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https://www.readbyqxmd.com/read/28446969/micrornas-pleiotropic-players-in-congenital-heart-disease-and-regeneration
#1
REVIEW
Sarah C Hoelscher, Stefanie A Doppler, Martina Dreßen, Harald Lahm, Rüdiger Lange, Markus Krane
Congenital heart disease (CHD) is the leading cause of infant death, affecting approximately 4-14 live births per 1,000. Although surgical techniques and interventions have improved significantly, a large number of infants still face poor clinical outcomes. MicroRNAs (miRs) are known to coordinately regulate cardiac development and stimulate pathological processes in the heart, including fibrosis or hypertrophy and impair angiogenesis. Dysregulation of these regulators could therefore contribute (I) to the initial development of CHD and (II) at least partially to the observed clinical outcomes of many CHD patients by stimulating the aforementioned pathways...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28444475/vertebral-abnormality-without-spine-curvature-deformity-on-prenatal-ultrasonography-sonographic-findings-and-postnatal-radiographic-correlations
#2
Mi Jin Song, Young-Hwa Kim
PURPOSE: To evaluate prenatal US features and postnatal radiographic findings of fetuses with a sonographically detected vertebral abnormality (VA) without spine-curvature deformity (SCD). METHODS: Twenty-six fetuses showing a VA without SCD on prenatal US at our ultrasound center for a 5-year period were retrospectively identified and evaluated for sonographic data and coexisting anomalies. Medical records and postnatal radiographs of all 16 live births were reviewed...
April 25, 2017: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28444210/initial-shunt-type-at-the-norwood-operation-impacts-myocardial-function-in-hypoplastic-left-heart-syndrome%C3%A2
#3
Hanna K Ruotsalainen, Jaana Pihkala, Jukka Salminen, Lisa K Hornberger, Heikki Sairanen, Tiina Ojala
OBJECTIVES: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: A population-based cohort of 63 Finnish children with HLHS (BT n  = 23, RV-PA n  = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0...
April 24, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28439384/characteristics-of-neonatal-pneumothorax-in-saudi-arabia-three-years-experience
#4
Abdulrahman Al Matary, Humariya H Munshi, Sameh Abozaid, Mostafa Qaraqei, Tariq A Wani, Amani K Abu-Shaheen
OBJECTIVES: To identify the incidence, clinical characteristics, predisposing factors, morbidity, and mortality among hospitalized neonates with pneumothorax. METHODS: The records of 2 204 infants admitted to the neonatal intensive care unit at King Fahad Medical City, Saudi Arabia, between 2011 and 2014 were reviewed. All newborns hospitalized in the neonatal intensive care unit with pneumothorax were included in the study. Participants were evaluated for baseline characteristics, predisposing factors of neonatal pneumothorax (NP), accompanying disorders, and mortality...
March 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28431733/neonatal-repair-of-persistent-fifth-aortic-arch-coarctation-and-interrupted-fourth-aortic-arch
#5
Enrico Cetrano, Angelo Polito, Matteo Trezzi, Adriano Carotti
Persistent left fifth aortic arch is a rare anomaly often associated with aortic coarctation. We report the case of a newborn presenting with signs of duct-dependent aortic coarctation. Echocardiography showed an interrupted fourth aortic arch, persistent left fifth aortic arch associated with aortic coarctation, and a restrictive arterial duct. Arch repair was accomplished using the fifth aortic arch as an in situ flap to enlarge the hypoplastic fourth aortic arch associated with coarctectomy and extended end-to-end anastomosis...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28431723/successful-biventricular-conversion-late-after-primary-one-and-one-half-ventricle-repair
#6
Takaya Hoashi, Masataka Kitano, Koji Kagisaki, Hajime Ichikawa
A 6-year-old girl with unbalanced atrioventricular septal defect, hypoplastic right ventricle, and severe common atrioventricular valve regurgitation developed patient-prosthetic mismatch. At 6 months old, she underwent primary one and one-half ventricle repair and replacement of left side atrioventricular valve. A catheter examination showed that her right ventricular end-diastolic volume increased from 39.4 mL/m(2) 1 year after to 70 mL/m(2) 3.5 years after the previous surgery. Thus, at the timing of redo left side atrioventricular valve replacement, she was converted successfully to biventricular circulation...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28426528/comparative-analysis-of-multicolor-flow-cytometry-and-immunohistochemistry-for-the-detection-of-disseminated-tumor-cells
#7
Eszter Szánthó, Bettina Kárai, Gergely Ivády, Judit Bedekovics, István Szegedi, Miklós Petrás, György Ujj, Anikó Ujfalusi, Csongor Kiss, János Kappelmayer, Zsuzsanna Hevessy
