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Prader-Willi syndrome (PWS) is a rare genetic disorder resulting from lack of expression of the paternally derived chromosome 15q11-13, associated with several complications, including pubertal disorders, short stature, hyperphagia, obesity, glucose metabolism abnormalities, scoliosis, obstructive sleep apnea syndrome (OSAS) and behavioral problems. We report the case of a girl affected by PWS who presented at the age of 5.9 with premature pubarche, accelerated linear growth and advanced bone age (BA).

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Starting from biochemical hypotheses on hepatic encephalopathy, which presuppose at the basis of this disease first of all some modifications of the plasmatic values of octopamine, ammonia, serotonin and of particular aromatic aminoacids, the Authors compared the levels of ammoniemia and of octopaminemia with eegraphic data, in two groups of cirrhotic patients, operated of porto-cava anastomosis (16 patients) ad of mesenterico-cava by-pass (24 patients). All this in order to value if there are significant correlations between these values and the cerebral damage and also if there are differences between the two types of operation. Attained data show a good correlation between neuropsychiatric symptomatology and importance of the cerebral damage noticeable at the E.

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