Relapse rate and predictors of relapse after cessation of glucocorticoid maintenance therapy in type 1 autoimmune pancreatitis: a multicenter retrospective study.

BACKGROUND: Type 1 autoimmune pancreatitis responds well to glucocorticoid therapy with a high remission rate. Moreover, glucocorticoid maintenance therapy can help prevent relapse. However, the relapse rate following cessation of long-term glucocorticoid therapy is unknown. The aim of this study was to clarify the relapse rate and predictors of relapse following long-term glucocorticoid therapy cessation. METHODS: We analyzed 94 patients who achieved remission after undergoing glucocorticoid therapy, discontinued treatment after at least 6 months of maintenance therapy, and were subsequently followed up for at least 6 months. The patients were divided into three groups based on treatment duration (< 18, 18-36, and ≥ 36 months), and their relapse rates were compared. Univariate and multivariate analyses of clinical factors were conducted to identify relapse predictors. RESULTS: After discontinuing glucocorticoid therapy, relapse was observed in 43 (45.7%) patients, with cumulative relapse rates of 28.2% at 1 year, 42.1% at 3 years, 47.0% at 5 years, and a plateau of 77.6% at 9 years. Of the 43 patients who relapsed, 25 (58.1%) relapsed within 1 year after after cessation of glucocorticoid therapy. Relapse and cumulative relapse rates did not differ significantly according to treatment duration. In the multivariate analysis, an elevated serum IgG4 level at the time of glucocorticoid cessation was found to be an independent predictor of relapse (hazard ratio, 4.511; p < 0.001). CONCLUSIONS: A high relapse rate occurred after cessation of glucocorticoid maintenance therapy, regardless of the duration of maintenance therapy, especially within the first year after cessation. However, the normalization of long-term serum IgG4 levels may be a factor in considering cessation.

Endoscopic placement of covered versus uncovered self-expandable metal stents for palliation of malignant gastric outlet obstruction.

OBJECTIVE: Stenting is an established endoscopic therapy for malignant gastric outlet obstruction (mGOO). The choice of stent (covered vs uncovered) has been examined in prior randomised studies without clear results. DESIGN: In a multicentre randomised prospective study, we compared covered (CSEMS) with uncovered self-expandable metal stents (UCSEMS) in patients with mGOO; main outcomes were stent dysfunction and patient survival, with subgroup analyses of patients with extrinsic and intrinsic tumours. RESULTS: Overall survival was poor with no difference between groups (probability at 3 months 49.7% for covered vs 48.4% for uncovered stents; log-rank for overall survival p=0.26). Within that setting of short survival, the proportion of stent dysfunction was significantly higher for uncovered stents (35.2% vs 23.4%, p=0.01) with significantly shorter time to stent dysfunction. This was mainly relevant for patients with extrinsic tumours (stent dysfunction rates for uncovered stents 35.6% vs 17.5%, p<0.01). Subgrouping was also relevant with respect to tumour ingrowth (lower with covered stents for intrinsic tumours; 1.6% vs 27.7%, p<0.01) and stent migration (higher with covered stents for extrinsic tumours: 15.3% vs 2.5%, p<0.01). CONCLUSIONS: Due to poor patient survival, minor differences between covered and uncovered stents may be less relevant even if statistically significant; however, subgroup analysis would suggest to use covered stents for intrinsic and uncovered stents for extrinsic malignancies.

Impact of sclerosing dacryoadenitis/sialadenitis on relapse during steroid therapy in patients with type 1 autoimmune pancreatitis.

OBJECTIVES: Steroids are the first-line drugs for induction of remission in patients with type 1 autoimmune pancreatitis (AIP), and the usefulness of steroid maintenance therapy to prevent relapse has recently been reported. However, even during steroid therapy, a relatively large percentage of patients relapse and the predictive factors for relapse have not yet been elucidated. The aim of this study was to clarify the predictive factors for relapse of AIP patients during steroid therapy. MATERIALS AND METHODS: The medical records of 76 patients with type 1 AIP with continued steroid therapy after induction of remission were analyzed retrospectively. The relapse rate during steroid therapy was evaluated, and the risk factors for relapse were investigated by univariate and multivariate analysis of clinical factors. RESULTS: Relapse occurred in 28.9% (22/76) of the patients. The cumulative relapse rates were 10.5% at 1 year, 25.0% at 3 years, 34.9% at 5 years, and 43.0% at 10 years. In multivariate analysis, presence of sclerosing dacryoadenitis/sialadenitis at the time of initial diagnosis of AIP was an independent risk factor for relapse (HR 3.475, p = .009). The cumulative relapse rates of patients with sclerosing dacryoadenitis/sialadenitis reached 21.4% at 1 year, 56.0% at 3 years, and 78.0% at 5 years. CONCLUSIONS: Sclerosing dacryoadenitis/sialadenitis was a predictive factor for relapse in type 1 AIP during steroid therapy; in such cases, strict follow-up is necessary with relapse in mind.

