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Full-Text Articles in Medicine and Health Sciences

Speckle-Tracking Echocardiographic Measures Of Right Ventricular Diastolic Function Correlate With Reference Standard Measures Before And After Preload Alteration In Children., Shahryar M. Chowdhury, Suma Goudar, G Hamilton Baker, Carolyn L. Taylor, Girish S. Shirali, Mark K. Friedberg, Andreea Dragulescu, Karen S. Chessa, Luc Mertens Jan 2017

Speckle-Tracking Echocardiographic Measures Of Right Ventricular Diastolic Function Correlate With Reference Standard Measures Before And After Preload Alteration In Children., Shahryar M. Chowdhury, Suma Goudar, G Hamilton Baker, Carolyn L. Taylor, Girish S. Shirali, Mark K. Friedberg, Andreea Dragulescu, Karen S. Chessa, Luc Mertens

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The accuracy of echocardiographic measures of right ventricular (RV) diastolic function has been sparsely studied. Our objective was to evaluate the correlation between echocardiographic and reference standard measures of RV diastolic function derived from micromanometer pressure analysis before and after preload alteration in children. Echocardiograms and micromanometer pressure analyses were prospectively performed before and after fluid bolus in children undergoing right heart catheterization. The isovolumic relaxation time constant (τ) and end-diastolic pressure (EDP) were measured. Conventional and speckle-tracking echocardiographic (STE) parameters of RV systolic and diastolic function were assessed. Normal saline bolus was given to increase RV EDP by 20 …


Relationship Between Hospital Procedure Volume And Complications Following Congenital Cardiac Catheterization: A Report From The Improving Pediatric And Adult Congenital Treatment (Impact) Registry., Natalie Jayaram, John A. Spertus, Michael L. O'Byrne, Paul S. Chan, Kevin F. Kennedy, Lisa Bergersen, Andrew C. Glatz Jan 2017

Relationship Between Hospital Procedure Volume And Complications Following Congenital Cardiac Catheterization: A Report From The Improving Pediatric And Adult Congenital Treatment (Impact) Registry., Natalie Jayaram, John A. Spertus, Michael L. O'Byrne, Paul S. Chan, Kevin F. Kennedy, Lisa Bergersen, Andrew C. Glatz

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BACKGROUND: The association between institutional volume and outcomes has been demonstrated for cardiac catheterization among adults, but less is known about this relationship for patients with congenital heart disease (CHD) undergoing cardiac catheterization.

METHODS: Within the IMPACT registry, we identified all catheterizations between January 2011 and March 2015. Hierarchical logistic regression, adjusted for patient and procedural characteristics, was used to determine the association between annual catheterization lab volume and occurrence of a major adverse event (MAE).

RESULTS: Of 56,453 catheterizations at 77 hospitals, an MAE occurred in 1014 (1.8%) of cases. In unadjusted analysis, a MAE occurred in 2.8% (123/4460) …


Biochemical And Biophysical Methods For Analysis Of Poly(Adp-Ribose) Polymerase 1 And Its Interactions With Chromatin., Maggie H. Chassé, Uma M. Muthurajan, Nicholas J. Clark, Michael A. Kramer, Srinivas Chakravarthy, Thomas Irving, Karolin Luger Jan 2017

Biochemical And Biophysical Methods For Analysis Of Poly(Adp-Ribose) Polymerase 1 And Its Interactions With Chromatin., Maggie H. Chassé, Uma M. Muthurajan, Nicholas J. Clark, Michael A. Kramer, Srinivas Chakravarthy, Thomas Irving, Karolin Luger

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Poly (ADP-Ribose) Polymerase I (PARP-1) is a first responder to DNA damage and participates in the regulation of gene expression. The interaction of PARP-1 with chromatin and DNA is complex and involves at least two different modes of interaction. In its enzymatically inactive state, PARP-1 binds native chromatin with similar affinity as it binds free DNA ends. Automodification of PARP-1 affects interaction with chromatin and DNA to different extents. Here we describe a series of biochemical and biophysical techniques to quantify and dissect the different binding modes of PARP-1 with its various substrates. The techniques listed here allow for high …


Life-Threatening Hypercalcemia During Prodrome Of Pneumocystis Jiroveci Pneumonia In An Immunocompetent Infant, Judith Sebestyen Vansickle, Tarak Srivastava, Uri S. Alon Jan 2017

Life-Threatening Hypercalcemia During Prodrome Of Pneumocystis Jiroveci Pneumonia In An Immunocompetent Infant, Judith Sebestyen Vansickle, Tarak Srivastava, Uri S. Alon

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Severe hypercalcemia in infants is usually attributed to genetic etiologies and less commonly to acquired ones. An 8-week-old girl presented with failure to thrive, mild respiratory distress, and life-threatening hypercalcemia (23.5 mg/dL). Serum 1,25(OH)2-D) level was elevated and parathyroid hormone undetectable. Evaluation for genetic mutations and malignant etiologies of hypercalcemia was negative. Treatment with intravenous hydration, loop diuretic, and calcitonin failed to correct the hypercalcemia, which was subsequently controlled with bisphosphonate therapy. Due to progressive respiratory deterioration, a bronchopulmonary lavage was done on day 17 of her hospitalization disclosing Pneumocystis jiroveci infection. The subsequent immunological investigation showed no abnormalities. She …


