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Hirata syndrome

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https://www.readbyqxmd.com/read/29425580/japanese-sleep-questionnaire-for-elementary-schoolers-jsq-es-validation-and-population-based-score-distribution
#1
Ayano Kuwada, Ikuko Mohri, Ryosuke Asano, Shigeyuki Matsuzawa, Kumi Kato-Nishimura, Ikuko Hirata, Takashi Mitsuhosi, Kuriko Kagitani-Shimono, Mariko Nakanishi, Masaya Tachibana, Yuko Ohno, Masako Taniike
OBJECTIVE: The Japanese Sleep Questionnaire for Elementary Schoolers (JSQ-ES) was developed to measure the sleep habits and disturbances of Japanese children. The current study aimed to present psychometric properties and describe the score distribution of the JSQ-ES. In addition, it examined correlations between the sleep and daytime behavior of school-aged children. METHOD: Guardians of 4369 elementary school children and 100 children diagnosed with sleep disorders in two clinics completed the JSQ-ES...
January 2018: Sleep Medicine
https://www.readbyqxmd.com/read/29409370/clinical-practice-guideline-for-sj%C3%A3-gren-s-syndrome-2017
#2
Takayuki Sumida, Naoto Azuma, Masafumi Moriyama, Hiroyuki Takahashi, Hiromitsu Asashima, Fumika Honda, Saori Abe, Yuko Ono, Tomoya Hirota, Shintaro Hirata, Yoshiya Tanaka, Toshimasa Shimizu, Hideki Nakamura, Atsushi Kawakami, Hajime Sano, Yoko Ogawa, Kazuo Tsubota, Koufuchi Ryo, Ichiro Saito, Akihiko Tanaka, Seiji Nakamura, Etsuko Takamura, Masao Tanaka, Katsuya Suzuki, Tsutomu Takeuchi, Noriyuki Yamakawa, Tsuneyo Mimori, Akiko Ohta, Susumu Nishiyama, Toshio Yoshihara, Yasunori Suzuki, Mitsuhiro Kawano, Minako Tomiita, Hiroto Tsuboi
OBJECTIVES: To develop clinical practice guideline (CPG) for Sjögren's syndrome (SS) based on recently available clinical and therapeutic evidences. METHODS: The CPG committee for SS was organized by the Research Team for Autoimmune Diseases, Research Program for Intractable Disease of the Ministry of Health, Labor and Welfare (MHLW), Japan. The committee completed a systematic review of evidences for several clinical questions and developed CPG for SS 2017 according to the procedure proposed by the Medical Information Network Distribution Service (Minds)...
February 7, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29321405/reversible-parkinsonism-and-multiple-cerebral-infarctions-after-pulmonary-endarterectomy-in-a-patient-with-antiphospholipid-syndrome
#3
Mitsumasa Okano, Kazuhiko Nakayama, Naoki Tamada, Yuto Shinkura, Ken-Ichi Yanaka, Hiroyuki Onishi, Hidekazu Tanaka, Toshiro Shinke, Hiroshi Tanaka, Yutaka Okita, Noriaki Emoto, Ken-Ichi Hirata
Antiphospholipid syndrome (APS) is a cause of chronic thromboembolic pulmonary hypertension (CTEPH) and it is associated with an increased risk of postoperative neurological complications. We experienced a case of reversible parkinsonism after pulmonary endarterectomy (PEA) and subsequent multiple cerebral infarctions under standard anticoagulation therapy in a patient with CTEPH associated with APS. Strict management using a combination of antiplatelet and anticoagulation therapy should be considered in patients with a high titer of triple antiphospholipid antibodies in the perioperative period...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29276674/upfront-triple-combination-therapy-induced-pulmonary-edema-in-a-case-of-pulmonary-arterial-hypertension-associated-with-sjogren-s-syndrome
#4
Kimikazu Takeuchi, Kazuhiko Nakayama, Mitsumasa Okano, Naoki Tamada, Hideya Suehiro, Yuto Shinkura, Kenichi Yanaka, Hiroyuki Onishi, Hidekazu Tanaka, Toshiro Shinke, Noriaki Emoto, Ken-Ichi Hirata
Clinical efficacy of combination therapy using vasodilators for pulmonary arterial hypertension (PAH) is well established. However, information on its safety are limited. We experienced a case of primary Sjogren's syndrome associated with PAH where the patient developed pulmonary edema immediately after the introduction of upfront triple combination therapy. Although the combination therapy successfully stabilized her pre-shock state, multiple ground glass opacities (GGO) emerged. We aborted the dose escalation of epoprostenol and initiated continuous furosemide infusion and noninvasive positive pressure ventilation (NPPV), but this did not prevent an exacerbation of pulmonary edema...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29249525/barth-syndrome-different-approaches-to-diagnosis
#5
Atsuko Imai-Okazaki, Yoshihito Kishita, Masakazu Kohda, Yukiko Yatsuka, Tomoko Hirata, Yosuke Mizuno, Hiroko Harashima, Keiichi Hirono, Fukiko Ichida, Atsuko Noguchi, Masayuki Yoshida, Chiho Tokorodani, Ritsuo Nishiuchi, Atsuhito Takeda, Akihiro Nakaya, Yasushi Sakata, Kei Murayama, Akira Ohtake, Yasushi Okazaki
The diagnosis of Barth syndrome is challenging owing to the wide phenotypic spectrum with allelic heterogeneity. Here we report 3 cases of Barth syndrome with phenotypic and allelic heterogeneity that were diagnosed by different approaches, including whole exome sequencing and final confirmation by reverse-transcription polymease chain reaction.
