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Background: Sarcopenia has been reported to be associated with short-term outcomes after gastric endoscopic submucosal dissection (ESD). The "strength, assistance with walking, rising from a chair, climbing stairs, and falls" (SARC-F) questionnaire has been widely used as a screening tool for sarcopenia; however, SARC-F combined with body mass index and age (SARC-F+EBM) has recently been reported to be more useful than SARC-F alone. This study aimed to investigate the association between sarcopenia, measured using SARC-F+EBM, and short-term outcomes after gastric ESD. Methods: Patients who underwent gastric ESD at our institution between May 2020 and June 2023 were included, and their medical records were reviewed retrospectively. A SARC-F+EBM score ≥ 12 indicated sarcopenia. We evaluated the incidence of adverse events and the length of hospital stay in the sarcopenia and non-sarcopenia groups. Results: Overall, 263 patients (64 and 199 in the sarcopenia and non-sarcopenia groups, respectively) were investigated. The incidence of adverse events with a Common Terminology Criteria for Adverse Events grade ≥ 3 was not significantly different between the sarcopenia and non-sarcopenia groups (6.2% vs. 8.5%, p = 0.791). The proportion of patients with an extended hospital stay (≥ 10 days) was significantly higher in the sarcopenia group than that in the non-sarcopenia group (12.5% [8/64] vs. 3.5% [7/199], p = 0.012). Multivariate analysis showed that sarcopenia and lesions that present technical difficulty in ESD were independent risk factors for extended hospital stays (≥ 10 days). Of the eight cases having extended hospital stays in the sarcopenia group, four were due to the management after gastric ESD, three were due to family circumstances, and one was due to decreased activities of daily living. Conclusions: Sarcopenia is not a predictor of adverse events associated with gastric ESD. However, patients with sarcopenia may be hospitalized for longer owing to non-ESD-related factors. [ABSTRACT FROM AUTHOR]

Purpose: Preoperative deep vein thrombosis (DVT) is a risk factor for postoperative venous thromboembolism (VTE), causing severe mortality. Early detection of preoperative DVT is essential to prevent postoperative VTE. However, little is known regarding preoperative DVT in patients undergoing major surgery. The present study aimed to determine the incidence and risk factors of preoperative DVT in patients admitted for total hip arthroplasty (THA). Methods: From August 2017 to September 2022, 243 patients admitted for THA at our institution were enrolled in this study. Patients medical records and preoperative laboratory data were retrospectively collected. According to the results of lower-limb ultrasonography, patients were divided into either the non-DVT (n = 136) or DVT (n = 43) group. The incidence of DVT and independent risk factors for preoperative DVT were investigated using univariate and multivariate logistic regression analyses. Results: The mean age was 74.0 ± 8.4 years. Preoperative DVT was diagnosed in 43 of the 243 (17.7%) patients. The risk of DVT was significantly high (p < 0.05) in patients with advanced age, increased D-dimer levels, and malnutrition status, as assessed by the Geriatric Nutritional Risk Index (GNRI). Multivariate analysis showed that advanced age, increased D-dimer level, and malnutrition status assessed by the GNRI were independent risk factors for preoperative DVT. Conclusion: A high incidence of preoperative DVT was observed in patients undergoing THA. Advanced age, increased D-dimer levels, and malnutrition assessed by the GNRI increased the risk of preoperative DVT. Screening high-risk subgroups for preoperative DVT is necessary to prevent postoperative VTE. [ABSTRACT FROM AUTHOR]

Summary: Immune-related sclerosing cholangitis (irSC) is relatively rare and its clinical characteristics are not well known. In this study, we aimed to summarize the clinical features of irSC. Clinical data were collected retrospectively from 1,393 patients with advanced malignancy treated with immune-checkpoint inhibitors (ICIs) between August 2014 and October 2021. We analyzed patients with immune-related adverse events of liver injury (liver-irAEs) and compared irSC and non-irSC groups. Sixty-seven patients (4.8%) had a liver-irAE (≥ grade 3) during the follow-up period (median, 262 days). Among these, irSC was observed in eight patients (11.9%). All patients in the irSC group were treated with anti-PD-1/PD-L1 antibodies. Compared with the non-irSC group, the irSC group showed mainly non-hepatocellular liver injury (87.5 % vs 50.8 %, P = 0.065), and had elevated serum inflammatory markers (e.g., CRP and NLR) and biliary enzymes (e.g., GGTP and ALP) at the onset of liver-irAEs. Furthermore, most patients with irSC had abdominal pain. In the non-irSC group, the liver injury of 23 patients improved only with the discontinuation of ICIs, and 22 patients improved with medication including prednisolone (PSL). Conversely, almost all patients (n=7) in the irSC group were treated with PSL, but only two patients experienced an improvement in liver injury. We found that irSC is characterized by a non-hepatocellular type of liver injury with abdominal pain and a high inflammatory response and is refractory to treatment. Further examination by imaging is recommended to detect intractable irSC in cases with these characteristics. [ABSTRACT FROM AUTHOR]

Background: Inflammatory bowel disease (IBD), which encompasses both ulcerative colitis (UC) and Crohn's disease (CD), is a risk factor for venous thromboembolism (VTE), which includes deep vein thrombosis (DVT) and pulmonary embolism (PE).Aim: To investigate the incidence of, and risk factors for, VTE in patients with IBD in Japan.Methods: This was a retrospective, non-interventional study in patients with IBD from the Japan Medical Data Center claims database. Incidence rates (IRs; unique patients with events per 100 patient-years) were calculated for VTE, DVT, and PE among the IBD, UC, and CD cohorts. Odds ratios of potential risk factors were calculated by univariate and multivariate analyses in a nested case-control design.Results: Overall, 16 273 patients with IBD were included: 13 585 with UC and 3443 with CD. VTE events occurred in 1.3%, 1.2%, and 1.9% of patients with IBD, UC, and CD, respectively. In patients with IBD, UC, and CD, IRs of VTE were 0.45, 0.40, and 0.64, respectively, IRs of DVT were 0.42, 0.38, and 0.61, respectively, and IRs of PE were 0.07, 0.07, and 0.11, respectively. In patients with IBD, treatment history (immunomodulators), cardiovascular risk (hypertension, high-density lipoprotein or diabetes mellitus, and history of coronary artery disease or heart failure), malignancy, and undergoing major surgery were identified as potential risk factors for VTE in the multivariate analysis, with similar risk factors reported for patients with UC and CD.Conclusions: This large study provides insight into the incidence and risk factors for VTE in patients with IBD from Japan. [ABSTRACT FROM AUTHOR]

The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0-179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to describe epidemiologic features of pediatric blunt renal trauma. Methods: We performed a retrospective analysis using the Japan Trauma Data Bank over 15 years. We included patients younger than 18 years with blunt renal trauma. We analyzed temporal trends and variations across age groups in patient characteristics, intervention, and in-hospital mortality. We also assessed factors associated with in-hospital mortality. Results: We identified 435 pediatric patients with blunt renal trauma. Their median age was 14 years and median Injury Severity Score (ISS) was 17. The most common mechanism of injury was traffic accident in all age groups. Nephrectomy was performed in 3.2%, and the overall in-hospital mortality was 4.6%, both of which showed decreasing trends. The most common mechanism of injury by age group was a pedestrian accident in infants/toddlers/preschoolers (43.5%), pedestrian accident in middle childhood (18.5%), bicycle accident in young teens (24.7%), and motorcycle accident in teenagers (41.2%). Sports-related injury was common in young teens (23.3%) and teenagers (15.2%). Factors such as ISS, shock, concomitant injury, and nephrectomy were associated with high in-hospital mortality. Conclusions: We described decreasing trends in nephrectomy and in-hospital mortality in pediatric blunt renal trauma and found traffic accident and sports-related injury were common in the pediatric population in Japan. [ABSTRACT FROM AUTHOR]

