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Hirata syndrome

Akiko Takeuchi, Takashi Matsushita, Kenzo Kaji, Yoshinobu Okamoto, Masahide Yasui, Masayoshi Hirata, Naoto Oishi, Akira Higashi, Mariko Seishima, Tomoya Asano, Manabu Fujimoto, Masataka Kuwana, Kazuhiko Takehara, Yasuhito Hamaguchi
OBJECTIVE: To identify and characterize a novel connective tissue disease (CTD)-related autoantibody (autoAb) directed against scaffold attachment factor B (SAFB). METHODS: AutoAb specificity was analyzed using RNA and protein-immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry. RESULTS: By immunoprecipitation assay, 10 sera reacted with a protein with a molecular weight of approximately 160 kDa...
September 24, 2016: Journal of Autoimmunity
Akihiro Nakajo, Hideo Arima, Munetsugu Hirata, Yoshie Yamashita, Yoshiaki Shinden, Naoki Hayashi, Yota Kawasaki, Takaaki Arigami, Yasuto Uchikado, Shinichiro Mori, Yuko Mataki, Masahiko Sakoda, Yuko Kijima, Yoshikazu Uenosono, Kosei Maemura, Shoji Natsugoe
INTRODUCTION: Endoscopic thyroidectomy is a well-established surgical technique that is mainly performed for benign thyroid disease. We considered that endoscopic surgery could also be widely indicated for the treatment of thyroid cancer. We herein describe our new bidirectional approach of video-assisted neck surgery (BAVANS) for complete central node dissection in endoscopic thyroid cancer surgery. METHODS: BAVANS involves two different directional pathways to the cervical lesion...
September 21, 2016: Asian Journal of Endoscopic Surgery
Keisuke Suzuki, Masayuki Miyamoto, Tomoyuki Uchiyama, Tomoyuki Miyamoto, Takeo Matsubara, Koichi Hirata
We herein report an elderly patient with a history of restless legs syndrome (RLS) who developed unpleasant sensations in the lower abdomen and perineum. The patient' s symptoms occurred during the evening and at rest and forced the patient to void, resulting in some symptom relief. Urological examinations and spinal magnetic resonance imaging findings were unremarkable. The adjunctive use of rotigotine resolved her symptoms. The symptoms were considered to be "restless bladder". Further studies are required to elucidate whether restless bladder is an identical condition to the clinical entities of other RLS variants or whether it represents an unusual feature of RLS...
2016: Internal Medicine
Masahiko Nakata, Keisuke Ishii, Masahiro Sumie, Mayumi Takano, Hiroko Hirata, Susumu Murata, Yuichiro Takahashi, Takeshi Murakoshi, Haruhiko Sago
OBJECTIVE: The aim of this study is to clarify the feasibility and efficacy of fetoscopic laser photocoagulation of placental vascular communicating vessels (FLP) on twin-twin transfusion syndrome (TTTS) after 26 weeks of gestation. MATERIALS AND METHODS: This is a prospective pilot study investigating all cases of TTTS between 26 weeks and 27 weeks during 2012 and 2013 in Japan. The primary endpoints in this study were the feasibility of FLP and rate of harmful complications for the mother's health...
August 2016: Taiwanese Journal of Obstetrics & Gynecology
David Church, Luís Cardoso, Sonia Bradbury, Catriona Clarke, Anna Stears, Anna Dover, David Halsall, Robert Semple
CONTEXT: Insulin-binding antibodies may produce severe dysglycaemia in insulin-naïve patients ('insulin autoimmune syndrome' (IAS) or Hirata disease), while rendering routine insulin assays unreliable. OBJECTIVE: To assess the performance of clinically used insulin assays and an optimal analytical approach in the context of IAS. DESIGN: Observational biochemical study of selected patients with hyperinsulinaemic hypoglycaemia. PATIENTS: Three patients without diabetes with recurrent spontaneous hyperinsulinaemic hypoglycaemia and 'positive' insulin antibodies...