Central extracorporeal membrane oxygenation requiring pulmonary arterial venting after near-drowning.

Extracorporeal membrane oxygenation (ECMO) is an effective respiratory and circulatory support in patients in refractory cardiogenic shock or cardiac arrest. Peripheral ECMO sometimes requires left heart drainage; however, few reports state that pulmonary arterial (PA) venting is required during ECMO support. We present a case of a 14-year-old boy who required PA venting during ECMO support after resuscitation from near-drowning in freshwater. A biventricular assist device with an oxygenator implantation was intended on day 1; however, we were unable to proceed because of increasing of pulmonary vascular resistance from the acute lung injury. Central ECMO with PA venting was then performed. On day 13, central ECMO was converted to biventricular assist device with an oxygenator, which was removed on day 16. This case suggests that PA venting during ECMO support may be necessary in some cases of respiratory and circulatory failure with high pulmonary vascular resistance after near-drowning.

The effect of respiratory-gated positron emission tomography/computed tomography in patients with pancreatic cancer.

Our aim of this study was to evaluate prospectively in patients with pancreatic cancer the effects of respiratory- gating during PET/CT acquisition on the determination of lesion volume and to measure tracer uptake. Our research included 36 patients diagnosed with pancreatic cancer. They underwent conventional whole-body PET/CT and subsequently respiratory-gated PET/CT of the upper abdomen. Based on list-mode PET acquisition data, respiratory-gated and non-gated images were created. Maximum standardized uptake values (SUVmax) and lesion volumes were compared between gated and non-gated images and also the rate of increase in SUVmax based on lesion size. Results showed that respiratory gating was successful in 34/36 patients. The median non-gated SUVmax was 6.2±2.1 and was 8.1±2.5 for respiratory- gated (P<0.01). Lesion volumes could be calculated in 27/34 patients. The median non-gated lesion volume was 5.82±5.57cm(3) and 4.31±4.56cm(3) for respiratory-gated (P<0.01). Furthermore, small lesions of ≤2cm had a significantly higher proportion of increased SUVmax compared to large lesions of >2cm (P=0.016). In conclusion, respiratory-gated PET/CT for patients with pancreatic cancer reduced respiratory motion artifacts and allowed significantly higher SUVmax to be obtained. In addition, the rate of increase in SUVmax tended to be higher in patients with pancreatic cancers of less or equal to 2cm diameter.

Observation of Atrial Fibrillation Dependent on an Intra-atrial Re-entrant Tachycardia Within the Right Atrium in a Repaired Tetralogy of Fallot.

Atrial fibrillation (AF) can occur predominantly associated with right atrial (RA) lesions in congenital heart disease, particularly when the RA cavity is dilated. RA electrical potentials occasionally appear organized during AF. We clearly mapped such areas circumscribed by an intra-atrial re-entrant circuit during an isoproterenol infusion, in a patient with a repaired tetralogy of Fallot, using an ultrahigh-density mapping system and its beat acceptance criteria function. Ablation of areas inside the re-entrant circuit successfully eliminated the AF. Our experience indicated that a macro-re-entrant tachycardia was a driver as well as a trigger of AF of this right-sided origin.

Chez les patients atteints d'une cardiopathie congénitale, la fibrillation auriculaire (FA) peut souvent survenir en association avec des lésions auriculaires droites (AD), en particulier lorsque la cavité AD est dilatée. Lors d'une FA, il peut arriver que les potentiels électriques AD semblent normaux. Chez un patient ayant une tétralogie de Fallot réparée, nous avons clairement cartographié des zones délimitées par un circuit de réentrée intra-auriculaire lors d'une perfusion d'isoprotérénol, et ce, à l'aide d'un système de cartographie à très haute densité et de ses critères d'acceptation liés aux battements cardiaques. L'ablation des régions se trouvant dans le circuit de réentrée a permis d'éliminer la FA avec succès. Notre expérience a démontré qu'une tachycardie macroréentrante avait été un facteur déterminant et même un déclencheur de la FA, laquelle est apparue à droite.

