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https://www.readbyqxmd.com/read/27916030/-short-term-outcomes-of-lung-transplant-recipients-using-organs-from-brain-death-donors
#1
W X He, C Jiang, X G Liu, W Huang, C Chen, L Jiang, B Yang, K Wu, Q K Chen, Y Yang, Y M Yu, G N Jiang
Objective: To assess short-term outcomes after lung transplantation with organs procured following brain death. Methods: Between April 2015 and July 2016, all 17 recipients after lung transplantation using organs from brain death donors (DBD) at Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University School of Medicine were enrolled in this study. All patients were male, aging (60±7) years, including 11 chronic obstructive pulmonary disease, 5 idiopathic pulmonary fibrosis, 1 silicosis...
December 1, 2016: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/27909566/prenatal-diagnosis-of-a-terminal-chromosome-1-q42-q44-deletion-original-case-report-and-review-of-the-literature
#2
C Van Linthout, V Emonard, J S Gatot, X Capelle, F Kridelka, P Emonts, M C Segghaye
Terminal chromosome 1q deletion is rarely reported but causes typical malformations that have been well described in childhood. Clinical features include facial dysmorphy, growth and/or psychomotor retardation, brain agenesis or hypoplasia of the corpus callosum, epilepsy and occasional urogenital or cardiac malformations. The diagnosis of this condition is usually made at birth. The rare cases of antenatal diagnosis were based on microcephaly and growth retardation. In the present case, the foetus presented with an hypoplasia of the corpus callosum, a dysmorphic profile and a single umbilical artery...
June 27, 2016: Facts, Views & Vision in ObGyn
https://www.readbyqxmd.com/read/27909513/assessment-of-sinoatrial-node-function-in-patients-with-persistent-and-long-standing-persistent-forms-of-atrial-fibrillation-after-maze-iii-procedure-combined-with-mitral-valve-operation
#3
Kulikov Aleksei A, Bokeria Leo A
RESEARCH OBJECTIVE: Assessment of sinoatrial node function after Maze III procedure combined with a mitral valve operation. METHODS: 100 patients were included in the research with persistent and long-standing persistent forms of atrial fibrillation (AF) and need of operative treatment concerning valve disease. The following preoperative preparation methods were executed to all patients: Electrocardiogram in 12 standard assignments;Two-dimensional echocardiographic with assessment of systolic and diastolic functions of the left ventricle, size of the left atrium and grade of valve disease;Transesophageal echocardiography for exclusion of blood clots in the left atrium and left atrial appendage;Coronary angiography for exclusion of coronary heart disease;Computer tomography for examination of cardiac chambers and anatomic characteristics of pulmonary veins...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909498/shock-lead-dislodgement-related-to-its-small-hair-pin-curve-in-a-pocket-a-case-of-ratchet-syndrome
#4
Yuka Taguchi, Kohei Matsushita, Toshiyuki Ishikawa, Yutaka Ogino, Hirooki Matsushita, Junya Hosoda, Katsumi Matsumoto, Satoshi Umemura
There have been few reports about ratchet syndrome. We report a case of ratchet syndrome caused by small hair-pin curve of lead that triggered the lead retract itself. A 69-year-old man with a past history of inferior wall myocardial infarction, presented with progressive congestive heart failure. He underwent implantation of cardiac resynchronization therapy with an implantable cardiac defibrillator (CRTD) at our hospital. At 33 days after implantation, shock lead dislodgement was revealed. X-ray showed that the lead tip was in left subclavian vein, leaving its screw out, and a large part of the proximal portion of the lead was retracted into the pocket, while the other two leads remained in appropriate positions and the device had not rotated...
