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Matsui S.,University of Toyama | Hebisawa A.,NHO Tokyo National Hospital | Sakai F.,Saitama University | Yamamoto H.,Shinshu University | And 15 more authors.
Respirology | Year: 2013

Background and objective Immunoglobulin G4 (IgG4)-related disease is a multi-organ disorder that can include the lungs. IgG4-related lung disease can present in various forms; the clinical, radiological and pathological features of patients with this disease have been assessed. Methods Forty-eight patients suspected of having IgG4-related lung disease, with a high serum concentration of IgG4 and abundant IgG4-positive plasma cell infiltration into the intrathoracic organs, were retrospectively evaluated. Their clinical features, chest imaging findings and pathological findings were examined, with final diagnoses made by an open panel conference. Results Of the 48 patients, 18 with extrathoracic manifestations were diagnosed as having IgG4-related lung disease. Most of these patients were middle-Aged to elderly men. IgG4-related lung disease was characterized by high serum concentrations of IgG and IgG4, normal white blood cell count and serum C-reactive protein concentration and a good response to corticosteroids. Common radiological findings included mediastinal lymphadenopathy and thickening of the perilymphatic interstitium, with or without subpleural and/or peribronchovascular consolidation. Pathological examination showed massive lymphoplasmacytic infiltration with fibrosis in and around the lymphatic routes, with distribution well correlated with radiological manifestations. Conclusions The findings suggest that the intrathoracic manifestations of IgG4-related lung disease develop through lymphatic routes of the lungs and show various clinical characteristics. Because some lymphoproliferative disorders show similar findings, the correlation of clinicoradiological and pathological characteristics is crucial for the diagnosis of IgG4-related lung disease. The clinicoradiological and pathological features of IgG4-RLD were assessed. Clinically, this disease is silent, although radiological and pathological examinations showed that it involves lymphatic routes, with spread observed on imaging and infiltration on pathological examination. Clinicoradiological and pathological correlation is necessary for the diagnosis of this disease. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.


Hasegawa K.,Brigham and Women's Hospital | Hasegawa K.,Massachusetts General Hospital | Hagiwara Y.,Kawaguchi Municipal Medical Center | Chiba T.,Obama Municipal Hospital | And 4 more authors.
Resuscitation | Year: 2012

Objectives: Emergency medicine is increasingly recognized as a medical specialty in Japan. However, comprehensive studies evaluating emergency airway management practice are lacking. We describe emergency department (ED) airway management using a large multi-center registry. Methods: We formed the Japanese Emergency Airway Network, a consortium of 10 academic and community medical centers in Japan, and prospectively collected data on ED intubations from April 2010 to February 2011. All patients undergoing emergency intubation were eligible for inclusion. Data were entered in real time by the intubator using a standardized data form. Variables included patient's age, sex, weight, indication for intubation, methods of intubation, drugs, level of training and specialty of the intubator, number of attempts, success or failure, and adverse events. We present descriptive data as proportions with 95% confidence intervals. Results: We recorded 1486 intubations (compliance rate 99%). Intubation was ultimately successful in 99.7%. The initial method of intubation varied substantially among the hospitals, including rapid sequence intubation (0-79%), sedation without paralysis (4-88%), paralysis without sedation (0-18%), and oral without medication (12-67%), in non-cardiac arrest encounters. Success rates in first and ≤3 attempts ranged from 40 to 83% and from 74 to 100%, respectively. The overall adverse event rate was 11%, without significant difference by the method used. Conclusions: In this multi-center study characterizing ED airway management across Japan, we observed a high overall success rate but a high degree of variation among hospitals in the methods of intubation and success rates. © 2011 Elsevier Ireland Ltd.


PubMed | Kawaguchi Municipal Medical Center, Tokyo Women's Medical University, Tokyo Medical and Dental University and University of Tsukuba
Type: Journal Article | Journal: American journal of medical genetics. Part A | Year: 2016

1p36 deletion syndrome is the most common terminal deletion syndrome, and the genomic regions that contribute to specific 1p36 deletion syndrome-related phenotypes were recently identified. Deletions in the 1p36 region have been documented in various tumor tissues, which indicates correlation between loss of heterozygosity of 1p36 and tumor development, and the existence of tumor suppressors in this region. Therefore, it was suspected that patients with 1p36 deletion syndrome have a higher risk of tumor development; however, only a few child cases of neuroblastoma with 1p36 deletion syndrome have been reported. We report the first case of 1p36 deletion syndrome with paraganglioma (PGL) and include genetic investigation. The 24-year-old woman with 1p36 deletion syndrome had severe intellectual disability, dilated cardiomyopathy, and distinct dysmorphic features, and presented with persistent vomiting accompanied by hypertension (178/115mmHg). Abdominal CT revealed a 4050mm retroperitoneal mass and substantial elevations of plasma and urine norepinephrine (15.4nmol/L and 1022mol/mol creatinine, respectively); abnormal uptake of


