Severe neonatal Marfan syndrome with a novel mutation in the intron of the FBN1 gene: A case report.

Rationale: Marfan syndrome (MFS) has been defined as a genetic disorder that affects various systems such as the musculoskeletal, orbital, and cardiovascular systems. Neonatal MFS is considered rare and the most severe form of MFS is characterized by rapidly progressive atrioventricular valve dysfunction, often leading to death during early childhood due to congestive heart failure.

Patient Concerns: A newborn with neonatal MFS and severe cardiac involvement.

Clinical Implications of Fragmented QRS Complex as an Outcome Predictor in Children with Idiopathic Dilated Cardiomyopathy.

Fragmented QRS (fQRS) is an easily evaluated noninvasive parameter in electrocardiograms (ECGs) for predicting cardiac adverse events. Our study aimed to evaluate whether fQRS could be used as a risk factor to predict a major adverse cardiac event (MACE) in children with idiopathic dilated cardiomyopathy (DCM). Our retrospective study enrolled 63 patients aged ≤ 19 years who were newly diagnosed with idiopathic DCM at Samsung Medical Center from 2003 to 2014, and followed up until December 2018.

Effects of low-dose topiramate on language function in children with migraine.

Purpose: This study aimed to verify the safety of low-dose topiramate on language development in pediatric patients with migraine.

Methods: Thirty newly diagnosed pediatric patients with migraine who needed topiramate were enrolled and assessed twice with standard language tests, including the Test of Language Problem Solving Abilities (TOPs), Receptive and Expressive Vocabulary Test, Urimal Test of Articulation and Phonology, and computerized speech laboratory analysis. Data were collected before treatment, and topiramate as monotherapy was sustained for at least 3 months.

Left ventricular hypertrophy and diastolic function in children and adolescents with essential hypertension.

Introduction: Left ventricular hypertrophy and diastolic dysfunction in children and adolescents with essential hypertension tend to be underdiagnosed. The aims of this study were to investigate left ventricular hypertrophy and diastolic dysfunction in the subjects with essential hypertension defined by ambulatory blood pressure monitoring.

Methods: A total of 38 Korean subjects aged 9-19 years without secondary causes of hypertension were reviewed.