Medical Specialties Commons^™

Neonatal Renal Failure In The Setting Of Anorectal Malformation: A Case Report And Literature Review., Wendy Jo Svetanoff, Asma Ahmed, Richard J. Hendrickson, Rebecca M. Rentea

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Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic kidneys to highlight the nuances and complex decision-making considerations required in taking care of this complex patient population. Our patient is a male twin born at 32 weeks' gestational age who …

Chronic Inflammation In Chronic Kidney Disease Progression: Role Of Nrf2., Peter Stenvinkel, Glenn M. Chertow, Prasad Devarajan, Adeera Levin, Sharon P. Andreoli, Sripal Bangalore, Bradley A. Warady

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Despite recent advances in the management of chronic kidney disease (CKD), morbidity and mortality rates in these patients remain high. Although pressure-mediated injury is a well-recognized mechanism of disease progression in CKD, emerging data indicate that an intermediate phenotype involving chronic inflammation, oxidative stress, hypoxia, senescence, and mitochondrial dysfunction plays a key role in the etiology, progression, and pathophysiology of CKD. A variety of factors promote chronic inflammation in CKD, including oxidative stress and the adoption of a proinflammatory phenotype by resident kidney cells. Regulation of proinflammatory and anti-inflammatory factors through NF-κB- and nuclear factor, erythroid 2 like 2 (Nrf2)-mediated …

Factors Associated With Reduced Infliximab Exposure In The Treatment Of Pediatric Autoimmune Disorders: A Cross-Sectional Prospective Convenience Sampling Study., Ryan S. Funk, Valentina Shakhnovich, Yu Kyoung Cho, Kishore Polireddy, Taina Hudson, Kyle Gress, Mara L. Becker

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BACKGROUND: Inadequate systemic exposure to infliximab (IFX) is associated with treatment failure. This work evaluated factors associated with reduced IFX exposure in children with autoimmune disorders requiring IFX therapy.

METHODS: In this single-center cross-sectional prospective study IFX trough concentrations and anti-drug antibodies (ADAs) were measured in serum from children diagnosed with inflammatory bowel disease (IBD) (n = 73), juvenile idiopathic arthritis (JIA) (n = 16), or uveitis (n = 8) receiving maintenance IFX infusions at an outpatient infusion clinic in a tertiary academic pediatric hospital. IFX concentrations in combination with population pharmacokinetic modeling were used to estimate IFX clearance. Patient …

Investigation Of Alpl Variant States And Clinical Outcomes: An Analysis Of Adults And Adolescents With Hypophosphatasia Treated With Asfotase Alfa., Priya S. Kishnani, Guillermo Del Angel, Shanggen Zhou, Eric T. Rush

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Background: Hypophosphatasia (HPP), a rare metabolic disease, can be inherited in an autosomal recessive (biallelic) or an autosomal dominant (monoallelic) manner. Most of the severe, early-onset, frequently lethal HPP in infants is acquired through recessive inheritance; less severe, later-onset, typically nonlethal HPP phenotypes are acquired through either dominant or recessive inheritance. HPP's variable clinical presentation arises from >400 identified ALPL pathogenic variants with likely variable penetrance, especially with autosomal dominant inheritance. This post hoc analysis investigated the relationship between ALPL variant state (biallelic and monoallelic) and clinical outcomes with asfotase alfa in HPP.

Methods: Data were pooled from two phase …

Recalibration Of The Renal Angina Index For Pediatric Septic Shock., Natalja L. Stanski, Hector R. Wong, Rajit K. Basu, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Michael Quasney, Bereketeab Haileselassie, Lakhmir S. Chawla, Stuart L. Goldstein

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Introduction: Sepsis-associated acute kidney injury (AKI) is a common diagnosis in children that is associated with poor outcomes. The lack of therapeutic options once present makes early identification of at-risk patients essential. The renal angina index (RAI) has been previously validated to predict severe AKI in heterogeneous populations of critically ill children. The performance of this score specifically in children with septic shock is unknown.

