Search

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism and platelet dysfunction. A subset of patients also show ceroid deposition, which can result in pulmonary fibrosis or granulomatous colitis. Whether this colitis may be considered Crohn's disease is under debate. We report a case of a patient with HPS associated with inflammatory bowel disease which affected the distal small bowel but not the colon. Ileitis was severe, and recurred rapidly after surgery. Search for mutations in HPS1, ADTB3A, HPS3, HPS4 and for CARD15 were negative. Symptoms and ileal ulcerations which recurred after surgery were successfully treated with azathioprine and infliximab.

Aims: Jejunoileitis might be a severe form of Crohn's disease (CD). The aim of the study was to evaluate clinical characteristics, therapeutics modalities and long-term outcome in CD patients with jejunoileitis (CDJI)., Methods: All patients with CDJI followed in the department of Gastroenterology from 1963 to 1999 were included and compared to matched (on Year of CD diagnosis) CD controls without jejunoileitis. Data were obtained from retrospective review of medical charts., Results: Eighteen patients with CDJI were compared to 36 matched CD controls. Median follow-up was 7.65 Years in both groups. At time of CDJI diagnosis the following signs were significantly more frequent in patients with jejunoileal CD than in controls: malnutrition (39% vs 3%), pain suggesting obstruction (33% vs 8%), vomiting (28% vs 5%). Patients with CDJI were more frequently male: M/F ratio=2.0/1.1 (P=0.33). Upper digestive involvement (esophagus, stomach and duodenum) (67% vs 36%, P=0.04) and small intestine strictures (61% vs 19%, P=0.06) were more frequent in CDJI. Initial management was more "aggressive" in CDJI than in controls: steroids in 62% vs 30%, azathioprine in 39% vs 3%, total parenteral nutrition in 28% vs 8% and surgery in 33% vs 17%. During follow-up, the need for azathioprine therapy and surgery were more frequent in CDJI than in controls (extensive small bowel resection in two patients). In 10 of 18 patients, jejunoileitis involvement was diagnosed with a median delay of 3.6 Years (range: 0.5-14.5) after CD diagnosis and at time of CD diagnosis in the 8 others; outcome after CDJI diagnosis was similar in these 2 groups., Conclusion: The main revealing signs of jejunoileitis in CD patients are obstruction and malnutrition. Patients with CDJI require more often azathioprine and surgery than CD patients without jejunoileitis. Jejunoileitis is a severe form of CD more frequently complicated by extensive small bowel resection.

Campylobacter jejuni is an enteropathogen with invasive ability which may be responsible for acute colitis. We report the case of a 38-year-old woman who was hospitalized for severe acute colitis. Stool specimen examination revealed Campylobacter jejuni. A laparotomy was performed due to clinical deterioration and toxic megacolon. Colic perforations were observed. The examination of the colectomy specimen revealed ulcerative pancolitis. This is the first published case of Campylobacter jejuni infection complicated by toxic megacolon and perforation.

The authors report the case of a 51-year-old woman who was hospitalized for severe acute colitis. Sub-total colectomy was performed, due to clinical deterioration and resistance to treatment. The examination of the surgery specimen revealed a herpes-virus type 2 as the responsible pathogen agent. Complementary aciclovir treatment cured the patient.

Bacterial translocation is the passage of viable endogenous bacteria from the gastrointestinal tract to mesenteric lymph nodes and other internal organs. The aim of this work was to study bacterial translocation in patients operated on for Crohn's disease. Twenty-eight patients, mean age 29 years, not having received any antibiotics since at least 8 days, presenting with ileal (n = 12), ileo-colonic (n = 14) or colonic (n = 2) Crohn's disease were studied. In 25 out of 28 cases (89%) indication for surgery was strictures inducing an upper small bowel distension in 9 out of 25 patients. Mesenteric lymph nodes and liver biopsies, portal blood samples and peritoneum swabs were harvested after laparotomy and before gut opening. Bacterial translocation, defined as the presence of intestinal bacteria in at least one of the specimens, was present in 8 out of 28 patients. This was found in lymph nodes draining surgical territories in 7 out of 8 cases. Bacterial strains involved in translocation included E. coli (n = 5), Enterococcus (n = 3), Clostridium perfringens (n = 2), Proteus (n = 2), and Bacteroides fragilis (n = 1). The rate of translocation differed neither according to Crohn's disease site nor with perforating or non perforating type of the disease. Five out of 9 patients operated on for strictures with proximal distension had a translocation. In conclusion, bacterial translocation was identified in 29% of patients operated on for Crohn's disease in this series. Distension of the intestine proximal to a digestive stricture could favor the occurrence of bacterial translocation in Crohn's disease.

Psoas abscess were found in 6 cases among 166 patients with Crohn's disease between 1985 and 1989; in one case, it was the first sign of Crohn's disease. Diagnosis was usually difficult and should be suspected on the following signs: lower abdominal quadrant pain, psoitis, abdominal mass, sciatica or pain along the course of the femoral nerve. Diagnosis was confirmed in nearly all cases by computerized axial tomography. Effective therapy combines drainage and bowel resection.