Coronary stenting for long complex lesion is effective but associated with complication. We compared the results of stenting between with multiple conventional stenting group (group A) and with single long stenting group (group B). Fifty patients were prospectively and randomly enrolled: 25 patients for each group. Each group showed no significant differences of clinical characteristics. One patient died of heart failure in each group, not associated with the procedure itself. One patients had cerebrovascular accident in each group. Five patients had major bleeding (2, group A; 3, group B). Angiographic success rate was 100% in each group and procedural success rate was 96% and 100% in group A and B, respectively. Angiographic and clinical restenosis rate at 6 months follow-up were 60%, 36% in group A and 65%, 44% in group B, respectively (p=S). Multivariate analysis showed that several factors affected the angiographic restenosis rate as follows; a) male gender (M:F=76.9%:25.0%, P<0.001), b) AMI (AMI:stable angina pectoris=72.7%:66.7%, P<0.001), c) lesion length d) residual stenosis. In conclusion, there were no statistical differences of restenosis and complication rate between the two groups. Our data support single long stenting is acceptable and economically more favorable for long diffuse lesion, compared to multiple conventional stenting.
Constriction, Pathologic ; Coronary Stenosis* ; Coronary Vessels* ; Follow-Up Studies ; Heart Failure ; Hemorrhage ; Humans ; Male ; Multivariate Analysis ; Prospective Studies ; Stents* ; Stroke

Uterine leiomyomas are the most common benign uterine tumors. They are estimated to be present in at least 20% of all women of reproductive age and may be asymptomatic in 40-50% of women older than 40 years of age. They may be subserosal, intramural, or submucosal in location within the uterus or located in the cervix, in the broad ligament, or on a pedicle. But it seems to be rare to experience a intraligamentary huge leiomyoma in the broad ligament.Recently we have experienced a case of intraligamentary huge leiomyoma with its weight of 3146 gm in 45 year-old primiparous woman. We present this case with a brief review of the literature.
Broad Ligament ; Cervix Uteri ; Female ; Humans ; Leiomyoma* ; Middle Aged ; Uterus

The vast majority of malignant tumors involving the vagina are secondary spread from primary malignant lesion of the cervix uteri, the sigmoid colon, the bladder and the vulva. Primary invasive carcinoma of the vagina remains among the rare gynecologic malignant tumor. The diagnosis of primary carcinoma of the vagina requires that the cervix and the vulva be intact and no clinical evidence of other primary tumors exist. Greater than 80-90% of all vaginal tumors are squamous cell type. We experienced two cases of primary vaginal cancer of 68 years old woman without any other gynecologic disease and 67 years old woman after hysterectomy for benign desease. We presented these cases with a brief review of related literatures.
Aged ; Cervix Uteri ; Colon, Sigmoid ; Diagnosis ; Female ; Genital Diseases, Female ; Humans ; Hysterectomy ; Neoplasms, Squamous Cell ; Urinary Bladder ; Vagina ; Vaginal Neoplasms* ; Vulva

PURPOSE: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center.METHODS: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed.RESULTS: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months–8.3 years). The median age of periodic fever onset was 3 years (range, 1–6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4–13.2 mg/dL) and 29 mm/hr (range, 16–49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy.CONCLUSIONS: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.
Adenoidectomy ; Anti-Bacterial Agents ; Blood Sedimentation ; C-Reactive Protein ; Child ; Diagnosis ; Fever ; Follow-Up Studies ; Hematologic Tests ; Humans ; Lymphadenitis ; Male ; Neutropenia ; Pharyngitis ; Pharynx ; Prednisolone ; Retrospective Studies ; Seoul ; Stomatitis, Aphthous ; Tonsillectomy ; Yemen

