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The Ministry of Health, Labor and Welfare mandated the creation of the business continuity plan (BCP) for disaster key hospitals on March 31, 2017. Supposing the hospital information system (HIS) failure occurred, the picture archiving and communication system (PACS) also suffers obstacles, we assumed building a new network was necessary for radiological examination images. The purpose of this study was to investigate whether building a new network for radiological examination images is necessary in an emergency. Using wireless fidelity (Wi-Fi), the new network consisting of one image server and two tablet terminals A and B was constructed. The study measured the portable image transfer time for various stages of the network. The results were as follows: Transfer time from the mobile X-ray unit to the image server was 4.12±0.86 s, that from the image server to the tablet device A was 5.14±0.71 s, and that from the image server to the tablet device B was 7.32±1.66 s. Therefore, the new network configuration can provide a reliable means of accessing radiological images during emergency situations when the HIS and PACS may experience obstacles or failures.

The patient was a 21-year-old man who had been diagnosed with Crohn's disease and received infliximab and azathioprine six years earlier. He was admitted with fever and fatigue. Peripheral blood examination showed LDH 2,473 U/l and thrombocytopenia, and contrast-enhanced computed tomography (CT) showed hepatosplenomegaly. Bone marrow biopsy and liver biopsy showed CD4+CD56+TCRγδ＋CD8 - atypical cells, leading to a diagnosis of hepatosplenic T-cell lymphoma (HSTCL). The patient was refractory to CHOP and DA-EPOCH, and therefore received cord blood transplantation with myeloablative conditioning. CT showed reduced in hepatosplenomegaly and peripheral blood examination showed LDH 165 U/l and plt 180,000/µl, so the patient was discharged on day117. HSTCL is a tumor of immature γδT cells with a Vδ1 mutation in the spleen, and immunodeficiency has been implicated in its pathogenesis. Patients with inflammatory bowel disease treated with azathioprine are known to have an increased risk of lymphoproliferative disease. In this case, use of immunosuppressive drugs for Crohn's disease may have caused malignant transformation of γδ cells in the intestinal epithelium. Although the patient was refractory to chemotherapy, he was able to achieve remission with early cord blood transplantation and long-term survival is expected.

Previous reports have shown that a low attenuated area(LAA)in chest CT reflects the prognosis or therapeutic outcomes for chronic obstructive pulmonary disease(COPD). COPD has been reported as one of the important predictive factors for postoperative pulmonary complications(PPCs). In this retrospective study, we analyzed 100 patients who underwent surgery for gastric or colorectal cancer between January 2020 and August 2022, and investigated the impact of the LAA volume ratio(LAAv%)on short term surgical outcomes. Twenty-two cases developed PPCs, and their Clavien-Dindo grades were Grade Ⅰ-Ⅱ(for 21 patients)and Grade Ⅳa(in 1). We found that high-LAAv% and high-BMI were independent risk factors of PPCs in uni- and multi-variate analyses. In a sub analysis of the limited high-LAAv% group, surgical time qA found to be an independent risk factor of PPCs, and it's cut-off value was calculated as 189 minutes(sensitivity 88.2%, specificity 42.4%). The incidence of PPCs might be avoidable in patients with high-LAAv% through selection of the surgical approach or applying respiratory function training when appropriate.

Haploidentical allogeneic hematopoietic stem cell transplantation from her brother was performed on a 41-year-old lady with no prior history of pemphigoid to treat recurrent AML. On day 59 following transplantation, she experienced esophageal stenosis. During immunosuppressive therapy for graft vs. host disease, this condition was controlled with periodic esophageal dilatation (GVHD). Her esophageal stricture, which required periodic dilatation, grew worse after she stopped immunosuppressive therapy because of recurrent AML. The esophageal mucosa was easily hemorrhagic and desquamative. Histologic analysis revealed that the squamous cell layers had been divided. Indirect immunofluorescence was negative for IgG and positive for IgA on the epidermal layers, while direct immunofluorescence showed a linear deposition of IgG on the basement membrane zone. It was determined through immunoblotting utilizing recombinant protein of BP180 C-terminal domain that both IgG and IgA antibodies were present, supporting the diagnosis of mucous membrane pemphigoid with anti-BP180. After allogeneic transplantation, basal epidermal cell destruction by GVHD may result in autoimmune blistering disorders, which expose basement membrane proteins and antigen presentation. A similar mechanism could apply to our situation. For rare GVHD cases, a thorough histological diagnosis is required.

