Medical Specialties Commons^™

Elective Tracheostomy In Critically Ill Children: A 10-Year Single-Center Experience From A Lower-Middle Income Country, Sidra Ishaque, Anwarul Haque, Saqib Hamid Qazi, Hamdan Mallick, Saad Nasir

Department of Paediatrics and Child Health

Objective: Tracheostomy is a commonly performed procedure amongst critically ill patients, especially in cases of prolonged mechanical ventilation (PMV). This study aimed to describe the indications, clinical characteristics, and outcomes of elective pediatric tracheostomies in critically ill children at our center.
Methods: A retrospective review of medical records of children who underwent elective tracheostomies in our pediatric intensive care unit (PICU) was conducted from January 2009 to June 2018. Data were extracted based on demographics, indications of tracheostomy, and patient outcomes. Results were reported as mean with standard deviation and as frequencies with percentage.
Results: Of the 3,200 patients admitted …

Post-Op Day 1 Fever Is Never Atelectasis, Elizabeth Boudiab, Paras Khandhar

Articles

Postop day 1 fever is never atelectasis. It has been blamed for decades while even the latest editions of surgical textbooks disseminate this myth1. Whilst both fever and atelectasis are extraordinarily common after surgery – occurring in up to 40% and 90% of patients, respectively2 this is NOT the cause on Day 1. It is time that we exonerate this myth.

Postop day 1 fever is never atelectasis, it is due to inflammation and trauma of surgery. The concept that atelectasis was causing fever was derived principally from a study showing that alveolar macrophages sampled from an atelectatic rat lung …

Polyspike Ictal-Onset Absence Seizures In A Pediatric Patient With Down Syndrome., Sherouk A. Abdelmoity, Mohammed Ilyas

Manuscripts, Articles, Book Chapters and Other Papers

Polyspike ictal-onset absence seizure has been reported in adult patients with genetic generalized epilepsy but is a novel pattern in the pediatric population. Absence seizures are usually associated with generalized spike-and-wave on EEG. However, we present the case of a 10-year-old girl with Down syndrome and developmental delays who presented with atypical absence seizure associated with an unusual electroencephalographic (EEG) pattern of polyspike ictal-onset. Recognition of this ictal pattern in the pediatric population, as previously reported in adult populations, is important as it can have therapeutic and prognostic implications.

Food Supplements To Reduce Stunting In Pakistan: A Process Evaluation Of Community Dynamics Shaping Uptake, Shehla Zaidi, Jai K. Das, Gul Nawaz Khan, Rabia Najmi Taha, Mashal Murad Shah, Sajid Bashir Soofi

Community Health Sciences

Background: There is an increasing interest in use of food supplements to prevent childhood stunting, however the evidence on the process indicators is scarce. We in this study explore the barriers to the effective implementation of food supplementation programs and the possible mitigation strategies which can guide the design of future programs.
Methods: We undertook a process evaluation of a stunting prevention food supplementation pilot program in rural Pakistan that distributed Wheat Soy Blend (WSB) to pregnant & lactating women, and Lipid-based Nutrient Supplement (LNS) and micronutrient powder (MNP) to < 5 years children. We used a mixed methods approach through a quantitative survey of 800 households and conducted 18 focused group discussion (FGDs) (with male and female caregivers), 4 FGDs (with Community Health Workers (CHWs)) and 22 key informant interviews (with district stakeholders) to evaluate the community side factors affecting uptake through five parameters: value, acceptability, receipt of supplement, usage and correct dosage.
Results: The findings show that proportionately few beneficiaries consumed the …

Multifocal Bone Pain, Recurrent Fevers And Anemia In A 15-Year-Old, Anna M. Martens, Catherine Ezzio, Amanda Goddard, Leah Mallory

Journal of Maine Medical Center

Introduction: Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disorder that primarily affects children with recurrent episodes of sterile osteomyelitis in one or more bones. Patients usually present with recurrent bone pain, though it is often a diagnosis of exclusion with most patients undergoing extensive work-up to arrive at a diagnosis.

