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Children's Mercy Kansas City

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Articles 1 - 30 of 1931

Full-Text Articles in Medical Specialties

Sexual Function In Women With Polycystic Ovary Syndrome: A Systematic Review And Meta-Analysis., Hester Pastoor, Aya Mousa, Hanneke Bolt, Wichor Bramer, Tania S. Burgert, Anuja Dokras, Chau Thien Tay, Helena J. Teede, Joop Laven May 2024

Sexual Function In Women With Polycystic Ovary Syndrome: A Systematic Review And Meta-Analysis., Hester Pastoor, Aya Mousa, Hanneke Bolt, Wichor Bramer, Tania S. Burgert, Anuja Dokras, Chau Thien Tay, Helena J. Teede, Joop Laven

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Polycystic ovary syndrome (PCOS) is a common and distressing endocrine disorder associated with lower quality of life, subfertility, diabetes, cardiovascular disease, depression, anxiety, and eating disorders. PCOS characteristics, its comorbidities, and its treatment can potentially influence sexual function. However, studies on sexual function in women with PCOS are limited and contradictory.

OBJECTIVE AND RATIONALE: The aim was to perform a systematic review of the published literature on sexual function in women with PCOS and assess the quality of the research and certainty of outcomes, to inform the 2023 International Guidelines for the Assessment and Management of PCOS.

SEARCH METHODS: …


Population Pharmacokinetic Analysis Of Atomoxetine And Its Metabolites In Children And Adolescents With Attention-Deficit/Hyperactivity Disorder., Shen Cheng, Mahmoud Al-Kofahi, J Steven Leeder, Jacob T. Brown May 2024

Population Pharmacokinetic Analysis Of Atomoxetine And Its Metabolites In Children And Adolescents With Attention-Deficit/Hyperactivity Disorder., Shen Cheng, Mahmoud Al-Kofahi, J Steven Leeder, Jacob T. Brown

Manuscripts, Articles, Book Chapters and Other Papers

Atomoxetine (ATX) is a non-stimulant used to treat attention-deficit/hyperactivity disorder (ADHD) and systemic exposure is highly variable due to polymorphic cytochrome P450 2D6 (CYP2D6) activity. The objective of this study was to characterize the time course of ATX and metabolites (4-hydroxyatomoxetine (4-OH); N-desmethylatomoxetine (NDA); and 2-carboxymethylatomoxetine (2-COOH)) exposure following oral ATX dosing in children with ADHD to support individualized dosing. A nonlinear mixed-effect modeling approach was used to analyze ATX, 4-OH, and NDA plasma and urine, and 2-COOH urine profiles obtained over 24-72 hours from children with ADHD (n = 23) following a single oral ATX dose. Demographics and CYP2D6 …


Approach For Defining Human Adenovirus Infection And Disease For Central Review Adjudication In Clinical Studies., Brian T. Fisher, Jesse Blumenstock, Craig L K Boge, Sydney Shuster, Alix E. Seif, Michael Green, Marian G. Michaels, Jessie L. Alexander, Monica I. Ardura, Tamara P. Miller, Diego R. Hijano, William J. Muller, Jennifer E. Schuster, Abby M. Green, Daniel E. Dulek, Adriana E. Kajon, Lara Danziger-Isakov May 2024

Approach For Defining Human Adenovirus Infection And Disease For Central Review Adjudication In Clinical Studies., Brian T. Fisher, Jesse Blumenstock, Craig L K Boge, Sydney Shuster, Alix E. Seif, Michael Green, Marian G. Michaels, Jessie L. Alexander, Monica I. Ardura, Tamara P. Miller, Diego R. Hijano, William J. Muller, Jennifer E. Schuster, Abby M. Green, Daniel E. Dulek, Adriana E. Kajon, Lara Danziger-Isakov

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Pediatric allogeneic hematopoietic cell transplant (allo-HCT) recipients are at risk for morbidity and mortality from human adenovirus (HAdV). HAdV can be detected in an asymptomatic state, referred to as infection or with signs or symptoms of illness, referred to as disease. Standardized case definitions are needed to distinguish infection from disease and allow for consistent reporting in both observational cohort studies and therapeutic clinical trials.

METHODS: A working group of experts in virology, transplant infectious disease, and HCT was assembled to develop HAdV infection and disease definitions with the degree of certainty (i.e., possible, probable, and proven). Definitions were …


Availability And Utilization Of Endoscopic Retrograde Cholangiopancreatography At Children's Hospitals., Sagar J. Pathak, Thomas M. Attard, Matthew Hall, Mustafa Arain, Melvin B. Heyman, Emily R. Perito May 2024

Availability And Utilization Of Endoscopic Retrograde Cholangiopancreatography At Children's Hospitals., Sagar J. Pathak, Thomas M. Attard, Matthew Hall, Mustafa Arain, Melvin B. Heyman, Emily R. Perito

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: No study has explored whether availability of endoscopic retrograde cholangiopancreatography (ERCP) is adequate and equitable across US children's hospitals. We hypothesized that ERCP availability and utilization differs by geography and patient factors.

METHODS: Healthcare encounter data from 2009 to 2019 on children with pancreatic and biliary diseases from the Pediatric Health Information System were analyzed. ERCP availability was defined as treatment at a hospital that performed pediatric ERCP during the year of service.

