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https://www.readbyqxmd.com/read/29905864/pathogenic-variant-in-ephb4-results-in-central-conducting-lymphatic-anomaly
#1
Dong Li, Tara L Wenger, Christoph Seiler, Michael E March, Alvaro Gutierrez-Uzquiza, Charlly Kao, Elizabeth Bhoj, Lifeng Tian, Misha Rosenbach, Yichuan Liu, Nora Robinson, Mechenzie Behr, Rosetta Chiavacci, Cuiping Hou, Tiancheng Wang, Marina Bakay, Renata Pellegrino da Silva, Jonathan A Perkins, Patrick Sleiman, Michael A Levine, Patricia J Hicks, Maxim Itkin, Yoav Dori, Hakon Hakonarson
Central conducting lymphatic anomaly (CCLA) is one of the complex lymphatic anomalies characterized by dilated lymphatic channels, lymphatic channel dysmotility, and distal obstruction affecting lymphatic drainage. We performed whole exome sequencing (WES) of DNA from a four-generation pedigree and examined the consequences of the variant by transfection of mammalian cells and morpholino and rescue studies in zebrafish. WES revealed a heterozygous mutation in EPHB4 (RefSeq NM_004444.4; c.2334 + 1G>C) and RNA-Seq demonstrated that the EPHB4 mutation destroys the normal donor site, which leads to the use of a cryptic splice donor that results in retention of the intervening 12-bp intron sequence...
June 14, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29858286/identification-and-characterization-of-hiv-specific-resident-memory-cd8-t-cells-in-human-lymphoid-tissue
#2
Marcus Buggert, Son Nguyen, Gonzalo Salgado-Montes de Oca, Bertram Bengsch, Samuel Darko, Amy Ransier, Emily R Roberts, Daniel Del Alcazar, Irene Bukh Brody, Laura A Vella, Lalit Beura, Sathi Wijeyesinghe, Ramin S Herati, Perla M Del Rio Estrada, Yuria Ablanedo-Terrazas, Leticia Kuri-Cervantes, Alberto Sada Japp, Sasikanth Manne, Shant Vartanian, Austin Huffman, Johan K Sandberg, Emma Gostick, Gregory Nadolski, Guido Silvestri, David H Canaday, David A Price, Constantinos Petrovas, Laura F Su, Golnaz Vahedi, Yoav Dori, Ian Frank, Maxim G Itkin, E John Wherry, Steven G Deeks, Ali Naji, Gustavo Reyes-Terán, David Masopust, Daniel C Douek, Michael R Betts
Current paradigms of CD8+ T cell-mediated protection in HIV infection center almost exclusively on studies of peripheral blood, which is thought to provide a window into immune activity at the predominant sites of viral replication in lymphoid tissues (LTs). Through extensive comparison of blood, thoracic duct lymph (TDL), and LTs in different species, we show that many LT memory CD8+ T cells bear phenotypic, transcriptional, and epigenetic signatures of resident memory T cells (TRMs ). Unlike their circulating counterparts in blood or TDL, most of the total and follicular HIV-specific CD8+ T cells in LTs also resemble TRMs Moreover, high frequencies of HIV-specific CD8+ TRMs with skewed clonotypic profiles relative to matched blood samples are present in LTs of individuals who spontaneously control HIV replication in the absence of antiretroviral therapy (elite controllers)...
June 1, 2018: Science Immunology
https://www.readbyqxmd.com/read/29794709/lymphovenous-anastomosis-for-the-treatment-of-chylothorax-in-infants-a-novel-microsurgical-approach-to-a-devastating-problem
#3
Jason M Weissler, Eugenia H Cho, Peter F Koltz, Martin J Carney, Maxim Itkin, Pablo Laje, L Scott Levin, Yoav Dori, Suhail K Kanchwala, Stephen J Kovach
With the expanding horizon of microsurgical techniques, novel treatment strategies for lymphatic abnormalities are increasingly reported. Described in this article is the first reported use of lymphovenous anastomosis surgery to manage recalcitrant chylothoraces in infants. Chylothorax is an increasingly common postoperative complication after pediatric cardiac surgery, with a reported incidence of up to 9.2 percent in infants. Although conservative nutritional therapy has a reported 70 percent success rate in this patient population, failed conservative management leading to persistent chylothorax is associated with a significant risk of multisystem complications and mortality...
