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Children's Mercy Kansas City

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Multi-Use Books In Waiting Rooms: Summary, Children's Mercy Kansas City Jan 2020

Multi-Use Books In Waiting Rooms: Summary, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


An Electronic Medical Records-Based Approach To Identify Idiosyncratic Drug-Induced Liver Injury In Children., Tracy L. Sandritter, Jennifer Goldman, Clayton J Habiger, James F. Daniel, Jennifer Lowry, Ryan T. Fischer Dec 2019

An Electronic Medical Records-Based Approach To Identify Idiosyncratic Drug-Induced Liver Injury In Children., Tracy L. Sandritter, Jennifer Goldman, Clayton J Habiger, James F. Daniel, Jennifer Lowry, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Drug-induced liver injury (DILI) is the leading cause of liver failure in the United States and the most common cause of drug recall. As opposed to the recognized direct toxicity of super-therapeutic acetaminophen or chemotherapeutic agents in children, limited data exists for pediatric populations on the incidence of idiosyncratic DILI (iDILI) that may develop independently of drug dose or duration of administration. To improve the detection of adverse drug reactions at our hospital, we utilized electronic medical records-based automated trigger tools to alert providers of potential iDILI. Clinical criteria concerning for iDILI were defined as serum ALT > 5x or serum …


Determining The Instructional Effectiveness Of Online Vaccine Education Modules: A Focus-Group Analysis, Sarah Williams, Shannon Clark, Sharon Humiston, Barbara Pahud, Donald Middleton, Kadriye O. Lewis Nov 2019

Determining The Instructional Effectiveness Of Online Vaccine Education Modules: A Focus-Group Analysis, Sarah Williams, Shannon Clark, Sharon Humiston, Barbara Pahud, Donald Middleton, Kadriye O. Lewis

Manuscripts, Articles, Book Chapters and Other Papers

Vaccine education for pediatric and family medicine residents is inadequate. Implementation of evidence-based instructional design methods for medical education is understudied. We conducted four focus groups with residents who had completed a novel immunization curriculum to explore their satisfaction with design, content and impact on confidence. Data were analyzed using thematic content analysis. Overall satisfaction with the curriculum was high. Residents valued the interactive design and content, reported improvement in confidence in discussing vaccines with parents, and shared recommendations for future iterations of the modules. Technical challenges were reported with the learning management system. Medical education modules developed using best …


Factors Affecting Acceptance Of At-Birth Point Of Care Hiv Testing Among Providers And Parents In Kenya: A Qualitative Study., Catherine Wexler, May Maloba, Melinda Brown, Natabhona Mabachi, Kathy Goggin, Brad Gautney, Beryne Odeny, Sarah Finocchario-Kessler Nov 2019

Factors Affecting Acceptance Of At-Birth Point Of Care Hiv Testing Among Providers And Parents In Kenya: A Qualitative Study., Catherine Wexler, May Maloba, Melinda Brown, Natabhona Mabachi, Kathy Goggin, Brad Gautney, Beryne Odeny, Sarah Finocchario-Kessler

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: At-birth and point-of-care (POC) HIV testing are emerging strategies to streamline infant HIV diagnosis and expedite ART initiation for HIV-positive infants. The purpose of this qualitative study was to evaluate factors influencing the provision and acceptance of at-birth POC testing among both HIV care providers and parents of HIV-exposed infants in Kenya.

METHODS: We conducted semi-structured interviews with 26 HIV care providers and 35 parents of HIV-exposed infants (including 23 mothers, 6 fathers, and 3 mother-father pairs) at four study hospitals prior to POC implementation. An overview of best available evidence related to POC was presented to participants prior …


Adapting The Diabetes Prevention Program For Low And Middle-Income Countries: Protocol For A Cluster Randomised Trial To Evaluate 'Lifestyle Africa'., Delwyn Catley, Thandi Puoane, Lungiswa Tsolekile, Ken Resnicow, Kandace Fleming, Emily A. Hurley, Joshua M. Smyth, Mara Z. Vitolins, Estelle V. Lambert, Naomi Levitt, Kathy Goggin Nov 2019

Adapting The Diabetes Prevention Program For Low And Middle-Income Countries: Protocol For A Cluster Randomised Trial To Evaluate 'Lifestyle Africa'., Delwyn Catley, Thandi Puoane, Lungiswa Tsolekile, Ken Resnicow, Kandace Fleming, Emily A. Hurley, Joshua M. Smyth, Mara Z. Vitolins, Estelle V. Lambert, Naomi Levitt, Kathy Goggin

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Low and middle-income countries like South Africa are experiencing major increases in burden of non-communicable diseases such as diabetes and cardiovascular conditions. However, evidence-based interventions to address behavioural factors related to these diseases are lacking. Our study aims to adapt the CDC's National Diabetes Prevention Program (DPP) within the context of an under-resourced urban community in Cape Town, South Africa.