Introduction: Rheumatoid arthritis (RA) is a chronic inflammatory disorder that causes swelling and pain in the joints. Pain management for RA becomes easier if patients are in clinical remission, although some may experience remnant pain despite adequate disease control. Duloxetine, a potent serotonin and norepinephrine reuptake inhibitor, has demonstrated pain relief effects in patients with osteoarthritis; however, the effectiveness of duloxetine for pain in patients with RA in clinical remission has not been investigated. The purpose of this study was to evaluate the efficacy of duloxetine for remnant pain in patients with RA in clinical remission Methods: This retrospective study included RA patients from a single institute in Japan from January 2011 to September 2019. Patients with RA who had a visual analog scale (PS-VAS) score > 30 mm and Clinical Disease Activity Index (CDAI) score ≤ 2.8 were selected and were grouped depending on whether duloxetine was administered (G-D) or not (G-C). Variables such as PS-VAS, patient's global assessment (PGA), C-reactive protein (CRP), CDAI, Simplified Disease Activity Index (SDAI), modified Health Assessment Questionnaire (mHAQ), and quality of life (QOL) score were measured at the initiation of duloxetine and at the first CDAI remission in the G-D and G-C groups, respectively. Thereafter, the variables were measured at week 12. Changes in PS-VAS were compared as a primary outcome using the one-sample t test for each group. Differences between the two groups were evaluated using the Mann–Whitney U test. Results: A total of 306 patients (G-C = 238; G-D = 68) were evaluated. PS-VAS, mHAQ, and QOL scores in both groups improved at week 12 from baseline, in which these scores in the G-D group demonstrated significantly higher improvements than those observed in the G-C group. At week 12, although CDAI scores significantly increased from baseline in both groups, patients were considered to have low disease activity. Conclusion: Duloxetine treatment in patients with RA in clinical remission appears to have comparable effectiveness on relieving remnant pain, improving activities of daily living and maintaining QOL. Duloxetine is not expected to have any effects on disease activity control. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax.Methods: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study.Results: Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmH2O, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax.Conclusions: The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients. [ABSTRACT FROM AUTHOR]

Purpose: Hirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan.Methods: Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan.Results: A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes.Conclusion: HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes. [ABSTRACT FROM AUTHOR]

Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor.Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment. What is Known: • There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions. • Data are scarce on their effectiveness in non-Asian children. What is New: • We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement. • Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities. [ABSTRACT FROM AUTHOR]

Purpose: Vena cava syndrome (VCS) from stenosis of the superior vena cava or inferior vena cava caused by compression from a malignant tumor is one of the typical clinical conditions in patients with advanced stage malignant disease. VCS is difficult to manage and painful, reducing patients' quality of life. Although several reports have investigated stent placement for VCS, this treatment has never been established as the standard because of the lack of evidence of the safety and efficacy. We conducted a phase II trial and a phase III randomized controlled trial to clarify the role of stent placement in managing patients with VCS.Methods: In the phase II trial, 28 eligible patients were treated with stent placement. The efficacy of stent placement for VCS was evaluated based on the reduction of patients' symptom scores during 14 days following treatment. Technical success, technical feasibility, overall survival, recurrence of symptoms, and adverse events were evaluated. In the phase III trial, 32 patients were enrolled and randomly assigned to the test (n = 16) and control groups (n = 16). The area under the symptom score curve was compared between the groups. The EQ-5D, SF-8, and adverse events were evaluated until discontinuation of the protocol treatment or 28 days after enrollment.Results: In the phase II trial, the median patients' symptom scores significantly decreased from 10.50 before the procedure to 3.00 after the procedure. Technical success and technical feasibility rates were 96.4% and 100%, respectively. The incidence of treatment-related grade 3 or higher adverse events was 14.3%. In the phase III trial, significant superiority of stent placement was observed in the test, compared to that in the control, group. There was no significant difference in most other evaluations between the groups.Conclusions: Stent placement significantly improved the symptoms of VCS; thus, it might be accepted as the standard treatment to manage the symptoms of VCS.Trial Registration: JIVROSG-0402, JIVROSG-0807. [ABSTRACT FROM AUTHOR]

We evaluated clinical outcomes of disseminated intravascular coagulation (DIC) in patients with hematological malignancies treated with synthetic protease inhibitors (SPIs) and compared the effects of gabexate mesilate (FOY) and nafamostat mesilate (FUT). We retrospectively examined 127 patients [acute myeloid leukemia (n = 48), acute lymphoblastic leukemia (n = 25), and non-Hodgkin lymphoma (n = 54)] with DIC, who were diagnosed according to Japanese Ministry of Health, Labour and Welfare criteria and treated with SPIs [FOY (n = 55) and FUT (n = 72)] at our hospital from 2006 to 2015. The DIC resolution rates on days 7 and 14 were 42.6% and 62.4%, respectively. No significant differences were observed in DIC resolution rates between the FUT and FOY groups [40.3% vs. 45.5% (day 7), P = 0.586; 56.3% vs. 69.8% (day 14), P = 0.179, respectively]. Multivariate analysis revealed that response to chemotherapy was the only independent predictor of DIC resolution on days 7 and 14 (ORR 2.81, 95% CI 1.32-5.98, P = 0.007; ORR 2.51, 95% CI 1.12-5.65, P = 0.026). Resolution of DIC was correlated with improvement of background hematological malignancies, and no significant differences were observed between the two SPIs. [ABSTRACT FROM AUTHOR]

The geriatric nutritional risk index (GNRI) is a simple and well-established nutritional assessment tool that is a significant prognostic factor for various cancers. However, the role of the GNRI in predicting clinical outcomes of diffuse large B cell lymphoma (DLBCL) patients has not been investigated. To address this issue, we retrospectively analyzed a total of 476 patients with newly diagnosed de novo DLBCL. We defined the best cutoff value of the GNRI as 96.8 using a receiver operating characteristic curve. Patients with a GNRI < 96.8 had significantly lower overall survival (OS) and progression-free survival (PFS) than those with a GNRI ≥ 96.8 (5-year OS, 61.2 vs. 84.4%, P < 0.001; 5-year PFS, 53.7 vs. 75.8%, P < 0.001). Multivariate analysis showed that performance status, Ann Arbor stage, serum lactate dehydrogenase, and GNRI were independent prognostic factors for OS. Among patients with high-intermediate and high-risk by National Comprehensive Cancer Network-International Prognostic Index (NCCN-IPI), the 5-year OS was significantly lower in patients with a GNRI < 96.8 than in those with a GNRI ≥ 96.8 (high-intermediate risk, 59.5 vs. 75.2%, P = 0.006; high risk, 37.4 vs. 64.9%, P = 0.033). In the present study, we demonstrated that the GNRI was an independent prognostic factor in DLBCL patients. The GNRI could identify a population of poor-risk patients among those with high-intermediate and high-risk by NCCN-IPI. [ABSTRACT FROM AUTHOR]