Disseminating cells of a primary solid tumor may represent the origin of metastases and relapses. We aimed at comparing the diagnostic efficacy of multicolor flow cytometry (MFC) and morphology/immunohistochemistry (IHC) in the detection of disseminated tumor cells in the bone marrow (BM) and body fluids of patients with solid tumors, and in pediatric neuroblastoma cases. We investigated 72 samples retrospecively from 50 patients by MFC. Morphology/IHC data were available in 48 cases. In the first cohort, 36 samples derived from 34 patients with various forms of suspected and proven solid tumors and in the second cohort, 36 samples of 16 children with suspected and proven neuroblastoma were analyzed at diagnosis or during follow-up in a 4-color setting by MFC, and the results were compared with those obtained by IHC...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28419546/connexin43-and-runx2-interact-to-affect-cortical-bone-geometry-skeletal-development-and-osteoblast-and-osteoclast-function
#8
Atum M Buo, Ryan E Tomlinson, Eric R Eidelman, Max Chason, Joseph P Stains
The coupling of osteoblasts and osteocytes by connexin43 (Cx43) gap junctions permits the sharing of second messengers that coordinate bone cell function and cortical bone acquisition. However, details of how Cx43 converts shared second messengers into signals that converge onto essential osteogenic processes are incomplete. Here, we use in vitro and in vivo methods to show that Cx43 and Runx2 functionally interact to regulate osteoblast gene expression and proliferation, ultimately affecting cortical bone properties...
April 17, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28417904/zebrafish-as-an-alternative-vertebrate-model-for-investigating-developmental-toxicity-the-triadimefon-example
#9
Maria Zoupa, Kyriaki Machera
Triadimefon is a widely used triazole fungicide known to cause severe developmental defects in several model organisms and in humans. The present study evaluated in detail the developmental effects seen in zebrafish embryos exposed to triadimefon, confirmed and expanded upon previous phenotypic findings and compared them to those observed in other traditional animal models. In order to do this, we exposed embryos to 2 and 4 µg/mL triadimefon and evaluated growth until 120 h post-fertilization (hpf) through gross morphology examination...
April 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28413549/challenges-in-a-case-of-ophthalmic-artery-aneurysm-associated-with-abnormal-internal-carotid-arteries
#10
Eduard B Dinca, Felix Brehar, Andrei Giovani, Alexandru V Ciurea
Ophthalmic artery aneurysms account for 5% of all cerebral aneurysms and are an important cause of morbidity and mortality related to subarachnoid hemorrhage. The diagnosis is often made only when the aneurysm is large enough to become symptomatic. They remain technically challenging for both neurosurgeon and interventional radiologist. We present the case of a 62-year-old woman admitted for transient loss of consciousness, followed by generalized tonic-clonic seizures. Computed tomography (CT) showed a subarachnoid hemorrhage (SAH), clinically graded as Hunt and Hess III...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28410632/evolution-of-left-ventricular-size-in-late-survivors-of-surgery-for-hypoplastic-left-heart-syndrome
#11
Markus Bjurbom, Ajay J Iyengar, Florian Moenkemeyer, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
BACKGROUND: Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. METHODS: Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after...
April 12, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#12
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28406545/diagnosing-nocturnal-paroxysmal-hemoglobinuria-a-single-center-4-year-experience
#13
T Mercier, T Devos, M Mukovnikova, N Boeckx
INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease and can present as a wide range of signs and symptoms. As such, the indication for diagnostic testing for PNH is not always straightforward. Therefore, we analyzed all first-time samples tested over a 56-month period to determine the clinical settings with a high probability of detecting a PNH clone. METHODS: We retrospectively analyzed 323 first-time PNH flow cytometry tests, including LDH, cytopenias, direct antiglobulin test (DAT), and clinical indication for testing as available at the time of testing...
April 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28402400/unplanned-cardiac-reoperations-and-interventions-during-long-term-follow-up-after-the-norwood-procedure%C3%A2
#14
Eva Sames-Dolzer, Gregor Gierlinger, Michaela Kreuzer, Julia Schrempf, Roland Gitter, Christoph Prandstetter, Gerald Tulzer, Rudolf Mair
OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients...