Methods/analysis: The new intervention (Lifestyle Africa) consists of 17 weekly sessions delivered by trained community health workers (CHWs). In addition to educational and cultural adaptations of DPP content, the programme adds novel components of text messaging and …


Nearly Complete Genome Sequences Of 17 Enterovirus D68 Strains From Kansas City, Missouri, 2018, Suman B. Pakala, Yi Tan, Ferdaus Hassan, Annie Mai, Robert H. Markowitz, Meghan H. Shilts, Seesandra V. Rajagopala, Rangaraj Selvarangan, Suman R. Das Nov 2019

Nearly Complete Genome Sequences Of 17 Enterovirus D68 Strains From Kansas City, Missouri, 2018, Suman B. Pakala, Yi Tan, Ferdaus Hassan, Annie Mai, Robert H. Markowitz, Meghan H. Shilts, Seesandra V. Rajagopala, Rangaraj Selvarangan, Suman R. Das

Manuscripts, Articles, Book Chapters and Other Papers

Here, we report 17 nearly complete genome sequences of enterovirus D68 (EV-D68) isolated from Kansas City, MO, in 2018. Phylogenetic analysis suggests that these strains belong to subclade B3, similar to the ones that caused the 2016 epidemics in the United States but different from the 2014 outbreak B1 strains.


Choice Of Conditioning Regimens For Bone Marrow Transplantation In Severe Aplastic Anemia., Nelli Bejanyan, Soyoung Kim, Kyle M. Hebert, Natasha Kekre, Hisham Abdel-Azim, Ibrahim A. Ahmed, Mahmoud Aljurf, Sherif M. Badawy, Amer Beitinjaneh, Jaap Jan Boelens, Miguel Angel Diaz, Christopher C. Dvorak, Shahinaz Gadalla, James Gajewski, Robert Peter Gale, Siddhartha Ganguly, Andrew R. Gennery, Biju George, Usama Gergis, David Gómez-Almaguer, Marta Gonzalez Vicent, Hasan Hashem, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Vikram Mathews, Paul J. Orchard, Michael Pulsipher, Olle Ringden, Kirk Schultz, Pierre Teira, Ann E. Woolfrey, Blachy Dávila Saldaña, Bipin Savani, Jacek Winiarski, Jean Yared, Daniel J. Weisdorf, Joseph H. Antin, Mary Eapen Oct 2019

Choice Of Conditioning Regimens For Bone Marrow Transplantation In Severe Aplastic Anemia., Nelli Bejanyan, Soyoung Kim, Kyle M. Hebert, Natasha Kekre, Hisham Abdel-Azim, Ibrahim A. Ahmed, Mahmoud Aljurf, Sherif M. Badawy, Amer Beitinjaneh, Jaap Jan Boelens, Miguel Angel Diaz, Christopher C. Dvorak, Shahinaz Gadalla, James Gajewski, Robert Peter Gale, Siddhartha Ganguly, Andrew R. Gennery, Biju George, Usama Gergis, David Gómez-Almaguer, Marta Gonzalez Vicent, Hasan Hashem, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Vikram Mathews, Paul J. Orchard, Michael Pulsipher, Olle Ringden, Kirk Schultz, Pierre Teira, Ann E. Woolfrey, Blachy Dávila Saldaña, Bipin Savani, Jacek Winiarski, Jean Yared, Daniel J. Weisdorf, Joseph H. Antin, Mary Eapen

Manuscripts, Articles, Book Chapters and Other Papers

Allogeneic bone marrow transplantation (BMT) is curative therapy for the treatment of patients with severe aplastic anemia (SAA). However, several conditioning regimens can be used for BMT. We evaluated transplant conditioning regimens for BMT in SAA after HLA-matched sibling and unrelated donor BMT. For recipients of HLA-matched sibling donor transplantation (n = 955), fludarabine (Flu)/cyclophosphamide (Cy)/antithymocyte globulin (ATG) or Cy/ATG led to the best survival. The 5-year probabilities of survival with Flu/Cy/ATG, Cy/ATG, Cy ± Flu, and busulfan/Cy were 91%, 91%, 80%, and 84%, respectively (P = .001). For recipients of 8/8 and 7/8 HLA allele-matched unrelated donor transplantation (n …


Aap To Honor Dr. Jackson With Lifetime Achievement Award, Children's Mercy Hospital Oct 2019

Aap To Honor Dr. Jackson With Lifetime Achievement Award, Children's Mercy Hospital

Our Story Continues

Mary Anne Jackson, MD, FAAP, FPIDS, FIDSA, is a rarity among physicians. Her curriculum vitae reads like a virtual “Who’s Who” in pediatric infectious diseases. She’s served as an associate editor of the American Academy of Pediatrics’ Red Book, a musthave reference for pediatricians across the nation.

Plus, she’s recently added the title of Interim Dean, University of Missouri-Kansas City School of Medicine, to her long list of accomplishments. She is also a Professor of Pediatrics and Pediatric Infectious Diseases specialist at Children’s Mercy Kansas City.

