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https://www.readbyqxmd.com/read/29876186/multi-modal-3d-simulation-makes-the-impossible-possible
#1
Ingrid M Ganske, Noah Schulz, Katie Livingston, Susan Goobie, John G Meara, Mark Proctor, Peter Weinstock
B.Y. was born full term after a large vertex encephalocele was diagnosed prenatally. The unique challenge to repairing B.Y.'s encephalocele was a microcephalic skull and large proportion of likely functional extracranial brain tissue, which would need to be preserved. At Boston Children's Hospital, a simulation-based collaborative presurgical planning and rehearsal process, using both digital and 3D printed models, enabled successful technical completion and outcome of an otherwise inoperable case.
April 2018: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/29870137/improving-venous-thromboembolism-management-in-children-undergoing-surgery
#2
EDITORIAL
James D O'Leary, Susan M Goobie
No abstract text is available yet for this article.
May 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29788842/veno-arterio-venous-ecmo-for-septic-cardiomyopathy-a-single-centre-experience
#3
Dominik J Vogel, Josie Murray, Adam Z Czapran, Luigi Camporota, Nicholas Ioannou, Chris I S Meadows, Peter B Sherren, Kathleen Daly, Nigel Gooby, Nicholas Barrett
INTRODUCTION: The role of extracorporeal support for patients with septic shock remains unclear. METHODS: We conducted a retrospective analysis of our single-centre experience with veno-arterio-venous extracorporeal membrane oxygenation (VAV ECMO) in adult patients with severe respiratory failure and septic cardiomyopathy. Clinical data was extracted from electronic medical records including a dedicated ECMO referral and follow-up database. RESULTS: Twelve patients were commenced on VAV ECMO for septic cardiomyopathy for a median of four days (IQR 3...
May 2018: Perfusion
https://www.readbyqxmd.com/read/29595531/sedative-and-analgesic-drug-sequestration-after-a-single-bolus-injection-in-an-ex-vivo-extracorporeal-membrane-oxygenation-infant-circuit
#4
Viviane G Nasr, Jonathan Meserve, Luis M Pereira, David Faraoni, Steve Brediger, Susan Goobie, Ravi Thiagarajan, James A DiNardo
Patient sedation and analgesia on extracorporeal membrane oxygenation (ECMO) is vital for safety and comfort. However, adsorption to the circuit may alter drug pharmacokinetics and remains poorly characterized. This study characterizes drug adsorption of morphine, fentanyl, midazolam, and dexmedetomidine in an ex vivo infant ECMO circuit utilizing polymethylpentene (PMP) membrane oxygenator (MO) with protein-bounded polyvinylchloride (PVC) tubing. Twelve closed-loop ex vivo ECMO circuits were prepared using P...
March 26, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29379194/psychosocial-impact-on-mothers-receiving-expanded-newborn-screening-results
#5
Kathleen O'Connor, Tara Jukes, Sharan Goobie, Jennifer DiRaimo, Greg Moran, Beth Katherine Potter, Pranesh Chakraborty, Charles Anthony Rupar, Srinitya Gannavarapu, Chitra Prasad
Expanded newborn screening (NBS) for genetic disorders has improved diagnosis of numerous treatable diseases, positively impacting children's health outcomes. However, research about the psychological impact of expanded NBS on families, especially mothers, has been mixed. Our study examined associations between maternal experiences of expanded NBS and subsequent psychosocial functioning and parenting stress in mothers whose infants received either true negative (TN), true positive (TP) or false positive (FP) results after a 4- to 6-month period...
April 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29178053/autosomal-recessive-agammaglobulinemia-due-to-a-homozygous-mutation-in-pik3r1
#6
Paoyun Tang, Julia E M Upton, Michelle A Barton-Forbes, Marina I Salvadori, Meghan P Clynick, April K Price, Sharan L Goobie
The role of class IA phosphoinositide 3 kinases (PI3Ks) in immune function and regulation continues to expand with the identification of greater numbers of genetic variants. This case report is the second reported case of a homozygous premature stop codon within the PIK3R1 gene leading to autosomal recessive agammaglobulinemia. The proband, born to consanguineous parents, presented at 10 months of age with a history of oropharyngeal petechiae and bleeding from the mouth, gums, and tear ducts. Initial investigations revealed thrombocytopenia, neutropenia and the absence of B cells...
January 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29120963/high-dose-versus-low-dose-tranexamic-acid-to-reduce-transfusion-requirements-in-pediatric-scoliosis-surgery
#7
Daniel J Johnson, Christine C Johnson, Susan M Goobie, Nina Nami, Joshua A Wetzler, Paul D Sponseller, Steven M Frank
BACKGROUND: Our objective was to quantify blood loss and transfusion requirements for high-dose and low-dose tranexamic acid (TXA) dosing regimens in pediatric patients undergoing spinal fusion for correction of idiopathic scoliosis. Previous investigators have established the efficacy of TXA in pediatric scoliosis surgery; however, the dosing regimens vary widely and the optimal dose has not been established. METHODS: We retrospectively analyzed electronic medical records for 116 patients who underwent spinal fusion surgery for idiopathic scoliosis by a single surgeon and were treated with TXA...
