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https://www.readbyqxmd.com/read/29775450/-congenital-megacalycosis-in-a-girl-with-unilateral-renal-agenesis
#1
Agnieszka Szmigielska, Grażyna Krzemień, Anna Zacharzewska, Teresa Dudek-Warchoł, Stanisław Warchoł
Renal agenesis occurs in pediatric population with the incidence 1:500- 2000 children. It is more often diagnosed in boys and on the left side of the body. Renal agenesis may be isolated or it may be a part of complex malformation syndrome. Megacalycosis is a very rare anomaly of urinary tract associated with abnormal structure of the kidney pyramids. AIM: The aim of the study was to present for the first time in the medical literature the case of a girl with unilateral renal agenesis and megacalycosis...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29775448/pyonephrosis-as-the-first-symptom-of-congenital-hydronephrosis-in-a-6-year-old-girl
#2
Teresa Dudek-Warchoł, Stanisław Warchoł, Przemysław Bombiński, Krzysztof Toth, Agnieszka Szmigielska, Garżyna Krzemień
Pyonephrosis in the course of hydronephrosis usually provides to total or near-total loss of renal function. In adults pyonephrosis usually results from urolithiasis. In children usually congenital urinary tract anomalies are present as contributing factors. CASE REPORT: 6-year old girl was admitted to the hospital because of 2-day history of high fever, abdominal pain, progressive deterioration of general condition and ultrasonographically (US) detected left hydronephrosis. US on admission showed grossly dilated left renal collecting system together with extensive echogenic debris and laboratory tests highly elevated inflammatory markers...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29775428/the-involvement-of-human-monogenic-cardiomyopathy-genes-in-experimental-polygenic-cardiac-hypertrophy
#3
Priscilla R Prestes, Francine Z Marques, Guillermo Lopez-Campos, Paul Lewandowski, Lea M D Delbridge, Fadi J Charchar, Stephen B Harrap
Hypertrophic cardiomyopathy thickens heart muscles reducing functionality and increasing risk of cardiac disease and morbidity. Genetic factors are involved, but their contribution is poorly understood. We used the hypertrophic heart rat (HHR), a unique normotensive polygenic model of cardiac hypertrophy and heart failure to investigate the role of genes associated with monogenic human cardiomyopathy. We selected 42 genes involved in monogenic human cardiomyopathies to study: 1) DNA variants, by sequencing the whole-genome of 13-week old HHR and age-matched normal heart rat (NHR), its genetic control strain; 2) mRNA expression, by targeted RNA-sequencing in left ventricles of HHR and NHR at five ages (2-days old, 4-, 13-, 33- and 50-weeks old) compared to human idiopathic dilated data; and 3) microRNA expression, with rat microRNA microarrays in left ventricles of 2-days old HHR and age-matched NHR...
May 18, 2018: Physiological Genomics
https://www.readbyqxmd.com/read/29773157/genetic-etiology-for-alcohol-induced-cardiac-toxicity
#4
James S Ware, Almudena Amor-Salamanca, Upasana Tayal, Risha Govind, Isabel Serrano, Joel Salazar-Mendiguchía, Jose Manuel García-Pinilla, Domingo A Pascual-Figal, Julio Nuñez, Gonzalo Guzzo-Merello, Emiliano Gonzalez-Vioque, Alfredo Bardaji, Nicolas Manito, Miguel A López-Garrido, Laura Padron-Barthe, Elizabeth Edwards, Nicola Whiffin, Roddy Walsh, Rachel J Buchan, William Midwinter, Alicja Wilk, Sanjay Prasad, Antonis Pantazis, John Baski, Declan P O'Regan, Luis Alonso-Pulpon, Stuart A Cook, Enrique Lara-Pezzi, Paul J Barton, Pablo Garcia-Pavia
BACKGROUND: Alcoholic cardiomyopathy (ACM) is defined by a dilated and impaired left ventricle due to chronic excess alcohol consumption. It is largely unknown which factors determine cardiac toxicity on exposure to alcohol. OBJECTIVES: This study sought to evaluate the role of variation in cardiomyopathy-associated genes in the pathophysiology of ACM, and to examine the effects of alcohol intake and genotype on dilated cardiomyopathy (DCM) severity. METHODS: The authors characterized 141 ACM cases, 716 DCM cases, and 445 healthy volunteers...