April 11, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28399114/lung-hypoplasia-in-newborn-rabbits-with-a-diaphragmatic-hernia-affects-pulmonary-ventilation-but-not-perfusion
#15
Andreas W Flemmer, Marta Thio, Megan J Wallace, Katie Lee, K Marcus J Kitchen, Lauren Kerr, Charles C Roehr, Andreas Fouras, Richard Carnibella, Jaccques J Jani, Philip DeKoninck, Arjan B tePas, James T Pearson, Stuart B Hooper
BACKGROUND: A congenital diaphragmatic hernia can result in severe lung hypoplasia that increases the risk of morbidity and mortality after birth, but little is known about the cardiorespiratory transition at birth. METHODS: Using phase contrast X-ray imaging and angiography, we examined the cardiorespiratory transition at birth in rabbit kittens with diaphragmatic hernias (DH). Surgery was performed on pregnant New Zealand white rabbits (n=18) at 25 days gestation to induce a left-sided DH...
April 11, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28396980/median-pericallosal-artery-with-double-proximal-origin-case-report-and-clinical-consideration
#16
Maryna A Kornieieva, Azmy M Hadidy, Suzan H Hinno
A 76-year-old male patient underwent magnetic resonance angiography of the head and neck vessels due to a recent incident of transitory ischemic attack. Cerebral angiogram uncovered the double proximal origin of the median unpaired pericallosal artery from the duplicated anterior communicating complex. The vessel curved around the corpus callosum and irrigated the paracentral lobule and the medial parietal cortical areas. The main trunks of the anterior cerebral arteries showed a branching pattern of the marginal callosal arteries, supplying orbital and frontal territories...
April 10, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28393581/the-effect-of-transport-on-the-physiologic-stability-of-neonates-withductal-dependent-single-ventricle-lesions
#17
Silvestre R Duran, Sanjeev Aggarwal, Girija Natarajan
OBJECTIVE: To compare the status of infants with hypoplastic left heart syndrome (HLHS) or pulmonary atresia-hypoplastic right heart (PA-HRH) before and following transport using the validated Transport Risk Index of Physiologic Stability (TRIPS) score. METHODS: In this retrospective review of infants with HLHS or PA-HRH transported to a Children's Hospital by a pediatric transport team, an increase in TRIPS score (temperature, blood pressure, respiratory status, and response to stimuli) following transport was defined as deterioration...
April 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28391520/neonatal-repair-of-left-atrial-diverticulum-with-gigantic-thrombus-without-cardiopulmonary-bypass
#18
Akihiko Higashida, Takaya Hoashi, Heima Sakaguchi, Hajime Ichikawa
A 5-day-old neonate with coarctation of the aorta, hypoplastic aortic arch, large apical muscular ventricular septal defect, and patent ductus arteriosus developed pulmonary over-circulation and systemic hypoperfusion underwent bilateral pulmonary artery banding through median sternotomy as a part of hybrid stage I palliation. At operation, left atrial diverticulum with gigantic thrombus formation at the base of the left atrial appendage was incidentally detected by intraoperative direct echocardiography, and therefore, was successfully resected with the whole thrombus inside it without use of cardiopulmonary bypass...
April 8, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28390600/sox10-mutation-causes-waardenburg-syndrome-associated-with-distinctive-phenotypic-features-in-an-iranian-family-a-clue-for-phenotype-directed-genetic-analysis
#19
Nazanin Jalilian, Mohammad Amin Tabatabaiefar, Hossein Alimadadi, Mohammad Reza Noori-Daloii
BACKGROUND: Waardenburg syndrome (WS) is a neurocristopathy characterized by hearing impairment and pigmentary disturbances in hair, eyes, and skin. WS is clinically heterogeneous and can be subdivided into four major types (WS1-WS4) where WS4 or Shah-Waardenburg is diagnosed when WS2 is accompanied by Hirschsprung disease (HD). Mutations of SOX10, EDN3/EDNRB have been identified in association with WS4. This study was aimed to determine the pathogenic variant in an Iranian pedigree affected with WS4...
May 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28387614/reduction-of-atlantoaxial-dislocation-prevented-by-pathological-position-of-the-transverse-ligament-in-fixed-irreducible-os-odontoideum-operative-illustrations-and-radiographic-correlates-in-41-patients
#20
Brian J Dlouhy, Bruno A Policeni, Arnold H Menezes
OBJECTIVE Os odontoideum (OO) is a craniovertebral junction (CVJ) abnormality in which an ossicle (small bone) is cranial to a hypoplastic dens by a variable gap. This abnormality can result in instability, which may be reducible or irreducible. What leads to irreducibility in OO is unclear. Therefore, the authors sought to better understand the causes of irreducibility in OO. METHODS A retrospective review was conducted, which identified more than 200 patients who had undergone surgical treatment for OO between 1978 and 2015 at the University of Iowa Hospitals and Clinics...
April 7, 2017: Journal of Neurosurgery. Spine
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