The AAP Section on Infectious Diseases will present Dr. Mary Anne Jackson with a …


Assessment And Improvement Of Data Collection Errors Through Inter-Departmental Collaboration, Charlott Williams, Kelli L. Behr, Mary Moffatt, Rangaraj Selvarangan Oct 2019

Assessment And Improvement Of Data Collection Errors Through Inter-Departmental Collaboration, Charlott Williams, Kelli L. Behr, Mary Moffatt, Rangaraj Selvarangan

Posters

Specific Aims:

  1. To maintain the ED team's high rate of accuracy in data collection
  2. To begin team participation in corrective action planning
  3. To improve interdepartmental problem solving

Conclusion

Open communication about errors among all collaborating departments, combing with a shared approach to solving them:

  • improved morale and perception of error tracking by the team
  • led to a decrease in errors overall, and
  • increased interdepartmental collaboration

When all members of the interdepartmental team work together with a positive approach to corrective action, improvement in error rates is a natural outcome of the solutions derived.


Improving Pneumococcal Vaccination Rates In High Risk Patients Across Multiple Specialty Divisions, Rachel Moran, Julia G. Harris, Claire Olsen, Rana El Feghaly, Liset Olarte, Douglas Blowey, Luke A. Harris Oct 2019

Improving Pneumococcal Vaccination Rates In High Risk Patients Across Multiple Specialty Divisions, Rachel Moran, Julia G. Harris, Claire Olsen, Rana El Feghaly, Liset Olarte, Douglas Blowey, Luke A. Harris

Posters

Pediatric patients with deficient immune systems or certain chronic medical conditions have an increased risk of acquiring invasive pneumococcal disease.

The 23-valent pneumococcal (PPSV23) vaccine provides protection against 23 pneumococcal serotypes and is recommended for patients aged 2 years or older who are high-risk for invasive pneumococcal disease.

Unfortunately, many high-risk patients are not properly vaccinated due to lack of provider knowledge or understanding of accountability between primary care and specialty providers.

The goal of this project was to improve PPSV23 vaccination rates by 10-20% across multiple Children's Mercy Kansas City specialty divisions.


Related And Unrelated Donor Transplantation For Β-Thalassemia Major: Results Of An International Survey., Chunfu Li, Vikram Mathews, Soyoung Kim, Biju George, Kyle Hebert, Hua Jiang, Changgang Li, Yiping Zhu, Daniel A. Keesler, Jaap Jan Boelens, Christopher C. Dvorak, Rajni Agarwal, Jeffery J. Auletta, Rakesh K. Goyal, Rabi Hanna, Kimberly Kasow, Shalini Shenoy, Angela R. Smith, Mark C. Walters, Mary Eapen Sep 2019

Related And Unrelated Donor Transplantation For Β-Thalassemia Major: Results Of An International Survey., Chunfu Li, Vikram Mathews, Soyoung Kim, Biju George, Kyle Hebert, Hua Jiang, Changgang Li, Yiping Zhu, Daniel A. Keesler, Jaap Jan Boelens, Christopher C. Dvorak, Rajni Agarwal, Jeffery J. Auletta, Rakesh K. Goyal, Rabi Hanna, Kimberly Kasow, Shalini Shenoy, Angela R. Smith, Mark C. Walters, Mary Eapen

Manuscripts, Articles, Book Chapters and Other Papers

We studied 1110 patients with β-thalassemia major aged ≤25 years who received transplants with grafts from HLA-matched related (n = 677; 61%), HLA-mismatched related (n = 78; 7%), HLA-matched unrelated (n = 252; 23%), and HLA-mismatched unrelated (n = 103; 9%) donors between 2000 and 2016. Ninety percent of transplants were performed in the last decade. Eight-five percent of patients received ≥20 transfusions and 88% were inadequately chelated. All patients received myeloablative-conditioning regimen. Overall and event-free survival were highest for patients aged ≤6 years and after HLA-matched related and HLA-matched unrelated donor transplantation. The 5-year probabilities of overall survival for …


Antibiotic Resistance And Molecular Characterization Of Bacteremia Escherichia Coli Isolates From Newborns In The United States., Bryan K. Cole, Marko Ilikj, Cindy B. Mccloskey, Susana Chavez-Bueno Jul 2019

Antibiotic Resistance And Molecular Characterization Of Bacteremia Escherichia Coli Isolates From Newborns In The United States., Bryan K. Cole, Marko Ilikj, Cindy B. Mccloskey, Susana Chavez-Bueno

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Escherichia coli is a major cause of neonatal sepsis. Contemporary antibiotic resistance data and molecular characterization of neonatal E. coli bacteremia isolates in the US are limited.

METHODS: E. coli blood isolates, antibiotic susceptibility data, and clinical characteristics were obtained from prospectively identified newborns from 2006 to 2016. The E. coli isolates were classified using an updated phylogrouping method and multi-locus sequence typing. The presence of several virulence traits was also determined.