RESULTS: From 2009 to 2019, 37,946 children (88,420 encounters) had a potential pancreatic or biliary indication for ERCP; 7066 ERCPs were performed. The commonest pancreatic diagnoses …


Recurrent Diffuse Alveolar Hemorrhage And Extracorporeal Membrane Oxygenation Utilization In A Hematopoietic Stem Cell Transplant Patient With Hunter's Syndrome., Nasreen Noor, Gene Peir, Asdis Finnsdottir Wagner, Jay Rilinger, Jenna Miller Apr 2024

Recurrent Diffuse Alveolar Hemorrhage And Extracorporeal Membrane Oxygenation Utilization In A Hematopoietic Stem Cell Transplant Patient With Hunter's Syndrome., Nasreen Noor, Gene Peir, Asdis Finnsdottir Wagner, Jay Rilinger, Jenna Miller

Manuscripts, Articles, Book Chapters and Other Papers

We describe the natural history of a three-month-old patient with Hunter Syndrome with hematopoietic stem cell transplant (HSCT) who developed recurrent diffuse alveolar hemorrhage (DAH) requiring extracorporeal membrane oxygenation (ECMO). The patient underwent HSCT with several complications, including veno-occlusive disease and DAH. He was managed with ECMO. Unfortunately, despite initial success he developed recurrent DAH and ultimately died. This is a novel report of this severe adverse event requiring ECMO following the use of HSCT in this rare patient population. We share the clinical strategies employed to address the complications associated with HSCT and the progression of his disease over …


Immunophenotypic Predictors Of Influenza Vaccine Immunogenicity In Pediatric Hematopoietic Cell Transplant Recipients., Justin Z. Amarin, Daniel E. Dulek, Joshua Simmons, Haya Hayek, James D. Chappell, Cindy Hager Nochowicz, Carrie L. Kitko, Jennifer E. Schuster, Flor M. Muñoz, Claire E. Bocchini, Elizabeth A. Moulton, Susan E. Coffin, Jason L. Freedman, Monica I. Ardura, Rachel L. Wattier, Gabriela Maron, Michael Grimley, Grant Paulsen, Lara Danziger-Isakov, Paul A. Carpenter, Janet A. Englund, Natasha B. Halasa, Andrew J. Spieker, Spyros A. Kalams Apr 2024

Immunophenotypic Predictors Of Influenza Vaccine Immunogenicity In Pediatric Hematopoietic Cell Transplant Recipients., Justin Z. Amarin, Daniel E. Dulek, Joshua Simmons, Haya Hayek, James D. Chappell, Cindy Hager Nochowicz, Carrie L. Kitko, Jennifer E. Schuster, Flor M. Muñoz, Claire E. Bocchini, Elizabeth A. Moulton, Susan E. Coffin, Jason L. Freedman, Monica I. Ardura, Rachel L. Wattier, Gabriela Maron, Michael Grimley, Grant Paulsen, Lara Danziger-Isakov, Paul A. Carpenter, Janet A. Englund, Natasha B. Halasa, Andrew J. Spieker, Spyros A. Kalams

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric hematopoietic cell transplant (HCT) recipients exhibit poor serologic responses to influenza vaccination early after transplant. To facilitate the optimization of influenza vaccination timing, we sought to identify B- and T-cell subpopulations associated with influenza vaccine immunogenicity in this population. We used mass cytometry to phenotype peripheral blood mononuclear cells collected from pediatric HCT recipients enrolled in a multicenter influenza vaccine trial comparing high- and standard-dose formulations over 3 influenza seasons (2016-2019). We fit linear regression models to estimate relationships between immune cell subpopulation numbers before vaccination and prevaccination to postvaccination geometric mean fold rises in antigen-specific (A/H3N2, A/H1N1, and …


Structural Variants Involving Mllt10 Fusion Are Associated With Adverse Outcomes In Pediatric Acute Myeloid Leukemia., Oussama Abla, Rhonda E. Ries, Tim Triche, Robert B. Gerbing, Betsy Hirsch, Susana Raimondi, Todd Cooper, Jason E. Farrar, Nathaniel Buteyn, Lauren M. Harmon, Hong Wen, Aniruddha J. Deshpande, E Anders Kolb, Alan S. Gamis, Richard Aplenc, Todd Alonzo, Soheil Meshinchi Apr 2024

Structural Variants Involving Mllt10 Fusion Are Associated With Adverse Outcomes In Pediatric Acute Myeloid Leukemia., Oussama Abla, Rhonda E. Ries, Tim Triche, Robert B. Gerbing, Betsy Hirsch, Susana Raimondi, Todd Cooper, Jason E. Farrar, Nathaniel Buteyn, Lauren M. Harmon, Hong Wen, Aniruddha J. Deshpande, E Anders Kolb, Alan S. Gamis, Richard Aplenc, Todd Alonzo, Soheil Meshinchi

Manuscripts, Articles, Book Chapters and Other Papers

MLLT10 gene rearrangements with KMT2A occur in pediatric acute myeloid leukemia (AML) and confer poor prognosis, but the prognostic impact of MLLT10 in partnership with other genes is unknown. We conducted a retrospective study with 2080 children and young adults with AML registered on the Children's Oncology Group AAML0531 (NCT00372593) and AAML1031 trials (NCT01371981). Transcriptome profiling and/or karyotyping were performed to identify leukemia-associated fusions associated with prognosis. Collectively, 127 patients (6.1%) were identified with MLLT10 fusions: 104 (81.9%) with KMT2A::MLLT10, 13 (10.2%) with PICALM::MLLT10, and 10 (7.9%) X::MLLT10: (2 each of DDX3X and TEC), with 6 partners (DDX3Y, CEP164, SCN2B, …


