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Lucile Salter Packard Children's Hospit... Publications | LitMetric

196 results match your criteria: "Lucile Salter Packard Children's Hospital[Affiliation]"

Neonatal G6PD deficiency (G6PDd) prevalence explains its recent recognition as a major contributory cause of extreme hyperbilirubinemia (EHB). Disparate global EHB burden reveals comparative racial prevalence of leading conditions, Rh negativity and G6PDd: 15-17% vs. < 5% in Caucasians, 4 to 8% vs.

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Newborn screening for biliary atresia using direct bilirubin: An implementation science study.

J Med Screen

September 2024

Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, UT Health San Antonio, San Antonio, TX, USA.

Article Synopsis
  • Biliary atresia (BA) is a serious liver condition in infants that requires early detection for better outcomes, with the ideal surgical intervention happening before 45 days of life, but the average age for surgery in this hospital was 60 days.
  • To improve early diagnosis, a new two-stage screening strategy was established that tested newborns for direct bilirubin levels shortly after birth and again at two weeks for those with elevated levels.
  • Over 16 months, 99.5% of infants were screened, identifying a small percentage with concerning bilirubin levels, leading to timely evaluations by gastroenterology and effectively ruling out BA by 28 days of life for most infants.
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Managing the Historic Burden of Kernicterus Mortality in India.

Indian J Pediatr

December 2024

Stanford University School of Medicine, Lucile Salter Packard Children's Hospital, Stanford, CA, 94305, USA.

Prevention of neonatal bilirubin injury exemplifies success of systems approach to avert adverse neonatal and childhood outcomes that rely on strategies including prenatal identification of Rhesus sensitization, universal maternal blood typing, risk assessment for neonatal extreme hyperbilirubinemia (EHB), unfettered access to safe, effective phototherapy, and application of patient safety principles. India's diverse landscape suggests varied real-time experiences of neonatal hyperbilirubinemia and consequent infant mortality rates (IMR). Utilizing Global Burden of Disease (GBD) database, the authors examined national and subnational trends, infant mortality timing, and the disease burden from hemolytic and perinatal jaundice over 30 y (1999 to 2019).

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Due to abnormal prenatal ultrasound findings of femoral shortening and flattened facial profile, a G2P0 pregnant patient underwent an amniocentesis at 15 weeks of gestation for proband-only exome sequencing. Bioinformatic filtering for genes included on the laboratory's extended skeletal dysplasia panel identified a heterozygous, likely pathogenic, frameshift variant in DVL1 NM_001330311.2:c.

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Predicting Stroke for Pediatric Patients Supported With Ventricular Assist Devices: A Pedimacs Report.

Ann Thorac Surg

October 2024

Division of Cardiovascular Surgery, Department of Surgery, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) provides detailed understanding on pediatric patients supported with ventricular assist devices (VADs). We sought to identify important variables affecting the incidence of stroke in pediatric VADs.

Methods: Between 2012 and 2022, 1463 devices in 1219 patients were reported to Pedimacs from 40 centers in patients aged <19 years at their first VAD implantation.

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Article Synopsis
  • Study Objective
  • : The study aimed to improve Tanzanian healthcare providers' knowledge of newborn care through an adaptive e-learning platform (aESNC) while evaluating its implementation success and assessing baseline provider knowledge.
  • Methodology
  • : Conducted over 6 months in Mwanza, Tanzania, the observational study measured the reach and effectiveness of aESNC using specific frameworks and models, while also examining factors influencing providers' completion of training.
  • Results
  • : The aESNC reached 85% of targeted providers, with a median clinical experience of 4 years. Providers showed 78% completion of initial learning, but 67% experienced inactivity. Higher baseline understanding and nursing officers were linked to better training completion.
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Foreign national patients and families can face life-limiting illness and end-of-life care far from home; this palliative need has not been well described. We present a case of a 20-year-old Ugandan patient diagnosed with metastatic alveolar rhabdomyosarcoma who presented to a pediatric academic medical center in California. Despite treatment, her disease progressed and she was unable to return to Uganda due to symptom burden.

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Background: Tube feeds are used commonly in children listed for heart transplant; however, rates of renourishment and development of feeding disorders are not sufficiently characterized.

Methods: Retrospective review of pediatric heart transplant recipients from January 1, 2014, to January 3, 2021. Demographics, anthropometric, and nutritional data were collected from heart transplant listing through 3 years post-transplant.