Polycythemia vera (PV) and essential thrombocythemia (ET) are associated with life-threatening thrombohemorrhagic events, and disease progression and development of non-hematological malignancies also reduce long-term survival. We retrospectively surveyed thrombohemorrhagic events and overall survival (OS) in 62 PV and 117 ET patients. The cumulative incidences of thrombohemorrhagic events in PV and ET patients were 11.3 and 10.3%, and the incidence rates were 2.42 and 1.85 per 100 person-years. The combined incidence rates of disease progression and development of non-hematological malignancies in PV and ET patients were 1.73 and 1.69 per 100 person-years. The incidence rates of thrombohemorrhagic events in our Japanese PV/ET patients were lower than those reported by most Western studies, but were comparable to those in the largest prospective observational study in ET patients. The combined incidence rates of disease progression and development of non-hematological malignancies were similar between Japanese and Western PV/ET patients. In ET patients, the conventional risk stratification model based on the presence of advanced age or history of thrombosis was useful to predict thrombosis risk, and both the conventional model and the International Prognostic Score of thrombosis in ET based on the above 2 risk factors plus increased leukocyte count could predict poor survival. [ABSTRACT FROM AUTHOR]

Purpose: The incidence of and risk factors for febrile neutropenia (FN) in Japanese non-Hodgkin B-cell lymphoma (B-NHL) patients receiving rituximab, cyclophosphamide, doxorubicin, vincristine, and predonisolone (R-CHOP) chemotherapy are unknown. We conducted this study to address this issue.Methods: In this single-center, retrospective, observational study, 466 patients with B-NHL who completed an R-CHOP regimen within a 7-year period and who planned to undergo at least three cycles of this regimen were analyzed. The following FN-related factors were assessed: fever, infection, disease state, neutrophil count, and prophylactic interventions such as use of antibiotics and/or granulocyte colony-stimulating factor (G-CSF). We simulated the FN incidence and 95% confidence interval (CI) of patients without prophylaxis with G-CSF (cycle 1) using bootstrap sampling.Results: The incidence of FN was 9.1% (42 of 462) in cycle 1 and 12.3% (57 of 462 patients) throughout all cycles, with 73.7% (42/57) developing FN during cycle 1. Risk factors for FN among patients with B-NHL treated with R-CHOP were albumin <35 g/L (p = 0.0047), relative dose intensity <85% (p = 0.0007), and lack of prophylaxis with G-CSF (p = 0.0006) in cycle 1. In the simulation analysis, the estimated FN incidence in cycle 1 was 16.2% (95% CI [10.9-22.2]).Conclusions: At 9.1% in cycle 1 and 12.3% throughout all cycles, the incidence of FN was lower than previously reported, possibly reflecting the appropriate use of G-CSF in this clinical setting. For patients with risk factors, the prophylaxis with G-CSF may decrease the occurrence of FN. [ABSTRACT FROM AUTHOR]

Purpose: Favorable long-term outcomes of the Zilver PTX drug-eluting stent (DES) in femoropopliteal lesions have been demonstrated. Chronic renal failure (CRF) has been shown to be a risk factor for restenosis and decreased limb salvage. The results of the DES in patients with CRF have not previously been reported. This study compares the results with the DES in patients with CRF and those without CRF.Methods: This retrospective analysis from the Zilver PTX Japan Post-Market Surveillance Study included 321 patients with CRF and 584 patients without CRF. Outcomes included freedom from target lesion revascularization (TLR) and patency.Results: Of the patients included in this subgroup analysis, 2-year data were available for 209 patients in the CRF group and 453 patients in the non-CRF group. The two groups were similar in terms of lesion length and the frequency of in-stent restenosis. Critical limb ischemia, severe calcification, and diabetes were more common in patients with CRF, whereas total occlusion was more common in patients without CRF. Freedom from TLR rates were 81.4 versus 84.9% (p = 0.24), and patency rates were 70.7 versus 70.3% (p = 0.95) in patients with and without CRF at 2 years, respectively.Conclusion: This is the first comparative study of the DES in femoropopliteal artery lesions in patients with and without CRF. These results indicate that the DES placed in femoropopliteal artery lesions of CRF patients is safe and effective with similar patency and TLR rates to patients without CRF.Level Of Evidence: Level 3, Post-Market Surveillance Study. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the rate and predictive factors of post-operative neurological deterioration in ossified yellow ligament (OYL) surgery.Methods: A retrospective review was conducted for all patients with thoracic OYL causing myelopathy requiring surgical decompression from January 1998 to December 2012. Clinical parameters under study included clinical presentation, distribution of OYL, pre-operative walking score, pre- and post-operative neurological status, status of intra-operative neurophysiological monitoring, and modified Japanese Orthopaedic Association (mJOA) score. Any complications were also recorded. All outcomes were measured at post-operative 1 week and at 2 years.Results: A total of 26 patients were included in this study. Most patients (92.3%) had Frankel grade D pre-operatively. The rate of neurological deterioration was 15.4% and was correlated with the presence of dural tear, extra-dural hematoma and spinal cord injury. Pre-operative walking score was prognostic of patients' walking ability in the post-operative period. Intra-operative monitoring of Somatosensory Evoked Potentials (SSEP) was found to be useful for monitoring spinal cord injury in OYL surgery, with a positive predictive value of 100% and a negative predictive value of 92.3%. The false negative rate of a SSEP signal drop was only 7.7% CONCLUSIONS: This is the first study exploring risk factors for post-operative neurological deterioration after surgery for thoracic OYL. The rate of neurological deficit is not small and prognostic factors for poor outcome include poor pre-operative walking score, presence of intra-operative dural tear, extra-dural hematoma and spinal cord injury, and intra-operative drop of SSEP signal. [ABSTRACT FROM AUTHOR]

Background: Among comorbidities, pulmonary hypoplasia (PH) is known as a significant risk factor for mortality in infants with end-stage kidney disease (ESKD). However, the final outcomes of infants with both ESKD and PH are still not well defined, as the diagnosis modality, and definition of PH severity remain ambiguous. Methods: Children initiating peritoneal dialysis during infancy from 1990 to 2015 were followed until death, date of last contact, or the end of 2015. We examined the long-term outcome of children with congenital pulmonary disorders by studying infants with prolonged respiratory disorders of greater than 28 days duration after birth and evaluated risk factors for mortality. Results: Forty-six children were followed (median follow-up, 9.23 years), and classified as children without ( n = 38; Group A) or with ( n = 8; Group B) a prolonged respiratory disorder. Overall actuarial 5 year survival rate in this cohort was 79.5 %. The survival curve in Group B showed a significant decline compared with Group A. Prolonged respiratory disorder was significantly associated with mortality by multivariate analysis (hazard ratio, 8.32). Conclusions: Infants who initiate peritoneal dialysis complicated by prolonged respiratory disorders have increased adverse risk factors for mortality; therefore, withholding aggressive treatment should be considered. [ABSTRACT FROM AUTHOR]

