[Lupus enteritis: an uncommon manifestation of systemic lupus erythematosus with favourable outcome on corticosteroids]. / L'entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique.

INTRODUCTION: Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis. CASE REPORTS: We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded. CONCLUSION: Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.

[Long term outcome following 26 surgical ampullectomies]. / Résultat à long terme de 26 ampullectomies chirurgicales.

BACKGROUND: Pancreaticoduodenectomy (PD) is the standard surgical treatment for malignant ampullomas but is still associated with a mortality and morbidity still ranging from 0 to 10% and from 15 to 40%, respectively. Ampullectomy is an alternative to PD for benign ampulloma or, in high-risk patients, for invasive carcinoma. The aim of this study was to report early and long term results of surgical ampullectomy for presumed benign ampullomas. PATIENTS AND METHODS: From 1981 to 2004, 26 patients from two institutions underwent surgical ampullectomy. Of the 26 patients, 8 had familial adenomatous polyposis (FAP). Surgical ampullectomy was indicated on a multisciplinary basis. RESULTS: Final pathological examination revealed 15 adenomas, 4 in situ adenocarcinomas, 2 endocrine tumors, and 5 other benign lesions. There was no postoperative mortality. Specific morbidity was 8% (N=2). Mean follow-up was 86+/-70 months (range: 3-204). Actuarial overall 5-year survival was 92%. There were 4 local recurrences (none in patients with FAP). Four patients died during follow-up (including 3 from initial disease). CONCLUSION: Ampullectomy is a good alternative to PD in case of benign or non-invasive malignant ampullary lesion, including in selected cases of FAP.

Gastrointestinal tract symptoms in Maternally Inherited Diabetes and Deafness (MIDD).

OBJECTIVE: To evaluate the prevalence and clinical consequences of gastro-intestinal manifestations in Maternally Inherited Diabetes and Deafness syndrome (MIDD). METHODS: We report the case of fatal intestinal pseudo-obstruction in a patient with severe MIDD. Using a standardized questionnaire, we evaluate the frequency of gastrointestinal tract (GIT) symptoms in 10 patients with MIDD (8 A3243G and 2 T14709C mutations of mitochondrial DNA). The reference population consisted of 50 patients with type 1 diabetes matched for disease duration. In 4 patients with digestive manifestations endoscopic examination of upper and lower GIT was performed allowing multiple biopsies for ultrastructural and molecular analysis. RESULTS: GIT symptoms were frequently reported in MIDD specially in patients bearing the mt 3243 mutation. The manifestations i.e. constipation, diarrhea or both, were more frequent in this subgroup than in type 1 diabetic population (88% vs 28%, p<0.05). Ileus is a rare and severe complication with a frequent fatal Issue. Ultrastructural analysis of the mucosa from oesophagus, stomach, duodenum, colon and rectum showed mild modifications such as accumulation of normal mitochondria and lipId droplets. Heteroplasmy levels were determined in 4 patients harboring the 3243 mutation. In three patients the percentage of mutated DNA increased from upper to lower GIT. CONCLUSIONS: Gastrointestinal symptoms are frequent in MIDD secondary to 3243 mutation. They might explain the lower body weight observed in these patients in comparison to reference diabetic populations. They can also lead to a severe complication namely the intestinal pseudo-obstruction.

Intraductal papillary or mucinous tumors (IPMT) of the pancreas: report of a case series and review of the literature.

