Tubulointerstitial nephritis and uveitis (TINU) syndrome is defined as the occurrence of tubulointerstitial nephritis and uveitis in the absence of other systemic diseases. Three pediatric cases have been reported in the Republic of Korea, and we now report a fourth case. A 15-year-old girl presented to the ophthalmology department with a 1-week history of bilateral ocular discomfort that worsened on the day of presentation with redness and pain in both eyes. She was diagnosed with bilateral uveitis, and her baseline examination revealed moderate renal dysfunction and mild proteinuria. A renal biopsy was performed and confirmed the diagnosis of TINU syndrome. She was started on steroid eye drops and a 12-week course of oral steroids at a dose of 40 mg/m2/day, which completely resolved the proteinuria and mild renal function to an estimated glomerular filtration rate of 60 mL/min/1.73 m2. However, the uveitis did not improve, and despite the addition of oral methotrexate as a second-line treatment, the uveitis remains unresponsive to treatment over 21 months. Further evaluation and treatment are ongoing, and active therapeutic intervention is suggested even at a pediatric age, considering the lack of improvement in renal function and uveitis to date.

Globally, there have been a small number of donor cell leukemia cases reported but, to our knowledge, none have been reported in Korea until now. Donor cell leukemia is a rare though well-recognized complication that occurs after allogenic hematopoietic stem cell transplantation (allo-HSCT). Herein, we report the case of an 18-year-old woman who developed acute lymphoblastic leukemia originating from donor cells after allo-HSCT.
Adolescent ; Anemia, Aplastic* ; Bone Marrow Transplantation* ; Bone Marrow* ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Korea ; Leukemia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Tissue Donors*

PURPOSE: The aim of the present study was to investigate the risk factors for the development of coronary artery lesions (CALs) and to determine whether hyponatremia is associated with CALs in children with Kawasaki disease (KD). METHODS: We retrospectively analyzed the data of 105 children with KD who were admitted to Ilsan Hospital between January 2000 and July 2011. RESULTS: Erythrocyte sedimentation rate (P = 0.013), total bilirubin levels (P = 0.017) were higher and serum sodium levels (P = 0.027) were lower in KD children with CALs than those without. White blood cell (WBC) counts (P = 0.006), neutrophil counts (P = 0.003) were higher and albumin levels (P = 0.009) were lower in KD children with hyponatremia than those without. On multiple logistic regression analysis, hyponatremia (P = 0.024) and intravenous immunoglobulin??resistance (P = 0.024) were independent risk factors for CALs in KD. Furthermore, serum sodium levels were correlated negatively with WBC counts (P = 0.004), neutrophil counts (P < 0.001), total bilirubin levels (P = 0.005) and positively with albumin levels (P = 0.009). CONCLUSION: Our study indicates that hyponatremia may reflect severe inflammation in children with KD.
Bilirubin ; Blood Sedimentation ; Cardiovascular Abnormalities ; Child* ; Coronary Vessels ; Humans ; Hyponatremia* ; Inflammation* ; Leukocytes ; Logistic Models ; Mucocutaneous Lymph Node Syndrome* ; Neutrophils ; Retrospective Studies ; Risk Factors ; Sodium

Early esophageal cancer is defined as a tumor invading the mucosa with or without lymph node or distant organ metastasis. In the current guidelines for early esophageal cancer, absolute indication for endoscopic resection include lesions limited to the epithelium or lamina propria mucosa not exceeding two-thirds of the circumference, and relative indications include lesions limited to the muscularis mucosa or the upper third of the submucosal layer and not accompanied by clinical evidence of lymph node metastasis. After endoscopic submucosal dissection for early esophageal cancer, locally recurrent cancer can occur, especially in the case of incomplete resection. Here, we report a rare case of a submucosal tumor-like recurrence after endoscopic resection of early esophageal cancer.
Endosonography ; Epithelium ; Esophageal Neoplasms ; Lymph Nodes ; Mucous Membrane ; Neoplasm Metastasis ; Recurrence

The safety and effectiveness of adalimumab was demonstrated in a phase 3 trial in Japanese patients with intestinal Behçet’s disease. The aim of this study was to evaluate the long-term safety and effectiveness of adalimumab in Japanese patients with intestinal Behçet’s disease.

The safety and effectiveness of adalimumab was demonstrated in a phase 3 trial in Japanese patients with intestinal Behçet’s disease. The aim of this study was to evaluate the long-term safety and effectiveness of adalimumab in Japanese patients with intestinal Behçet’s disease.