Background: The significance of HBV reactivation during immunosuppressive therapy was evaluated in three nationwide cohorts including patients with previously resolved HBV (prHBV) infection.Methods: The clinical features of 1061 patients with acute liver failure (ALF) or late-onset hepatic failure (LOHF) were retrospectively examined, focusing on those who experienced HBV reactivation. Additionally, 420 patients with prHBV infection were prospectively enrolled: 203 received immunosuppressive therapies immediately after enrollment, while the remaining 217 were enrolled after having received immunosuppressive therapies without the occurrence of HBV reactivation. The serum HBV-DNA levels were prospectively monitored every month, and the incidences of HBV reactivation, defined as a serum HBV-DNA level of 1.3 log IU/ml or more, were evaluated.Results: In the retrospective study, persistent HBV infection was found in 90 patients, and HBV reactivation was responsible for liver injuries in 50 patients including 23 receiving immunosuppressive therapies (26 with HBs-antigen positivity, 7 with prHBV infection). None of seven patients with prHBV infection were rescued. In the prospective studies, HBV reactivation occurred in ten patients, but preemptive entecavir administration prevented liver injury. The cumulative reactivation rate was 3.2 % at 6 months, and the increase of the rate compared to that at 6 months was +1.5 % at 48 months.Conclusions: HBV reactivation during immunosuppression was responsible for liver injuries in a quarter of the ALF/LOHF patients with persistent HBV infection. Early serum HBV-DNA monitoring may improve patient prognosis, since HBV reactivation typically occurs within 6 months of the start of immunosuppressive therapies in patients with prHBV infection. [ABSTRACT FROM AUTHOR]

Background: The lower rectum close to the dentate line has distinct characteristics, making endoscopic submucosal dissection (ESD) of tumors challenging. We assessed clinical outcomes of ESD for such patients with hemorrhoids.Methods: Sixty-four patients (mean age, 68 years) underwent ESD for anorectal tumors close to the dentate line. We divided patients into those with (Group A, 45 patients) and without hemorrhoids (Group B, 19 patients). We examined en bloc and histological en bloc resection rates, procedure time, complication rates, and postoperative prognosis after ESD.Results: The mean tumor size was 43 mm. Histologic diagnoses were adenoma (42 %, 27/64), carcinoma in situ (44 %, 28/64), and T1 carcinoma (14 %, 9/64). There was no significant difference in en bloc resection (93 %, 42/45 vs. 95 %, 18/19) or postoperative bleeding rates (16 %, 7/45 vs. 11 %, 2/19) between Groups A and B, respectively. The mean procedural durations were 120 and 124 min, respectively, in Groups A and B. No perforations occurred. There was no significant difference in postoperative anal pain rate between Groups A (18 %, 8/45) and B (16 %, 3/19), and it resolved within a few days in all cases. There was one case of stricture in Group B. Two patients with T1 carcinoma underwent additional surgery, one underwent chemotherapy, and five had no additional treatment. No recurrence occurred during the follow-up period of 38 months.Conclusions: ESD is safe and effective for anorectal tumors close to the dentate line in patients with hemorrhoids. [ABSTRACT FROM AUTHOR]

Okinawa Prefecture, located in the subtropics, is an area of endemic adult T-cell leukemia-lymphoma (ATL) in Japan. We retrospectively analyzed 659 patients with aggressive ATL in seven institutions in Okinawa between 2002 and 2011. The median patient age was 68 years. More patients were aged ≥90 years (2.6 %), in this study, than in a nationwide survey (<1 %). The median survival time (MST) of the entire cohort was 6.5 months. Of the 217 patients who had a clinical status similar to that stated in the eligibility criteria of JCOG9801 (a randomized phase III study comparing VCAP-AMP-VECP with CHOP-14), 147 who received the CHOP regimen had a poorer MST than those in the CHOP-14 arm of JCOG9801 (8 vs 11 months). The prevalence of strongyloidiasis in the ATL patients was much higher (12.4 %) than in the historical cohort who visited the University of the Ryukyus Hospital (3.4 %). Furthermore, strongyloidiasis may be associated with ATL-related deaths. These findings suggest that, compared with other areas in Japan, in Okinawa, the proportion of patients aged ≥90 years with clinical features of aggressive ATL is higher, outcomes are poorer, and the disease is associated with a higher prevalence of strongyloidiasis. [ABSTRACT FROM AUTHOR]

There are some reports regarding hepatitis B virus (HBV) reactivation in patients with myeloma who are HBV carriers or who have had a resolved HBV infection, and there is no standard prophylaxis strategy for these patients. We performed a retrospective multicenter study to determine the incidence and characteristics of HBV reactivation in patients with multiple myeloma. We identified 641 patients with multiple myeloma who had been treated using novel agents and/or autologous stem cell transplantation with high-dose chemotherapy between January 2006 and June 2014 at nine Japanese hospitals. The patients' characteristics, laboratory data, and clinical courses were retrieved and statistically analyzed. During a median follow-up of 101 weeks, one of eight (12.5 %) HBV carriers developed hepatitis and 9 of 99 (9.1 %) patients with resolved HBV infection experienced HBV reactivation; the cumulative incidences of HBV reactivation at 2 years (104 weeks) and 5 years (260 weeks) were 8 and 14 %, respectively. The nine cases of reactivation after resolved HBV infection had received entecavir as preemptive therapy or were carefully observed by monitoring their HBV DNA levels, and none of these cases developed hepatitis. Among patients with multiple myeloma, HBV reactivation was not rare. Therefore, long-term monitoring of HBV DNA levels is needed to prevent hepatitis that is related to HBV reactivation in these patients. [ABSTRACT FROM AUTHOR]

Background: Both colorectal neoplasms and atherosclerosis can be induced by common visceral fat accumulation. However, the association between these diseases at the advanced stage, colorectal cancer and cardiovascular/cerebrovascular diseases, has not been elucidated.Aims: This study aimed to investigate the association between colorectal cancer and vascular diseases in relation to obesity and metabolic disorders, using a nationwide database of hospitalized patients in Japan. We used non-cardiac gastric cancer patients as a reference, because they were considered to be neutral for obesity or metabolic disorders.Methods: We identified 54,591 patients with colorectal cancer and 19,565 patients with gastric cancer from the Diagnosis Procedure Combination database. The data collected included: sex; age; body mass index (BMI); smoking status; comorbidity; and medication data, comprising hypertension, diabetes, hyperlipidemia, coronary artery disease, and stroke. We compared these data in a cross-sectional setting by multivariate analyses.Results: Multivariate logistic regression analyses showed that female sex, increased BMI, hypertension [odds ratio (OR) 1.11; 95 % confidence interval (CI) 1.07-1.15; P < 0.0001], and diabetes (OR 1.17; 95 % CI 1.12-1.23; P < 0.0001) were more associated with colorectal cancer than with gastric cancer. Smoking, aspirin use (OR 0.85; 95 % CI 0.79-0.92; P < 0.0001), and coronary artery disease (OR 0.90; 95 % CI 0.86-0.95; P = 0.0001) were inversely associated with colorectal cancer.Conclusions: Obesity and metabolic disorders were more associated with colorectal cancer than with non-cardiac gastric cancer, while coronary artery disease (CAD) was inversely associated. Some mechanisms involving separate populations of colorectal cancer and CAD under visceral fat accumulation might be suggested. [ABSTRACT FROM AUTHOR]

