Arrhythmic Risk and Clinical Features in Catecholaminergic Polymorphic Ventricular Tachycardia: Results From a Multicenter Study in Korea.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but life-threatening inherited arrhythmia in children, often associated with sudden cardiac death (SCD). Risk stratification remains challenging. This study aimed to assess clinical characteristics, genetic profiles, and risk factors for adverse outcomes in Korean pediatric CPVT patients.

Late development of intra-atrial reentrant tachycardia in lateral tunnel Fontan patients and the preventive role of prophylactic cryoablation.

Objective: Intra-atrial reentrant tachycardia is an important late-onset complication in patients undergoing the Fontan procedure. However, the protective effects of prophylactic cryoablation against late-onset intra-atrial reentrant tachycardia are unclear. This study investigated the late development of intra-atrial reentrant tachycardia in patients undergoing the lateral tunnel Fontan procedure and the role of prophylactic cryoablation.

Severe protein C deficiency in a newborn caused by a homozygous pathogenic variant in the PROC gene: a case report.

Background: Severe protein C deficiency is a rare and inherited cause of thrombophilia in neonates. Protein C acts as an anticoagulant, and its deficiency results in vascular thrombosis. Herein, we report a case of protein C deficiency with a homozygous pathogenic variant in a term neonate, with good outcomes after proper treatment.

Lacrimo-auriculo-dento-digital syndrome: A novel mutation in a Korean family and review of literature.

Background: Lacrimo-auriculo-dento-digital (LADD) syndrome is a rare autosomal dominant disorder caused by mutations in one of the three genes: fibroblast growth factor receptor 2 (FGFR2), FGFR3, or FGF10. Affected patients have hypoplasia/aplasia of lacrimal ducts/glands, hypoplasia/aplasia of salivary glands, dental anomalies, ear anomalies, hearing loss, and digital anomalies.

Case Presentation: Proband was an 11-year-old male with xerostomia, xerophthalmia, and a referring diagnosis of Sjogren syndrome.

The Associations between Antenatal Corticosteroids and In-Hospital Outcomes of Preterm Singleton Appropriate for Gestational Age Neonates according to the Presence of Maternal Histologic Chorioamnionitis.

Introduction: This study aimed to assess the association between antenatal corticosteroid (ACS) and in-hospital outcomes of preterm singleton appropriate for gestational age (AGA) infants according to the presence of maternal histologic chorioamnionitis (HCA).

Methods: A retrospective study was performed with singleton AGA neonates of 23+0 to 33+6 weeks' gestation born between 2007 and 2014. We compared the clinical outcomes according to the presence of HCA and ACS use.