OBJECTIVE: Despite a better understanding of these conditions, intraductal papillary or mucinous tumors (IPMT) of the pancreas still present difficulty relating to the predictive factors of malignancy and the risk of relapse after surgical resection. The aim of this study was to report on our experience and to compare it to previously published cases. METHODS: We studied retrospectively 26 patients (mean age 60.3 yr) presenting with IPMT. Of the 26 patients, 19 had surgical resection and seven did not. The main clinical feature was acute pancreatitis occurring in 38% of the patients. Segmental pancreatectomy was performed in all the cases. At pathological assessment of resection margins, tumor resection was considered as complete in 17 cases. Margins exhibited benign mucinous involvement, and resection was considered to be incomplete in one multifocal case and in one case with diffuse spread of the tumor. RESULTS: A total of 11 tumors were benign and five were malignant. Carcinomas were invasive in four cases (two invading the pancreatic parenchyma, one the duodenum, and one the peripancreatic nodes) and in situ in one case. Malignancy was not diagnosed preoperatively except when invasion was evident (duodenal spread). Although main pancreatic duct type and obstructive jaundice appeared as suggestive features for the risk of malignancy, no reliable preoperative predictive factors for malignancy could be identified as regarding to clinical parameters, biological examinations, carcinoembryonic antigen or CA19-9 levels in serum or in pure pancreatic juice, imaging, and cytological methods. Within 40.8 months mean follow-up after surgery (range 2-96 months), three patients (16%), two with malignant and one with benign tumor, had tumor relapse after respectively 7, 27, and 14 months. Margins were positive without malignant features in the two malignant cases and negative in the other case. Tumor relapse was malignant with diffuse spreading in the three cases, and the patients died within 34 months after surgical resection. CONCLUSIONS: Our series and the review of the literature indicate that preoperative indicators of malignancy in IPMT are still lacking. Concerning resection margins, complete tumor resection is usually possible by segmental pancreatectomy. Malignant relapses are not exceptional. Incomplete resection and diffuse or multifocal tumor represent poor prognostic factors. Total pancreatectomy should be considered in such cases.

Biliopancreatic fistulas complicating pancreatic pseudocysts: a report of three cases demonstrated by endoscopic retrograde cholangiopancreatography.

Three patients were found to have fistulation of the pancreatic and common bile ducts, complicating chronic pancreatitis in one patient and acute pancreatitis in two patients. Closure of the fistula was achieved with biliary and pancreatic stenting in one patient; the other two patients were treated surgically because endoscopic treatment had failed. The clinical and radiological features of this exceptional complication are presented, with a brief review of the topic.

[Pancreatic intubation ifn pancreas divisum]. / Intubation pancréatique dans le pancréas divisum.

AIM: Long-term results of endoscopic pancreatic stenting in pancreas divisum is still debated. The aim of this retrospective study was to evaluate the efficacy of dorsal duct stenting in patients presenting with acute recurrent pancreatitis. PATIENTS AND METHODS: Between 1980 and 1998, among 34 patients presenting with recurrent acute pancreatitis associated with pancreas divisum, 21 were treated by pancreatic stenting during a mean time of 11 months. There were 13 men and eight women (mean age: 50 years). RESULTS: The median follow-up was 50 (range 11-105) months. The number of patients presenting with acute pancreatitis before pancreatic stenting, at the end of stenting and at the end of the follow-up was respectively 21/21 (100%), 2/19 (10%) and 2/18 (11%) (P < 0.01). The number of patients presenting with chronic pain before stenting, at the end of stenting and at the end of the follow-up was respectively 17/21 (80%), 6/19 (31%) and 5/18 (27%) (P = 0.07). The overall morbidity rate was 8/21 patients (38%) including mainly acute pancreatitis (three cases); all but one complication were managed conservatively. CONCLUSION: In patients with pancreas divisum, dorsal duct stenting decreases the rate of recurrent acute pancreatitis but the improvement of chronic pain appears less obvious.

[Small bowel perforation and occlusion after migration of biliary and cystogastric prosthesis]. / Perforation et occlusion du grêle après migration de prothèse biliaire et de prothèse kystogastrique.

We report on two cases of intestinal complications associated with the migration of biliary and cystogastrostomy stents. In the first case, intestinal perforation occurred after migration of an Amsterdam type biliary stent. In the second case, a double pig tail endoprosthesis induced intestinal occlusion which was successfully managed without surgery. Complications after intestinal migration of biliopancreatic stents are very rare and usually involve the colon. Our observations point out the potential gravity of intestinal migration of biliopancreatic stents and, as in the second case, the possible resolution of stent related intestinal occlusion after medical management.

Dynamic anal endosonography may challenge defecography for assessing dynamic anorectal disorders: results of a prospective pilot study.

