246 results match your criteria: "Children's Specialized Hospital.[Affiliation]"

Status of vitamins and minerals in children with screening-identified celiac disease: A case-control study.

J Pediatr Gastroenterol Nutr

March 2024

Department of Biostatistics, Research Services Adminsitration, Research Center, King Fahad Medical City, Riyadh, Riyadh, Saudi Arabia.

Objectives: Micronutrient deficiencies characterize classical "late-diagnosed" celiac disease (CeD). This study aimed to identify the prevalence of micronutrient deficiencies among children with "early-diagnosed" screening-identified CeD to determine the clinical value of routine testing for deficiencies in those patients.

Methods: A case-control study was conducted on screening-identified CeD patients diagnosed during a mass screening study (84 patients, mean age 11.

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The purpose of the work - to investigate the peculiarities of the clinical course of Hirschsprung's disease in children of the first year of life and to determine the significance of symptoms in the verification of the disease. From 1980 to 2021, at the pediatric surgery clinic of the National Medical University named after O.O.

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Fitusiran, a subcutaneous investigational small interfering RNA therapeutic, targets antithrombin to rebalance hemostasis in people with hemophilia A or B (PwHA/B), irrespective of inhibitor status. This phase 3, open-label study evaluated the efficacy and safety of fitusiran prophylaxis in males aged ≥12 years with hemophilia A or B, with or without inhibitors, who received prior bypassing agent (BPA)/clotting factor concentrate (CFC) prophylaxis. Participants continued their prior BPA/CFC prophylaxis for 6 months before switching to once-monthly 80 mg fitusiran prophylaxis for 7 months (onset and efficacy periods).

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Resurgence is a temporary increase in a previously suppressed target behavior following a worsening in reinforcement conditions. Previous studies have examined how higher rates or magnitudes of alternative reinforcement affect suppression of the target behavior and subsequent resurgence. However, there has been no investigation of the effects of higher versus lower qualities of alternative reinforcement on resurgence.

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Carbonic Anhydrase II Deficiency: Unusual Presentation of the Arabic Mutation. A Case Report.

Glob Pediatr Health

February 2024

Department of Pediatrics, King Saud Medical City, Riyadh, Saudi Arabia.

Carbonic anhydrase II deficiency is an extremely rare inborn error of metabolism that constitutes a triad osteopetrosis, renal tubular acidosis and intracerebral calcification. Unlike other subtypes of osteopetrosis, the presence of developmental delay and relative infrequency of skeletal fractures may not be a typical signs of symptoms indolent trajectory. This case report demonstrates a 11-year-old boy who had a bilateral midshaft tibial fracture despite low mechanism of injury.

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The purpose of this review is to summarize recent literature on the use of concurrent-chains arrangements in the assessment of preference for interventions (or intervention components) in the applied literature. The types of interventions and participants are described briefly, and procedural variations, ethical considerations, and recommendations for future research are discussed.

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Objective: The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis.

Patients And Methods: Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022.

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Objective: The study evaluated whether pain intensity and extent, balance-confidence, functional mobility, and balance (eg, functional reach) are potential risk factors for recurrent falls among adults with a lower-limb amputation.

Design: Cross-sectional study.

Setting: Research laboratory.

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The literature offers few recommendations for sequencing exposure to treatment conditions with individuals with multiply maintained destructive behavior. Identifying relative preference for the functional reinforcers maintaining destructive behavior may be one means of guiding that decision. The present study presents a preliminary attempt at developing a robust relative preference and reinforcer assessment for individuals with multiply maintained destructive behavior.

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Objective: The COVID-19 pandemic disproportionately harmed Latino families; however, its effects on their stress and media routines remain understudied. We examined economic and parenting stress patterns during the COVID-19 pandemic and estimated associations between these forms of stress and nonadherence to American Academy of Pediatrics (AAP) infant media exposure recommendations among Latino families. We also explored how nonadherence with AAP recommendations varied with COVID-19 cases.

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Celiac Disease and Intussusception: A Common Association in Children.

