신제희 교수

Live immunization is contraindicated in patients with DiGeorge syndrome (DGS). We retrospectively investigated the occurrence of adverse events after live immunization in patients with DGS in Korea. The data of patients matching the International Classification of Disease-10 code of DGS (D82.1) at Severance Hospital Seoul, Korea, were extracted; patients without genetically diagnosed DGS were excluded. Based on T cell immunity status, the included patients were categorized into group A (CD3 < 50

BACKGROUND: Infections are considered risk factors for autoimmune inflammatory rheumatic diseases (AIRDs), the incidence of which is considered to have been impacted by the COVID-19 pandemic. The impact of non-pharmaceutical interventions (NPIs) on the incidence of AIRDs and their associated health care services and medical expenses in Korea was investigated. METHODS: We included all AIRD cases reported between January 2016 and February 2021 based on the National Health Insurance Service data. W

Abstract Background Non-typhoidal salmonella (NTS) infections usually have a self-limiting course, but can cause invasive NTS (iNTS) diseases, including bacteremia, meningitis, osteomyelitis or other focal infection. However, information on NTS infections in children is scarce in Asian countries. We investigated the differences in clinical features and antimicrobial susceptibility patterns of NTS infections in Korean children. Methods From November 2006 to May 2021, we collected NTS cases isolat

Purpose: Diagnosing Leigh syndrome (LS), a representative mitochondrial disease, remains challenging. Mitochondrial DNA (mtDNA)-associated LS, which is maternally inherited, has relatively well-known genetic variants. We evaluated the usefulness of brain magnetic resonance spectroscopy (MRS) for the initial diagnosis of mtDNA-associated LS using data from LS patients.Methods: The study involved LS patients who visited Gangnam Severance Hospital between 2006 and 2018. Based on patients’ clinical

Abstract Background Tetracycline (TC) is not recommended for pediatric use due to the risk of permanent teeth discoloration and enamel dysplasia. We aimed to assess the incidence of dental abnormality in Korean children and investigate whether the risk was greater in tetracycline-exposed children compared to the general population. Methods This nationwide, population-based study using Health Insurance Review and Assessment service database included children (aged 0-12 years) who were prescribed

Abstract Background Patients with primary antibody deficiency are known to be associated with a higher risk of SARS-CoV-2 infection, complications, and mortality. Tixagevimab/Cilgavimab prophylaxis has shown efficacy in reducing hospitalization and mortality. We aim to describe the clinical characteristics and outcomes of COVID-19 cases in primary antibody deficiency patients receiving tixagevimab/cilgavimab prophylaxis in South Korea. Methods This is a descriptive analysis of COVID-19 infection

Purpose: Magnetic resonance spectroscopy (MRS) is a diagnostic tool used to detect abnormal accumulation of lactate in the brain parenchyma in various metabolic diseases. This study evaluated the clinical roles of brain MRS in the initial assessment of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) caused by impairment of the mitochondrial respiratory chain. Methods: Twenty-five patients with the A3243G mutation among 34 MELAS patients referred to the pediatri

Abstract Background DiGeorge syndrome (DGS) is a syndrome accompanied by congenital heart defect, hypoparathyroidism and immunodeficiency of varying severity. Live vaccination is generally contraindicated in patients with DGS. However, in real clinical practice, there are cases in which live vaccines are immunized before the diagnosis of DGS or are incidentally immunized. We collected these cases and investigated adverse events (AEs), especially infections caused by the vaccine strains. Methods

Abstract Background Despite its clinical usefulness, the use of tetracycline antibiotics (TCs) in children has historically been limited because of the risk of permanent tooth discoloration and enamel deformity, although age restrictions have varied across countries (e.g., contraindicated under 8 years of age in Taiwan, USA, and Canada, and under 12 years of age in Korea and UK). We explored whether the incidence of dental abnormalities in Korean children with a history of TCs use differed by ag