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Research Notes: PWS Abstracts - 1979 and prior years[ 2007 | 2006 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995 | 1994 | 1993 | 1992 | 1991 | 1990 | 1980-1989 | 1979 and prior ] Masui. 1979 May. No abstract available. Acta Paediatr Scand. 1978 Nov. The sexual maturation in the Prader-Labhart-Willi (PLW) syndrome was investigated in 14 patients, 10 females and 4 males. A wide variability in the pattern of pubertal development was found including delayed puberty in 5 patients and normal puberty in 4 patients; sexual precocity was also observed in 5 patients, true precocious puberty in one patient and incomplete sexual precocity in the form of precocious pubarche in 4 patients. In 5 patients, 3 of them with precocious pubarche, the appearance of the pubertal signs was followed by a delay or arrest in their future development. An LH-RH stimulation test was performed in 11 patients. In the 6 patients who eventually developed normal puberty, the basal levels and the peak responses of both LH and FSH were within the range of those observed in normal controls of the same pubertal stage. In 4 patients showing marked delay or arrest of puberty, the basal levels were normal or low and the responses of LH and FSH to LH-RH were blunted. Priming with repeated LH-RH stimulation in one of the male patients led to an augmented LH response, suggesting a hypothalamic hypogonadotrophism. It is concluded that the lack of uniformity in the pattern of sexual maturation in the PLW syndrome is due to a variability in the location and extent of a hypothalamic lesion, which may comprise an active process continuing beyond the perinatal period. Diabetes. 1977 Sep. Studies of fat mobilization and transport are reported in six patients with the Prader-Willi syndrome. Two patients had carbohydrate intolerance. One of these had a low and the other an augmented insulin response to glucose challenge. Following challenge with glucose, three of the four nondiabetics had normal insulin responses or increased responses consistent with their obesity; the other nondiabetic had insulinopenia. Measurements of the effects of norepinephrine, insulin, glucose, and 5-methylpyrazole-3-carboxylic acid on plasma levels of FFA, glycerol, and ketones provide no evidence for abnormal regulation of mobilization of fat from adipose tissue. Measurements of plasma lipids and postheparin lipolytic activity are consistent with normal uptake of fat into adipose tissue, and normal fatty acid composition of adipose tissue gives no evidence for abnormal lipogenesis. Am J Dis Child. 1977 Jul. The families of 39 patients with Prader-Willi syndrome were examined for similarly affected individuals. The pertinent findings include (1) one sibling more profoundly affected than the patients usually described, (2) one first cousin more mildly affected than usual, (3) a suggestive history of increased frequency of spontaneous late miscarriage (17%) from the mothers of affected offspring, and (4) an occurrence rate of 1.6% in proband siblings. N Engl J Med. 1977 Apr 7. Four adolescents or young adults with the Prader-Willi syndrome (hypotonia, mental retardation, hypogonadism and obesity) received a protein-sparing modified fast consisting of 1.5 g of meat protein per kilogram of ideal body weight and meeting vitamin, mineral and fluid requirements. Evaluation of nitrogen and energy metabolism revealed the development of starvation ketosis and a positive nitrogen balance. Serial whole-body potassium measurements in two patients confirmed preservation of lean tissue despite continuing loss of weight. Clinical diabetes mellitus in two subjects was rapidly ameliorated by the regimen. Short-term weight loss greater than 18 kg occurred in three of the four subjects, and reduced weight persisted during observation periods of 26 to 44 months. This degree of outpatient diet adherence by mentally deficient subjects, who do not normally experience satiety, suggests that hunger is eliminated or at least reduced by modified, protein-sparing fasting. Rev Clin Esp. 1976 Dec 15. No abstract available. J Am Diet Assoc. 1976 Apr. Dietary adherence of eight children with the Prader-Willi syndrome was studied in the home environment. Weight changes were recorded at two-week intervals, and measured two-week dietary records were completed twice during the study by the parents of seven of the children. An eighth child was similarly followed for three months, and one dietary record was obtained. Calories, protein, fat, and carbohydrate contents were calculated, and related to recorded weight changes to determine which diets were most practical in controlling weight gain. Caloric requirements of children with the Prader-Willi syndrome appear to be much lower than those of healthy, active children of comparable ages. Age, degree of obesity, familial relationships, and probably, composition of the diet influenced the effectiveness of a given diet. Each family designed a diet which took into consideration the family's eating habits, as well as the needs of the Prader-Willi child. Frequent contact with the dietitian enabled each family to try new food preparation ideas. The effectiveness and acceptability of a low caloric, very low-carbohydrate diet should be tested over long periods in Prader-Willi children whose obesity is being managed in a non-institution setting. Anesthesiology. 1976 Feb. No abstract available. Anesthesiology. 1975 Nov. No abstract available. J Clin Endocrinol Metab. 1975 Aug. Two women with the Prader-Labhart-Willi syndrome are presented. The gonadotropin response to LH-RH administration was studied prior to, immediately following, and 6 months after a 6-week trial of clomiphene citrate, 200 mg per day for 21 days in divided doses, followed by 100 mg for 21 days in divided doses, followed by 100 mg per day for 14 days in divided doses, and followed by 50 mg per day in a single dose for an additional 14 days. During therapy, the basal gonadotropin and estradiol concentrations rose from prepubertal levels to those of mature women in midmenstrual cycle. However, 6 months after cessation of treatment, the basal gondadotropin and estradiol levels had returned to the prepubertal range. The initial response to LH-RH in the 2 patients differed in that one was clearly prepubertal and the other indistinguishable from the broad range of the adult normal response. The LY and FSH responses to LH-RH administration was greater after 6 weeks of clomiphene citrate therapy than they were either before (both patients) or 6 months after treatment (1 patient). We conclude that there is heterogeneity in the response to LH-RH administration in the Prader-Labhart-Willi Syndrome, just as there is in other syndromes of hypogonadotropic hypogonadism. A normal adult response of gonadotropins to the administration of LH-RH was acheived during clomiphene citrate therapy. J Ment Defic Res. 1975 Jun. Three patients with Prader-Willi syndrome are reported. The subjects presented chemical diabetes mellitus. All patients were hypersentsiive to exogenous insulin and they showed poor adrenal medullary response to hypoglycaemics. It was postulated that the poor adrenal medullary response to the insulin injection may be a contributing factor in the excessive hypoglycaemic response, and that the diabetes mellitus observed in the patients could be due to inactivation of endogenous insulin. J Clin Endocrinol Metab. 1974 Dec. No abstract available. Dtsch Med Wochenschr. 1974 May 31. No abstract available. Rev Invest Clin. 1974 Jan-Mar. Neuroendocrinology. 1974. No abstract available. Acta Med Iugosl. 1974. No abstract available. Acta Neuropathol (Berl). 1972. No abstract available. Orv Hetil. 1971 Jul 18. No abstract available. J Neurol Sci. 1969 Jul-Aug. No abstract available. Dev Med Child Neurol. 1969 Jun. No abstract available. Acta Derm Venereol. 1968. No abstract available. Arch Kinderheilkd. 1966 Apr. No abstract available. [ 2007 | 2006 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995 | 1994 | 1993 | 1992 | 1991 | 1990 | 1980-1989 | 1979 and prior ] |