Diagnostic Ability and Safety of Repeated Pancreatic Juice Cytology Using an Endoscopic Nasopancreatic Drainage Catheter for Pancreatic Ductal Adenocarcinoma: A Multicenter Prospective Study.

Pathological examination is essential for the diagnosis and treatment of pancreatic ductal adenocarcinoma (PDAC). Moreover, a reliable pathological diagnosis is extremely important for improving prognosis, especially in early-stage PDAC. This study prospectively evaluated the usefulness of repeated pancreatic juice cytology (PJC) using an endoscopic nasopancreatic drainage (ENPD) catheter for the diagnosis of PDAC. We enrolled 82 patients suspected of having resectable PDAC, based on imaging studies, and judged the necessity for cytology. The diagnostic yield of up to six repeated PJCs and the incidence of complications, such as pancreatitis, was evaluated. A total of 60 patients were diagnosed with PDAC. The overall sensitivity and specificity were 46.7% and 95.5%, respectively. The cumulative positivity rate increased with the number of sampling sessions, reaching 58.3% in the sixth session. The sensitivity was significantly higher in the pancreatic head than in the pancreatic tail (p = 0.043). Additionally, it was 100% in four patients with a tumor size ≤10 mm. Pancreatitis occurred in six patients (7.3%), all of whom were treated conservatively. In the diagnosis of PDAC, repeated PJC using an ENPD catheter revealed a cumulative effect of sensitivity up to six times and an excellent diagnostic yield for small PDAC.

Effects of hepatitis B virus infection on the interferon response in immunodeficient human hepatocyte chimeric mice.

Complementary DNA microarray analysis of human livers cannot exclude the influence of the immunological response. In this study, complementary DNA microarray analysis was performed under immunodeficient conditions with human hepatocyte chimeric mice, and gene expression profiles were analyzed by hepatitis B virus (HBV) infection and/or interferon treatment. The expression levels of 183 of 525 genes upregulated by interferon treatment were significantly suppressed in response to HBV infection. Suppressed genes were statistically significantly associated with the interferon signaling pathway and pattern recognition receptors in the bacteria/virus recognition pathway (P = 1.0 × 10(-8) and P = 1.2 × 10(-8), respectively). HBV infection attenuated virus recognition and interferon response in hepatocytes, which facilitated HBV escape from innate immunity.

A single nucleotide polymorphism in activated Cdc42 associated tyrosine kinase 1 influences the interferon therapy in hepatitis C patients.

BACKGROUND & AIMS: Cdc42 is a Rho family GTPase protein and was recently implicated in mediating hepatitis C virus (HCV) infectivity. This study examines the association between Cdc42-related gene and interferon (IFN) therapy in HCV patients. METHODS: We analyzed the associations between the outcome of IFN therapy and 17 tagging single nucleotide polymorphisms (SNPs) within two genes involved in Cdc42 signaling (CDC42 and ACK1). A total of 295 out of the 409 study subjects were sustained responders (SR) and 114 were non-responders (NR). Replication was performed using an independent set of 794 IFN-treated patients. RESULTS: SNP rs2278034 [A/G] in intron 11 of activated Cdc42 associated tyrosine kinase (ACK) 1 was associated with the outcome of IFN therapy (p=6.4 × 10(-4)). Replication analysis confirmed the association (p=2.2 × 10(-3)) for patients treated with IFN monotherapy, but the association was not significant for pegylated-IFN-plus ribavirin therapy. Analysis using published HapMap expression data revealed that ACK1 expression correlates with IFN-stimulated gene (ISG) expression independently of ethnicity, but the relationship between rs2278034 and ACK1 expression was observed only within Asian populations. Over-expression of ACK1, but not the kinase-inactive mutant, increased ISG transcription in Huh7 cells. ACK1 expression enhanced the IFN-stimulated response element (ISRE) and interferon-γ-activated site (GAS) promoter activity through tyrosine phosphorylation of signal transducers and activators of transcription (STAT) 1. Furthermore, ACK1 over-expression in HCV-N replicon cells inhibited HCV replication. CONCLUSIONS: SNP rs2278034 in ACK1 is associated with IFN therapy outcome in patients with HCV. ACK1 may play a role in innate and IFN-induced antiviral action against HCV.

