[ENTEROLITHS CAUSED BY REGIONAL ENTERITIS].
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Two patients are presented with upper airway obstruction associated with regional enteritis (Crohn's disease). Two mechanisms for this obstruction are proposed. The first is involvement of the cricoarytenoid joint with the inflammatory process and the second is extensive edema of the upper airway due to submucosal involvement with the disease process. We were unable to find any previous reports of upper airway obstruction associated with regional enteritis. The data from these patients suggests that Crohn's disease should be included in the differential diagnosis of chronic persistent laryngeal edema.
The clinical experiences with a few patients diagnosed as regional enteritis by x-ray and histological examination and treated as such during a period of 15-40 years, have furnished some interesting data. It appears that in some instances, regional enteritis may gradually resolve completely or "burn" itself out, while in others it may be asymptomatic for years only to reappear suddenly with marked abdominal symptoms. An asymptomatic course can occur without steroid therapy. Initial surgery may be "curative" in some for many years; in others for only a short time.
The 35th and 36th cases of adenocarcinoma arising in small bowel affected with regional enteritis are presented. The pathology of all reported cases is reviewed. An association between regional enteritis and carcinoma of the small bowel is suggested.
Forty-three cases of regional enteritis of the duodenum were found in the world literature. Regional duodenitis is relatively uncommon; in one large series of 600 cases of regional enteritis only three involved the duodenum.At the University of Alberta Hospital, in a three-year period (1962 to 1965), the authors encountered five patients with regional duodenitis, demonstrating a spectrum of clinical, radiologic and pathologic characteristics of this disease. The description of these patients brings the world's total to 48 reported cases.Two of these patients had symptoms of severe duodenal obstruction and were relieved by bypass procedures and vagotomy; one required surgery because of co-existent obstructive ileal disease: and two patients have improved on corticoids and salicylazosulfapyridine without surgery.In our experience treatment with corticoids and salicylazosulfapyridine is beneficial.Four of the five patients remain in a state of mild to moderate nutritional impairment and have evidence of intestinal malabsorption. In the fifth case the period of followup is too short to permit assessment.
Four cases of carcinoma of the small bowel following chronic regional enteritis are presented. Search of the medical literature revealed 31 other cases reported up to the present. We must entertain the possibility that chronic regional enteritis may predispose to the development of cancer of the small bowel.
A biphasic malignant neoplasm, carcinosarcoma, arose in a segment of ileum involved with regional enteritis. The tumor spread throughout the abdomen, and the patient died nine months after diagnosis. The spectrum of intraepithelial dysplasia, carcinoma with varying degrees of differentiation, and sarcomatous-appearing elements suggests that the histogenesis of this tumor is epithelial. This report provides further documentation of the relationship between regional enteritis and unusual neoplasms of the intestine.
Although intra-abdominal abscesses are relatively frequent in the setting of regional enteritis, liver abscesses are rare. Pylephlebitis, steroid administration, and biliary fistulization have been suggested as inciting factors. This report describes the first attempted percutaneous drainage of a liver abscess which was a complication of regional enteritis. The percutaneous drainage proved inadequate therapy due to significant disease outside the liver involving the bowel. A review of all cases reported to date in the literature suggests that although percutaneous drainage might prove to be a satisfactory temporizing measure, all patients will require surgical exploration for definitive therapy.
We had a patient with primary acquired hypogammaglobulinemia (PAH), nodular lymphoid hyperplasia, giardiasis, and the subsequent development of regional enteritis (RE). The case is of interest because the development of regional enteritis in a patient with PAH has not been previously reported, to our knowledge. The interaction of hypogammaglobulinemia and RE is discussed from an immunologic point of view. The report adds RE to the many known bowel diseases associated with PAH.
Two patients with regional enteritis who developed renal failure due to amyloidosis are described. The literature is reviewed briefly and the hope expressed that this association will be recognized more frequently in the future.