June 2018: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29793847/improvement-in-ldl-is-associated-with-decrease-in-non-calcified-plaque-volume-on-coronary-cta-as-measured-by-automated-quantitative-software
#4
Balaji Tamarappoo, Yuka Otaki, Mhairi Doris, Yoav Arnson, Heidi Gransar, Sean Hayes, John Friedman, Louise Thomson, Frances Wang, Alan Rozanski, Piotr Slomka, Damini Dey, Daniel Berman
BACKGROUND: Computed tomography coronary angiography (CTA) can be used for assessment of plaque characteristics; however, quantitative assessment of changes in plaque composition in response to LDL lowering has not been performed with CTA. We sought to assess the association between LDL reduction and changes in plaque composition with quantitative CTA. METHODS: Quantification of total, calcified, non-calcified and low-density non-calcified plaque volumes (TPV, CPV, NCPV and LD-NCPV) was performed using semi-automated software in 234 vessels from 116 consecutive patients (89 men, 60 ± 10 years) with baseline LDL>70 mg/dl...
May 7, 2018: Journal of Cardiovascular Computed Tomography
https://www.readbyqxmd.com/read/29784622/non-invasive-fractional-flow-reserve-in-vessels-without-severe-obstructive-stenosis-is-associated-with-coronary-plaque-burden
#5
Mhairi K Doris, Yuka Otaki, Yoav Arnson, Balaji Tamarappoo, Markus Goeller, Heidi Gransar, Frances Wang, Sean Hayes, John Friedman, Louise Thomson, Piotr Slomka, Damini Dey, Daniel Berman
AIMS: Non-invasive fractional flow reserve derived from coronary CT angiography (FFRCT ) has been shown to be predictive of lesion-specific ischemia as assessed by invasive fractional flow reserve (FFR). However, in practice, clinicians are often faced with an abnormal distal FFRCT in the absence of a discrete obstructive lesion. Using quantitative plaque analysis, we sought to determine the relationship between an abnormal whole vessel FFRCT (V-FFRCT ) and quantitative measures of whole vessel atherosclerosis in coronary arteries without obstructive stenosis...
May 7, 2018: Journal of Cardiovascular Computed Tomography
https://www.readbyqxmd.com/read/29776653/accuracy-of-phase-contrast-velocity-mapping-proximal-and-distal-to-stent-artifact-during-cardiac-magnetic-resonance-imaging
#6
Catherine M Avitabile, Matthew A Harris, Ravi S Doddasomayajula, Steven G Chopski, Matthew J Gillespie, Yoav Dori, Andrew C Glatz, Mark A Fogel, Kevin K Whitehead
Little data are available on the accuracy of phase-contrast magnetic resonance imaging (PC-MRI) velocity mapping in the vicinity of intravascular metal stents other than nitinol stents. Therefore, we sought to determine this accuracy using in vitro experiments. An in vitro flow phantom was used with 3 stent types: (1) 316L stainless steel, (2) nitinol self-expanding, and (3) platinum-iridium. Steady and pulsatile flow was delivered with a magnetic resonance imaging-compatible pump (CardioFlow 5000, Shelley Medical, London, Ontario, Canada)...
April 20, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29687529/lymphatic-imaging-and-intervention-in-a-pediatric-population-anesthetic-considerations
#7
David R Jobes, Lauren A Brown, Yoav Dori, Maxim Itkin, Susan C Nicolson
The recent adoption of an improved lymphatic access technique coupled with Dynamic Contrast-enhanced Magnetic Resonance Lymphangiography has introduced the ability to diagnose and treat severe lymphatic disorders unresponsive to other therapies. All pediatric patients presenting for lymphatic procedures require general anesthesia presenting challenges in managing highly morbid and comorbid conditions both from logistical as well as medical aspects. General anesthesia is used because of the procedural requirement for immobility to accurately place needles and catheters, treat pain secondary to contrast and glue injections, and to accommodate additional procedures...
June 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29618662/human-mait-cells-exit-peripheral-tissues-and-recirculate-via-lymph-in-steady-state-conditions
#8
Valentin Voillet, Marcus Buggert, Chloe K Slichter, Julia D Berkson, Florian Mair, Mary M Addison, Yoav Dori, Gregory Nadolski, Maxim G Itkin, Raphael Gottardo, Michael R Betts, Martin Prlic
Mucosal-associated invariant T cells (MAIT cells) recognize bacterial metabolites as antigen and are found in blood and tissues, where they are poised to contribute to barrier immunity. Recent data demonstrate that MAIT cells located in mucosal barrier tissues are functionally distinct from their blood counterparts, but the relationship and circulation of MAIT cells between blood and different tissue compartments remains poorly understood. Previous studies raised the possibility that MAIT cells do not leave tissue and may either be retained or undergo apoptosis...
