Parsonage-Turner syndrome due to autochthonous acute genotype 3f hepatitis E virus infection in a nonimmunocompromised 55-year-old patient.

Hepatitis E virus (HEV) infection is an emerging autochthonous disease in industrialized countries. Extra-hepatic manifestations, in particular neurologic manifestations, have been reported in HEV infection. Only a few cases of hepatitis E-associated Parsonage-Turner syndrome have been reported, and HEV genotypes were rarely determined. Here, we report the case of a Parsonage-Turner syndrome associated with an acute autochthonous HEV infection in a 55-year-old immunocompetent patient. HEV genomic RNA was detected in serum and cerebrospinal fluid samples (CSF), and molecular phylogenetic analysis of HEV was performed. The interest of this case lies in its detailed description notably the molecular analysis of HEV RNA isolated from serum and CSF. HEV infection should be considered in diagnostic investigations of neurologic manifestations associated with liver function perturbations.

Update on Anti-Saccharomyces cerevisiae antibodies, anti-nuclear associated anti-neutrophil antibodies and antibodies to exocrine pancreas detected by indirect immunofluorescence as biomarkers in chronic inflammatory bowel diseases: results of a multicenter study.

AIM: Anti-Saccharomyces cerevisiae antibodies (ASCA), anti-nuclear associated anti-neutrophil antibodies (NANA) and antibodies to exocrine pancreas (PAB), are serological tools for discriminating Crohn's disease (CrD) and ulcerative colitis (UC). Like CrD, coeliac disease (CoD) is an inflammatory bowel disease (IBD) associated with (auto) antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS: Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence (IIF). RESULTS: ASCA+/NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD. PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION: ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.

Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia.

We study two authentic cases of protein-losing enteropathy, the diagnosis of which was facilitated using Given M2A videocapsule endoscopy. The first case corresponded to a primary intestinal lymphangiectasia confirmed by jejunum biopsies and the second one to a protein-losing enteropathy with lymphatic abnormalities secondary to a chronic constrictive pericarditis. In the first case, the mucosa of jejunum presented with a diffuse oedematous aspect, whitish villi, white curved lines probably related to submucosal dilated lymphatics and lacteal juice. In the second case, capsule endoscopy showed oedematous aspect of jejunum mucosa associated with white curved lines similar to those observed in the first case. Videocapsule endoscopy is useful in cases of protein-losing enteropathy to identify presence of intestinal lymphangiectasia and to specify their localisation after ruling out other disorders liable to induce protein-losing gastrointestinal syndrome.

[Diagnostic value of high resolution sonography in Crohn's disease and ulcerative colitis]. / Valeur diagnostique de l'échographie de haute fréquence au cours de la maladie de Crohn et de la rectocolite hémorragique.

OBJECTIVE: To evaluate the diagnostic accuracy of high resolution sonography in patients with inflammatory bowel disease (MICI). PATIENTS AND METHODS: In patients with Crohn's disease (n = 48), ulcerative colitis (n = 23), indeterminate colitis (n = 3), inflammatory (n = 21) and non-inflammatory (n = 23) controls, high resolution sonography was performed and compared to colonoscopy (+/- retrograde ileoscopy) and/or baryum studies of the small bowel and the colon. RESULTS: Diagnosis of intestinal inflammation or not was correct in 69/74 MICI patients (sensitivity: 94.4%, specificity: 66.7%, global accuracy: 93.2%). Segment location was accurate in 58/74 (sensitivity: 80.3%, specificity: 66.7%, global accuracy: 79.7), more frequently in Crohn's disease, than in ulcerative colitis. Five out of six complications of Crohn's disease were diagnosed. In Crohn's disease, the method was more accurate in case of colonic or ileocolonic involvement. CONCLUSION: High resolution sonography is a reliable diagnostic tool for the detection of intestinal inflammation and related complications in MICI. In can be of value in the follow-up and seems particularly interesting in the case of temporary contraindication of invasive methods.

Frequent factor II G20210A mutation in idiopathic portal vein thrombosis.

BACKGROUND & AIMS: Despite extensive investigations of portal vein thrombosis, no underlying cause is identifiable in up to 30% of patients. A recently described mutation of the prothrombin gene at nucleotide position 20210 is associated with history of venous thrombosis and was assessed in this study. METHODS: We compared the frequency of factor II G20210A and factor V G1691A (factor V Leiden) mutations in 10 patients with idiopathic portal vein thrombosis, 10 patients with nonidiopathic portal vein thrombosis, 60 patients with deep vein thrombosis of the legs, and 42 control subjects. RESULTS: The frequency of factor II G20210A mutation was increased in patients with idiopathic portal vein thrombosis (40.0%; confidence interval, 3.1%-76.9%) compared with controls (4.8%; confidence interval, 0%-11.5%) or patients with nonidiopathic portal vein thrombosis or deep vein thrombosis (P = 0.0001). In contrast, the frequency of the factor V G1691A mutation was similar in subjects with portal vein thrombosis and in controls but was increased in patients with deep vein thrombosis (P = 0.0001). CONCLUSIONS: The factor II G20210A mutation is frequent in patients with idiopathic portal vein thrombosis and should therefore be assessed under this circumstance.