May 22, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29769710/jdp2-overexpression-provokes-cardiac-dysfunction-in-mice
#5
Jacqueline Heger, Julia Bornbaum, Alona Würfel, Christian Hill, Nils Brockmann, Renáta Gáspár, János Pálóczi, Zoltán V Varga, Márta Sárközy, Péter Bencsik, Tamás Csont, Szilvia Török, Baktybek Kojonazarov, Ralph Theo Schermuly, Kerstin Böngler, Mariana Parahuleva, Peter Ferdinandy, Rainer Schulz, Gerhild Euler
The transcriptional regulator JDP2 (Jun dimerization protein 2) has been identified as a prognostic marker for patients to develop heart failure after myocardial infarction. We now performed in vivo studies on JDP2-overexpressing mice, to clarify the impact of JDP2 on heart failure progression. Therefore, during birth up to the age of 4 weeks cardiac-specific JDP2 overexpression was prevented by doxycycline feeding in transgenic mice. Then, JDP2 overexpression was started. Already after 1 week, cardiac function, determined by echocardiography, decreased which was also resembled on the cardiomyocyte level...
May 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29769452/acute-glaucoma-attack-following-microvascular-decompression-surgery-for-trigeminal-neuralgia
#6
Kenichi Amagasaki, Masami Nagayama, Saiko Watanabe, Naoyuki Shono, Hiroshi Nakaguchi
Microvascular decompression (MVD) is widely accepted as an effective surgical method to treat trigeminal neuralgia (TN), but the risks of morbidity and mortality must be considered. We experienced a case of acute angle-closure glaucoma attack following MVD for TN in an elderly patient, considered to be caused by lateral positioning during and after the surgery. A 79-year-old female underwent MVD for right TN in the left lateral decubitus position, and TN disappeared after the surgery. Postoperatively, the patient tended to maintain the left lateral decubitus position to prevent wound contact with the pillow, even after ambulation...
May 17, 2018: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/29767239/time%C3%A2-dependent-and-independent-effects-of-thyroid-hormone-administration-following-myocardial-infarction-in-rats
#7
Ioanna Iliopoulou, Iordanis Mourouzis, George I Lambrou, Dimitra Iliopoulou, Dimitrios-Dionysios Koutsouris, Constantinos Pantos
Cardiac function is reduced following myocardial infarction (MI) due to myocardial injury and alterations in the viable non‑ischemic myocardium, a process known as cardiac remodeling. The current treatments available for patients with acute MI (AMI) reduce infarct size, preserve left ventricular (LV) function and improve survival; however, these treatments do not prevent remodeling, which can lead to heart failure. The aim of the present study was to investigate the effects of thyroid hormone (TH) treatment following MI in an in vivo rat model...
May 11, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29764897/diagnostic-yield-of-genetic-testing-in-young-athletes-with-t-wave-inversion
#8
Nabeel Sheikh, Michael Papadakis, Mathew Wilson, Aneil Malhotra, Carmen Adamuz, Tessa Homfray, Lorenzo Monserrat, Elijah R Behr, Sanjay Sharma
Background -T-wave inversion (TWI) is common in patients with cardiomyopathy. However, up to 25% of athletes of African/Afro-Caribbean descent (black athletes) and 5% of white athletes also have TWI of unclear clinical significance despite comprehensive clinical evaluation and long-term follow-up. The aim of this study was to determine the diagnostic yield from genetic testing, beyond clinical evaluation, when investigating athletes with TWI. Methods -We investigated 50 consecutive asymptomatic black and 50 white athletes aged 14-35-years-old with TWI and a normal echocardiogram who were referred to a UK tertiary center for cardiomyopathy and sports cardiology...