RESULTS: Forty-three newborns with E. coli bacteremia were identified. Mean gestational age was 32.3 (SD±5.4) weeks. Median age was 7 days (interquartile range 0-10). Mortality (28%) occurred …


2019-2020 Pediatric Subspecialty Trainees, Children's Mercy Hospital Jul 2019

2019-2020 Pediatric Subspecialty Trainees, Children's Mercy Hospital

Residents and Fellows Portraits

Photo composites of the graduate pediatric trainees in allergy/immunology, child abuse pediatrics, child neurology, clinical child psychology, developmental-behavioral pediatrics, epilepsy; headache medicine; hospice & palliative care, laboratory genetics & genomics, neonatal-perinatal medicine, pediatric adolescent gynecology, pediatric anesthesiology, pediatric bioethics, pediatric cardiology, pediatric clinical pharmacology, pediatric critical care medicine, pediatric cardiac congenital surgery; pediatric dentistry, pediatric emergency medicine, pediatric EM/child abuse; pediatric endocrinology, pediatric gastroenterology, pediatric hematology/oncology, pediatric hospital medicine, pediatric infectious diseases, pediatric nephrology, pediatric ophthalmology, pediatric optometry, pediatric otolaryngology, pediatric pulmonology, pediatric rehabilitation, pediatric sports medicine, pediatric surgery, pharmacy, sleep medicine, pediatric residency and med/peds residency.


The Concentration Of Total Nucleated Cells In Harvested Bone Marrow For Transplantation Has Decreased Over Time., Nicole L. Prokopishyn, Brent R. Logan, Deidre M. Kiefer, Jennifer A. Sees, Pintip Chitphakdithai, Ibrahim A. Ahmed, Paolo N. Anderlini, Amer M. Beitinjaneh, Christopher Bredeson, Jan Cerny, Saurabh Chhabra, Andrew Daly, Miguel Angel Diaz, Nosha Farhadfar, Haydar A. Frangoul, Siddhartha Ganguly, Dennis A. Gastineau, Usama Gergis, Gregory A. Hale, Peiman Hematti, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Hemant S. Murthy, Maxim Norkin, Richard F. Olsson, Mona Papari, Bipin N. Savani, Jeffrey Szer, Edmund K. Waller, Baldeep Wirk, Jean A. Yared, Michael A. Pulsipher, Nirali N. Shah, Galen E. Switzer, Paul V. O'Donnell, Dennis L. Confer, Bronwen E. Shaw Jul 2019

The Concentration Of Total Nucleated Cells In Harvested Bone Marrow For Transplantation Has Decreased Over Time., Nicole L. Prokopishyn, Brent R. Logan, Deidre M. Kiefer, Jennifer A. Sees, Pintip Chitphakdithai, Ibrahim A. Ahmed, Paolo N. Anderlini, Amer M. Beitinjaneh, Christopher Bredeson, Jan Cerny, Saurabh Chhabra, Andrew Daly, Miguel Angel Diaz, Nosha Farhadfar, Haydar A. Frangoul, Siddhartha Ganguly, Dennis A. Gastineau, Usama Gergis, Gregory A. Hale, Peiman Hematti, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Hemant S. Murthy, Maxim Norkin, Richard F. Olsson, Mona Papari, Bipin N. Savani, Jeffrey Szer, Edmund K. Waller, Baldeep Wirk, Jean A. Yared, Michael A. Pulsipher, Nirali N. Shah, Galen E. Switzer, Paul V. O'Donnell, Dennis L. Confer, Bronwen E. Shaw

Manuscripts, Articles, Book Chapters and Other Papers

Bone marrow (BM) is an essential source of hematopoietic stem cell grafts for many allogeneic hematopoietic cell transplant (HCT) recipients, including adult patients (for specific diseases and transplantation strategies) and the majority of pediatric recipient. However, since the advent of granulocyte colony-stimulating factor-mobilized peripheral blood stem cell (PBSC) grafts, there has been a significant decrease in the use of BM in HCT, thought to be due mainly to the increased logistical challenges in harvesting BM compared with PBSCs, as well as generally no significant survival advantage of BM over PBSCs. The decreased frequency of collection has the potential to impact …


Use Of The Who Access, Watch, And Reserve Classification To Define Patterns Of Hospital Antibiotic Use (Aware): An Analysis Of Paediatric Survey Data From 56 Countries., Yingfen Hsia, Brian R. Lee, Ann Versporten, Yonghong Yang, Julia Bielicki, Charlotte Jackson, Jason Newland, Herman Goossens, Nicola Magrini, Mike Sharland, Garpec And Global-Pps Networks Jul 2019

Use Of The Who Access, Watch, And Reserve Classification To Define Patterns Of Hospital Antibiotic Use (Aware): An Analysis Of Paediatric Survey Data From 56 Countries., Yingfen Hsia, Brian R. Lee, Ann Versporten, Yonghong Yang, Julia Bielicki, Charlotte Jackson, Jason Newland, Herman Goossens, Nicola Magrini, Mike Sharland, Garpec And Global-Pps Networks

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Improving the quality of hospital antibiotic use is a major goal of WHO's global action plan to combat antimicrobial resistance. The WHO Essential Medicines List Access, Watch, and Reserve (AWaRe) classification could facilitate simple stewardship interventions that are widely applicable globally. We aimed to present data on patterns of paediatric AWaRe antibiotic use that could be used for local and national stewardship interventions.

METHODS: 1-day point prevalence survey antibiotic prescription data were combined from two independent global networks: the Global Antimicrobial Resistance, Prescribing, and Efficacy in Neonates and Children and the Global Point Prevalence Survey on Antimicrobial Consumption and …


Provider Education And Rapid Antigen Detection Test Use In An Academic Pediatric Clinic, Steven Dahl May 2019

Provider Education And Rapid Antigen Detection Test Use In An Academic Pediatric Clinic, Steven Dahl

Research Days

No abstract provided.


