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https://www.readbyqxmd.com/read/28719384/anesthetic-management-of-an-infant-with-postnatally-diagnosed-tracheal-agenesis-undergoing-tracheal-reconstruction-a-case-report
#1
Brittany L Willer, Kayla G Bryan, Daiva E Parakininkas, Michael R Uhing, Susan R Staudt, Kathleen M Dominguez, Michael E McCormick, Michael E Mitchell, John C Densmore, Keith T Oldham, Richard J Berens
A term infant born cyanotic failed multiple intubation attempts and tracheostomy placement. After esophageal intubation resulted in the ability to ventilate, he was presumed to have tracheal agenesis and distal bronchoesophageal fistula. He was transferred to our institution where he was diagnosed with Floyd Type II tracheal agenesis. He underwent staged tracheal reconstruction. He was discharged to home at 4 months of age with a tracheostomy collar, cervical spit fistula, and gastrostomy tube. He represents the sole survivor-to-discharge of tracheal agenesis in the United States...
July 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28713984/downregulation-of-microrna%C3%A2-199a%C3%A2-5p-protects-cardiomyocytes-in-cyanotic-congenital-heart-disease-by-attenuating-endoplasmic-reticulum-stress
#2
Yang Zhou, Wei-Kun Jia, Zhao Jian, Liang Zhao, Chen-Cheng Liu, Yong Wang, Ying-Bin Xiao
Chronic hypoxia is a key pathological change in patients with cyanotic congenital heart defect (CCHD). It has been demonstrated that enhanced myocardial unfolded protein response (UPR) increases the capacity to buffer endoplasmic reticulum (ER) stress and to avoid subsequent apoptosis caused by the hypoxia that underlies CCHD. The present study was performed to determine the regulatory role of microRNAs (miRNAs) in this cytoprotective UPR process. The results revealed that miR‑199a‑5p was markedly downregulated in the cardiac tissue of patients with CCHD and in human myocardial cells cultured in hypoxic conditions...
July 6, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28701598/the-blalock-and-taussig-shunt-revisited
#3
REVIEW
Usha Kiran, Shivani Aggarwal, Arin Choudhary, B Uma, Poonam Malhotra Kapoor
The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28688566/dysphagia-in-healthy-children-characteristics-and-management-of-a-consecutive-cohort-at-a-tertiary-centre
#4
Orysya Svystun, Wendy Johannsen, Rabin Persad, Justine M Turner, Carina Majaesic, Hamdy El-Hakim
OBJECTIVE: Whereas the literature is replete with reports on complex children with dysphagia (DP), the parameters characterizing non-neurologically impaired (NNI) children have been underreported, leaving a substantial knowledge gap. We set to characterize a consecutive cohort of NNI children, their management, and outcomes. METHODS: We undertook a retrospective case series. Children (<18 years old) attending a tertiary multidisciplinary swallowing clinic were eligible...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28685697/does-pharmacological-therapy-still-play-a-role-in-preventing-sudden-death-in-surgically-treated-tetralogy-of-fallot
#5
Gabriele Bronzetti, Maurizio Brighenti, Marco Bonvicini
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, with a familial recurrence risk of 3%. Despite the performing of an optimal surgical repair, TOF patients may feature a poor medium and long-term survival rate: atrial re-entrant tachycardia will develop in more than 30% of patients and high-grade ventricular arrhythmias will be seen in about 10% of patients. These life-threatening arrhythmias and consequent sudden death continue to represent serious complications following TOF repair...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28680470/a-case-of-successfully-managed-pregnancy-in-a-patient-with-complex-cyanotic-congenital-heart-disease
#6
J Y Liu, W K Tan, E L Tan, J L Tan, L K Tan
Medical advances have increased survival of patients with congenital heart disease. However, cardiac disease in pregnancy carries significant maternal and fetal risks, posing enormous challenges to obstetricians. Cyanotic congenital heart disease is associated with maternal complications such as arrhythmias, thromboembolic events and death. Fetal complications include small for gestational age, miscarriage and prematurity. Cyanotic congenital heart disease patients who continue their pregnancies require holistic multidisciplinary team care with early and coordinated planning for delivery...