Appendectomy Versus Non-Operative Treatment For Acute Uncomplicated Appendicitis In Children: Study Protocol For A Multicentre, Open-Label, Non-Inferiority, Randomised Controlled Trial., Nigel J. Hall, Simon Eaton, Olivier Abbo, Alexis P. Arnaud, Marianne Beaudin, Mary Brindle, Andreana Bütter, Dafydd Davies, Tim Jancelewicz, Kathy Johnson, Richard Keijzer, Eveline Lapidus-Krol, Martin Offringa, Nelson Piché, Risto Rintala, Erik Skarsgard, Jan F. Svensson, Wendy J. Ungar, Tomas Wester, Andrew R. Willan, Augusto Zani, Shawn D. St Peter, Agostino Pierro Jan 2017

Appendectomy Versus Non-Operative Treatment For Acute Uncomplicated Appendicitis In Children: Study Protocol For A Multicentre, Open-Label, Non-Inferiority, Randomised Controlled Trial., Nigel J. Hall, Simon Eaton, Olivier Abbo, Alexis P. Arnaud, Marianne Beaudin, Mary Brindle, Andreana Bütter, Dafydd Davies, Tim Jancelewicz, Kathy Johnson, Richard Keijzer, Eveline Lapidus-Krol, Martin Offringa, Nelson Piché, Risto Rintala, Erik Skarsgard, Jan F. Svensson, Wendy J. Ungar, Tomas Wester, Andrew R. Willan, Augusto Zani, Shawn D. St Peter, Agostino Pierro

Manuscripts, Articles, Book Chapters and Other Papers

Background: Appendectomy is considered the gold standard treatment for acute appendicitis. Recently the need for surgery has been challenged in both adults and children. In children there is growing clinician, patient and parental interest in non-operative treatment of acute appendicitis with antibiotics as opposed to surgery. To date no multicentre randomised controlled trials that are appropriately powered to determine efficacy of non-operative treatment (antibiotics) for acute appendicitis in children compared with surgery (appendectomy) have been performed.

Methods: Multicentre, international, randomised controlled trial with a non-inferiority design. Children (age 5-16 years) with a clinical and/or radiological diagnosis of acute uncomplicated appendicitis …


Prediction Of Cyp2d6 Phenotype From Genotype Across World Populations., Andrea Gaedigk, Katrin Sangkuhl, Michelle Whirl-Carrillo, Teri Klein, J Steven Leeder Jan 2017

Prediction Of Cyp2d6 Phenotype From Genotype Across World Populations., Andrea Gaedigk, Katrin Sangkuhl, Michelle Whirl-Carrillo, Teri Klein, J Steven Leeder

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PURPOSE: Owing to its highly polymorphic nature and major contribution to the metabolism and bioactivation of numerous clinically used drugs, CYP2D6 is one of the most extensively studied drug-metabolizing enzymes and pharmacogenes. CYP2D6 alleles confer no, decreased, normal, or increased activity and cause a wide range of activity among individuals and between populations. However, there is no standard approach to translate diplotypes into predicted phenotype.

METHODS: We exploited CYP2D6 allele-frequency data that have been compiled for Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines (>60,000 subjects, 173 reports) in order to estimate genotype-predicted phenotype status across major world populations based on …


Components Of Effective Cognitive-Behavioral Therapy For Pediatric Headache: A Mixed Methods Approach., Emily F. Law, Sarah E. Beals-Erickson, Emma Fisher, Emily A. Lang, Tonya M. Palermo Jan 2017

Components Of Effective Cognitive-Behavioral Therapy For Pediatric Headache: A Mixed Methods Approach., Emily F. Law, Sarah E. Beals-Erickson, Emma Fisher, Emily A. Lang, Tonya M. Palermo

Manuscripts, Articles, Book Chapters and Other Papers

Internet-delivered treatment has the potential to expand access to evidence-based cognitive-behavioral therapy (CBT) for pediatric headache, and has demonstrated efficacy in small trials for some youth with headache. We used a mixed methods approach to identify effective components of CBT for this population. In Study 1, component profile analysis identified common interventions delivered in published RCTs of effective CBT protocols for pediatric headache delivered face-to-face or via the Internet. We identified a core set of three treatment components that were common across face-to-face and Internet protocols: 1) headache education, 2) relaxation training, and 3) cognitive interventions. Biofeedback was identified as …


Ambulatory Blood Pressure, Left Ventricular Hypertrophy, And Allograft Function In Children And Young Adults After Kidney Transplantation., Gilad Hamdani, Edward J. Nehus, Coral D. Hanevold, Judith Sebestyen Van Sickle, Robert Woroniecki, Scott E. Wenderfer, David K. Hooper, Douglas Blowey, Amy Wilson, Bradley A. Warady, Mark M. Mitsnefes Jan 2017

