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Neonatal palliative

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https://www.readbyqxmd.com/read/29896780/hybrid-strategies-for-high-risk-non-hypoplastic-left-heart-syndrome-patients
#1
Syed M Rehman, Antonio Ravaglioli, Kanchana Singappuli, Kevin Roman, James Gnanapragasam, Duminda Samarasinghe, Nicola Viola
Single-stage repair of complex cardiac abnormalities in high-risk neonates presents formidable challenges. The majority of hybrid strategies involving bilateral pulmonary artery banding (bPAB) with or without patent ductus arteriosus (PDA) stenting is described in the setting of hypoplastic left heart syndrome. We present a series of cases describing two-stage repair with initial palliative hybrid procedures involving bPAB with or without PDA stenting. This allows weight gain and stabilization of circulation before complete repair, provides good results, and may overcome risk factors associated with single-stage repair in neonates...
June 12, 2018: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/29887165/nurses-experiences-of-end-of-life-photography-in-nicu-bereavement-support
#2
Sara Martel, Lori Ives-Baine
PURPOSE: To qualitatively explore neonatal intensive care nurses' experiences with end-of-life photography as part of their bereavement support work with families. DESIGN AND METHODS: An Interpretive Phenomenological Analysis with data collected through a focus group (n = 6) and one semi-structured interview (n = 1) with neonatal nurses from a Level 3/4 NICU in a Canadian pediatric hospital. RESULTS: Participants' comfort with EOL photography developed over time through exposure to bereavement scenarios and positive experiences with families...
June 7, 2018: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/29886666/-techniques-and-experience-which-improve-the-surgical-outcomes-of-critical-and-complex-congenital-heart-disease-in-neonates
#3
Y Pan, B Jia
Objective: To discuss the experience of early surgical intervention to critical and complex congenital heart diseases (CHD) in neonates. Methods: A retrospective study of clinical records of patients with congenital heart diseases underwent surgical intervention in one single pediatric cardiac center was performed. From January 2009 to December 2017, 841 critical and complex CHD neonates were identified at Department of Cardiovascular Surgery, Children's Hospital of Fudan University, of which 6.5% were premature...
June 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29886041/outcomes-after-acute-symptomatic-seizures-in-children-admitted-to-a-neonatal-neurocritical-care-service
#4
Hannah C Glass, Adam L Numis, Dawn Gano, Varun Bali, Elizabeth E Rogers
BACKGROUND: Neonatal seizures due to acute brain injury are associated with high rates of death, disability, and epilepsy. Our objective was to examine incidence of and risk factors for epilepsy among survivors of acute symptomatic neonatal seizures who were cared for by a neonatal neurocritical care service. METHODS: Neonates with acute symptomatic seizures who were admitted to UCSF Benioff Children's Hospital Neuro-Intensive Care Nursery from July 2008 to June 2014 were considered for inclusion...
April 20, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29788322/outcomes-and-risk-factors-associated-with-tricuspid-valve-repair-in-children-with-hypoplastic-left-heart-syndrome
#5
Bahaaldin Alsoufi, Raina Sinha, Courtney McCracken, Janet Figueroa, Firat Altin, Kirk Kanter
OBJECTIVES: Tricuspid valve (TV) competence is important for successful palliation of hypoplastic left heart syndrome (HLHS). We report our experience with TV repair in HLHS patients with a focus on TV and right ventricular (RV) function and associated clinical outcomes. METHODS: From 2002 to 2012, 219 neonates with HLHS underwent the Norwood operation. Thirty patients who underwent TV repair at various stages comprised our current series cohort. Echocardiographic and clinical data were reviewed to determine the effectiveness of TV repair and outcomes of the patients...
May 19, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29787960/late-presentation-of-ectopia-vesica-with-malignant-transformation-a-case-report-and-review
#6
Sami Eldirdiri, Rehab M Elmushly, Sami G Elazhary
INTRODUCTION: Exstrophy of the bladder is a rare congenital anomaly usually treated in neonatal or childhood period. When combined with renal agenesis and presents for the first time in the adulthood with malignant transformation, is an extreme rarity. CASE PRESENTATION: We present a case of 65 years single male who presented with a right irreducible inguinal hernia and an unreconstructed Ectopia Vesicae with fungating tumor. He was anemic with impaired renal function, left renal agenesis and right sided hydronephrosis, hydroureter and distal ureteric stricture...
