12 results match your criteria: "Department of Pediatrics National Taiwan University Hospital[Affiliation]"

Aromatic l-amino acid decarboxylase (AADC) deficiency is a rare inherited disorder that affects neurotransmitter biosynthesis. A founder mutation c.714 + 4A > T (IVS6 + 4A > T) is prevalent in the Chinese population.

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Article Synopsis
  • Alagille syndrome (ALGS) is a complex disorder that varies in how it affects individuals, and this study looked at clinical outcomes and genetic factors in 25 children over 7.3 years.
  • About 36% of the children in the jaundice-free (JF) group had better health outcomes compared to those with progressive disease (PD), who had more serious issues and higher need for liver transplants.
  • The study found distinct genetic mutations in the ALGS patients and highlighted that they generally had less severe liver damage and more growth challenges compared to children with biliary atresia at the time of liver transplant.
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Background Protracted cerebral hypoperfusion following cardiac arrest (CA) may cause poor neurological recovery. We hypothesized that inhaled carbon dioxide (CO) could augment cerebral blood flow (CBF) and improve post-CA neurological outcomes. Methods and Results After 6-minute asphyxia-induced CA and resuscitation, Wistar rats were randomly allocated to 4 groups (n=25/group) and administered with different inhaled CO concentrations, including control (0% CO), 4% CO, 8% CO, and 12% CO.

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Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents.

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Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study.

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Background In most post-cardiac arrest patients, the autoregulation mechanism of cerebral blood flow (CBF) is dysregulated. We examined whether recovery of CBF by adjusting mean arterial pressure mitigates post-cardiac arrest neuronal damage. Methods and Results Wistar rats that underwent 8-minute asphyxia-induced cardiac arrest and resuscitation were computer-randomized to norepinephrine or control groups.

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Aim: To assess the clinical trial and real-world data for adjunctive perampanel in adolescents and develop consensus recommendations to guide the use of perampanel in this population in clinical practice.

Methods: In May 2015, 15 epilepsy experts attended a Consensus Development Meeting to assess the clinical trial data for perampanel, specific to the adolescent age group (12-17 years) and develop consensus treatment recommendations.

Results And Discussion: Analysis of the adolescent subgroup data of three pivotal placebo-controlled, double-blind, phase 3 trials investigating perampanel in patients with ongoing focal epileptic seizures despite receiving one to three antiepileptic drugs found that perampanel 4-12 mg was superior to placebo.

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To evaluate the current status of hepatitis A virus (HAV) infection, a seroepidemiologic study to detect the prevalence of antibodies against HAV (anti-HAV) has conducted in Taipei City, from March to July, 1989. Serum specimens were collected from 602 male and 503 female apparently healthy children age 12 and under. The prevalence rate of anti-HAV was 27.

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