September 2, 2016: Clinical Endocrinology
Mychelle Kytchia Rodrigues Nunes Duarte, Jéssica Nayara Góes de Araújo, Victor Hugo Rezende Duarte, Katiene Macêdo de Oliveira, Juliana Marinho de Oliveira, Antonio Augusto Ferreira Carioca, Raul Hernandes Bortolin, Adriana Augusto Rezende, Mario Hiroyuki Hirata, Rosário Domingues Hirata, Dan Linetzky Waitzberg, Severina Carla Vieira Cunha Lima, André Ducati Luchessi, Vivian Nogueira Silbiger
BACKGROUND: The fatty acid profile is associated with the risk and progression of several diseases, probably via mechanisms including its influence on gene expression. We previously reported a correlation between ECHDC3 upregulation and the severity of acute coronary syndrome. Here, we assessed the relationship of serum fatty acid profile and ECHDC3 expression with the extent of coronary lesion. METHODS: Fifty-nine individuals aged 30 to 74 years and undergoing elective cinecoronariography for the first time were enrolled in the present study...
2016: Lipids in Health and Disease
Isamu Murata, Mai Goto, Masahiro Komiya, Risa Motohashi, Momoko Hirata, Yutaka Inoue, Ikuo Kanamoto
Crush syndrome (CS) is the systemic manifestation of muscle cell damage resulting from pressure and crushing. It is associated with a high mortality rate, even when patients are treated with conventional therapy. We demonstrated the utility of intramuscular administration of dexamethasone (DEX) in disaster medical care by using a model of CS to characterize the pharmacokinetics and biochemical parameters. We compared intravenous (IV) and intramuscular (IM) injection. The IM sites were the right anterior limb (AL), bilateral hind limbs (bHL), and unilateral hind limb (uHL)...
2016: Biological & Pharmaceutical Bulletin
Takaaki Shinohara, Ryogo Nakamura, Etsuhiro Nakao, Hitoshi Hirata
We retrospectively reviewed 12 patients (3 men and 9 women, with a mean age of 72 years) who were surgically treated for carpal tunnel syndrome associated with Kienböck disease. All patients except 1 were incidentally diagnosed with Kienböck disease and had little or no wrist pain. Radiographic tests revealed advanced Kienböck disease in all patients. Intraoperative findings indicated that the site of maximum compression on the median nerve was located at the level of the carpal tunnel inlet in 11 patients, and the volar dislocated fragment of the lunate was located proximally adjacent to the floor of the carpal tunnel inlet...
August 2016: Nagoya Journal of Medical Science
Renata Caroline Costa de Freitas, Raul Hernandes Bortolin, Mariana Borges Lopes, Mario Hiroyuki Hirata, Rosario Dominguez Crespo Hirata, Vivian Nogueira Silbiger, André Ducati Luchessi
Genetic and epigenetic variability may influence the efficacy and safety of antiplatelet therapies, including clopidogrel. Therefore, the miRNA-mRNA interactions and drug toxicity were investigated in silico using available microarray data. Expressions profiles of platelet miRNA (GSE59488) from acute coronary syndrome and mRNA in peripheral blood cells (GSE32226) from coronary artery disease patients were used to miRNA-target mRNA integrated analysis by Ingenuity Pathways Analysis 6 software (IPA). Results showed that ST13 mRNA is regulated by hsa-miR-107 (miR-103-3p); BTNL3 and CFD mRNAs are regulated by hsa-miR-4701-3p (miR-1262); SLC7A8 is regulated by hsa-miR-145-5p (miR-145-5p); and SENP5 mRNA is regulated by hsa-miR-15b-5p (miR-16-5p) and hsa-miR-26a-5p (miR-26a-5p)...
November 15, 2016: Gene
Ryuki Matsuura, Shin-Ichiro Hamano, Yuko Hirata, Atsuko Oba, Kotoko Suzuki, Kenjiro Kikuchi
PURPOSE: To evaluate factors influencing the efficacy and safety of intravenous immunoglobulins (IVIG) therapy for West syndrome. METHODS: We investigated seizure outcomes in 70 patients who received IVIG treatment for West Syndrome during the first 3months after the onset of epileptic spasms. IVIG was administered for 3 consecutive days (initial IVIG treatment) at dosages ranging from 100 to 500mg/kg/day. If spasms disappeared within 2weeks of the initial treatment, maintenance IVIG treatment was commenced...
