Abstract
Three brothers, ages 12, 7 and 6 years with a striking similar history of psychomotor retardation, developmental defects in speech and reading, low I.Q.'s behavioral disabilities and hyperactivity were found to be retarded in growth and skeletal maturation. There was a positive family history of short stature and mental retardation. The oldest sibling had panhypopituitarism as indicated by lack of increase in serum HGH during insulin, arginine and deep sleep, low FBI and T4, low 24 hr. urinary 17KGS and lack of response to metyrapone. Administration of 25U of ACTH produced an increase in plasma cortisol levels from 3.2 meg/100 ml to 19.4. The other two had normal increases in serum HGH and normal thyroid and adrenal functions. A normal increase in serum TSH after administration of 7 μg/kg of TRF in the three indicated an intact pituitary gland. The increase in nitrogen balance, plasma FFA, insulin release, and the marked calciuria and hydroxyprolinuria produced by administration of HGH ruled out peripheral unresponsiveness to HGH. It is possible that a genetically determined brain lesion affected the hypothalamic centers controlling the anterior pituitary gland, resulting in a multiple deficiency of releasing factors in the oldest and a decreased secretion of an unknown hypothalamic factor involved in promoting growth in the other two. (Supported by NIH Grant RR-318)
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