We investigated the cytotoxicity on a mast cell line (C57 cells) of water-soluble extracts of coarse (>3 µm, PM >3 ) and fine (0.05-3 µm, PM 0.05-3 ) atmospheric particulates collected from April 2016 to March 2019 in Fukuoka, Japan. We examined the direct cytotoxicity with punched-out membrane filter fragments of PM >3 and PM 0.05-3 collected from April 2019 to March 2021, without extraction of the components. Also, cell proliferation and degranulation assays were conducted under conditions which caused no cytotoxicity with water-soluble extracts of PM >3 from FY2016 and PM >3 direct samples from FY2019. The findings revealed the significant direct cytotoxicity of many PM >3 and all PM 0.05-3 samples, with higher cytotoxicity for PM 0.05-3 (FY2019-2020). These results were different from the cytotoxicity effects of water-soluble extracts of PM >3 and PM 0.05-3 samples (FY2016) in previous studies. In addition, inhibition of cell proliferation and induction of degranulation were significantly induced in a few PM >3 samples, showing a correlation with the suspended particulate matter (SPM) concentrations. This method using punched-out membrane filters is convenient and useful for assessing the direct effects of atmospheric particles on a small scale.

We report the case of an 82-year-old male with subacute sensorimotor neuropathy associated with Epstein-Barr virus (EBV) infection, who presented with diplopia followed by gait disturbance due to limb weakness. Pathological findings of a biopsied cervical lymph node showed a high frequency of EBV-positive cells. EBV-DNA was detected in blood. A nerve conduction study suggested a mixture of axonal damage and demyelination. Brain MRI showed multiple microbleeds in cerebellar cortices, but cerebrospinal fluid EBV-PCR was negative, suggesting bleeding due to EBV-related vasculitis. Corticosteroid therapy improved the neurological symptoms and the patient was able to walk independently four months later. The main pathogenesis of the neuropathy in this case is likely to be a mixture of vasculitic neuropathy and immune-mediated demyelinating neuropathy, which are considered to be due to EBV reactivation.

A 62-year-old woman was presented at our hospital with visual disturbance. An ocular examination revealed bilateral Roth spots. Laboratory data revealed leukocytosis (236,200 µl) with an excess blast (11%). Physical examination and computed tomography (CT) showed systemic lymphadenopathy. A bone marrow examination revealed a composition of 9.2% blast. Chromosomal analysis on bone marrow cells revealed 46,XX,t (3;12)(q26.2;p13),t (9;22)(q34.1;q11.2) in 80% of metaphases (16/20). Inguinal lymph node biopsy revealed diffuse proliferation of myeloperoxidase (MPO)-positive abnormal cells. Fluorescence in situ hybridization analysis was used to detect the BCR-ABL1 fusion gene and split the signals of MECOM and ETV6. She was diagnosed with de-novo chronic myeloid leukemia (CML) extramedullary blast crisis. She received tyrosine kinase inhibitor (TKI) combination chemotherapy and allogeneic hematopoietic stem cell transplantation and achieved a major molecular response. In this study, we reported a case of CML in blast-phase initially presenting as extramedullary, in which cytogenetic and molecular analyses were useful in the staging method.

In 1993, a mutation in the superoxyde dismutase gene, SOD1, was found causative for familial ALS, suggesting that free radical-related injury may be involved in ALS pathogenesis. Therefore, clinical trials were conducted with ALS patients using a free radical scavenger, edaravone, which was already approved for acute phase treatment of cerebral infarction in Japan. Because edaravone showed a therapeutic effect in suppressing the progression of ALS symptoms, it was approved as a new therapeutic agent in Japan, in June, 2015. In this article, we discuss the recent progress of basic and clinical research for the development of new ALS treatments.

Fanconi anemia (FA) is a genetic disorder characterized by progressive bone marrow failure, increased susceptibility to leukemia and cancer, and genomic instabilities. Protein products encoded by 22 FA genes, identified till date, cooperate in a molecular pathway called the FA pathway to repair DNA interstrand cross-links induced by chemotherapeutic agents, such as mitomycin C and cisplatin. An accumulating number of studies have shown several new functional aspects of the FA pathway, particularly in the context of the pathogenesis of bone marrow failure. This review focuses on the following topics: (1) aldehydes as intrinsic interstrand cross-linkers; (2) cytokine-induced hematopoietic stress; (3) increased transforming growth factor-β signaling; (4) mitochondrial functions of FA proteins. These findings are expected to offer new therapeutic opportunities for bone marrow failure in FA.

