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https://www.readbyqxmd.com/read/29931985/liver-transplantation-in-hemophilia-a-and-von-willebrand-disease-type-3-perioperative-management-and-post-transplant-outcome
#1
Cristina Alonso Madrigal, Manuela Dobón Rebollo, Viviana Laredo de la Torre, Luis Palomera Bernal, Francisco Agustín García Gil
INTRODUCTION: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). MATERIAL AND METHODS: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3...
June 22, 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29929811/liver-resection-and-transplantation-in-caroli-disease-and-syndrome
#2
R Fahrner, S G C Dennler, F Dondorf, M Ardelt, F Rauchfuss, U Settmacher
INTRODUCTION: Caroli disease (CD) is a congenital dilatation of the intrahepatic bile ducts. In combination with liver fibrosis or cirrhosis, it is called Caroli syndrome (CS). Infectious complications and intrahepatic cholangiocarcinoma are secondary problems. The aim of this study was to analyse the clinical pattern and outcome in patients with CD/CS who underwent liver surgery. METHODS: Between January 2004 and December 2016, 21 patients with CD/CS were treated with liver resection or transplantation (LTX) and post-operative data of patients with CD/CS were retrospectively analysed in a database...
June 18, 2018: Journal of Visceral Surgery
https://www.readbyqxmd.com/read/29929666/catheter-ablation-for-atrial-fibrillation-in-adults-with-congenital-heart-disease-lessons-learned-from-more-than-10-years-following-a-sequential-ablation-approach
#3
Christian Sohns, Jan-Hendrik Nürnberg, Joachim Hebe, Wolfgang Duckeck, Rodolfo Ventura, Frank Konietschke, Cong Cao, Jürgen Siebels, Marius Volkmer
OBJECTIVES: This study aimed to evaluate the impact, safety, and success of atrial fibrillation (AF) ablation in adults with congenital heart disease (ACHD) transferring ablation strategies established in normal hearts. BACKGROUND: AF is an emerging arrhythmia in ACHD. METHODS: Fifty-seven consecutive ACHD (median age 51.1 ± 14.8 years) with drug-refractory AF were analyzed who underwent catheter ablation between 2004 and 2017. CHD was classified according to its complexity into mild (61...
June 2018: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29929532/candidate-plasma-biomarkers-for-predicting-ascending-aortic-aneurysm-in-bicuspid-aortic-valve-disease
#4
Oliver J Harrison, Felino Cagampang, Sunil K Ohri, Christopher Torrens, Kareem Salhiyyah, Amit Modi, Narain Moorjani, Anthony D Whetton, Paul A Townsend
BACKGROUND: Bicuspid aortic valve (BAV) disease is the most common congenital cardiac abnormality affecting 1-2% of the population and is associated with a significantly increased risk of ascending aortic aneurysm. However, predicting which patients will develop aneurysms remains a challenge. This pilot study aimed to identify candidate plasma biomarkers for monitoring ascending aortic diameter and predicting risk of future aneurysm in BAV patients. METHODS: Plasma samples were collected pre-operatively from BAV patients undergoing aortic valve surgery...
June 22, 2018: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29927492/congenital-aqueduct-stenosis-progressive-brain-findings-in-utero-to-birth-in-the-presence-of-severe-hydrocephalus
#5
Beth Kline-Fath, Monica S Arroyo, Maria A Calvo-Garcia, Paul S Horn, Cameron Thomas
PURPOSE: To evaluate the effects of progressive hydrocephalus on the developing brain in a cohort of fetuses diagnosed with congenital aqueduct stenosis (CAS) by comparing prenatal MRI and postnatal imaging. METHODS: This IRB approved single center retrospective review of prenatally diagnosed children with CAS interrogated changes in the brain between prenatal and postnatal imaging and analyzed statistics utilizing SAS ® software package version 9.3. RESULTS: Thirty fetuses imaged at a mean gestational age of 26 weeks had aqueduct obstruction confirmed by postnatal imaging...
June 21, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29926279/the-possibility-of-postmortem-magnetic-resonance-imaging-for-the-diagnostics-of-lung-hypoplasia
#6
U N Tumanova, V M Lyapin, A A Burov, A I Shchegolev, G T Sukhikh
We explored the possibility of using postmortem MRI for the diagnostics of lung hypoplasia associated with innate diaphragmatic hernia in neonates. The main experimental group consisted of 17 newborns with innate diaphragmatic hernia including 10 non-operated newborns and 7 newborns died after surgery for innate diaphragmatic hernia. It was demonstrated that postmortem MRI allows objective quantitative assessment of the absolute and relative dimensions of the lungs in the thoracic cavity and thereby reveals their hypoplasia, which contributes to the determination of tanatogenesis...
