97 results match your criteria: "Chang Gung Children's Hospital and Chang Gung Memorial Hospital[Affiliation]"

Dravet-like syndrome with PCDH19 mutations in Taiwan - A multicenter study.

Pediatr Neonatol

August 2024

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Taoyuan, Taiwan; Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address:

Article Synopsis
  • Protocadherin-19 (PCDH19) epilepsy is a rare condition primarily affecting females, characterized by early-onset seizures and developmental delays due to mutations in the PCDH19 gene on the X chromosome.
  • A study of fifteen female patients in Taiwan revealed that seizures typically began between 4 months and 2 years of age, often occurring in clusters, with varying levels of intellectual disability among the patients.
  • The research identified distinct mutations in the PCDH19 gene and highlighted the use of multiple antiseizure medications, with some patients achieving seizure freedom, but no specific medication class proven most effective for this syndrome.
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Background: SARS-CoV-2 posed a threat to children during the early phase of Omicron wave because many patients presented with febrile seizures. The study aimed to investigate predicting factors for acute encephalopathy of children infected by SARS-CoV-2 Omicron variant presenting with febrile seizures.

Methods: The retrospective study analyzed data from pediatric patients who visited the emergency department of Chang Gung Memorial Hospital in Taiwan between April and July 2022.

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Ferritin as an Effective Predictor of Neurological Outcomes in Children With Acute Necrotizing Encephalopathy.

Pediatr Neurol

March 2024

Division of Pediatric Critical Care Medicine, Department of Pediatrics, Chang Gung Memorial Hospital at Linko, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address:

Background: Acute necrotizing encephalopathy (ANE) is a fulminant disease with poor prognosis. Cytokine storm is the important phenomenon of ANE that affects the brain and multiple organs. The study aimed to identify whether hyperferritinemia was associated with poor prognosis in patients with ANE.

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Risk factors of Omicron variant associated acute encephalitis/encephalopathy in children.

J Microbiol Immunol Infect

December 2023

Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, Taoyuan, Taiwan; Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address:

Background: Outbreak of Omicron BA.2 in Taiwan led to an increased number of acute encephalitis/encephalopathy cases in children and several fatal cases drew public attention. In pre-Omicron period, pediatric cases of COVID-19-associated acute encephalitis have been reported and during Omicron epidemic, febrile convulsions, encephalitis were mentioned more frequently.

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Introduction: Sjogren's syndrome is an autoimmune disease that commonly involves exocrinopathy. Although studies have reported psychiatric manifestations resulting from Sjogren's syndrome, few studies have focused on such manifestations in pediatric patients. Herein, we present a case of an adolescent girl with depression and involuntary self-harm behaviors related to Sjogren's syndrome with central nervous system involvement.

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Automatic and human level Graf's type identification for detecting developmental dysplasia of the hip.

Biomed J

April 2024

Department of Orthopedic Surgery, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; Bone and Joint Research Center, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address:

Background: Developmental dysplasia of the hip (DDH) is a common congenital disorder that may lead to hip dislocation and requires surgical intervention if left untreated. Ultrasonography is the preferred method for DDH screening; however, the lack of experienced operators impedes its application in universal neonatal screening.

Methods: We developed a deep neural network tool to automatically register the five keypoints that mark important anatomical structures of the hip and provide a reference for measuring alpha and beta angles following Graf's guidelines, which is an ultrasound classification system for DDH in infants.

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MRI of fatal course of acute hemorrhagic leukoencephalitis in a child with SARS-CoV-2 omicron BA 2.0 infection.

Neuroradiology

July 2023

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.

We present a pediatric case of acute hemorrhagic leukoencephalitis associated with SARS-CoV-2 Omicron BA 2.0 infection. A previously healthy girl presented with ataxia and diplopia three weeks after the COVID-19 confirmation from a nasopharyngeal swab.