Background/Aims: Crohn’s disease (CD) with recurrent inflammation can cause intestinal fibrostenosis due to dysregulated deposition of extracellular matrix. However, little is known about the pathogenesis of fibrostenosis. Here, we performed a differential proteomic analysis between normal, inflamed, and fibrostenotic specimens of patients with CD and investigated the roles of the candidate proteins in myofibroblast activation and fibrosis. Methods: We performed two-dimensional difference gel electrophoresis and identified candidate proteins using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry and orbitrap liquid chromatography-mass spectrometry. We also verified the levels of candidate proteins in clinical specimens and examined their effects on 18Co myofibroblasts and Caco-2 intestinal epithelial cells. Results: We identified five of 30 proteins (HSP72, HSPA5, KRT8, PEPCK-M, and FABP6) differentially expressed in fibrostenotic CD. Among these proteins, the knockdown of heat shock protein 72 (HSP72) promoted the activation and wound healing of myofibroblasts. Moreover, knockdown of HSP72 induced the epithelial-mesenchymal transition of intestinal epithelial cells by reducing E-cadherin and inducing fibronectin and α-smooth muscle actin, which contribute tofibrosis. Conclusions HSP72 is an important mediator that regulates myofibroblasts and epithelial-mesenchymal transition in fibrosis of CD, suggesting that HSP72 can serve as a target for antifibrotic therapy.

Background/Aims: The purpose of the current study was to examine the anti-inflammatory effects of CKD-506, a novel histone deacetylase 6 inhibitor, on human peripheral blood mononuclear cells (PBMCs) and CD4+ T cells and to explore the relationship between CKD-506 and gut epithelial barrier function. Methods: Lipopolysaccharide-stimulated human PBMCs from inflammatory bowel disease (IBD) patients were treated with CKD-506, and tumor necrosis factor (TNF)-α expression was measured using an enzyme-linked immunosorbent assay. The proliferation of CD4+ T cells from IBD patients was evaluated using flow cytometric analysis. The effects of CKD-506 on gut barrier function in a cell line and colon organoids, based on examinations of mRNA production, goblet cell differentiation, and E-cadherin recovery, were investigated using quantitative reverse transcription polymerase chain reaction, immunofluorescence, and a fluorescein isothiocyanatedextran permeability assay. Results: Secretion of TNF-α, a pivotal pro-inflammatory mediator in IBD, by lipopolysaccharidetriggered PBMCs was markedly decreased by CKD-506 treatment in a dose-dependent manner and to a greater extent than by tofacitinib or tubastatin A treatment. E-cadherin mRNA expression and goblet cell differentiation increased significantly and dose-dependently in HT-29 cells in response to CKD-506, and inhibition of E-cadherin loss after TNF-α stimulation was significantly reduced both in HT-29 cells and gut organoids. Caco-2 cells treated with CKD-506 showed a significant reduction in barrier permeability in a dose-dependent manner. Conclusions The present study demonstrated that CKD-506 has anti-inflammatory effects on PBMCs and CD4 T cells and improves gut barrier function, suggesting its potential as a smallmolecule therapeutic option for IBD.

PURPOSE: Pulmonary function tests are useful and important methods for evaluating patients with respiratory diseases. To assess lung function, we need to establish normal values of lung function, which vary according to population, age, gender and growth, particularly in children. This study was undertaken to establish normal predicted values of pulmonary function tests and to predict renewed reference values by spirometry in Korean school children. METHODS: Spirometric forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), forced expiratory flow at 25% to 75% of FVC (FEF25%-75%), and peak expiratory flow rate (PEFR) were measured in 406 healthy elementary school children (age, 6-12 years old) in May, 2012. Multiple linear regression analysis was performed for each spirometric parameter against age, height, weight and body surface area (BSA) separately for boys and girls, and derived reference equations and coefficients for all the spirometric parameters. RESULTS: All the measured spirometric parameters were significantly correlated with age, height, weight, and BSA. FVC was higher in boys aged 6-10 years than girls at ages matched. Height showed the highest correlation coefficient for all the spirometric parameters followed by BSA, weight and age in both genders. The normal predicted values of FVC and FEV1 at 130 cm in this study using renewed reference equations were approximately in the middle compared to previous studies. CONCLUSION: We reported here the renewed reference equations for normal predicted values of pulmonary function tests with high coefficients of determination based on updated data in healthy Korean children.
Body Surface Area ; Child* ; Female ; Forced Expiratory Volume ; Humans ; Linear Models ; Lung ; Peak Expiratory Flow Rate ; Reference Values* ; Respiratory Function Tests* ; Spirometry ; Vital Capacity