Methods: A secondary analysis of a multicenter, prospective, observational study of 379 children with septic shock to determine the ability of the RAI to predict severe AKI at day 3, and to assess for the …

Global Trends In Norovirus Genotype Distribution Among Children With Acute Gastroenteritis., Jennifer L. Cannon, Joseph Bonifacio, Filemon Bucardo, Javier Buesa, Leesa Bruggink, Martin Chi-Wai Chan, Tulio M. Fumian, Sidhartha Giri, Mark D. Gonzalez, Joanne Hewitt, Jih-Hui Lin, Janet Mans, Christian Muñoz, Chao-Yang Pan, Xiao-Li Pang, Corinna Pietsch, Mustafiz Rahman, Naomi Sakon, Rangaraj Selvarangan, Hannah Browne, Leslie Barclay, Jan Vinjé

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Noroviruses are a leading cause of acute gastroenteritis (AGE) among adults and children worldwide. NoroSurv is a global network for norovirus strain surveillance among children(genotype and polymerase type) and uploaded 1,325 dual-typed sequences to the NoroSurv web portal during 2016-2020. More than 50% of submitted sequences were GII.4 Sydney[P16] or GII.4 Sydney[P31] strains. Other common strains included GII.2[P16], GII.3[P12], GII.6[P7], and GI.3[P3] viruses. In total, 22 genotypes and 36 dual types, including GII.3 and GII.20 viruses with rarely reported polymerase types, were detected, reflecting high strain diversity. Surveillance data captured in NoroSurv enables the monitoring of trends in norovirus strains …

Critical Need For Pharmacologic Treatment Options In Nafld: A Pediatric Perspective., Chance S. Friesen, Sherwin S. Chan, Jonathan B. Wagner, Chelsea Hosey-Cojocari, Iván L Csanaky, Valentina Shakhnovich

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Nonalcoholic fatty liver disease (NAFLD) affects up to 70% of children with obesity and has become the number one etiology for liver transplant in the United States. Early, effective intervention is critical to prevent disease progression into adulthood. Yet, it is seldom achieved through lifestyle modification alone. Thus, children must be included in NAFLD pharmacology trials, which, to date, continue to focus primarily on adult populations. This commentary serves as a call to action.

Real-Time Gastrointestinal Infection Surveillance Through A Cloud-Based Network Of Clinical Laboratories., Juliana M. Ruzante, Katherine Olin, Breda Munoz, Jeff Nawrocki, Rangaraj Selvarangan, Lindsay Meyers

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Acute gastrointestinal infection (AGI) represents a significant public health concern. To control and treat AGI, it is critical to quickly and accurately identify its causes. The use of novel multiplex molecular assays for pathogen detection and identification provides a unique opportunity to improve pathogen detection, and better understand risk factors and burden associated with AGI in the community. In this study, de-identified results from BioFire® FilmArray® Gastrointestinal (GI) Panel were obtained from January 01, 2016 to October 31, 2018 through BioFire® Syndromic Trends (Trend), a cloud database. Data was analyzed to describe the occurrence of pathogens causing AGI across United …

Health-Related Quality Of Life Across Recent Pediatric Obesity Classification Recommendations., William R. Black, Kelsey B. Borner, Marshall T. Beauchamp, Ann M. Davis, Meredith Dreyer, Brooke Sweeney, Sarah Hampl

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Extreme body mass index (BMI) values (i.e., above the 97th and below the 3rd percentiles) are inaccurately represented on the Centers for Disease Control and Prevention's growth curves, which may limit the utility of BMI percentile and BMI z-score for capturing changes in clinical outcomes for patients at extreme weights. Modeling child obesity severity based upon the percentage of BMI in excess of the 95th percentile (BMI95pct) has been proposed as an improved metric to better capture variability in weight at extreme ends of growth curves, which may improve our understanding of relationships between weight status and changes in clinical …

The Prevalence Of Hyperpalatable Baby Foods And Exposure During Infancy: A Preliminary Investigation., Kai Ling Kong, Tera L. Fazzino, Kaitlyn M. Rohde, Katherine S. Morris

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Objective: To characterize the prevalence of hyperpalatable foods (HPF) among baby foods in the U.S. and examine the prevalence of HPF exposure and consumption from both baby food and adult food sources among infants aged 9-15 months.

Methods: A U.S. baby food database as well as baby foods from three 24-h dietary recalls of 147 infants were used to identify baby foods as HPF per previous publication. HPF exposure was defined as intake of any HPF during the 3-day measurement period. To determine the extent of HFP consumption, % kilocalorie (kcal) intake from HPF was characterized.