Clinical Findings: This 15-year-old female presented with months of progressive multifocal bone pain, 22-pound weight loss and recurrent fevers. Work-up as an outpatient included a positive screening assay and IgM Western blot Panel positive for Lyme disease, treated with a 28-day course doxycycline without resolution of the symptoms, …

2020-2021 Pediatric Subspecialty Trainees, Children's Mercy Hospital

Residents and Fellows Portraits

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

Screening And Treatment Outcomes In Adults And Children With Type 1 Diabetes And Asymptomatic Celiac Disease: The Cd-Diet Study., Farid H Mahmud, Antoine B M Clarke, Kariym C Joachim, Esther Assor, Charlotte Mcdonald, Fred Saibil, Heather A Lochnan, Zubin Punthakee, Amish Parikh, Andrew Advani, Baiju R Shah, Bruce A Perkins, Caroline S Zuijdwijk, David R Mack, Dror Koltin, Emilia N De Melo, Eugene Hsieh, Geetha Mukerji, Jeremy Gilbert, Kevin Bax, Margaret L Lawson, Maria Cino, Melanie D Beaton, Navaaz A Saloojee, Olivia Lou, Patricia H Gallego, Premysl Bercik, Robyn L Houlden, Ronnie Aronson, Susan E Kirsch, William G Paterson, Margaret A Marcon

Paediatrics Publications

OBJECTIVE: To describe celiac disease (CD) screening rates and glycemic outcomes of a gluten-free diet (GFD) in patients with type 1 diabetes who are asymptomatic for CD.

RESEARCH DESIGN AND METHODS: Asymptomatic patients (8-45 years) were screened for CD. Biopsy-confirmed CD participants were randomized to GFD or gluten-containing diet (GCD) to assess changes in HbA

RESULTS: Adults had higher CD-seropositivity rates than children (6.8% [95% CI 4.9-8.2%,

CONCLUSIONS: CD is frequently observed in asymptomatic patients with type 1 diabetes, and clinical vigilance is warranted with initiation of a GFD.

Delivering Water, Sanitation And Hygiene Interventions To Women And Children In Conflict Settings: A Systematic Review, Daina Als, Sarah Meteke, Marianne Stefopulos, Michelle F. Gaffey, Mahdis Kamali, Mariella Munyuzangabo, Shailja Shah, Reena P. Jain, Amruta Radhakrishnan, Fahad J. Siddiqui, Anushka Ataullahjan, Zulfiqar Ahmed Bhutta

Woman and Child Health

Background: Access to safe water and sanitation facilities and the adoption of effective hygiene practices are fundamental to reducing maternal and child morbidity and mortality globally. In armed conflict settings, inadequate water, sanitation and hygiene (WASH) infrastructure poses major health risks for women and children. This review aimed to synthesise the existing information on WASH interventions being delivered to women and children in conflict settings in low-income and middle-income countries (LMICs) and to identify the personnel, sites and platforms being used to deliver such interventions.
Methods: We conducted a systematic search for publications indexed in four databases, and grey literature …

Caregiver Perspective On Sensory Diet And Therapeutic Listening For Children With Autism Spectrum Disorder And/Or Sensory Processing Disorder, Mikayla M. Brett, Lynette Zulquairnain, Katlyn Ribant

Pediatrics

Due to the high prevalence of Autism Spectrum Disorder (ASD) and Sensory Processing Disorder (SPD), there is a high demand for more research on effective interventions in order to improve quality of life and engagement in meaningful occupations for individuals with ASD and/or SPD. Sensory Diet (SD) and Therapeutic Listening © (TL) are interventions commonly utilized with individuals with ASD and/or SPD, however, there is limited research on their combined use. The purpose of this study is to determine the effectiveness of the combined occupational therapy (OT) interventions of SD and TL on the occupational performance of children with ASD …

Bortezomib With Standard Chemotherapy For Children With Acute Myeloid Leukemia Does Not Improve Treatment Outcomes: A Report From The Children's Oncology Group., Richard Aplenc, Soheil Meshinchi, Lillian Sung, Todd Alonzo, John Choi, Brian Fisher, Robert Gerbing, Betsy Hirsch, Terzah Horton, Samir Kahwash, John Levine, Michael Loken, Lisa Brodersen, Jessica Pollard, Susana Raimondi, Edward Anders Kolb, A S. Gamis

Manuscripts, Articles, Book Chapters and Other Papers

New therapeutic strategies are needed for pediatric acute myeloid leukemia (AML) to reduce disease recurrence and treatment-related morbidity. The Children's Oncology Group Phase III AAML1031 trial tested whether the addition of bortezomib to standard chemotherapy improves survival in pediatric patients with newly diagnosed AML. AAML1031 randomized patients younger than 30 years of age with de novo AML to standard treatment with or without bortezomib. All patients received the identical chemotherapy backbone with either four intensive chemotherapy courses or three courses followed by allogeneic hematopoietic stem cell transplantation for high-risk patients. For those randomized to the intervention arm, bortezomib 1.3 mg/m2 …

