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https://www.readbyqxmd.com/read/29470938/diagnostic-performance-of-mr-elastography-for-liver-fibrosis-in-children-and-young-adults-with-a-spectrum-of-liver-diseases
#1
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
https://www.readbyqxmd.com/read/29468569/illness-identity-in-adults-with-a-chronic-illness
#2
Leen Oris, Koen Luyckx, Jessica Rassart, Liesbet Goubert, Eva Goossens, Silke Apers, Seher Arat, Joris Vandenberghe, René Westhovens, Philip Moons
The present study examines the concept of illness identity, the degree to which a chronic illness is integrated into one's identity, in adults with a chronic illness by validating a new self-report questionnaire, the Illness Identity Questionnaire (IIQ). Self-report questionnaires on illness identity, psychological, and physical functioning were assessed in two samples: adults with congenital heart disease (22-78 year old; n = 276) and with multisystem connective tissue disorders (systemic lupus erythematosus or systemic sclerosis; 17-81 year old; n = 241)...
February 21, 2018: Journal of Clinical Psychology in Medical Settings
https://www.readbyqxmd.com/read/29468473/clinical-aspects-of-myocardial-fibrosis-in-adults-with-ebstein-s-anomaly
#3
Aleksandra Ciepłucha, Olga Trojnarska, Anna Kociemba, Magdalena Łanocha, Mikolaj Barczynski, Szymon Rozmiarek, Lucyna Kramer, Malgorzata Pyda
Heart failure and arrhythmia are common complications in adults with Ebstein's anomaly. They may result not only from hemodynamic alterations, but also from myocardial fibrosis. Late gadolinium enhancement (LGE) by CMR enables the evaluation of myocardial fibrosis. The aim of the study was to asses the presence of LGE and its relation to clinical outcome. We studied a group of 37 unoperated adults aged 43.0 ± 14.4 years with Ebstein's anomaly from the congenital heart disease outpatient clinic. Study protocol included: cardiopulmonary test, assessment of supraventricular arrhythmia (SVA), and CMR with evaluation of cardiac chambers' morphology and function, and presence of LGE...
February 21, 2018: Heart and Vessels
https://www.readbyqxmd.com/read/29465640/context-appropriate-interventions-to-prevent-syphilis-a-narrative-review
#4
Thomas A Peterman, Susan Cha
BACKGROUND: The success of an intervention to prevent syphilis will depend on the context and the goal of the intervention. To help programs choose interventions, we reviewed major changes in context and types of interventions that may be effective. METHODS: We reviewed the literature on the changing context of syphilis in the United States and interventions to prevent syphilis, focusing on papers that included evidence of effectiveness. RESULTS: Populations acquiring syphilis are constantly changing...
February 13, 2018: Sexually Transmitted Diseases
https://www.readbyqxmd.com/read/29465639/multi-state-syphilis-outbreak-among-american-indians-2013-2015
#5
Virginia B Bowen, Thomas A Peterman, Dinorah L Calles, Antoine Thompson, Robert Kirkcaldy, Melanie Taylor
This article summarizes a multi-state outbreak of heterosexual syphilis, including 134 cases of syphilis in adults and adolescents and at least two cases of congenital syphilis, which occurred on an American Indian reservation in the United States during 2013-2015. In addition to providing salient details about the outbreak, the article seeks to document the case-finding and treatment activities undertaken, their relative success or failure, and the lessons learned from a coordinated, multiagency response. Of 134 adult cases of syphilis, 40% were identified by enhanced, interagency contact tracing and partner services; 26% through symptomatic testing; and 16% through screening of asymptomatic individuals as the result of an electronic medical record (EMR) screening prompt...
February 13, 2018: Sexually Transmitted Diseases
https://www.readbyqxmd.com/read/29464136/anesthetic-management-of-pheochromocytoma-resection-in-adults-with-single-ventricle-physiology
#6
Giancarlo Suffredini, Natalia Diaz-Rodriguez, Krishnan Chakravarthy, Aarti Mathur, Heather K Hayanga, Steve M Frank, Richard E Ringel, Stephen Freiberg, Viachaslau M Barodka, Jochen Steppan
Survival rates for patients with palliated congenital heart disease are increasing, and an increasing number of adults with cyanotic congenital heart disease (CCHD) might require surgical resection of pheochromocytoma-paraganglioma (PHEO-PGL). A recent study supports the idea that patients with a history of CCHD and current or historical cyanosis might be at increased risk for developing PHEO-PGL. We review the anesthetic management of two adults with single-ventricle physiology following Fontan palliation presenting for PHEO-PGL resection and review prior published case reports...
December 8, 2017: Curēus
https://www.readbyqxmd.com/read/29463466/right-ventricular-dysfunction-in-congenitally-corrected-transposition-of-the-great-arteries-and-risk-of-ventricular-tachyarrhythmia-and-sudden-death
#7
Suraj Kapa, Vaibhav Vaidya, David O Hodge, Christopher J McLeod, Heidi M Connolly, Carole A Warnes, Samuel J Asirvatham
BACKGROUND: It is unknown whether systemic right ventricular (SRV) dysfunction confers increased risk for sudden death in congenitally corrected transposition of the great arteries (ccTGA). We sought to define risk of ventricular arrhythmias and sudden death attributable to systemic right ventricular dysfunction in ccTGA. METHODS: The study cohort is comprised of adult patients with a diagnosis of ccTGA. Clinical information and clinical outcomes including ICD therapy, incidence of VT/VF, and cause of death were reviewed...
