Is there an excess risk for colorectal cancer in patients with ulcerative colitis and concomitant primary sclerosing cholangitis? A population based study.

BACKGROUND: Patients with ulcerative colitis have an increased risk of colorectal cancer. Duration, age, and extent of the disease at diagnosis are the only established risk factors. Patients with ulcerative colitis and concomitant primary sclerosing cholangitis (PSC) have been reported to have a higher frequency of colonic DNA aneuploidy and/or dysplasia than expected, findings indicating an increased risk of colorectal cancer compared with other patients with ulcerative colitis. METHODS: A population based cohort consisting of 125 patients with a verified diagnosis of PSC was followed up by linkage to the Swedish Cancer Registry for the occurrence of colorectal cancer. RESULTS: There were 12 colorectal cancers. Six cancers were diagnosed prior to the diagnosis of PSC. Among the 104 patients with an intact colon at the time of the diagnosis of PSC there was a cumulative risk for colorectal cancer of 16% after 10 years. Among the 58 patients with a diagnosis of ulcerative colitis and colorectal cancer prior to the diagnosis of PSC, there were five colorectal cancers corresponding to a cumulative risk of 25% after 10 years. CONCLUSIONS: Patients with ulcerative colitis and concomitant PSC seem to constitute a subgroup with a high risk for colorectal cancer.

Survival and risk of cholangiocarcinoma in patients with primary sclerosing cholangitis. A population-based study.

BACKGROUND: Endoscopic retrograde cholangiopancreatography was introduced in the early 1970s, making a more reliable diagnosis of primary sclerosing cholangitis (PSC) possible. Since then decreased survival and increased risk of cholangiocarcinoma have been reported. However, no population-based studies have quantified these outcomes. METHODS: A population-based cohort of 125 patients with a verified PSC diagnosis was followed up through linkage to the Swedish Death Registry and the Swedish Cancer Registry for occurrence of death and cholangiocarcinoma. RESULTS: The diagnosis of PSC was associated with a substantially decreased survival, with an overall 10-year survival of 68.8%. Patients with a diagnosis of inflammatory bowel disease (IBD) had a somewhat better prognosis, 71.8%, compared with 60% for patients without. Fourteen subsequent cholangiocarcinomas yielded a cumulative risk of 11.2% 10 years after diagnosis. Sex, duration of IBD, and colectomy influenced neither the survival nor the cholangiocarcinoma risk. CONCLUSION: Patients with PSC have a substantially decreased survival, which is most pronounced among patients without IBD.

Perianal Paget's disease: report of five cases.

Paget's disease of the anus is a rare perianal disorder. The condition is often associated with underlying invasive carcinoma. The prognosis is poor when rectal adenocarcinoma is present. Five own cases of perianal Paget's disease are presented. In two of our cases an underlying adenocarcinoma was found in the anorectum. Adenocarcinoma is sweat gland ducts was found in one case. One patient developed an adenocarcinoma in the anorectal junction four years after the Paget diagnose. In only one of our cases no underlying adenocarcinoma was found.

A combined electromyographic and cineradiologic investigation in patients with defecation disorders.

Records from 20 patients on whom defecography and electromyography were performed simultaneously because of defecation disorders were analyzed. According to the electromyographic investigation, the patients could be divided into three main groups: 1) normal sphincter reaction; 2) paradoxical sphincter reaction; and 3) combined reaction. Group A was characterized by a marked reduction of muscular activity during emptying and a pronounced closing reflex after emptying. This was followed by return of normal tonic activity. Patients in group B had no relaxation of the sphincters during emptying but a pronounced increased activity in the external sphincter and the puborectalis muscle. They also had severe emptying difficulties at defecography. No closing reflex was seen. In group C the electrical activity in the sphincters increased during moderate straining and when emptying was complete a clear closing reflex was seen. In this study, a dynamic visualization of the defecation together with a registration of electromyographic activity in the striated anal sphincters was performed. It was shown that patients with paradoxical sphincter reaction were lacking a closing reflex after emptying was complete. This has not been reported previously and is important evidence for the paradoxical defecation pattern. It was also shown that the patients with rectoceles had paradoxical sphincter reaction.

Intussusception of the rectum and the solitary ulcer syndrome.

A general review is given of the intussusception of the rectum, the paradoxical contraction of the pelvic floor syndrome and of the solitary ulcer syndrome. The latter is in most cases a symptom of the other two as is the descending perineum syndrome. The conditions are diagnosed by defecography, proctosigmoidoscopy and EMG. The etiologies are unknown. The treatment is either conservative or operative.

Disturbances in the defecation mechanism with special reference to intussusception of the rectum (internal procidentia).

Anorectal disorders that disturb normal defecation are described, especially intussusception of the rectum (internal procidentia). A review of 190 patients, half of whom were treated operatively and the other half conservatively, is presented. Diagnostic procedures, symptoms, and indications for operations are evaluated. We believe that intussusception of the rectum is a relatively common cause of difficult emptying of the rectum and, when the correct diagnosis is established, operation presents a fair chance for improvement.

