Pediatrics Commons^™

Expanded-Access Use Of Elamipretide In A Patient With Membrane Protein-Associated Neurodegeneration, Jorge Patino, Anna Haertling Clearman, Lindsey Miller, Mary Kay Koenig

Faculty, Staff and Student Publications

This case report presents a progressively declining 17-year-old patient with membrane protein-associated neurodegeneration who demonstrated symptomatic improvements in her dysarthria, dysphagia, and gait, and objective improvements in her 6-minute walk test and 5 times sit-to-stand test during elamipretide treatment.

The Role Of Occipital Condyle And Atlas Anomalies On Occipital Cervical Fusion Outcomes In Chiari Malformation Type I With Syringomyelia: A Study From The Park-Reeves Syringomyelia Research Consortium, Alexander T Yahanda, Joyce Koueik, Laurie L Ackerman, P David Adelson, Gregory W Albert, Philipp R Aldana, Tord D Alden, Richard C E Anderson, David F Bauer, Tammy Bethel-Anderson, Karin Bierbrauer, Douglas L Brockmeyer, Joshua J Chern, Daniel E Couture, David J Daniels, Brian J Dlouhy, Susan R Durham, Richard G Ellenbogen, Ramin Eskandari, Herbert E Fuchs, Gerald A Grant, Patrick C Graupman, Stephanie Greene, Jeffrey P Greenfield, Naina L Gross, Daniel J Guillaume, Todd C Hankinson, Gregory G Heuer, Mark Iantosca, Bermans J Iskandar, Eric M Jackson, George I Jallo, James M Johnston, Bruce A Kaufman, Robert F Keating, Nickalus R Khan, Mark D Krieger, Jeffrey R Leonard, Cormac O Maher, Francesco T Mangano, Jonathan Martin, J Gordon Mccomb, Sean D Mcevoy, Thanda Meehan, Arnold H Menezes, Michael S Muhlbauer, Brent R O'Neill, Greg Olavarria, John Ragheb, Nathan R Selden, Manish N Shah, Chevis N Shannon, Joshua S Shimony, Matthew D Smyth, Scellig S D Stone, Jennifer M Strahle, Mandeep S Tamber, James C Torner, Gerald F Tuite, Elizabeth C Tyler-Kabara, Scott D Wait, John C Wellons, William E Whitehead, Tae Sung Park, David D Limbrick, Raheel Ahmed

Faculty, Staff and Student Publications

Objective: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF).

Methods: The authors analyzed the prevalence of condylar …

Use Of Clearguard Hd Caps In Pediatric Hemodialysis Patients., Amy E. Nau, Troy Richardson, Diana Cardwell, Jennifer Ehrlich, Jyothsna Gattineni, Melisha Hanna, Mahima Keswani, Emily Neibauer, Kelly Nitz, Raymond Quigley, Michelle Rheault, Rebekah Sims, Mayna Woo, Bradley A. Warady

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Bloodstream infections (BSIs) are a leading cause of hospitalizations and mortality among patients receiving hemodialysis (HD) therapy, especially those with a central venous catheter (CVC) for dialysis access. The use of chlorhexidine impregnated catheter caps (ClearGuard) has been associated with a decrease in the rate of HD catheter-related BSIs (CA-BSIs) in adults; similar data have not been published for children.

METHODS: We compared CA-BSI data from participating centers within the Standardizing Care to Improve Outcomes in Pediatric Endstage Kidney Disease (SCOPE) collaborative based on the center's use of ClearGuard caps for patients with HD catheter access. Centers were characterized …

Preeclampsia, Fetal Growth Restriction, And 24-Month Neurodevelopment In Very Preterm Infants., Jennifer Check, Coral Shuster, Julie Hofheimer, Marie Camerota, Lynne M. Dansereau, Lynne M. Smith, Brian S. Carter, Sheri A. Dellagrotta, Jennifer Helderman, Howard Kilbride, Cynthia M. Loncar, Elisabeth Mcgowan, Charles R. Neal, T Michael O'Shea, Steven L. Pastyrnak, Stephen J. Sheinkopf, Barry M. Lester

Manuscripts, Articles, Book Chapters and Other Papers

IMPORTANCE: Preeclampsia has direct influences on a developing fetus and may impact postnatal health, and fetal growth restriction (FGR) is often seen co-occurring with preeclampsia. The development of children born very preterm after preeclampsia diagnosis with and without FGR is not well characterized.

