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Research Notes: PWS Abstracts - 1991[ 2007 | 2006 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995 | 1994 | 1993 | 1992 | 1991 | 1990 | 1980-1989 | 1979 and prior ] Minerva Pediatr. 1991 Sep. Five children (3 boys and 2 girls) ranging in age form 5-12 years and suffering from Prader-Willi syndrome have been evaluated. In each subject the Authors have examined auxological parameters and the following hormonal values: GH after two pharmacological stimuli tests, gonadotropins after LHRH, TSH and prolactin after TRH, cortisol rhythm, testosterone after hCG in males, thyroid hormones and steroids. The results have shown a height less than 3 degrees centile only in a subjects and ranging from 10 degrees-50 degrees in the others, a weight greater than 97 degrees centile for the height age in all, a low response in GH to both stimuli in two subjects, an increased response to LHRH in FSH in two subjects. All other endocrine evaluations were in the normal range with the exception of insulin that resulted augmented in spite of normal glycaemic values. In conclusion, our data would suggest the existence of an eventual alteration of the hypothalamus-pituitary structures. Nutr Rev. 1991 Aug. A ventilator-dependent, extremely obese adolescent patient with Prader-Willi syndrome was nutritionally managed using a parenteral, protein-sparing modified fast. A solution containing only amino acids, electrolytes, vitamins, and macro- and micro-minerals helped create a ketotic state and facilitate weaning from the ventilator. This case demonstrates an unusual strategy for achieving weight loss and the challenge to provide adequate protein intake while limiting fluid intake. J R Soc Med. 1991 Apr. Acta Paediatr Scand. 1991 Jan. This report describes the polysomnographic findings and the respiratory alterations during sleep in a 20-year-old patient with the Prader-Willi syndrome. Nocturnal recordings and a variant of the multiple sleep latency test showed excessive daytime sleepiness, sleep onset rapid eye movement episodes, snoring and sleep apnea. Treatment with nasal continuous positive airway pressure normalized the respiratory pattern and the sleep structure, except for rapid eye movement sleep onset. Whereas upper airway obstruction and obesity may explain the respiratory disorders, as shown by their resolution with continuous positive airway pressure treatment, hypothalamic dysfunction could play a role in the disruption of the normal nonrapid eye movement/rapid eye movement sleep periodicity. Genet Couns. 1991. In this study we document the behavioral/emotional problems of 27 Prader-Willi syndrome (PWS) subjects assessed with the Achenbach Child Behaviour Checklist. Compared with normal subjects of the same age and sex, PW subjects showed significantly more problem behaviour. Of the PWS subjects 87% had total problem scores in the clinical range. No significant difference was found in the proportion of Prader-Willi patients scored in the clinical range on the Internalizing over the Externalizing syndrome. The need for systematic attention towards behavioral/emotional problems when PWS patients enter adolescence is emphasized. [ 2007 | 2006 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995 | 1994 | 1993 | 1992 | 1991 | 1990 | 1980-1989 | 1979 and prior ] |