A patient with regional enteritis and recurrent uric acid nephrolithiasis was treated with allopurinol. While on 600 mg of allopurinol daily, she began to pass many small, soft, yellow stones. Analysis of the stones by liquid chromatographic and gas chromatograph/mass spectrometric techniques revealed that their major constituent was oxypurinol, a metabolite of allopurinol. Metabolic studies of the patient indicated that increasing doses of allopurinol were associated with increases in xanthine and oxypurinol excretion, while uric acid excretion was not reduced. This case illustrates a complication of high-dose allopurinol therapy in the treatment of uric acid nephrolithiasis.
Thirty-two lambs with regional enteritis were examined. Eighteen were received alive and examined clinically, while the remaining were dead on arrival. One of the sick lambs recovered without treatment. Occurrence and epidemiology, clinical signs, clinical pathology, necropsy findings, light microscopy, immunoperoxidase studies, scanning electron microscopy and transmission electron microscopy are reported. The disease was characterised by stretching of the fore- and hindlimbs, ill thrift, intermittent diarrhoea, leucocytosis, thickened ileal wall and hyperplasia of the ileal mucosa. None of the methods applied revealed any causal agents. Possible aetiological factors are discussed.
The classical features of regional enteritis--a thickened edematous mesentery and edema of the submucosa--have been demonstrated by computed tomography. Future experience may confirm its value in separating inflammatory and neoplastic diseases of the small bowel.
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Lymphatic obstruction is constantly present in regional enteritis, but its role in the pathogenesis is poorly understood. To clarify this the mesenterial lymphatics of the terminal ileum were obstructed in 21 pigs chosen for experiments because spontaneous ileitis is common in pigs. Long-lasting obstruction was produced by injecting formaline into regional lymph nodes. The growth of the pigs was retarded. Their ileum became reddish and oedematous with numerous intra-abdominal adhesions, and mucosal ulcerations. Eleven pigs developed fistulae; none had chylous ascites. Histologically the subserosa was infiltrated by round and giant cells giving an impression of granulomatous inflammation. Many lymphatics were thrombosed. The lymphoid tissue was hyperplastic. These changes became more prominent with extensive subserosal fibrosis after three weeks, when the oedema diminshed. Transmural inflammation with round cell aggregations was then the most typical feature. Purulent infection with crypt abcesses appeared near the fistulae. If the function of lymphatics is destroyed, transport of particles too big to enter blood vessels is inhibited. This produces chronic irritation and oedema with declining tissue nutrition and oxygenation, thus causing fibrosis. Moreover, the intestinal lumen is a source of infections, and the oedematous tissue is easily infected. This may lead to mucosal ulcerations and, progressively, to fistulae.
Sera were collected from 108 patients with inflammatory bowel disease and assayed for carcinoembryonic antigen (CEA) and alpha(1)-fetoprotein (AFP). Seven (14%) of 51 patients with ulcerative colitis had a positive test for CEA and one of these had associated carcinoma of the colon. Ten (19%) of 52 patients with regional enteritis were also seropositive. The sera of 4 (9%) of 47 patients with ulcerative colitis and 2 (5%) of 41 patients with regional enteritis contained small amounts of AFP. Of two unclassified patients one had a positive CEA and the other a positive AFP. No serum was positive for both CEA and AFP. In addition, multiple samples were available for sequential analysis in eight CEA-positive patients but there was no apparent relationship between seropositivity and disease activity. Continued follow-up is now in progress to determine the significance of detectable fetal antigen levels in inflammatory bowel disease.
It has long been known that Crohn's disease may occur in association with certain forms of malignancy, specifically adenocarcinoma and lymphoma of the bowel and carcinoma of the vulva and perianal area. Here, the authors report the rare occurrence of extraintestinal Hodgkin's disease in a patient with documented regional enteritis of five years' duration. They propose explanations for this unusual combination of disease, reported in the literature only once previously, and review the pathophysiology of lymphoma and regional enteritis.