A 54-year-old female complained of oral erosion. A flaccid blister appeared on the trunk 2 months after the onset. The high titer of the anti-desmoglein 1 antibody in the absence of Nikolsky's sign led to the diagnosis of pemphigus vulgaris. The lymphadenopathy in the mesenteric and para-aortic regions indicated the possibility of paraneoplastic pemphigus. The steroid pulse therapy and therapeutic plasma exchange were ineffective. As CT-guided intraperitoneal lymph node biopsy revealed follicular lymphoma, R-CHOP therapy was performed. Although partial remission was attained accompanied by an improvement in the skin and mucosal findings after four courses of R-CHOP therapy, an occlusive ventilatory disturbance, possibly attributed to bronchiolitis obliterans, appeared 4 months after the treatment initiation. Although the treatment with tacrolimus was attempted, it was not feasible to be continued because of opportunistic infection, and the patient died 9 months after the onset of the skin lesion. Although specific anti-plakin antibodies were negative, this case was diagnosed as paraneoplastic pemphigus due to follicular lymphoma and complicated by obstructive bronchiolitis based on the clinical findings. The accumulation of similar cases is needed to establish effective treatment strategies.

In recent years, the appearance of MALDI-TOF MS made it possible to identify bacterial species in a short time. However, the sensitivity test has not largely shortened the time. When ESBL is suspected by routine examination, we speculate that there are many institutions conducting the inspection method conforming to the CLSI inspection procedure. When carrying out bacteria identification, susceptibility test and ESBL confirmation test from blood culture-positive specimens, the expected examination days are required for about 3 to 4 days, which is not quick. We report on bacterial species identification by MALDI-TOF MS from blood culture positive specimen bottle and ESBL rapid decision with fully automated urine analyzer.

The concept of "watershed shift"(WS)has been proposed as a cause of the ischemic complications following a superficial temporal artery-middle cerebral artery(STA-MCA)bypass operation performed for the management of moyamoya disease. Previous reports have observed that only 1.2-5.7% of the patients who underwent a bypass operation for the management of moyamoya disease developed cerebral infarction secondary to the WS phenomenon. To date, the WS phenomenon has not been objectively proven on imaging studies. We describe a 39-year-old woman who presented with right facial palsy and aphasia. Magnetic resonance imaging revealed cerebral infarction in the left frontal lobe secondary to moyamoya disease. Three days after undergoing the left STA-MCA bypass procedure, she showed deterioration in aphasia secondary to the occurrence of cerebral hyperperfusion syndrome(CHPS). Diffusion-weighted imaging(DWI)performed on postoperative day(POD)1 and 5 showed no area of high signal intensity. DWI performed on POD 8 showed an area of high signal intensity in the deep white matter of the left parietal lobe outside the range of the craniotomy. Postoperative fusion images of computed tomography angiography and DWI performed on POD 8 showed that the blood flow through the MCA from the bypass graft and that through the posterior cerebral artery crossed each other at the surface of the subcortical infarction. In the present case, the WS could be directly confirmed on imaging studies, and the cerebral infarction may have occurred secondary to WS concomitant with CHPS. Clinicians need to be aware of the WS phenomenon even after performing a direct bypass to treat adults with moyamoya disease.