June 20, 2018: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/29925723/regenerative-therapy-for-patients-with-congenital-heart-disease
#7
Naritaka Kimura
Congenital heart disease (CHD) is the most common birth defect, affecting 1 in 100 babies. Among CHDs, single ventricle (SV) physiologies, such as hypoplastic left heart syndrome and tricuspid atresia, are particularly severe conditions that require multiple palliative surgeries, including the Fontan procedure. Although the management strategies for SV patients have markedly improved, the prevalence of ventricular dysfunction continues to increase over time, especially after the Fontan procedure. At present, the final treatment for SV patients who develop heart failure is heart transplantation; however, transplantation is difficult to achieve because of severe donor shortages...
June 19, 2018: Keio Journal of Medicine
https://www.readbyqxmd.com/read/29925120/-occupational-therapy-in-orthopedics-and-accident-surgery
#8
Ute Hirsch, Jörg Zobel
Ergotherapy is part of the multidisciplinary treatment concept in the field of orthopedics and trauma surgery. The occupational therapist treats patients together with doctors, nurses, physiotherapists, psychologists, social workers/pedagogues, speech therapists and other professional groups · with congenital malformations,. · with chronic diseases,. · with degenerative diseases,. · after accidental injuries or surgical interventions.. Therapy takes place in terms of client centricity and sees the patient as a full member of the team...
June 2018: Die Rehabilitation
https://www.readbyqxmd.com/read/29924007/intravenous-anesthesia-with-nitroglycerin-inhalation-for-surgical-abortion-in-a-patient-with-severe-congenital-heart-disease-and-low-oxygen-saturation-a-case-report
#9
Zhengfeng Gu, Lian Xin, Zhiping Wang, Jun Wang
INTRODUCTION: Congenital heart disease and pulmonary arterial hypertension are perilous to a gravida for the high morbidity and mortality. CASE PRESENTATION: We report an extremely rare case of a 27-year-old gravida with congenital heart disease and severe pulmonary arterial hypertension of 115 mmHg. Arterial blood gas analysis revealed her oxygen saturation (SpO2) of 67.8% and oxygen partial pressure of 40.0 mmHg, which were severely low. The patient was diagnosed as having gestation combined with congenital heart disease, severe pulmonary arterial hypertension, and hypoxemia...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29923988/coexistence-of-double-gallbladder-with-cholangiocarcinoma-a-case-report
#10
Wei Chen, Ruoling Han
RATIONALE: Gallbladder duplication is a rare congenital disorder, which could cause an increasing risk of complications during surgery. The coexistence of cholangiocarcinoma with double gallbladder is extremely rare, which might lead to an even higher possibility of misdiagnosis and postsurgery complications. PATIENT CONCERNS: A 58-year-old female was presented with abdominal pain and jaundice. Abdominal ultrasonography showed duplication of gallbladder, one of which with a thickened wall and a rough surface...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29923964/revision-surgery-for-undercorrected-blepharoptosis-after-frontalis-sling-operation-using-autogenous-fascia-lata
#11
Ju-Hyang Lee, Kyung In Woo, Yoon-Duck Kim
PURPOSE: Undercorrected blepharoptosis can be encountered after frontalis sling operation. Revision surgery for undercorrection has commonly involved introducing a new sling material. We describe and evaluate a simple surgical technique to correct undercorrection by adjusting preexisting fascia. METHODS: This is a retrospective interventional case series of patients undergoing sling revision between February 2010 and February 2017. Skin incision was made on the previous incision line...
June 18, 2018: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29923938/predictive-factors-for-central-line-associated-bloodstream-infections-in-pediatric-cardiac-surgery-patients-with-chylothorax
#12
Stephanie G Waterhouse, Jeffrey E Vergales, Mark R Conaway, Laura Lee
OBJECTIVES: To assess the prevalence of central line-associated bloodstream infections in pediatric patients with and without chylothorax after cardiac surgery and identify risk factors that predict those patients at highest risk for developing a central line-associated bloodstream infection. DESIGN: Retrospective single-center cohort study. SETTING: A PICU located within a tertiary-care academic pediatric hospital. PATIENTS: All pediatric patients admitted to the PICU after cardiac surgery between 2008 and 2014...
June 19, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29922523/infective-endocarditis-associated-with-atrial-septal-defect-in-an-intravenous-drug-abuser-a-case-report
#13
Maha Jahangir, Marrium Nawaz, Fareha Jabbar, Fahad Khan, Nimra Hasnain
Atrial septal defect (ASD) is a common congenital abnormality, which accounts for 20-40% of all the adult patients with congenital heart diseases. Due to the slow velocity of shunt flow, ASD has a negligible risk for infective endocarditis (IE). However, intravenous drug abuse (IVDA) is a potential cause for IE. IE remains a diagnostic and therapeutic challenge. Our case report demonstrates the atypical presentation of IE in an ASD patient. The diagnosis was made on the basis of modified Duke criteria, and blood cultures were found out to be positive for methicillin-resistant Staphylococcus aureus (MRSA)...