Mealtime Insulin Bolus Score Increases Prior To Clinic Visits In Youth With Type 1 Diabetes., Andrew Mcconville, Amy E. Noser, Mark A. Clements, Susana R. Patton

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Multiple studies confirm the occurrence of 'white coat adherence' (WCA), a term describing an increase in engagement with self-care tasks just prior to a scheduled clinic appointment, across cohorts with multiple chronic conditions. In youth with type 1 diabetes (T1D), research also shows an increase in self-monitoring blood glucose frequency ahead of youths' clinic visits. While studies show preliminary evidence for the occurrence of WCA in youth with T1D, no study has examined the effect of WCA and mealtime insulin dosing behaviors in youth with T1D. The frequency of mealtime insulin bolusing score (BOLUS) is an objective measure of …

Igf1r Immunohistochemistry In Ewing's Sarcoma As Predictor Of Response To Targeted Therapy., Elizabeth Gonzalez, Marilyn Bui, Atif Ahmed

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Ewing's sarcoma is an aggressive malignancy of bone and soft tissue in children and young adults. Despite advances in modern therapy, metastasis can occur and results in high mortality. The objective of this study was to identify whether the signaling transduction proteins, insulin growth factor receptor (IGF1R) and S6 kinase (S6K), can predict poor prognosis in Ewing's sarcoma.

Methods: After the Institutional Research Board approval, immunohistochemical experiments on tissue microarray slides containing 32 archived Ewing's sarcoma tumor samples were performed with antibodies against IGF1Rb and p-S6K. Immunohistochemical staining results were correlated with patients' clinical data including clinical stage and …

Igf1r Immunohistochemistry In Ewing's Sarcoma As Predictor Of Response To Targeted Therapy., Elizabeth Gonzalez, Marilyn Bui, Atif Ahmed

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Ewing's sarcoma is an aggressive malignancy of bone and soft tissue in children and young adults. Despite advances in modern therapy, metastasis can occur and results in high mortality. The objective of this study was to identify whether the signaling transduction proteins, insulin growth factor receptor (IGF1R) and S6 kinase (S6K), can predict poor prognosis in Ewing's sarcoma.

Methods: After the Institutional Research Board approval, immunohistochemical experiments on tissue microarray slides containing 32 archived Ewing's sarcoma tumor samples were performed with antibodies against IGF1Rb and p-S6K. Immunohistochemical staining results were correlated with patients' clinical data including clinical stage and …

New Mexico Children's Alliance Children's Advocacy Centers Needs Assessment, Theresa H. Cruz, Leona Woelk, Ivy Vitanzos Cervantes, Emily Guerra

Reports & Documents

No abstract provided.

Building A Contextually-Relevant Understanding Of Resilience Among African American Youth Exposed To Community Violence., Briana Woods-Jaeger, Emily Siedlik, Amber Adams, Kaitlin Piper, Paige O'Connor, Jannette Berkley-Patton

Manuscripts, Articles, Book Chapters and Other Papers

Studies consistently demonstrate that African American youth experience disproportionate levels of community violence, which is associated with negative health and well-being outcomes among these youth. The frequency and severity of community violence exposure is a unique challenge for these youth and requires tailored approaches to promote resilience after community violence exposure. However, limited research exists that operationalizes resilience after community violence based on the unique context and lived experience of African American youth. Developing a more contextually relevant understanding of resilience is critical to reducing health inequities experienced by African American youth and promoting their well-being. Five focus groups were …

Congenital Abnormalities Of The Lower Airways And Lungs, Christopher M. Oermann

Presentations

Goals and Objectives. Participants will be able to: Describe the 5 stages of fetal lung development. Summarize the prenatal identification and management of congenital abnormalities of the lower airways and lung (CALAL). Explain the classification of CALAL. Compare the clinical characteristics, pathology, and management of common CALAL. Discuss the long term prognosis of CALAL

Risk Factors For Nonaccidental Burns In Children, Dalya M Ferguson, Tayler D Parker, Vanessa E Marino, Elisa I Garcia, Seyed A Arshad, Pranali S Kamat, Caroline M Anding, Kuojen Tsao, Rebecca G Girardet, Mary T Austin

Faculty, Staff and Student Publications

BACKGROUND: The relative influences of baseline risk factors for pediatric nonaccidental burns have not been well described. We evaluated baseline characteristics of pediatric nonaccidental burn patients and their primary caretakers.