Pediatr Emerg Care

June 2024

Department of Biostatistics, Research Services Administration, Research Center, King Fahad Medical City, Riyadh, Saudi Arabia.

Objectives: In young childhood, intestinal intussusception (IS) is the most common cause of small bowel obstruction. A lead point such as Meckel diverticulum, polyps, tumors, enlarged lymph nodes, cystic fibrosis, and Schoenlein-Henoch purpura are recognized causes. Association between celiac disease (CD) and IS has been well recognized in adults but rarely in children.

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Performance of deamidated gliadin peptide antibodies as first screening for celiac disease in the general pediatric population.

Front Pediatr

November 2023

Department of Medical Translational Sciences & European Laboratory for the Investigation of Food-Induced Diseases, University Federico II, Naples, Italy.

Background: Celiac serology has evolved, with the identification of newer antibodies against deamidated gliadin peptides (DGP) [e.g., anti-DGP, immunoglobulin A (IgA), and immunoglobulin G (IgG) types] with sensitivity and specificity in detecting celiac disease (CeD) that are equivalent to anti-tissue transglutaminase [anti-tissue transglutaminase (TTG) IgA]-based tests, particularly in populations with high pretest probability of CeD (prevalence of CeD > 50% of the population under study).

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Background: Reach Out and Read (ROR) is a multi-component pediatric literacy promotion intervention. However, few studies link ROR components to outcomes. We examine associations between receipt of (1) multiple ROR components and (2) clinician modeling, a potential best practice, with enhanced home literacy environments (EHLEs) among Latino families.

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Non-invasive prenatal testing (NIPT) is a pioneering technique that has consistently advanced the field of prenatal testing to detect genetic abnormalities and conditions with the aim of decreasing the incidence and prevalence of inherited conditions. NIPT remains a method of choice for common autosomal aneuploidies, mostly trisomy 21, and several monogenic disorders. The advancements in gene sequencing techniques have expanded the panel of conditions where NIPT could be offered.

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Background: The 2020-2029 strategic plan for the Patient-Centered Outcomes Research Trust Fund calls for addressing data infrastructure gaps that are critical for studying issues around intellectual and developmental disabilities (I/DD). Specifically, the plan calls for data collection on economic factors that affect person-centered approaches to health care decision-making. Among people with I/DD and their caregivers, such economic factors may include financial costs of care, decreased opportunities for leisure and recreation, income losses associated with caregiving, and foregone opportunities for skill acquisition or other human capital investments.

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Human MCTS1-dependent translation of JAK2 is essential for IFN-γ immunity to mycobacteria.

Cell

November 2023

Laboratory of Human Genetics of Infectious Diseases, INSERM UMR1163, Necker hospital for sick children, 75015 Paris, France; Paris Cité University, Imagine Institute, 75015 Paris, France; St. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, NY 10065, USA; Howard Hughes Medical Institute, New York, NY 10032, USA; Department of Pediatrics, Necker Hospital for Sick Children, AP-HP, 75015 Paris, France. Electronic address:

Article Synopsis
  • * This deficiency results in the impaired translation of important proteins like JAK2, affecting cellular responses to specific cytokines such as IL-23 and IL-12, particularly in T lymphocytes and phagocytes.
  • * The impaired response to IL-23 reduces the production of IFN-γ by certain immune cells during mycobacterial infections, highlighting a crucial link between MCTS1 deficiency and mycobacterial disease susceptibility.
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Peripheral neuropathy (PN) and peripheral arterial disease (PAD) are life-limiting comorbidities among adults with lower-limb loss that may not be adequately addressed in current care models. The objective of this study was to evaluate underreporting of PN and PAD among adults with lower-limb loss. We conducted a secondary analysis of a cross-sectional dataset of community-dwelling adults with unilateral lower-limb loss seen in an outpatient Limb Loss Clinic (n = 196; mean age = 56.

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Objectives: Connexin43 (Cx43) is involved in the inflammation of many tissue types. Dental caries is infectious disease resulting from mineralized tissue dissolution by a specific bacterial population, causing pulp inflammation. However, Cx43's role in dental pulp remains unclear.