December 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29103924/ceftaroline-pharmacokinetics-and-pharmacodynamics-in-patients-with-cystic-fibrosis
#8
Emily E Barsky, Luis M Pereira, Keri J Sullivan, Alanna Wong, Alexander J McAdam, Gregory S Sawicki, Gregory P Priebe, Susan M Goobie
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) is a prevalent pathogen in patients with cystic fibrosis (CF) associated with increased morbidity. Ceftaroline fosamil is an intravenous (IV) cephalosporin with activity against MRSA. There are minimal data regarding dosing in the CF population. The objective of this study was to determine the pharmacokinetic and pharmacodynamic profile of IV ceftaroline in patients with CF. METHODS: We conducted a single-center prospective study of children and young adults with CF receiving ceftaroline (15mg/kg IV up to 600mg every 8h) as part of treatment for a CF pulmonary exacerbation between June 2016 and April 2017...
November 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28991109/patient-blood-management-in-pediatric-cardiac-surgery-a-review
#9
Jill M Cholette, David Faraoni, Susan M Goobie, Victor Ferraris, Nabil Hassan
Efforts to reduce blood product transfusions and adopt blood conservation strategies for infants and children undergoing cardiac surgical procedures are ongoing. Children typically receive red blood cell and coagulant blood products perioperatively for many reasons, including developmental alterations of their hemostatic system, and hemodilution and hypothermia with cardiopulmonary bypass that incites inflammation and coagulopathy and requires systemic anticoagulation. The complexity of their surgical procedures, complex cardiopulmonary interactions, and risk for inadequate oxygen delivery and postoperative bleeding further contribute to blood product utilization in this vulnerable population...
October 5, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28822975/choosing-wisely-canada-the-canadian-college-of-medical-geneticists-ccmg-list-of-five-items-physicians-and-patients-should-question
#10
Elaine Goh, Andrea Guerin, Joanna Lazier, Sharan Goobie, Tanya N Nelson, Ron Agatep, Victoria Mok Siu, Karen Y Niederhoffer, Julie Richer
No abstract text is available yet for this article.
February 2018: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28696996/tranexamic-acid-what-is-known-and-unknown-and-where-do-we-go-from-here
#11
EDITORIAL
Susan M Goobie, Steven M Frank
No abstract text is available yet for this article.
September 2017: Anesthesiology
https://www.readbyqxmd.com/read/28557286/incidence-and-predictors-of-massive-bleeding-in-children-undergoing-liver-transplantation-a-single-center-retrospective-analysis
#12
Benjamin Kloesel, Pete G Kovatsis, David Faraoni, Vanessa Young, Heung Bae Kim, Khashayar Vakili, Susan M Goobie
BACKGROUND: Liver transplantation represents a major surgery involving a highly vascular organ. Reports defining the scope of bleeding in pediatric liver transplants are few. AIMS: We conducted a retrospective analysis of liver transplants performed at our pediatric tertiary care center to quantify blood loss, blood product utilization, and to determine predictors for massive intraoperative bleeding. METHODS: Pediatric patients who underwent isolated liver transplantation at Boston Children's Hospital between 2011 and 2016 were included...
July 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28211198/safety-of-antifibrinolytics-in-cranial-vault-reconstructive-surgery-a-report-from-the-pediatric-craniofacial-collaborative-group
#13
Susan M Goobie, Franklyn P Cladis, Chris D Glover, Henry Huang, Srijaya K Reddy, Allison M Fernandez, David Zurakowski, Paul A Stricker, Heike Gries
BACKGROUND: Antifibrinolytic therapy significantly decreases blood loss and transfusion in pediatric cranial vault reconstructive surgery; however, concern regarding the side effects profile limits clinical use. AIMS: The aim was to utilize the Pediatric Craniofacial Surgery Perioperative Registry database to identify the safety profile of antifibrinolytic therapy for cranial vault reconstructive surgery by reporting the incidence of adverse events as they relate to exposure to tranexamic acid and aminocaproic acid compared to no exposure to antifibrinolytics...
March 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28203768/tranexamic-acid-still-far-to-go
#14
S M Goobie
No abstract text is available yet for this article.
March 1, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/27992637/association-of-preoperative-anemia-with-postoperative-mortality-in-neonates-reply
#15
Susan M Goobie, David Faraoni, James A DiNardo
No abstract text is available yet for this article.