Antiviral Cellular Therapy For Enhancing T-Cell Reconstitution Before Or After Hematopoietic Stem Cell Transplantation (Aces): A Two-Arm, Open Label Phase Ii Interventional Trial Of Pediatric Patients With Risk Factor Assessment., Michael D. Keller, Patrick J. Hanley, Yueh-Yun Chi, Paibel Aguayo-Hiraldo, Christopher C. Dvorak, Michael R. Verneris, Donald B. Kohn, Sung-Yun Pai, Blachy J. Dávila Saldaña, Benjamin Hanisch, Troy C. Quigg, Roberta H. Adams, Ann Dahlberg, Shanmuganathan Chandrakasan, Hasibul Hasan, Jemily Malvar, Mariah A. Jensen-Wachspress, Christopher A. Lazarski, Gelina Sani, John M. Idso, Haili Lang, Pamela Chansky, Chase D. Mccann, Jay Tanna, Allistair A. Abraham, Jennifer L. Webb, Abeer Shibli, Amy K. Keating, Prakash Satwani, Pawel Muranski, Erin Hall, Michael J. Eckrich, Evan Shereck, Holly Miller, Ewelina Mamcarz, Rajni Agarwal, Satiro N. De Oliveira, Mark T. Vander Lugt, Christen L. Ebens, Victor M. Aquino, Jeffrey J. Bednarski, Julia Chu, Suhag Parikh, Jennifer Whangbo, Michail Lionakis, Elias T. Zambidis, Elizabeth Gourdine, Catherine M. Bollard, Michael A. Pulsipher Apr 2024

Antiviral Cellular Therapy For Enhancing T-Cell Reconstitution Before Or After Hematopoietic Stem Cell Transplantation (Aces): A Two-Arm, Open Label Phase Ii Interventional Trial Of Pediatric Patients With Risk Factor Assessment., Michael D. Keller, Patrick J. Hanley, Yueh-Yun Chi, Paibel Aguayo-Hiraldo, Christopher C. Dvorak, Michael R. Verneris, Donald B. Kohn, Sung-Yun Pai, Blachy J. Dávila Saldaña, Benjamin Hanisch, Troy C. Quigg, Roberta H. Adams, Ann Dahlberg, Shanmuganathan Chandrakasan, Hasibul Hasan, Jemily Malvar, Mariah A. Jensen-Wachspress, Christopher A. Lazarski, Gelina Sani, John M. Idso, Haili Lang, Pamela Chansky, Chase D. Mccann, Jay Tanna, Allistair A. Abraham, Jennifer L. Webb, Abeer Shibli, Amy K. Keating, Prakash Satwani, Pawel Muranski, Erin Hall, Michael J. Eckrich, Evan Shereck, Holly Miller, Ewelina Mamcarz, Rajni Agarwal, Satiro N. De Oliveira, Mark T. Vander Lugt, Christen L. Ebens, Victor M. Aquino, Jeffrey J. Bednarski, Julia Chu, Suhag Parikh, Jennifer Whangbo, Michail Lionakis, Elias T. Zambidis, Elizabeth Gourdine, Catherine M. Bollard, Michael A. Pulsipher

Manuscripts, Articles, Book Chapters and Other Papers

Viral infections remain a major risk in immunocompromised pediatric patients, and virus-specific T cell (VST) therapy has been successful for treatment of refractory viral infections in prior studies. We performed a phase II multicenter study (NCT03475212) for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell transplant with refractory viral infections using partially-HLA matched VSTs targeting cytomegalovirus, Epstein-Barr virus, or adenovirus. Primary endpoints were feasibility, safety, and clinical responses (>1 log reduction in viremia at 28 days). Secondary endpoints were reconstitution of antiviral immunity and persistence of the infused VSTs. Suitable VST …


Durability Of Original Monovalent Mrna Vaccine Effectiveness Against Covid-19 Omicron-Associated Hospitalization In Children And Adolescents - United States, 2021-2023., Laura D. Zambrano, Margaret M. Newhams, Regina M. Simeone, Amanda B. Payne, Michael Wu, Amber O. Orzel-Lockwood, Natasha B. Halasa, Jemima M. Calixte, Pia S. Pannaraj, Kanokporn Mongkolrattanothai, Julie A Boom, Leila C Sahni, Satoshi Kamidani, Kathleen Chiotos, Melissa A Cameron, Aline B Maddux, Katherine Irby, Jennifer E. Schuster, Elizabeth H. Mack, Austin Biggs, Bria M. Coates, Kelly N. Michelson, Katherine E. Bline, Ryan A. Nofziger, Hillary Crandall, Charlotte V. Hobbs, Shira J. Gertz, Sabrina M. Heidemann, Tamara T. Bradford, Tracie C. Walker, Stephanie P. Schwartz, Mary Allen Staat, Samina S. Bhumbra, Janet R. Hume, Michele Kong, Melissa S. Stockwell, Thomas J. Connors, Melissa L. Cullimore, Heidi R. Flori, Emily R. Levy, Natalie Z. Cvijanovich, Matt S. Zinter, Mia Maamari, Cindy Bowens, Danielle M. Zerr, Judith A. Guzman-Cottrill, Ivan Gonzalez, Angela P. Campbell, Adrienne G. Randolph, Overcoming Covid-19 Investigators Apr 2024