September 15, 2016: Journal of the Neurological Sciences
Hiroto Tsuboi, Isao Matsumoto, Shinya Hagiwara, Tomoya Hirota, Hiroyuki Takahashi, Hiroshi Ebe, Masahiro Yokosawa, Mizuki Yagishita, Hidenori Takahashi, Izumi Kurata, Ayako Ohyama, Fumika Honda, Hiromitsu Asashima, Haruka Miki, Naoto Umeda, Yuya Kondo, Shintaro Hirata, Kazuyoshi Saito, Yoshiya Tanaka, Yoshiro Horai, Hideki Nakamura, Atsushi Kawakami, Takayuki Sumida
OBJECTIVE: To clarify the efficacy and safety of abatacept for secondary Sjögren's syndrome (SS) associated with rheumatoid arthritis (RA). METHODS: The primary endpoint of this open-labeled, prospective, observational multicenter study for secondary SS with RA was the remission rate of Simplified Disease Activity Index (SDAI) at 52 weeks after initiation of abatacept. The secondary endpoints included Saxon's test and Schirmer's test. Adverse events and adherence rate during the study period were also analyzed...
July 26, 2016: Modern Rheumatology
Katsuyuki Iwatsuki, Akihito Yoshida, Takaaki Shinohara, Tomonori Nakano, Jun-Ichi Uemura, Sae Goto, Masaaki Hirayama, Minoru Hoshiyama, Hitoshi Hirata
OBJECTIVE: The recovery function of somatosensory evoked magnetic fields (SEFs) was recorded to investigate excitatory and inhibitory balance in the somatosensory cortex of patients with carpal tunnel syndrome. METHODS: SEFs were recorded in patients and controls. Recordings were taken following median nerve stimulation with single and double pulses with interstimulus intervals of 10-200ms. The root mean square for the N20m component following the second stimulation was analyzed...
August 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Ryota Niikura, Takako Serizawa, Atsuo Yamada, Shuntaro Yoshida, Mariko Tanaka, Yoshihiro Hirata, Kazuhiko Koike
The number of cancer patients undergoing dialysis has been increasing, and the number of these patients on chemotherapy is also increasing. Imatinib is an effective and safe therapy for KIT-positive gastrointestinal stromal tumors (GIST), but the efficacy and safety of imatinib in dialysis patients remain unclear. Because clinical trials have not been conducted in this population, more investigations are required. We report on a 75-year-old Japanese man undergoing dialysis who presented with massive tarry stool from a duodenal GIST...
January 2016: Case Reports in Gastroenterology
Jun-Ichi Suzuki, Yasushi Imai, Mieko Aoki, Daishi Fujita, Norifumi Takeda, Norio Aoyama, Kouji Wakayama, Yuichi Ikeda, Hidetoshi Kumagai, Hiroshi Akazawa, Yuichi Izumi, Mitsuaki Isobe, Issei Komuro, Yasunobu Hirata
Marfan syndrome (MFS) is a systemic connective tissue disorder that is caused by mutations of fibrillin-1. While MFS patients are at a high risk of periodontitis and aortic diseases, little causal information has been provided to date. To clarify the relationship, their oral condition and sinus of Valsalva (SoV) were evaluated.The subjects were patients with MFS (n = 33) who attended the University of Tokyo Hospital. We divided them into two groups; MFS patients with highly dilated (the diameters were equal to or more than 39 mm) SoV (high group, n = 18) and MFS patients with mildly dilated (less than 39 mm) SoV (mild group, n = 15)...
July 27, 2016: International Heart Journal
Atsuyuki Ikeda, Yumiko Yasuhara, Daizen Hirata, Mari Teramura, Takeharu Nakamura, Yoko Oiwa, Osamu Araki, Hidemasa Azechi, Ryuta Nishitai, Hiroyuki Kokuryu
We report a case of resected hepatocellular carcinomas (HCCs) after drug-eluting bead transarterial chemoembolization (DEB-TACE). A 67-year-old man with alcoholic liver disease was diagnosed with HCCs. Serological markers for hepatitis B and C viruses were negative. Among tumor markers, alpha-fetoprotein was 2.7ng/mL, and protein induced by vitamin K absence II was 868mAU/mL. Two HCCs were detected using dynamic computed tomography: one was 9cm in diameter in S8 and the other was 2cm in diameter in S4. We performed DEB-TACE using HepaSphere(®) and epirubicin...