OBJECTIVE: To examine the associations of preeclampsia and FGR with developmental and/or behavioral outcomes in a cohort of very preterm infants.

DESIGN, SETTING, AND PARTICIPANTS: In this cohort study, infants in the prospective Neonatal Neurobehavior and Outcomes in Very Preterm Infants study were enrolled between April 2014 and June 2016 from 9 US university-affiliated neonatal intensive care …

Including Young Children In Their Food Allergy Care: A Pilot Study., Jodi Shroba, Susan F. Mcelroy

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE: Food allergy education is often directed toward adult caregivers. However, once children go to school, they must participate in their food allergy care to remain safe. The purpose of this study was to assess food allergy knowledge and test an educational intervention targeted toward the child. We hypothesized that child-based teaching will be equal in safety and knowledge outcomes compared with standard parental education.

METHODS: Twenty-nine children between the ages of 5-11 years and their caregivers were enrolled. Child subjects completed a food allergy knowledge questionnaire. Each caregiver/child dyad was randomized to receive parent-targeted education (control) or child-targeted education …

The Use Of Hearing Tests To Assess Otitis Media With Effusion In Children With Down Syndrome., Mackenzie O'Donnell, Nasrin Sultana, Nasreen Talib, Jason May, Michael Slogic

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Down syndrome is associated with an increased risk for otitis media with effusion (OME), a childhood condition in which fluid accumulates in the middle ear, potentially leading to hearing loss. The American Academy of Pediatrics Down syndrome guidelines and the American Academy of Otolaryngology - Head and Neck Surgery OME guidelines recommend hearing testing to assess the hearing status of children with Down syndrome diagnosed with OME.

METHODS: Through an Institutional Review Board approved retrospective chart review at Children's Mercy, this project assessed how clinical factors affect the frequency in which children with Down syndrome receive hearing testing after …

The Safeboosc-Iii Trial And The Future Of Cerebral Oximetry-Guided Interventions In Preterm Infants-Time To Pause And Reset?, Krishna Dummula, Vishal Pandey, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

Comment on

Cerebral Oximetry Monitoring in Extremely Preterm Infants. N Engl J Med. 2023 Apr 20;388(16):1501-1511. doi: 10.1056/NEJMoa2207554.PMID: 37075142 Clinical Trial.

Perbedaan Pengaruh Media Edukasi Terhadap Kepatuhan Orang Tua Anak Cerebral Palsy Untuk Melaksanakan Home Program, Khabib Abdullah, Ichsanul Amalia, Aji Sukma Wijaya

Jurnal Fisioterapi Terapan Indonesia

Cerebral palsy (CP) is a condition of movement and function disorders in children that requires continuous physiotherapy. One factor in the successful development of children with CP is the existence of a home program/independent therapy carried out by parents at home. In providing the home program, physiotherapists at YPCP Surabaya gave leaflets to parents. However, this was less effective because it was easily damaged, so the home program media was replaced with video. The effectiveness of both in increasing parental compliance in carrying out the home program every day is not yet known. Objective: to find out the difference in …

Occupational Therapy In Stroke: A Case Study Using The Bobath Frame Of Reference And Task Oriented Approach, Reza Nur Arsyi, Afifah Fadhilah Nursyam, Muhammad Luthfi, Hermito Gidion, Ayleen Alicia Kosasi, Erna Faryza Mohd Poot, Rosilah Wahab, Nur Atiqah Azman

Jurnal Fisioterapi Terapan Indonesia

Stroke is a sudden neurological disorder caused by vascular injury to the brain, leading to significant functional limitations. This case study examines the use of occupational therapy interventions, specifically the Bobath and Task Oriented approaches, for a 43-year-old male with left hemiparesis to enhance independence in motorcycling. The patient experienced various limitations, including vestibular function, range of motion, muscle tone and strength, postural control, and fine motor skills. Using the Ashworth scale and the Canadian Occupational Performance Measure (COPM) for assessment, occupational therapy interventions showed progress in several aspects, although the primary goal has not yet been fully achieved. This …

Terapi Latihan Pada Fase Satu Pasca Operasi Rekonstruksi Ruptur Anterior Cruciate Ligamentum Sinistra, Aunike Arum Meta Wurgani, Irawan Wibisono