April 5, 2018: JCI Insight
https://www.readbyqxmd.com/read/29580790/recognition-of-neonatal-lymphatic-flow-disorder-fetal-mr-findings-and-postnatal-mr-lymphangiogram-correlation
#9
David M Biko, Jordan A Johnstone, Yoav Dori, Teresa Victoria, Edward R Oliver, Maxim Itkin
RATIONALE AND OBJECTIVES: This study aimed to describe prenatal and postnatal imaging features and outcomes of neonates with neonatal lymphatic disorders (NLDs). MATERIALS AND METHODS: An institutional review board-approved search of the radiology database for patients with NLD identified five patients. Inclusion criteria include prenatal imaging (fetal magnetic resonance [MR] imaging and ultrasound) and postnatal three-dimensional T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) and dynamic contrast-enhanced MR lymphangiography within 6 months of life...
March 23, 2018: Academic Radiology
https://www.readbyqxmd.com/read/29134238/a-comparison-of-anterograde-versus-retrograde-approaches-for-neonatal-balloon-aortic-valvuloplasty
#10
Namrita Mozumdar, Edmund Burke, Melissa Schweizer, Matthew J Gillespie, Yoav Dori, Hari K Narayan, Jonathan J Rome, Andrew C Glatz
In neonates requiring balloon aortic valvuloplasty, both anterograde and retrograde approaches are feasible. A recent comparison of these two approaches is lacking. A retrospective cohort study of neonates at a single center undergoing BAV from 9/00 to 7/14 was performed. Records were reviewed including pre- and post-intervention echocardiograms and catheterization data. Comparisons of acute efficacy and procedural safety were made based on type of approach utilized. Forty-two neonates underwent BAV. Eleven cases utilized exclusively an anterograde approach, while 31 included a retrograde approach (including 4 with both approaches used)...
March 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29098350/reintervention-burden-and-vessel-growth-after-surgical-reimplantation-of-a-pulmonary-artery-during-childhood
#11
Stephan J Wu, Tacy Downing, Christopher Mascio, Matthew J Gillespie, Yoav Dori, Jonathan J Rome, Andrew C Glatz
Children requiring reimplantation of a branch pulmonary artery (PA) are at risk for postoperative stenosis and impaired growth of the reimplanted PA. Outcomes and risk factors for reintervention and impaired growth are incompletely described. We reviewed data on patients who underwent reimplantation of a branch PA between 1/1/99 and 5/1/15 at a single center. The primary outcome was reintervention to treat postoperative stenosis. The secondary outcome was "catch-up" growth (faster diameter growth of the affected PA compared with the unaffected PA from the preoperative to follow-up measurements...
February 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/28938967/increasing-propensity-to-pursue-operative-closure-of-atrial-septal-defects-following-changes-in-the-instructions-for-use-of-the-amplatzer-septal-occluder-device-an-observational-study-using-data-from-the-pediatric-health-information-systems-database
#12
MULTICENTER STUDY
Michael L O'Byrne, Russell T Shinohara, Elena K Grant, Joshua P Kanter, Matthew J Gillespie, Yoav Dori, Jonathan J Rome, Andrew C Glatz
Concern for device erosion following transcatheter treatment of atrial septal defects (TC-ASD) led in 2012 to a United States Food and Drug Administration panel review and changes in the instructions for use of the Amplatzer Septal Occluder (ASO) device. No studies have assessed the effect of these changes on real-world practice. To this end a multicenter observational study was performed to evaluate trends in the treatment of ASD. METHODS: A retrospective observational study was performed using data from the Pediatric Health Information Systems database of all patients with isolated ASD undergoing either TC-ASD or operative ASD closure (O-ASD) from January 1, 2007, to September 30, 2015, hypothesizing that the propensity to pursue O-ASD increased beginning in 2013...
October 2017: American Heart Journal
https://www.readbyqxmd.com/read/28619193/protein-losing-enteropathy-in-patients-with-congenital-heart-disease
#13
Maxim Itkin, David A Piccoli, Gregory Nadolski, Jack Rychik, Aaron DeWitt, Erin Pinto, Jonathan Rome, Yoav Dori
BACKGROUND: Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected. OBJECTIVES: The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE. METHODS: This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE...
June 20, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28494978/post-operative-chylothorax-in-patients-with-congenital-heart-disease
#14
Jill J Savla, Maxim Itkin, Joseph W Rossano, Yoav Dori
BACKGROUND: Post-operative chylothorax in patients with congenital heart disease is a challenging problem with substantial morbidity and mortality. Currently, the etiology of chylothorax is poorly understood and treatment options are limited. OBJECTIVES: This study aimed to report lymphatic imaging findings, determine the mechanism of chylothorax after cardiac surgery, and analyze the outcomes of lymphatic embolization. METHODS: We conducted a retrospective review of 25 patients with congenital heart disease and post-operative chylothorax who presented for lymphatic imaging and intervention between July 2012 and August 2016...