Ambulatory Blood Pressure, Left Ventricular Hypertrophy, And Allograft Function In Children And Young Adults After Kidney Transplantation., Gilad Hamdani, Edward J. Nehus, Coral D. Hanevold, Judith Sebestyen Van Sickle, Robert Woroniecki, Scott E. Wenderfer, David K. Hooper, Douglas Blowey, Amy Wilson, Bradley A. Warady, Mark M. Mitsnefes

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Hypertension is a common complication and is an important risk factor for graft loss and adverse cardiovascular outcomes in pediatric kidney transplantation. Ambulatory blood pressure monitoring (ABPM) is the preferred method to characterize blood pressure status.

METHODS: We conducted a retrospective review of a large cohort of children and young adults with kidney transplant to estimate the prevalence of abnormal ambulatory blood pressure (ABP), assess factors associated with abnormal ABP, and examine whether ambulatory hypertension is associated with worse allograft function and left ventricular hypertrophy (LVH).

RESULTS: Two hundred twenty-one patients had ABPM, and 142 patients had echocardiographic results …


Biomechanical Evaluation Of A Growth-Friendly Rod Construct., Sarah Galvis, Josh Arnold, Erin Mannen, Benjamin Wong, Hadley Sis, Eileen Cadel, John Anderson, Dennis Anderson, Paul Arnold, Elizabeth Friis Jan 2017

Biomechanical Evaluation Of A Growth-Friendly Rod Construct., Sarah Galvis, Josh Arnold, Erin Mannen, Benjamin Wong, Hadley Sis, Eileen Cadel, John Anderson, Dennis Anderson, Paul Arnold, Elizabeth Friis

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Distraction-type rods mechanically stabilize the thorax and improve lung growth and function by applying distraction forces at the rib, spine, pelvis, or a combination of locations. However, the amount of stability the rods provide and the amount the thorax needs is unknown.

METHODS: Five freshly frozen and thawed cadaveric thoracic spine specimens were tested for lateral bending, flexion/extension, and axial rotation in displacement control (1°/sec) to a load limit of ±5 Nm for five cycles after which a growth-friendly unilateral rod was placed in a simulated rib-to-lumbar attachment along the right side. The specimens were tested again in the …


Validation Of Patient-Reported Outcomes Measurement Information System Short Forms For Use In Childhood-Onset Systemic Lupus Erythematosus., Jordan T. Jones, Adam C. Carle, Janet Wootton, Brianna Liberio, Jiha Lee, Laura E. Schanberg, Jun Ying, Esi Morgan Dewitt, Hermine I. Brunner Jan 2017

Validation Of Patient-Reported Outcomes Measurement Information System Short Forms For Use In Childhood-Onset Systemic Lupus Erythematosus., Jordan T. Jones, Adam C. Carle, Janet Wootton, Brianna Liberio, Jiha Lee, Laura E. Schanberg, Jun Ying, Esi Morgan Dewitt, Hermine I. Brunner

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To validate the pediatric Patient-Reported Outcomes Measurement Information System short forms (PROMIS-SFs) in childhood-onset systemic lupus erythematosus (SLE) in a clinical setting.

METHODS: At 3 study visits, childhood-onset SLE patients completed the PROMIS-SFs (anger, anxiety, depressive symptoms, fatigue, physical function-mobility, physical function-upper extremity, pain interference, and peer relationships) using the PROMIS assessment center, and health-related quality of life (HRQoL) legacy measures (Pediatric Quality of Life Inventory, Childhood Health Assessment Questionnaire, Simple Measure of Impact of Lupus Erythematosus in Youngsters [SMILEY], and visual analog scales [VAS] of pain and well-being). Physicians rated childhood-onset SLE activity on a VAS and completed …


Comparison Of Allergens Collected From Furnace Filters And Vacuum Floor Dust., Ryan Allenbrand, Charles S. Barnes, Mubeen Mohammed, Luke Gard, Freddy Pacheco, Kevin Kennedy, Anita Didonna, Jay M. Portnoy, Chitra Dinakar Jan 2017

Comparison Of Allergens Collected From Furnace Filters And Vacuum Floor Dust., Ryan Allenbrand, Charles S. Barnes, Mubeen Mohammed, Luke Gard, Freddy Pacheco, Kevin Kennedy, Anita Didonna, Jay M. Portnoy, Chitra Dinakar

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No abstract provided.


