Pediatrics Commons^™

A Rare Case Of Combined Immunodeficiency Syndrome With Otomastoiditis, Parotitis & Facial Nerve Palsy, Parth Garg, Amulya T M, Prakash B.G., Sandhya D

Digital Journal of Clinical Medicine

This article presents a case study of a 4-month-old female baby with combined immunodeficiency syndrome complicated by bilateral otomastoiditis, right-sided parotitis, and facial nerve palsy. The patient presented with symptoms of fever, cold, earache, and ear discharge. Clinical examination revealed redness and swelling in the post-auricular region, along with bloody discharge from the right ear. Further investigations, including blood tests, culture sensitivity, ultrasound scans, and imaging studies, confirmed the diagnosis. The patient was treated with appropriate antibiotics, antifungals, and supportive care. The article discusses the challenges in diagnosing and managing primary immunodeficiency diseases (PIDs), emphasising the importance of early recognition …

Bone Growth Induction In Mucopolysaccharidosis Iva Mouse, Estera Rintz, Angélica María Herreño-Pachón, Betul Celik, Fnu Nidhi, Shaukat Khan, Eliana Benincore-Flórez, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is caused by a deficiency of the N-acetylgalactosamine-6-sulfate-sulfatase (GALNS) enzyme, leading to the accumulation of glycosaminoglycans (GAG), keratan sulfate (KS) and chondroitin-6-sulfate (C6S), mainly in cartilage and bone. This lysosomal storage disorder (LSD) is characterized by severe systemic skeletal dysplasia. To this date, none of the treatment options for the MPS IVA patients correct bone pathology. Enzyme replacement therapy with elosulfase alpha provides a limited impact on bone growth and skeletal lesions in MPS IVA patients. To improve bone pathology, we propose a novel gene therapy with a small peptide as a growth-promoting …

Infant Effortful Control Predicts Bmi Trajectories From Infancy To Adolescence, Lillybelle K. Deer, Jenalee R. Doom, Kylie K. Harrall, Deborah H. Glueck, Laura M. Glynn, Curt A. Sandman, Elysia Poggi Davis

Psychology Faculty Articles and Research

Background

Effortful control, or the regulation of thoughts and behaviour, is a potential target for preventing childhood obesity.

Objectives

To assess effortful control in infancy through late childhood as a predictor of repeated measures of body mass index (BMI) from infancy through adolescence, and to examine whether sex moderates the associations.

Methods

Maternal report of offspring effortful control and measurements of child BMI were obtained at 7 and 8 time points respectively from 191 gestational parent/child dyads from infancy through adolescence. General linear mixed models were used.

Results

Effortful control at 6 months predicted BMI trajectories from infancy through adolescence, …

Achondroplasia Natural History Study (Clarity): 60-Year Experience In Orthopedic Surgery From Four Skeletal Dysplasia Centers, Nickolas J Nahm, W G Stuart Mackenzie, William G Mackenzie, Ethan Gough, S Shahrukh Hashmi, Jacqueline T Hecht, Janet M Legare, Mary Ellen Little, Peggy Modaff, Richard M Pauli, David F Rodriguez-Buritica, Maria Elena Serna, Cory J Smid, Julie Hoover-Fong, Michael B Bober

Faculty, Staff and Student Publications

BACKGROUND: The purpose of this study was to describe the frequency and risk factors for orthopedic surgery in patients with achondroplasia. CLARITY (The Achondroplasia Natural History Study) includes clinical data from achondroplasia patients receiving treatment at four skeletal dysplasia centers in the United States from 1957 to 2018. Data were entered and stored in a Research Electronic Data Capture (REDCap) database.

RESULTS: Information from one thousand three hundred and seventy-four patients with achondroplasia were included in this study. Four hundred and eight (29.7%) patients had at least one orthopedic surgery during their lifetime and 299 (21.8%) patients underwent multiple procedures. …

A Shared Decision-Making Process Utilizing A Decision Coach In Pediatric Epilepsy Surgery, Michelle Kregel, Natalie Evans, Bonnie Wooten, Craig Campbell, Sandrine De Ribaupierre, Andrea Andrade

Paediatrics Publications

BACKGROUND & RATIONALE: The process to evaluate candidacy for epilepsy surgery is lengthy and stressful for caregivers, therefore the decision can be challenging. There is not a lot of information in regard to how families of a child living with epilepsy navigate the stressful decision during surgical candidacy evaluation. With difficult decisions comes the possibility of increased decisional conflict in both the child and the family.

METHODS: A project designed to provide greater knowledge to the family was conducted utilizing the shared decision-making (SDM) process to assist families in the decision-making during surgical candidacy evaluation; this was done using a …

The Role Of Geography And Distance On Physician Follow-Up After A First Hospitalization With A Diagnosis Of A Schizophrenia Spectrum Disorder: A Retrospective Population-Based Cohort Study In Ontario, Canada, Kelly Anderson, Martin Rotenberg, Peter Gozdyra, Paul Kurdyak

Data and Test Instruments

No abstract provided.

