Background: Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods: We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results: A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.
Anthracyclines ; Cardiotoxicity ; Dexrazoxane ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence ; Korea ; Multivariate Analysis ; Neoplasms, Second Primary ; Risk Factors* ; Stem Cell Transplantation

BACKGROUND AND OBJECTIVES: Various methods to induce tracheal stenosis in an animal model have been introduced. However, most methods use non-physiologic mechanical or chemical injury to tracheal mucosa or cartilage. In this study, we sought to develop an animal model of tracheal stenosis using a segmented endotracheal tube. MATERIALS AND METHOD: Nine New Zealand White Rabbits were included in this feasibility study. A segmented 1.5 cm LEVIN-Tube (16 French) was inserted into tracheal lumen via tracheotomy site and fixed with a nylon tape circumferentially tied around the trachea. The tube was removed transorally one week later and the tracheal lumen was observed with bronchoscopy every week. Rabbits were sacrificed two weeks after the tube removal and the trachea was evaluated with histologic image. Three rabbits underwent tracheotomy and closure only to evaluate possible impact of tracheotomy procedure to tracheal stenosis (sham surgery). RESULTS: None of the 6 rabbits showed significant complications or death during the study. No significant change of tracheal lumen was identified in 3 sham models. The mean grade of stenosis was 57.2±9.9% (range, 43-70%). Histologic image showed thickening and fibrosis of lamina propria with relatively intact tracheal cartilage framework. CONCLUSION: We developed an animal model of tracheal stenosis using a segmented endotracheal tube fixed with a nylon tape. Since this model has similar pathophysiology to prolonged endotracheal intubation, it may be used in various studies related to tracheal stenosis.
Animals* ; Bronchoscopy ; Cartilage ; Constriction, Pathologic ; Feasibility Studies ; Fibrosis ; Intubation, Intratracheal ; Methods ; Models, Animal* ; Mucous Membrane ; Nylons ; Rabbits ; Trachea ; Tracheal Stenosis* ; Tracheotomy

BACKGROUND: From May to July 2015, the Republic of Korea experienced the largest outbreak of Middle East respiratory syndrome (MERS) outside the Arabian Peninsula. A total of 186 patients, including 36 deaths, had been diagnosed with MERS-coronavirus (MERS-CoV) infection as of September 30th, 2015. MATERIALS AND METHODS: We obtained information of patients who were confirmed to have MERS-CoV infection. MERS-CoV infection was diagnosed using real-time reverse-transcriptase polymerase chain reaction assay. RESULTS: The median age of the patients was 55 years (range, 16 to 86). A total of 55.4% of the patients had one or more coexisting medical conditions. The most common symptom was fever (95.2%). At admission, leukopenia (42.6%), thrombocytopenia (46.6%), and elevation of aspartate aminotransferase (42.7%) were observed. Pneumonia was detected in 68.3% of patients at admission and developed in 80.8% during the disease course. Antiviral agents were used for 74.7% of patients. Mechanical ventilation, extracorporeal membrane oxygenation, and convalescent serum were employed for 24.5%, 7.1%, and 3.8% of patients, respectively. Older age, presence of coexisting medical conditions including diabetes or chronic lung disease, presence of dyspnea, hypotension, and leukocytosis at admission, and the use of mechanical ventilation were revealed to be independent predictors of death. CONCLUSION: The clinical features of MERS-CoV infection in the Republic of Korea were similar to those of previous outbreaks in the Middle East. However, the overall mortality rate (20.4%) was lower than that in previous reports. Enhanced surveillance and active management of patients during the outbreak may have resulted in improved outcomes.
Antiviral Agents ; Aspartate Aminotransferases ; Coronavirus Infections* ; Disease Outbreaks ; Dyspnea ; Extracorporeal Membrane Oxygenation ; Fever ; Humans ; Hypotension ; Leukocytosis ; Leukopenia ; Lung Diseases ; Middle East Respiratory Syndrome Coronavirus ; Middle East* ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Republic of Korea* ; Respiration, Artificial ; Thrombocytopenia