Novel Hla-Dp Region Susceptibility Loci Associated With Severe Acute Gvhd., Rakesh K. Goyal, S J. Lee, T Wang, M Trucco, M Haagenson, S R. Spellman, M Verneris, R E. Ferrell Jan 2017

Novel Hla-Dp Region Susceptibility Loci Associated With Severe Acute Gvhd., Rakesh K. Goyal, S J. Lee, T Wang, M Trucco, M Haagenson, S R. Spellman, M Verneris, R E. Ferrell

Manuscripts, Articles, Book Chapters and Other Papers

Despite HLA allele matching, significant acute GvHD remains a major barrier to successful unrelated donor BMT. We conducted a genome-wide association study (GWAS) to identify recipient and donor genes associated with the risk of acute GvHD. A case-control design (grade III-IV versus no acute GvHD) and pooled GWA approach was used to study European-American recipients with hematological malignancies who received myeloablative conditioning non-T-cell-depleted first transplantation from HLA-A, -B, -C, -DRB1, -DQB1 allele level (10/10) matched unrelated donors. DNA samples were divided into three pools and tested in triplicate using the Affymetrix Genome-wide SNP Array 6.0. We identified three novel susceptibility …


Should School Boards Discontinue Support For High School Football?, Lewis H. Margolis, Greg Canty, Mark Halstead, John Lantos Jan 2017

Should School Boards Discontinue Support For High School Football?, Lewis H. Margolis, Greg Canty, Mark Halstead, John Lantos

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A pediatrician is asked by her local school board to help them decide whether to discontinue their high school football program. She reviews the available evidence on the risks of football and finds it hopelessly contradictory. Some scholars claim that football is clearly more dangerous than other sports. Others suggest that the risks of football are comparable to other sports, such as lacrosse, ice hockey, or soccer. She finds very little data on the long-term sequelae of concussions. She sees claims that good coaching and a school culture that prioritizes the health of athletes over winning can reduce morbidity from …


International Consensus (Icon) On: Clinical Consequences Of Mite Hypersensitivity, A Global Problem., Mario Sánchez-Borges, Enrique Fernandez-Caldas, Wayne R. Thomas, Martin D. Chapman, Bee Wah Lee, Luis Caraballo, Nathalie Acevedo, Fook Tim Chew, Ignacio J. Ansotegui, Leili Behrooz, Wanda Phipatanakul, Roy Gerth Van Wijk, Demoly Pascal, Nelson Rosario, Motohiro Ebisawa, Mario Geller, Santiago Quirce, Susanne Vrtala, Rudolf Valenta, Markus Ollert, Giorgio Walter Canonica, Moises A. Calderón, Charles S. Barnes, Adnan Custovic, Suwat Benjaponpitak, Arnaldo Capriles-Hulett Jan 2017

International Consensus (Icon) On: Clinical Consequences Of Mite Hypersensitivity, A Global Problem., Mario Sánchez-Borges, Enrique Fernandez-Caldas, Wayne R. Thomas, Martin D. Chapman, Bee Wah Lee, Luis Caraballo, Nathalie Acevedo, Fook Tim Chew, Ignacio J. Ansotegui, Leili Behrooz, Wanda Phipatanakul, Roy Gerth Van Wijk, Demoly Pascal, Nelson Rosario, Motohiro Ebisawa, Mario Geller, Santiago Quirce, Susanne Vrtala, Rudolf Valenta, Markus Ollert, Giorgio Walter Canonica, Moises A. Calderón, Charles S. Barnes, Adnan Custovic, Suwat Benjaponpitak, Arnaldo Capriles-Hulett

Manuscripts, Articles, Book Chapters and Other Papers

Since mite allergens are the most relevant inducers of allergic diseases worldwide, resulting in significant morbidity and increased burden on health services, the International Collaboration in Asthma, Allergy and Immunology (iCAALL), formed by the American Academy of Allergy, Asthma and Immunology (AAAAI), the American College of Allergy, Asthma and Immunology (ACAAI), the European Academy of Allergy and Clinical Immunology (EAACI), and the World Allergy Organization (WAO), has proposed to issue an International Consensus (ICON) on the clinical consequences of mite hypersensitivity. The objectives of this document are to highlight aspects of mite biology that are clinically relevant, to update the …


Immunodeficiency-Associated Lymphoid Hyperplasia As A Cause Of Intussusception In A Case Of Activated Pi3k-Δ Syndrome., Daniel Mettman, Isabelle Thiffault, Chitra Dinakar, Carol J. Saunders Jan 2017

Immunodeficiency-Associated Lymphoid Hyperplasia As A Cause Of Intussusception In A Case Of Activated Pi3k-Δ Syndrome., Daniel Mettman, Isabelle Thiffault, Chitra Dinakar, Carol J. Saunders

Manuscripts, Articles, Book Chapters and Other Papers

Activated PI3K-δ syndrome refers to a recently described primary immunodeficiency syndrome consisting of recurrent sinopulmonary infections, lymphadenopathy, mucosal lymphoid aggregates, increased susceptibility to Epstein-Barr virus and cytomegalovirus, and increased incidence of B-cell lymphomas. Variants in PIK3CD, which encodes the phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit delta isoform, enhance membrane association and kinase activity, resulting in increased signal transduction through the PI3K-Akt pathway. Whole-exome sequencing revealed a pathogenic PIK3CD variant in a patient with history of immunologic impairment, recurrent sinopulmonary infections, and lymphoid hyperplasia presenting as intussusception. This case illustrates that while lymphoid hyperplasia secondary to immunodeficiency is most often unsurprising and …