May 16, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28183988/molecular-imaging-of-vulnerable-coronary-plaque-a-pathophysiologic-perspective
#15
Sandeep Krishnan, Yuka Otaki, Mhairi Doris, Leandro Slipczuk, Yoav Arnson, Mathieu Rubeaux, Damini Dey, Piotr Slomka, Daniel S Berman, Balaji Tamarappoo
Atherothrombotic events in coronary arteries are most often due to rupture of unstable plaque resulting in myocardial infarction. Radiolabeled molecular imaging tracers directed toward cellular targets that are unique to unstable plaque can serve as a powerful tool for identifying high-risk patients and for assessing the potential of new therapeutic approaches. Two commonly available radiopharmaceuticals-18 F-FDG and 18 F-NaF-have been used in clinical research for imaging coronary artery plaque, and ongoing clinical studies are testing whether there is an association between 18 F-NaF uptake and future atherothrombotic events...
March 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/27993320/novel-lymphatic-imaging-techniques
#16
REVIEW
Yoav Dori
The lymphatic system plays an important role in immune regulation, transport of metabolites, and fluid balance. The key circulatory role of the lymphatic system is to transport fluid from tissue back into the venous system via lymphovenous connections. Despite the centuries-old recognition of this key role, there has been poor understanding of lymphatic flow pathophysiology because of a lack of a simple reliable noninvasive clinical lymphatic imaging method. This lack of clinical imaging has limited the treatment options for patients with lymphatic flow disorders...
December 2016: Techniques in Vascular and Interventional Radiology
https://www.readbyqxmd.com/read/27662847/implantation-of-the-medtronic-harmony-transcatheter-pulmonary-valve-improves-right-ventricular-size-and-function-in-an-ovine-model-of-postoperative-chronic-pulmonary-insufficiency
#17
Rosanne C Schoonbeek, Satoshi Takebayashi, Chikashi Aoki, Toru Shimaoka, Matthew A Harris, Gregory L Fu, Timothy S Kim, Yoav Dori, Jeremy McGarvey, Harold Litt, Wobbe Bouma, Gerald Zsido, Andrew C Glatz, Jonathan J Rome, Robert C Gorman, Joseph H Gorman, Matthew J Gillespie
BACKGROUND: Pulmonary insufficiency is the nexus of late morbidity and mortality after transannular patch repair of tetralogy of Fallot. This study aimed to establish the feasibility of implantation of the novel Medtronic Harmony transcatheter pulmonary valve (hTPV) and to assess its effect on pulmonary insufficiency and ventricular function in an ovine model of chronic postoperative pulmonary insufficiency. METHODS AND RESULTS: Thirteen sheep underwent baseline cardiac magnetic resonance imaging, surgical pulmonary valvectomy, and transannular patch repair...
October 2016: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/27599255/diagnosis-and-treatment-of-lymphatic-plastic-bronchitis-in-adults-using-advanced-lymphatic-imaging-and-percutaneous-embolization
#18
Maxim G Itkin, Francis X McCormack, Yoav Dori
RATIONALE: Plastic bronchitis is a condition characterized by expectoration of branching bronchial casts. Although the mechanism of cast formation in adults with plastic bronchitis remains poorly understood, abnormal pulmonary lymphatic flow resulting in molding of congealing lymphatic fluids in the airway has been documented as a cause of the disease in children with congenital heart disease. OBJECTIVES: To use advanced lymphatic imaging techniques, including dynamic contrast-enhanced magnetic resonance (MR) lymphangiography (DCMRL) and intranodal lymphangiography, to investigate the mechanism of cast formation in adult patients with plastic bronchitis, and to evaluate the therapeutic outcome of percutaneous lymphatic embolization for these patients...
October 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27423853/etiology-and-new-treatment-options-for-patients-with-plastic-bronchitis
#19
EDITORIAL
Yoav Dori, Maxim Itkin
No abstract text is available yet for this article.
August 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27297208/cerebral-lipiodol-embolism-after-lymphatic-embolization-for-plastic-bronchitis
#20
Matthew P Kirschen, Yoav Dori, Maxim Itkin, Daniel J Licht, Rebecca Ichord, Arastoo Vossough
An adolescent with plastic bronchitis due to congenital heart disease had altered mental status after an interventional lymphatic procedure in which lipiodol contrast was used. Neuroimaging revealed cerebral lipiodol embolization due to direct shunting between lymphatic channels and pulmonary veins. Cerebral lipiodol embolization is a potential neurologic morbidity associated with interventional lymphatic procedures.
September 2016: Journal of Pediatrics
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