June 2017: Obstetric Medicine
https://www.readbyqxmd.com/read/28677480/preoperative-venovenous-ecmo-in-an-infant-with-late-diagnosis-of-hypoplastic-left-heart-syndrome
#7
Lena K Eschenbach, Jelena Kasnar-Samprec, Kilian Ackermann, Christian Schreiber, Rudiger Lange, Julie Cleuziou
Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28674625/gender-related-differences-in-self-reported-dental-care-in-adults-with-congenital-heart-disease-at-increased-risk-of-infective-endocarditis
#8
Susann Schmidt, Marlies Ramseier-Hadorn, Corina Thomet, Kerstin Wustmann, Markus Schwerzmann
OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of infective endocarditis (IE). Women with CHD have a lower IE risk, potentially due to gender-related differences in dental care. We aimed to assess self-reported dental hygiene measures in adults with CHD, and to identify factors associated with good oral hygiene. METHODS AND RESULTS: Descriptive study includes 187 adults with CHD at increased risk of IE. The patients' IE knowledge was assessed using an adapted version of the Leuven Knowledge Questionnaire for CHD...
2017: Open Heart
https://www.readbyqxmd.com/read/28664753/atypical-cyanotic-breath-holding-spells-in-an-infant-with-16p11-2-microdeletion-syndrome
#9
Jayalakshmi Bhat, Jose Martinez, Paul Maertens
No abstract text is available yet for this article.
June 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/28663364/platelet-count-and-mean-platelet-volume-predict-outcome-in-adults-with-eisenmenger-syndrome
#10
Agustin C Martin-Garcia, Deepa Rj Arachchillage, Aleksander Kempny, Rafael Alonso-Gonzalez, Ana Martin-Garcia, Anselm Uebing, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Pedro Luis Sanchez, Konstantinos Dimopoulos, Michael A Gatzoulis
OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years...
June 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28651675/responses-of-mothers-of-children-with-chd-quality-of-life-anxiety-and-depression-parental-attitudes-family-functionality
#11
Fatos Alkan, Tamay Sertcelik, Sermin Yalın Sapmaz, Erhan Eser, Senol Coskun
Introduction The aim of this study was to evaluate the anxiety and depression status, family functions, parenting attitudes, and quality of life in the mothers of children with CHD. METHOD: The study enrolled 120 mothers: 40 of children with cyanotic CHD, 40 of children with non-cyanotic CHD, and 40 of healthy controls. Short Form-36 for quality of life, Hospital Anxiety-Depression Scale for anxiety and depression, Family Assessment Device for the detection of problems affecting family functions, and Parental Attitude Research Instrument for measuring child-rearing attitudes were used in the study...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28634172/identifying-an-oxygenation-index-threshold-for-increased-mortality-in-acute-respiratory-failure
#12
Brandon G Hammond, Pamela Garcia-Filion, Paul Kang, Mounica Y Rao, Brigham C Willis, Heidi J Dalton
BACKGROUND: The objective of this work was to examine current oxygenation index (OI) data and outcomes using electronic medical record data to identify a specific OI value associated with mortality. METHODS: This study was a retrospective electronic medical record data review from the pediatric ICU of Phoenix Children's Hospital, with data mining for variables to calculate OIs on subjects age 1 month to 20 y mechanically ventilated >24 h, excluding those with known intracardiac shunts or cyanotic heart disease...
June 20, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28632506/adenotonsillectomy-for-the-management-of-pulmonary-hypertension-in-a-patient-with-complex-congenital-heart-disease-a-case-report
#13
Adam C Adler, Yuan-Jiun Nicole Chao
Pulmonary hypertension is a feared complication in congenital heart disease patients. Patients with pulmonary hypertension are at risk for major perioperative cardiopulmonary complications when undergoing any surgical procedure, especially airway and laparoscopic procedures. We present the anesthetic management for a 2-year old with Down syndrome and complex cyanotic congenital heart disease undergoing tonsillectomy and adenoidectomy for severe obstructive sleep apnea.