April 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27904840/surgical-experience-on-chronic-constrictive-pericarditis-in-african-setting-review-of-35-years-experience-in-cote-d-ivoire
#5
Koffi Herve Yangni-Angate, Yves Tanauh, Christophe Meneas, Florent Diby, Anicet Adoubi, Manga Diomande
BACKGROUND: Surgical experience with chronic constrictive pericarditis (CCP) is rarely documented in Africa; the aim of this study is therefore to review our African experience with CCP from 1977 to 2012 in terms of clinical and surgical outcomes and risk factors of early death after pericardiectomy. METHODS: This retrospective study is related to 120 patients with CCP; there were 72 men and 48 women with an average age at 28.8±10.4 years standard deviation (SD) (8-51 years)...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27899087/paradoxical-physiological-responses-to-propranolol-in-a-rett-syndrome-patient-a-case-report
#6
P J Santosh, L Bell, K Lievesley, J Singh, F Fiori
BACKGROUND: Rett Syndrome (RTT), caused by a loss-of-function in the epigenetic modulator: X-linked methyl-CpG binding protein 2 (MeCP2), is a pervasive neurological disorder characterized by compromised brain functions, anxiety, severe mental retardation, language and learning disabilities, repetitive stereotyped hand movements and developmental regression. An imbalance in the sympathetic and the parasympathetic nervous system (dysautonomia) and the resulting autonomic storms is a frequent occurrence in patients with RTT...
November 29, 2016: BMC Pediatrics
https://www.readbyqxmd.com/read/27899069/gastric-dilatation-and-intestinal-obstruction-mimicking-acute-coronary-syndrome-with-dynamic-electrocardiographic-changes
#7
H M M T B Herath, Anne Thushara Matthias, B S D P Keragala, W A E Udeshika, Aruna Kulatunga
BACKGROUND: ST elevation myocardial infarction is a medical emergency and the electrocardiogram is a part of the mainstay in the initial diagnosis. A variety of non-cardiac conditions have been known to mimic the electrocardiographic changes seen in acute coronary syndrome. We present a patient presenting with acute partial intestinal obstruction causing gastric distension and intestinal dilatation who also had dynamic electrocardiographic changes, mimicking anterior ST elevation myocardial infarction...
November 29, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27896330/biventricular-thrombus-in-hypereosinophilic-syndrome-presenting-with-shortness-of-breath
#8
Abdul Baqi, Shahan Waheed, Fateh Ali Tipoo, Aamir Hameed Khan
A 48 years old male presented to clinic with 12 months of low grade fever with shortness of breath which has progressively worsened with no associated weight loss, night sweats or loss of appetite. There was no prior history of chronic illness before the current illness. Laboratory workup revealed a high white blood cell count with predominant eosinophils. Chest X-ray was normal. Transthoracic echocardiography and Cardiac Magnetic Resonance showed biventricular thrombi. On further extensive workup the findings were consistent with hypereosinophilic syndrome...
June 2016: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27884380/association-of-endothelial-dysfunction-and-cytotoxin-associated-gene-a-positive-helicobacter-pylori-in-patients-with-cardiac-syndrome-x
#9
COMMENT
Yousef Rasmi, Hadi Rouhrazi, Ebrahim Khayati-Shal, Alireza Shirpoor, Ehsan Saboory
BACKGROUND: Existence of coronary endothelial dysfunction has been demonstrated in patients with cardiac syndrome X (CSX). In addition, Helicobacter pylorus (H. pylori) has been associated with CSX. We aimed to assess the possible association of endothelial dysfunction and cytotoxin-associated gene A-positive H. pylori (CagA+) infection in CSX patients. METHODS: Fifty-six patients with CSX (23 male/33 female; age: 51.25 ± 8.86 years) who were anti-H. pylori IgG-positive [H...
October 2016: Biomedical Journal
https://www.readbyqxmd.com/read/27817779/-identification-of-a-novel-jag1-mutation-in-a-family-affected-by-alagille-syndrome
#10
Ying Cheng, Shu-Tao Zhao, Li Guo, Mei Deng, Qing Zhou, Yuan-Zong Song
Alagille syndrome (ALGS) is an autosomal dominant disorder which is mainly caused by JAG1 gene mutation and can affect multiple systems including the liver, heart, eyes, skeleton and face. This paper reports the clinical and genetic features of an ALGS patient. A 2-year-and-9-month-old boy was referred to the hospital with the complaint of abnormal liver function and heart murmur discovered over two years. Jaundice of the skin and sclera was not observed. The child had a prominent forehead, left esotropia, depressed nasal bridge and micromandible...