Impact Of A Mobile Device-Based Clinical Decision Support Tool On Guideline Adherence And Mental Workload Among Trainee And Attending Physicians, Katherine M. Richardson May 2019

Impact Of A Mobile Device-Based Clinical Decision Support Tool On Guideline Adherence And Mental Workload Among Trainee And Attending Physicians, Katherine M. Richardson

Presentations

This study evaluated PedsGuide, an app used to evaluate serious bacterial infections in febrile infants.


The Disproportionate Impact Of Obesity Among Hospitalized Children, Kathyrn Kyler May 2019

The Disproportionate Impact Of Obesity Among Hospitalized Children, Kathyrn Kyler

Research Days

No abstract provided.


Herpes Simplex Virus Whole Genome Sequencing For Antiviral Resistance In A Child With Dock8 Deficiency And Chronic Infection, Sean Stout May 2019

Herpes Simplex Virus Whole Genome Sequencing For Antiviral Resistance In A Child With Dock8 Deficiency And Chronic Infection, Sean Stout

Research Days

No abstract provided.


Chronic Cavitary Pulmonary Aspergillosis With Aspergilloma In A Previously Healthy Teenager, Michael D. Mcdowell Jr May 2019

Chronic Cavitary Pulmonary Aspergillosis With Aspergilloma In A Previously Healthy Teenager, Michael D. Mcdowell Jr

Research Days

No abstract provided.


Optimization Of Surgical Prophylaxis In Penicillin-Allergic Labeled Patients, Katie A. Vandervelde May 2019

Optimization Of Surgical Prophylaxis In Penicillin-Allergic Labeled Patients, Katie A. Vandervelde

Research Days

No abstract provided.


Viral Whole Genome Sequencing For Antiviral Resistance In A Child With Dock8 Deficiency And Recurrent Hsv-1, Sean Stout, A. L. Greninger, Rangaraj Selvarangan, A. F. Freeman, Brandon D. Newell, Erin Stahl, Dwight Yin May 2019

Viral Whole Genome Sequencing For Antiviral Resistance In A Child With Dock8 Deficiency And Recurrent Hsv-1, Sean Stout, A. L. Greninger, Rangaraj Selvarangan, A. F. Freeman, Brandon D. Newell, Erin Stahl, Dwight Yin

Posters

We present the case of a child with dedicator of cytokinesis 8 (DOCK8) deficiency and chronic, resistant HSV-1 mucocutaneous infections to illustrate the clinical utility of viral whole genome sequencing to detect active and latent HSV resistance mutations.


Dehydrated Hereditary Stomatocytosis Causing Sever Ascites Leading To Pulmonary Hypoplasia And Respiratory Insufficiency In A Neonate, Michael D. Mcdowell Jr, Christopher M. Oermann May 2019

Dehydrated Hereditary Stomatocytosis Causing Sever Ascites Leading To Pulmonary Hypoplasia And Respiratory Insufficiency In A Neonate, Michael D. Mcdowell Jr, Christopher M. Oermann

Posters

Care Presentation: EM is a 32.5-week EGA infant who was born with sever congenital ascites (following repeated prenatal paracentesis) identified on prenatal ultrasound. Initial chest-abdomen imaging is shown in Figure 1. He required CPAP at delivery but was weaned of high flow nasal cannula with intermittent episodes of prolonged intubation during repeat paracentesis. Paracentesis demonstrated simple ascites rather than chylous fluid. Lymphangiograms demonstrated transient lymphatic leakage with resolution. Genetic evaluation revealed a pathogenic mutation in the PIEZO 1 gene for autosomal dominant DHS. This disorder had reduced penetrance and variable expressivity and symptoms range for absence of clinical features to …


Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections In Children With Cystic Fibrosis., Peter Jorth, Zarmina Ehsan Md, Amir Rezayat, Ellen Caldwell, Christopher Pope, John J. Brewington, Christopher H. Goss, Dan Benscoter, John P. Clancy, Pradeep K. Singh Apr 2019

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections In Children With Cystic Fibrosis., Peter Jorth, Zarmina Ehsan Md, Amir Rezayat, Ellen Caldwell, Christopher Pope, John J. Brewington, Christopher H. Goss, Dan Benscoter, John P. Clancy, Pradeep K. Singh

Manuscripts, Articles, Book Chapters and Other Papers

Culture and sequencing have produced divergent hypotheses about cystic fibrosis (CF) lung infections. Culturing suggests that CF lungs are uninfected before colonization by a limited group of CF pathogens. Sequencing suggests diverse communities of mostly oral bacteria inhabit lungs early on and diversity decreases as disease progresses. We studied the lung microbiota of CF children using bronchoscopy and sequencing, with measures to reduce contamination. We found no evidence for oral bacterial communities in lung lavages that lacked CF pathogens. Lavage microbial diversity varied widely, but decreases in diversity appeared to be driven by increased CF pathogen abundance, which reduced the …