December 15, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29082550/reversed-rivero-carvallo-s-sign-confirmed-by-blood-flow-analysis-using-cardiac-magnetic-resonance-imaging-in-a-patient-with-straight-back-syndrome
#6
Makiko Suto, Shumpei Mori, Tatsuya Nishii, Yu Izawa, Shinsuke Shimoyama, Hiroki Konishi, Kensuke Matsumoto, Hidekazu Tanaka, Ken-Ichi Hirata
An asymptomatic 45-year-old man was referred to our hospital for detailed evaluation of a systolic ejection murmur. The intensity of the murmur increased on deep expiration and decreased on deep inspiration, showing so-called reversed Rivero-Carvallo's sign. Using cardiac magnetic resonance imaging, we demonstrated a characteristic respiratory-induced change in peak flow velocity in the right ventricular outflow tract, which was the basic mechanism of the reversed Rivero-Carvallo's sign in a case with straight back syndrome...
October 29, 2017: Echocardiography
https://www.readbyqxmd.com/read/29070754/a-70-year-old-woman-presenting-with-restless-shoulder-following-posterior-internal-capsule-infarction
#7
Takeo Matsubara, Keisuke Suzuki, Madoka Okamura, Tomohiko Shiina, Masayuki Miyamoto, Toshiki Nakamura, Koichi Hirata
A 70-year-old woman noticed difficulty in speech and weakness of the left upper and lower limb upon awakening. Neurological examination showed dysarthria and left hemiparesis. No sensory disturbance was observed. Brain MRI revealed acute infarction in the right posterior limb of the internal capsule. On the hospital day 1, she developed the abnormal sensations restricted to the bilateral shoulders, resulting in difficulty initiating sleep. On laboratory data, renal function and serum hemoglobin and ferritin levels were normal...
October 26, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29021475/successful-pulmonary-artery-embolization-for-the-management-of-hemoptysis-in-a-patient-with-eisenmenger-syndrome-caused-by-patent-ductus-arteriosus
#8
Naoki Tamada, Kazuhiko Nakayama, Yuto Shinkura, Ken-Ichi Yanaka, Naoto Katayama, Takuya Okada, Masato Yamaguchi, Kensuke Matsumoto, Hidekazu Tanaka, Toshiro Shinke, Noriaki Emoto, Ken-Ichi Hirata
The patient was a 19-year-old woman who was diagnosed with patent ductus arteriosus complicating Eisenmenger syndrome at a previous medical institution. She was referred to our hospital and arranged for lung transplantation. She developed hemoptysis after the introduction of i.v. epoprostenol, which was administered as a bridging treatment while the patient awaited lung transplantation. She continued to suffer from recurrent hemoptysis, even after switching from i.v. epoprostenol to i.v. treprostinil. Angiography of the systemic and pulmonary arteries revealed the vessel responsible for the recurrent hemoptysis and pulmonary artery embolization was successfully performed...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28975585/polymorphisms-in-genes-involved-in-the-leptin-melanocortin-pathway-are-associated-with-obesity-related-cardiometabolic-alterations-in-a-southern-chilean-population
#9
Victor Manriquez, Jorge Aviles, Luis Salazar, Nicolas Saavedra, Pamela Seron, Fernando Lanas, Cristina Moreno Fajardo, Mario Hiroyuki Hirata, Rosario Dominguez Crespo Hirata, Alvaro Cerda
BACKGROUND: Polymorphisms in genes encoding proteins of the leptin-melanocortin pathway have been associated with obesity. The involvement of these polymorphisms with changes in body mass index (BMI) and anthropometric measures could also imply a contribution to the risk of metabolic syndrome (MetS) and metabolic alterations. We evaluated the relationship of leptin-melanocortin system polymorphisms with obesity, MetS, and other metabolic alterations in Southern Chilean individuals. METHODS: Two-hundred individuals were grouped as normoweight (BMI 18...
