of the energy which normally is captured by the synthesis of ATP is lost as heat, and is thus not available for tissue anabolism. It is postulated that this waste of energy is the cause of the hyperthermia, the physical underdevelopment and the abnormal bone structure in children with osteogenesis imperfecta. After the onset of puberty, metabolic rates normalized in our patients and the clinical progression of the disease became arrested. This study suggests that osteogenesis imperfecta is a disease caused by abnormalities in metabolic processes in many tissues, and it does not seem to be a disease which affects connective tissue alone.

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