Kawasaki Disease And Mis-C At A Community Children’S Hospital In Pennsylvania: A Five-And-A-Half Year Retrospective Study, Kelsey Kaplan Do, Kayle Barndt Do, Ruchi Gupta Md, Kris Rooney Md, Kyle Shaak Mph

Department of Pediatrics

No abstract provided.

Caregivers Of Youth In Foster Care In Texas And Their Role In Contraception Decision-Making For Adolescents In Care, Shelby Edmondson, Ana Belen Conrado, Hilda Loria, Jill Mcleigh, Jasmin Tiro, Simon Lee, Jenny Francis

Caregiver Needs and Supports

Few studies address the experiences of caregivers of adolescents in foster care. This study highlights a range of caregiver attitudes about contraception for adolescents in care. Provider training regarding contraception should include strategies to manage caregiver beliefs and extreme reactions to contraception use among youth in care.

For questions about this study or others, please email [email protected]

childrens.com/fostercare

Diabetes-Related Quality Of Life Assessment In Children Following Total Pancreatectomy With Islet Autotransplantation., Jacob M. Redel, Lindsey Hornung, Deborah Elder, Jaimie D. Nathan, Sarah Corathers, Kristin L. Rich, Maisam Abu-El-Haija

Manuscripts, Articles, Book Chapters and Other Papers

Total pancreatectomy with islet autotransplantation (TPIAT) can improve pain and reduce functional impairment associated with acute recurrent or chronic pancreatitis. However, long-term glucose monitoring and insulin therapy are often required, which can adversely affect the quality of life. We sought to evaluate diabetes-related quality of life (DR-QOL) in youth who underwent TPIAT and compare it to the youth with new-onset type 1 diabetes (T1D). The Pediatric Quality of Life Inventory™ 3.2 Diabetes Module (PedsQL™ DM) was used to assess DR-QOL in 46 youth (< 20 years old) who underwent TPIAT. The PedsQL™ DM scores were analyzed for statistically significant changes and minimally important clinical differences (MCID) over time post-TPIAT. Scores at 12 months (n = 52) and 24 months (n = 58) after diagnosis of T1D. The diabetes …

Divergent Clinical Presentations And Outcomes Among Children And Adolescents With Kaposi Sarcoma In Malawi And Tanzania, Liane R Campbell, Allison Silverstein, Erin Peckham-Gregory, William Kamiyango, Jimmy Villiera, Casey L Mcatee, Jason M Bacha, Carrie L Kovarik, Parth S Mehta, Toni Chanroo, Asulwisye Kapesa, Beatrice Malingoti, Rizine Mzikamanda, Nmazuo W Ozuah, Carl E Allen, Michael E Scheurer, Nader K El-Mallawany

Center for Medical Ethics and Health Policy Staff Publications

Objectives: The Kaposi sarcoma (KS) T0 versus T1 staging classification does not address the unique clinical features of paediatric KS in human gammaherpesvirus 8 (HHV-8) endemic regions of Africa. This study seeks to define patterns of childhood KS using a paediatric-specific approach.

Methods: The Lilongwe paediatric KS staging classification categorizes disease based on clinical phenotype: stage 1 = mild/moderate KS limited to cutaneous/oral involvement, stage 2 = primarily lymphadenopathic disease, stage 3 = woody edema KS, stage 4 = visceral and/or severe/disseminated mucocutaneous disease. Characteristics and outcomes were evaluated from paediatric referral centres in Lilongwe, Malawi, and Mbeya, Tanzania.

Results: …

Central Hypothyroidism Following Neonatal Graves' Presentation, Emily Metzinger, Kelsee Halpin

Posters

Background: Neonatal Graves’ disease is a rare disorder of neonatal autoimmune hyperthyroidism secondary to the transplacental passage of thyroid-stimulating hormone receptor antibodies. Although neonatal Graves’ disease is transient, it is associated with cardiac dysfunction, intrauterine growth restriction, prematurity, craniosynostosis, and developmental delay and can be life-threatening. Additionally, infants with neonatal Graves’ disease can rarely present with primary or central hypothyroidism later in infancy or childhood. This is suspected to be due to the disruption in the hypothalamic-pituitary-thyroid axis or development of the thyroid gland. Clinical Case: Here we describe a case of neonatal Graves’ disease that has been complicated by …

Supporting Fathers And Partners In The Nicu, Tiffany Gladdis

Posters

The NICU can be an incredibly stressful environment for both the infant and its parents. Unlike many traumatic events, the NICU experience can often feel like an ongoing trauma, with the stay often involving highs and lows, new diagnoses, changes in prognosis, multiple procedures, and a rollercoaster of emotions. Additionally, the NICU can also trigger trauma responses for parents who have had previous traumatic experiences. When working with families in the NICU, it is important to use a Trauma Informed Care lens to ensure that the whole family is understood and adequately supported (Sanders & Hall, 2007). Perinatal Mood and …

Endometriosis Eras, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Dexmedetomidine Alters The Inflammatory Profile Of Rat Microglia In Vitro, Michael C Scott, Candice M Haase, Scott D Olson, Charles S Cox

