Medical Specialties Commons^™

Biomarkers In Patients With Mucopolysaccharidosis Type Ii And Iv., Honoka Fujitsuka, Kazuki Sawamoto, Hira Peracha, Robert W. Mason, William Mackenzie, Hironori Kobayashi, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji Orii, Tadao Orii, Toshiyuki Fukao, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Glycosaminoglycans (GAGs), dermatan sulfate (DS), heparan sulfate (HS), and keratan sulfate (KS), are the primary biomarkers in patients with mucopolysaccharidoses (MPS); however, little is known about other biomarkers. To explore potential biomarkers and their correlation with GAGs, blood samples were collected from 46 MPS II patients, 34 MPS IVA patients, and 5 MPS IVB patients. We evaluated the levels of 8 pro-inflammatory factors (EGF, IL-1β, IL-6, MIP-1α, TNF-α, MMP-1, MMP-2, and MMP-9), collagen type II, and DS, HS (HS0S, HSNS), and KS (mono-sulfated, di-sulfated) in blood. Eight biomarkers measured were significantly elevated in untreated MPS II patients, compared with those …

Whole Genome Metagenomic Analysis Of The Gut Microbiome Of Differently Fed Infants Identifies Differences In Microbial Composition And Functional Genes, Including An Absent Crispr/Cas9 Gene In The Formula-Fed Cohort, Matthew D. Di Guglielmo, Karl Franke, Courtney Cox, Erin L. Crowgey

Department of Pediatrics Faculty Papers

Background: Advancements in sequencing capabilities have enhanced the study of the human microbiome. There are limited studies focused on the gastro-intestinal (gut) microbiome of infants, particularly the impact of diet between breast-fed (BF) versus formula-fed (FF). It is unclear what effect, if any, early feeding has on short- term or long-term composition and function of the gut microbiome.

Results: Using a shotgun metagenomics approach, differences in the gut microbiome between BF (n = 10) and FF (n = 5) infants were detected. A Jaccard distance principle coordinate analysis was able to cluster BF versus FF infants based on the presence …

Nurturing Environments And Nutrient-Rich Diets May Improve Cognitive Development: Analysis Of Cognitive Trajectories From Six To Sixty Months From The Mal-Ed Study (Or10-01-19), Benjamin Mccormick, Laura Caulfield, Stephanie Richard, Laura Pendergast, Laura Murray-Kolb, Mal-Ed Network Investigators

Department of Paediatrics and Child Health

Objectives: To identify clusters of cognitive developmental trajectories and associated differentiating factors of children aged 6 to 60 months old in 5 low to middle-income sites.
Methods: We followed 835 children and assessed anthropometry at enrolment (≤ 17 d old); bi-weekly illness data (0–24 and 60 mo); non-diarrheal and diarrheal stools (0–24 mo) analyzed for a panel of enteropathogens; quantitative complementary food intakes (9–24 and 60 mo); micronutrient status (Fe, Zn, Vit A; 7, 15, and 24 mo); quality of the child's home environment (6, 24, and 60 mo) and maternal reasoning ability and depressive symptoms via questionnaire. Child cognitive …

A Neonate With Upper Gi Bleeding, Suneeta Khemani, Huma Ghulam Qadir, Mumtaz Malik, Faiqa Hassan

Department of Paediatrics and Child Health

Bleeding in the newborn is often a serious problem because of cardiovascular effects associated with a loss of blood and/or the damaging effects of bleeding on neonatal tissues, especially the brain. Gastric polyps are described as abnormal lesions that originate in the gastric epithelium or submucosa and protrude into the stomach lumen. Clinical presentation of gastric polyps in children varies widely, from incidental endoscopic finding to massive gastrointestinal bleeding. However, acute gastrointestinal bleeding in association with hyperplastic gastric polyps has been reported very rarely. We report a case of unusual upper gastrointestinal bleeding in newborn baby on 2nd day of …

The Man With 2 Hearts: 25 Years From Heterotopic To Orthotopic Heart Transplantation, Elizabeth L Godfrey, Michael L Kueht, Abbas Rana, O H Frazier