Objectives　In Vietnam, the number of patients with non-communicable diseases (NCDs) has been increasing in recent years in association with the country's remarkable economic growth and corresponding changes in its population's lifestyle. The purposes of this research were to identify the challenges in the prevention and control of NCDs in Vietnam and to discuss countermeasures for NCDs in Vietnam and Japan.Methods　As a 2015 Regional Public Health Overall Promotion Project, an investigation team consisting of 11 public health physicians visited Hanoi, the capital of Vietnam, and its vicinities from January 11, 2016 to January 15, 2016. In Hanoi and its vicinities, we visited local healthcare institutions, such as the World Health Organization(WHO) Representative Office in Vietnam and Ministry of Health of Vietnam, and discussed the prevention and control of NCDs in Vietnam and Japan.Results　According to a survey in 2014, 73% of people of all age groups in Vietnam died from NCDs and the number of people suffering from NCDs has been sharply increasing in recent years. Major behavioral risk factors are dietary risks, tobacco smoke, alcohol use, and physical inactivity. There are four main problems with prevention and control of NCDs: 1) low awareness among the people of NCDs, 2) regional disparity of medical services, 3) shortage of healthcare staff members with professional knowledge, and 4) poor NCD surveillance.　In Vietnam, an NCD program with screening methods and medical guidelines for respective diseases was developed in 2002. However, it only covered tertiary prevention and did not fully describe the primary and secondary prevention measures. Currently, with the technical assistance of the WHO, the implementation of countermeasures emphasizing prevention and control to reduce NCD risk factors has only just begun.Conclusion　It was considered that educating each person in Vietnam on NCD prevention measures would be necessary and that a national policy, like Healthy Japan 21 of Japan, and a nationwide screening project, such as specific medical checkups, could serve as a useful reference. We found that public health activities in Japan to penetrate a region mainly involving public health nurses had played important roles for Japanese people's health. Furthermore, Japan shares with Vietnam the challenges including the shortage of human resources, and therefore, the securement of healthcare staff members who confront health challenges and the enhancement of their abilities is required.

Neuropathic pain associated with cancer, diabetic neuropathy, and postherpetic neuralgia is a type of intractable chronic pain characterized by mechanical allodynia and abnormal pain hypersensitivity evoked by innocuous stimuli. However, this disorder has no specific treatment. We previously showed that the purinergic receptor P2X4 (P2X4R), a subtype of ATP-gated nonselective cation channels, is highly upregulated in spinal microglia after peripheral nerve injury, and blocking the function of P2X4R reverses mechanical allodynia. In the present study, we screened a chemical library of 1979 clinically approved compounds (a gift from the Drug Discovery Initiative at the University of Tokyo) aimed at achieving "Eco-Pharma," which refers to seeking new effects of existing drugs. We demonstrated that duloxetine, a serotonin and noradrenaline reuptake inhibitor, has an inhibitory effect on rat and human P2X4R. In rat primary cultured microglial cells, duloxetine also inhibited P2X4R-mediated responses. Moreover, intrathecal administration of duloxetine in a model of neuropathic pain reversed nerve injury-induced mechanical allodynia. Based on those results, we suggest that the inhibition of P2X4R expressed in microglial cells may be involved in the antiallodynic effect of duloxetine in neuropathic pain. Furthermore, in this review, we discuss a new strategy for drug discovery called "Green Pharma" (a merger of "Eco-Pharma" and "Green chemistry" and referring to the development of eco-friendly pharmaceuticals).

Identification of bacteria of sepsis or bacteremia is a useful result for treatment policy. In recent years, bacterial identification has become possible from blood culture bottles by MALDI‒TOF, but it is not as accurate as bacterial identification from agar colonies. Blood culture pretreatment kit (MALDI Sepcityper Kit) is currently on sale from Bruker. However, the current situation has not reached good accuracy. This time, a new blood culture pretreatment kit appeared, so I studied. Up to now, the blood culture pretreatment kit was only MALDI Sepcityper Kit using enzyme digestion method. Rapid BAC pro (Nittobo) is a pretreatment kit using nanomaterials. This time, comparison examination (total number 40 samples) was done. Among them, 33 specimens were identified by MALDI Sepcityper Kit. There were 21 specimens that could be identified by rapid BAC pro. In this study, rapid BAC pro did not show superior results over MALDI Sepcityper Kit.