May 15, 2018: Circulation
https://www.readbyqxmd.com/read/29762932/pulmonary-hypertension-as-a-possible-cause-of-paradoxical-low-flow-low-gradient-aortic-stenosis
#9
Yuta Watanabe, Haruhiko Higashi, Katsuji Inoue, Jun Aono, Takafumi Okura, Jitsuo Higaki, Shuntaro Ikeda
Paradoxical low-flow, low-gradient aortic stenosis (LFLG AS) is recognized as a subtype of aortic stenosis. A small left ventricular (LV) cavity with marked LV concentric remodeling leads to a reduced stroke volume in this condition. The case is reported of a paradoxical LFLG AS patient who was undergoing treatment for pulmonary hypertension (PH) and interstitial pneumonia associated with scleroderma. Echocardiography demonstrated enlargement of the right ventricle and a diminished LV cavity. Moreover, the aortic valve opening was restricted despite a preserved LV ejection fraction (61%)...
September 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29762929/trileaflet-mitral-valve-treated-with-the-mitraclip%C3%A2-system
#10
Miguel Rodríguez-Santamarta, Rodrigo Estévez-Loureiro, Tomás Benito-González, Javier Gualis, Carmen Garrote, Armando Pérez de Prado, Felipe Fernández-Vázquez
A 79-year-old woman with a history of ischemic dilated cardiomyopathy, severely depressed left ventricular ejection fraction and significant mitral regurgitation (MR) was admitted to the authors´ institution for percutaneous mitral valve repair. Transesophageal echocardiography (TEE) revealed the presence of a posterior mitral cleft at the P2 level, causing a trileaflet mitral valve that contributed significantly to the regurgitant jet. The procedure was performed under general anesthesia and guided by real-time three-dimensional TEE...
September 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29761889/a-novel-human-s10f-hsp20-mutation-induces-lethal-peripartum-cardiomyopathy
#11
Guan-Sheng Liu, George Gardner, George Adly, Min Jiang, Wen-Feng Cai, Chi Keung Lam, Fawzi Alogaili, Nathan Robbins, Jack Rubinstein, Evangelia G Kranias
Heat shock protein 20 (Hsp20) has been shown to be a critical regulator of cardiomyocyte survival upon cardiac stress. In this study, we investigated the functional significance of a novel human Hsp20 mutation (S10F) in peripartum cardiomyopathy. Previous findings showed that cardiac-specific overexpression of this mutant were associated with reduced autophagy, left ventricular dysfunction and early death in male mice. However, this study indicates that females have normal function with no alterations in autophagy but died within a week after 1-4 pregnancies...
May 15, 2018: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29761081/enhanced-aortic-pressure-wave-reflection-in-patients-with-aortic-coarctation-after-aortic-arch-repair
#12
REVIEW
Tomoaki Murakami
Background: In patients with aortic coarctation after successful aortic arch repair, it is well known that early-onset cardiovascular diseases can develop. Summary: We studied the pressure waveform in patients after aortic arch repair focusing on a pressure wave reflection. In patients after aortic arch repair, the repaired portion generates a new reflected pressure wave. As a result, the newly generated pressure wave causes aortic pressure augmentation, loss of pressure amplification, and left ventricular hypertrophy with fibrosis...
March 2018: Pulse (Basel, Switzerland)
https://www.readbyqxmd.com/read/29757579/isolated-low-grade-prenatally-detected-unilateral-hydronephrosis-do-we-need-long-term-follow-up
#13
Osama M Sarhan, Ahmed El Helaly, Abdulhakim Al Otay, Mustafa Al Ghanbar, Ziad Nakshabandi
PURPOSE: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. MATERIALS AND METHODS: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis...
April 30, 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29755824/olfactory-neuroblastoma-a-rare-cause-of-external-ophthalmoplegia-proptosis-and-compressive-optic-neuropathy
#14
Ömer Kartı, Mehmet Özgür Zengin, Ozan Çelik, Taşkın Tokat, Tuncay Küsbeci
Olfactory neuroblastoma (ONB), which is a neuroectodermal tumor of the nasal cavity, is a rare and locally aggressive malignancy that may invade the orbit via local destruction. In this study, we report a patient with proptosis, external ophthalmoplegia, and compressive optic neuropathy caused by ONB. A detailed clinical examination including ocular imaging and histopathological studies were performed. The 62-year-old female patient presented to our clinic with complaints of proptosis and visual deterioration in the left eye...