Results: Only 12% of …

Efficacy Of Weight Reduction On Pediatric Nonalcoholic Fatty Liver Disease: Opportunities To Improve Treatment Outcomes Through Pharmacotherapy., Chance S. Friesen, Chelsea Hosey-Cojocari, Sherwin S. Chan, Iván L. Csanaky, Jonathan B. Wagner, Brooke Sweeney, Alec Friesen, Jason D. Fraser, Valentina Shakhnovich

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Obesity is the single greatest risk factor for nonalcoholic fatty liver disease (NAFLD). Without intervention, most pediatric patients with NAFLD continue to gain excessive weight, making early, effective weight loss intervention key for disease treatment and prevention of NAFLD progression. Unfortunately, outside of a closely monitored research setting, which is not representative of the real world, lifestyle modification success for weight loss in children is low. Bariatric surgery, though effective, is invasive and can worsen NAFLD postoperatively. Thus, there is an evolving and underutilized role for pharmacotherapy in children, both for weight reduction and NAFLD management. In this perspective article, …

Hydronephrosis Classifications: Has Utd Overtaken Apd And Sfu? A Worldwide Survey., Santiago Vallasciani, Anna Bujons Tur, John Gatti, Marcos Machado, Christopher S. Cooper, Marie Klaire Farrugia, Huixia Zhou, Mohammed El Anbari, Pedro-José Lopez

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Objective: To collect baseline information on the ultrasonographic reporting preferences.

Method: A 13-multiple choice questionnaire was designed and distributed worldwide among pediatric urologists, pediatric surgeons, and urologists. The statistical analysis of the survey data consisted of 3 steps: a univariate analysis, a bivariate and a multivariate analysis.

Results: Three hundred eighty participants responded from all the continents. The bivariate analysis showed the significant differences in the geographical area, the years of experience and the volume of cases. Most of the physicians prefer the SFU and APD systems because of familiarity and simplicity (37 and 34%, respectively). Respondents noted that their …

Leveraging Institutional Support To Build An Integrated Multidisciplinary Care Model In Pediatric Inflammatory Bowel Disease., Jennifer Verrill Schurman, Craig A. Friesen

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While the biopsychosocial nature of inflammatory bowel disease (IBD) is now well accepted by clinicians, the need for integrated multidisciplinary care is not always clear to institutional administrators who serve as decision makers regarding resources provided to clinical programs. In this commentary, we draw on our own experience in building successful integrated care models within a division of pediatric gastroenterology (GI) to highlight key considerations in garnering initial approval, as well as methods to maintain institutional support over time. Specifically, we discuss the importance of making a strong case for the inclusion of a psychologist in pediatric IBD care, justifying …

Delta-Like 4 Is Required For Pulmonary Vascular Arborization And Alveolarization In The Developing Lung., Sheng Xia, Heather Menden, Nick Townley, Sherry M. Mabry, Jeffrey J. Johnston, Michael F. Nyp, Daniel P. Heruth, Thomas Korfhagen, Venkatesh Sampath

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The molecular mechanisms by which endothelial cells (ECs) regulate pulmonary vascularization and contribute to alveolar epithelial cell development during lung morphogenesis remain unknown. We tested the hypothesis that delta-like 4 (DLL4), an EC Notch ligand, is critical for alveolarization by combining lung mapping and functional studies in human tissue and DLL4-haploinsufficient mice (Dll4+/lacz). DLL4 expressed in a PECAM-restricted manner in capillaries, arteries, and the alveolar septum from the canalicular to alveolar stage in mice and humans. Dll4 haploinsufficiency resulted in exuberant, nondirectional vascular patterning at E17.5 and P6, followed by smaller capillaries and fewer intermediate blood vessels at P14. Vascular …

Heartburn In Children And Adolescents In The Presence Of Functional Dyspepsia And/Or Irritable Bowel Syndrome Correlates With The Presence Of Sleep Disturbances, Anxiety, And Depression., Jennifer Colombo, Amanda D. Deacy, Jennifer Verrill Schurman, Craig A. Friesen