A 47-year-old woman was transferred to our hospital in June 2014 in hemorrhagic shock due to rupture of a huge right renal angiomyolipoma (AML). Selective right renal arterial embolization performed that same day reversed the shock immediately. Despite the huge abdominal tumor, the patient was discharged 2 weeks later after refusing any further treatment.Two weeks later she noticed the abdominal tumor growing. One month after discharge, she was readmitted due to dyspnea caused by restriction of her breathing by the growing tumor mass. A CT revealed a massive increase in tumor size with internal liquefaction, a thrombus in the left common iliac vein, and a 12 mm aneurysm in the right renal artery. The patient requested removal of the abdominal tumor since her ADL had deteriorated. We decided to perform a right nephrectomy with consideration of the left common iliac vein thrombus and right renal arterial aneurysm.As a precaution against pulmonary embolism in case the left common iliac vein thrombus dislodged, a retrievable inferior vena cava (IVC) filter was inserted before surgery. We were also concerned about possible rupture of the right renal aneurysm, so the right renal artery was embolized before surgery. After these procedures, a right nephrectomy was performed via a transperitoneal approach.The surgery was uneventful. The tumor weighed about 11 kg including 7,000 mL of bloody fluid. The IVC filter was removed the day after surgery, but the thrombus in the left common iliac vein remained, and an anticoagulant was started. Three months later, the thrombus had disappeared, and the anticoagulant was discontinued six months after surgery.According to the treatment guidelines for deep vein thrombosis, anticoagulants are the drugs of choice. IVC filters are seldom used to prevent pulmonary embolism. We initially administered an anticoagulant for the thrombus in the left iliac vein. However, an increase in abdominal tumor size suggested the drug had caused internal rebleeding and it had to be discontinued. Ultimately, we used a temporary retrievable IVC filter during the right nephrectomy with success.There is currently no consensus on when to use an IVC filter. Moreover, very little data exists on the use of an IVC filter during the perioperative period. Therefore, given the risk of potential thromboembolism, although we were able to use it successfully in our surgery, it should not be employed without a thorough benefit-risk assessment.

A 74-year-old man with hepatitis C virus (HCV)-related cirrhosis was admitted because of dyspnea. Laboratory investigations revealed severe proteinuria (4.0g/day), low serum albumin level, and cryoglobulinemia. Computed tomography showed massive pleural effusion and ascites. Because these effusions were leaky and the hepatic reserve was relatively intact, we thought these were mainly caused by nephrotic syndrome. Renal biopsy revealed membranoproliferative glomerulonephritis with mesangial proliferation and excessive matrix deposition. Based on these histopathological findings and the presence of cryoglobulinemia, a diagnosis of HCV-related nephropathy was performed. Therefore, antiviral therapy was initiated with direct-acting antiviral (DAA) agents (daclatasvir+asunaprevir). Serum HCV-RNA level was observed to be negative at week 8, which was followed by an alleviation of proteinuria and a gradual decrease in the pleural effusion and ascites. HCV-related nephropathy should be considered in the differential diagnosis of patients with chronic hepatitis C and refractory ascites. DAA agents are effective in the treatment of these patients.

A 51-year-old woman complaining of weakness in the limbs was diagnosed as having a duodenal gastrinoma on performing a further evaluation. Surgical resection was performed with selective arterial calcium injection for localization. During preoperative hospitalization, she experienced recurrent severe vomiting and diarrhea after endoscopy, leading to acute kidney injury. To our knowledge, this is the first report of gastrinoma with post-endoscopy symptom exacerbation. Although the etiology is unknown, the findings in this case suggest that sufficient fluid replacement, sedation, and high-dose proton pump inhibitor administration should be taken into consideration when performing endoscopy in gastrinoma patients.

Although immunoglobulin light chain (LC) plays a critical role in AL amyloidosis, detailed characteristics of deposited LC are not well known. In this study, LC peptides were analyzed by a combination of laser microdissection, liquid chromatography, and mass spectrometry (LC-MS/MS) in 65 patients with AL amyloidosis. Constant regions of LC were detected in all patients. Kappa- or lambda-types were detected in 20 and 45 patients, respectively. Various types of constant regions of LC were found; however, IGLC3 and IGKC4 were the most frequently detected. Besides LC, apolipoproteins and vitronectin were repeatedly found in amyloid lesions. These results suggest marked heterogeneity in terms of subtype of constant regions of LC in amyloid lesions. Immunohistochemistry identified LC in approximately half of patients in whom LC was detected by LC-MS/MS. This finding indicates superiority of LC-MS/MS over IHC for the detection of LC.

Kumamoto Earthquakes in 2016 severely affected medical circumstances and condition of each patient with neuro-muscular diseases, in addition to having destroyed life circumstances of local residence. Number of neuro-muscular disease patients admitted to the Department of Neurology, Kumamoto University, the only university hospital in the prefecture, increased approximately twice compared to usual years. Most of the related facilities were able to admit emergency patients with neuro-muscular diseases although the hospital buildings were damaged in various degrees. A number of issues remained unsolved as to emergency contact system, securement of emergency beds for severe neuro-muscular diseases, and information system for these patients.

