[Epidemiologic data, clinical and prognostic features of autosomal dominant polycystic kidney disease in a French region]. / Données épidémiologiques, cliniques et pronostiques de la maladie polykystique autosomique dominante dans une région française.

Epidemiological study on autosomal dominant polycystic kidney disease (ADPKD) was undertaken in a French region from 1988 to 1993. This survey was led in a population of 410,000 inhabitants and 84 kindreds with ADPKD and 296 affected members were studied. Prevalence of ADPKD in the studied region was calculated to 1/1111 inh. Renal prognosis was evaluated according to the Kaplan-Meier method in 296 affected subjects of whom 212 were members of propositus kindreds. In our region 17% of patients had ESRD by the age of 50 years, 47% by the age of 60 years and 70% by the age of 70. No significance difference was found between males and females. The influence of the sex of the parent from whom ADPKD was received on the renal prognosis of the disease in affected descendants was evaluated. Anticipation of ESRD for at least one offspring inheriting ADPKD from parent was found in 15 (38%) out of 39 families, without genetic imprinting linked to gender. Mean survival to ESRD for fathers transmitting ADPKD to offspring (52 +/- 10 years) was significantly earlier compared to that for mothers transmitting ADPKD (61 +/- 10 years, p < 0.001), therefore that for siblings inheriting the disease from their fathers (sons: 49 +/- 7 years, daughters: 51 +/- 9 years) and for those inheriting ADPKD from their mothers (sons: 57 +/- 10 yrs, p < 0.01, daughters: 55 +/- 6 yrs, p < 0.02). Prevalence of de novo mutations was evaluated to 1/135,000 inh. Adult polycystic disease of the liver (APLD) was studied in 82 kindreds with 158 ADPKD affected members by ultrasonography and/or CT. In patients with APLD, 49/84 (58.3%) were females compared to 46/74 (62.2%) in those without APLD. Familial APLD (at least 2 affected members and all with APLD) was demonstrated in 22/27 APLD kindreds (81.5%). Familial ADPKD without APLD (at least 2 affected members and all without APLD) was demonstrated in 12/12 kindreds (100%). Renal prognosis of ADPKD in 84 APLD pts was compared to that in 71 non-APLD pts, in whom mean age was not different at the time of the study. In APLD pts 28/84 (33.3%) had reached ESRD compared to 23/71 (32.3%) non-APLD pts (ns). The occurrence of stroke in ADPKD patients was documented in 24/231 pts (10.4%) from 11/82 kindreds (13.4%). Family history of cerebro-vascular event was found in 4/11 kindreds (36%).

Laparoscopic gastrectomy for obstructing duodenal ulcer.

A case of bilateral truncal vagotomy and antrectomy for pyloric stenosis is presented. Other procedures for laparoscopic treatment of peptic ulcer disease are discussed.

[Treatments of inguinal hernias by celioscopy. Preliminary results apropos of 162 cases]. / Cures de hernies de l'aine par coelioscopie. Résultats préliminaires à propos de 162 cas.

Mesch insertion is a reliable means for repairing inguinal hernias. Results in 162 patients treated laparoscopically via an intraperitoneal approach are reviewed herein. Analgesics were not required after the first postoperative day in 71% of patients. Fourteen patients were treated on a day-care basis. There were two recurrences due to inadequate fixation of the mesch to Cooper's ligament. The two patients who developed an infection did not require removal of the patch and had a strong, completely healed wall at the follow-up evaluation after four and ten months, respectively. The number of patients and duration of follow-up are still inadequate. However, these preliminary data warrant continued use and evaluation of this technique.

[A case of polypoid solitary colonic plasmocytoma]. / Un cas de plasmocytome solitaire du côlon à forme polypoïde.

One case of polypoid solitary plasmacytoma in the sigmoid colon of a 60 year-old woman is presented. Plasmacytomas are rarely observed in the gastrointestinal tract and are especially uncommon in the côlon. Eight cases were published since 1972. They show a mean age of 46.3 years, a sex-ratio of 1/1, varying revealing symptoms, and no preferential colonic location. Two differential diagnosis must be discussed: an inflammatory pseudotumor, and a multiple myeloma related tumor. Multiple myeloma must be ruled out by a complete radiographic and biological research. Surgery is the only therapy described and seems to be efficient.

[Tubular gastrostomy using celioscopy]. / Gastrostomie tubulée sous coelioscopie.

Surgical feeding gastrostomy is attended by a significant morbidity and mortality. Today, the percutaneous endoscopic route is regarded as the best method, but it is not feasible in case of pharyngo-oesophageal stenosis and has its own drawbacks. A new method using laparoscopy to perform a Depage-Janeway gastrostomy is presented here. The theoretical advantages of this method are clear: the limited parietal damage should avoid cicatricial complications, and the limited ventilatory aggression should reduce the amplitude of respiratory complications. Moreover, in surgical practice this is a simple, rapid and efficient operation.

[Giant diverticulum of the colon. Apropos of a case disclosed by complication]. / Le diverticule géant du côlon. A propos d'un cas révélé par une complication.

The authors report a case of giant colonic diverticulum. This is a rare disease which can be revealed by a complication. Surgery is absolutely necessary.

[Therapeutic diagram in advanced cancers of the esophagus]. / Notre schéma thérapeutique dans les cancers avancés de l'oesophage.

Only palliative treatment may be contemplated when advanced oesophageal cancers present with dysphagia. Operability depends on respiratory, hepatic and nutritional status. Resectability may be assessed on the information provided by bronchoscopy, thoracic CT scan and surgical exploration. Advanced resectable oesophageal cancers require oesophagectomy without thoracotomy and radiotherapy. We performed 93 oesophagectomies in 106 advanced oesophageal cancers with a mortality rate of less than 2%. Non resectable advanced oesophageal cancers require bypass procedures. We performed 13 in the 106 cases. Inoperable advanced oesophageal cancers require radiotherapy in the absence of a fistula, laser therapy or an endoprosthesis for dysphagia.

[Rectal stenosis. A rare complication of chronic salpingitis caused by an intrauterine device]. / La sténose rectale. Une complication rare des salpingites chroniques sur dispositifs intra-utérins.

The authors describe one case of rectal stenosis complicating chronic salpingitis in a patient carrying an intrauterine device. This observation is peculiar in that the inflammatory fibrous reaction is very intense, spreading all over the pelvis and forming a pseudotumoral mass sheathing the rectum. The clinical signs were mainly digestive, including a rectal syndrome: cramplike pelvic pain before defecation, tenesmus, constipation, abdominal pain and induration of the anterior aspect of the rectum observed during the clinical examination. Radiological examinations (barium enema, ultrasound, CT) show a tissue mass within the pelvis, with considerable thickening of the rectal wall. Ultrasound-guided biopsy in the pelvis yielded only nonspecific inflammatory signs with dominant fibrosis. The diagnosis of rectal stenosis caused by chronic salpingitis complicating the presence of an IUD was made only during surgery.

[Precocious puberty associated with Silver's syndrome]. / Puberte precoce et syndrome de Silver: une association inhabituelle.

Precocious puberty has been reported as an important feature of the Silver-Russell syndrome. The present case refers to a boy entered puberty at the chronological age of 11 years and the bone age of 6 years 6/12. Criteria for precocious puberty in this syndrome are discussed by comparison with previously published cases, and it is concluded that abnormalities in clinical pubertal development are quite unusual in this syndrome.