Significance of diminished factor XIII in Crohn's disease.

OBJECTIVE: Coagulation factor XIII is a plasma transglutaminase involved in crosslinking of fibrin, the last step of the coagulation system and a connective tissue factor contributing to the wound healing process. It circulates as a heterotetrameric molecule consisting of two identical proenzyme subunits (factor XIIIA) and two carrier protein subunits (factor XIIIS). The aim of this study was to determine the disease features associated with the diminution of factor XIII in Crohn's disease. METHODS: Factor XIIIA and factor XIIIS levels were assessed in patients presenting with Crohn's disease, ulcerative colitis, infectious colitis, or diverticulitis, in patients with rheumatoid arthritis, and in control subjects. Prothrombin fragment 1 + 2 assay, as a marker of the generation of thrombin and measurement of C-terminal telopeptide of type I collagen as an estimate of degradation of collagen type I, were performed. RESULTS: Factor XIIIA was significantly decreased in Crohn's disease, in ulcerative colitis, and in infectious colitis by comparison with subjects presenting with diverticulitis, normal, and rheumatoid subjects p = 0.0001). Factor XIIIS was unmodified in patients with Crohn's disease by comparison with controls but was reduced in those presenting with intestinal bleeding (p = 0.0002). In Crohn's disease, the lowest level of factor XIIIA was observed in patients with intestinal bleeding (p = 0.0003). Factor XIIIA was correlated with the Van Hees index (r = -0.5661; p = 0.0001) and with the C-terminal telopeptide of type I collagen (r = -0.4110; p = 0.0011) but not with prothrombin fragment 1 + 2. The multiple regression analysis showed that only Van Hees index and intestinal bleeding were independent variables for explaining the diminution of Factor XIIIA in Crohn's disease. CONCLUSIONS: Factor XIIIA subunit is an indicator of Crohn's disease activity. Our study suggests that a low factor XIIIA level is related to the presence of intestinal lesions and might be linked to intestinal repair mechanisms; loss in intestinal lumen could be also involved, especially in patients with intestinal bleeding.

Antral and fundic D-cell numbers in Helicobacter pylori infection.

OBJECTIVES: Helicobacter pylori infection is associated with an exaggeration of gastrin release following meals or bombesin stimulation attributed to a defect of somatostatin secretion of antral D-cells. Nevertheless, these modifications of gastric physiology do not explain the increase of gastric acid secretion which is only observed in duodenal ulcer patients. The inhibitory effect of somatostatin secretion of fundic D-cells on parietal cells is well known. The aim of our prospective study was to compare the number of fundic D-cells and likewise the number of antral G-cells and D-cells between patients with duodenal ulcer and healthy subjects with and without H. pylori infection. METHODS: The numbers of D-cells and G-cells were compared between 19 infected patients with duodenal ulcer and 20 healthy subjects, 10 with and 10 without H. pylori infection. Fundic mucosal biopsy specimens were examined using immunohistochemical techniques specific for the presence of somatostatin, antral mucosal biopsy specimens for the presence of gastrin and somatostatin. RESULTS: The number of G-cells was significantly lower (P = 0.0012) in duodenal ulcer patients by comparison with infected subjects and controls. The number of antral D-cells was significantly less (P < 0.0001) in duodenal ulcer patients (mean of 10 random fields = 0.45 +/- 0.04) than in either asymptomatic infected patients (0.65 +/- 0.07) or uninfected controls (0.88 +/- 0.10). The number of fundic D-cells was significantly lower (P < 0.0001) in duodenal ulcer patients (mean = 0.20 +/- 0.03) than in either asymptomatic infected subjects (0.29 +/- 0.05) or controls (0.73 +/- 0.09); here the difference between the two groups of infected subjects was not significant. Multivariate analysis showed that the presence of H. pylori infection of the fundic mucosa did not influence the number of fundic D-cells. CONCLUSION: Changes in the number of fundic and antral D-cells induced by H. pylori infection did not explain abnormalities of gastric acid secretion usually observed in duodenal ulcer patients; it is suggested that pre-existing abnormalities in the regulation of parietal cell or increase of parietal cell mass are involved.

[Isolation of Helicobacter pylori: necessity of control of transport conditions]. / Isolement d'Helicobacter pylori: nécessité du contrôle des conditions de transport.