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Article Synopsis
  • The study focuses on the impact of anti-glutamic acid decarboxylase (anti-GAD) antibodies in children with encephalitis/encephalopathy, highlighting the limited research on their clinical relevance.
  • A total of 37 hospitalized children were analyzed and categorized into high-titer and low-titer groups based on their anti-GAD antibody levels.
  • High titers were linked to initial symptoms like language difficulties and ataxia, but the antibody levels did not correlate with overall severity or patient outcomes; immunotherapy led to fluctuating antibody levels and variable clinical responses.*
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Article Synopsis
  • BFPP is a rare genetic disorder linked to the ADGRG1 gene, with a specific missense variant (p.Leu290Pro) causing altered protein function.
  • The study examined three siblings with BFPP, reviewing their medical histories, EEG recordings, and treatment options that included medication and surgery.
  • Significant EEG findings included high-amplitude rhythmic activity during infancy, which diminished with age, and the treatments had varying degrees of success in managing seizures.
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Electrographic Seizures in Neonates with a High Risk of Encephalopathy.

Children (Basel)

May 2022

Study Group for Intensive and Integrated Care of Pediatric Central Nervous System, Department of Pediatrics (iCNS Study Group), Chang Gung Memorial Hospital, Taoyuan 333, Taiwan.

Background: Neonatal encephalopathy is caused by a wide variety of acute brain insults in newborns and presents with a spectrum of neurologic dysfunction, such as consciousness disturbance, seizures, and coma. The increased excitability in the neonatal brain appears to be highly susceptible to seizures after a variety of insults, and seizures may be the first clinical sign of a serious neurologic disorder. Subtle seizures are common in the neonatal period, and abnormal clinical paroxysmal events may raise the suspicion of neonatal seizures.

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The Frequency of Clinical Seizures in Paroxysmal Events in a Neonatal Intensive Care Unit.

Children (Basel)

February 2022

Study Group for Intensive and Integrated Care of Pediatric Central Nervous System, Chang Gung Children's Hospital, Taoyuan 333, Taiwan.

Background: In general clinical practice, neonatal seizures are identified visually by direct clinical observation. The study aimed to examine the frequency of clinical seizures in paroxysmal events in a neonatal intensive care unit.

Methods: We conducted a prospective study of continuous video-EEG monitoring in a neonatal intensive care unit between January 2017 and December 2020.

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Background: Transcranial Doppler ultrasound is a sensitive, real time tool used for monitoring cerebral blood flow; it could provide additional information for cerebral perfusion in cerebral resuscitation during post cardiac arrest care. The aim of the current study was to evaluate the utility of a point-of-care transcranial Doppler ultrasound management algorithm on outcomes in pediatric asphyxial out-of-hospital cardiac arrest.

Methods: This retrospective cohort study was conducted in two tertiary pediatric intensive care units between January 2013 and June 2018.

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Background: Patients with epilepsy have a higher mortality rate than the general population. Up-to-date estimates of epilepsy incidence, prevalence, and medication use are critical to assist policymaking.

Methods: Using the National Taiwan Insurance Research Database, the standardized incidence and prevalence of epilepsy were estimated in each calendar year from 2007 to 2015.

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Article Synopsis
  • Dravet syndrome (DS) is a rare epilepsy disorder that significantly impacts both patients and their caregivers, yet there is a lack of reliable measures to assess this impact in Taiwan.
  • A cross-sectional survey conducted in Taiwan revealed that while seizure frequency tends to decrease with age, caregivers face major challenges such as increased household responsibilities, financial strain, and the need for constant care.
  • Caregivers reported significant concerns about independence, seizure control, and the effects on siblings, highlighting the need for greater awareness and support for families affected by DS.
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Genetics and clinical correlation of Dravet syndrome and its mimics - experience of a tertiary center in Taiwan.

Pediatr Neonatol

September 2021

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address:

Article Synopsis
  • Dravet syndrome is a serious condition marked by severe seizures starting in infancy, often related to mutations in the SCN1A gene, though similar symptoms can arise from other gene mutations.
  • In a study of 36 patients, about 41.7% had SCN1A mutations, and those patients typically experienced earlier and more severe seizure activity compared to those with different mutations, particularly PCDH19.
  • The average time taken to get a genetic diagnosis was quite long, around 6.6 years, indicating a need for improved awareness and testing for Dravet syndrome and its variants.
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Measurement of optic nerve sheath diameter by ultrasound in healthy term neonates.

Pediatr Neonatol

November 2021

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address:

Background: Optic nerve sheath diameter (ONSD) ultrasound is a noninvasive and repeatable tool to dynamically evaluate intracranial pressure with high diagnostic accuracy; however, data in neonates are scarce. The aim of this study was to determine the reference value of ONSD and potential influencing factors in healthy term neonates.