Faculty, Staff and Student Publications

BACKGROUND: Microglia are a primary mediator of the neuroinflammatory response to neurologic injury, such as that in traumatic brain injury. Their response includes changes to their cytokine expression, metabolic profile, and immunophenotype. Dexmedetomidine (DEX) is an α

METHODS: Primary microglia were isolated from Sprague-Dawley rats and cultured. Microglia were activated using multiple mediators: lipopolysaccharide (LPS), polyinosinic-polycytidylic acid (Poly I:C), and traumatic brain injury damage-associated molecular patterns (DAMP) from a rat that sustained a prior controlled cortical impact injury. After activation, cultures were treated with DEX. At the 24-h interval, the cell supernatant and cells were collected for the following studies: …

Bronchiolitis, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Clinical Practice Guidelines)

No abstract provided.

Factors Predictive Of Being Bullies Or Victims Of Bullies In Us Elementary Schools, Paul L Morgan, George Farkas, Adrienne D Woods, Yangyang Wang, Marianne M Hillemeier, Yoonkyung Oh

Faculty, Staff and Student Publications

We analyzed a population-representative cohort (N=13,611; Mage at kindergarten, first, and second grade = 67.5, 79.5, and 91.5 months, respectively) to identify kindergarten to second grade factors predictive of being bullies or victims during third to fifth grade. We did so by estimating a block recursive structural equation model (SEM) with three sets of predictors. These were: (a) individual and school socio-demographics; (b) family distress and harsh parenting; and (c) individual behavior and achievement. Relations between each of the included variables and the bullying outcomes were simultaneously estimated within the SEM. Thus, each variable served as a control …

Creating A Data Dictionary For Pediatric Autonomic Disorders, Jeffrey R Boris, Hasan Abdallah, Shelley Ahrens, Gisela Chelimsky, Thomas C Chelimsky, Philip R Fischer, John E Fortunato, Raewyn Gavin, Janice L Gilden, Renato Gonik, Blair P Grubb, Kelsey M Klaas, Erin Marriott, Lauren E Marsillio, Marvin S Medow, Lucy Norcliffe-Kaufmann, Mohammed T Numan, Erin Olufs, Laura A Pace, Paul T Pianosi, Pippa Simpson, Julian M Stewart, Sally Tarbell, Natalie R Van Waning, Debra E Weese-Mayer

Faculty, Staff and Student Publications

PURPOSE: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders.

METHODS: Our group created the list of terms for the dictionary. Definitions were prioritized to be obtained from established sources with which …

Evolving Antibiotic Resistance In Group B Streptococci Causing Invasive Infant Disease: 1970–2021, Elizabeth Marie Sabroske, Misu Ailin Sanson Iglesias, Marcia Rench, Trevor Moore, Hanna Harvey, Morven Edwards, Carol J Baker, Anthony R Flores

Faculty, Staff and Student Publications

BACKGROUND: We sought to define the frequency of antibiotic resistance over time in a collection of invasive GBS isolates derived from infant early-onset disease (EOD), late-onset disease (LOD), and late-late onset disease (LLOD).

METHODS: A multicenter retrospective review of infants born from 1970 to 2021 with GBS isolated from blood, cerebrospinal fluid, synovial fluid, cellulitis, or bone. All isolates were serotyped and antimicrobial susceptibility testing performed using disk diffusion.

RESULTS: The most common serotypes in our 2017 isolates were III (n = 1112, 55.1%), Ia (n = 445, 22%), Ib (n = 182, 9%) and II (n = 146, 7.2%). …

Red Blood Cell Transfusion Thresholds For Anemia Of Prematurity, Lindsay F Holzapfel, Matthew A Rysavy, Edward F Bell

Faculty, Staff and Student Publications

Anemia of prematurity affects the majority of preterm infants, particularly extremely low birthweight infants. Anemia of prematurity arises from both innate and iatrogenic causes and results in more than 80% of extremely preterm infants receiving red blood cell transfusions during the first month after birth. Multiple randomized controlled trials were conducted to evaluate the effect of using lower versus higher transfusion thresholds based on hemoglobin levels. These trials showed no difference in the primary outcome of neurodevelopmental impairment at 2 years of age between lower and higher thresholds. However, some uncertainties about transfusion thresholds remain. This review elaborates the following: …

Impact Of Transfused Citrate On Pathophysiology In Massive Transfusion, Jacob B Schriner, J Michael Van Gent, M Adam Meledeo, Scott D Olson, Bryan A Cotton, Charles S Cox, Brijesh S Gill

Faculty, Staff and Student Publications

This narrative review article seeks to highlight the effects of citrate on physiology during massive transfusion of the bleeding patient.

DATA SOURCES: A limited library of curated articles was created using search terms including "citrate intoxication," "citrate massive transfusion," "citrate pharmacokinetics," "hypocalcemia of trauma," "citrate phosphate dextrose," and "hypocalcemia in massive transfusion." Review articles, as well as prospective and retrospective studies were selected based on their relevance for inclusion in this review.

STUDY SELECTION: Given the limited number of relevant studies, studies were reviewed and included if they were written in English. This is not a systematic review nor a …