METHODS: A single-center retrospective cohort study was conducted of pediatric (age < 17) burn patients from July 1, 2013, to June 30, 2018. The primary outcome was nonaccidental burn, defined as burn secondary to abuse or neglect as determined by the inpatient child protection team or Child Protective Services. Univariate and multivariate analyses were performed.

RESULTS: Of 489 burn patients, 47 (9.6%) suffered nonaccidental burns. Nonaccidental burn patients more frequently had a history of Child Protective Services involvement (48.9% vs 9.7%, P < .001), as did their primary caretakers (59.6% vs 10.9%, P < .001). Non-Hispanic black children had higher rates of Child Protective Services referral (50.7% vs 26.7%, P < .001) and nonaccidental burn diagnosis (18.9% vs 5.6%, P < .001) than children of other races/ethnicities. On multivariate analysis, caretaker involvement with CPS (odds ratio 7.53, 95% confidence interval 3.38-16.77) and non-Hispanic black race/ethnicity (odds ratio 3.28, 95% confidence interval 1.29-8.36) were associated with nonaccidental burn.

CONCLUSION: Caretaker history of Child Protective Services involvement and non-Hispanic black race/ethnicity were associated with increased odds of pediatric nonaccidental burn. Prospective research is necessary to determine whether these represent true risk …

Efficacy Of A Novel Mitochondrial-Derived Peptide In A Porcine Model Of Myocardial Ischemia/Reperfusion Injury, Thomas E. Sharp, Zhenwei Gong, Amy Scarborough, Eric S. Goetzman, Murtuza J. Ali, Pablo Spaletra, David J. Lefer, Radhika H. Muzumdar, Traci T. Goodchild

School of Medicine Faculty Publications

With the complexities that surround myocardial ischemia/reperfusion (MI/R) injury, therapies adjunctive to reperfusion that elicit beneficial pleiotropic effects and do not overlap with standard of care are necessary. This study found that the mitochondrial-derived peptide S14G-humanin (HNG) (2 mg/kg), an analogue of humanin, reduced infarct size in a large animal model of MI/R. However, when ischemic time was increased, the infarct-sparing effects were abolished with the same dose of HNG. Thus, although the 60-min MI/R study showed that HNG cardioprotection translates beyond small animal models, further studies are needed to optimize HNG therapy for longer, more patient-relevant periods of cardiac …

Atrx Deletion In Neurons Leads To Sexually Dimorphic Dysregulation Of Mir-137 And Spatial Learning And Memory Deficits., Renee J. Tamming, Vanessa Dumeaux, Yan Jiang, Sarfraz Shafiq, Luana Langlois, Jacob Ellegood, Lily R. Qiu, Jason P. Lerch, Nathalie G. Bérubé

Paediatrics Publications

ATRX gene mutations have been identified in syndromic and non-syndromic intellectual disabilities in humans. ATRX is known to maintain genomic stability in neuroprogenitor cells, but its function in differentiated neurons and memory processes remains largely unresolved. Here, we show that the deletion of neuronal Atrx in mice leads to distinct hippocampal structural defects, fewer presynaptic vesicles, and an enlarged postsynaptic area at CA1 apical dendrite-axon junctions. We identify male-specific impairments in long-term contextual memory and in synaptic gene expression, linked to altered miR-137 levels. We show that ATRX directly binds to the miR-137 locus and that the enrichment of the …

Host Immune Response To Enterovirus And Parechovirus Systemic Infections In Children., Anjana Sasidharan, Wail M. Hassan, Christopher J. Harrison, Ferdaus Hassan, Rangaraj Selvarangan

Manuscripts, Articles, Book Chapters and Other Papers

Background: Enterovirus (EV) and parechovirus type A3 (PeV-A3) cause infections ranging from asymptomatic to life-threatening. Host immune responses in children, particularly innate responses to PeV-A3, remain largely unknown. The aim of this study was to determine aspects of the cytokine/chemokine responses to EV and PeV-A3 in cerebrospinal fluid (CSF) and plasma obtained from children with systemic/central nervous system infection.