June 19, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28628576/the-insight-from-foresight-near-infrared-spectroscopy-in-cyanotic-congenital-heart-disease
#14
Adam C Adler, Stephen A Stayer
No abstract text is available yet for this article.
July 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28623384/the-role-of-cardiopulmonary-exercise-testing-for-decision-making-in-patients-with-repaired-tetralogy-of-fallot
#15
REVIEW
Frederic Dallaire, Rachel M Wald, Ariane Marelli
Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital heart disease...
June 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28619123/chd-and-respiratory-syncytial-virus-global-expert-exchange-recommendations
#16
Robert M R Tulloh, Constancio Medrano-Lopez, Paul A Checchia, Claudia Stapper, Naokata Sumitomo, Matthias Gorenflo, Eun Jung Bae, Antonio Juanico, Juan M Gil-Jaurena, Mei-Hwan Wu, Talal Farha, Ali Dodge-Khatami, Rocky Tsang, Gerard Notario, Colleen Wegzyn
BACKGROUND: Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings. METHODS: An international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology...
June 16, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28614229/familial-clustering-of-congenital-deafness-patent-ductus-arteriosus-eisenmenger-complex-and-differential-cyanosis-a-case-report
#17
Ting-Wei Lin, Chih-Wei Tseng, Chi-Yao Huang, Kuo-Yang Wang, Kae-Woei Liang
RATIONALE: Few studies had reported syndromes that include patent ductus arteriosus (PDA) with Eisenmenger syndrome and congenital deafness clustered in male siblings without facial, skeletal, or mental abnormalities. PATIENT CONCERNS: Two brothers, who were deaf and had PDA with Eisenmenger complex, were first seen at our Cardiology clinic at the ages of 25 and 41, respectively. They presented with progressive dyspnea on exertion. Upon physical examination, both brothers had clubbing and/or cyanotic toes, normal fingers, and without facial, skeletal, ophthalmological, or mental abnormalities...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28601176/cyanotic-congenital-heart-disease-essential-primer-for-the-practicing-radiologist
#18
REVIEW
Evan J Zucker, Jeffrey L Koning, Edward Y Lee
The cyanotic congenital heart diseases are a rare and heterogeneous group of disorders, often requiring urgent neonatal management. Although echocardiography is the mainstay for imaging, continued technological advances have expanded the role for computed tomography and magnetic resonance imaging, helping to limit invasive cardiac catheterization. In this article, the authors review the broad spectrum of cyanotic congenital heart disease, focusing on the utility of advanced noninvasive imaging modalities while highlighting key clinical features and management considerations...
July 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28598000/peptidomic-analysis-of-maternal-serum-to-identify-biomarker-candidates-for-prenatal-diagnosis-of-tetralogy-of-fallot
#19
Bin Zhuang, Yin Hu, Xuemei Fan, Mengmeng Li, Jingai Zhu, Heng Liu, Li Cao, Dong Liang, Jingjing Zhang, Zhangbin Yu, Shuping Han
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. To identify endogenous peptides possibly involved in the progression of TOF, we performed comparative peptidomic profiling of maternal serum between normal fetuses and fetuses suffering from TOF. A total of 278 differentially expressed peptides, including 94 over-expressed peptides and 184 under-expressed peptides, originating from 227 protein precursors were identified by Liquid Chromatography/Mass Spectrometry (LC/MS) in maternal serum of fetuses with TOF compared to normal controls...
June 9, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28595379/does-the-prophylactic-and-therapeutic-use-of-beta-blockers-in-preoperative-patients-with-tetralogy-of-fallot-significantly-prevent-and-treat-the-occurrence-of-cyanotic-spells
#20
Eliana Fanous, Gábor Mogyorósy
A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Does the use of beta-blockers significantly prevent and treat the occurrence of cyanotic spells in preoperative infants with tetralogy of Fallot?' Altogether, 80 papers were found using the reported search, of which 6 represented the best evidence to answer the clinical question. The author, journal, date, country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated...
June 7, 2017: Interactive Cardiovascular and Thoracic Surgery
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