Relationship between colorectal neoplasia and primary sclerosing cholangitis in ulcerative colitis.
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Biomedical subjects
Publications and source records attributed to F W Nugent.
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During a 13-year period, 213 patients with ulcerative colitis who had no clinical or endoscopic evidence of colonic carcinoma were enrolled in a biopsy surveillance program for dysplasia and carcinoma. The aims of the study were to determine whether such a program could decrease the cancer risk in this group of patients, to determine whether patients with a low risk of carcinoma could be identified, thus enabling them to retain their colon, and to accomplish these goals with a reasonable expenditure of resources. Eighteen patients had dysplasia detected in the initial biopsy specimens; 15 of these patients underwent colectomy, and 7 had unsuspected carcinoma (1 Dukes' stage A, 2 stage B, and 4 stage C). Eleven patients had dysplasia detected during follow-up; 7 of these patients had colectomy, and only 1 patient had carcinoma (Dukes' B). Dysplasia developed in 5 of 20 patients with indefinite changes on initial biopsy samples; 3 of these patients underwent colectomy, and 1 patient had carcinoma (Dukes' B). There was no difference in the prevalence of dysplasia between patients with left-sided disease and patients with extensive disease. With the exception of 2 patients with inadequate surveillance, there has been no clinical evidence of carcinoma in any of the 148 patients whose biopsy results remained negative throughout the study; carcinoma has not developed in any of 175 patients without dysplasia on initial biopsy sample. All 4 patients who died of carcinoma had high-grade dysplasia in their initial colonoscopic biopsy samples. It is concluded that a biopsy surveillance program can be an effective aid in helping control the risk of carcinoma in patients with long-standing ulcerative colitis, that the short-term risk of carcinoma for patients with negative biopsy results is low and colectomy for risk of carcinoma can be deferred in this group, and that patients with extensive and left-sided disease share the same risk of the development of dysplasia.
Of 178 patients with sclerosing cholangitis treated since 1950, 88 patients had associated inflammatory bowel disease, 72 had no such history, and 18 had iatrogenic injury or stone disease. A total of 233 biliary operations were performed, with a 75% rate of temporary improvement after initial operation. Subsequent operations resulted in a lower success rate and a higher mortality rate. Radiologic findings included predominant extrahepatic, intrahepatic, and diffuse disease in 29%, 28%, and 43% of patients, respectively; no survival differences were noted. Seventy-five of one hundred three deaths (73%) were related to liver failure, bleeding, or sepsis. Of 14 patients undergoing portosystemic shunt, 13 died of surgical complications or related disease. Orthotopic liver transplantation was performed in 16 patients and resulted in eight deaths, mainly in patients who had previously undergone extensive surgical treatment. No survival differences were seen between the patients with inflammatory bowel disease, those without the condition, or those who had colectomy. Surgical treatment in patients with sclerosing cholangitis should be minimized. Orthotopic liver transplantation should be offered as the treatment of choice for patients with portal hypertension, refractory cholangitis, advanced cirrhosis, or progressive liver failure.
Hepatotoxicity secondary to the administration of thiabendazole has been rare since this drug was produced in 1964. In 14 of 15 patients reported previously in the literature, severe intrahepatic cholestasis resolved within seven months of the onset of illness. A recent report documented the progression to cirrhosis in a 15th patient. We report the second case of a patient with intrahepatic cholestasis that developed after treatment with thiabendazole and progressed to severe micronodular cirrhosis.
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Prior studies confirm the increased incidence of carcinoma of the colon in chronic ulcerative colitis. The authors reviewed clinical and histologic data retrospectively in 23 patients with colon carcinoma and chronic ulcerative colitis. Twenty-two of these patients had dysplasia of colonic epithelium remote from the cancer. The authors prospectively reviewed clinical data and rectal and colonoscopic biopsy specimens on 36 patients with chronic ulcerative colitis, 12 with Crohn's colitis, and 12 with miscellaneous disorders. Eight patients with chronic ulcerative colitis had dysplasia; 6 have had colectomy, and 2 of these had carcinoma. No patient without chronic ulcerative colitis had dysplasia. Patients with chronic ulcerative colitis should have periodic rectal and colonoscopic biopsies, and those with moderate to marked dysplasia require colectomy because of the increased risk of colon carcinoma.