October 3, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28960464/pde3-inhibitor-attenuates-rat-sinusoidal-obstruction-syndrome-through-inhibition-of-platelet-aggregation-in-disse-s-space
#10
Takashi Miyata, Hidehiro Tajima, Miki Hirata, Shin-Ichi Nakanuma, Isamu Makino, Hironori Hayashi, Katsunobu Oyama, Tomoharu Miyashita, Hiroyuki Takamura, Itasu Ninomiya, Sachio Fushida, Shoichi Iseki, Shin-Ichi Harada, Tomohiko Wakayama, Tetsuo Ohta
BACKGROUND AND AIM: Sinusoidal obstruction syndrome (SOS) is a serious drug-induced liver injury. However, the pathophysiology of the disease remains unclear. This study investigated the effects of cilostazol (CZ), a phosphodiesterase III inhibitor, in a monocrotaline (MCT)-induced rat model of SOS. METHODS: Male Wistar rats were administrated MCT to induce SOS. Rats were divided into control, MCT, and MCT+CZ groups. In the MCT+CZ group, CZ was administered at 48 h, 24 h, and 30 min prior to and 8 h and 24 h after MCT administration...
September 28, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28936753/long-term-results-of-bilateral-pulmonary-artery-banding-versus-primary-norwood-procedure
#11
Yasutaka Hirata, Hiroaki Miyata, Norimichi Hirahara, Arata Murakami, Hideaki Kado, Kisaburo Sakamoto, Shunji Sano, Shinichi Takamoto
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included...
January 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/28929616/noninvasive-assessment-of-left-ventricular-end-diastolic-pressure-by-deceleration-time-of-early-diastolic-mitral-annular-velocity-in-patients-with-heart-failure
#12
Kazushi Takemoto, Kumiko Hirata, Takeshi Hozumi, Takashi Tanimoto, Makoto Orii, Yasutsugu Shiono, Yoshiki Matsuo, Yasushi Ino, Hironori Kitabata, Takashi Kubo, Atsushi Tanaka, Takashi Akasaka
OBJECTIVE: To examine the diagnostic ability of the deceleration time of early mitral annular velocity (e'DT) as determined by tissue Doppler velocity image, a method for assessing LV filling pressure. BACKGROUNDS: Estimation of LV filling pressure by Doppler echocardiography requires a combination of various parameters. Therefore, there remains a need for a simple index in LV filling pressure estimation. The e' is known to be reduced and delayed with increased LV filling pressure during development of heart failure...
September 2017: Echocardiography
https://www.readbyqxmd.com/read/28923775/immunoadsorption-using-immusorba-tr-and-ph
#13
REVIEW
Ryuichiro Hirano, Noriko Hirata
Immusorba TR (IM-TR) and PH (IM-PH) were developed as immunoadsorbents from nonbiological materials as affinity ligands for removal of pathogenic substances. The immunoadsorbents in IM-TR and IM-PH are immobilized on a polyvinyl alcohol gel with tryptophan and phenylalanine, respectively, as a ligand. IM-TR is mainly clinically applied to autoimmune neurological diseases such as myasthenia gravis, Guillain-Barré syndrome, and multiple sclerosis. IM-PH is also applied to neurological diseases but mainly to rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus...
August 30, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28917079/insulin-autoimmune-syndrome-hirata-s-disease-in-an-italian-patient-a-case-report-and-review-of-the-literature
#14
REVIEW
Simona Censi, Maria Paola Albergoni, Nicoletta Gallo, Mario Plebani, Marco Boscaro, Corrado Betterle
We describe the case of a 54-year-old Caucasian Italian male experiencing episodes of hypoglycemia, occurring mainly after meals. He had never been exposed to insulin and was taking ramipril, flecainide and acetylsalicylic acid. An oral glucose tolerance test (OGTT) showed high blood glucose levels diagnostic for diabetes mellitus at 120 min and hypoglycemia with inappropriately high insulin levels at 240 min. The 72-h fasting test, abdominal computed tomography (CT) and positron emission tomography-CT were normal...
September 16, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28904256/current-surgical-outcomes-of-congenital-heart-surgery-for-patients-with-down-syndrome-in-japan
#15
Takaya Hoashi, Norimichi Hirahara, Arata Murakami, Yasutaka Hirata, Hajime Ichikawa, Junjiro Kobayashi, Shinichi Takamoto
BACKGROUND: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28892298/impact-of-hysteroscopic-surgery-for-isthmocele-associated-with-cesarean-scar-syndrome
#16
Shunichiro Tsuji, Fuminori Kimura, Akiyoshi Yamanaka, Tetsuro Hanada, Kimiko Hirata, Akie Takebayashi, Akiko Takashima, Ayumi Seko-Nitta, Takashi Murakami
AIM: Cesarean scar syndrome (CSS) is characterized by increased risk of postmenstrual abnormal uterine bleeding, dysmenorrhea, and infertility, due to a post-cesarean scar defect known as an isthmocele. This study aimed to assess the impact of hysteroscopic surgery on isthmocele associated with CSS. METHODS: Eighteen patients with CSS were enrolled. Surgical methods included resection of the inferior edge and superficial cauterization of the isthmocele via hysteroscopic surgery...