April 15, 2018: Curēus
https://www.readbyqxmd.com/read/29922233/selecting-infants-with-cryptorchidism-and-high-risk-of-infertility-for-optional-adjuvant-hormonal-therapy-and-cryopreservation-of-germ-cells-experience-from-a-pilot-study
#14
Jorgen Thorup, Erik Clasen-Linde, Lihua Dong, Simone Hildorf, Stine Gry Kristensen, Claus Yding Andersen, Dina Cortes
Introduction: Orchiopexy for congenital cryptorchid testes is recommended between ½ and 1 year of age to preserve testicular germ cell maturation. Early operation is not enough to preserve fertility in 22 and 36% of cases. Aim of this study was to set up a protocol for optional adjuvant hormonal therapy after orchiopexy and thereafter cryopreservation of testicular biopsies from infants with bilateral cryptorchidism and high infertility risk. Materials and methods: We included 17 boys with bilateral cryptorchidism, normal FSH, and impaired germ cell number per tubular transverse section (G/T) in testicular biopsies at orchiopexy, 7 months to 3½ years old...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29922027/large-nonapical-right-ventricular-diverticulum-in-a-patient-with-atrioventricular-septal-defect
#15
Michael Weidenbach, Bardo Wannenmacher, Christian Paech, Robert Wagner
Congenital diveticula and aneurysm of the heart are rare and most often located at the apex of the left ventricle. They pose a significant risk for cardiac failure and arrhythmias. In contrast, nonapical diverticula of the right ventricle (RV) have a much more benign course. We present a child with Trisomy 21, atrioventricular septal defect, and large nonapical diverticulum of the RV that was neither addressed during surgery nor needed any medical treatment during 1-year follow-up.
May 2018: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/29922014/developing-congenital-heart-surgery-in-india-the-travails-and-triumphs-of-a-pioneer
#16
Rao R Ivatury
Professor IM Rao, from All India Institute of Medical Sciences, New Delhi, was a pioneering cardiac surgeon who, against overwhelming odds, developed congenital heart surgery in India. He made many contributions to the evolution of his specialty, spanning more than four decades and three countries. This is a brief report of his professional life and accomplishments.
May 2018: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/29920635/review-of-early-postoperative-metrics-for-children-undergoing-resection-of-congenital-pulmonary-airway-malformations-and-report-of-pleuropulmonary-blastoma-at-a-single-institution
#17
Robert J Vandewalle, Joseph C Easton, R Cartland Burns, Brian W Gray, Frederick J Rescorla
PURPOSE:  The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB). METHODS:  An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed. RESULTS:  A total of 51 patients with a pathologic diagnosis of CPAM ( n  = 45; 88...
June 19, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29915955/population-pharmacokinetic-modeling-of-remifentanil-in-infants-with-unrepaired-tetralogy-of-fallot
#18
Jing Chang, Yang Shen, Yue Huang, Ying Sun, Mei-Hua Cai, Jing Niu, Li-Ming Zhang, Ji-Jian Zheng, Ma-Zhong Zhang
BACKGROUND: Although there is literature suggesting that pathophysiologic changes in children with congenital heart disease alter the pharmacokinetics of anesthetics and may result in dosage adjustment, limited information exists regarding the pharmacokinetics of remifentanil in infants with unrepaired tetralogy of Fallot (TOF). The objectives of the current analysis were to characterize the population pharmacokinetics of remifentanil in infants, and to evaluate the effects of TOF on remifentanil's pharmacokinetics...
June 19, 2018: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/29914536/very-late-presentation-of-anomalous-origin-of-the-left-coronary-artery-from-the-pulmonary-artery-case-report
#19
Pairoj Chattranukulchai, Jule Namchaisiri, Monravee Tumkosit, Sarinya Puwanant, Yongkasem Vorasettakarnkij, Suphot Srimahachota, Smonporn Boonyaratavej
BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. The enlarged right coronary artery provides retrograde collaterals to supply the left ventricle then preferentially directs into the lower pressure pulmonary artery system causing coronary steal phenomenon. Few patients who survive through adulthood without surgery must have abundant, well-formed functioning collaterals with adequate perfusion of the left ventricle...
June 18, 2018: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29914284/congenital-heart-defects-the-10-year-experience-at-a-single-center
#20
Emine Aydin, Ebru Aypar, Ahmet Oktem, Ozgur Ozyuncu, Murat Yurdakok, Murat Guvener, Metin Demircin, M Sinan Beksac
OBJECTIVE: We aimed to evaluate congenital heart disease (CHD) cases according to EUROCAT subgroup classification that were diagnosed during the prenatal period in our center. METHODS: CHDs that were prenatally diagnosed using ultrasonography and confirmed by fetal echocardiography were reviewed over a 10-year period. Subgroup classification was finalized at the post-partum period in terms of the EUROCAT guide 1.3. Congenital heart defect subtypes and obstetric outcomes (gestational week at delivery, birth weight, gender, extracardiac structural abnormalities, karyotype results if performed) were analyzed...
June 18, 2018: Journal of Maternal-fetal & Neonatal Medicine
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