Unlabelled: We report the treatment outcomes of 5 cases of adult-onset Ewing sarcoma(ES)managed between 2011 and 2014. We examined prognostic factors including the primary lesion, tumor size, metastatic status, and serum LDH levels., Results: The locations of the primary lesions included the limbs in 1 case and the trunk in 4; the cases in the trunk had a worse prognosis than that in the limbs. Tumor size was greater than 8 cm in only 1 patient, who also displayed evidence of metastases at presentation and high LDH levels. All the patients received chemotherapy consisting of alternating vincristine, doxorubicin, and cyclophosphamide(VDC)and etoposide and ifosfamide(IE). Surgery was selected for the treatment of 4 patients, and radiotherapy was administered to 1 patient for local treatment of the tumor. A median follow-up duration of 31.6 months revealed the 2-year overall survival rate and progression-free survival rate to be 80.0%., Conclusions: The prognosis of patients with adult-onset ES is poor; however, combined modality therapy, including VDC-IE, was demonstrated to improve the outcome of patients in the present study. Nevertheless, the patient with tumor size exceeding 8 cm, metastasis, and high LDH levels, relapsed 1 year after treatment, as reported previously. Further investigation is required to clarify the factors affecting prognosis in adults, and to develop effective therapies for patients with a poor prognosis.

Liver cancer is classified into primary and metastatic liver cancer. In Japan, hepatocellular carcinoma (HCC) accounts for about 95% and intrahepatic cholangiocarcinoma for about 4% of primary liver cancer. Recently, the prevalence of and mortality due to liver cancer have been rapidly decreasing in Japan. The main etiology of HCC is chronic infection of hepatitis B virus (HBV) and hepatitis C virus (HCV). However, the rate of these viral infections is decreasing, but the rate of HCC caused by non-alcoholic steato- hepatitis (NASH) is gradually increasing. The characteristic pathogeneses are multi-centric carcinogenesis, intrahepatic metastasis, and multi-step carcinogenesis. These pathogeneses are related to high rates of recurrence of HCC even after curative treatment. Clinical symptoms of HCC are rare in Japan because most patients with HCC are diagnosed by surveillance or incidental imaging analysis. HCC is diagnosed by not pathology but also classical images defined as showing early staining during the arterial phase and hypoattenuation compared with the surrounding non-tumorous liver during the portal or equilibrium phase (washout) on dynamic CT or MR. Three tumor markers: a-fetoprotein (AFP), protein induced by vitamin K absence or antagonist-II (PIVKA-II), and LCA-reactive a-fetoprotein isoform (AFP-L3), support the diagnosis. The surveillance of high-risk groups is recommended in Japanese Clinical Practice Guidelines for Hepatocellular Carcinoma 2013. Treatments for HCC are selected by considering the tumor progression and hepatic reserve and consist of surgery(hepatic resection, liver transplantation), local ablation therapy, transarterial chemoembolization (TACE), chemotherapy (sorafenib, hepatic arterial infusion chemotherapy), and radiation therapy. The algorithm for treatment selection is presented in the Japanese Clinical Practice Guidelines for Hepatocellular Carcinoma 2013. [Review].

Echographic examination for leg vein thromboembolism was carried out in 123 patients scheduled for thoracic surgery. Preventive measures for thromboembolism were conducted after the risk assessment. Echography was done after surgery in 72 cases, most of which were cases of lung malignant tumors, and thromboembolism was detected in 4 cases. Thus, the incidence rate of venous thromboembolism was 5.6%( 4/72). There was no patients who developed pulmonary thromboembolism during the examination period, suggesting reasonable risk assessment and preventive measures in our procedure.

