Long-term Result of Treatment of Prader-Willi Syndrome by Scopinaro's Bilio-pancreatic Diversion. Study of Three Cases and the Effect of Dextrofenfluramine on the Postoperative Evolution.

The Prader-Willi Syndrome shortens the life of patients due to the morbid obesity which it entails. The compulsive hyperphagia associated with it makes a dietetic treatment or a gastroplasty difficult. This study presents the case histories of three patients suffering from the Prader-Willi syndrome who were operated on by means of a Scopinaro's bilio-pancreatic diversion. Following a marked reduction the first year, the weight loss stabilized and then tended to diminish. The observation of three cases which continued for two and a half to six years did not reveal any considerable metabolic problems. The deficiency of iron, vitamins D and B12 as well as folic acid had to be made up by supplementation. These results are comparable with the most favorable ones in the literature. Even if the effect on the weight loss is not spectacular, the operation manages to hold off the development of the obesity, inexorable for those with the Prader-Willi syndrome, and prevents lethal complications, without having notable side effects. Lifting coercive dietary measures improves the quality of life.

[Recurrence of a benign pleural mesothelioma 18 years after excision. Review of a series of 33 patients with a primary tumor of the pleura from 1958 to 1986]. / Récidive d'un mésothéliome pleural "bénin" 18 ans après l'exérèse. Revue d'une série de 33 malades atteints d'une tumeur primitive de la plèvre de 1958 à 1986.

A 60 year old woman developed recurrence of a localized pleural mesothelioma 18 years after initial exeresis. Based on data from 33 cases of primary tumor of pleura treated in the UCH, Vaudois, Switzerland between 1958 and June 1986, symptomatology, treatment and prognosis of pleural mesothelioma a are discussed. Findings suggest the need for long-term follow up surveillance of patients with benign mesothelioma.

[Saturnism caused by skin absorption of a drug]. / Saturnisme par résorption cutanée médicamenteuse.

A case of chronic lead poisoning is described in a woman presenting with abdominal pain due to cutaneous absorption of a lead soap. We have found no other case of intoxication by this route in the literature.

[Pseudo-obstruction and colonic ischemia]. / Pseudo-obstruction et ischémie colique.

We report the case of a 78 year old male schizophrenic with pseudo-obstruction of the colon complicated by patent-vessel ischaemia that was diagnosed after sub-total colectomy. Surgery was motivated by the chance discovery of a pneumo-peritoneum without peritonitis and without any evidence of perforation in the gastro-intestinal tract. We believe that early surgery is required in patients with pseudo-obstruction of the colon, to prevent complications such as ischaemia of the distended bowel wall which, though rare, is also well recognised.

[Continuous thoracic drainage and suction. Comparative study of the performance and price of multiple-use systems (separate bottles) and single-use (compact systems) disposable systems on the market]. / Drainage thoracique continu et aspiratif. Etude comparative des performances et du prix des systèmes à usages multiples (bocaux séparés) et à usage unique (systèmes compacts) disponibles sur le marché.

A study of 12 pleural drainage systems has been conducted. The system was disposable in 8 instances and allowed multiple use in 4. Each system was analyzed in relation to 15 performance criteria and 15 safety criteria. The cost of use per patient was compared. In conclusion, compact and disposable pleural drainage units can be recommended since they (a) offer superior technical performance, (b) are more reliable, (c) offer increased safety, (d) are easier to monitor, (e) are much safer with respect to cross-infection, (f) are less noisy in some cases, (g) are more useful in apportioning responsibility between surgeons and nursing staff, and (h) are less costly.

[Diffuse digestive ganglioneuromatosis]. / Ganglioneuromatose digestive diffuse.

Discovery of intestinal ganglioneuromatosis implies the presence of the MEN IIb syndrome or, more rarely, von Recklinghausen's neurofibromatosis. The two conditions are due to dysfunction of the neural crest. This very rare intestinal pathology is illustrated by two observations. The intestinal motility disorders, due to the diffuse proliferation of the peripheral autonomous nervous system which ganglioneuromatosis represents, may have serious implications and indeed prove fatal. The etiologic role of excessive nerve growth factor production in these two entities is mentioned. The physiopathologic implications of an increase in vasoactive intestinal polypeptide, and of possible ectopic secretion of calcitonin by ganglioneuromatosis, are discussed.

[Medullary cancer of the thyroid gland]. / Les carcinomes médullaires de la thyroïde.

Thirty-one cases of medullary carcinoma of the thyroid have been studied over the past fifteen years at the University Hospital of the Canton of Vaud, Switzerland (CHUV). Twenty cases were of sporadic nature and eleven presented as part of the familial MEN II syndrome (multiple endocrine neoplasia), one of which showed the classical features of the rare MEN IIb type. It is important to distinguish between the familial and sporadic cases, because membership of the former group implies the investigation of associated endocrinopathies (pheochromocytoma, hyperparathyreoidism) and study of the family tree as the syndrome is autosomal dominant. Medullary carcinoma of the thyroid is a constant feature of the MEN II syndrome and is the cause of premature death in these patients. The familial type should be suspected if the carcinoma appears early in life, is located in the superior pole of the thyroid or is bilateral or multicentric, if the histology shows hyperplasia of the C cells and, of course, if there is a history of surgery for pheochromocytoma or hyperparathyroidism. Although total thyroidecomy is the rule for these familial cases, its role is debatable in sporadic medullary carcinoma of the thyroid. Postoperative follow-up of these patients is based on serum calcitonin determination, as this is an extremely sensitive marker. The ten year survival rate is 50%, with the worst prognosis in MEN IIb type.