Durability Of Original Monovalent Mrna Vaccine Effectiveness Against Covid-19 Omicron-Associated Hospitalization In Children And Adolescents - United States, 2021-2023., Laura D. Zambrano, Margaret M. Newhams, Regina M. Simeone, Amanda B. Payne, Michael Wu, Amber O. Orzel-Lockwood, Natasha B. Halasa, Jemima M. Calixte, Pia S. Pannaraj, Kanokporn Mongkolrattanothai, Julie A Boom, Leila C Sahni, Satoshi Kamidani, Kathleen Chiotos, Melissa A Cameron, Aline B Maddux, Katherine Irby, Jennifer E. Schuster, Elizabeth H. Mack, Austin Biggs, Bria M. Coates, Kelly N. Michelson, Katherine E. Bline, Ryan A. Nofziger, Hillary Crandall, Charlotte V. Hobbs, Shira J. Gertz, Sabrina M. Heidemann, Tamara T. Bradford, Tracie C. Walker, Stephanie P. Schwartz, Mary Allen Staat, Samina S. Bhumbra, Janet R. Hume, Michele Kong, Melissa S. Stockwell, Thomas J. Connors, Melissa L. Cullimore, Heidi R. Flori, Emily R. Levy, Natalie Z. Cvijanovich, Matt S. Zinter, Mia Maamari, Cindy Bowens, Danielle M. Zerr, Judith A. Guzman-Cottrill, Ivan Gonzalez, Angela P. Campbell, Adrienne G. Randolph, Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric COVID-19 vaccination is effective in preventing COVID-19-related hospitalization, but duration of protection of the original monovalent vaccine during SARS-CoV-2 Omicron predominance merits evaluation, particularly given low coverage with updated COVID-19 vaccines. During December 19, 2021-October 29, 2023, the Overcoming COVID-19 Network evaluated vaccine effectiveness (VE) of ≥2 original monovalent COVID-19 mRNA vaccine doses against COVID-19-related hospitalization and critical illness among U.S. children and adolescents aged 5-18 years, using a case-control design. Too few children and adolescents received bivalent or updated monovalent vaccines to separately evaluate their effectiveness. Most case-patients (persons with a positive SARS-CoV-2 test result) were unvaccinated, despite …


Chromatin Profiles Are Prognostic Of Clinical Response To Bortezomib-Containing Chemotherapy In Pediatric Acute Myeloid Leukemia: Results From The Cog Aaml1031 Trial., Anneke D. Van Dijk, Fieke W. Hoff, Yihua Qiu, Stefan E. Hubner, Robin L. Go, Vivian R. Ruvolo, Amanda R. Leonti, Robert B. Gerbing, Alan S. Gamis, Richard Aplenc, Edward A. Kolb, Todd A. Alonzo, Soheil Meshinchi, Eveline S J M De Bont, Terzah M. Horton, Steven M. Kornblau Apr 2024

Chromatin Profiles Are Prognostic Of Clinical Response To Bortezomib-Containing Chemotherapy In Pediatric Acute Myeloid Leukemia: Results From The Cog Aaml1031 Trial., Anneke D. Van Dijk, Fieke W. Hoff, Yihua Qiu, Stefan E. Hubner, Robin L. Go, Vivian R. Ruvolo, Amanda R. Leonti, Robert B. Gerbing, Alan S. Gamis, Richard Aplenc, Edward A. Kolb, Todd A. Alonzo, Soheil Meshinchi, Eveline S J M De Bont, Terzah M. Horton, Steven M. Kornblau

Manuscripts, Articles, Book Chapters and Other Papers

The addition of the proteasome inhibitor bortezomib to standard chemotherapy did not improve survival in pediatric acute myeloid leukemia (AML) when all patients were analyzed as a group in the Children's Oncology Group phase 3 trial AAML1031 (NCT01371981). Proteasome inhibition influences the chromatin landscape and proteostasis, and we hypothesized that baseline proteomic analysis of histone- and chromatin-modifying enzymes (HMEs) would identify AML subgroups that benefitted from bortezomib addition. A proteomic profile of 483 patients treated with AAML1031 chemotherapy was generated using a reverse-phase protein array. A relatively high expression of 16 HME was associated with lower EFS and higher 3-year …


Something's Gnawing At Me, Catharine Kral, Brian Harvey Apr 2024

Something's Gnawing At Me, Catharine Kral, Brian Harvey

Posters

17-year-old female presented to Sports Medicine clinic with a chief complaint of bilateral hip pain and difficulty walking who was found to have bilateral ovarian teratoma.


