Faculty Bibliography

OBJECTIVES: Reports of Crohn's disease (CD)-associated colorectal carcinoma are being cited in the medical literature with increasing frequency. Our aim was to identify subgroups of patients with risk factors that may account for this. METHODS: We reviewed the medical records of 16 patients with the simultaneous diagnosis of CD and colorectal carcinoma and, in addition, reviewed previously reported cases of CD-associated colorectal carcinoma. RESULTS: Eight male and eight female patients presented with 18 carcinomas: four right colon, four transverse, two descending colon, and eight rectal lesions. Median age at presentation was 48 yr. The mean duration of CD before presentation of carcinoma was 19.7 yr. Two lesions were discovered in strictured bowel segments. Two patients had multiple cancers. One had simultaneous cecal and left colon adenocarcinomas. The other underwent resection of a right colon lesion and 5 yr later presented with transverse colon carcinoma. Eight patients had rectal cancer; all were diagnosed preoperatively. Six of these patients had a history of severe perianal CD. Six had undergone multiple incision and drainage procedures for perirectal abscesses and fistulas. Two developed malignancies in defunctionalized rectal stumps. One of these patients presented with simultaneous squamous rectal carcinoma and papillary bile duct cholangiocarcinoma. CONCLUSIONS: Gastrointestinal malignancy in association with CD has been reported. Symptoms of chronic inflammatory disease may obscure clinical manifestations of occult malignancy and thereby delay diagnosis. Crohn's patients with long-standing anorectal or perianal disease and stricture may well warrant surveillance endoscopy and biopsy of involved areas with the hope of earlier detection and treatment of these rectal cancers

Crohn's disease and neutropenia unrelated to drug therapy is a rare association. We present a case of Crohn's disease associated with cyclic neutropenia. Our patient, observed over 10 yr, is unique due to the lack of recurrent bacterial infections or other associated sequelae of the neutropenic state. There was no consistent association between white blood cell cycling and exacerbations of her Crohn's disease. However, the latter did appear to worsen the neutropenic state. A clear pattern of cyclic neutropenia became apparent only when the disease was quiescent and all medications had been discontinued. We report this case for its value in pursuing clinically and therapeutically appropriate options, including surgery and drugs with neutropenic potential, in patients with Crohn's disease and, possibly, white blood cell abnormalities.

We have compared the effect of ursodeoxycholic acid with placebo on the clinical state, blood liver chemistries and serum and urinary bile acids in four patients with primary biliary cirrhosis. All parameters were evaluated monthly, and bile acid composition was measured by capillary gas-liquid chromatography. At the time of admission, all patients showed intense pruritus, and their serum alkaline phosphatase, AST and ALT levels were elevated 4.3, 2.7 and 2.3 times over control values. Serum bile acids were elevated almost 38-fold with 2.5 times more cholic acid than chenodeoxycholic acid. Urinary bile acid output was elevated 28 times the control values, and 36% were 1 beta-hydroxycholic acid, 1 beta-hydroxydeoxycholic acid and hyocholic acid (3 alpha,6 alpha, 7 alpha-trihydroxy-5 beta-cholanoic acid). Three months of placebo administration did not significantly affect the clinical or biochemical presentations, and the serum and urinary bile acid composition did not change. In contrast, ursodeoxycholic acid feeding (12 to 15 mg per kg per day) for 6 months abolished pruritus in two and lessened itching in two subjects and reduced serum alkaline phosphatase, AST and ALT levels by 21, 35 and 47%, respectively. The mean values for the total serum bile acid concentrations in these patients declined 26% from the pretreatment value, but the proportion of ursodeoxycholic acid increased from 3 to 40% of the total bile acids; thus, total fasting serum endogenous bile acid levels decreased almost 50%. Similar changes were noted in the urinary bile acids, in which ursodeoxycholic acid became the major bile acid, and approximately 18% were hydroxylated at C-1, C-6 and C-21.(ABSTRACT TRUNCATED AT 250 WORDS)

Isolated granulomatous disease was identified in a gastrectomy specimen after a gastrointestinal hemorrhage in a 61-year-old white woman. She died postoperatively. No other areas of granulomatous tissue were identified at autopsy. Ten years previously, pulmonary sarcoidosis was proven on lung biopsy. Conceivable, other reported cases of unexplained 'idiopathic' granulomatous disease of the stomach may represent residua of prior disseminated sarcoidosis

Extensive occlusive mesenteric vascular disease is described in a 41-year-old man, resulting in aphthous ulcers of the stomach, small bowel, and colon. Ischemic disease was confirmed angiographically and ultimately by surgical intervention. No evidence of inflammatory bowel disease was detected. Occlusive vascular disease must be considered in the differential diagnosis of aphthous ulcers of the gastrointestinal tract. The risks of angiography may be warranted in the presence of atypical and unresponsive inflammatory bowel disease, especially when it coexists with vascular disease elsewhere

Three separate episodes of free perforation of the duodenum and stomach are described in a 47-year-old white man with histological evidence of gastroduodenal Crohn's disease, co-existing with peptic disease. His mother died of Crohn's disease after ostomy surgery and two brothers required ileostomy for inflammatory bowel disease. In 1972, the patient underwent a bypass gastroenterostomy for obstructing Crohn's disease of the descending duodenum. In 1977, 1978, and 1982, three free perforations occurred first in the duodenum and then twice at the gastrojejunostomy site. This occurred while on continuous Cimetidine therapy as well as steroids and after vagotomy. The basal and maximum acid outputs, secretin stimulation test, and serum gastrins were normal throughout his clinical course. This unique situation of acid peptic perforation in the presence of active Crohn's disease, poses an etiological dilemma and signals the vulnerability of the diseased stomach and duodenum in transmural disease