Niemann-Pick disease type C (NPC) is a neurovisceral lysosomal storage disorder caused by mutations in the NPC1 and NPC2 genes. These mutations cause the accumulation of unesterified cholesterol and other lipids in the lysosomes. NPC has a broad spectrum of clinical manifestations, depending on the age of onset. A 15-day-old infant presented at the Seoul National University Children's Hospital with neonatal cholestasis and hepatosplenomegaly, with the onset of jaundice at 5 days of age. Despite supportive treatment, the patient was considered for a liver transplant because of progressive liver failure. Unfortunately, the patient died from gastrointestinal bleeding before undergoing the transplant. The neonatal cholestasis gene panel revealed two novel likely pathogenic variants in the NPC1 gene (c.1145C>G [p.Ser382*] and c.2231_2233del [p.Val744del]). The patient was diagnosed with NPC, and both parents were found to be carriers of each variant. In infants presenting with neonatal cholestasis, a gene panel can help diagnose NPC.

Regulation of matrix metalloproteinases (MMPs) is important for many physiological processes involving cancers, inflammation, tissue remodeling and skin aging. Here, we report the novel finding that the expression of MMP1 mRNA is downregulated by the overexpression of miR-526b which is a member of chromosome 19 microRNA cluster (C19MC). Our analysis using reporter constructs containing the 3' untranslated region (3' UTR) of MMP1 and its mutant form showed that the region from 377-383 in the 3' UTR of MMP1 is critical for targeting by miR-526b. In addition, the expression pattern of miR-526b and MMP1 mRNA showed reverse relation between adult dermal and neonatal fibroblasts. We show for the first time that miR-526b, an miRNA belonging to C19MC, can target the 377-383 region of the MMP1 3' UTR.
3' Untranslated Regions ; Adult ; Base Sequence ; Cell Line ; Down-Regulation ; Fibroblasts/metabolism ; *Gene Expression Regulation ; HeLa Cells ; Humans ; Matrix Metalloproteinase 1/*genetics ; MicroRNAs/*genetics ; RNA, Messenger/*genetics

Endocannabinoids can affect multiple cellular targets, such as cannabinoid (CB) receptors, transient receptor potential cation channel, subfamily V, member 1 (TRPV1) and peroxisome proliferator-activated receptor gamma (PPARgamma). The stimuli to induce adipocyte differentiation in hBM-MSCs increase the gene transcription of the CB1 receptor, TRPV1 and PPARgamma. In this study, the effects of three endocannabinoids, N-arachidonoyl ethanolamine (AEA), N-arachidonoyl dopamine (NADA) and 2-arachidonoyl glycerol (2-AG), on adipogenesis in hBM-MSCs were evaluated. The adipocyte differentiation was promoted by AEA whereas inhibited by NADA. No change was observed by the treatment of non-cytotoxic concentrations of 2-AG. The difference between AEA and NADA in the regulation of adipogenesis is associated with their effects on PPARgamma transactivation. AEA can directly activate PPARgamma. The effect of AEA on PPARgamma in hBM-MSCs may prevail over that on the CB1 receptor mediated signal transduction, giving rise to the AEA-induced promotion of adipogenesis. In contrast, NADA had no effect on the PPARgamma activity in the PPARgamma transactivation assay. The inhibitory effect of NADA on adipogenesis in hBM-MSCs was reversed not by capsazepine, a TRPV1 antagonist, but by rimonabant, a CB1 antagonist/inverse agonist. Rimonabant by itself promoted adipogenesis in hBM-MSCs, which may be interpreted as the result of the inverse agonism of the CB1 receptor. This result suggests that the constantly active CB1 receptor may contribute to suppress the adipocyte differentiation of hBM-MSCs. Therefore, the selective CB1 agonists that are unable to affect cellular PPARgamma activity inhibit adipogenesis in hBM-MSCs.
Adipocytes* ; Adipogenesis ; Dopamine* ; Endocannabinoids ; Ethanolamine ; Felodipine ; Glycerol ; Humans ; Mesenchymal Stromal Cells* ; PPAR gamma ; Receptor, Cannabinoid, CB1 ; Receptors, Cannabinoid* ; Signal Transduction ; Transcriptional Activation