Single Center Retrospective Comparison Of Bivalirudin And Heparin For Therapeutic Anticoagulation In Pediatric Patients, Clayton Habiger, Shannon L. Carpenter Apr 2024

Single Center Retrospective Comparison Of Bivalirudin And Heparin For Therapeutic Anticoagulation In Pediatric Patients, Clayton Habiger, Shannon L. Carpenter

Posters

Background: The pharmacological choice for continuous anticoagulation therapy in pediatric patients has classically been unfractionated heparin. However, with heparin there are risks of treatment resistance in pediatrics given differences in developmental hemostasis, and contemporary medications such as bivalirudin offer a valuable alternative. Bivalirudin binds to thrombin and has shown encouraging results compared to heparin in pediatric patients receiving ECMO, but no comparison has been made in patients receiving therapeutic anticoagulation. Three years ago, Children’s Mercy Pediatric Hematology department encouraged bivalirudin use over heparin use for therapeutic anticoagulation requiring a continuous infusion. Objectives: Compare clinical and laboratory outcomes in patients receiving …


Multicenter Study Of Long-Term Outcomes And Quality Of Life In Phace Syndrome After Age 10., Mitchell Braun, Ilona J. Frieden, Dawn H. Siegel, Elizabeth George, Christopher P. Hess, Christine K. Fox, Sarah L. Chamlin, Beth A. Drolet, Denise Metry, Elena Pope, Julie Powell, Kristen Holland, Caden Ulschmid, Marilyn G. Liang, Kelly K. Barry, Tina Ho, Chantal Cotter, Eulalia Baselga, David Bosquez, Surabhi Neerendranath Jain, Jordan K. Bui, Irene Lara-Corrales, Tracy Funk, Alison Small, Wenelia Baghoomian, Albert C. Yan, James R. Treat, Griffin Stockton Hogrogian, Charles Huang, Anita Haggstrom, Mary List, Catherine C. Mccuaig, Victoria Barrio, Anthony J. Mancini, Leslie P. Lawley, Kerrie Grunnet-Satcher, Kimberly A. Horii, Brandon D. Newell, Amy J. Nopper, Maria C. Garzon, Margaret E. Scollan, Erin F. Mathes Apr 2024

Multicenter Study Of Long-Term Outcomes And Quality Of Life In Phace Syndrome After Age 10., Mitchell Braun, Ilona J. Frieden, Dawn H. Siegel, Elizabeth George, Christopher P. Hess, Christine K. Fox, Sarah L. Chamlin, Beth A. Drolet, Denise Metry, Elena Pope, Julie Powell, Kristen Holland, Caden Ulschmid, Marilyn G. Liang, Kelly K. Barry, Tina Ho, Chantal Cotter, Eulalia Baselga, David Bosquez, Surabhi Neerendranath Jain, Jordan K. Bui, Irene Lara-Corrales, Tracy Funk, Alison Small, Wenelia Baghoomian, Albert C. Yan, James R. Treat, Griffin Stockton Hogrogian, Charles Huang, Anita Haggstrom, Mary List, Catherine C. Mccuaig, Victoria Barrio, Anthony J. Mancini, Leslie P. Lawley, Kerrie Grunnet-Satcher, Kimberly A. Horii, Brandon D. Newell, Amy J. Nopper, Maria C. Garzon, Margaret E. Scollan, Erin F. Mathes

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To characterize long-term outcomes of PHACE syndrome.

STUDY DESIGN: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics. Data not available in charts were collected from patients directly. Likert scales were used to assess the impact of specific findings. Patient-Reported Outcomes Measurement Information System (PROMIS) scales were used to assess quality of life domains.

RESULTS: A total of 104/153 (68%) individuals contacted participated in the study at a median of 14 years of age (range 10-77 years). There were infantile hemangioma …


Increasing Palliative Care Team Involvement In Pediatric Hematopoietic Stem Cell Transplant Patients, Meagan Vacek, Lisa Tarbell, Melissa List, Erin Hall, Jennifer Linebarger, Kelstan Ellis, Gina Jones, Joel Thompson Apr 2024

Increasing Palliative Care Team Involvement In Pediatric Hematopoietic Stem Cell Transplant Patients, Meagan Vacek, Lisa Tarbell, Melissa List, Erin Hall, Jennifer Linebarger, Kelstan Ellis, Gina Jones, Joel Thompson

Posters

Background: Palliative care facilitates communication, helps with physical and psychological symptom management, and assists in goals of care and advance care planning discussions.1 Multiple organizations, including the American Academy of Pediatrics and American Society of Clinical Oncology encourage palliative care engagement and their involvement with hematopoietic stem cell transplant (HSCT) patients has been shown to be beneficial as HSCT is associated with a high degree of morbidity and possible mortality.2-3 Objective: To increase the number of PaCT consults for patients receiving HSCT for our targeted diagnoses from 48% to 75% by December 2023. Methods: Chart review was conducted for patients …


Early Cholestasis And Vitamin K Deficiency Secondary To Biliary Atresia Presenting As Coagulopathy And Thymic Hemorrhage, Abbey Elsbernd, Lauren Amos Md Apr 2024

Early Cholestasis And Vitamin K Deficiency Secondary To Biliary Atresia Presenting As Coagulopathy And Thymic Hemorrhage, Abbey Elsbernd, Lauren Amos Md

Posters

Background Bleeding and progressive coagulopathy are established symptoms of early cholestasis, including extrahepatic biliary atresia. We present a patient with bleeding secondary to vitamin K deficiency and cholestasis-induced fat malabsorption with unusual presentation of mediastinal mass. Objective Describe a case of late-onset vitamin K deficiency and cholestasis ¬¬presenting as jaundice and coagulopathy with associated bleeding into thymic structures Design/Method Case report Results A 4-week-old term male infant was brought to the ED with fussiness and bruising. Prior to presentation, he was afebrile and followed regularly with his primary care provider for jaundice below phototherapy thresholds. Infant received vitamin K at …