November 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27816333/ischemic-stroke-due-to-hypoperfusion-in-a-patient-with-a-previously-unrecognized-danon-disease
#11
Marco Marino, Olimpia Musumeci, Giuseppe Paleologo, Maria Cucinotta, Alba Migliorato, Carmelo Rodolico, Antonio Toscano
Danon disease, an X-linked multisystemic disorder, is due to deficiency of Lysosome-Associated Membrane Protein 2 (LAMP2). It is usually characterized by hypertrophic cardiomyopathy, mental retardation and skeletal myopathy, sometimes also with atypical features. A 20-year-old man with cognitive impairment was admitted to the Emergency Room because of a sudden chest pain. ECG showed Wolff-Parkinson-White syndrome; echocardiography revealed hypertrophic cardiomyopathy, and, shortly after, he experienced a cardiac arrest followed by an occipital ischemic stroke...
October 5, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27801321/-clinical-characteristics-and-long-term-therapeutic-effects-of-60-patients-with-idiopathic-hypereosinophilic-syndrome-in-a-single-center
#12
S Q Qu, T J Qin, Z F Xu, Y Zhang, X F Ai, B Li, H L Zhang, L W Fang, L J Pan, N B Hu, Z J Xiao
Objective: To analyze the long term outcome of patients with hypereosinophilic syndrome(HES). Methods: The clinical characteristics and efficacy of 60 newly diagnosed HES patients who received corticosteroids(CS)monotherapy were retrospectively analyzed. The survival and death causes of patients were obtained by follow- up. Results: Of all 60 HES patients, 45 were male and 15 female. The median age was 38(11-80)years old. The most frequent organ involvement of HES occurred in cutaneous(55.0%), gastrointestinal(40...
October 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27767272/myocardial-scintigraphy-a-method-for-detecting-cardiac-comorbidity-in-psoriasis-patients
#13
Markus Zutt, Hagen Rudolph, Kjell Matthias Kaune, Werner Wosniok, Ulrich Gärtner, Rainer Linke
BACKGROUND: In recent years, cardiac comorbidities in psoriasis patients have increasingly moved into the focus of clinical research. The objective of the present study was to evaluate myocardial scintigraphy as a screening method in patients with psoriasis. PATIENTS AND METHODS: Assessment of various comorbidities in 50 psoriasis patients without clinical symptoms of cardiac disease. Myocardial scintigraphy was employed to detect cardiac risk/exercise-induced ischemia...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27754772/determinants-of-improvement-in-left-ventricular-diastolic-function-following-a-1-year-lifestyle-modification-program-in-abdominally-obese-men-with-features-of-the-metabolic-syndrome
#14
Jacinthe Leclerc, Marie Arsenault, Jean-Pierre Després, Patrice Brassard, Valérie Gaudreault, Jean Bergeron, Natalie Alméras, Angelo Tremblay, Audrey Auclair, Marie-Kristelle Ross, Stéphanie Denault-Bissonnette, Paul Poirier
BACKGROUND: Abdominal obesity and presence of the metabolic syndrome (MetS) are associated with cardiac abnormalities. Among those, left ventricular diastolic dysfunction (LVDD) is the most frequently encountered in clinical practice. Few studies evaluated the reversibility of LVDD by an approach promoting lifestyle modifications in abdominally obese subjects with MetS. METHODS: We assessed the impact of a 1-year lifestyle modification program combining nutritional and physical activity counseling on LVDD and metabolic profile of abdominally obese men with MetS...
December 2016: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/27739212/ehlers-danlos-syndrome-with-lethal-cardiac-valvular-dystrophy-in-males-carrying-a-novel-splice-mutation-in-flna
#15
Marco Ritelli, Silvia Morlino, Edoardo Giacopuzzi, Giulia Carini, Valeria Cinquina, Nicola Chiarelli, Silvia Majore, Marina Colombi, Marco Castori
Filamin A is an X-linked, ubiquitous actin-binding protein whose mutations are associated to multiple disorders with limited genotype-phenotype correlations. While gain-of-function mutations cause various bone dysplasias, loss-of-function variants are the most common cause of periventricular nodular heterotopias with variable soft connective tissue involvement, as well as X-linked cardiac valvular dystrophy (XCVD). The term "Ehlers-Danlos syndrome (EDS) with periventricular heterotopias" has been used in females with neurological, cardiovascular, integument and joint manifestations, but this nosology is still a matter of debate...
