PubMed | Funabashi Municipal Medical Center, Hidaka General Hospital, Asahi General Hospital, Chiba Childrens Hospital and 23 more.
Type: Journal Article | Journal: PloS one | Year: 2016
Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+)/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+) release activated Ca(2+) (CRAC) channel mediating store-operated Ca(2+) entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+)/NFAT pathway in the pathogenesis of this disorder.
Suzuki H.,Wakayama Medical University |
Terai M.,Tokyo Women's Medical University |
Hamada H.,Tokyo Women's Medical University |
Honda T.,Tokyo Women's Medical University |
And 14 more authors.
Pediatric Infectious Disease Journal | Year: 2011
BACKGROUND: There are still no definite treatments for refractory Kawasaki disease (KD). In this pilot study, we evaluated the use of cyclosporin A (CyA) treatment in patients with refractory KD. METHODS: We prospectively collected clinical data of CyA treatment (4-8 mg/kg/d, oral administration) for refractory KD patients using the same protocol among several hospitals. Refractory KD is defined as the persistence or recurrence of fever (37.5°C or more of an axillary temperature) at the end of the second intravenous immunoglobulin (2 g/kg) following the initial one. RESULTS: Subjects were enrolled out of 329 KD patients who were admitted to our 8 hospitals between January 2008 and June 2010. Among a total of 28 patients of refractory KD treated with CyA, 18 (64.3%) responded promptly to be afebrile within 3 days and had decreased C-reactive protein levels, the other 4 became afebrile within 4 to 5 days. However, 6 patients (21.4%) failed to become afebrile within 5 days after the start of CyA and/or high fever returned after becoming afebrile within 5 days. Although hyperkalemia developed in 9 patients at 3 to 7 days after the start of CyA treatment, there were no serious adverse effects such as arrhythmias. Four patients (1.2%), 2 before and the other 2 after the start of CyA treatment, developed coronary arterial lesions. CONCLUSION: CyA treatment is considered safe and well tolerated, and a promising option for patients with refractory KD. Further investigations will be needed to clarify optimal dose, safety, and timing of CyA treatment. Copyright © 2011 by Lippincott Williams & Wilkins.
Wada T.,Kanazawa University |
Kanegane H.,University of Toyama |
Ohta K.,National Hospital Organization |
Katoh F.,Tokyo Women's Medical University |
And 9 more authors.
Cytokine | Year: 2014
X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency characterized by increased vulnerability to Epstein-Barr virus infection. XLP type 1 is caused by mutations in SH2D1A, whereas X-linked inhibitor of apoptosis (XIAP) encoded by XIAP/. BIRC4 is mutated in XLP type 2. In XIAP deficiency, hemophagocytic lymphohistiocytosis (HLH) occurs more frequently and recurrence is common. However, the underlying mechanisms remain mostly unknown. We describe the characteristics of the cytokine profiles of serum samples from 10 XIAP-deficient patients. The concentration of interleukin (IL)-18 was strikingly elevated in the patients presented with HLH, and remained high after the recovery from HLH although levels of other pro-inflammatory cytokines approached the normal range. Longitudinal examination of two patients demonstrated marked exacerbation of IL-18 levels during every occasion of HLH. These findings may suggest the association between HLH susceptibility and high serum IL-18 levels in XIAP deficiency. © 2013 Elsevier Ltd.
Yao K.,Fukuoka University |
Doyama H.,Ishikawa Prefectural Central Hospital |
Gotoda T.,Tokyo Medical University |
Ishikawa H.,Kyoto Prefectural University of Medicine |
And 6 more authors.
Gastric Cancer | Year: 2014
Background: Curative treatment of patients with gastric cancer requires reliable detection of early gastric cancer. Magnifying endoscopy with narrow-band imaging (M-NBI) is useful for the accurate preoperative diagnosis of early gastric cancer. However, the role of M-NBI in screening endoscopy has not been established. The aims of this study were to determine the feasibility and limitations of M-NBI in screening endoscopy.Methods: We conducted a multicenter prospective uncontrolled trial of patients undergoing routine screening endoscopy patients. We determined the diagnostic accuracy, sensitivity and specificity of M-NBI according to the degree of certainty and need for biopsy, as assessed using the VS (vessel plus surface) classification system. We analyzed the endoscopic and histopathological characteristics of both false negative and false positive high confidence M-NBI diagnoses. We then developed a provisional diagnostic strategy based on the diagnostic performance and limitations identified in this study.Results: A total of 1097 patients were enrolled in the study. We analyzed 371 detected lesions (20 cancers and 351 non-cancers). The accuracy, sensitivity and specificity of high confidence M-NBI diagnoses were 98.1, 85.7 and 99.4 %, respectively. The false negative case was a pale mucosal lesion with tissue diagnosis of signet-ring cell carcinoma. Exclusion of pale mucosal lesions increased the accuracy, sensitivity and specificity of high confidence M-NBI diagnoses to 99.4, 100 and 99.4 %, respectively. We therefore propose a practical strategy targeting non-pale mucosal lesions.Conclusions: With a refined strategy considering its limitations, M-NBI can act as an “optical biopsy” in screening endoscopies. © 2014, The International Gastric Cancer Association and The Japanese Gastric Cancer Association.