Unlabelled: No large cohort study has yet determined the incidence of acute kidney injury (AKI) in children with heart failure treated with renin-angiotensin system (RAS) inhibitors. We thus retrospectively analyzed the incidence and risk factors for severe AKI (stages 2-3 according to the Kidney Disease Improving Global Outcomes (KDIGO) guidelines) at our institutions from 2008 to 2011. Among 312 children (162 boys; median age, 7.3 months), 59 cases of AKI occurred in 45 children. The incidence of AKI was 14.3 cases per 100 person-years overall (follow-up 413.6 person-years), or 27.3, 16.8, and 4.5 cases per 100 person-years in children aged <1, 1-3, and ≥4 years, respectively. Among them, 23 (39.0 %) children had metabolic acidosis and 14 (23.7 %) had hyperkalemia. Younger age, myocardial disease, cyanotic congenital heart disease, use of spironolactone, and cardiac surgery were independent risk factors for AKI. Furthermore, 37.3 % of children suffered dehydration during AKI.Conclusion: AKI incidence is relatively high in children, particularly younger children, with heart failure treated using RAS inhibitors. Careful monitoring of renal function and serum electrolytes is essential. Proper management of fluid balance after infection and cardiac surgery may reduce the risk of AKI. Temporary discontinuation in RAS inhibitors should be considered during dehydration or surgery.What Is Known: • Angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) are the two main classes of renin-angiotensin system (RAS) inhibitors used to treat hypertension, heart failure, and chronic kidney disease. Acute kidney injury (AKI) and hyperkalemia are potentially life-threatening complications associated with the use of ACEIs and ARBs. Some reports have suggested that dehydration and cardiac surgery are risk factors for AKI in children. However, no large-scale cohort studies have determined the incidence of AKI, its risk factors, and its outcomes in children with heart failure treated with ACEIs and/or ARBs. What is new: • In this retrospective cohort study, we determined the incidence, severity, and risk factors for severe AKI in children with heart failure treated with ACEIs and/or ARBs. The incidence of AKI in these children was relatively high (14.3 episodes per 100 person-years). In addition, younger age, myocardial disease, cyanotic congenital heart disease, concomitant use of spironolactone, and cardiac surgery were risk factors for AKI. Furthermore, 37.3 % of children had dehydration during AKI episodes. • Our results suggested that appropriate fluid balance after infection and cardiac surgery might reduce the risk of AKI and its complications. Temporary discontinuation or reductions in the levels of ACEIs and/or ARBs during dehydration or before surgery may also be warranted in these patients. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to assess the technical success rate and adverse events (AEs) associated with computed tomography (CT)-guided percutaneous gastrostomy for patients with head and neck cancer (HNC).Materials and Methods: This retrospective study included patients with HNC who had undergone CT-guided percutaneous gastrostomy between February 2007 and December 2013. Information regarding the patients' backgrounds, CT-guided percutaneous gastrostomy techniques, technical success rate, and AEs were obtained from the medical records. In all patients, the stomach was punctured under CT fluoroscopy with a Funada gastropexy device.Results: During the study period, 177 patients underwent CT-guided percutaneous gastrostomy. The most common tumor location was the oral cavity, followed by the pharynx and maxilla. The indication for CT-guided percutaneous gastrostomy were tumor obstruction in 78 patients, postoperative dysphagia in 55 patients, radiation edema in 43 patients, and cerebral infarction in 1 patient. The technical success rate was 97.7 %. The overall mean procedure time was 25.3 min. Major AEs occurred in seven patients (4.0 %), including bleeding (n = 4), colonic injury (n = 1), gastric tear (n = 1), and aspiration pneumonia (n = 1). Minor AEs occurred in 15 patients (8.5 %), which included peristomal leakage (n = 6), irritation (n = 4), inadvertent removal (n = 2), peristomal hemorrhage (n = 1), peristomal infection (n = 1), and wound granulation (n = 1). The mean follow-up period was 111 days (range 1-1106 days).Conclusion: Our study suggests that CT-guided gastrostomy may be suitable in patients with HNC. [ABSTRACT FROM AUTHOR]

Respiratory syncytial virus (RSV) can cause life-threatening complications of lower respiratory tract infection (LRTI) in young children with malignancies, but reports remain limited. We performed a retrospective nationwide survey to clarify the current status of RSV disease among infants with hematological malignancies. Clinical course, treatment, and outcome of patients with hematological malignancies who suffered from RSV infections at the age of <24 months during anti-tumor therapy from April 2006 to March 2009 were investigated by sending a questionnaire to all member institutions of the Japanese Pediatric Leukemia/Lymphoma Study Group (JPLSG). Twelve patients with acute leukemia were identified as having experienced RSV disease. The primary diseases were acute myeloid leukemia (n = 8) and acute lymphoblastic leukemia (n = 4). RSV infection occurred pre- or during induction therapy (n = 8) and during consolidation therapy (n = 4). Eight patients developed LRTI, four of whom had severe pneumonia or acute respiratory distress syndrome; these four patients died despite receiving intensive care. In our survey, the prognosis of RSV disease in pediatric hematological malignancies was poor, and progression of LRTI in particular was associated with high mortality. In the absence of RSV-specific therapy, effective prevention and treatment strategies for severe RSV disease must be investigated. [ABSTRACT FROM AUTHOR]

Background: The criterion for performing a renal biopsy in children with idiopathic nephrotic syndrome (NS) showing microscopic hematuria at onset remains controversial. Methods: To determine an adequate renal biopsy criterion in children with NS showing hematuria, the optimal cutoff for the maximum red blood cell (RBC) range in urine sediment to separate minimal change disease (MCD) from other glomerular changes was obtained by receiver operating characteristic analysis. We studied 29 children with NS showing hematuria who were screened from 1,320 patients who underwent renal biopsies between January 2001 and September 2011. Patients were divided into two groups according to the cutoff value to verify its validity. Results: The optimal maximum RBC range was 30-49/high-power field (HPF). In group 1 (RBC ≤29/HPF, n = 14), 3 patients showed nephritis and the other 11 patients showed MCD. In group 2 (RBC ≥30/HPF, n = 15), 1 patient showed focal segmental glomerulosclerosis, 12 showed nephritis, and the other 2 showed MCD. These findings indicated that the ratio of non-MCD/MCD was significantly higher in group 2 than in group 1 ( P < 0.01). Conclusions: The use of maximum RBC range (30-49/HPF) for a criterion of renal biopsy in patients with NS showing hematuria may be reasonable for clinical practice. [ABSTRACT FROM AUTHOR]