Jurnal Fisioterapi Terapan Indonesia

Anterior Cruciate Ligament (ACL) reconstruction is a surgical procedure involving the grafting of the ACL using tendon tissue. This procedure aims to restore knee stability and joint fixation following an ACL injury. Post-ACL reconstruction often leads to complications such as pain, edema, reduced range of motion, and muscle weakness. The physiotherapy program in this case aims to evaluate the effects of physiotherapy interventions, including quadriceps setting, hamstring setting, straight leg raise (SLR), heel slide, ankle pumping, bridging, and prone hang exercises, in reducing edema and pain, as well as improving the range of motion and muscle strength in the knee …

Jump To Box Kombinasi Core Plank Knee To Elbow Terhadap Power Otot Tungkai, Maya Triyanita

Jurnal Fisioterapi Terapan Indonesia

Leg power training is defined as a movement to get the jump in athletes doing the maximum jump smash. Badminton requires leg muscle explosive power because it is the muscle's ability to overcome loads and with a high speed of contraction. This study aims to determine differences in the effect of jump to box training, a combination of core plank knee to elbow on increasing leg power in badminton athletes. This study used an experimental design method with a pre-test and post-test design. The treatment group was given the Jump To Box Combination Core Plank Knee To Elbow exercise. The …

Randomized Controlled Trial Of Enteral Vitamin D Supplementation (Vides) In Infants <28 Weeks Gestational Age Or <1000 G Birth Weight: Study Protocol, Mar Romero-Lopez, Jon E Tyson, Mamta Naik, Claudia Pedroza, Lindsay F Holzapfel, Elenir Avritscher, Ricardo Mosquera, Amir Khan, Matthew Rysavy

Faculty, Staff and Student Publications

BACKGROUND: Vitamin D is necessary to develop healthy lungs and other organs early in life. Most infants born before 28 weeks' gestation have low vitamin D levels at birth and a limited intake during the first month. Enteral vitamin D supplementation is inexpensive and widely used. The appropriate supplementation regimen for extremely preterm infants is controversial, and the effect of different regimens on their blood levels and outcomes is unclear.

METHODS: Randomized, blinded comparative effectiveness trial to compare two vitamin D supplementation regimens for inborn infants(400 IU/day with established feedings) or increased supplementation (800 IU/day with any feedings) during the …

A Systematic Review Of Telehealth Utilization For Bowel Management Programs In Pediatric Colorectal Surgery., Elizaveta Bokova, Ismael Elhalaby, Seth Saylors, Irene Isabel P. Lim, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Recent advancements in pediatric surgery have embraced telehealth (TH) modalities, transitioning from traditional in-person consultations to virtual care. This shift has broadened access to healthcare, potentially enhancing affordability, patient and caregiver satisfaction, and clinical outcomes. In pediatric colorectal surgery, telehealth has been effectively utilized to support Bowel Management Programs (BMPs) for children suffering from constipation and fecal incontinence. A systematic review was conducted to assess the effectiveness of virtual BMPs, analyzing studies from January 2010 to December 2023, sourced from MEDLINE (via PubMed), Embase, and the Cochrane Library, with five studies included. Remote BMPs, implemented through video or telephone consultations, …

Keep Your Summer Fun Safe, Maggie Mckay, Laura Kemerling

Parent-ish Podcast

Laura Kemerling, MSN, RN, C-NPT, CPST, discusses summer safety and how to prevent injuries - no matter what your summer fun entails!

Click here to stream the podcast episode.

Postnatal Corticosteroid Therapy In Bronchopulmonary Dysplasia - Why Animal Studies Disagree With Clinical Trials?, Anantha Harijith, Thomas M. Raffay, Rita M. Ryan

Faculty Scholarship

Abstract: The systematic review and meta-analysis of newborn animal models by Irene Lok et al. is the first to extensively summarize the literature regarding postnatal systemic corticosteroid use on lung development of newborn rodent models. The meta-analysis showed that the use of postnatal corticosteroids resulted in a reduction in body weight along with persistent alveolar simplification. The most frequently used corticosteroid was dexamethasone. Corticosteroids have been extensively used in clinical trials in preterm newborns. Trials using early systemic administration of corticosteroids reduced the rate of BPD or mortality with no increase in the rates of cerebral palsy. Use of late …