Effect Of Aging And Predonation Comorbidities On The Related Peripheral Blood Stem Cell Donor Experience: Report From The Related Donor Safety Study., Michael A. Pulsipher, Brent R. Logan, Pintip Chitphakdithai, Deidre M. Kiefer, Marcie L. Riches, J Douglas Rizzo, Paolo Anderlini, Susan F. Leitman, James W. Varni, Hati Kobusingye, Raeanne M. Besser, John P. Miller, Rebecca J. Drexler, Aly Abdel-Mageed, Ibrahim A. Ahmed, Luke P. Akard, Andrew S. Artz, Edward D. Ball, Ruthee-Lu Bayer, Carolyn Bigelow, Brian J. Bolwell, E Randolph Broun, Nancy J. Bunin, David C. Delgado, Katharine Duckworth, Christopher C. Dvorak, Theresa E. Hahn, Ann E. Haight, Parameswaran N. Hari, Brandon M. Hayes-Lattin, David A. Jacobsohn, Ann A. Jakubowski, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Michael Linenberger, Mark R. Litzow, Walter Longo, Margarida Magalhaes-Silverman, John M. Mccarty, Joseph P. Mcguirk, Shahram Mori, Vinod K. Prasad, Scott D. Rowley, Witold B. Rybka, Indira Sahdev, Jeffrey R. Schriber, George B. Selby, Paul J. Shaughnessy, Shalini Shenoy, Thomas Spitzer, William T. Tse, Joseph P. Uberti, Madhuri Vusirikala, Edmund K. Waller, Daniel J. Weisdorf, Gregory A. Yanik, Willis H. Navarro, Mary M. Horowitz, Galen E. Switzer, Bronwen E. Shaw, Dennis L. Confer Apr 2019

Effect Of Aging And Predonation Comorbidities On The Related Peripheral Blood Stem Cell Donor Experience: Report From The Related Donor Safety Study., Michael A. Pulsipher, Brent R. Logan, Pintip Chitphakdithai, Deidre M. Kiefer, Marcie L. Riches, J Douglas Rizzo, Paolo Anderlini, Susan F. Leitman, James W. Varni, Hati Kobusingye, Raeanne M. Besser, John P. Miller, Rebecca J. Drexler, Aly Abdel-Mageed, Ibrahim A. Ahmed, Luke P. Akard, Andrew S. Artz, Edward D. Ball, Ruthee-Lu Bayer, Carolyn Bigelow, Brian J. Bolwell, E Randolph Broun, Nancy J. Bunin, David C. Delgado, Katharine Duckworth, Christopher C. Dvorak, Theresa E. Hahn, Ann E. Haight, Parameswaran N. Hari, Brandon M. Hayes-Lattin, David A. Jacobsohn, Ann A. Jakubowski, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Michael Linenberger, Mark R. Litzow, Walter Longo, Margarida Magalhaes-Silverman, John M. Mccarty, Joseph P. Mcguirk, Shahram Mori, Vinod K. Prasad, Scott D. Rowley, Witold B. Rybka, Indira Sahdev, Jeffrey R. Schriber, George B. Selby, Paul J. Shaughnessy, Shalini Shenoy, Thomas Spitzer, William T. Tse, Joseph P. Uberti, Madhuri Vusirikala, Edmund K. Waller, Daniel J. Weisdorf, Gregory A. Yanik, Willis H. Navarro, Mary M. Horowitz, Galen E. Switzer, Bronwen E. Shaw, Dennis L. Confer

Manuscripts, Articles, Book Chapters and Other Papers

The development of reduced-intensity approaches for allogeneic hematopoietic cell transplantation has resulted in growing numbers of older related donors (RDs) of peripheral blood stem cells (PBSCs). The effects of age on donation efficacy, toxicity, and long-term recovery in RDs are poorly understood. To address this we analyzed hematologic variables, pain, donation-related symptoms, and recovery in 1211 PBSC RDs aged 18 to 79 enrolled in the Related Donor Safety Study. RDs aged > 60 had a lower median CD34+ level before apheresis compared with younger RDs (age > 60, 59 × 106/L; age 41 to 60, 81 × 106/L; age 18 to 40, …


Co-Localization Of Autophagy-Related Protein P62 With Cancer Stem Cell Marker Dclk1 May Hamper Dclk1'S Elimination During Colon Cancer Development And Progression., Badal Chandra Roy, Ishfaq Ahmed, Satish Ramalingam, Venkatakrishna Jala, Bodduluri Haribabu, Prabhu Ramamoorthy, John Ashcraft, Joseph Valentino, Shrikant Anant, Venkatesh Sampath, Shahid Umar Mar 2019

Co-Localization Of Autophagy-Related Protein P62 With Cancer Stem Cell Marker Dclk1 May Hamper Dclk1'S Elimination During Colon Cancer Development And Progression., Badal Chandra Roy, Ishfaq Ahmed, Satish Ramalingam, Venkatakrishna Jala, Bodduluri Haribabu, Prabhu Ramamoorthy, John Ashcraft, Joseph Valentino, Shrikant Anant, Venkatesh Sampath, Shahid Umar