Background: Prednisolone, the first-line treatment for children with nephrotic syndrome, causes severe side effects. One of these side effects is ocular hypertension, which can result in severe and permanent visual disturbance. However, the exact prevalence, severity and timing of development of ocular hypertension have yet to be fully explored in this pediatric patient group. Methods: In this retrospective cohort study, children with nephrotic syndrome treated with prednisolone for their first episode were analyzed. Intraocular pressure was screened with an iCare® tonometer and confirmed with Goldmann applanation tonometry before the initiation of prednisolone treatment and at 1 and 4 weeks thereafter. Results: A total of 26 children with nephrotic syndrome were included in this study, of whom eight (30.8 %) required treatment with eye drops for ocular hypertension. The median time interval between the diagnosis of ocular hypertension and start of treatment was 9 (range 5-31) days. At relapse of nephrotic syndrome, all children who had undergone treatment for ocular hypertension in their first episode again required treatment for ocular hypertension. Conclusions: Routine ophthalmologic examination should be conducted from the early phase after the start of prednisolone treatment. In addition, children with episodes of ocular hypertension may be at greater risk of its reappearance with relapse of the nephrotic syndrome. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to analyze the radiological outcomes of bipolar hemiarthroplasty after displaced femoral neck fractures of non-arthritic hip joints in rheumatoid arthritis patients. Methods: We retrospectively investigated 25 hip joints in 23 rheumatoid arthritis patients who underwent bipolar hemiarthroplasty for displaced femoral neck fracture of non-arthritic hip joints. All patients were female with an average age of 69.8 years (range 51-83 years). Mean follow-up duration was 8.4 years (range 5-12 years). Radiographs taken immediately, 1 year after surgery and most recently, were collected for each case. Radiographic measurement of the migration distance of the outer-head prosthesis in the direction of vertical, horizontal and medial to Köhler's line was undertaken at 1 year after surgery and most recently. Results: No patients had hip-related pain after surgery. No case indicated apparent central migration and >3-mm migration of the hemisphere in each direction. There was no significant change in migration distance between evaluation at 1 year after surgery and most recently. Conclusions: We conclude that risk of acetabular protrusion appears to be low in patients of rheumatoid arthritis treated with bipolar hemiarthroplasty for displaced femoral neck fractures of non-arthritic hip joints in the medium term. [ABSTRACT FROM AUTHOR]

Study design:Retrospective chart review.Objective:To evaluate the clinical outcomes and factors influencing patient satisfaction with Malone antegrade continence enema (MACE) in patients with spina bifida.Setting:Japan.Methods:We performed retrospective analysis of 21 patients with spina bifida who underwent surgical creation of an MACE stoma. Clinical outcomes were evaluated by medical records, operative notes and mailed questionnaires. Patient satisfaction scores (SSs) were measured on a modified visual analog scale (VAS) from 1 to 10, and the factors influencing the SS were analyzed.Results:A 100% return rate for the mailed questionnaires was achieved. All patients underwent in situ appendicocecostomy with cecal plication. There was only one complication that required surgical revision. Regarding fecal continence, the overall success rate was 90%. Although 4 patients (19%) had severe irrigation pain and 4 patients (19%) found the washout time intolerably long, 18 (85%) of them were satisfied with the MACE procedure. Age at operation, experience of retrograde colonic enema (RCE), experience of stomal leakage, increased comfort at school or workplace and increased comfort at sleepovers significantly influenced SSs.Conclusion:MACE is a valuable option in achieving fecal continence in patients with spina bifida, with most patients being satisfied with the procedure. In our analysis, younger age at operation, previous experience of RCE, no stomal leakage and improvement of quality of life (enhanced comfort at school, workplace and sleepovers) significantly influenced the high satisfaction after MACE. [ABSTRACT FROM AUTHOR]

Purpose: Congestive heart failure (CHF) alters the pharmacokinetics of various drugs, including cardiovascular agents, due to decreased cardiac output and decreased renal blood flow. The purpose of this study was to evaluate the influence of CHF on the clearance of vancomycin, a glycopeptide antibacterial agent. Methods: After reviewing more than 1,500 clinical charts of patients who received vancomycin therapy and whose serum vancomycin level was monitored, we identified 101 patients who also had the left ventricular ejection fraction (LVEF) assessed at that time. The fluorescence polarization immunoassay method was used to measure vancomycin serum concentrations in these patients 1 h after the end of vancomycin infusion and just before the next administration. Using these two measurements, we calculated the pharmacokinetic parameters using the Bayesian estimator. Results: Patients with an LVEF of <40 % (16 patients) or those with an LVEF of ≥ 40 % and <60 % (40 % ≤ LVEF < 60 % ; 32 patients) had a significantly lower vancomycin clearance than patients with LVEF of ≥60 % (53 patients) (2.29 ± 0.95 or 2.79 ± 0.99 vs. 3.50 ± 1.04 L/h; p < 0.001 or p < 0.01, respectively). Vancomycin clearance was strongly correlated not only with estimated creatinine clearance (CLcr) in patients with an LVEF of <40 % ( r = 0.828) and 40 % ≤ LVEF < 60 % ( r = 0.773), but also with an LVEF in patients with a CLcr of <60 mL/min ( r = 0.646). Consistent with these findings, multiple regression analysis revealed that CLcr, LVEF and body weight were important independent variables for vancomycin clearance ( r = 0.649). Conclusions: Vancomycin clearance decreased with decreasing cardiac function (LVEF) and decreasing CLcr. This finding suggests that vancomycin clearance is affected by cardiac function and would be predicted not only CLcr but also by LVEF. [ABSTRACT FROM AUTHOR]

Introduction: At present, the risk of future hemorrhage or ischemic insult from vertebral artery (VA) dissection cannot be estimated from available imaging data. We investigated the relationship between symptoms and the angiographic patterns of the dissecting site on balloon test occlusion (BTO) to develop guidelines for clinical decision-making. Methods: We retrospectively reviewed 18 patients with unilateral VA dissection who presented with subarachnoid hemorrhage (SAH) or cerebral infarction. We analyzed the angiographic findings at the dissecting site on contralateral VA angiograms during BTO of the affected VA, classified the angiographic patterns into two types, and compared the symptoms they presented. Results: Patients with dissection opacified from the distal to the proximal side are more likely to present with cerebral infarction than SAH. Conversely, patients with dissection opacified from the proximal to the distal side had a significantly higher incidence of SAH. Conclusions: Angiographic findings at the dissecting site on contralateral VA angiograms during BTO of the affected VA are helpful for considering the treatment and prognosis of patients with VA dissecting aneurysms. [ABSTRACT FROM AUTHOR]

The management of Henoch-Schönlein purpura nephritis (HSPN) is controversial. It has been revealed that some patients develop end-stage renal disease and aggressive treatment with drugs such as steroids is increasing, and some of them may be overzealous. At our institutes, our treatment decisions are based on the clinical and pathological severity of the case in an attempt to limit the indications for aggressive therapies such as steroids and immunosuppressive agents. Here, we retrospectively examined the efficacy of treatment for HSPN. Renal biopsy was performed in patients with nephrotic syndrome or persistent proteinuria for more than 3 months and patients were classified by treatment. Patients ( n = 31) with moderately severe HSPN (histological grade I-III and serum albumin [Alb] >2.5 g/dl) were treated with angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers. Patients ( n = 19) with HSPN exceeding grade III or Alb ≤ 2.5 g/dl received combination therapy comprising prednisolone, immunosuppressants, warfarin, and dipyridamole. All patients showed resolution of proteinuria without renal dysfunction during the observation period (3.76 ± 0.37 years). Our findings support those of some earlier reports that treatment strategies for HSPN should depend on the histological and clinical severity. Furthermore, aggressive therapies, particularly combination therapies, are unnecessary for moderate-severe HSPN. [ABSTRACT FROM AUTHOR]