Mc-Ppea As A New And More Potent Inhibitor Of Clp-Induced Sepsis And Pulmonary Inflammation Than Fk866., Peixin Huang, Mark W Lee, Keivan Sadrerafi, Daniel P. Heruth, Li Q. Zhang, Dev Maulik, Shui Qing Ye Jan 2017

Mc-Ppea As A New And More Potent Inhibitor Of Clp-Induced Sepsis And Pulmonary Inflammation Than Fk866., Peixin Huang, Mark W Lee, Keivan Sadrerafi, Daniel P. Heruth, Li Q. Zhang, Dev Maulik, Shui Qing Ye

Manuscripts, Articles, Book Chapters and Other Papers

Our previous study indicated that overexpression of nicotinamide phosphoribosyltransferase (NAMPT) aggravated acute lung injury, while knockdown of NAMPT expression attenuated ventilator-induced lung injury. Recently, we found that meta-carborane-butyl-3-(3-pyridinyl)-2E-propenamide (MC-PPEA, MC4), in which the benzoylpiperidine moiety of FK866 has been replaced by a carborane, displayed a 100-fold increase in NAMPT inhibition over FK866. Here, we determined the effects of MC4 and FK866 on cecal ligation and puncture (CLP) surgery-induced sepsis in C57BL/6J mice. MC4 showed stronger inhibitory effects than FK866 on CLP-induced mortality, serum tumor necrosis factor α (TNFα) levels, pulmonary myeloperoxidase activity, alveolar injury, and interleukin 6 and interleukin1β messenger …


Erratum To: Scaling Up Strategies Of The Chronic Respiratory Disease Programme Of The European Innovation Partnership On Active And Healthy Ageing (Action Plan B3: Area 5)., J Bousquet, J Farrell, G Crooks, P Hellings, E H. Bel, M Bewick, N H. Chavannes, J Correia De Sousa, A A. Cruz, T Haahtela, G Joos, N Khaltaev, J Malva, A Muraro, M Nogues, S Palkonen, S Pedersen, C Robalo-Cordeiro, B Samolinski, T Strandberg, A Valiulis, A Yorgancioglu, T Zuberbier, A Bedbrook, W Aberer, M Adachi, A Agusti, C A Akdis, M Akdis, J Ankri, A Alonso, I Annesi-Maesano, I J. Ansotegui, J M. Anto, S Arnavielhe, H Arshad, C Bai, I Baiardini, C Bachert, A K. Baigenzhin, C Barbara, E D. Bateman, B Beghé, A Ben Kheder, K S. Bennoor, M Benson, K C. Bergmann, T Bieber, C Bindslev-Jensen, L Bjermer, H Blain, F Blasi, A L. Boner, M Bonini, S Bonini, S Bosnic-Anticevitch, L P. Boulet, R Bourret, P J. Bousquet, F Braido, A H. Briggs, C E. Brightling, J Brozek, R Buhl, P G. Burney, A Bush, F Caballero-Fonseca, D Caimmi, M A. Calderon, P M. Calverley, P A M Camargos, G W. Canonica, T Camuzat, K H. Carlsen, W Carr, A Carriazo, T Casale, A M. Cepeda Sarabia, L Chatzi, Y Z. Chen, R Chiron, E Chkhartishvili, A G. Chuchalin, K F. Chung, G Ciprandi, I Cirule, L Cox, D J. Costa, A Custovic, R Dahl, S E. Dahlen, U Darsow, G De Carlo, F De Blay, T Dedeu, D Deleanu, E De Manuel Keenoy, P Demoly, J A. Denburg, P