January 31, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29463390/managing-congenital-heart-disease-in-the-obstetric-patient
#8
REVIEW
Heidi M Connolly
OBJECTIVE: Cardiovascular disease is the major cause of pregnancy-related maternal mortality in the United States, and congenital heart disease (CHD) is the most common form of structural heart disease affecting women of childbearing age. Most females born with CHD will reach childbearing age and consider pregnancy. Adult CHD and maternal-fetal medicine (MFM) specialists managing women with CHD should provide preconception counseling, cardiovascular risk assessment prior to pregnancy that estimates maternal and fetal risk, management during pregnancy, and in the peripartum period and also know the potential complications and special circumstances that may occur in the post-partum period...
February 2018: Seminars in Perinatology
https://www.readbyqxmd.com/read/29463283/neonatal-diagnosis-of-isolated-absence-of-the-right-pulmonary-artery-a-case-report-and-review-of-the-literature
#9
Akamin Raymond, Ettore Pedretti, Giuseppina Privitera, Cristina Cicero, Giacomo Biasucci
BACKGROUND: Unilateral absence of the pulmonary artery (UAPA) is a rare congenital malformation often associated with other cardiac anomalies; however it may occur as an isolated lesion. Isolated absence of the right pulmonary artery is twice more frequent than that of the left pulmonary artery. Patients with isolated UAPA are usually asymptomatic at birth; thereafter they may develop a progression of symptoms such as exercise intolerance, dyspnea, chest pain, hemoptysis and recurrent pulmonary infections...
February 20, 2018: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29460643/para-ovarian-adrenal-rest-tumors-gynecologic-manifestations-of-untreated-congenital-adrenal-hyperplasia
#10
Jessica M Sisto, Fong W Liu, Mitchell E Geffner, Michael L Berman
Congenital adrenal hyperplasia (CAH) is an inherited disorder of adrenal steroidogenesis often diagnosed in infancy. Gynecologists may encounter adult patients with CAH due to the clinical effects of increased androgens, e.g. hirsutism, clitoromegaly, oligomenorrhea, or, rarely, pelvic masses. This case report reviews the association of para-ovarian adrenal rest tumors with CAH, and the role of gynecologists in their evaluation and treatment. A 23-year-old woman with CAH (21-hydroxyase deficiency) untreated for the past 5 years presented with a pelvic mass and elevated serum testosterone (1433 ng/dL) and plasma ACTH (1117 pg/mL)...
February 20, 2018: Gynecological Endocrinology
https://www.readbyqxmd.com/read/29459613/complete-annular-pancreas-with-concurrent-entero-pancreatico-biliary-symptoms-in-adult-a-case-report
#11
M M Chowdhury, A A Ullah, R Karim, A Ahmed, S Mohammed, S A Sobhan, A A Farmidi, F Zuwaida, R Pradan, R Mahmud, B Rai, S Pervin, R Habib
Complete Annular pancreas (AP) is a rare congenital anomaly, often presented and operated at the early age of life. Adult presentation group usually presents with either biliary or duodenal or pancreatic symptoms. We report a case of 43 years old female presenting with concurrent enteric, biliary and pancreatic symptoms admitted on April 2016 in Hepatobiliary and Pancreatic Surgery Department of BSMMU, Dhaka, Bangladesh. A complete type of annular pancreas with partial duodenal stenosis and dilated common bile duct was observed during laparotomy...
January 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29458311/double-superior-vena-cava-presentation-of-two-cases-and-review-of-the-literature
#12
Christos Farazi-Chongouki, Ioannis Dalianoudis, Anestis Ninos, Pantelis Diamantopoulos, Dimitrios Filippou, Stefanos Pierrakakis, Panagiotis Skandalakis
INTRODUCTION: Various anomalies in the development of the great thoracic veins of the embryo can be incidentally discovered in the normal adult. Duplication of superior vena cava (SVC) is a rare abnormality, but the most common thoracic venous congenital anomaly. Case reports-methods: We present two cases in the intensive care unit of our hospital, of asymptomatic patients who underwent an uneventful central line placement in the left subclavian vein. The track of the catheter, as shown in the X-ray, was misplaced to the left of the aorta and further investigation with computed tomography angiography confirmed a persistent left SVC...