The aim of the study was the influence of transport conditions of antrum biopsies on the diagnostis of Helicobacter pylori infection by mean of bacteriological methods. We compare bacteriological to anatomopathological methods of Helicobacter pylori infection during two periods which differed by the transport conditions for antrum biopsies. During the first period (P1), 57 specimens were carried to the laboratory at room temperature and physiological serum immersion was inconstant. During the second period (P2), 56 specimens were all correctly immerged into 5 ml of physiological serum, in a 15 mm diameter tube which was vertically maintained in ice. Anatomopathological methods were considered as the reference method for the diagnostic of Helicobacter pylori infection. The sensibility of bacteriological culture was increased from 59% (P1) to 84% (P2) (p < 0.05) when specificity was unchanged. Furthermore sensibility of bacteriological direct examination was increased from 41% (P1) to 61% (P2). Complete biopsy immersion and ice transport of samples appear as necessary conditions for the optimal diagnostic of Helicobacter infections by mean of bacteriological methods.

Prothrombin fragment 1 + 2 and thrombin-antithrombin III complex as markers of activation of blood coagulation in inflammatory bowel diseases.

OBJECTIVES AND METHODS: The aims of the present work were to assess the presence of thrombin generation in Crohn's disease and in ulcerative colitis by using the prothrombin fragment 1 + 2 and the thrombin-antithrombin III complex assays and to study the possible relationships between these markers and disease activity. RESULTS: Prothrombin fragment 1 + 2 and thrombin-antithrombin III complex were significantly raised in patients with Crohn's disease (n = 69) and with ulcerative colitis (n = 25) as compared with healthy controls (n = 50). In Crohn's disease these two markers of thrombin generation were correlated with the Van Hees index (P < 0.05 and P < 0.001, respectively); values were significantly different from controls even in the patient group displaying the lowest disease activity (P < 0.001). No correlation was found with tumour necrosis factor alpha and C-reactive protein; nevertheless patients with C-reactive protein less than or equal to 10 mg/l had significant lower values of prothrombin fragment 1 + 2 (P < 0.03). In ulcerative colitis prothrombin fragment 1 + 2 and thrombin-antithrombin III complex were significantly increased by comparison with controls, were higher in patients with pancolitis and correlated with C-reactive protein (P < 0.002 and P < 0.009, respectively). CONCLUSION: These data show that prothrombin fragment 1 + 2 and thrombin-antithrombin III complex are increased in inflammatory bowel diseases and suggest that thrombin generation might be an early event in their pathogenesis.

Delta-opioid receptor agonists inhibit neuromuscular transmission in human colon.

The present study was undertaken to investigate the possible role of delta-opioid receptors in the neuroregulation of human colonic motility by using a superfusion model. Spontaneous mechanical activity and responses to electrical transmural nerve stimulation of both longitudinal and circular muscle strips from the human sigmoid colon were studied. Exogenously added delta-opioid receptor agonists did not modify spontaneous contractile activities of either type of strip. Nerve stimulation induced a triphasic response composed of an initial contraction followed by a relaxation and an off-contraction. This response was mediated by cholinergic excitatory nerves and non-adrenergic, non-cholinergic excitatory and inhibitory nerves. [Met5]Enkephalin and the synthetic delta-opioid receptor agonist [D-Pen2,D-Pen5]enkephalin (DPDPE) significantly decreased the amplitude of the initial contraction and of the off-contraction. The effects of both delta-opioid receptor agonists were reduced in the presence of either the delta-opioid receptor antagonist, ICI 174864, or another delta-opioid receptor antagonist, naltrindole. ICI 174864 prevented neither the effects of a natural kappa-opioid receptor agonist, dynorphin-(1-13) nor those of the mu-opioid receptor agonist, PL017. Therefore, these data suggest that delta-opioid receptors might be involved in the neuroregulation of smooth muscle of human colon and may mediate inhibition of cholinergic and non-cholinergic excitatory transmission within the myenteric plexus.

Prevalence and significance of anticardiolipin antibodies in Crohn's disease.

Crohn's disease is a chronic inflammatory bowel syndrome in which thrombotic complications occur in the active phase. Phospholipid-binding antibodies such as anticardiolipin antibodies and lupus anticoagulants have been shown to be associated with thrombosis. Their presence has been assessed in a group of 50 patients with Crohn's disease among whom 44 had active disease. The overall prevalence of anticardiolipin antibodies was about 22%, while none of these patients had lupus anticoagulant. Anticardiolipin antibodies have been observed in both active and quiescent CD and their presence does not seem to be related to the site of CD lesions. The presence of phospholipid-binding antibodies could be a sign of vascular alterations that are potentially thrombogenic per se, and their predictive value with respect to the specific inflammatory syndrome of Crohn's disease is discussed.