PubMed | Izumiotsu Municipal Hospital, Oyo Electrical Co., Doshisha University and Tottori University
Type: Journal Article | Journal: Journal of medical ultrasonics (2001) | Year: 2015
To verify the measurement of cortical bone thickness at the distal radius in vivo using an ultrasonic method.The method for estimating cortical bone thickness was derived from experiments with in vitro bovine specimens. Propagation time of echo waves and propagation time of slow waves were used for the estimation. The outside diameter of cortical bone and the cortical bone thickness at the distal 5.5 % site of radius were measured with the new ultrasonic bone measurement system, and the results were compared with X-ray pQCT clinical measurements.There was a high positive correlation (r: 0.76) between the cortical bone thickness measured by the new ultrasonic system and the X-ray pQCT results.We will be able to measure not only cancellous bone density but also cortical bone thickness in vivo using ultrasonic waves (without X-ray) safely and repeatedly.
PubMed | Osaka Medical Center and Research Institute for Maternal and Child Health, Wakayama Medical University, Kyoto University and Izumiotsu Municipal Hospital
Type: Journal Article | Journal: Pediatric research | Year: 2016
Maternal intrauterine infection/inflammation represents the major etiology of preterm delivery and the leading cause of neonatal mortality and morbidity. The aim of this study was to investigate the anti-inflammatory properties of thioredoxin-1 in vivo and its potential ability to attenuate the rate of inflammation-induced preterm delivery.Two intraperitoneal injections of lipopolysaccharide from Escherichia coli were administered in pregnant mice on gestational day 15, with a 3-h interval between the injections. From either 1h before or 1h after the first lipopolysaccharide injection, mice received three intravenous injections of either recombinant human thioredoxin-1, ovalbumin, or vehicle, with a 3-h interval between injections.Intraperitoneal injection of lipopolysaccharide induced a rise of tumor necrosis factor-, interferon-, monocyte chemotactic protein 1, and interleukin-6 in maternal serum levels and provoked preterm delivery. Recombinant human thoredoxin-1 prevented the rise in these proinflammatory cytokine levels. After the inflammatory challenge, placentas exhibited severe maternal vascular dilatation and congestion and a marked decidual neutrophil activation. These placental pathological findings were ameliorated by recombinant human thioredoxin-1, and the rate of inflammation-induced preterm delivery was attenuated.Thioredoxin-1 may thus represent a novel effective treatment to delay inflammation-induced preterm delivery.
Nomura N.,Izumiotsu Municipal Hospital |
Itoh T.,Izumiotsu Municipal Hospital
Japanese Journal of Chest Diseases | Year: 2015
A 64-year-old man was admitted to our hospital with appetite loss, general malaise, and disturbed consciousness after a visit to a hot spring. He had high fever (40°C), chest radiographs showed broad infiltration in the left lower lung field, and blood examinations demonstrated elevated serum CRP and CPK levels, renal dysfunction, and liver dysfunction. Since the patient showed positive results for the Legionella antigen and high myoglobin titers in urine, we diagnosed severe Legionella pneumonia associated with rhabdomyolysis and multiple organ dysfunction. We started intravenous steroid administration in addition to pazufloxacin and tazobactam/piperacillin. However, on the 5th disease day, the patient's condition worsened, and the PCT level also increased. We determined that the PCT level was correlated with the severity of the condition, and used it as an index while continuing to treat the patient, whose condition improved subsequently.
Itoh T.,Izumiotsu Municipal Hospital |
Hamanaka Y.,Izumiotsu Municipal Hospital
Japanese Journal of Chest Diseases | Year: 2012
A 49-year-old man was admitted to the hospital with complaints of fever and dry cough. Chest X-ray film revealed ground-glass attenuation in both lung fields, so he was transferred to our hospital for further examination. Chest CT film showed ground-glass attenuation in both lung fields. A transbronchial lung biopsy revealed infiltration of lymphocytes and neutrophils, thickening of alveolar walls and proliferation of type II pneumocytes. By careful history taking, he admitted he had taken sodium chondroitin sulfate for one month before admission. The lymphocyte-stimulation test was positive for sodium chondroitin sulfate. Based on the above findings, we diagnosed this case as drug-induced pneumonia caused by sodium chondroitin sulfate. He recovered after discontinuation of the drug without corticosteroid therapy.
Itoh T.,Izumiotsu Municipal Hospital |
Nomura N.,Izumiotsu Municipal Hospital
Japanese Journal of Chest Diseases | Year: 2016
An 82-year-old woman was admitted to our hospital for further investigation of left pleural effusion. Her pleural fluid showed no evidence of malignancy or infectious disease. Serum immunological tests were positive for antinuclear antibody and anti-dsDNA IgG. Careful history taking revealed that she had been experiencing joint pain in her fingers for 1 month before admission. Based on the above findings, we diagnosed this case as systemic lupus erythematosus (SLE). The pleural effusions resolved with prolonged corticosteroid treatment.
Itoh T.,Izumiotsu Municipal Hospital
Japanese Journal of Chest Diseases | Year: 2013
A 41-year-old woman was referred to our hospital for further examination of pulmonary shadows during a lung cancer screening. Chest X-ray films revealed wandering infiltrations, and a bronchoalveolar lavage showed an increase in the number of lymphocytes and eosinophils. By careful history taking, she admitted she had taken Bofu-tsusho-san intermittently for three months before the lung cancer screening. Based on the above findings, we diagnosed this case as druginduced pneumonitis caused by Bofu-tsusho-san. The patient recovered after discontinuation of the drug without corticosteroid therapy.