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Implementation of a Pilot Clinic for Pediatric to Adult Cancer Survivorship Transitions.

J Adolesc Young Adult Oncol

December 2023

Division of Pediatric Hematology, Oncology, Stem Cell Transplantation & Regenerative Medicine, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California, USA.

Childhood cancer survivors are recommended to have lifelong survivorship care, yet many become disengaged during pediatric to adult care transitions. We implemented a pilot clinic for adult survivors of pediatric or adolescent and young adult (AYA) leukemia transitioning to adult-focused survivorship care. The clinic featured AYA-specific care, bidirectional communication with primary care, and a quality improvement (QI) cycle.

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Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) provides detailed understanding on pediatric patients supported with ventricular assist devices (VADs). We sought to identify important variables affecting mortality in pediatric VADs.

Methods: Patients aged <19 years, from 2012 to 2021, were included.

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Background: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network.

Methods: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected.

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Objectives: Data on triage practices of children admitted to Princess Marina Hospital in Gaborone, Botswana is limited. The inpatient triage, assessment, and treatment score was developed for low resource settings to predict mortality in children. We assess its performance among children admitted to Princess Marina Hospital and their demographic, clinical, and risk factors for death.

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Background: Clinical trials represent a significant risk in the commercialization of surgical technologies. There is incentive for companies to mitigate their regulatory risk by targeting 510K over Premarket Approval (PMA) pathways in order to limit the scope, complexity and cost of clinical trials. As such, not all companies will publish clinical data in the scientific literature.

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Purpose Of Review: This review summarizes recent investigations into the cellular and molecular effects of skeletal aging on the inflammatory response and stem cell function after fracture.

Recent Findings: Proper regulation of the inflammatory phase of fracture healing is essential. Aging is associated with chronic inflammation, which inhibits bone formation and promotes bone resorption.

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Background: Nocardia infections are rare opportunistic infections in SOT recipients, with few reported pediatric cases. Pediatric patients with single ventricle congenital heart defects requiring HT may be more susceptible to opportunistic infections due to a decreased T-cell repertoire from early thymectomy and potential immunodeficiencies related to their congenital heart disease. Other risk factors in SOT recipients include the use of immunosuppressive medications and the development of persistent lymphopenia, delayed count recovery and/or lymphocyte dysfunction.

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Introduction/aims: Dysferlinopathy demonstrates heterogeneity in muscle weakness between patients, which can progress at different rates over time. Changing muscle strength due to disease progression or from an investigational product is associated with changing functional ability. The purpose of this study was to compare three methods of strength testing used in the Clinical Outcome Study (COS) for dysferlinopathy to understand which method and which muscle groups were most sensitive to change over time.

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Dysferlinopathy is a muscular dystrophy with a highly variable functional disease progression in which the relationship of function to some patient reported outcome measures (PROMs) has not been previously reported. This analysis aims to identify the suitability of PROMs and their association with motor performance.Two-hundred and four patients with dysferlinopathy were identified in the Jain Foundation's Clinical Outcome Study in Dysferlinopathy from 14 sites in 8 countries.

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Children on ventricular assist device (VAD) support can present several unique challenges, including small patient size, univentricular or biventricular congenital heart disease (1V- or 2V-CHD) and need for biventricular VAD (BiVAD) support. While cardiac catheterization can provide valuable information, it is an invasive procedure with inherent risks. We sought to evaluate the safety of catheterization in pediatric patients on VAD support.

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Background: Vegetable or plant-based sources of protein may confer health benefits in children with progressive kidney disease. Our aims were to understand the effect of the proportion of vegetable protein intake on changes in estimated GFR and to understand the effect of the proportion of vegetable protein intake on serum levels of bicarbonate, phosphorus, and potassium.

Methods: Children with baseline eGFR between 30 and 90 mL/min/1.

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Aim: The purpose of this study was to assess for an association between higher body mass index and disease severity, morbidity and mortality in children admitted for an acute respiratory distress and failure.

Methods: A single-institution retrospective cross-sectional study performed in the United States evaluating paediatric patients, 2-20 years of age, admitted for diagnoses related to acute respiratory distress and acute respiratory failure. Main outcomes include disease severity as assessed using the respiratory component of the Paediatric Early Warning Score (PEWS) with adjustment for altered mental status (Resp-PEWS + AMS), hospital or intensive care length of stay (LOS) and death.

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