We herein describe a 2-year-old boy with severe congenital neutropenia (SCN) who was successfully treated with reduced-intensity bone marrow transplantation (HSCT). He had suffered recurrent episodes of bacterial pneumonia from 12 months of age, and was found to have severe neutropenia with white blood cell counts below 100/μl. The patient harbored a heterozygous missense mutation in ELANE exon 4 (p.Gln134Pro, NM_001972.2: c.401A>C). This was a novel mutation. Due to intractable pneumonia and severe persistent neutropenia, reduced-intensity HSCT was performed from an HLA-matched sibling donor. The preparative regimen consisted of melphalan, fludarabine, and 4 Gy of total body irradiation. Hematopoietic engraftment was rapidly obtained, i.e., by day +14, and complete donor chimerism was subsequently achieved. The lung complications observed pre-transplantation markedly improved after neutrophil recovery, i.e., by day +60. We concluded that HSCT is a useful treatment for SCN patients, especially for those at high risk of leukemic transformation. Fludarabine-based reduced-intensity HSCT may represent a safe and effective therapeutic option for patients with SCN who need HSCT even if they have intractable infectious complications.

Cell replacement therapy is attractive as a novel strategy for stroke patients. To realize this therapy, safer and more effective cell resources are now required. Since both embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs) can retain high replication competence and pluripotency when they differentiate into various kinds of cells, they are regarded as a promising cell source for cell replacement therapy. Recent progress includes the combination of novel transcriptional factors that can convert somatic cells to various kinds of mature neuronal cells and neural stem cells without requiring embryonic stem fate. In this paper, we would like to discuss the advantage, issues, and possibility of clinical application of these cells for cell replacement therapy for post-stroke patient.

Langerhans cell sarcoma (LCS) is an extremely rare neoplasm of Langerhans cell origin characterized by systemic involvement and a poor prognosis. There are, however, few reports of LCS with central nervous system involvement. We experienced a patient with LCS recurrence in the brain that appeared during systemic chemotherapy. The brains lesions eventually responded to total cranial irradiation. A 60-year-old female presented with systemic lymphadenopathy. LCS was diagnosed based on neck lymph node biopsy findings. Two cycles of ESHAP induced marked regression of her lymphadenopathy, but FDG-PET/CT scan revealed new lesions in the central nervous system and her disorientation gradually worsened. We administered 37.5 Gy of total cranial irradiation which improved her consciousness and shrank the brain tumors as demonstrated by MRI. The patient's clinical course indicates that radiation therapy may be effective for central nervous system involvement of LCS even if the lesion is resistant to systemic chemotherapy.

Neurolymphomatosis is a rare manifestation of malignant lymphoma. The involvement of peripheral nerves has mostly been described as dissemination of a systemic lymphoma. In contrast, primary peripheral nerve lymphoma is extremely rare. A 68-year-old man presented in January 2014 with a sensory disturbance in the left lower extremity. There were no obvious findings on MRI or CT that could account for his symptoms. After 1 year of symptomatic treatment, the patient was managed conservatively for an additional year. However, his symptoms worsened. FDG-PET/CT showed high FDG uptake in the left sciatic nerve. Biopsy of the lesion revealed diffuse large B cell lymphoma.

We report the anesthetic management of microlaryngeal surgery in children using tubeless total intravenous anesthesia (TIVA) without endotracheal intubation under spontaneous breathing. In 9 patients (median age : 4.9 yr. range 1 months-14 years, body weight : 17 kg, range 3-61 kg), 19 procedures were performed with TIVA using propofol and remifentanil. The median time from the start of TIVA to rigid laryngoscope insertion was 11 minutes. Propofol 15.7 mg x kg(-1) x hr(-1) and remifentanil 0.05 μg x kg(-1) x min(-1) was infused in this interval. Laryngospasm was observed in two cases, but it responded to a bolus of propofol (0.5-1.0 mg x kg(-1)) and additional topical anesthesia with 1% lidocaine. Three children were found apneic after a bolus administration of remifentanil or after increasing the rate of remifentanil infusion accompanied with desaturation and their tracheae were intubated. The spontaneous respiration technique using TIVA without intubation provides an excellent view of the operative field while allowing stable anesthesia. Further studies are required to establish the optimal dose of propofol and remifentanil and the timing of rigid laryngoscope insertion.

An 84-year-old man was diagnosed with IgG4-related autoimmune pancreatitis and sclerosing cholangitis with jaundice. Endoscopic nasobiliary drainage was performed, but hemorrhagic shock due to multiple duodenal ulcers occurred about a week later. After several endoscopic hemostasis, he was given corticosteroids. Histopathology of duodenal ulcer biopsies showed IgG4-positive plasma cell infiltration. Reports about duodenal ulcers with IgG4-related disease are very rare and we consider this case valuable.