Diagnosis Of Mosaic Rasopathy In A Child With Rhabdomyosarcoma, Meagan Vacek, Paige Johnson, Midhat S. Farooqi, Kristi M. Canty, Dihong Zhou, Brendan Lanpher, Wendy Allen-Rhoades, Erin M. Guest Apr 2024

Diagnosis Of Mosaic Rasopathy In A Child With Rhabdomyosarcoma, Meagan Vacek, Paige Johnson, Midhat S. Farooqi, Kristi M. Canty, Dihong Zhou, Brendan Lanpher, Wendy Allen-Rhoades, Erin M. Guest

Posters

Background: Phacomatosis pigmentokeratotica (PPK) is a subtype of epidermal nevus syndrome characterized by the co-existence of a sebaceous nevus and a speckled lentiginous nevus and described in approximately 30 cases in literature. PPK is now recognized as a mosaic RASopathy due a postzygotic mutation in the Ras-Raf-MEK-ERK pathway. RAS variants are also known to contribute to tumorigenesis, in some pediatric cancers, including rhabdomyosarcoma. Objective: Describe the presentation and evaluation of a child with pelvic rhabdomyosarcoma and evolving skin lesions found to have a rare mosaic-RASopathy. Design/Method: Case Report Results: The patient is a former 32-week premature female who presented to …


Testing For Bleeding Disorders In Child Abuse: Adherence To Aap Recommendations And Results Of Testing, Lyndsey Hultman, Angela Doswell, Henry T. Puls, Shannon L. Carpenter, Angela Bachim, Kristine Campbell, Daniel Lindberg, Joanne Wood, James Anderst Apr 2024

Testing For Bleeding Disorders In Child Abuse: Adherence To Aap Recommendations And Results Of Testing, Lyndsey Hultman, Angela Doswell, Henry T. Puls, Shannon L. Carpenter, Angela Bachim, Kristine Campbell, Daniel Lindberg, Joanne Wood, James Anderst

Presentations

Background: The American Academy of Pediatrics (AAP) recommends evaluations for bleeding disorders in children who have bruising and/or intracranial hemorrhage (ICH) concerning for abuse. Adherence to these recommendations is not known nor are the frequencies of identification of bleeding disorders or laboratory findings suggestive of a bleeding disorder. Objective: Using a multicenter research network (CAPNET), the objectives of this study were to characterize (1) the adherence to AAP recommendations for bleeding disorder testing in total and across CAPNET sites and (2) the frequency of identification of specific bleeding disorders and laboratory values concerning for bleeding disorders. Methods: We performed a …


Influence Of Novel Cyp2c-Haplotype On Proton Pump Inhibitor Pharmacokinetics In Children., Kathyrn Kyler, Andrea Gaedigk, Susan Abdel-Rahman, Vincent S. Staggs, Robin E. Pearce, Paul C. Toren, J Steven Leeder, Valentina Shakhnovich Apr 2024

Influence Of Novel Cyp2c-Haplotype On Proton Pump Inhibitor Pharmacokinetics In Children., Kathyrn Kyler, Andrea Gaedigk, Susan Abdel-Rahman, Vincent S. Staggs, Robin E. Pearce, Paul C. Toren, J Steven Leeder, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

In this brief report, we provide an analysis of the influence of a novel CYP2C haplotype (CYP2C:TG) on proton pump inhibitor (PPI) pharmacokinetics (PK) in children. The CYP2C:TG haplotype has been proposed to be associated with increased CYP2C19 activity. We sought to determine if this CYP2C:TG haplotype resulted in similar alterations in metabolism for proton pump inhibitors, which are primarily metabolized by CYP2C19. In a cohort of 41 children aged 6-21 participating in a PPI pharmacokinetic study, effects of the CYP2C:TG allele were assessed by fitting two linear regression models for each of the six PK outcomes assessed, the second …


Accuracy Of Influenza Icd-10 Diagnosis Codes In Identifying Influenza Illness In Children., James W. Antoon, Tess Stopczynski, Justin Z. Amarin, Laura S. Stewart, Julie A. Boom, Leila C. Sahni, Marian G. Michaels, John V. Williams, Janet A. Englund, Eileen J. Klein, Mary A. Staat, Elizabeth P. Schlaudecker, Rangaraj Selvarangan, Jennifer E. Schuster, Geoffrey A. Weinberg, Peter G. Szilagyi, Ariana Perez, Heidi L. Moline, Andrew J. Spieker, Carlos G. Grijalva, Samantha M. Olson, Natasha B. Halasa Apr 2024

Accuracy Of Influenza Icd-10 Diagnosis Codes In Identifying Influenza Illness In Children., James W. Antoon, Tess Stopczynski, Justin Z. Amarin, Laura S. Stewart, Julie A. Boom, Leila C. Sahni, Marian G. Michaels, John V. Williams, Janet A. Englund, Eileen J. Klein, Mary A. Staat, Elizabeth P. Schlaudecker, Rangaraj Selvarangan, Jennifer E. Schuster, Geoffrey A. Weinberg, Peter G. Szilagyi, Ariana Perez, Heidi L. Moline, Andrew J. Spieker, Carlos G. Grijalva, Samantha M. Olson, Natasha B. Halasa

Manuscripts, Articles, Book Chapters and Other Papers

IMPORTANCE: Studies of influenza in children commonly rely on coded diagnoses, yet the ability of International Classification of Diseases, Ninth Revision codes to identify influenza in the emergency department (ED) and hospital is highly variable. The accuracy of newer International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) codes to identify influenza in children is unknown.