Manuscripts, Articles, Book Chapters and Other Papers

Autophagy may play a critical role in colon cancer stem cells (CCSCs)-related cancer development. Here, we investigate whether accumulation of infection/injury-induced CCSCs due to impaired autophagy influences colon cancer development and progression. When Apc++ mice were infected with Citrobacter rodentium (CR; 109CFUs), we discovered presence of autophagosomes with increases in Beclin-1, LC3B and p62 staining during crypt hyperplasia. Apc1638N/+ mice when infected with CR or subjected to CR+AOM treatment, exhibited increased colon tumorigenesis with elevated levels of Ki-67, β-catenin, EZH2 and CCSC marker Dclk1, respectively. AOM/DSS treatment of Apc1638N/+ mice phenocopied CR+AOM treatment as colonic tumors exhibited pronounced changes in …


Second Case Of Hoip Deficiency Expands Clinical Features And Defines Inflammatory Transcriptome Regulated By Lubac., Hirotsugu Oda, David B. Beck, Hye Sun Kuehn, Natalia Sampaio Moura, Patrycja Hoffmann, Maria Ibarra, Jennifer Stoddard, Wanxia Li Tsai, Gustavo Gutierrez-Cruz, Massimo Gadina, Sergio D. Rosenzweig, Daniel L. Kastner, Luigi D. Notarangelo, Ivona Aksentijevich Mar 2019

Second Case Of Hoip Deficiency Expands Clinical Features And Defines Inflammatory Transcriptome Regulated By Lubac., Hirotsugu Oda, David B. Beck, Hye Sun Kuehn, Natalia Sampaio Moura, Patrycja Hoffmann, Maria Ibarra, Jennifer Stoddard, Wanxia Li Tsai, Gustavo Gutierrez-Cruz, Massimo Gadina, Sergio D. Rosenzweig, Daniel L. Kastner, Luigi D. Notarangelo, Ivona Aksentijevich

Manuscripts, Articles, Book Chapters and Other Papers

Background: HOIP is the catalytic subunit of the linear ubiquitination chain assembly complex (LUBAC) that is essential for NF-κB signaling and thus proper innate and adaptive immunity. To date only one patient with HOIP deficiency has been reported with clinical characteristics that include autoinflammation, immunodeficiency, amylopectinosis, and systemic lymphangiectasia. Case: We sought to identify a genetic cause of a disease for an 8 year-old girl who presented with early-onset immune deficiency and autoinflammation. Methods: Targeted next generation sequencing of 352 immune-related genes was performed. Functional studies included transcriptome analysis, cytokine profiling, and protein analysis in patients' primary cells. Results: We …


Effect Of Conditioning Regimen Dose Reduction In Obese Patients Undergoing Autologous Hematopoietic Cell Transplantation., Claudio G. Brunstein, Marcelo C. Pasquini, Soyoung Kim, Mingwei Fei, Kehinde Adekola, Ibrahim A. Ahmed, Mahmoud Aljurf, Vaibhav Agrawal, Jeffrey J. Auletta, Minoo Battiwalla, Nelli Bejanyan, Joseph Bubalo, Jan Cerny, Lynette Chee, Stefan O. Ciurea, Cesar Freytes, Shahinaz M. Gadalla, Robert Peter Gale, Siddhartha Ganguly, Shahrukh K. Hashmi, Peiman Hematti, Gerhard Hildebrandt, Leona A. Holmberg, Oscar B. Lahoud, Heather Landau, Hillard M. Lazarus, Marcos De Lima, Vikram Mathews, Richard Maziarz, Taiga Nishihori, Maxim Norkin, Richard Olsson, Ran Reshef, Seth Rotz, Bipin Savani, Harry C. Schouten, Sachiko Seo, Baldeep M. Wirk, Jean Yared, Shin Mineishi, John Rogosheske, Miguel-Angel Perales Mar 2019

Effect Of Conditioning Regimen Dose Reduction In Obese Patients Undergoing Autologous Hematopoietic Cell Transplantation., Claudio G. Brunstein, Marcelo C. Pasquini, Soyoung Kim, Mingwei Fei, Kehinde Adekola, Ibrahim A. Ahmed, Mahmoud Aljurf, Vaibhav Agrawal, Jeffrey J. Auletta, Minoo Battiwalla, Nelli Bejanyan, Joseph Bubalo, Jan Cerny, Lynette Chee, Stefan O. Ciurea, Cesar Freytes, Shahinaz M. Gadalla, Robert Peter Gale, Siddhartha Ganguly, Shahrukh K. Hashmi, Peiman Hematti, Gerhard Hildebrandt, Leona A. Holmberg, Oscar B. Lahoud, Heather Landau, Hillard M. Lazarus, Marcos De Lima, Vikram Mathews, Richard Maziarz, Taiga Nishihori, Maxim Norkin, Richard Olsson, Ran Reshef, Seth Rotz, Bipin Savani, Harry C. Schouten, Sachiko Seo, Baldeep M. Wirk, Jean Yared, Shin Mineishi, John Rogosheske, Miguel-Angel Perales

