김성헌 교수

In the last decade, great advances have been made in understanding the genetic basis for focal segmental glomerulosclerosis (FSGS). Animal models using specific gene disruption of the slit diaphragm and cytoskeleton of the foot process mirror the etiology of the human disease. Many animal models have been developed to understand the complex pathophysiology of FSGS. Therefore, we need to know the usefulness and exact methodology of creating animal models. Here, we review classic animal models and

Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of

Background: Little information exists regarding the differences in the clinical and laboratory characteristics of Kikuchi-Fujimoto disease (KFD) according to age. Objective: To evaluate the clinical and laboratory characteristics of KFD according to age. Methods: The relevance of sex, age, clinical features, laboratory findings, courses, and follow-up results were retrospectively evaluated in patients diagnosed with KFD at Pusan National University Hospital between 2010 and 2020. Results: Eighty

BACKGROUND: The aim of this study was to describe clinical and laboratory characteristics of urinary tract infection (UTI) without significant pyuria in young children aged 2-24 months. METHODS: The subjects consisted of infants and young children with febrile UTI treated at Pusan National University Children's Hospital, Korea. Group A included 283 patients with definite UTI who fulfilled the revised American Academy of Pediatrics diagnostic criteria, and group B included 19 patients with presum

We mapped spatially varying intramolecular electronic structures on a pentacene-gold interface using scanning tunneling spectroscopy. Along with ab initio calculations based on density functional theory, we found that the directional nature of the d orbitals of Au atoms plays an important role in the interaction at the pentacene-gold contact. The gold-induced interface states are broadened and shifted by various pentacene-gold distances determined by the various registries of a pentacene molecul

Focal segmental glomerulosclerosis (FSGS) is a nephrotic syndrome. Up to around 80% of cases of primary FSGS are resistant to steroid treatment. A large proportion of patients with steroid-resistant FSGS progress to end-stage renal disease. The purpose of treatment is to obtain a complete remission of proteinuria, a necessary step that precedes improved renal survival and reduces the risk of progression to chronic kidney disease. When this is not possible, the secondary goal is a partial remissi

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse cutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis. Objective We investigated the differences in clinical characteristics of drug-induced SJS/TEN depending on the type of drug in a single center. Methods The relevance of sex, age, culprit drugs, clinical features, courses, treatment options, and follow-up results were retr

Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan Nation

-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal

Corticosteroids are potent anti-inflammatory and anti-allergic agents used in the treatment of various inflammatory diseases, including allergic diseases. Allergic reactions caused by corticosteroids, however, have been reported. Among these, delayed reactions to topical steroids are more common, whereas immediate reactions to systemic steroids are rare. Herein, we report the case of a 10-year old boy with methylprednisolone-induced anaphylaxis, in which the patient had a positive oral challenge

Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while single photon emission computed tomograp

BACKGROUND: Cephalosporin is the most commonly used empirical agent for urinary tract infections (UTIs) in children. However, increasing use of cephalosporins can lead to an increase in resistant pathogens. This study therefore aims to investigate the effects of monotherapy with ampicillin-sulbactam as an alternative to cephalosporins. METHODS: All 2- to 24-month-old patients who were hospitalized at Pusan National University Children's Hospital due to a first episode of a febrile UTI during the