OBJECTIVE: To determine the accuracy of ICD-10 influenza discharge diagnosis codes in the pediatric ED and inpatient settings.

DESIGN, SETTING, AND PARTICIPANTS: Children younger than 18 years presenting to the ED or inpatient settings with fever and/or respiratory symptoms at 7 …


Improving Frequency Of Peer Review Of Abnormal Genital Exam Findings In Patients Undergoing Sexual Abuse Evaluation, Lyndsey Hultman, Terra N. Frazier, Jennifer Hansen Apr 2024

Improving Frequency Of Peer Review Of Abnormal Genital Exam Findings In Patients Undergoing Sexual Abuse Evaluation, Lyndsey Hultman, Terra N. Frazier, Jennifer Hansen

Presentations

Background/Objectives: 2023 Child Advocacy Center (CAC) accreditation standards require that “all medical professionals providing services to CAC clients must demonstrate that 100% of all findings deemed abnormal or “diagnostic” of trauma from sexual abuse have undergone expert review by an advanced medical consultant”. Prior to program implementation, scheduled Case Review occurred among the authors’ institution child abuse pediatricians (CAPs), but typical practice was to review cases upon CAP request. The authors’ institution faced practice standard implementation barriers of high case volumes and collaboration with multiple different CACs. Our primary aim was to increase peer review of abnormal exams from a …


An Increased Failure Rate Of Asparaginase Desensitization With Calaspargase Pegol, Jillian Fry, Erin M. Guest, Keith J. August Apr 2024

An Increased Failure Rate Of Asparaginase Desensitization With Calaspargase Pegol, Jillian Fry, Erin M. Guest, Keith J. August

Posters

Background: In late 2022, pegaspargase (SS-PEG) became unavailable to patients younger than 22 years in the United States, leaving calaspargase pegol (SC-PEG) as the only available long-acting asparaginase formulation. SC-PEG has been compared to SS-PEG in two randomized, pediatric clinical trials and found to have similar rates of adverse events with comparable rates of event-free survival. Our institution uses a desensitization protocol for patients who experience a hypersensitivity reaction to asparaginase. Here we review our experience with desensitization since the transition to SC-PEG from SS-PEG. Objectives: To compare the rate of hypersensitivity reactions, success of desensitization protocols, and need for …


Anesthetic And Multidisciplinary Management Of A 2-Staged Liver Transplant​, Kasey Brooks, Lisa Conley, Brandon Layton Apr 2024

Anesthetic And Multidisciplinary Management Of A 2-Staged Liver Transplant​, Kasey Brooks, Lisa Conley, Brandon Layton

Posters

We present a case of a 2 staged liver transplant on a 12-month-old 8kg male with newly diagnosed mitochondrial depletion syndrome causing acute liver failure, refractory lactic acidosis, and cardiopulmonary collapse requiring ECMO. The patient’s past medical history at the time of presentation included history of prematurity at 32 weeks, developmental delay, and a brother with an undiagnosed pancreatic disorder. The patient was brought in by parents to the emergency department with a cough, minimal PO intake for 5 days, and increased fatigue who was found to be URI positive. Initial labs were concerning for anemia, elevated bilirubin, elevated AST/ALT, …


Anterior Hip Pain In Adolescent Female Wrestler, Melanie Kennedy Apr 2024

Anterior Hip Pain In Adolescent Female Wrestler, Melanie Kennedy

Posters

No abstract provided.


Ovarian Torsion, Children's Mercy Kansas City Apr 2024

Ovarian Torsion, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.


Transition Skills Lab: A Telehealth Intervention For Improving Healthcare Transition In Youth With Special Health Care Needs, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux Apr 2024

Transition Skills Lab: A Telehealth Intervention For Improving Healthcare Transition In Youth With Special Health Care Needs, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux

Posters

Introduction: Few interventions exist to provide hands-on practice of key health management tasks to youth with special health care needs (YSHCN). The Transition Skills Lab was adapted from Jerlym Porter’s work and piloted as a virtual intervention in YSHCN. Methods: Sixteen young adults participated in the virtual Transition Skills Lab, during which they practiced three key patient competencies pertaining to health care tasks: reading an insurance card, reading a pill bottle label, and scheduling an initial visit with an adult doctor. Participants were then asked to rate their confidence on skills taught within the intervention on a four-point scale from …


Telehealth Implementation Of A Young Adult Ibd Clinic: Uptake, Benefits To Patient Care, And Challenges, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux Apr 2024

Telehealth Implementation Of A Young Adult Ibd Clinic: Uptake, Benefits To Patient Care, And Challenges, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux

Posters

Introduction: Since the COVID-19 pandemic, pediatric psychologists have been at the forefront of telemedicine expansion to ensure access for patients and families to needed mental health services. This has required adaptations across the care continuum – from scheduling to service delivery to documentation. This poster aims to summarize the telehealth (TH) application of a Young Adult IBD Clinic (YAC-IBD) that provides transition preparation to youth with Inflammatory Bowel Disease (IBD). Methods: In April 2020, the YAC-IBD was converted from in-person visits to a virtual visit format. Since then, 119 youth ages 17-21 (inclusive) have been seen in this clinic. This …