BACKGROUND AND STUDY AIMS: The diagnosis of anorectal dynamic disorders and perineal insufficiency has been mainly based up to now on the use of defecography. Here, we performed a prospective blinded study to evaluate the accuracy of a new procedure, dynamic anorectal endosonography (DAE), by comparing it with defecography. PATIENTS AND METHODS: A total of 43 women (mean age 53), presenting with outlet obstruction, were prospectively enrolled in our study. The DAE was performed with a 7.5-Mhz linear probe (Toshiba, Tokyo, Japan) with basal and straining recordings. None of the DAE or defecography operators was informed about the results of the respective other investigation. RESULTS: Defecography showed a descending perineum in 29 patients (68%), a rectocele in 25 patients (58%), and a rectal intussusception in eight patients (18%). The rate of concordance between the diagnosis of descending urinary bladder at DAE and descending perineum at defecography was 35/43 (80%), and that between the descending puborectal muscle at DAE and descending perineum at defecography was 40/43 (93 %). The rates of concordance between DAE and defecography for the diagnosis of rectocele and rectal intussusception were 27/43 patients (57%) and 34/43 patients (80%), respectively. In the last 15 patients, the DAE technique was adapted in order to improve the accuracy for diagnosing rectocele by filling the rectum with water. This improved sensitivity from 36 to 86% and accuracy from 57 to 87%. CONCLUSIONS: DAE is a new imaging approach to anorectal dynamic disorders, providing a highly reliable means of diagnosing perineal insufficiency as well as rectocele. DAE should be substituted for previous methods since it makes it possible at the same time to assess the anal sphincters and to avoid pelvic irradiation.

[Lymphocytic colitis and Gougerot-Sjögren syndrome. Report of two cases]. / Colite lymphocytaire et syndrome de Gougerot-Sjögren. A propos de deux cas.

INTRODUCTION: Microscopic colitis describes a subset of patients with chronic watery diarrhea of unknown origin, and normal endoscopic findings and microscopic evidence of an inflammatory infiltrate in the colonic mucosa. We report two cases associated with sicca syndrome. EXEGESIS: A 56-year-old woman and a 76-year-old man presented with a history of lymphocytic colitis associated with sicca syndrome. Drugs or infectious agents were not implicated in the cause of lymphocytic colitis, suggesting that sicca syndrome may be involved in the pathogenesis of microscopic colitis. CONCLUSION: These cases suggest that sicca syndrome should be detected in patients with lymphocytic colitis.

[Pancreatic intubation by endoscopy in chronic calcifying pancreatitis]. / Intubation pancréatique per endoscopique dans la pancréatite chronique calcifiante.

BACKGROUND: The aim of our study was to assess the efficacy of endoscopic pancreatic stenting on pain and frequency of acute pancreatitis in patients with calcifying chronic pancreatitis. PATIENTS: Between 1987 and 1996, 70 patients underwent endoscopic management for calcifying chronic pancreatitis. RESULTS: Endoscopic stent placement was successful in 59 patients (85%). 46 patients were followed for a mean duration of 29 +/- 26.4 months after stent removal. The presence of pancreatic pseudocysts in 26 patients appeared to modify pain improvement rate but did not influence the decrease in the frequency of acute pancreatitis. An improvement of pancreatic pain occurred at the end of stenting and after removal of the stent in respectively 44% and 38% of the patients without pseudocysts and in 85% (P < 0.02) and 65% of the patients presenting with pancreatic pseudocysts. Before stenting, 43/70 (61%) patients had at least two episodes of acute pancreatitis. After removal of the stent, only 5/46 (11%) had one episode of acute pancreatitis. Improvement of weight loss and diabetes was observed in respectively 28 cases (52%), 7 cases (39%) at the end of pancreatic stenting and was still persisting in 21 (51%) and 5 cases (26%) after removal of the stent. CONCLUSION: After pancreatic stenting, the improvement of pain appears to be moderate in the absence of pancreatic pseudocyst. Nevertheless, the improvement of pain is maintained after stent removal. Moreover, the frequency of acute pancreatitis is significantly decreased after pancreatic stenting.