February 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/27991736/kmt2d-p-gln3575his-segregating-in-a-family-with-autosomal-dominant-choanal-atresia-strengthens-the-kabuki-charge-connection
#16
Lauren Badalato, Sali M K Farhan, Allison A Dilliott, Dennis E Bulman, Robert A Hegele, Sharan L Goobie
Choanal atresia is rarely reported in Kabuki syndrome, but is a common feature of CHARGE syndrome. Otherwise, the two conditions have a number of overlapping features, and the molecular links between them have recently been elucidated. Here, we report a case of a mother and her two children who presented with congenital choanal atresia. We performed whole exome sequencing on DNA from the mother and her two unaffected parents, and identified a de novo, novel variant in KMT2D. KMT2D p.Gln3575His segregated with disease status in the family, and is associated with a unique and conserved phenotype in the affected family members, with features overlapping with Kabuki and CHARGE syndromes...
January 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27977460/perioperative-outcomes-and-management-in-pediatric-complex-cranial-vault-reconstruction-a-multicenter-study-from-the-pediatric-craniofacial-collaborative-group
#17
MULTICENTER STUDY
Paul A Stricker, Susan M Goobie, Franklyn P Cladis, Charles M Haberkern, Petra M Meier, Srijaya K Reddy, Thanh T Nguyen, Lingyu Cai, Marcia Polansky, Peter Szmuk, John Fiadjoe, Codruta Soneru, Ricardo Falcon, Timothy Petersen, Courtney Kowalczyk-Derderian, Nicholas Dalesio, Stefan Budac, Neels Groenewald, Daniel Rubens, Douglas Thompson, Rheana Watts, Katherine Gentry, Iskra Ivanova, Mali Hetmaniuk, Vincent Hsieh, Michael Collins, Karen Wong, Wendy Binstock, Russell Reid, Kim Poteet-Schwartz, Heike Gries, Rebecca Hall, Jeffrey Koh, Carolyn Bannister, Wai Sung, Ranu Jain, Allison Fernandez, Gerald F Tuite, Ernesto Ruas, Oleg Drozhinin, Lisa Tetreault, Bridget Muldowney, Karene Ricketts, Patrick Fernandez, Lisa Sohn, John Hajduk, Brad Taicher, Jessica Burkhart, Allison Wright, Jane Kugler, Lea Barajas-DeLoa, Meera Gangadharan, Veronica Busso, Kayla Stallworth, Susan Staudt, Kristen L Labovsky, Chris D Glover, Henry Huang, Helena Karlberg-Hippard, Samantha Capehart, Cynthia Streckfus, Kim-Phuong T Nguyen, Peter Manyang, Jose Luis Martinez, Jennifer K Hansen, Heather Mitzel Levy, Alyssa Brzenski, Franklin Chiao, Pablo Ingelmo, Razaz Mujallid, Olutoyin A Olutoye, Tariq Syed, Hubert Benzon, Adrian Bosenberg
BACKGROUND: The Pediatric Craniofacial Collaborative Group established the Pediatric Craniofacial Surgery Perioperative Registry to elucidate practices and outcomes in children with craniosynostosis undergoing complex cranial vault reconstruction and inform quality improvement efforts. The aim of this study is to determine perioperative management, outcomes, and complications in children undergoing complex cranial vault reconstruction across North America and to delineate salient features of current practices...
February 2017: Anesthesiology
https://www.readbyqxmd.com/read/27870741/relationship-between-preoperative-anemia-and-in-hospital-mortality-in-children-undergoing-noncardiac-surgery
#18
David Faraoni, James A DiNardo, Susan M Goobie
BACKGROUND: The relationship between preoperative anemia and in-hospital mortality has not been investigated in the pediatric surgical population. We hypothesized that children with preoperative anemia undergoing noncardiac surgery may have an increased risk of in-hospital mortality. METHODS: We identified all children between 1 and 18 years of age with a recorded preoperative hematocrit (HCT) in the 2012, 2013, and 2014 American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) pediatric databases...
December 2016: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/27510707/multivariable-predictors-of-substantial-blood-loss-in-children-undergoing-craniosynostosis-repair-implications-for-risk-stratification
#19
Petra M Meier, David Zurakowski, Susan M Goobie, Mark R Proctor, John G Meara, Vanessa J Young, Gary F Rogers, James A DiNardo
BACKGROUND: Operative treatment of craniosynostosis is associated with substantial blood loss, often requiring transfusion of packed red blood cells (PRBC) and coagulation products. AIMS: The aim of this prospective study was to analyze thromboelastographic (TEG) parameters and platelet fibrinogen product to determine predictors of substantial blood loss, and the need for PRBC transfusion and coagulation products. METHODS: With IRB approval, we enrolled 120 children undergoing craniosynostosis repair with a standardized anesthetic, fluid management, and TEG measurements at predefined times...
October 2016: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27461765/the-translational-value-of-chart-reviews-and-the-need-for-trials
#20
EDITORIAL
Andrew Davidson, Susan M Goobie
No abstract text is available yet for this article.
September 2016: Paediatric Anaesthesia
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