[Treatment of lithiasis of the common bile duct by endoscopic sphincterotomy and laparoscopic cholecystectomy]. / Le traitement de la lithiase de la voie biliaire principale par sphinctérotomie endoscopique et cholécystectomie laparoscopique.

This paper evaluates the treatment of common bile duct stones by endoscopic sphincterotomy (SE) and laparoscopic cholecystectomy (CL). 733 patients presenting with symptomatic cholelithiasis were operated on between March 1990 April 1993; 131 (18%) of them had a preoperative suspicion of common bile duct stones (LVBP): jaundice for 41, biliary acute pancreatitis for 27 and altered liver function tests for 63. 131 retrograde cholangiographies (CPRE) were attempted with an associated SE (113 cases) in the presence of LVBP, biliary pancreatitis, enlargement of common bile duct and appearance of forced papilla. CL was performed 24 to 48 hours later. CPRE +/- SE had no mortality; 1 patient presented a retroduodenal perforation of CBD, requiring surgery. 58 cases (44.2%) of LVBP were diagnosed, without a statistically significant difference according to the clinical pattern. In the group with altered liver function tests only alkaline phosphatase was significantly predictive of LVBP. There was no mortality or morbidity related to CL; conversion rate was 9.8%; 4 of 12 cases of conversion were related to persistence of stones in the common bile duct, without any possibility of laparoscopic extraction. Mean hospital stay was 7.4 days. Efficacy of this sequential method of treatment of LVBP was 91.3%: this method seems satisfactory, not dangerous and minimally invasive, and should be indicated for pre-operative suspected common bile duct stones.

Regulatory role of enteric kappa opioid receptors in human colonic motility.

The effects of different kappa opioid agonists and antagonists on spontaneous mechanical activities and responses to electrical transmural nerve stimulation of both longitudinal and circular muscle strips from the human sigmoid colon were studied. A superfusion apparatus was used to record isometric contractions. Exogenously added kappa agonists did not modify spontaneous contractile activities on either type of strip. Nerve stimulation induced a triphasic response composed of a first contraction (C1) followed by a relaxation (C2) and an off-contraction (C3); this response was mediated by cholinergic excitatory nerves and non-adrenergic, non-cholinergic excitatory and inhibitory nerves. Dynorphin 1-13 and the synthetic kappa agonist trans-3,4-dichloro-N-methyl-N-(2- [1pyrolidinyl]-cyclohexyl) dramatically decreased the amplitude of the excitatory components C1 and C3. The effects of both kappa agonists were blocked in presence of the kappa antagonist Nor-Binaltorphimine. The delta antagonist ICI 174864 did not prevent the inhibition of the contractions C1 and C3 induced by dynorphin. Therefore, these data suggest that kappa receptors are involved in the neuroregulation of smooth muscle of human colon and mediate inhibition of cholinergic and non-cholinergic excitatory transmission within myenteric plexus.

Intestinal carcinoid tumor and myotonic dystrophy. A new association?

The occurrence of multiple carcinoid tumors of the small bowel with liver metastases is reported in a patient with a previous myotonic dystrophy. In addition to the association of myotonic dystrophy with multiple endocrine adenomatosis type 2A, hyperparathyroidism, and neurofibromatosis, this case report gives further evidence for the propensity of such patients to develop neuroendocrine tumors.

[Non-surgical treatment of cysts and pseudocysts of the pancreas. A study of a series of 33 cases]. / Traitement non chirurgical des kystes et pseudo-kystes du pancréas. Etude d'une série de 33 cas.

Pancreatic cysts and pseudocysts had to be treated by surgery until 15 years ago. Nowadays they can be aspirated (or drained) either endoscopically or by ultrasonic and computed tomographic guided punctures. The aim of this study was to see if these non-surgical treatments were efficient enough among the actual treatments. From 1984 to 1988, 33 patients were admitted in one single institution with a pancreatic cyst or pseudocyst, 22 of which were a consequence on an acute pancreatitis and 11 complicated a chronic pancreatitis. Ten cysts were connected with the Wirsung channel while 11 others were not, but the ERCP failed to give any accurate information on this point in 12 cases. As a first treatment, we abstained of any invasive procedure in 9 cases, 18 were treated by percutaneous aspiration guided by ultrasound and 6 patients underwent surgery. The mean follow-up was 30 months. All the patients who had no treatment remained symptom free; their cysts improved in 5 cases and disappeared in 4 cases. Among the cysts treated by percutaneous aspiration, 4 had to be operated, 5 disappeared, 3 improved and 6 recurred; the percutaneous aspiration obtained a lasting symptoms' relief for these patients, whatever the connection the cyst had with the Wirsung channel. This study suggests that percutaneous aspiration or drainage guided by ultrasound may be a treatment of the highest quality for acute and chronic pancreatitis cystic formations. However, it seems to act upon the symptoms rather than upon the cystic formations themselves.