Laron's dwarfism: Studies on the nature of the defect

作者: M. Joycelyn Elders , John T. Garland , William A. Daughaday , Delbert A. Fisher , John E. Whitney

DOI: 10.1016/S0022-3476(73)80485-8

关键词: EndocrinologyProlactinAdipose tissueDwarfismGrowth hormone secretionFatty acidGrowth hormoneInternal medicineSlow rateSomatomedinBiologyPediatrics, Perinatology, and Child Health

摘要: Laron's syndrome is characterized by severe dwarfism, high circulating levels of immunoreactive growth hormone, and a failure to generate somatomedin in response administration human hormone. Studies conducted 7 1/2-year-old boy with the indicate that hypothalamic-pituitary mechanisms controlling hormone secretion are intact. However, those suppression release seem be inoperative. The exogenous failed produce an acute metabolic as measured mineral retention, nitrogen generation, or free fatty acid from adipose tissue; however, unsustained long-term was observed. resembled normal immunologically has usual prolactin-like activity. These data suggest primary defect tissue unresponsiveness An abnormality structure which not essential for prolactin activity immunoreactivity slow rate degradation these patients cannot excluded available data.

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