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Background: Prader-Willi Syndrome (PrWS) is an uncommon neuroendocrine disorder of genetic origin, described in 1956 by Prader, Labhart and Willi. The main clinical manifestations in the adult are mental retardation, hyperphagia with gross obesity, hypogonadismcriptorhismus and short stature. The life expectancy of the affected individual ranges between 20 and 30 years - rarely beyond - due to complications related to excessive obesity. Sustained dieting combined with behavior modification programs, as well as gastric restrictive surgery for obesity, proved to have a high failure rate in PrWS, due to the patients' inability to cooperate in changing their eating habits. Methods: Billiopancreatic Diversion (BPD), which does not require the patient's cooperation in changing eating habits after surgery, was performed in two PrWS patients (13- and 22-years-old), both with excessive obesity, severe respiratory distress, day sleepiness and limited mobility. Results: Two years after surgery, the 13-year-old had lost 80% of her overweight, while the 22-year-old, after 1 year, had lost 34%. Recent laboratory tests showed normal data in both patients. Their respiratory distress had subsided completely, their mobility improved dramatically, and their self-image and alertness enhanced. Conclusion: BPD resulted in an improved quality of life in these patients. [ABSTRACT FROM AUTHOR]

Liver damage following biliopancreatic diversion (BPD) for obesity is an infrequent, dangerous and, when left untreated, even potentially fatal complication. The laboratory expression of developing liver damage is the progressive deterioration of the liver enzymes over 70 i.u., persisting and progressing beyond 6 months after surgery. Until recently, the treatment for this complication consisted of hospitalization for total parenteral nutrition and then, if this proved unsatisfactory, surgical reversal. In this report, a new therapeutical approach to this condition is presented. Four patients who, following a BPD for obesity, developed deterioration of the liver enzymes (AST, ALT over 70 i.u.) which persisted and progressed beyond the first 6 months after surgery, were given a treatment consisting of the ambulatory administration of 'Viokase' tablets, an uncoated pancreas extract with a high protease content (30,000 i.u.) taken with protein-rich meals. After 2-4 months of Viokase treatment, in all four patients the liver enzymes returned to normal levels: AST from 160, 86, 120 and 89 i.u. to 26, 20, 24 and 27 i.u. Two patients who complained of diarrheic stools (ten per day) received metronidazole for several days, dramatically improving their condition and returning their daily stool count to normal. [ABSTRACT FROM AUTHOR]

Thirteen patients, following a bilio-pancreatic diversion procedure for obesity, developed severe nutritional complications such as protein deficiency, anemia, hypocalcemia and/or gastro-intestinal problems (uncontrollable diarrhea). They were subjected to a new therapeutic approach-the administration of pancreas extract tablets Viokase, containing protease, lipase and amylase. This, together with protein-rich food resulted after 2-4 weeks of treatment in a drastic reduction in the number of daily stools: from 10-12 per day to 4-6 per day, as well as the disappearance of the accompanying foul smell and the malodourous gas emissions. After a further 4-8 weeks of treatment of those patients with protein deficiency, the serum protein-albumin levels as well as the Hgb and Ca levels rose to near normal values: protein from 4.8-5.5 g% to 6-6.5 g%; albumin from 1.8-3 g% to 3.4 g% and above; Hgb from 7-9 g/dl to 11-12 g/dl; and Ca from 7.5-7.8 mg% to 8-9 mg%. None of the patients treated with pancreas extract for protein deficiency required rehospitalization for intravenous protein replacement, nor was there any need observed for operative revision or takedown in these patients. [ABSTRACT FROM AUTHOR]