The Texas Heart Institute Journal

Substantial technological advances in mechanical circulatory support have caused a shift in the management of end-stage heart failure. From the 1970s through the 1990s, heterotopic heart transplantation was routinely performed in patients in whom orthotopic transplantation was likely to fail. Heterotopic heart transplantation is now performed less often because modern mechanical circulatory assist devices are routinely used as bridges to orthotopic transplantation; regardless, the operation has helped numerous patients who would not otherwise have received adequate allografts. We describe the case of a man with idiopathic nonischemic cardiomyopathy who, at age 17, was given an ABO- and size-matched heterotopic allograft …

Use Of Best Practice Alerts To Improve Adherence To Evidence-Based Screening In Pediatric Diabetes Care, Daniel Desalvo, Sara K. Bartz, Blair Mockler, Rona Y. Sonabend

Journal of Nursing & Interprofessional Leadership in Quality & Safety

Background: Youth with type 1 diabetes (T1D) are at increased risk for comorbid autoimmune conditions and long-term complications. To help with early identification of these complications, the American Diabetes Association (ADA) has published evidence-based screening guidelines. The aim of our quality improvement intervention was to improve and sustain adherence to the ADA recommended screening guidelines to >90% for youth with T1D in the Texas Children’s Hospital (TCH) Diabetes Center by utilizing best practice alerts (BPA) within the electronic medical record (EMR).

Methods: In accordance with the ADA Standards of Care screening guidelines for youth with T1D, we analyzed the database …

Abcb1 Snp Predicts Outcome In Patients With Acute Myeloid Leukemia Treated With Gemtuzumab Ozogamicin: A Report From Children's Oncology Group Aaml0531 Trial., Roya Rafiee, Lata Chauhan, Todd A. Alonzo, Yi-Cheng Wang, Ahlam Elmasry, Michael R. Loken, Jessica Pollard, Richard Aplenc, Susana Raimondi, Betsy A. Hirsch, Irwin D. Bernstein, A S. Gamis, Soheil Meshinchi, Jatinder K. Lamba

Manuscripts, Articles, Book Chapters and Other Papers

Gemtuzumab-ozogamicin (GO), a humanized-anti-CD33 antibody linked with the toxin-calicheamicin-γ is a reemerging and promising drug for AML. Calicheamicin a key element of GO, induces DNA-damage and cell-death once the linked CD33-antibody facilitates its uptake. Calicheamicin efflux by the drug-transporter PgP-1 have been implicated in GO response thus in this study, we evaluated impact of ABCB1-SNPs on GO response. Genomic-DNA samples from 942 patients randomized to receive standard therapy with or without addition of GO (COG-AAML0531) were genotyped for ABCB1-SNPs. Our most interesting results show that for rs1045642, patients with minor-T-allele (CT/TT) had better outcome as compared to patients with CC …

The Role Of Outdoor Tree Pollen Levels On Healthcare Visits For Respiratory Infections In Infants And Toddlers: A Case Cross-Over Analysis, Adam J. Van Mason

Research Days

No abstract provided.

A Retrospective Review Of Project Instruct: Improving Sexual And Reproductive Health Amongst At-Risk Teens, April Mcneill Md

Research Days

No abstract provided.

Family Entropy: Understanding The Organization Of The Home Environment And Impact On Health Behaviors And Weight In School-Age Children, Carolyn Bates

Research Days

No abstract provided.

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

Research Days

No abstract provided.

Measuring Clinical Weight Loss In Young Children With Severe Obesity: Comparison Of Outcomes Using Zbmi, Modified Zbmi, And Percent Of 95th Percentile, Carolyn Bates

Research Days

No abstract provided.

Heavy Menstrual Bleeding In Adolescent Females With Platelet Function Disorders, Lauren Amos Md

Research Days

No abstract provided.

Pediatric Ppi Use And Fractures, Nathan R. Fleishman Md, Thomas M. Attard, Troy E. Richardson

Research Days

No abstract provided.

Clinical Utility Of Exon Deletion/Duplication Microarray Testing - A Children’S Mercy Kansas City Two-Year Experience, Binu Porath

Research Days

No abstract provided.

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

Research Days

No abstract provided.