May 10, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29779802/feasibility-of-initiating-early-enteral-nutrition-after-congenital-heart-surgery-in-neonates-and-infants
#7
Rajat Kalra, Rohit Vohra, Malti Negi, Reena Joshi, Neeraj Aggarwal, Mridul Aggarwal, Raja Joshi
OBJECTIVE: To assess the feasibility of initiating enteral nutrition support with first 24 h of congenital heart repairs in neonates and Infants and its impact on outcomes following surgery. DESIGN: It is a prospective randomized control single blind study. SETTING: It is a single centre prospective study carried out in a tertiary care centre at Pediatric cardiac intensive care unit. PATIENTS: All patients with the cyanotic congenital heart disease with increase pulmonary blood flow, weighing less than 5 kg and undergoing congenital heart repair during the study period were included in the study...
June 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29775603/genetic-and-extracardiac-anomalies-are-associated-with-inferior-single-ventricle-palliation-outcomes
#8
Bahaaldin Alsoufi, Courtney McCracken, Matthew Oster, Subhadra Shashidharan, Kirk Kanter
BACKGROUND: We aim to examine the effect of genetic syndromes and extracardiac anomalies (GS/EC) on single ventricle (SV) palliation with focus on hospital death, interstage mortality, and progression towards subsequent palliation stages. METHODS: 530 neonates with SV underwent first-stage palliation: Norwood (n=284,53%), shunt (n=173,33%), band (n=73,14%). Outcomes were compared between those with GS/EC (n=121,23%) and without GS/EC (n=409,77%). Regression analyses were adjusted for other risk factors (age, gender, prematurity, weight, SV anomaly, first-stage palliation surgery)...
May 15, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#9
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29754124/predictors-of-postoperative-rehabilitation-therapy-following-congenital-heart-surgery
#10
Ana Ubeda Tikkanen, Meena Nathan, Lynn A Sleeper, Marisa Flavin, Ana Lewis, Donna Nimec, John E Mayer, Pedro Del Nido
BACKGROUND: Patients with congenital heart disease are at risk of motor, cognitive, speech, and feeding difficulties after cardiac surgery. Rehabilitation therapy could improve functional outcomes in this population if applied in the acute postcardiac surgical in-hospital stay. However, information on the types of acute postcardiac surgery therapy needs in children is scarce. Our goal was to describe rehabilitation therapy following congenital heart surgery and pre/intraoperative factors associated with need for therapy...
May 12, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29752389/pregnancy-in-women-with-a-fontan-circulation-a-systematic-review-of-the-literature
#11
Alvaro Garcia Ropero, Shankar Baskar, Jolien W Roos Hesselink, Andrea Girnius, Dominica Zentner, Lorna Swan, Magalie Ladouceur, Nicole Brown, Gruschen R Veldtman
BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience. METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation...
May 2018: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/29740195/prenatal-counseling-and-parental-decision-making-following-a-fetal-diagnosis-of-trisomy-13-or-18
#12
Phoebe Winn, Krishna Acharya, Erika Peterson, Steven Leuthner
OBJECTIVES: To evaluate parental decisions following a prenatal diagnosis of trisomy 13 (T13) or trisomy 18 (T18), prenatal counseling received, and pregnancy outcomes. STUDY DESIGN: Single-center, retrospective cohort study of families with a prenatal diagnosis of T13 or T18 from 2000 to 2016. RESULTS: Out of 152 pregnancies, 55% were terminated. Twenty percent chose induction with palliative care, 20% chose expectant management, 2% chose full interventions, and 3% were lost to follow-up...
May 8, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29734172/preemptive-delivery-and-immediate-resection-for-fetuses-with-high-risk-sacrococcygeal-teratomas
#13
Heron D Baumgarten, Juliana S Gebb, Nahla Khalek, Julie S Moldenhauer, Mark P Johnson, William H Peranteau, Holly L Hedrick, N Scott Adzick, Alan W Flake
INTRODUCTION: Fetuses with "high-risk" sacrococcygeal teratoma (SCT) have a mortality rate of 40-50%. While fetal surgery may benefit select fetuses prior to 27 weeks' gestation, many fetuses die due to consequences of rapid tumor growth after 27 weeks. Here we report our experience applying "preemptive" delivery to fetuses who manifest signs of decompensation between 27 and 32 weeks. METHODS: A retrospective review of SCT fetuses delivered between 2010 and 2016 at ≤32 weeks' gestation was performed...