Gastrin and somatostatin in plasma and gastric biopsy specimens in pernicious anemia.

Five patients with megaloblastic anemia due to deficiency of vitamin B12 and achlorhydria and six normal controls participated in the study. The patients with pernicious anemia (PA) had elevated plasma gastrin levels, as expected, and lower plasma somatostatin (SST) levels than the control patients. The amount of gastrin and SST in the antral mucosa did not differ in the two groups of patients. In the fundic mucosa, the patients with PA had increased tissue concentrations of both gastrin and SST as compared with the tissue concentrations in the controls. These findings of hormone tissue concentrations were correlated to the number of argyrophilic cells. Thus, in the antral mucosa the number of argyrophilic cells did not differ in patients with PA and in the controls. In the fundic mucosa, however, the number of argyrophilic cells was significantly elevated in patients with PA as compared with the controls.

Squamous-cell carcinoma of the anus. A follow-up study of 65 patients.

The five-year survival rate in 72 patients who underwent treatment for squamous-cell carcinoma of the anus in Stockholm County (1972 to 1978) has been studied. There were no differences in survival rates after rectum-preserving treatment (mainly consisting of irradiation +/- cytostatics +/- local excision) as compared with extensive surgery (abdominoperineal resection), even if the tumor size, location, and differentiation were taken into consideration. It therefore seems appropriate to suggest irradiation +/- cytostatics as the therapy of choice regardless of size, location, and differentiation of the tumor.

Randomised double blind trial of somatostatin in the treatment of massive upper gastrointestinal haemorrhage.

In order to evaluate the effect of somatostatin in the treatment of massive upper gastrointestinal bleeding a randomised double blind trial of 95 patients has been undertaken during a 28 months period. Patients with oesophageal varices have been excluded as well as patients with diabetes. All patients were endoscoped within eight hours of admission to the hospital, whereupon the source of bleeding and types of stigmata were assessed. Forty six patients, chosen at random, were given a 72 hour infusion of somatostatin, while the remaining 49 patients received infusion of placebo. The two groups were well matched for sex, age, and source of bleeding. On the day after admission, an additional endoscopy was performed at which eight patients in the somatostatin group and 16 in the placebo group were found to have a persistent bleeding. A total of five patients in the somatostatin group and 14 in the placebo group underwent surgery (Fisher's exact test, 2-tail, p = 0.04). Rebleeding occurred in six patients in the somatostatin group, of whom five experienced rebleeding after completion of the somatostatin treatment. In the placebo group, rebleeding occurred in five patients, of whom four rebled on the day after admission. The need for blood transfusions and the mortality rate did not differ significantly between the two groups. No toxic side effects were found as a result of the infusion of somatostatin. In this study, somatostatin reduced the number of patients needing surgery with massive upper gastrointestinal bleeding.

The effect of cholecystokinin on postoperative bowel function.

Cholecystokinin (CCK) was used in a randomized double-blind trial to evaluate its effect on post-operative paralytic ileus. Sixty patients were admitted to the study. The return of postoperative intestinal motility was registered with subjective and/or objective methods. No differences were found between the CCK group and the placebo group.

Endoscopic sclerotherapy v. conservative management of bleeding oesophageal varices. A 5-year prospective controlled trial of emergency and long-term treatment.

A comparative trial was made of conservative therapy (balloon tamponade and/or vasopression infusion) alone (control group) or with addition of acute and serial endoscopic injection sclerotherapy (ST group). The 107 unselected patients, mainly with alcoholic cirrhosis, were randomly allocated to these groups, which were comparable as regards Child's grading and clinical and laboratory findings. For emergency ST a fibreoptic endoscope and Williams sheath were used. In initial control of index bleed and hospital mortality the two groups did not differ significantly. The median follow-up was 15 and 28 months (minimum 1 year). Supplementary ST (mainly out-patient) gave variceal eradication in 34 of 41 patients, with most failures in persistent alcoholics. A calculated risk factor for rebleeds was 3.6 times higher in the controls than in the ST group. Mortality rate showed no intergroup difference. The cause of death mainly was variceal bleeding in the controls, but not in the ST group. Major complications of treatment occurred in c. 15% of all patients.

Non-surgical control of upper gastrointestinal bleeding.

The non-surgical methods to control upper gastrointestinal bleeding include some recently developed endoscopic techniques (laser photocoagulation and electrocoagulation). Among drugs proposed to be effective in controlling upper gastrointestinal bleeding, somatostatin is considered particularly promising. The experience with these techniques and with somatostatin is reviewed.

Long-term effects of papillotomy.

Forty-three patients who were papillotomied according to Doubilet were followed up for 20-25 years after the papillotomy in an attempt to predict the long-term effects of endoscopic papillotomy (EPT). The indications for the initial papillotomy was common bile duct stones in 20 patients, and elevated serum amylase in 23. The papillotomy was performed in conjunction with a cholecystectomy. No negative effects were found at follow-up, and this study therefore seems to show that the long-term risks of EPT are minimal.