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ABSTRACT: The aim of this study was to assess the relationship of heartburn in pediatric patients with functional dyspepsia (FD) and irritable bowel syndrome (IBS) with gastrointestinal symptoms, sleep disturbances, and psychologic distress.The overlap in symptoms of FD, IBS, and gastroesophageal reflux disease (GERD) predicts greater symptom severity and decreased quality of life and presents opportunities for improved diagnostic classification and personalized therapeutics.A cross-sectional observational study of 260 pediatric patients with abdominal pain was conducted. Patients completed standardized questionnaires assessing clinical symptoms, sleep quality, and psychologic symptoms during routine clinical care. Questionnaire data were compared for patients reporting heartburn and …

Reliability Of The Cutting Alignment Scoring Tool (Cast) To Assess Trunk And Limb Alignment During A 45-Degree Side-Step Cut., Lauren S. Butler, Eryn K. Milian, Amie Deverna, Kevin Latz, Henry B. Ellis, Alexa R. Martinez, Kristin Hayden, Christian Gerstenkorn, Sara C. Carpenito, Charles W. Wyatt, Dai Sugimoto

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Background: Three-dimensional (3D) motion analysis is considered the gold standard for evaluating human movement. However, its clinical utility is limited due to cost, operating expertise, and lengthy data processing time. Numerous qualitative scoring systems have been introduced to assess trunk and lower extremity biomechanics during functional tasks. However, the reliability of qualitative scoring systems to evaluate cutting movements is understudied. Purpose/Hypotheses: To assess the inter-rater and intra-rater reliability of the Cutting Alignment Scoring Tool (CAST) among sports medicine providers and to evaluate rater agreement of each component of the CAST. The hypotheses were: 1) there would be good-to-excellent inter-rater and …

Comorbidities And Complications In Adults With Pyruvate Kinase Deficiency., Audra N. Boscoe, Yan Yan, Elizabeth Hedgeman, Eduard J. Van Beers, Hanny Al-Samkari, Wilma Barcellini, Stefan W. Eber, Bertil Glader, Hassan M. Yaish, Satheesh Chonat, Mukta Sharma, Kevin H M Kuo, Ellis J. Neufeld, Heng Wang, Madeleine Verhovsek, Sujit Sheth, Rachael F. Grace

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OBJECTIVES: Pyruvate kinase (PK) deficiency is caused by PKLR gene mutations, leading to defective red blood cell glycolysis and hemolytic anemia. Rates of comorbidities and complications by transfusion history and relative to the general population remain poorly quantified.

METHODS: Data for patients aged ≥ 18 years with two confirmed PKLR mutations were obtained from the PK deficiency Natural History Study (NCT02053480). Frequencies of select conditions were compared with an age- and sex-matched cohort from a general insured US population without PK deficiency.

RESULTS: Compared with the matched population (n = 1220), patients with PK deficiency (n = 122) had significantly …

Mechanical Support Of Superior Cavopulmonary (Glenn) Physiology To Heart Transplantation, Edo K S Bedzra, Aliessa P. Barnes, Brian Birnbaum, James D. St. Louis

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No abstract provided.

Quality Outcomes For Pediatric Colorectal Surgery Treated During Short-Term International Medical Service Trips At A Dedicated Site In Honduras., Wilfried Krois, Rebecca M. Rentea, Pastora X. Hernandez, Juan Craniotis-Rios, Richard J. Wood, Marc A. Levitt, Carlos A. Reck-Burneo

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INTRODUCTION: Short-term international medical service trips (MSTs) provide specialized care in resource-constrained countries. There are limited data on immediate and long-term reported outcomes following specialty MST. We hypothesized that dedicated collaborative MST team and host institution produce outcomes and results comparable to those of high-income settings. Our primary aim was to analyze the long-term surgical and functional outcomes of our specialty-specific MSTs following five years of annual MST in Honduras.