Methods: A total of 74 salvaged CSF samples (27 with EV, 23 with PeV-A3, and 24 with neither EV nor PeV-A3) and 35 paired blood samples (10 with EV, 14 with PeV-A3, and 11 with neither) were studied. Concentrations of cytokines …

Transient Hyponatremia Of Prematurity Caused By Mild Bartter Syndrome Type Ii: A Case Report., Subhrata Verma, Rahul Chanchlani, Victoria Mok Siu, Guido Filler

Paediatrics Publications

BACKGROUND: Bartter syndrome subtypes are a group of rare renal tubular diseases characterized by impaired salt reabsorption in the tubule, specifically the thick ascending limb of Henle's loop. Clinically, they are characterized by the association of hypokalemic metabolic alkalosis, hypercalciuria, nephrocalcinosis, increased levels of plasma renin and aldosterone, low blood pressure and vascular resistance to angiotensin II. Bartter syndrome type II is caused by mutations in the renal outer medullary potassium channel (ROMK) gene (KCNJ1), can present in the newborn period and typically requires lifelong therapy.

CASE PRESENTATION: We describe a case of a prematurely born female infant presenting with …

Evolving Role For Pharmacotherapy In Nafld/Nash, Suzanna L. Attia, Samir Softic, Marialena Mouzaki

Pediatrics Faculty Publications

Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent, dynamic disease that occurs across the age spectrum and can lead to cirrhosis and hepatocellular carcinoma. There are currently no US Food and Drug Administration (FDA) approved treatments for NAFLD; however, this is a field of active research. This review summarizes emerging pharmacotherapies for the treatment of adult and pediatric NAFLD. Investigated pharmacotherapies predominantly target bile acid signaling, insulin resistance, and lipid handling within the liver. Three drugs have gone on to phase III trials for which results are available. Of those, obeticholic acid is the single agent that demonstrates promise …

Effects Of A Postnatal Atrx Conditional Knockout In Neurons On Autism-Like Behaviours In Male And Female Mice., Nicole Martin-Kenny, Nathalie G Bérubé

Paediatrics Publications

BACKGROUND: Alpha-thalassemia/mental retardation, X-linked, or ATRX, is an autism susceptibility gene that encodes a chromatin remodeler. Mutations of ATRX result in the ATR-X intellectual disability syndrome and have been identified in autism spectrum disorder (ASD) patients. The mechanisms by which ATRX mutations lead to autism and autistic-like behaviours are not yet known. To address this question, we generated mice with postnatal Atrx inactivation in excitatory neurons of the forebrain and performed a battery of behavioural assays that assess autistic-like behaviours.

METHODS: Male and female mice with a postnatal conditional ablation of ATRX were generated using the Cre/lox system under the …

Seasonal Influence On Postoperative Hip Fracture Complications: Retrospective Cohort Of More Than 1000 Patients From A Tertiary-Care University Hospital, Obada Hasan, Mashal Amin, Fatima Mahmood, Amna Rabbani, Umar Rabbani, Shahryar Noordin

Department of Paediatrics and Child Health

Background: Hip fractures account for one of the most debilitating conditions affecting the general population amongst the developed and developing nations. Investigators aimed to study the influence of the ongoing season i.e. whether patients operated upon in summer or winter, on post-operative complications occurring within 30 days of surgical procedure for hip fractures at a tertiary care hospital and level 1 trauma center.
Materials and methods: Investigators conducted a non-funded, non-commercialized retrospective cohort of 1045 patients with hip fractures managed surgically. Primary exposure was surgical procedures undertaken during the summer months (April-September). Our primary outcome was determining post-operative complication rates …

Do New Trainees Pose A Threat To Postoperative Complications After Hip Fracture Surgeries? Retrospective Cohort Of 1045 Patients Over A Decade At A University Hospital, Obada Hasan, Mashal Amin, Umar Rabbani, Amna Rabbani, Fatima Mahmood, Shahryar Noordin

Department of Paediatrics and Child Health

Introduction: Induction of new residents and surgical trainees in most institutes occurs once a year. Fresh residents with no experience, may pose a threat to the surgical procedure outcome and there can be a potential increase in patients' morbidity and mortality as a result of this turnover. Literature is inconclusive about this effect. Our aim was to study the "new residents' induction effect" on postoperative complications after hip fracture surgeries.
Methodology: This is non funded non commercialized study from a university hospital. Investigators studied a retrospective cohort of 1045 adult hip fracture patients who were operated at our tertiary care …

Education And Safety For Children With Autism To Prevent Elopement (E.S.C.A.P.E.) Kits, Silvia Pereira-Smith, Jessie Montezuma, Nicole Vonada, Laura A. Carpenter, Andrea Boan