[Management of gastrointestinal malformations in children with asplenia syndrome].

Asplenia syndrome (AS) is a complex disorder that includes a variety of cardiovascular and gastrointestinal malformations (GMs), and its prognosis remains poor. We treated 40 AS patients between 2000 and 2010, 10 of whom underwent surgery to correct GMs. These comprised 7 hiatus hernias (HH), 1 intestinal malrotation (IM), 1 stomach volvulus, and 1 ompalocele. In this review, we consider the optimal surgical timing and methods of management, especially for HH and IM. Surgery was performed in 6 of 7 HH patients. Four of those underwent laparotomy, 1 thoracotomy, and 1 laparoscopy. The Toupet method or fixation of the His angle or hiatus closure was selected in each case. Because the stomach intruding into the mediastinum may cause pulmonary venous obstruction and respiratory impairment, surgical repair is preferably performed prior to Glenn surgery. Among the 9 IMs, 8 were nonrotational and required no treatment, and only 1 required surgical treatment. GMs in AS are difficult to manage because of the unique anatomy, meaning there are no fixed surgical techniques. Each patient must be carefully examined preoperatively, and the optimum surgical method selected. Close collaboration with cardiovascular surgeons is vital when determining the timing and method of surgery.

Clinical Analysis of Early-Stage Pancreatic Cancer and Proposal for a New Diagnostic Algorithm: A Multicenter Observational Study.

Early diagnosis of pancreatic ductal adenocarcinoma (PDAC) is challenging but essential for improving its poor prognosis. We established a multicenter study to clarify the clinicopathological features, and to propose new algorithm for early diagnosis of PDAC. Ninety-six patients with stage 0 and IA PDAC were enrolled from 13 high-volume centers. Overall, 70% of the patients were asymptomatic. The serum pancreatic enzyme levels were abnormal in half of the patients. The sensitivity of endoscopic ultrasonography (EUS) for detecting small PDAC was superior to computed tomography and magnetic resonance imaging (MRI) (82%, 58%, and 38%, respectively). Indirect imaging findings were useful to detect early-stage PDAC; especially, main pancreatic duct stenosis on MRI had the highest positive rate of 86% in stage 0 patients. For preoperative pathological diagnosis, the sensitivity of endoscopic retrograde cholangiopancreatography (ERCP)-associated pancreatic juice cytology was 84%. Among the stage IA patients, EUS-guided fine-needle aspiration revealed adenocarcinoma in 93% patients. For early diagnosis of PDAC, it is essential to identify asymptomatic patients and ensure close examinations of indirect imaging findings and standardization of preoperative pathological diagnosis. Therefore, a new diagnostic algorithm based on tumor size and imaging findings should be developed.

A polymorphism in MAPKAPK3 affects response to interferon therapy for chronic hepatitis C.

BACKGROUND & AIMS: This study aimed to identify host single nucleotide polymorphisms (SNPs) that are associated with the efficacy of interferon (IFN) therapy in patients with chronic hepatitis C. METHODS: We examined whether 116 tagging-SNPs from 13 genes that are involved in type I IFN signaling associate with the outcome of IFN therapy in Japanese case-control groups; the study included 468 sustained responders and 587 nonresponders. RESULTS: We identified 2 SNPs (rs3792323 [A/T] and rs616589 [G/A]), located in intron 2 of mitogen-activated protein kinase-activated protein kinase 3 (MAPKAPK3) that were associated with the outcome of IFN therapy in patients infected with hepatitis C virus (HCV) genotype 1b (P = 4.6 x 10(-5) and 4.8 x 10(-5), respectively). The 2 SNPs were in strong linkage disequilibrium and multivariate logistic regression analysis showed that rs3792323 is an independent factor associated with the IFN efficacy (genotype 1b; P = .0011). MAPKAPK3 is a kinase involved in the mitogen and stress responses, but the biological significance of MAPKAPK3 in IFN responses is poorly understood. By using an allele-specific transcript quantification assay in liver biopsy, we showed that allele-specific expression of MAPKAPK3 messenger RNA, corresponding to the risk allele for nonresponse, was significantly higher than that of the other allele. Luciferase reporter assay data indicated that overexpression of MAPKAPK3 inhibits IFN-alfa-induced gene transcription via IFN-stimulated response element and IFN-gamma-activated site. CONCLUSIONS: The SNP rs3792323 in MAPKAPK3 associates with the outcome of IFN therapy in patients with HCV genotype 1b. Our functional analyses indicate that MAPKAPK3 inhibits IFN-alfa-induced antiviral activity.

Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome.

OBJECTIVES: Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown. METHODS: From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children's Hospital Japan with significant (3-4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome. RESULTS: Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0-2) and those with a poor outcome (3-4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage. CONCLUSION: Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.

Overlooked muscle cramps in patients with chronic liver disease: in relation to the prevalence of muscle cramps.

INTRODUCTION: Muscle cramps are common comorbidities in chronic liver disease (CLD). Although the prevalence of these has been reported in patients with liver cirrhosis (LC), that of CLD is unknown. In this study, we aimed to clarify the prevalence and characteristics of muscle cramps in individual CLD. PATIENTS AND METHODS: A total of 432 patients with CLD who visited our hospital were enrolled. The existence of muscle cramps, frequency, time zone, duration, and the degree of pain were investigated using a medical interview questionnaire. RESULTS: The median age of the patients was 65 years and 48.6% of the patients were women. The prevalence of muscle cramps was 25.9%. Age, female sex, lower BMI, existence of comorbid diseases, and liver fibrosis were associated significantly with muscle cramps. In LC, muscle cramps were significantly frequent, and the severity and duration of these were significantly stronger and longer compared with chronic hepatitis. Female sex [odds ratio (OR): 2.26; P=0.014], diabetes (OR: 29.4; P<0.001), chronic kidney disease (OR: 8.33; P=0.004), and lower BMI (OR: 0.853; P<0.001) were independent factors associated with muscle cramps in CLD. Muscle mass indices were significantly lower among nonalcoholic fatty liver disease patients with muscle cramps, female patients, elderly patients, and patients with advanced fibrosis. CONCLUSION: The prevalence of muscle cramps was relatively high in CLD. Female sex, comorbid diabetes, and chronic kidney disease are associated with muscle cramps in CLD. Furthermore, reduced muscle mass is related to muscle cramps in nonalcoholic fatty liver disease.

Expression of multidrug resistance-associated protein 2 is involved in chemotherapy resistance in human pancreatic cancer.

Despite the recent introduction of the new anticancer agents gemcitabine (GEM) and TS-1, as well as combination regimens such as GEM plus cisplatin (CDDP), pancreatic cancer treatment remains relatively ineffective. Both intrinsic and acquired resistance to chemotherapy are major roadblocks to the successful treatment of pancreatic cancer patients. The aims of this study were to examine the expression of multidrug resistance-associated proteins (MRPs) MRP1, MRP2 and MRP3 and to evaluate the correlation between MRP2 expression and CDDP resistance in human pancreatic cancer. Five human pancreatic cancer cell lines and several surgically resected pancreatic cancer tissues were subjected to reverse-transcriptase (RT)-PCR, real-time PCR and immunohistochemical analysis. While MRP1 and MRP2 mRNA was expressed in all cell lines, MRP3 mRNA was only detected in two cell lines. In resected pancreatic cancer tissues, only MRP2 mRNA was expressed and it was overexpressed compared with normal pancreatic tissues. MRP2 protein expression was observed in 77.5% (31/40) of cancer tissues, primarily in the cytoplasm of cancer cells, but was not observed in normal pancreatic tissue. Two CDDP-resistant pancreatic cancer cell line SUIT-2 variants, SUIT-2-CD3 and SUIT-2-CD4, were established by continuously administering 10 nM CDDP to SUIT-2 cell lines for 3 and 4 months, respectively. Incubation of these cells with CDDP in the presence of anti-MRP2 antibody or the MRP2 inhibitor MK-571 in a growth inhibition assay demonstrated that the CDDP-resistant variants were more resistant to CDDP than the parent cell line and this resistance was diminished by either anti-MRP2 antibody or MK-571. Moreover, RT-PCR and real-time PCR revealed that while induction of MRP2 mRNA expression was increased in CDDP-resistant compared with parent cells, MRP1 and MRP3 expression remained unchanged. These observations suggest that MRP2 may correlate to intrinsic and acquired resistance for CDDP in human pancreatic cancer.

Right atrial isomerism with infracardiac total anomalous pulmonary venous connection complicated by hiatal hernia.