Study design:A retrospective, multicenter study.Objectives:To investigate the relationship between bowel and bladder management methods and symptomatic autonomic dysreflexia (AD) during hospitalization in patients with spinal cord injury (SCI).Setting:Twenty-eight Rosai hospitals in Japan.Methods:The study subjects were 571 patients with SCI who had been admitted to 28 Rosai hospitals between April 1997 and March 2007 for rehabilitation therapy and fulfilled the following criteria: (1) SCI at or above sixth thoracic level, (2) discharged from hospital after more than 4 months of admission for initial injury and (3) lack of pressure ulcers, deep venous thrombosis, ureteral and renal stones or heterotopic ossification throughout hospitalization to exclude possible influence of these complications on cardiovascular reflexes. The study subjects were examined for the incidence of symptomatic AD according to age, sex, ASIA Impairment Scale, injury level, bowel and bladder management techniques at discharge.Results:The Rosai Hospital registry included 3006 persons with SCI during 1997-2007, and 571 patients fulfilled the above criteria. The highest incidence of symptomatic AD was diagnosed in subjects using reflex voiding and in those using manual removal of stool. By contrast, the lowest incidence of symptomatic AD was in those on continent spontaneous voiding and continent spontaneous defecation.Conclusion:Medical staff should evaluate the presence of AD in patients with SCI at or above the T6 level under bladder and bowel management such as reflex voiding and manual removal of stool. [ABSTRACT FROM AUTHOR]

A nationwide survey was conducted for identifying ataxia-telangiectasia (AT) patients in Japan. Eighty-nine patients were diagnosed between 1971 and 2006. Detailed clinical and laboratory data of 64 patients including affected siblings were collected. Analyses focused on malignancy, therapy-related toxicity, infection, and hematological/immunological parameters. The phenotypic variability of AT was assessed by comparing 26 affected siblings from 13 families. Malignancy developed in 22% of the cases and was associated with a high rate of severe therapy-related complications: chemotherapy-related cardiac toxicity in 2 children, and severe hemorrhagic cystitis requiring surgery in 2 patients. The frequency of serious viral infections correlated with the T cell count. Hypogammaglobulinemia with hyper-IgM (HIGM) was recorded in 5 patients, and 3 patients developed panhypogammaglobulinemia. Differences in immunological parameters were noted in siblings. Four patients showed an HIGM phenotype, in contrast to their siblings with normal IgG and IgM levels. The patients with HIGM phenotype showed reduced levels of TRECs and CD27+CD20+ memory B cells. The findings suggest that hitherto unidentified modifier genes or exogenous environmental factors can influence the overall immune responses. Our data along with future prospective study will lead to better understanding of the hematological/immunological phenotypes and to better care of the patients. [ABSTRACT FROM AUTHOR]

Purpose: To determine the prevalence and progression of Barrett's epithelium and associated risk factors in Japan.Methods: The study population comprised 869 cases. Endoscopic Barrett's epithelium was diagnosed based on the Prague C & M Criteria. The correlations of clinical factors with the prevalence and progression of endoscopic Barrett's epithelium were examined.Results: Endoscopic Barrett's epithelium was diagnosed in 374 cases (43%), in the majority of which the diagnosis was short-segment Barrett's esophagus. The progression of Barrett's epithelium was identified in 47 cases. In univariate and multiple logistic regression analyses, aging, smoking habit, and erosive esophagitis were significantly associated with the prevalence of Barrett's epithelium, whereas aging and erosive esophagitis, especially severe erosive esophagitis, were significant contributing factors to the progression of Barrett's epithelium.Conclusions: Forty-three percent of the total study population was diagnosed as having endoscopic Barrett's epithelium. During the follow-up period, 12.6% of the cases with Barrett's epithelium exhibited progression which was associated with aging and severe erosive esophagitis. [ABSTRACT FROM AUTHOR]

Anemia in cancer patients has been under-recognized and little studied in Japan. To gain some insight into cancer-related anemia in Japanese patients undergoing outpatient chemotherapy, we performed a single-center retrospective study of the prevalence and incidence of anemia in 148 patients with solid tumors treated at the Kyoto University Hospital Outpatient Oncology Unit. We classified the cases of anemia in accordance with the National Cancer Institute Common Terminology Criteria for Adverse Events (version 3.0). Of 148 patients, 65 (44%) were anemic at the start of chemotherapy, 19 (13%) of whom had anemia of grade 2 or higher. Chemotherapy further increased the number of anemic patients, with 125 (84%) being anemic at some point during chemotherapy, and 61 (41%) of these having anemia of grade 2 or higher. Among the 83 patients without anemia at the start of chemotherapy, 60 (72%) developed anemia during chemotherapy, 15 (18%) of whom had anemia of grade 2 or higher. This is the first report showing a high prevalence and incidence of anemia in Japanese patients undergoing outpatient chemotherapy. Better recognition and management of cancer-related anemia are required in Japan. To this end, randomized controlled trials evaluating the effects of erythropoietic agents on patients' survival and quality of life are necessary. [ABSTRACT FROM AUTHOR]

Bacterial infection is one of the most important causes of morbidity and mortality after unrelated cord blood transplantation (CBT). In the present study, we studied 101 adult patients with respect to the incidence, outcome, and risk factors for bacterial bloodstream infection (BSI) within 30 days after CBT using a myeloablative conditioning regimen. Bacterial BSI occurred in 12 patients within 30 days after CBT. The cumulative incidence of bacterial BSI was 12%. The median time of onset was day +6 (range, day -1 to day +13) after CBT. In all patients, the neutrophil count was 0/microL at the onset of bacterial BSI. Eight (67%) and 4 (33%) of the isolates were Gram-positive and Gram-negative bacteria, respectively. Only 2 (17%) of the 12 patients who had bacterial BSI died within 100 days after CBT. No risk factors for the occurrence of bacterial BSI within 30 days after CBT were identified. The low mortality rate for bacterial BSI in the neutropenic period appeared to be associated with the low incidence (6%) of transplantation-related death at day +100 in our study patients. Early diagnosis of bacterial BSI and prompt treatment with effective antibiotics are necessary for neutropenic adult patients after myeloablative CBT. [ABSTRACT FROM AUTHOR]

To clarify the clinical significance of a serologic HLA-DR mismatch after unrelated-donor transplantation, we evaluated for hematologic malignancies 123 cases of unrelated bone marrow transplantation carried out in a single institution between 1995 and 2004. Of the patients in these cases, 12 were serologically mismatched at the single HLA-DR locus. Eighty-two patients who received HLA-matched transplantations were used as controls. Conditioning consisted of a conventional total body irradiation-based regimen or a fludarabine-based reduced-intensity regimen. Prophylaxis for graft-versus-host disease (GVHD) consisted of tacrolimus plus short-term methotrexate. Graft failure did not develop. With a median follow-up of 42 months (range, 11-99 months), rates of overall survival, nonrelapse mortality, and relapse at 4 years were 63%, 38%, and 0%, respectively, all of which were comparable with those after HLA-matched transplantation. The frequency of acute GVHD of grades II to IV was 75%, significantly higher than after HLA-matched transplantation (42%, P = .046), and there was a trend toward an increased incidence of acute GVHD of grades III to IV after serologically HLA-DR-mismatched unrelated transplantation (27% versus 10%, P = .093). Chronic GVHD developed in 4 of 11 evaluable patients, an incidence comparable with that after HLA-matched transplantation. In summary, serologically HLA-DR-mismatched unrelated transplantation is feasible and might be an acceptable alternative for the Japanese population, although the higher incidence of acute GVHD is notable. [ABSTRACT FROM AUTHOR]