Back Pain In Adolescent Idiopathic Scoliosis: Frequency And Risk Factors, Stefan Sarkovich, Claudia Leonardi, Matthew Darlow, Davis Martin, Peter Issa, Tara Soria, Amy Bronstone, Carter Clement

School of Public Health Faculty Publications

Purpose: Although back pain is commonly reported in patients with adolescent idiopathic scoliosis (AIS), factors that influence the presence and severity of back pain in AIS, including curve-specific variables, have not been well studied. This study aims to describe the prevalence and severity of back pain in AIS patients and determine the extent to which patient characteristics, including curve-specific factors, are associated with a higher risk of back pain in AIS. Methods: The study was a retrospective medical records review of adolescents (aged 10–17 years) diagnosed with AIS between 01/01/2018 and 12/31/2021 at an academic tertiary children’s hospital. Patients with …

A Case Of Severe Anaphylactic Reaction After Administration Of Diagnostic-Dose Ferumoxytol In A Pediatric Patient, Fam Ekladious, David Saul

Jefferson Hospital Staff Papers and Presentations

We describe a case of anaphylaxis during administration of intravenous (IV) ferumoxytol as a magnetic resonance imaging (MRI) contrast agent in a 4-year-old patient with complicated past medical history including YARS genetic mutation with resultant liver failure and deceased donor liver transplantation, stage IV chronic kidney disease (CKD), and hypertension. The patient was noted to have labored breathing 4 min after initiation of ferumoxytol infusion and was subsequently rapidly intubated and returned to the intensive care unit (ICU) for monitoring. Anaphylactic reactions to therapeutic doses of ferumoxytol led to issuance of a black box warning by the FDA in 2015. …

The Impact Of Childhood Adversity On Life Course Alcohol Use Patterns And Health Status Among People Living With Hiv, Rayna E. Gasik, Aubrey Spriggs Madkour, Simone J. Skeen, Gretchen Clum, Tishawn Francis, Erica Felker-Kantor, Tekeda Ferguson, David A. Welsh, Patricia E. Molina, Katherine P. Theall

School of Public Health Faculty Publications

Adverse childhood experiences (ACEs) and financial hardship are associated with increased likelihood of heavier alcohol use and health challenges in adulthood among persons living with HIV (PWH). We examined whether retrospectively captured lifetime drinking trajectories are a pathway through which childhood hardships affect current health in a sample of 365 adult PWH. Childhood economic hardship and ACEs were used as main predictors. Measures of alcohol use included age at first drink and lifetime drinking trajectories. Health indicators included health-related quality of life, frailty, number of comorbidities, and symptoms of anxiety, depression, and post-traumatic stress disorder (PTSD). Structural equation modeling (SEM) …

The Effect Of Early Postnatal Auditory Stimulation On Outcomes In Preterm Infants, Juliann M. Di Fiore, Gloria Liu, Kenneth A. Loparo, Cynthia F. Bearer

Faculty Scholarship

Abstract: Preterm infants are deprived of in utero sensory stimulation during the third trimester, an important period of central nervous system development. As a result, maturational trajectories are often reduced in infants born preterm. One such system affected is the brain including the auditory and respiratory control pathways. During normal pregnancy the intrauterine environment attenuates external auditory stimuli while exposing the fetus to filtered maternal voice, intra-abdominal sounds, and external stimuli. In contrast, during the third trimester of development, preterm infants are exposed to a vastly different soundscape including non-attenuated auditory sounds and a lack of womb related stimuli, both …

Moral Distress And Pediatric Palliative Care., Sunny Jeong, Angie Knackstedt, Jennifer Linebarger, Brian S. Carter

Manuscripts, Articles, Book Chapters and Other Papers

Moral distress is a complex phenomenon whereby a person feels tension, constraint, or conflict with an action or circumstance because it goes against their individual or the perceived collective (e.g., community, organizational, or professional association's) moral stance. In pediatric healthcare settings, managing and mitigating feelings of moral distress can be particularly difficult to navigate through because of the intricate dynamics between the pediatric patient, parent and/or legal guardians, and clinicians. The proactive integration of an experienced pediatric palliative care (PPC) team can be an appropriate step toward reducing clinicians feeling overwhelmed by various case-specific and team management issues that contribute …