[Natural history of non alcoholic and non familial chronic pancreatitis. Results of a multicentre study]. / Evolution de la pancréatite chronique non alcoolique et non familiale.Résultats d'une étude multicentrique.

UNLABELLED: The natural history and complications of non alcoholic chronic pancreatitis (NACP) is poorly understood compared to that of alcoholic chronic pancreatitis (ACP). PATIENTS AND METHODS: From April 1993 to April 1996, 77 patients with NACP were prospectively evaluated in 17 French centres. This population was compared to a cohort of 417 patients with ACP. RESULTS: No significant difference was observed with respect to mean age between NACP and ACP (43 +/- 20 vs 44 +/- 11 years, respectively). The median patient follow-up time was also comparable: 7 years (1-28) and 6 years (1-34) respectively for NACP and ACP. There were significantly more males in the ACP group (9/1 in ACP group and 1.3/1 in NACP group; P<10(- 7) ). Patients with NACP were less likely to have calcifications (58% vs 77%; P=0.01), pseudocysts (19 vs 47%, P<0.001), portal vein thrombosis (5 vs 16%, P<0.02). Importantly, patients with NACP required less surgical procedures than those with ACP (26% vs 44%, P=0.004). The actuarial death rate at 15 years was 0% in the NACP group compared to 20.5% in those with ACP (no CP related death). CONCLUSION: NACP has a less severe disease progression, fewer complications and requires less surgical interventions than ACP. The lower actuarial survival rate in patients with ACP correlates with the extra-pancreatic complications encountered in patients with alcohol related diseases and not with the evolution of CP itself.

Role of the NHE3 isoform of the Na+/H+ exchanger in sodium absorption by the rabbit gallbladder.

The absorption of water and electrolytes by the gallbladder seems to be largely dependent upon a Na+/H+ exchange at the apical membrane of the gallbladder epithelium. To find out if the exchanger involved is the NHE3 isoform, as in other absorbing epithelia, two studies were performed using the rabbit gallbladder. First, we studied 22Na absorption in Ussing chambers with Krebs buffer as a control solution, and in the presence of amiloride (100, 200 or 1000 microM), ethyl-isopropyl-amiloride (EIPA, 1 or 5 microM), or the phorbol ester, phorbol 12-myristate 13-acetate (PMA, 1 microM). A net mucosal-to-serosal Na+ flux was observed with control buffer. No inhibition of this net flux was observed with 5 microM EIPA, and the IC50 for amiloride was found to be 200 microM. PMA induced a reduction of absorption by 30% that was prevented by incubation with calphostin C. Resistance to amiloride and EIPA, and inhibition by PMA are consistent with the involvement of the NHE3 isoform. The second study involved reverse-transcriptase polymerase chain reaction (RT-PCR) of total gallbladder RNA, with two primers designed to amplify a 645-base-pair fragment from NHE3 mRNA. A cDNA fragment of the expected size was actually obtained from gallbladder RNA, while RT-PCR of RNA from the liver, which does not contain NHE3, gave negative results. A sequence of 492 nucleotides of the amplified product was determined, which was almost superimposable onto the known sequence of the corresponding fragment of rabbit NHE3. It is concluded that, in rabbit gallbladder, neutral NaCl absorption is, at least in part, dependent on the NHE3 isoform of the Na+/H+ exchanger.

[Hepatic granulomatosis associated with mononucleosis syndrome secondary to cytomegalovirus infection: apropos of 2 cases in healthy adults]. / Granulomatose hépatique associée à un syndrome mononucléosique secondaire à une infection à cytomégalovirus: à propos de deux cas chez l'adulte sain.

In this study, the authors report two observations of granulomatous hepatitis. The secondary appearance of a mononucleosis syndrome, three weeks after the onset of fever, in healthy adults, evoked the diagnosis of a cytomegalovirus infection. The authors insist on the histologic and virologic differences of the CMV infection between the healthy adults and the immunodepressed patients. They also note the difficulties of the diagnosis of the CMV infection in healthy adults.