May 7, 2018: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/29718863/endoscopic-retrograde-cholangiopancreatography-in-adult-patients-with-biliary-atresia-process-compliant-case-series
#14
Jong Jin Hyun, Shayan S Irani, Richard A Kozarek
INTRODUCTION: Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29628851/renal-oxalate-stones-in-children-with-zellweger-spectrum-disorders
#15
Hamdan Hammad Alhazmi
Peroxisomal biogenesis disorders due to PEX gene defects are classified into many subgroups, of which Zellweger spectrum disorders (ZSDs) represent the major subgroup. The ZSDs are clinical and biochemical disorders divided into three phenotypes: neonatal, adolescence, or adult. Clinical presentations vary with severity of the condition. Metabolic abnormalities occur due to functional peroxisomal defects that could be detected in blood and urine. No cure or definitive management exists to date; only supportive and palliative measures are applied to prevent worse sequelae...
April 2018: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29605371/an-approach-to-successful-slide-tracheoplasty-in-the-low-birth-weight-neonate-with-single-lung
#16
Matthew S Yong, Joanne Harrison, Robert G Berkowitz, Sarath Ranganathan, Igor E Konstantinov
The advent of the slide tracheoplasty technique and a multi-disciplinary approach has improved outcomes of congenital tracheal stenosis. However, tracheal surgery in younger patients with pulmonary malformations, especially low birth-weight neonates, has been associated with increased mortality. Patients with very low birth-weight, pulmonary malformations and prematurity may be palliated prior to definitive tracheal surgery due to the poor prognosis. We report a successful and unique approach of delaying tracheal reconstruction to allow growth and development in the premature, very low birth-weight neonate (1046g) with left lung agenesis...
May 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29549119/the-case-for-early-use-of-rapid-whole-genome-sequencing-in-management-of-critically-ill-infants-late-diagnosis-of-coffin-siris-syndrome-in-an-infant-with-left-congenital-diaphragmatic-hernia-congenital-heart-disease-and-recurrent-infections
#17
Nathaly M Sweeney, Shareef A Nahas, Shimul Chowdhury, Miguel Del Campo, Marilyn C Jones, David P Dimmock, Stephen F Kingsmore, Rcigm Investigators
Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2...
March 16, 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29544408/optimization-of-the-assisted-bidirectional-glenn-procedure-for-first-stage-single-ventricle-repair
#18
Aekaansh Verma, Mahdi Esmaily, Jessica Shang, Richard Figliola, Jeffrey A Feinstein, Tain-Yen Hsia, Alison L Marsden
BACKGROUND: First-stage single-ventricle palliation is challenging to manage, and significant interstage morbidity and mortality remain. Prior computational and in vitro studies of the assisted bidirectional Glenn (ABG), a novel first-stage procedure that has shown potential for early conversion to a more stable augmented Glenn physiology, demonstrated increased pulmonary flow and oxygen delivery while decreasing cardiac work, as compared to conventional stage-1 alternatives. This study aims to identify optimal shunt designs for the ABG to improve pulmonary flow while maintaining or decreasing superior vena caval (SVC) pressure...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29520463/22q11-2-deletion-status-and-perioperative-outcomes-for-tetralogy-of-fallot-with-pulmonary-atresia-and-multiple-aortopulmonary-collateral-vessels
#19
Laura Mercer-Rosa, Okan U Elci, Nelangi M Pinto, Ronn E Tanel, Elizabeth Goldmuntz
Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11...
June 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29515081/a-devised-option-of-neonatal-palliation-for-compromised-tetralogy-of-fallot-with-absent-pulmonary-valve-syndrome
#20
Hiroo Kinami, Kiyozo Morita, Yoshihiro Ko, Gen Shinohara, Kazuhiro Hashimoto
Neonatal primary repair of tetralogy of Fallot (TOF) with absent pulmonary valve (APV) syndrome is associated with high mortality rates. Our plan involves a staged repair that avoids one-stage intracardiac repair (ICR), with a first palliation that closes the main pulmonary orifice using an expanded polytetrafluoroethylene (ePTFE) patch, pulmonary arterioplication, and an adjustable Blalock-Taussig (BT) shunt. This strategy was used for a neonatal case with TOF/APV syndrome with hypoplastic left ventricle (LV)...
March 7, 2018: Annals of Thoracic and Cardiovascular Surgery
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