Devillier, A Didier, A T. Dinh-Xuan, R Djukanovic, D Dokic, H Douagui, G Dray, R Dubakiene, S R. Durham, M S. Dykewicz, Y El-Gamal, R Emuzyte, L M. Fabbri, M Fletcher, A Fiocchi, A Fink Wagner, J Fonseca, W J. Fokkens, F Forastiere, P Frith, M Gaga, A Gamkrelidze, J Garces, J Garcia-Aymerich, B Gemicioğlu, J E. Gereda, S González Diaz, M Gotua, I Grisle, L Grouse, Z Gutter, M A. Guzmán, L G. Heaney, B Hellquist-Dahl, D Henderson, A Hendry, J Heinrich, D Heve, F Horak, J O' B Hourihane, P Howarth, M Humbert, M E. Hyland, M Illario, J C. Ivancevich, J R. Jardim, E J. Jares, C Jeandel, C Jenkins, S L. Johnston, O Jonquet, K Julge, K S. Jung, J Just, I Kaidashev, M R. Khaitov, O Kalayci, A F. Kalyoncu, T Keil, P K. Keith, L Klimek, B Koffi N'Goran, V Kolek, G H. Koppelman, M L. Kowalski, I Kull, P Kuna, V Kvedariene, B Lambrecht, S Lau, D Larenas-Linnemann, D Laune, L T T Le, P Lieberman, B Lipworth, J Li, K Lodrup Carlsen, R Louis, W Macnee, Y Magard, A Magnan, B Mahboub, A Mair, I Majer, M J. Makela, P Manning, S Mara, G D. Marshall, M R. Masjedi, P Matignon, M Maurer, S Mavale-Manuel, E Melén, E Melo-Gomes, E O. Meltzer, A Menzies-Gow, H Merk, J P. Michel, N Miculinic, F Mihaltan, B Milenkovic, G M Y Mohammad, M Molimard, I Momas, A Montilla-Santana, M Morais-Almeida, M Morgan, R Mösges, J Mullol, S Nafti, L Namazova-Baranova, R Naclerio, A Neou, H Neffen, K Nekam, B Niggemann, G Ninot, T D. Nyembue, R E. O'Hehir, K Ohta, Y Okamoto, K Okubo, S Ouedraogo, P Paggiaro, I Pali-Schöll, P Panzner, N Papadopoulos, A Papi, H S. Park, G Passalacqua, I Pavord, R Pawankar, R Pengelly, O Pfaar, R Picard, B Pigearias, I Pin, D Plavec, D Poethig, W Pohl, T A. Popov, F Portejoie, P Potter, D Postma, D Price, K F. Rabe, F Raciborski, F Radier Pontal, S Repka-Ramirez, S Reitamo, S Rennard, F Rodenas, J Roberts, J Roca, L Rodriguez Mañas, C Rolland, M Roman Rodriguez, A Romano, J Rosado-Pinto, N Rosario, L Rosenwasser, M Rottem, D Ryan, M Sanchez-Borges, G K. Scadding, H J. Schunemann, E Serrano, P Schmid-Grendelmeier, H Schulz, A Sheikh, M Shields, N Siafakas, Y Sibille, T Similowski, F E R Simons, J C. Sisul, I Skrindo, H A. Smit, D Solé, T Sooronbaev, O Spranger, R Stelmach, P J. Sterk, J Sunyer, C Thijs, T To, A Todo-Bom, M Triggiani, R Valenta, A L. Valero, E Valia, E Valovirta, E Van Ganse, M Van Hage, O Vandenplas, T Vasankari, B Vellas, J Vestbo, G Vezzani, P Vichyanond, G Viegi, C Vogelmeier, T Vontetsianos, M Wagenmann, B Wallaert, S Walker, D Y. Wang, U Wahn, M Wickman, D M. Williams, S Williams, J Wright, B P. Yawn, P K. Yiallouros, O M. Yusuf, A Zaidi, H J. Zar, M E. Zernotti, L Zhang, N Zhong, M Zidarn, J Mercier Jan 2017