We conducted the first nationwide survey to clarify the clinical features, treatment methods, and prognoses for polycythemia vera (PV) and essential thrombocythemia (ET). A 1-page questionnaire was mailed to members of the Japanese Elderly Leukemia and Lymphoma Study Group (JELLSG). Surveys on 647 patients (PV, 266 patients; ET, 381 patients) were returned and analyzed. Thrombotic events at diagnosis and during follow-up occurred at rates of 15.4% and 8.5%, respectively, in PV cases and 17.6% and 8.7% in ET cases. Splenomegaly was observed in only 28.8% of PV patients and 10.8% of ET patients. The leukocyte alkaline phosphatase score was elevated in only 46.2% of PV patients. The incidences of abnormal karyotypes were less than 10% in both PV and ET cases. The rates of transformation to myelofibrosis were 2.6% in both PV and ET cases, and acute leukemia was noted in 1.1% of PV patients and 2.9% of ET patients. Prognostic factors were thrombotic history for PV and thrombotic history and age (>or=60 years) for ET. The present study clearly demonstrated clinical differences between Japanese and Western patients for PV and ET. Concerning the treatment of PV and ET, the study revealed considerable variation among Japanese hematologists. These results suggest the necessity of developing treatment guidelines according to risk stratification that are suitable for Japanese PV and ET patients. [ABSTRACT FROM AUTHOR]

A retrospective study was performed to determine the prevalence of Helicobacter pylori (H pylori) infection, the effect of H pylori eradication on platelet counts, and the characteristic clinical features of chronic immune or idiopathic thrombocytopenic purpura (ITP) with H pylori infection. H pylori infection was found in 300 patients, a group that was significantly older (P < .005) and had more cases of hyperplastic megakaryocytes in the bone marrow (P = .01) than patients without H pylori infection. H pylori eradication therapy was performed in 207 H pylori-positive ITP cases, and the platelet count response was observed in 63% of the successful eradication group and in 33% of the unsuccessful eradication group (P < .005). In the successful group, the complete remission and partial remission rates were 23% and 42%, respectively, 12 months after eradication. In the majority of responders, the platelet count response occurred 1 month after eradication therapy, and the increased platelet count continued without ITP treatment for more than 12 months. H pylori eradication therapy was effective even in refractory cases, which were unresponsive to splenectomy. In conclusion, H pylori infection was involved in most ITP patients older than 40 years in Japan, and eradication therapy should be the first line of treatment in H pylori-positive ITP patients. [ABSTRACT FROM AUTHOR]

Background: Although minimally invasive surgery has achieved worldwide acceptance in various fields, laparoscopic surgery for pancreatic diseases has been reported only rarely. The purpose of this study was to evaluate the outcomes and feasibility of laparoscopic pancreatic surgery.Methods: Fifteen patients, comprising eight men and seven women with an average age of 54 years, underwent laparoscopic pancreatic surgery. Distal pancreatectomy was indicated for solid tumors ( n = 4), cystic lesions ( n = 3), and chronic pancreatitis ( n = 2). Cystogastrostomy was performed for pseudocysts ( n = 4) and enucleation for insulinomas ( n = 2). The lesions varied in size from 1 to 9 cm (2.9 +/- 2.4 cm) and were located in the pancreatic head ( n = 2), body ( n = 3), or tail ( n = 10). For distal pancreatectomy, the splenic artery was divided and the parenchyma was transected with a linear stapler. Laparoscopic ultrasonography was used to determine the distance between the tumor and the main pancreatic duct for enucleation as well as to localize the lesion for distal pancreatectomy. Cystogastrostomy, 4.5 cm in length, was also performed with the linear stapler through the window of the lesser omentum.Results: Mean operation time was 249 +/- 70 min (293 +/- 58 min in distal pancreatectomy, 185 +/- 14 min in enucleation, 204 +/- 50 min in cystogastrostomy), and mean blood loss was 138 +/- 184 g (213 +/- 227 g, 75 +/- 35 g, 38 +/- 48 g, respectively). Two distal pancreatectomies (13%) were converted to open surgery due to severe peripancreatic inflammation. There was no related mortality, but there were two cases (15%) of pancreatic fistula, one in a distal pancreatectomy case and the other in an enucleation case, and both were treated conservatively.Conclusions: Laparoscopic pancreatic surgery is safe and feasible for patients with benign tumors and cystic lesions. [ABSTRACT FROM AUTHOR]

Unlabelled: Despite intravenous immunoglobulin therapy, a certain percentage of patients with Kawasaki disease (KD) still develop coronary arterial lesions (CAL). In an effort to find new combined therapies to reduce the incidence of CAL, we focused on the oedema which can be an important sign of the increased vascular permeability in KD. A total of 127 patients with KD were included in the retrospective study. Serial weekly changes in serum sodium and albumin levels from the 1st to the 4th week of illness were examined. In addition, the maximum rate of increase in body weight from admission to the 14th day of illness was evaluated. Serum sodium levels (mEq/l) in only the 2nd week of illness were significantly lower in patients with CAL than in those without CAL (mean +/- SD, 135.5+/-4.5 versus 138.0+/-2.4, P<0.05). Serum albumin levels in all 4 weeks were significantly lower in patients with CAL than in those without CAL ( P<0.001). The maximum rate (%) of increase in body weight from admission to the 14th day of illness was significantly higher in patients with CAL than in those without CAL (ranges and median values, 0-12.3 (7.0) versus 0-10.3 (3.2), P<0.001).Conclusion: these results suggest that water retention in the acute phase of Kawasaki disease may be a risk factor for CAL, and water intake of both infusion and oral intake should be kept to a minimum in order to avoid progressive oedema. [ABSTRACT FROM AUTHOR]

Purpose: The Japan Adult Leukemia Study Group (JALSG) AML201 protocols are regimens for remission induction and consolidation chemotherapy of acute myeloid leukemia (AML) and have been widely accepted in Japan since 2001. Management of infectious complications during chemotherapy has a key role in the supportive care of AML patients.Methods: By using case report forms collected in December 2001 and December 2005, we retrospectively analyzed the infectious complications in adult patients treated by using the JALSG AML201 protocols against AML (excluding promyelocytic leukemia).Results: Of 980 patients, 80.2% experienced febrile neutropenia (FN), 8.3% bacteremia/fungemia, and 10.3% pulmonary infection at least once during remission-induction chemotherapy. Gram-positive bacteremia accounted for 65.1% of bacteremia/fungemia in 2001-2005, compared with 38.2% in 1987-1991 and 45.9% in 1992-1995. Of 750 patients, 81.9% experienced FN, 21.9% bacteremia/fungemia, and 9.1% pulmonary infection at least once during consolidation chemotherapy. During consolidation chemotherapy, bacteremia/fungemia and pulmonary infection were significantly more frequent in the high-dose cytarabine (HDAC) arm than in the conventional multiagent arm (25.9 vs. 17.9% and 12.7 vs. 7.7%, respectively). Invasive pulmonary aspergillosis accounted for 15.8% of pulmonary infections during remission induction and 19.7% during consolidation chemotherapy.Conclusions: Our data suggest that patterns of infectious complications have changed between 1987 and 2005, possibly because of chemoprophylaxis with oral fluoroquinolones and improved diagnosis of invasive pulmonary aspergillosis by serum antigen analysis. [ABSTRACT FROM AUTHOR]