MUSC Conference Presentations

Per a study published in 2019, two-thirds of patients with autism seen by the Division of Developmental-Behavioral Pediatrics (DBP) at MUSC engaged in elopement behavior, a potentially life-threatening behavior also known as wandering, that can lead to serious injury or death. E.S.C.A.P.E. kits were created by Dr. Pereira and the MUSC DBP autism research team in 2017 to help prevent elopement. Original kits included a door/window alarm, child ID kit, stop sign visual aids, ID bracelet, and printed materials with additional prevention tips. Initial survey data showed that 17 of 22 caregivers who completed surveys after receiving a kit reported …

Diabetes Conversation Map - A Novel Tool For Diabetes Management Self-Efficacy Among Type 2 Diabetes Patients In Pakistan: A Randomized Controlled Trial, Rubina Qasim, Sarfaraz Masih, Mohammad Tahir Yousafzai, Hakim Shah, Abdul Manan, Yousaf Shah, Muhammad Yaqoob, Abida Razzaq, Ajmal Khan, Atiya Rahman Khan Rohilla

Department of Paediatrics and Child Health

Background: This study aimed to measure the effect of diabetes education using the novel method of "diabetes conversation map (DCM)" as compared to routine counselling (RC) on diabetes management self-efficacy (DMSE) among patients living with type 2 diabetes in Karachi, Pakistan.
Methods: A parallel arm randomized controlled trial among patients with type 2 diabetes aged 30-60 years, with HbA1c > 7%, diagnosed for at least 5 yrs., was conducted at the national institute of diabetes and endocrinology in Karachi, Pakistan. A total 123 type 2 diabetes patients were randomized into DCM (n = 62) or RC (n = 61). Four weekly …

Failure To Thrive - An Overlooked Manifestation Of Kmt2b-Related Dystonia: A Case Presentation., Andrew Ng, Serena Galosi, Lisa Salz, Terence Wong, Caitlin Schwager, Shivarajan Amudhavalli, Rose N. Gelineau-Morel, Shimul Chowdhury, Rady Children’S Institute For Genomic Medicine Investigators, Jennifer Friedman

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: KMT2B-related dystonia is a recently described form of childhood onset dystonia that may improve with deep brain stimulation. Prior reports have focused on neurologic features including prominent bulbar involvement without detailing general health consequences that may result from orolingual dysfunction. We describe a family with novel KMT2B mutation with several members with failure to thrive to highlight this non-neurologic, but consequential impact of mutation in this gene.

CASE PRESENTATION: We present a case of a 15-year old female who was admitted and evaluated for failure to thrive. On exam, she had severe speech dysfluency, limited ability to protrude the …

Investigation Of Japanese Encephalitis Virus As A Cause Of Acute Encephalitis In Southern Pakistan, April 2015-January 2018, Tazeen Fatima, Abida Rais, Erum Khan, Susan L. Hills, Trudy V. Chambers, Aneeta Hotwani, Shahida Qureshi, Saad Shafqat, Saima Malik, Farah Qamar, Fatima Mir, Anthony A. Marfin, Anita K. M. Zaidi, Asif Raza Khowaja, Sadia Shakoor

Department of Pathology and Laboratory Medicine

Background: Japanese encephalitis (JE) occurs in fewer than 1% of JE virus (JEV) infections, often with catastrophic sequelae including death and neuropsychiatric disability. JEV transmission in Pakistan was documented in 1980s and 1990s, but recent evidence is lacking. Our objective was to investigate JEV as a cause of acute encephalitis in Pakistan.
Methods: Persons aged ≥1 month with possible JE admitted to two acute care hospitals in Karachi, Pakistan from April 2015 to January 2018 were enrolled. Cerebrospinal fluid (CSF) or serum samples were tested for JEV immunoglobulin M (IgM) using the InBios JE DetectTM assay. Positive or equivocal samples …

Pediatric Outpatient Utilization By Differing Medicaid Payment Models In The United States., Therese L Canares, Ari Friedman, Jonathan Rodean, Rebecca R Burns, Deena Berkowitz, Matt Hall, Elizabeth Alpern, Amanda Montalbano

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: In the United States (US), Medicaid capitated managed care costs are controlled by optimizing patients' healthcare utilization. Adults in capitated plans utilize primary care providers (PCP) more than emergency departments (ED), compared to fee-for-service (FFS). Pediatric data are lacking. We aim to determine the association between US capitated and FFS Medicaid payment models and children's outpatient utilization.

METHODS: This retrospective cohort compared outpatient utilization between two payment models of US Medicaid enrollees aged 1-18 years using Truven's 2014 Marketscan Medicaid database. Children enrolled > 11 months were included, and were excluded for eligibility due to disability/complex chronic condition, lack of …