September 11, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28883502/activation-of-endoplasmic-reticulum-stress-in-granulosa-cells-from-patients-with-polycystic-ovary-syndrome-contributes-to-ovarian-fibrosis
#17
Nozomi Takahashi, Miyuki Harada, Yasushi Hirota, Emi Nose, Jerilee Mk Azhary, Hiroshi Koike, Chisato Kunitomi, Osamu Yoshino, Gentaro Izumi, Tetsuya Hirata, Kaori Koga, Osamu Wada-Hiraike, R Jeffrey Chang, Shunichi Shimasaki, Tomoyuki Fujii, Yutaka Osuga
Recent studies report the involvement of intra-ovarian factors, such as inflammation and oxidative stress, in the pathophysiology of polycystic ovary syndrome (PCOS), the most common endocrine disorder of reproductive age women. Endoplasmic reticulum (ER) stress is a local factor that affects various cellular events during a broad spectrum of physiological and pathological conditions. It may also be an important determinant of pro-fibrotic remodeling during tissue fibrosis. In the present study, we showed that ER stress was activated in granulosa cells of PCOS patients as well as in a well-established PCOS mouse model...
September 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28827009/characterizing-restless-legs-syndrome-and-leg-motor-restlessness-in-patients-with-parkinson-s-disease-a-multicenter-case-controlled-study
#18
Keisuke Suzuki, Yasuyuki Okuma, Tomoyuki Uchiyama, Masayuki Miyamoto, Ryuji Sakakibara, Yasushi Shimo, Nobutaka Hattori, Satoshi Kuwabara, Toshimasa Yamamoto, Yoshiaki Kaji, Shigeki Hirano, Ayaka Numao, Koichi Hirata
BACKGROUND: We investigated the prevalence and impact of restless legs syndrome (RLS) and leg motor restlessness (LMR) in patients with Parkinson's disease (PD) in a multicenter study. METHODS: A total of 436 PD patients and 401 age- and sex-matched controls were included in this study. RLS was diagnosed based on four essential features. LMR was diagnosed when a participant exhibited the urge to move his or her legs but did not meet the four essential features of RLS...
August 14, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28790247/-pulmonary-mucoepidermoid-carcinoma-with-hilar-lymph-node-metastasis-complicated-by-myelodysplastic-syndrome
#19
Yoshinari Matsuda, Hiroshi Moriyama, Masashi Inaba, Yoshihiko Kubo, Masahiro Kitada, Satoshi Hayashi, Kei Ishibashi, Satoshi Okazaki, Nana Takahashi, Shunsuke Yasuda, Masahiro Abe, Satoshi Hirata
A 65-year-old woman was found to have a mass shadow on chest computed tomography. The patient had been diagnosed as having myelodysplastic syndrome 3 years before admission. She was transferred to our hospital for further examination of the mass in the lower lobe of left lung. The mass was positron emission tomography-positive (SUVmax of 8.6)suggesting a malignant neoplasm. Serum concentrations of carcinoembryonic antigen was elevated to 8.7 ng/ml. Preoperative laboratory studies showed anemia (hemoglobin 6...
August 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28768969/werner-syndrome-and-diabetes-mellitus-accompanied-by-adrenal-cortex-cancer
#20
Momoyo Nishioka, Shinji Kamei, Tomoe Kinoshita, Junpei Sanada, Yoshiro Fushimi, Shintaro Irie, Yurie Hirata, Akihito Tanabe, Hidenori Hirukawa, Tomohiko Kimura, Atsushi Obata, Fuminori Tatsumi, Kenji Kohara, Masashi Shimoda, Shuhei Nakanishi, Tomoatsu Mune, Kohei Kaku, Hideaki Kaneto
Werner syndrome is a rare genetic disease characterized by progeria, diabetes mellitus, cataracts and various types of malignancy. However, there are few reports showing adrenal cortex cancer in subjects with Werner syndrome. We herein report an extremely rare case of Werner syndrome accompanied by adrenal cortex cancer. Based on the data obtained from blood samples, computed tomography, magnetic resonance imaging and (131)I adosterol scintigraphy, we diagnosed this subject with adrenal cortex cancer and Cushing's syndrome...
2017: Internal Medicine
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