April 2018: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/29755095/-mediastinal-bronchial-artery-aneurysm-associated-with-bronchiectasis-report-of-a-case
#15
Katsutoshi Adachi, Tomohito Tarukawa
The patient was a 66-year-old man who had treated with bronchiectasis. He was admitted to our hospital because of hemoptysis. A chest plain computed tomography (CT) showed bronchiectasis in the left lower lobe and nodular lesion in the mediastinum. Chest contrast CT revealed a left bronchial artery aneurysm, and hyperplastic left bronchial arteries dilating and winding toward the left lower lobe. We performed selective angiography of the bronchial artery, revealing an aneurysm, and we performed 3 times of bronchial arterial embolization( BAE)...
May 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29755073/-beating-mitral-valve-replacement-for-functional-mitral-regurgitation-after-cardiac-resynchronization-therapy-report-of-a-case
#16
Hiroshi Kumano, Keisuke Shuntoh, Akimitsu Yamaguchi
A 72-year-old man was admitted to our hospital because of dyspnea. He had a history of cardiac resynchronization therapy for congestive heart failure due to dilated cardiomyopathy 6 months previously. Echocardiography revealed severe functional mitral regurgitation which had been mild 6 months before. Because medical treatment was ineffective, on-pump beating mitral valve replacement using a bioprosthesis was performed to prevent reperfusion injury. The patient was easily weaned from cardiopulmonary bypass with low-dose inotropic support and scheduled intra-aortic balloon pump...
March 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29754919/balloon-expandable-stents-for-recoarctation-of-the-aorta-in-small-children-two-centre-experience
#17
K Gendera, P Ewert, D Tanase, S Georgiev, T Genz, P Bambul Heck, T Moszura, I Malcic, J Cleuziou, A Eicken
BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents...
July 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29754460/prognostic-evaluation-of-the-elastic-properties-of-the-ascending-aorta-in-dilated-cardiomyopathy
#18
Edoardo Sciatti, Enrico Vizzardi, Ivano Bonadei, Davide Fabbricatore, Francesco Prati, Mattia Pagnoni, Marco Metra
BACKGROUND: Nowadays there is an increased interest in the role of aortic stiffness in the pathophysiology of heart failure (HF), since it is a major determinant of left ventricular (LV) performance. We aimed at assessing the predictive value of the aortic stiffness parameters, measured by echocardiography, in patients affected by non-ischemic dilated cardiomyopathy (DCM) regarding three end-points: death, HF rehospitalization, combined death or HF rehospitalization in a long-term follow-up...
May 13, 2018: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29754170/cardiac-support-device-asd-delivers-bone-marrow-stem-cells-repetitively-to-epicardium-has-promising-curative-effects-in-advanced-heart-failure
#19
Shizhong Yue, Muhammad Naveed, Wang Gang, Dingding Chen, Zhijie Wang, Feng Yu, Xiaohui Zhou
Ventricular restraint therapy is a non-transplant surgical option for the management of advanced heart failure (HF). To augment the therapeutic applications, it is hypothesized that ASD shows remarkable capabilities not only in delivering stem cells but also in dilated ventricles. Male SD rats were divided into four groups (n = 6): normal, HF, HF + ASD, and HF + ASD-BMSCs respectively. HF was developed by left anterior descending (LAD) coronary artery ligation in all groups except normal group. Post-infarcted electrocardiography (ECG) and brain natriuretic peptide (BNP) showed abnormal heart function in all model groups and HF + ASD-BMSCs group showed significant improvement as compared to other HF, HF + ASD groups on day 30...
May 12, 2018: Biomedical Microdevices
https://www.readbyqxmd.com/read/29753831/medical-and-dietary-management-of-eosinophilic-esophagitis
#20
REVIEW
Quan M Nhu, Seema S Aceves
OBJECTIVE: Eosinophilic esophagitis (EoE) is a disease of chronic, allergen-driven, T-helper 2 (Th2) immune-mediated inflammation that progresses to fibrostenosis of the esophagus if left untreated. There are currently no FDA-approved drugs for the treatment of EoE. This review focuses on medical and dietary management of EoE. DATA SOURCES: Manuscripts on EoE treatments are identified on PubMed. STUDY SELECTIONS: Original research, randomized control trials, retrospective studies, meta-analyses, case series, and on occasions, case reports of high relevance, are selected and reviewed...
May 10, 2018: Annals of Allergy, Asthma & Immunology
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