October 14, 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27708714/the-presence-of-two-rare-genomic-syndromes-1q21-deletion-and-xq28-duplication-segregating-independently-in-a-family-with-intellectual-disability
#16
Kyungsoo Ha, Yiping Shen, Tyler Graves, Cheol-Hee Kim, Hyung-Goo Kim
BACKGROUND: 1q21 microdeletion syndrome is a rare contiguous gene deletion disorder with de novo or autosomal dominant inheritance patterns and its phenotypic features include intellectual disability, distinctive facial dysmorphism, microcephaly, cardiac abnormalities, and cataracts. MECP2 duplication syndrome is an X-linked recessive neurodevelopmental disorder characterized by intellectual disability, global developmental delay, and other neurological complications including late-onset seizures...
2016: Molecular Cytogenetics
https://www.readbyqxmd.com/read/27708590/qt-interval-derived-measurements-in-patients-with-cardiac-syndrome-x-compared-to-coronary-artery-disease
#17
Mohamed F Lutfi
Previous studies assessing effect of ischemia on ventricular repolarization are mostly directed toward patients with coronary artery disease (CAD); however, similar reports on cardiac syndrome X (CSX) are scarce. Whether microvascular dysfunction of CSX and ischemia induced by CAD produce comparable effect on ventricular repolarization is unclear and deserve further studies. In the present study, ECG measures of ventricular repolarization were compared between CAD and CSX patients (40 subjects in each group)...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27665334/eosinophilic-myocarditis-associated-with-eosinophilic-pneumonia-and-eosinophilia-following-antibiotic-and-narcotic-analgesic-treatment
#18
Aziz İnan Çelik, Ali Deniz, Mustafa Tangalay, Muhammet Buğra Karaaslan, Emine Bağır Kılıç
Eosinophilic myocarditis (EM) is a rare form of myocarditis that usually presents with heart failure due to eosinophilic infiltration. EM is often a component of hypereosinophilic syndrome (HES). HES is a rare disorder characterized by persistent, marked eosinophilia combined with organ system dysfunction. A 38-year-old man was admitted to emergency services with left inguinal pain and fever, and was hospitalized with diagnosis of nephrolithiasis and urinary tract infection. Intravenous antibiotic therapy of 3 grams meropenem per day and analgesic of 50 mg pethidine per day were administered...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/27649277/familial-deletion-of-the-hoxa-gene-cluster-associated-with-hand-foot-genital-syndrome-and-phenotypic-variability
#19
Emir Tas, Jessica Sebastian, Suneeta Madan-Khetarpal, Philip Sweet, Alexander N Yatsenko, Nijole Pollock, Aleksandar Rajkovic, Francis X Schneck, Svetlana A Yatsenko, Selma Feldman Witchel
Hand-Foot-Genital syndrome is a rare autosomal dominant condition characterized by distal limb anomalies and urogenital malformations. This disorder is associated with loss-of-function mutations in the HOXA13 gene. HOXA13 plays an important role in the development of distal limbs and lower genitourinary tract of the fetus. We report a novel familial 589 kb deletion in the 7p15.2 region identified in a male toddler and his mother. The proband had severe penoscrotal hypospadias, mild skeletal anomalies of the hands and feet, cardiac, renal, and gastrointestinal anomalies...
September 20, 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27630835/lipid-profile-in-cardiac-syndrome-x-association-with-helicobacter-pylori
#20
Yousef Rasmi, Javad Zeynalzadeh, Alireza Shirpoor, Mirhossein Seyedmohammadzad, Reza Hajhosseini
INTRODUCTION: Chronic inflammation caused by Helicobacter pylori (H.pylori) infection has a pathogenic role in Cardiac Syndrome X (CSX). In addition, it has shown that bacterial infection may affect blood lipids. AIM: To assess if H.pylori affects the level of lipid profile in CSX. MATERIALS AND METHODS: Eighty-eight CSX patients and 97 healthy controls were enrolled. The Total Cholesterol (TC), Triglyceride (TG), Lipoprotein A (LP{A}), Low Density Lipoprotein (LDL), High Density Lipoprotein (HDL), Apoprotein A1 (APOA1), and Apoprotein B (APOB) was estimated colorimetrically...
July 2016: Journal of Clinical and Diagnostic Research: JCDR
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