Steffen Bülow

Mandibular osteomas in familial polyposis coli

Orthopantomograms of the mandible were performed on 46 patients with familial polyposis coli having no clinical signs of Gardners syndrome and on 46 control patients matched according to age and sex. Thirty-five patients (76.1 per cent) and two (4.3 per cent) controls had osteomas (P<0.0005). It is concluded that orthopantomography of the mandible is a valuable diagnostic supplement to prophylactic proctosigmoidoscopic examination of first-degree relatives of polyposis patients. Due to the frequent occurrence of mandibular osteomas in polyposis patients without clinically detectable extracolonic manifestations, it is suggested that “Gardners syndrome” is no longer considered a clinical entity.

Cimetidine for Severe Gastroduodenal Haemorrhage: A Randomized Controlled Trial

During a period of 12 months, 88 patients with severe haemorrhage from gastric or duodenal ulcers or from erosive gastritis completed a double-blind trial of either cimetidine or placebo. Only patients needing immediate blood transfusion were admitted to the trial. It was found that in patients with severe bleeding from gastric or duodenal ulcers neither the severity of bleeding nor the incidence of emergency surgery was reduced by cimetidine. Furthermore, the treatment did not improve the mortality rate. It is concluded that patients with severe bleeding from gastric or duodenal ulcers will not benefit from immediate treatment with intravenous cimetidine.

Dental anomalies in familial adenomatous polyposis coli

Forty-seven Danish and 50 Finnish patients with familial adenomatous polyposis coli (FPC) were studied by panoramic tomography (PTG) of the mandible, which showed dental abnormalities in 17% of the cases. Eleven patients (11%) had supernumerary teeth and/or compound osteomas, and nine patients (9%) had impacted permanent teeth. The frequency of the dental anomalies was statistically significantly higher in FPC patients than in the normal population; it is therefore concluded that this dental abnormality should be included in the list of extracolonic manifestations that may occur in any FPC patient. The frequency of dental anomalies was higher in Finnish than in Danish patients, probably owing to a higher frequency of extracolonic manifestations in the Finnish series.

The risk of developing rectal cancer after colectomy and ileorectal anastomosis in Danish patients with polyposis coli

Fifty-eight Danish polyposis patients had been treated with colectomy and ileorectal anastomosis by the end of 1982. Three of these patients developed rectal cancer despite regular proctoscopic control, but one was probably an overlooked synchronous cancer. The cumulative risk at five and ten years after operation (actuarial method) was 3.5 and 13.3 per cent (95 per cent confidence limits 0–10.3 and 0–70.3 per cent, respectively), which seem to be acceptably low figures. This study supports the assumption that colectomy and ileorectal anastomosis is still the operation of choice in polyposis patients without rectal cancer.

Mandibular Osteomas in Colorectal Cancer

Orthopantomography of the mandible was carried out on 50 patients with colorectal cancer without known familial disposition. Twelve patients (24%) had osteomas, as against 5% in the control group (P less than 0.001). Since an increased occurrence of mandibular osteomas has earlier been demonstrated in cases of familial polyposis coli and the family cancer syndrome, we must conclude that genetic factors are presumably of more importance in the development of colorectal cancer than previously assumed.

Hereditary colon cancer syndromes: an in vitro study

Assays of in vitro biological properties associated with cellular transformation revealed differences in cultured skin cells within the heritable colon cancer syndromes with and without polyposis coli. Such evidence suggested that an in vitro panel of biological assays could be used to recognize genotypes which increase the risk of colon cancer prior to clinical expression and supported the concept, long assumed on clinical differences, of genetic heterogeneity within the heritable colon cancer syndromes.

The Danish polyposis register: Description of the methods of detection and evaluation of completeness

A manual Danish register of patients with familial polyposis coli and their family members was established in 1971. The methods of collection of propositi, preparation of pedigrees, collection of call-up cases, and the organization of the register are described. A country-wide prophylactic proctosigmoidoscopic examination of first-degree relatives, aged 10 to 59 years, resulted in the detection of 42 call-up cases and by December 1982, 319 cases of polyposis were registered in 94 families. An evaluation of the number of collected propositi and call-up cases shows almost complete registration.

The Incidence and Prevalence of Familial Polyposis Coli in Denmark

Based on the Danish Polyposis Register, the frequency of familial polyposis coli was estimated at the end of 1982. The mean annual incidence rate of polyposis coli has remained rather constant at approximately I×10−6×yr−1 since the forties. The life-time risk of developing polyposis coli is about 1×10−4. The point prevalence rate was 26×10−6 at the end of 1982. The number of cases found in sibs and children of probands made it probable that a nation-wide prophylactic examination of first degree relatives resulted in a complete identification of affected family members. The completeness of the Danish Polyposis Register in the period 1976–82 was estimated to be 90% and the validity of the diagnosis is considered to be 100%.

In Vitro Tetraploidy in Patients with Non-Hereditary Colorectal Adenoma and Carcinoma: Support for a Genetic Influence on the Pathogenesis of Colorectal Carcinoma

One hundred and seventy-two patients (76 patients with colorectal cancer, 46 with adenoma of the colon or rectum, and 50 controls) were investigated for in vitro tetraploidy in dermal fibroblast monolayer cultures. The incidence of increased in vitro tetraploidy (IVT) among patients with adenoma or carcinoma was significantly different from that of controls. There was no difference in the incidence of IVT between the adenoma patients and those with colorectal cancer. IVT was investigated among different subgroups of adenoma and colorectal cancer patients and was only found to be related to an inherited tendency to colorectal cancer.

Chromosomal studies in familial polyposis coli

A study of five patients with familial polyposis coli did not demonstrate any structural abnormalities of prometaphase chromosomes from methotrexate synchronized peripheral blood lymphocytes. No chromosomal heritable fragile sites were observed, nor was sister chromatid exchange increased.