February 19, 2018: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/29457362/a-multinational-observational-investigation-of-illness-perceptions-and-quality-of-life-among-patients-with-a-fontan-circulation
#13
Christina E Holbein, Nicholas D Fogleman, Kevin Hommel, Silke Apers, Jessica Rassart, Philip Moons, Koen Luyckx, Maayke A Sluman, Junko Enomoto, Bengt Johansson, Hsiao-Ling Yang, Mikael Dellborg, Raghavan Subramanyan, Jamie L Jackson, Werner Budts, Adrienne H Kovacs, Stacey Morrison, Martha Tomlin, Kathy Gosney, Alexandra Soufi, Katrine Eriksen, Corina Thomet, Malin Berghammer, Luis Alday, Edward Callus, Susan M Fernandes, Maryanne Caruana, Samuel Menahem, Stephen C Cook, Gwen R Rempel, Kamila White, Paul Khairy, Shelby Kutty, Gruschen Veldtman
OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents...
February 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29457357/improving-outpatient-advance-care-planning-for-adults-with-congenital-or-pediatric-heart-disease-followed-in-a-pediatric-heart-failure-and-transplant-clinic
#14
Lindsay A Edwards, Christine Bui, Antonio G Cabrera, Jill Ann Jarrell
OBJECTIVE: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology. DESIGN: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions. PATIENTS AND SETTING: Patients ≥18 years of age seen in the HF and Transplant Clinic for follow-up visit were included in analysis...
February 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29456231/late-onset-choreoathetotic-syndrome-following-heart-surgery-in-adults-with-end-stage-renal-disease
#15
Mohamed Amine Hamzi, Kawtar Hassani, Driss El Kabbaj
Choreoathetotic syndrome is a rare complication of open cardiac surgery that is seen usually in children after surgery for congenital cardiac anomalies. Here, we report two cases of adult patients with end-stage renal disease (ESRD) on regular hemodialysis who developed acute choreoathetotic syndrome few days after cardiac surgeries under cardiopulmonary bypass (CPB). Improvement was seen after an interval with complete resolution in one case. Investigations of the cause have been noncontributory. Long CPB time seems to be the main identified risk factor in these cases...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29455367/adolescents-and-young-adults-living-with-congenital-heart-disease-coping-stress-reactivity-and-emotional-functioning
#16
Jennifer D Monti, Jamie L Jackson, Kathryn Vannatta
Living with congenital heart disease (CHD) presents survivors with numerous stressors, which may contribute to emotional problems. This study examined (a) whether coping with CHD-related stress predicts symptoms of depression and anxiety, and (b) whether associations between coping and emotional distress are moderated by involuntary stress reactivity. Adolescents and young adults diagnosed with CHD (Mage = 26.4) were recruited from pediatric and adult outpatient cardiology clinics. Participants (N = 168) completed online self-report measures...
February 17, 2018: Journal of Clinical Psychology in Medical Settings
https://www.readbyqxmd.com/read/29455160/children-and-adolescents-experiences-of-primary-lymphoedema-semistructured-interview-study
#17
Camilla S Hanson, Johanna Newsom, Davinder Singh-Grewal, Nicholas Henschke, Margaret Patterson, Allison Tong
BACKGROUND: Congenital lymphoedema is a lifelong condition that has detrimental physical and psychosocial outcomes for young patients and burdensome treatment responsibilities that may hamper patients' motivation for self-management. There is limited research from the perspective of young people with primary lymphoedema. We aimed to describe the experiences and views of children and adolescents with lymphoedema to inform patient-centred practice. METHODS: Twenty patients (aged 8-21 years) with primary lymphoedema were purposively sampled from two paediatric clinics in Sydney, Australia, to participate in a semistructured interview...
February 17, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29454526/predicting-sexual-problems-in-young-adults-with-an-anorectal-malformation-or-hirschsprung-disease
#18
M J Witvliet, S van Gasteren, D van den Hondel, E Hartman, Lwe van Heurn, Afw van der Steeg
AIM: The aim of this study was to examine the prevalence of sexual dysfunction and distress and to assess whether sexual functioning could be predicted by psychosocial factors in childhood and adolescence in patients with an anorectal malformation or Hirschsprung disease. MATERIAL AND METHODS: In 1998 patients completed a psychosocial questionnaire: The Self-Perception profile. To assess the prevalence of sexual distress and sexual functioning in adulthood (2015) the Female Sexual Function Index (FSFI), The Female Sexual Distress Scale (FSDS) and the International Index of Erectile Functioning (IIEF) were used...
January 31, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29453776/syndromic-sebaceous-nevus-current-findings
#19
Oumama El Ezzi, Anthony S de Buys Roessingh, Michèle Bigorre, Guillaume Captier
BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan...
February 16, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29453587/trimorphic-extreme-clubfoot-deformities-and-their-management-by-triple-surgical-skin-expanders-dolar-dolarz-and-dolarz-e-evidence-based-mega-corrections-without-arthrodesis
#20
Rattan L Mittal
AIM/PURPOSE: Extreme congenital club foot deformities are common in developing countries, presenting at birth, persisting in children, adolescents and adults; as untreated/under-corrected by conservative and/or surgical means. Scores of confusing names exist in literature for such deformities with no good treatment available; mostly advocating unacceptable arthrodesis. The author researched this grey area for more than 40 years and successfully innovated improved surgical corrections, more acceptable to patients...
February 17, 2018: International Orthopaedics
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