Adiponectin secretion-promoting compounds have therapeutic potentials in human metabolic diseases. Diallyl biphenyl-type neolignan compounds, magnolol, honokiol, and 4-O-methylhonokiol, from a Magnolia officinalis extract were screened as adiponectin-secretion promoting compounds in the adipogenic differentiation model of human bone marrow mesenchymal stem cells (hBM-MSCs). In a target identification study, magnolol, honokiol, and 4-O-methylhonokiol were elucidated as PPARα and PPARγ dual modulators. Diallyl biphenyl-type neolignans affected the transcription of lipid metabolism-associated genes in a different way compared to those of specific PPAR ligands. The diallyl biphenyl-type neolignan structure provides a novel pharmacophore of PPARα/γ dual modulators, which may have unique therapeutic potentials in diverse metabolic diseases.

Purpose: Few studies have reported the prevalence of inflammatory bowel disease unclassified (IBDU) among Korean pediatric IBD (PIBD) population. To address this gap, we used two tertiary centers and nationwide population-based healthcare administrative data to estimate the prevalence of Korean pediatric IBDU at the time of diagnosis. Methods: We identified 136 patients aged 2–17 years with newly diagnosed IBD (94 Crohn’s disease [CD] and 42 ulcerative colitis [UC]) from two tertiary centers in Korea between 2005 and 2017. We reclassified these 136 patients using the revised Porto criteria. To estimate the population-based prevalence, we analyzed Korean administrative healthcare data between 2005 and 2016, which revealed 3,650 IBD patients, including 2,538 CD and 1,112 UC. By extrapolating the reclassified results to a population-based dataset, we estimated the prevalence of PIBD subtypes. Results: Among the 94 CD, the original diagnosis remained unchanged in 93 (98.9%), while the diagnosis of one (1.1%) patient was changed to IBDU. Among the 42 UC, the original diagnosis remained unchanged in 13 (31.0%), while the diagnoses in 11 (26.2%), 17 (40.5%), and one (2.4%) patient changed to atypical UC, IBDU, and CD, respectively. The estimated prevalences of CD, UC, atypical UC, and IBDU in the Korean population were 69.5%, 9.4%, 8.0%, and 13.1%, respectively. Conclusion This study is the first in Korea to estimate the prevalence of pediatric IBDU.This prevalence (13.1%) aligns with findings from Western studies. Large-scale prospective multicenter studies on PIBDU are required to examine the clinical features and outcomes of this condition.

BACKGROUND AND OBJECTIVES: Sudden cardiac death (SCD) is one of the most common causes of death in patients with heart failure (HF). However, there are no available data on SCD in previous Korean HF registries. Additionally, although widely used, the utility of left ventricular (LV) ejection fraction (EF) in risk stratification for SCD is limited. SUBJECTS AND METHODS: The Korean non-invasive Risk Evaluation study for sudden cardiac DEath From INfarction or heart failurE (KREDEFINE) is the first Korean prospective, nationwide multicenter registry, primarily focused on SCD. The registry consists of 2 groups of patients presenting with (1) acute HF or (2) acute myocardial infarction (MI) at 25 tertiary referral cardiovascular centers. Using the HF-group data of the K-REDEFINE registry, the incidence and risk factors of SCD in patients with HF will be assessed. In particular, the efficacy of Holter-based ECG variables, such as T-wave alternans (marker of repolarization heterogeneity) and heart rate turbulence/ variability (maker of autonomic function), in risk stratification for SCD will be evaluated. Other cardiovascular outcomes will also be analyzed, including atrioventricular arrhythmias, HF-related admission, stroke, and overall deaths. CONCLUSION AND PERSPECTIVE: The K-REDEFINE registry will pave the way for better management of patients with HF at high risk of SCD by elucidating the burden and risk factors of SCD and the clinical utility of various non-invasive ambulatory ECG-based parameters in risk stratification for SCD in this patient population.
Arrhythmias, Cardiac ; Cause of Death ; Death, Sudden, Cardiac* ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Incidence ; Infarction* ; Myocardial Infarction ; Prospective Studies ; Referral and Consultation ; Registries ; Risk Factors ; Stroke