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Predictors of biliary atresia outcome: Saudi National Study (2000 - 2018).

Saudi J Gastroenterol

November 2023

College of Medicine, Alfaisal University; Division of Pediatric Gastroenterology, Children's Specialized Hospital, Prince Abdullah Bin Khaled Celiac Disease Research Chair, Department of Pediatrics, Faculty of Medicine, King Saud University, Riyadh, Saudi Arabia.

Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. Here, we analyzed the data from the Saudi national BA study (204 BA cases diagnosed between 2000 and 2018) to identify the prognostic factors of BA outcomes.

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Background: We utilized the data from the Saudi national biliary atresia (BA) study (2000-2018) to describe the clinical, biochemical, imaging, and histopathological features of BA and the perioperative clinical practices among local pediatric gastroenterologists.

Methods: This is a retrospective, multicenter, nationwide study that included 10 tertiary care governmental hospitals including the four liver transplant (LT) centers in different regions across Saudi Arabia.

Results: BA was diagnosed in 204 infants (106 females; 10% preterm).

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Disease patterns among Saudi children undergoing colonoscopy for lower gastrointestinal bleeding: Single tertiary care center experience.

Saudi J Gastroenterol

November 2023

Division of Pediatric Gastroenterology, Children's Specialized Hospital, King Fahad Medical City, Riyadh; Alfaisal University; Prince Abdullah Bin Khaled Celiac Disease Research Chair, Department of Pediatrics, Faculty of Medicine, King Saud University, Saudi Arabia.

Article Synopsis
  • A study was conducted to assess the effectiveness of colonoscopy in diagnosing lower gastrointestinal bleeding (LGIB) in Saudi children over a decade, utilizing data from King Fahad Medical City.
  • Pediatric patients aged 0-14 years who underwent colonoscopy for LGIB were analyzed, revealing inflammatory bowel disease (IBD) as the most common cause (32%), with other conditions like colonic lymphonodular hyperplasia (CLNH) and juvenile polyps following.
  • The results indicated high diagnostic accuracy for colonoscopy, with a sensitivity of 97% and a specificity of 100%, highlighting its effectiveness in identifying the underlying causes of LGIB in this population.
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Cycling between the availability and unavailability of reinforcement for alternative responding has successfully reduced resurgence in basic laboratory evaluations, but this approach represents a marked departure from current standards of care when treating problem behavior, warranting careful translation before its use clinically. Therefore, with extinction arranged for target responding across groups in Phase 2, we evaluated the effects of cycling between the availability and unavailability of differential reinforcement of alternative behavior (DRA) using a computer-based task with adult humans recruited through Amazon MTurk. Two control groups experienced constant DRA in Phase 2, with one group experiencing a dense DRA schedule and another group experiencing a lean DRA schedule.

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Previous research has examined the predictive validity of heart rate on destructive behavior; however, such research has yet to improve clinical practice or enhance our understanding of the relation between physiology and destructive behavior. The purpose of this study was to examine the predictive validity of heart rate on varying topographies and functions of destructive behavior while controlling antecedent and consequent events through functional analysis. We first demonstrated the reliability of the Polar H10 heart rate monitor and assessed the feasibility of its use in simulated functional analysis sessions.

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Self-feeding with utensils represents an important step in a child's progression toward age-typical eating and emerges in the absence of intervention for most children. In contrast, children with feeding disorders may lack the skill or motivation to self-feed, which impedes progress toward age-typical eating. In the current study, experimenters used a multielement design to evaluate negative reinforcement in the form of meal termination to transition six participants with a feeding disorder from caregiver-fed to self-fed bites and drinks.

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Behavioral momentum theory (BMT) suggests that resurgence of destructive behavior may be at least partly determined by the rate of alternative reinforcement, with lean schedules of reinforcement producing less resurgence than dense schedules. Findings from basic and translational studies have been mixed, and the effects of alternative reinforcement rate on resurgence remain unclear. In the current study, we conducted a within-subject evaluation of resurgence during extinction with four children following functional communication training using dense and lean (BMT-informed) schedules of alternative reinforcement.

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