It has not yet been determined whether chronic exposure to relatively low doses of pioglitazone increases risk of bladder cancer. We aimed to assess the risk of bladder cancer associated with pioglitazone in Korean patients. This was a retrospective cohort study of diabetic patients who had > or = 2 clinic visits between November 2005 and June 2011 at one of four tertiary referral hospitals in Korea. A prevalent case-control analysis nested within the cohort was conducted to further adjust confounders. A total of 101,953 control patients and 11,240 pioglitazone-treated patients were included, in which there were 237 and 30 cases of incidental bladder cancer (64.9 and 54.9 per 100,000 person-years; age, sex-adjusted HR 1.135, 95% confidence interval [CI] 0.769-1.677), respectively. In the prevalent case-control analysis nested within the cohort, use of pioglitazone for a duration of > 6 months, but not ever use of pioglitazone, was associated with an increased rate of bladder cancer as compared to never use of pioglitazone. In conclusion, we failed to exclude the possible association between use of pioglitazone for a duration of > 6 months and bladder cancer.
Aged ; Asian Continental Ancestry Group ; Case-Control Studies ; Cohort Studies ; Diabetes Mellitus, Type 2/complications/*drug therapy ; Female ; Humans ; Hypoglycemic Agents/*therapeutic use ; Male ; Middle Aged ; Prevalence ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Tertiary Care Centers ; Thiazolidinediones/*therapeutic use ; Urinary Bladder Neoplasms/complications/*diagnosis/epidemiology

Coronary artery milking is defined as a systolic compression of the coronary artery, usually resulting from myocardial bridging. Rarely, congenital heart disease, pulmonary hypertension and ventricular aneurysm can lead to coronary artery milking, and there has been a reported case of left anterior descending coronary artery milking after coronary stenting. Percutaneous coronary intervention (PCI) is an emerging strategy for treatment of stenosis in the left main stem. We report a case of symtomatic systolic milking at the left main stem treated by direct stenting during PCI in a patient with unstable angina pectoris.
Aneurysm ; Angina, Unstable ; Constriction, Pathologic ; Coronary Vessels ; Humans ; Hypertension ; Milk ; Myocardial Bridging ; Percutaneous Coronary Intervention ; Pulmonary Heart Disease ; Stents

Disseminated Mycobacterium avium complex (MAC) infection can occur in immunocompromised patients, and rarely in immunocompetent subjects. Due to the extensive distribution of the disease, clinical presentation of disseminated MAC may mimic malignancies, and thorough examinations are required in order to make accurate diagnosis. We report a case of disseminated Mycobacterium intracellulare disease in an immunocompetent patient, which involved the lung, lymph nodes, spleen, and multiple bones. F-18 fluorodeoxyglucose positron-emission tomography imaging showed multiple hypermetabolic lesions, which are suggestive of typical hematogenous metastasis. However, there was no evidence of malignancy in serial biopsies, and M. intracellulare was repeatedly cultured from respiratory specimens and bones. Herein, we should know that disseminated infection can occur in the immunocompetent subjects, and it can mimic malignancies.
Biopsy ; Humans ; Hybridization, Genetic ; Hydrazines ; Immunocompetence ; Immunocompromised Host ; Lung ; Lymph Nodes ; Mycobacterium ; Mycobacterium avium Complex ; Mycobacterium avium-intracellulare Infection ; Neoplasm Metastasis ; Nontuberculous Mycobacteria ; Positron-Emission Tomography ; Spleen

Knee pain is rarely an initial symptom of colon cancer, because most bone metastases develop in the late stage of cancer. Therefore, patients with colorectal cancer usually present with gastrointestinal symptoms. Recently, we experienced a 46-year-old female with rectal cancer that presented as knee pain due to tibial metastasis without other organ metastasis. She was evaluated for knee pain and diagnosed with a rectal cancer after biopsies of the tibial and rectal masses. We report the first such case in Korea with a review of the literature.
Biopsy ; Colon ; Colonic Neoplasms ; Colorectal Neoplasms ; Female ; Humans ; Knee ; Korea ; Middle Aged ; Neoplasm Metastasis ; Rectal Neoplasms ; Tibia