[Non-specific chronic ulcerous jejuno-ileitis. Presentation of a new case].
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Biomedical subjects
Publications and source records attributed to J Belaiche.
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UNLABELLED: The high frequency of familial Crohn's disease (CD) suggests a genetic predisposition. The most recent data from epidemiology and molecular biology are consistent with a multifactorial, polygenic inheritance with a possible genetic heterogeneity. The aim of our study was, first to compare familial and sporadic CD on the basis of the type and location of the disease, and age at diagnosis, and second, to evaluate among families, the concordance rate for the type and location of the disease. PATIENTS AND METHODS: 18 families with 2 (n = 16) and 3 (n = 2) affected first degree relatives were studied. They were compared to a population of 154 sporadic CD coming from the same gastroenterology unit. RESULTS: Age at diagnosis was the same in sporadic and familial CD. There was an increased frequency of ileal (p = 0.02), and fibro stenotic (p = 0.005) CD and a decreased frequency of colonic (p = 0.006) and inflammatory (p = 0.02) disease, in familial CD. There was a significant increase in concordance rate for fibrostenotic disease (p < 0.001) and a decrease for inflammatory disease (p < 0.01), among the families. The observed concordance rate for the location of the disease was not significantly different from the expected one. In conclusion, these data suggest that CD may be heterogenous and that different clinical patterns may be determined either by genetic or environmental factors.
Cyclosporin (CsA) has been proposed in the management of patients with acute ulcerative colitis (UC) in whom standard therapy failed and who were candidates for colectomy. Seven academic hospitals contributed to this retrospective study that included 29 patients (median age: 33 y. (15-74 y.); 12 females and 17 males). The median duration of the disease was 4 y. (0.3 to 33 y.). Before initiating CsA, patients were unresponsive to treatment including i.v. corticosteroids (n = 29), 5-ASA or salazopyrine (n = 19), azathioprine (n = 3), antibiotics (n = 14). The i.v. mean dose was 4 mg/kg/day and was adapted to blood level. Concomitant treatment included corticosteroids (n = 27). The median duration of i.v. CsA administration was 10 days (4 to 41 days). At the end of CsA administration, a global improvement was described in 20 patients while a surgery had to be performed immediately in 8 patients because of exacerbation of symptoms (n = 7) or perforation (n = 1). One other patient (74 y.) died because of Pneumocystis carinii infection. For the responders, maintenance therapy included: tapering dose of steroids (n = 12), azathioprine (n = 12), 5-ASA or salazopyrine (n = 10), methotrexate (n = 1) or oral CsA (n = II). The median duration of follow-up was 12 months (4 to 48 months). Among the 20 responders, 7 were subsequently referred for colectomy either electively (n = 3) or because of recurrence of the disease (n = 4). Among the 12 patients treated by azathioprine as a maintenance therapy, only 3 had to be referred for surgery (25%). Among the 8 patients who did not receive azathioprine, 4 were subsequently referred for a colectomy (50%) (NS). In patients with acute refractory UC who received CsA, the short-term efficacy (avoidance of immediate colectomy) was obtained in 20 out of 29 patients (69%). However, after a median follow-up of 12 months, only 13 patients were colectomy free (45%).
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Despite recent improvements in the management of Crohn's disease, steroids are still the most efficacious treatment in flare ups of the disease. However they have significant side effects and are only effective in the short term. There is no consensus regarding initial dose or duration of corticotherapy. With 1 mg/Kg a day of oral prednisolone given for 3 to 7 weeks, 92% of the patients achieve clinical remission. Topical acting oral corticosteroids such as budesonide seem to represent an important therapeutic advance due to their better tolerance. The promising results of budesonide in mild and moderate flare ups need to be confirmed and its indication in severe disease beside high dose prednisolone has to be clarified.
UNLABELLED: A first prospective epidemiological study of IBD was conducted in the area of Liège. The duration of the study was short taking into account the small size of the population (1 million inhabitants). Therefore we carried out a 3 years prospective study. METHOD: Private and public gastroenterologists completed a questionnaire for each new case they diagnosed between 01.06.1993 and 31.05.1996. RESULTS: During that period 270 IBD patients were identified: 137 (51%) had Crohn's disease (CD), 111 (41%) had ulcerative colitis (UC) including 32 proctitis (29% of UC) and 22 (8%) had unclassified colitis. The mean annual incidence per 10(5) was 4.5 for CD and 3.6 for UC. The female/male ratio was 1,6 for CD and 0,5 for UC. The median age at the time of diagnosis was 30 years for CD and 39 years for UC. The mean time between the onset of symptoms and the diagnosis was 6,5 months for CD and only 4,8 months for UC. Family history of IBD was found in 15% of patients with CD and in 7% of UC. CONCLUSIONS: These data show a high incidence of IBD in the area of Liege. These results confirm those reported during the first year of the prospective study which were similar to those observed in North-western France. Contrary to the other countries of Northern Europe, the incidence of UC is lower than the one of CD. Belgium appears to be a privileged country to undertake a national register and to study epidemiological aspects of IBD.