Manuscripts, Articles, Book Chapters and Other Papers

Data are limited on whether to adjust high-dose chemotherapy before autologous hematopoietic cell transplant (autoHCT) in obese patients. This study explores the effects of dose adjustment on the outcomes of obese patients, defined as body mass index (BMI) ≥ 30 kg/m2. Dose adjustment was defined as a reduction in standard dosing ≥20%, based on ideal, reported dosing and actual weights. We included 2 groups of US patients who had received autoHCT between 2008 and 2014. Specifically, we included patients with multiple myeloma (MM, n = 1696) treated with high-dose melphalan and patients with Hodgkin or non-Hodgkin lymphomas (n = 781) …


Neutralizing Antibody Against Enterovirus D68 In Children And Adults Before 2014 Outbreak, Kansas City, Missouri, Usa, Christopher J. Harrison, William C. Weldon, Barbara A. Pahud, Mary Anne Jackson, M. Steven Oberste, Rangaraj Selvarangan Mar 2019

Neutralizing Antibody Against Enterovirus D68 In Children And Adults Before 2014 Outbreak, Kansas City, Missouri, Usa, Christopher J. Harrison, William C. Weldon, Barbara A. Pahud, Mary Anne Jackson, M. Steven Oberste, Rangaraj Selvarangan

Manuscripts, Articles, Book Chapters and Other Papers

© 2019, Centers for Disease Control and Prevention (CDC). All rights reserved. We evaluated enterovirus D68 seroprevalence in Kansas City, Missouri, USA, from samples obtained during 2012-2013. Neutralizing antibodies against Fermon and the dominant 2014 Missouri isolate were universally detected. Titers increased with age. Widespread circulation of enterovirus D68 occurred before the 2014 outbreak. Research is needed to determine a surrogate of protection.


Characteristics Of Late Fatal Infections After Allogeneic Hematopoietic Cell Transplantation., Maxim Norkin, Bronwen E. Shaw, Ruta Brazauskas, Heather R. Tecca, Helen L. Leather, Juan Gea-Banacloche, Rammurti T. Kamble, Zachariah Defilipp, David A. Jacobsohn, Olle Ringden, Yoshihiro Inamoto, Kimberly A. Kasow, David Buchbinder, Peter Shaw, Peiman Hematti, Raquel Schears, Sherif M. Badawy, Hillard M. Lazarus, Neel Bhatt, Biljana Horn, Saurabh Chhabra, Kristin M. Page, Betty Hamilton, Gerhard C. Hildebrandt, Jean A. Yared, Vaibhav Agrawal, Amer M. Beitinjaneh, Navneet Majhail, Tamila Kindwall-Keller, Richard F. Olsson, Helene Schoemans, Robert Peter Gale, Siddhartha Ganguly, Ibrahim A. Ahmed, Harry C. Schouten, Jane L. Liesveld, Nandita Khera, Amir Steinberg, Ami J. Shah, Melhem Solh, David I. Marks, Witold Rybka, Mahmoud Aljurf, Andrew C. Dietz, Usama Gergis, Biju George, Sachiko Seo, Mary E D Flowers, Minoo Battiwalla, Bipin N. Savani, Marcie L. Riches, John R. Wingard Feb 2019

Characteristics Of Late Fatal Infections After Allogeneic Hematopoietic Cell Transplantation., Maxim Norkin, Bronwen E. Shaw, Ruta Brazauskas, Heather R. Tecca, Helen L. Leather, Juan Gea-Banacloche, Rammurti T. Kamble, Zachariah Defilipp, David A. Jacobsohn, Olle Ringden, Yoshihiro Inamoto, Kimberly A. Kasow, David Buchbinder, Peter Shaw, Peiman Hematti, Raquel Schears, Sherif M. Badawy, Hillard M. Lazarus, Neel Bhatt, Biljana Horn, Saurabh Chhabra, Kristin M. Page, Betty Hamilton, Gerhard C. Hildebrandt, Jean A. Yared, Vaibhav Agrawal, Amer M. Beitinjaneh, Navneet Majhail, Tamila Kindwall-Keller, Richard F. Olsson, Helene Schoemans, Robert Peter Gale, Siddhartha Ganguly, Ibrahim A. Ahmed, Harry C. Schouten, Jane L. Liesveld, Nandita Khera, Amir Steinberg, Ami J. Shah, Melhem Solh, David I. Marks, Witold Rybka, Mahmoud Aljurf, Andrew C. Dietz, Usama Gergis, Biju George, Sachiko Seo, Mary E D Flowers, Minoo Battiwalla, Bipin N. Savani, Marcie L. Riches, John R. Wingard

Manuscripts, Articles, Book Chapters and Other Papers

We analyzed late fatal infections (LFIs) in allogeneic stem cell transplantation (HCT) recipients reported to the Center for International Blood and Marrow Transplant Research. We analyzed the incidence, infection types, and risk factors contributing to LFI in 10,336 adult and 5088 pediatric subjects surviving for ≥2 years after first HCT without relapse. Among 2245 adult and 377 pediatric patients who died, infections were a primary or contributory cause of death in 687 (31%) and 110 (29%), respectively. At 12 years post-HCT, the cumulative incidence of LFIs was 6.4% (95% confidence interval [CI], 5.8% to 7.0%) in adults, compared with 1.8% …