Erratum To: Scaling Up Strategies Of The Chronic Respiratory Disease Programme Of The European Innovation Partnership On Active And Healthy Ageing (Action Plan B3: Area 5)., J Bousquet, J Farrell, G Crooks, P Hellings, E H. Bel, M Bewick, N H. Chavannes, J Correia De Sousa, A A. Cruz, T Haahtela, G Joos, N Khaltaev, J Malva, A Muraro, M Nogues, S Palkonen, S Pedersen, C Robalo-Cordeiro, B Samolinski, T Strandberg, A Valiulis, A Yorgancioglu, T Zuberbier, A Bedbrook, W Aberer, M Adachi, A Agusti, C A Akdis, M Akdis, J Ankri, A Alonso, I Annesi-Maesano, I J. Ansotegui, J M. Anto, S Arnavielhe, H Arshad, C Bai, I Baiardini, C Bachert, A K. Baigenzhin, C Barbara, E D. Bateman, B Beghé, A Ben Kheder, K S. Bennoor, M Benson, K C. Bergmann, T Bieber, C Bindslev-Jensen, L Bjermer, H Blain, F Blasi, A L. Boner, M Bonini, S Bonini, S Bosnic-Anticevitch, L P. Boulet, R Bourret, P J. Bousquet, F Braido, A H. Briggs, C E. Brightling, J Brozek, R Buhl, P G. Burney, A Bush, F Caballero-Fonseca, D Caimmi, M A. Calderon, P M. Calverley, P A M Camargos, G W. Canonica, T Camuzat, K H. Carlsen, W Carr, A Carriazo, T Casale, A M. Cepeda Sarabia, L Chatzi, Y Z. Chen, R Chiron, E Chkhartishvili, A G. Chuchalin, K F. Chung, G Ciprandi, I Cirule, L Cox, D J. Costa, A Custovic, R Dahl, S E. Dahlen, U Darsow, G De Carlo, F De Blay, T Dedeu, D Deleanu, E De Manuel Keenoy, P Demoly, J A. Denburg, P Devillier, A Didier, A T. Dinh-Xuan, R Djukanovic, D Dokic, H Douagui, G Dray, R Dubakiene, S R. Durham, M S. Dykewicz, Y El-Gamal, R Emuzyte, L M. Fabbri, M Fletcher, A Fiocchi, A Fink Wagner, J Fonseca, W J. Fokkens, F Forastiere, P Frith, M Gaga, A Gamkrelidze, J Garces, J Garcia-Aymerich, B Gemicioğlu, J E. Gereda, S González Diaz, M Gotua, I Grisle, L Grouse, Z Gutter, M A. Guzmán, L G. Heaney, B Hellquist-Dahl, D Henderson, A Hendry, J Heinrich, D Heve, F Horak, J O' B Hourihane, P Howarth, M Humbert, M E. Hyland, M Illario, J C. Ivancevich, J R. Jardim, E J. Jares, C Jeandel, C Jenkins, S L. Johnston, O Jonquet, K Julge, K S. Jung, J Just, I Kaidashev, M R. Khaitov, O Kalayci, A F. Kalyoncu, T Keil, P K. Keith, L Klimek, B Koffi N'Goran, V Kolek, G H. Koppelman, M L. Kowalski, I Kull, P Kuna, V Kvedariene, B Lambrecht, S Lau, D Larenas-Linnemann, D Laune, L T T Le, P Lieberman, B Lipworth, J Li, K Lodrup Carlsen, R Louis, W Macnee, Y Magard, A Magnan, B Mahboub, A Mair, I Majer, M J. Makela, P Manning, S Mara, G D. Marshall, M R. Masjedi, P Matignon, M Maurer, S Mavale-Manuel, E Melén, E Melo-Gomes, E O. Meltzer, A Menzies-Gow, H Merk, J P. Michel, N Miculinic, F Mihaltan, B Milenkovic, G M Y Mohammad, M Molimard, I Momas, A Montilla-Santana, M Morais-Almeida, M Morgan, R Mösges, J Mullol, S Nafti, L Namazova-Baranova, R Naclerio, A Neou, H Neffen, K Nekam, B Niggemann, G Ninot, T D. Nyembue, R E. O'Hehir, K Ohta, Y Okamoto, K Okubo, S Ouedraogo, P Paggiaro, I Pali-Schöll, P Panzner, N Papadopoulos, A Papi, H S. Park, G Passalacqua, I Pavord, R Pawankar, R Pengelly, O Pfaar, R Picard, B Pigearias, I Pin, D Plavec, D Poethig, W Pohl, T A. Popov, F Portejoie, P Potter, D Postma, D Price, K F. Rabe, F Raciborski, F Radier Pontal, S Repka-Ramirez, S Reitamo, S Rennard, F Rodenas, J Roberts, J Roca, L Rodriguez Mañas, C Rolland, M Roman Rodriguez, A Romano, J Rosado-Pinto, N Rosario, L Rosenwasser, M Rottem, D Ryan, M Sanchez-Borges, G K. Scadding, H J. Schunemann, E Serrano, P Schmid-Grendelmeier, H Schulz, A Sheikh, M Shields, N Siafakas, Y Sibille, T Similowski, F E R Simons, J C. Sisul, I Skrindo, H A. Smit, D Solé, T Sooronbaev, O Spranger, R Stelmach, P J. Sterk, J Sunyer, C Thijs, T To, A Todo-Bom, M Triggiani, R Valenta, A L. Valero, E Valia, E Valovirta, E Van Ganse, M Van Hage, O Vandenplas, T Vasankari, B Vellas, J Vestbo, G Vezzani, P Vichyanond, G Viegi, C Vogelmeier, T Vontetsianos, M Wagenmann, B Wallaert, S Walker, D Y. Wang, U Wahn, M Wickman, D M. Williams, S Williams, J Wright, B P. Yawn, P K. Yiallouros, O M. Yusuf, A Zaidi, H J. Zar, M E. Zernotti, L Zhang, N Zhong, M Zidarn, J Mercier