Antimicrobial Duration Stewardship Project: A Qi Approach, Joshua Saucedo, Rana El Feghaly, Amanda Nedved, Leslie Hueschen, Marsha Dannenburg, Tanis Stewart, Patty Philips, Jennifer Floyd Apr 2024

Antimicrobial Duration Stewardship Project: A Qi Approach, Joshua Saucedo, Rana El Feghaly, Amanda Nedved, Leslie Hueschen, Marsha Dannenburg, Tanis Stewart, Patty Philips, Jennifer Floyd

Presentations

A QI project aimed at increasing the percentage of children discharged home from the emergency department with a diagnosis of community acquired pneumonia (CAP) or acute bacterial rhinosinusitis (ABRS) treated with an optimal antibiotic duration (5 days or less for CAP, 7 days or less for ABRS) from 22% to 70% by July 2025. Additionally, assess for differences in care based on demographic and socioeconomic factors such as the area deprivation index. National guidelines and CMH clinical pathways recommend short antibiotic durations (5-7 days) for most infections (5 days for community acquired pneumonia, 5-7 days for acute bacterial rhinosinusitis). At …


Investigating The Use Of Finerenone In Children With Chronic Kidney Disease And Proteinuria: Design Of The Fiona And Open-Label Extension Studies., Franz Schaefer, Giovanni Montini, Hee Gyung Kang, Johan Vande Walle, Joshua Zaritsky, Michiel F. Schreuder, Mieczyslaw Litwin, Andrea Scalise, Helen Scott, James Potts, Pablo Iveli, Stefanie Breitenstein, Bradley A. Warady Mar 2024

Investigating The Use Of Finerenone In Children With Chronic Kidney Disease And Proteinuria: Design Of The Fiona And Open-Label Extension Studies., Franz Schaefer, Giovanni Montini, Hee Gyung Kang, Johan Vande Walle, Joshua Zaritsky, Michiel F. Schreuder, Mieczyslaw Litwin, Andrea Scalise, Helen Scott, James Potts, Pablo Iveli, Stefanie Breitenstein, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Proteinuria is a modifiable risk factor for chronic kidney disease (CKD) progression in children. Finerenone, a selective, non-steroidal, mineralocorticoid receptor antagonist (MRA) has been approved to treat adults with CKD associated with type 2 diabetes mellitus (T2DM) following results from the phase III clinical trials FIDELIO-DKD (NCT02540993) and FIGARO-DKD (NCT02545049). In a pre-specified pooled analysis of both studies (N = 13,026), finerenone was shown to have an acceptable safety profile and was efficacious in decreasing the risk of adverse kidney and cardiovascular outcomes and of proteinuria.

OBJECTIVE: FIONA and the associated open-label extension (OLE) study aim to demonstrate that …


Pediatric Beta Blocker Therapy: A Comprehensive Review Of Development And Genetic Variation To Guide Precision-Based Therapy In Children, Adolescents, And Young Adults., Mollie Walton, Jonathan B. Wagner Mar 2024

Pediatric Beta Blocker Therapy: A Comprehensive Review Of Development And Genetic Variation To Guide Precision-Based Therapy In Children, Adolescents, And Young Adults., Mollie Walton, Jonathan B. Wagner

Manuscripts, Articles, Book Chapters and Other Papers

Beta adrenergic receptor antagonists, known as beta blockers, are one of the most prescribed medications in both pediatric and adult cardiology. Unfortunately, most of these agents utilized in the pediatric clinical setting are prescribed off-label. Despite regulatory efforts aimed at increasing pediatric drug labeling, a majority of pediatric cardiovascular drug agents continue to lack pediatric-specific data to inform precision dosing for children, adolescents, and young adults. Adding to this complexity is the contribution of development (ontogeny) and genetic variation towards the variability in drug disposition and response. In the absence of current prospective trials, the purpose of this comprehensive review …


Infant Appendicitis: A Case Presentation Of Appendicitis In A Nine-Month-Old Infant With Respiratory Syncytial Virus (Rsv) And Otitis Media And Review Of Literature., Leslie Hueschen, April Mcneill-Johnson Mar 2024

Infant Appendicitis: A Case Presentation Of Appendicitis In A Nine-Month-Old Infant With Respiratory Syncytial Virus (Rsv) And Otitis Media And Review Of Literature., Leslie Hueschen, April Mcneill-Johnson

Manuscripts, Articles, Book Chapters and Other Papers

Appendicitis in children < 5 years old is uncommon and even less common in children < 1 year old. Symptoms of appendicitis can be non-specific and mimic other common pediatric diseases, causing delays in diagnosis. Without a timely diagnosis, young children with appendicitis are at risk of developing worsening disease, intra-abdominal abscess, perforation, and bacteremia. We submit a case of a nine-month-old male infant presenting with fever, vomiting, and irritability seen the day prior and treated for otitis media, who was ultimately diagnosed with appendicitis with concomitant viral infection (respiratory syncytial virus and human rhinovirus/enterovirus) and treated with intravenous antibiotics and laparoscopic appendectomy. This case illustrates how easy it is to misdiagnose infant appendicitis due to factors including normal developmental speech barriers, nonspecific presentations, and overlap of symptoms with many other common childhood illnesses, along with a challenging abdominal examination. Delay in diagnosis leads to increased rates of perforation and complications. Providers should trust abnormal physical examination findings, especially abdominal guarding against palpation, and keep a wide differential diagnosis in order to diagnose appendicitis in young children.