2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
Tetsushi Yamamoto, Hidekazu Tanaka, Chiyo Kurimoto, Takamitsu Imanishi, Nobuhide Hayashi, Jun Saegusa, Akio Morinobu, Ken-Ichi Hirata, Seiji Kawano
Cardiac involvement in hypereosinophilic syndrome (HES) patients entails significant morbidity and mortality. Left ventricular (LV) endocardial damage is important for the development of cardiac involvement in HES patients. However very early stage LV endocardial damage, such as prior to the first stage of an acute necrotic stage, remains uncertain. We studied 32 HES patients, all with normal conventional echocardiographic findings. Global radial and circumferential strain (GRS and GCS) were determined for each peak global strain curve from the mid-LV short-axis view, and global longitudinal strain (GLS) was averaged each peak global strain curve from standard apical views by means of two-dimensional speckle-tracking method...
May 30, 2016: International Journal of Cardiovascular Imaging
Kenji Tabara, Hiromi Fujita, Atau Hirata, Daisuke Hayasaka
No abstract text is available yet for this article.
September 21, 2016: Japanese Journal of Infectious Diseases
Kimihiko Banno, Sayaka Omori, Katsuya Hirata, Nobutoshi Nawa, Natsuki Nakagawa, Ken Nishimura, Manami Ohtaka, Mahito Nakanishi, Tetsushi Sakuma, Takashi Yamamoto, Tsutomu Toki, Etsuro Ito, Toshiyuki Yamamoto, Chikara Kokubu, Junji Takeda, Hidetoshi Taniguchi, Hitomi Arahori, Kazuko Wada, Yasuji Kitabatake, Keiichi Ozono
Chromosomal aneuploidy and specific gene mutations are recognized early hallmarks of many oncogenic processes. However, the net effect of these abnormalities has generally not been explored. We focused on transient myeloproliferative disorder (TMD) in Down syndrome, which is characteristically associated with somatic mutations in GATA1. To better understand functional interplay between trisomy 21 and GATA1 mutations in hematopoiesis, we constructed cellular disease models using human induced pluripotent stem cells (iPSCs) and genome-editing technologies...
May 10, 2016: Cell Reports
Kohji Tanakaya, Tatsuro Yamaguchi, Hideki Ishikawa, Takao Hinoi, Yoichi Furukawa, Keiji Hirata, Yoshihisa Saida, Mototsugu Shimokawa, Masami Arai, Nagahide Matsubara, Naohiro Tomita, Kazuo Tamura, Kokichi Sugano, Chikashi Ishioka, Teruhiko Yoshida, Hideyuki Ishida, Toshiaki Watanabe, Kenichi Sugihara
AIM: To elucidate the causes of cancer death in Japanese families with Lynch syndrome (LS). METHODS: The distributions of cancer deaths in 485 individuals from 67 families with LS (35, 30, and two families with MutL homologue 1 (MLH1), MSH2, and MSH6 gene mutations, respectively), obtained from the Registry of the Japanese Society for Cancer of the Colon and Rectum were analyzed. RESULTS: Among 98 cancer deaths of first-degree relatives of unknown mutation status, 53%, 19%, 13% (among females), 7% (among females) and 5% were due to colorectal, gastric, uterine, ovarian, and hepatobiliary cancer, respectively...
April 2016: Anticancer Research
Nozomi Takahashi, Miyuki Harada, Yasushi Hirota, Lin Zhao, Osamu Yoshino, Yoko Urata, Gentaro Izumi, Masashi Takamura, Tetsuya Hirata, Kaori Koga, Osamu Wada-Hiraike, Tomoyuki Fujii, Yutaka Osuga
Vascular endothelial growth factor A (VEGFA) is crucial for ovarian angiogenesis, but its excess production induces ovarian hyperstimulation syndrome (OHSS). The aim of this study was to determine whether endoplasmic reticulum (ER) stress regulates VEGFA expression in granulosa cells, and whether its activation is involved in OHSS development. The expression of the spliced form of X-box-binding protein 1 [XBP1(S)], induced by ER stress, in cumulus cells from OHSS patients was higher than that in cumulus cells from non-OHSS patients...
June 15, 2016: Molecular and Cellular Endocrinology
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