PubMed | Kawaguchi Municipal Medical Center, Kurume University and Nihon University
Type: Journal Article | Journal: Indian journal of dermatology | Year: 2015

We report a 79-year-old Japanese man who developed subepidermal blistering skin disease after an 8-year history of psoriasis. Histology of a bullous lesion revealed a subepidermal blister with a mixed inflammatory cell infiltrate and fibrin nets. Indirect immunofluorescence using normal human skin sections revealed IgG and IgA autoantibodies in the patient serum, which bound to the epidermal side of 1M NaCl-split skin sections. Immunoblot analysis revealed that both IgA and IgG antibodies reacted with the BP180 NC16a domain and the 120-kDa LAD-1 and that IgG antibodies also reacted with the BP180 C-terminal domain and laminin gamma-1. These findings indicated that autoantibodies to laminin gamma-1 and multiple epitopes in BP180 ectodomain played a role in the pathogenesis of this unique autoimmune subepidermal blistering skin disease associated with psoriasis.


Taki A.,Tokyo Medical and Dental University | Abe M.,Tokyo Medical and Dental University | Abe M.,Jobu University | Komaki M.,Tokyo Medical and Dental University | And 4 more authors.
Congenital Anomalies | Year: 2012

We hypothesized that gene expression in placenta and umbilical vessels are affected by intrauterine environment and some of the expression in umbilical vessels originating from the fetus could reflect fetal condition of these complicated pregnancies. Expression of angiogenesis-related factors and inflammatory cytokines were examined in placenta and umbilical vessels to clarify the effects of intrauterine environment of pregnancies complicated by preeclampsia and chorioamnionitis/funisitis. Forty-six preterm cesarean section deliveries were classified into three groups based on maternal condition during prenatal monitoring: preeclampsia (PE) (n=11), chorioamnionitis/funisitis (CAM) (n=8), and preterm control (PC) (n=27). Angiogenesis-related factors and inflammatory cytokines in placentas, umbilical arteries and umbilical veins were analyzed by RT-PCR and immunohistochemistry. We demonstrated that Ang-2, Tie-2, and Dll4 increase in the placentas of PE compared to PC for the first time, and we confirmed the findings of previous reports showing the high expression of HIF-1α, sFlt-1, endoglin, leptin, and AT1R. Expression of angiogenesis-related factors, including HIF-1α, VEGF, angiopoietin, and TGF-β systems, and inflammatory cytokines, such as TNF-α and IL-6, increased in umbilical vessels of PE. Umbilical veins of CAM showed a higher Dll4 level than did PC. In preeclampsia, abnormal expressions of angiogenesis-related factors related to lifestyle diseases in adulthood were seen in the placenta and umbilical vessels as compared to PC. Chorioamnionitis/funisitis showed only upregulation of DII4 in umbilical veins. © 2012 Japanese Teratology Society.


Araki T.,Kawaguchi Municipal Medical Center | Nakata H.,Kawaguchi Municipal Medical Center | Kusunoki S.,Kinki University
Clinical Neurology | Year: 2010

We describe two patients who developed progressive ascending paralysis associated with Guillain-Barré syndrome (GBS) during late pregnancy. A 25-year-old woman in the 30th week of gestation (GW) developed diarrhea followed by GBS and weakness of the bilateral facial muscles. Serum IgM antibody titers against cytomegalovirus (CMV) were high. Respiratory insufficiency developed at GW 31 requiring cesarean section and artificial ventilation. The facial palsy and limb paralysis persisted thereafter. Serum anti-GM2 IgM and anti-GalNAc-GD1a IgM antibodies were positive so immunoadsorption therapy (IAT) was applied. These antibody titers decreased with clinical improvement after IAT. The baby was healthy and did not have CMV. The other patient was a 24-year-old woman at GW 28 in whom GBS developed after a common cold. Right facial muscles were also weak and serum anti-GM2 IgM antibody was positive. Cesarean section was performed because of uterine bleeding. The clinical findings improved thereafter and the baby was healthy. The findings show that the course of GBS that develops after CMV infection can be severe and accompanied by respiratory insufficiency.


Hara H.,Kawaguchi Municipal Medical Center
Japanese Journal of Clinical Radiology | Year: 2012

As respiratory disorders are very common in children, chest radiography is the initial imaging examination and the most frequent pediatric procedure performed in the radiology department. Familiarity with the normal appearances of the pediatric chest, particularly expiration, airway, and variation of the normal thymus is essential to avoid errors of overinterpretation. This article provided an approach to evaluate the pediatric chest radiography, and also described the important signs, and useful findings which may be overlooked.