A 31-day-old girl with right atrial isomerism, a single atrium, a single right ventricle, pulmonary atresia, patent ductus arteriosus, coarctation of the pulmonary artery, infracardiac total anomalous pulmonary venous connection, and a large hiatal hernia was referred to our institution. We successfully performed a first-stage open palliation comprising the creation of a systemic-to-pulmonary arterial shunt, pulmonary artery angioplasty, and correction of the total anomalous pulmonary venous connection. During cardiopulmonary bypass, the hiatal hernia was also repaired through the median sternotomy itself without another laparotomy. The postoperative course was uneventful, and we avoided the potential complications of pulmonary venous obstruction and enteral feeding. She underwent a bidirectional Glenn operation and Fontan completion at 5 and 19 months of age, respectively.

Infection of human hepatocyte chimeric mouse with genetically engineered hepatitis C virus and its susceptibility to interferon.

We developed a reverse genetics system of hepatitis C virus (HCV) genotypes 1a and 2a using infectious clones and human hepatocyte chimeric mice. We inoculated cell culture-produced genotype 2a (JFH-1) HCV intravenously. We also injected genotype 1a CV-H77C clone RNA intrahepatically. Mice inoculated with HCV by both procedures developed measurable and transmissible viremia. Interferon (IFN) alpha treatment resulted in greater reduction of genotype 2a HCV levels than genotype 1a, as seen in clinical practice. Genetically engineered HCV infection system should be useful for analysis of the mechanisms of resistance of HCV to IFN and other drugs.

A case of staged Norwood procedure for a unique form of truncus arteriosus, interrupted aortic arch with hypoplastic tricuspid valve and right ventricle and anomalous left coronary artery orifice: first reported case.

We present the first reported case of truncus arteriosus, interrupted aortic arch with hypoplastic tricuspid valve and right ventricle and anomalous left coronary artery orifice in a neonate in whom successful palliation was performed using two-staged procedure. The first-stage palliation was the bilateral pulmonary artery band at age 2 days. The second-stage palliation was a Norwood procedure concomitant with ASD creation and patch augmentation of the pulmonary artery confluence at 22 days. The patient is doing well, with unobstructed aortic arch and mild truncal valve regurgitation.

Use of 4-Fr versus 6-Fr Nasobiliary Catheter for Biliary Drainage: A Prospective, Multicenter, Randomized, Controlled Study.

Background and Aim. Endoscopic nasobiliary drainage (NBD) effects according to diameter remain unclear. We aimed to assess the drainage effects of the 4-Fr and 6-Fr NBD catheters. Methods. This prospective, multicenter, randomized, controlled study was conducted at Hiroshima University Hospital and related facilities within Hiroshima Prefecture. Endoscopic retrograde cholangiopancreatography (ERCP) in 246 patients revealed acute cholangitis, obstructive jaundice, and/or extrahepatic cholestasis; 4-Fr or 6-Fr NBD catheters were randomly allocated and placed in these patients. The primary endpoint was the efficacy of NBD based on the technical success rate and clinical success (rates of change in blood test and amount of bile output). Secondary endpoints included the spontaneous catheter displacement rate and nasal discomfort. Results. The technical success rate and clinical success did not differ significantly between groups. No spontaneous catheter displacement was noted in either group. Nasal discomfort due to catheter placement was significantly lower in the 4-Fr group versus the 6-Fr group (24 h after ERCP: 2.4 versus 3.5 cm, P = 0.005; 48 h after ERCP: 2.2 versus 3.1 cm, P = 0.01). Conclusion. The 4-Fr NBD catheter was not inferior to 6-Fr NBD catheter in terms of clinical success; the 4-Fr NBD catheter was useful to reduce nasal discomfort.

Senning operation for very low birth weight infant with transposition of the great arteries: one of the smallest cases in the world.

We report a case of a Senning operation for very low birth weight infant weighing 1,168 g with transposition of the great arteries. The patient underwent a Senning operation on 62 days, 1,700 g after the first palliation. In this case, the orifice of the left anterior descending artery was located in sinus 1 (left posterior facing sinus), but we could not find orifices of both right coronary artery and left circumflex artery before the Senning operation. The surgical procedure of the Senning operation is typical one, but we used flesh autopericardial patch to cover the roof of the new pulmonary vein chamber to get an enough size. The patient recovered with no cardiac events after the repair.