Methods: We retrospectively reviewed 250 full-term neonates who underwent cranial ultrasound as part of selective newborn screening over a 2-year period.

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High-flow nasal cannulas (HFNCs) show potential in the application of positive pressure, improving gas exchange, and decreasing work of breathing in patients with acute respiratory distress. The aims of this study were to elucidate the indications for HFNC therapy in children of all ages and diagnoses, and to evaluate the efficacy and risk factors for failure of HFNC therapy in children with acute respiratory distress with hypoxia in a pediatric intensive care unit. We conducted this retrospective cohort study at a tertiary pediatric intensive care unit between January 1, 2018 and December 31, 2020.

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Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan.

Pediatr Neonatol

March 2021

Division of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan. Electronic address:

Background: Craniopharyngiomas are benign tumors of embryologic origin located in the sellar region. Patients have both neurological and endocrinological symptoms. Symptoms may be subtle in the early clinical course, which leads to delayed diagnosis.

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Developmental and epileptic encephalopathies: Is prognosis related to different epileptic network dysfunctions?

Epilepsy Behav

June 2022

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Study Group for Intensive and Integrated Care of Pediatric Central Nervous System (iCNS Group), Chang Gung Children's Hospital, Taoyuan, Taiwan. Electronic address:

Developmental and epileptic encephalopathies are a group of rare, severe epilepsies, which are characterized by refractory seizures starting in infancy or childhood and developmental delay or regression. Developmental changes might be independent of epilepsy. However, interictal epileptic activity and seizures can further deteriorate cognition and behavior.

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Factors Associated With In-hospital Mortality of Children With Acute Fulminant Myocarditis on Extracorporeal Membrane Oxygenation.

Front Pediatr

August 2020

Division of Pediatric Critical Care Medicine, and Pediatric Sepsis Study Group, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.

To analyze the factors associated with in-hospital mortality of children with acute fulminant myocarditis on venoarterial extracorporeal membrane oxygenation (VA-ECMO). This was a retrospective cohort study using chart reviews of patients diagnosed with acute fulminant myocarditis at the pediatric intensive care unit of two tertiary medical centers between January 1, 2005 and December 31, 2017. The inclusion criteria for this study were: (1) age from 1 month to 18 years; (2) diagnosed with acute myocarditis; (3) cardiogenic shock and need vasoactive-inotropic score ≥20 within 48 h after the use of vasoactive-inotropic agents; and (4) the need for ECMO placement.

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The aim of this study was to determine the frequency, timing, and predictors of rewarming seizures in a cohort of children undergoing therapeutic hypothermia after resuscitation. We retrospectively reviewed consecutive pediatric patients undergoing therapeutic hypothermia after resuscitation admitted to our pediatric intensive care unit between January 2000 and December 2019. Continuous electroencephalographic monitoring was performed during hypothermia (24 h for cardiac aetiologies and 72 h for asphyxial aetiologies), rewarming (72 h), and then an additional 12 h of normothermia.

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Valproic acid is the most high-risk teratogenic antiepileptic drug, and it may lead to fetal major congenital malformations. However, it is still used in women of childbearing age with epilepsy. The aim of this study was to report our experience of discontinuing or lowering valproic acid by adding levetiracetam, a low-risk teratogenic antiepileptic drug.

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The aim of the current study was to identify early clinical predictors of neurologic outcome in children with asphyxial out-of-hospital cardiac arrest (OHCA) treated with therapeutic hypothermia. The present retrospective cohort study of comatose children treated with therapeutic hypothermia or normothermia after asphyxial OHCA was conducted between January 2010 and June 2018. All children aged between 1 month and 18 years of age, with a history of at least 3 min of chest compressions were eligible for inclusion.

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Purpose: The role of methylprednisolone pulse therapy as adjuvant treatment of Streptococcus pneumoniae meningitis complicated by cerebral infarction has rarely been reported.

Methods: We reported a case report and also performed a systematic literature review.

Results: A 1-year 2-month-old boy who presented with high fever, status epilepticus, and septic shock was diagnosed with cerebral infarction caused by Streptococcus pneumoniae meningitis on magnetic resonance imaging (MRI).

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