METHODS: We performed a single-institution retrospective analysis of 56 children who underwent colorectal and pelvic reconstructive operations between 2014 and 2018. Demographics, diagnosis, comorbidities, type of repair, long-term complications, and …

Assessment Of Nutritional Status In Children With Kidney Diseases-Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., Christina L. Nelms, Vanessa Shaw, Larry A. Greenbaum, Caroline Anderson, An Desloovere, Dieter Haffner, Michiel J S Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, Lesley Rees, José Renken-Terhaerdt, Jetta Tuokkola, Johan Vande Walle, Rukshana Shroff, Bradley A. Warady

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In children with kidney diseases, an assessment of the child's growth and nutritional status is important to guide the dietary prescription. No single metric can comprehensively describe the nutrition status; therefore, a series of indices and tools are required for evaluation. The Pediatric Renal Nutrition Taskforce (PRNT) is an international team of pediatric renal dietitians and pediatric nephrologists who develop clinical practice recommendations (CPRs) for the nutritional management of children with kidney diseases. Herein, we present CPRs for nutritional assessment, including measurement of anthropometric and biochemical parameters and evaluation of dietary intake. The statements have been graded using the American …

Assessment Of Down Syndrome-Associated Arthritis: A Survey Of Down Syndrome Clinic Providers., Jordan T. Jones, Chelsey Smith, Nasreen Talib

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No abstract provided.

Exploring Pediatric Tele-Rheumatology Practices During Covid-19: A Survey Of The Prcoin Network., Y Ingrid Goh, Danielle R. Bullock, Janalee Taylor, Rajdeep Pooni, Tzielan C. Lee, Sheetal S. Vora, Cagri Yildirim-Toruner, Esi M. Morgan, Nancy Pan, Julia G. Harris, Andrew Warmin, Kendra Wiegand, Jon M. Burnham, Fatima Barbar-Smiley

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Healthcare providers were rapidly forced to modify the way they practiced medicine during the coronavirus disease 2019 (COVID-19) pandemic. Many providers transitioned from seeing their patients in person to virtually using telemedicine platforms with limited training and experience using this medium. In pediatric rheumatology, this was further complicated as musculoskeletal exams typically require hands-on assessment of patients. The objective of this study was to examine the adoption of telemedicine into pediatric rheumatology practices, to assess its benefits and challenges, and to gather opinions on its continued use. A survey was sent to the lead representatives of each Pediatric Rheumatology Care …

Relationships Between Disaccharidase Deficiencies, Duodenal Inflammation And Symptom Profile In Children With Abdominal Pain., Jennifer Colombo, Chance S. Friesen, Uttam Garg, Craig A. Friesen, William San Pablo

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Abdominal pain has been associated with disaccharidase deficiencies. While relationships with individual symptoms have been assessed, relationships between disaccharidase deficiencies and symptom complexes or inflammation have not been evaluated in this group. The primary aims of the current study were to assess relationships between disaccharidase deficiency and symptoms or symptom complexes and duodenal inflammation, respectively. Patients with abdominal pain who underwent endoscopy with evaluation of disaccharidase activity levels were identified. After excluding all patients with inflammatory bowel disease, celiac disease, H. pylori, or gross endoscopic lesions, patients were evaluated for disaccharidase deficiency frequency. Disaccharidase were compared between patients with and …

Ispd Guidelines For Peritoneal Dialysis In Acute Kidney Injury: 2020 Update (Paediatrics)., Peter Nourse, Brett Cullis, Fredrick Finkelstein, Alp Numanoglu, Bradley A. Warady, Sampson Antwi, Mignon Mcculloch

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1.1 Peritoneal dialysis is a suitable renal replacement therapy modality for treatment of acute kidney injury in children. (1C)2. Access and fluid delivery for acute PD in children.2.1 We recommend a Tenckhoff catheter inserted by a surgeon in the operating theatre as the optimal choice for PD access. (1B) (optimal)2.2 Insertion of a PD catheter with an insertion kit and using Seldinger technique is an acceptable alternative. (1C) (optimal)2.3 Interventional radiological placement of PD catheters combining ultrasound and fluoroscopy is an acceptable alternative. (1D) (optimal)2.4 Rigid …

Effect Of Crohn's Disease On Villous Length And Cyp3a4 Expression In The Pediatric Small Intestine., Carrie A. Vyhlidal, Brian D. Chapron, Atif Ahmed, Vivekanand Singh, Rebecca Casini, Valentina Shakhnovich

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Changes in absorptive capacity and first-pass metabolism in the small intestine affect oral drug bioavailability. Characterization of such changes as a consequence of inflammation is important for developing physiologically-based pharmacokinetic (PBPK) models for inflammatory bowel disease. We sought to elucidate the impact of small intestinal Crohn's disease (CD) on villous length and CYP3A4 expression in children. Freshly frozen duodenal and terminal ileum (TI) biopsies from 107 children (1-19 years) with and without CD were evaluated for active inflammation. Villous length and CYP3A4 mRNA/protein expression were compared among regions of active and inactive inflammation in CD and controls. A twofold reduction …

Adolescent Access To Firearms-This Is Definitely Our Lane., Shayla Sullivant, M Denise Dowd

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No abstract provided.

