By Randell C. Alexander, James W. Hanson (auth.), Louise R. Greenswag R.N., PH.D., Randell C. Alexander M.D., PH.D. (eds.)
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Example text
Somatomedin-C levels and 24-hour pulsatile secretory patterns of GH have not yet been reported in PWS subjects but are presently being studied. Thyroid studies are usually normal, although tertiary hypothalamic hypothyroidism with a normal or low thyroid-stimulating hormone (TSH) level and a low thyroxine level is possible. 26 Karen Rubin and Suzanne B. 2. Potential indications for hormonal intervention in PWS. TABLE Microphallus Cryptorchidism Incomplete pubertal development ? Osteoporosis ? Short stature Treatment of Hypogonadism Hormonal intervention in PWS may be indicated for management of microphallus, cryptorchidism, and incomplete pubertal development.
This discrepancy between what the PWS child wants to do and what he or she is able to do leads to frustrating conflicts that may result in depression and psychotic reactions. Obesity, always an emotional burden for anyone living in our society, is even greater for the emotionally labile PWS child. Another variant of PWS may be noted where the perception of satiety is lacking. In the author's opinion, these individuals will eat as long as food is in sight, but are not as distressed by a painful hunger feeling and have less violent outbursts of anger.
L (1975). Metabolic and genetic syndromes of overgrowth. , pp. 1324-1328). B. Saunders. M. (1980). Endocrine function in the Prader-Willi syndrome. Clinical Endocrinology, 12, 81-89. W. (1985). Effect of estradiol treatment monitored by serum concentration on growth and development in Turner syndrome. Pediatric Research, 19, 620. , & Hansmann, M. (1980). Pregnancies following chro- 3. Hypogonadism and Osteoporosis 33 nic intermittent (pulsatile) administration of Gn-RH by means of a portable pump ("Zyklomat").