The metabolic consequences and the clinical results of total pancreatectomy for end-stage chronic pancreatitis are detailed. This experience, accumulated between the years 1952 and 1976, adds 26 cases to the world's literature, which now contains 53 cases reported singly or in small series. The patients were selected on the basis of far-advanced, incapacitating disease in three patients and the failure of 62 previous related operations in 23 patients. Total pancreatectomy was performed with a zero hospital mortality but with serious inhospital morbidity. After discharge, 24 patients required 55 hospital readmissions and 18 related surgical operations; 12 patients have died, five of unrelated causes. Of 14 living patients, five consider themselves to be in good to excellent condition, six in fair condition, and three in poor condition. Twelve thought they were improved over their preoperative state. Our estimate of the result in living patients was similar. In those 12 who died, only four had satisfactory results. The results are also evaluated relative to level of activity, presence of abdominal pain, use of narcotics, weight, alcohol consumption, and the efficiency of endocrine and exocrine replacement. In general, the procedure is effective for relief or amelioration of pain, but the patients' personalities preclude complete adaptation to the apancreatic state rehabilitation. A failure to thrive is the common denominator.
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Crohn's disease of the duodenum is uncommon, occurring in approximately 2% of patients with Crohn's disease. Approximately 165 cases have been reported in small series in the literature. Our report includes 36 patients, most of whom had symptoms of duodenal disease coincident with or after obvious disease elsewhere in the gastrointestinal tract, although occasionally duodenal disease developed first and rarely disease was confined to the duodenum. Upper abdominal pain and symptoms of gastroduodenal obstruction are the commonest patterns of presentation. Significant weight loss is common, and occasionally major upper gastrointestinal bleeding occurs. The commonest pattern of involvement was contiguous disease of the proximal duodenum and distal stomach. Endoscopically, diffuse granularity, nodularity, and ulceration are seen accompanied by lack of distensibility of the involved area. Granulomas are rarely found in endoscopic biopsies. A bypass procedure was carried out on 18 patients, 15 of whom continue to be free of symptoms with an average follow-up of 6-6 years. When symptoms of obstruction dictate, operative bypass is accompanied by favourable long-term results in the large majority of patients.
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Three types of colitis are now recognized. In the late 1950's and early 1960's, 51 per cent of our patients having colectomy had classic chronic ulcerative colitis. Of our patients operated on at that time, 39 per cent had Crohn's colitis and 10 per cent had colitis of indeterminate type. These three types of colitis should be differentiated because of the high risk of cancer of the colon in patients with long-standing chronic ulcerative colitis. We have not seen an increased risk of carcinoma associated with Crohn's colitis. We performed resection with anastomosis in approximately one third of patients with Crohn's colitis, but recurrent disease developed in two thirds. In contrast, recurrent Crohn's disease developed in only 5.7 per cent of patients having colectomy with ileostomy. Toxic megacolon associated with Crohn's colitis has been seen only in the early clinical stage of the disease. From the standpoint of management and prognosis, attempts to differentiate between the two major types of colitis are appropriate. We have outlined some of the basic clinical and pathologic differences between these two types of colitis.
Serum lysozyme (muramidase) concentrations were measured in three groups of patients: control, ulcerative colitis and proctitis, and Crohn's disease. The mean +/-SD for each group was: control, 7 +/- 2; ulcerative colitis and proctitis, 7 +/- 2; and Crohn's disease, 10 +/- 4. Although a significant difference was seen between values in patients with Crohn's disease and values observed in those with ulcerative colitis or control patients, an important overlap was found between these groups. Further studies are necessary to explain the disparate results between this study and previous reports.
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