Readiness To Change And Prospective Effects Of Weight Management Programs In Pediatric Nonalcoholic Fatty Liver Disease., Voytek Slowik, Heather Wasserkrug, Ryan T. Fischer, Mark Connelly, Amanda D. Deacy, Sarah Hampl, James F. Daniel

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Non-alcoholic fatty liver disease (NAFLD) is an increasing problem in pediatrics with limited treatment options. We prospectively assessed outcomes in patients managed in a hepatology clinic (HC) alone vs. those managed in combination with a multidisciplinary weight management program (MWMP). We describe each group's readiness to change at the time of NAFLD diagnosis. Patients diagnosed with NAFLD were given a modified Stages of Change Readiness and Treatment Eagerness Scale (SOCRATES) at enrollment (T1) to assess readiness to change. They were then followed at 3-9 months (T2) and at 10-15 months (T3). Linear mixed models were used to evaluate changes in …

The Efficacy And Tolerability Of Auto-Stimulation-Vns In Children With Lennox-Gastaut Syndrome., Sherouk A. Abdelmoity, Ayman Abdelmoity, Sean M. Riordan, Christian Kaufman, Jean-Baptist Lepichon, Ahmed Abdelmoity

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OBJECTIVE: Lennox-Gastaut syndrome (LGS) is a severe drug-resistant epilepsy (DRE) of childhood. The Vagus Nerve Stimulator (VNS) is established as a safe and effective treatment for DRE. This study assesses efficacy and tolerability of the auto-stimulation VNS models in pediatric patients with LGS.

METHODS: This is a retrospective chart review of a cohort of pediatric patients (Age 1-18 years old) with LGS implanted with an auto-stimulation VNS model at a single level four pediatric epilepsy center. Patient responder's rate was measured as seizure reduction over baseline and improvements in five quality-of-life measures as reported by the patients and families. Efficacy …

Ikaneat: Protocol For A Randomized Controlled Trial Of Megestrol As A Component Of A Pediatric Tube Weaning Protocol., Sarah T. Edwards, Paul E. Hyman, Hayat Mousa, Amanda Bruce, Jose Cocjin, Kelsey Dean, Kandace Fleming, Rebecca Swinburne Romine, Ann M. Davis

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BACKGROUND: Although tube feeding routinely saves the lives of children who do not eat by mouth, chronic tube feeding can be a burden to patients, caregivers, and families. Very few randomized trials exist regarding the best methods for weaning children from their feeding tubes.

METHODS: The current paper describes a randomized controlled trial of an empirically supported outpatient treatment protocol for moving children from tube to oral eating called iKanEat. Specifically, we describe the methods of randomized double-blind, placebo-controlled trial which includes a 4-week course of megestrol, the only medication used in the iKanEat protocol, to determine whether the addition …

Sirolimus Therapy And Follow-Up In A Patient With Severe Congenital Hyperinsulinism Following Subtotal Pancreatectomy, Qiong Chen, Yongxing Chen, Xiaohong Wang, Haihua Yang, Yingxian Zhang, Xiaojing Liu, Yun Yan, Haiyan Wei

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Congenital hyperinsulinism (CHI) is the most common cause of severe, persistent hypoglycemia in neonates and infants. If the patient does not respond to medical treatment the currently available treatment is subtotal pancreatectomy, but some patients still experience severe hypoglycemia after surgery. Sirolimus, a mammalian target of rapamycin inhibitor has recently been reported to be effective in the treatment of insulinoma and CHI patients. Here we report a patient with CHI who had prolonged hypoglycemia after subtotal pancreatectomy. The patient had a heterozygous mutation in ABCC8 but was unresponsive to an optimal dose of diazoxide (15 mg/ kg/day) and octreotide (30 …