Manuscripts, Articles, Book Chapters and Other Papers

[This corrects the article DOI: 10.1186/s13601-016-0116-9.].


Yap And The Hippo Pathway In Pediatric Cancer., Atif Ahmed, Abdalla D. Mohamed, Melissa Gener, Weijie Li, Eugenio Taboada Jan 2017

Yap And The Hippo Pathway In Pediatric Cancer., Atif Ahmed, Abdalla D. Mohamed, Melissa Gener, Weijie Li, Eugenio Taboada

Manuscripts, Articles, Book Chapters and Other Papers

The Hippo pathway is an important signaling pathway that controls cell proliferation and apoptosis. It is evolutionarily conserved in mammals and is stimulated by cell-cell contact, inhibiting cell proliferation in response to increased cell density. During early embryonic development, the Hippo signaling pathway regulates organ development and size, and its functions result in the coordinated balance between proliferation, apoptosis, and differentiation. Its principal effectors, YAP and TAZ, regulate signaling by the embryonic stem cells and determine cell fate and histogenesis. Dysfunction of this pathway contributes to cancer development in adults and children. Emerging studies have shed light on the upregulation …


Multiple Targets For Novel Therapy Of Fsgs Associated With Circulating Permeability Factor., Virginia J. Savin, Mukut Sharma, Jianping Zhou, David Genochi, Ram Sharma, Tarak Srivastava, Amna Ilahe, Pooja Budhiraja, Aditi Gupta, Ellen T. Mccarthy Jan 2017

Multiple Targets For Novel Therapy Of Fsgs Associated With Circulating Permeability Factor., Virginia J. Savin, Mukut Sharma, Jianping Zhou, David Genochi, Ram Sharma, Tarak Srivastava, Amna Ilahe, Pooja Budhiraja, Aditi Gupta, Ellen T. Mccarthy

Manuscripts, Articles, Book Chapters and Other Papers

A plasma component is responsible for altered glomerular permeability in patients with focal segmental glomerulosclerosis. Evidence includes recurrence after renal transplantation, remission after plasmapheresis, proteinuria in infants of affected mothers, transfer of proteinuria to experimental animals, and impaired glomerular permeability after exposure to patient plasma. Therapy may include decreasing synthesis of the injurious agent, removing or blocking its interaction with cells, or blocking signaling or enhancing cell defenses to restore the permeability barrier and prevent progression. Agents that may prevent the synthesis of the permeability factor include cytotoxic agents or aggressive chemotherapy. Extracorporeal therapies include plasmapheresis, immunoadsorption with protein A …


Bleeding Meckel's Diverticulum In Children: The Diagnostic Value Of Double-Balloon Enteroscopy., Lan-Lan Geng, Pei-Yu Chen, Qiang Wu, Hui-Wen Li, Ding-You Li, Min Yang, Si-Tang Gong Jan 2017

Bleeding Meckel's Diverticulum In Children: The Diagnostic Value Of Double-Balloon Enteroscopy., Lan-Lan Geng, Pei-Yu Chen, Qiang Wu, Hui-Wen Li, Ding-You Li, Min Yang, Si-Tang Gong

Manuscripts, Articles, Book Chapters and Other Papers

Background. Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract. The purpose of this study was to evaluate the diagnostic value and safety of double-balloon enteroscopy (DBE) for bleeding MD in children. Methods. We included consecutive children who were highly suspected of MD between 2012 and 2013. All patients underwent Meckel's scan. DBE was performed for patient with negative Meckel's scan. An exploratory laparoscopy was performed in children with positive Meckel's scan or DBE. Results. 42 patients met the inclusion criteria. 40 patients were confirmed to have MD by exploratory laparoscopy. Meckel's scan …


A Novel Compound-Heterozygous Epithelial Cell Adhesion Molecule Mutation In Tufting Enteropathy., Valentina Shakhnovich, Darrell Dinwiddie, Amber Hildreth, Thomas M. Attard, Stephen Kingsmore Jan 2017

A Novel Compound-Heterozygous Epithelial Cell Adhesion Molecule Mutation In Tufting Enteropathy., Valentina Shakhnovich, Darrell Dinwiddie, Amber Hildreth, Thomas M. Attard, Stephen Kingsmore

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.


A Singleton Infant With Bilateral Renal Agenesis And Normal Pulmonary Function., Lovya George, Winston M. Manimtim, Jotishna Sharma Jan 2017

A Singleton Infant With Bilateral Renal Agenesis And Normal Pulmonary Function., Lovya George, Winston M. Manimtim, Jotishna Sharma

Manuscripts, Articles, Book Chapters and Other Papers

Bilateral renal agenesis leads to anhydramnios and other sequelae including pulmonary hypoplasia. There have been rare case reports of normal pulmonary function in the presence of bilateral renal agenesis in monoamniotic discordant twins, but this has never been reported in a singleton pregnancy. The few reported cases in twins have all been fatal in the neonatal period with no reported cases of survival beyond 2 months. We describe the first case of a singleton infant with bilateral renal agenesis who had normal pulmonary function and did well on peritoneal dialysis for 4 years while awaiting a renal transplant.


Pediatric Ovarian Growing Teratoma Syndrome., Rebecca M. Rentea, Aaron Varghese, Atif Ahmed, Alexander Kats, Michelle Manalang, Tazim Dowlut-Mcelroy, Richard J. Hendrickson Jan 2017

Pediatric Ovarian Growing Teratoma Syndrome., Rebecca M. Rentea, Aaron Varghese, Atif Ahmed, Alexander Kats, Michelle Manalang, Tazim Dowlut-Mcelroy, Richard J. Hendrickson

Manuscripts, Articles, Book Chapters and Other Papers

Ovarian immature teratoma is a germ cell tumor that comprises less than 1% of ovarian cancers and is treated with surgical debulking and chemotherapy depending on stage. Growing teratoma syndrome (GTS) is the phenomenon of the growth of mature teratoma elements with normal tumor markers during or following chemotherapy for treatment of a malignant germ cell tumor. These tumors are associated with significant morbidity and mortality due to invasive and compressive growth as well as potential for malignant transformation. Current treatment modality is surgical resection. We discuss a 12-year-old female who presented following resection of a pure ovarian immature teratoma …