Nakano S.,Kawaguchi Municipal Medical Center | Otsuka M.,Kawaguchi Municipal Medical Center | Mibu A.,Kawaguchi Municipal Medical Center | Oinuma T.,Kawaguchi Municipal Medical Center
Clinical Breast Cancer | Year: 2015

Conclusion Cytology findings for small breast lesions should be considered only when imaging and cytology indicate benign lesions. Therefore, pathological examination without cytological examination should be the initial approach.Results Ultrasonography-guided FNAC was performed before VAB in 248 cases (54.5%). In 133 cases (53.6%), the results of FNAC were inconclusive. Pathological examinations using VAB revealed malignant and benign lesions in 199 and 256 cases, respectively. Of the 256 benign cases, we performed excisional biopsy in 17 cases (6.6%) and repeated VAB in 8 cases (3.1%). Excisional biopsy revealed malignant lesions in 2 cases. The reason for excisional biopsy was overdiagnosis using FNAC in 6 cases (35%). In all cases of repeated VAB, the pathological diagnosis was benign. The reason for repeated VAB was excision of the lesions in 5 cases (62.5%). The false positive and false negative rates of FNAC were 16.7% and 3.4%, respectively, whereas those of VAB were 0% and 1.0%, respectively.Background In recent years, pathological diagnoses have been increasingly required, especially in small breast lesions, because malpractice lawsuits concerning erroneous cytological diagnoses have been commonly reported. Here, we retrospectively evaluated the significance of FNAC and VAB for small breast lesions using ultrasonography guidance.Patients and Methods A total of 1383 cases for which ultrasonography-guided VAB was performed between June 1996 and December 2012 were reviewed. Of these, 455 small breast lesions (239 nonpalpable and 216 nonmass lesions) were included in the study. © 2015 The Authors. Published by Elsevier Inc. All rights reserved.


Iida T.,Jikei University School of Medicine | Iida T.,Kawaguchi Municipal Medical Center | Nakabayashi Y.,Kawaguchi Municipal Medical Center | Okui N.,Jikei University School of Medicine | And 4 more authors.
Anticancer Research | Year: 2014

A 63-year-old woman was admitted to our Hospital for treatment of pancreatic head ductal carcinoma, and underwent pancreaticoduodedectomy (PD) in October 2007. At one month after surgery, she received systemic adjuvant chemotherapy using S-1 for three months. Because the serum carbohydrate antigen 19-9 (CA19-9) value was elevated at 23 months after surgery, the patient underwent systemic chemotherapy using gemcitabine. The serum CA19-9 decreased, but abdominal Computed Tomography (CT) revealed a hepatic metastasis in the ventrolateral segment of left hepatic lobe at 28 months after surgery. The chemotherapy was changed to oral S-1. At 35 months after surgery, abdominal CT revealed reduction of liver metastasis and that the serum CA19-9 was normalized, but chemotherapy had to be withdrawn because of severe myelosuppression. Because of her good general condition, the patient underwent partial hepatectomy for the liver metastasis. Histopathological examination demonstrated a complete response. Thirty six months after hepatectomy and 6 years after PD, the patient remains well without recurrence. We herein report a case of successful treatment for metachronous liver metastasis from pancreatic ductal carcinoma after PD by chemotherapy and hepatectomy and review the current literature.


PubMed | Kawaguchi Municipal Medical Center
Type: Journal Article | Journal: Pediatrics international : official journal of the Japan Pediatric Society | Year: 2016

This study reviewed the medical records of very low-birthweight infants (VLBWI) followed up to adolescence, with emphasis on inguinal hernia (IH), to discuss the ideal time for IH repair in VLBWI.Medical records from 274 VLBWI treated in the neonatal intensive care unit (NICU) between 1994 and 1999 were collected retrospectively. Outpatient data after NICU discharge were included. Additionally, a follow-up study was undertaken via questionnaire sent to the families of VLBWI in 2011.During NICU hospitalization, IH was diagnosed in 39 of 274 VLBWI, and two developed incarceration. Thirty-eight VLBWI were discharged with known hernia, and elective repair was performed in 19. Three developed incarceration before elective repair at the mean age of 316 days. In the remaining 18 cases of IH, spontaneous regression occurred at a mean age of 180 days and repair was not performed. New IH appeared in 25 VLBWI after NICU discharge.The high incidence of IH in VLBWI was confirmed during follow up to adolescence. The rate of incarceration was low and the incidence of spontaneous regression was high. Observation up to 6 months without surgery, with the expectation of spontaneous regression, is one option to avoid unnecessary surgery, but repair should be performed before 10 months to reduce the risk of incarceration. Further large, prospective, and randomized controlled studies with a long follow up are necessary to validate the present results and to define the ideal time for IH repair in VLBWI.

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