Attitudes Of Emergency Medical Service (Ems) Providers Towards Pediatric Death And Outside-Of-Hospital Do Not Resuscitate (Dnr) Orders, Clay Yaklin

Research Days

No abstract provided.

Adhd Clinic Outcomes: Assessing Dose Response Over Time, Caroline Pittard

Research Days

No abstract provided.

Prevalence Of Sexual Minorities Among Adolescents Evaluated For Polycystic Ovary Syndrome, Cintya Schweisberger

Research Days

No abstract provided.

Changes In Pt, Aptt And Platelet Counts After Ich Resulting From Accidental Trauma, Sara Kilbride

Research Days

No abstract provided.

Racial/Ethnic Demographics Of Participants In Clinical Trials Of Biologics Used For Asthma, Susamita Kesh

Research Days

No abstract provided.

Parental Observation Of Environmental Exposures In The Home Of Children With Asthma, Hannah N. Neuhaus

Research Days

No abstract provided.

Initial Evaluation Of Adolescent Females Hospitalized With Heavy Menstrual Bleeding, Lauren Amos Md

Research Days

No abstract provided.

Guided Mentorship: Enhancing Pediatric Resident’S Skills In Cardiopulmonary Resuscitation, Alyssa Stoner

Research Days

No abstract provided.

Characterization Of Low Resource Utilization Observation Status Hospitalizations Within Children's Hospitals, David C. Synhorst

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

Presentations

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

A High-Throughput Screen Indicates Gemcitabine And Jak Inhibitors May Be Useful For Treating Pediatric Aml, Christina D. Drenberg, Anang Shelat, Jinjun Dang, Anitria Cotton, Shelley J. Orwick, Mengyu Li, Jae Yoon Jeon, Qiang Fu, Daelynn R. Buelow, Marissa Pioso, Shuiying Hu, Hiroto Inaba, Raul C. Ribeiro, Jeffrey E. Rubnitz, Tanja A. Gruber, R. Kiplin Guy, Sharyn D. Baker

Pharmaceutical Sciences Faculty Publications

Improvement in survival has been achieved for children and adolescents with AML but is largely attributed to enhanced supportive care as opposed to the development of better treatment regimens. High risk subtypes continue to have poor outcomes with event free survival rates < 40% despite the use of high intensity chemotherapy in combination with hematopoietic stem cell transplant. Here we combine high-throughput screening, intracellular accumulation assays, and in vivo efficacy studies to identify therapeutic strategies for pediatric AML. We report therapeutics not currently used to treat AML, gemcitabine and cabazitaxel, have broad anti-leukemic activity across subtypes and are more effective relative to the AML standard of care, cytarabine, both in vitro and in vivo. JAK inhibitors are selective for acute megakaryoblastic leukemia and significantly prolong survival in multiple preclinical models. Our approach provides advances in the development of treatment strategies for pediatric AML.

Challenges Of Training And Delivery Of Pediatric Surgical Services In Developing Economies: A Perspective From Pakistan, Amir Humza Sohail, Muhammad Hassaan Arif Maan, Mohammed Sachal, Muhammad Soban

Medical College Documents

Background: As the pediatric population requiring health services rises globally, developing countries are struggling to cater to the growing burden of non-communicable diseases - particularly those requiring specialized surgical care.
Main body: Despite the literature supporting specialized pediatric surgical care, the developing world is far from meeting the American Pediatric Surgical Association (APSA) Manpower taskforce recommendation of at least 1 qualified pediatric surgeon per 100,000 patients (0-15 years-old). In Pakistan, there is an unmet surgical need in the pediatric population due to a multitude of short shortcomings, notably in quality and quantity of the training programs on offer, and urgent …

The Effect Of Antihypertensive Medications Dosing On Blood Pressure Control And Left Ventricular Hypertrophy In Children With Chronic Kidney Disease, Benjamin A. Matta

Research Days

No abstract provided.

Relationship Between Hemolytic Index Early In Life And Development Of Abnormal Transcranial Doppler Velocities In Pediatric Patients With Sickle Cell Anemia, Derrick L. Goubeaux

Research Days

No abstract provided.