Osteochondritis dissecans of the knee occurs twice as often in males as in females. The peak incidence is about fifteen years of age, and most patients are first diagnosed before the age of eighteen. The etiology of osteochondritis dissecans is still unknown, but the most accepted theories are trauma, ischemia, abnormal ossification within the physes, constitutional or genetic predisposition, and a combination of these. Osteochondritis dissecans of the knee associated with gout was first reported by Carrabba in 1969. And no additional case has been reported. The purpose of this paper is to present an unusual case of osteochondritis dissecans of the knee associated with gout.
Female ; Genetic Predisposition to Disease ; Gout* ; Humans ; Incidence ; Ischemia ; Knee* ; Male ; Osteochondritis Dissecans* ; Osteochondritis*

No abstract available.
Female ; Humans

Kimura's disease is a rare chronic inflammatory disease of the lymphoid organs and this presents as painless nodules in the head and neck area. It occurs endemically in the Far East Asia and sporadically in the West. Its exact etiology is unknown and it has no potential to transform into malignant disease. However, it is often confused with malignant disease such as malignant lymphoma. We report a case of NK/T cell lymphoma patient who had Kimura's disease in the right thigh 3 years ago. She then suffered from sudden dyspnea. Radiological studies revealed multiple small nodules scattered in both lung fields. NK/T cell lymphoma was diagnosed by open lung biopsy. In addition, Epstein-Barr virus (EBV) was detected in the previous site of Kimura's disease and lung nodule by molecular pathologic examination. To the best of our knowledge, this is the first case of NK/T cell lymphoma complicating Kimura's disease with coexisting chronic EBV infection.
Asia ; Biopsy ; Dyspnea ; Epstein-Barr Virus Infections ; Far East ; Follow-Up Studies* ; Head ; Herpesvirus 4, Human ; Humans ; Lung ; Lymphoma* ; Neck ; Thigh

This study was to investigate the effects of individual and district-level socioeconomic status (SES) on the development of cognitive impairment among the elderly. A 3-year retrospective observational analysis (2010–2013) was conducted which included 136,217 community-dwelling healthy elderly who participated in the Seoul Dementia Management Project. Cognitive impairment was defined as 1.5 standard deviations below the norms on the Mini-mental status examination. In the individual lower SES group, the cumulative incidence rate (CIR) of cognitive impairment was 8.7% (95% confidence interval [CI], 8.64–8.70), whereas the CIR in the individual higher SES group was 4.1% (95% CI, 4.08–4.10). The CIR for lower district-level SES was 4.7% (95% CI, 4.52–4.86), while that in the higher district-level SES was 4.3% (95% CI, 4.06–4.44). There were no additive or synergistic effects between individual and district-level SES. From this study, the individual SES contributed 1.9 times greater to the development of cognitive impairment than the district-level SES, which suggests that individual SES disparities could be considered as one of the important factors in public health related to cognitive impairment in the elderly.
Aged* ; Cognition Disorders ; Dementia ; Humans ; Incidence ; Public Health ; Retrospective Studies ; Seoul* ; Social Class

A new alpha-pyrone derivative, violapyrone J (1), and along with the two known violapyrones B (2) and C (3) were isolated from the fermentation broth of a marine actinomycete Streptomyces sp. SC0718. The structure of violapyrone J (1) was elucidated from 1D and 2D NMR spectroscopic analyses.
Actinobacteria* ; Fermentation ; Streptomyces*

AbstractThe demand for natural substances with anti-melanogenic activity is increasing due to the recent interest in skin whitening. Intensive investigation on the culture broth of Streptomyces sp. SCO-736, a marine bacterium from the Antarctica coast, has led to the isolation of a new natural product named antaroide (1). The chemical structure was established through the interpretation of MS, UV, and NMR spectroscopic data. Antaroide is a nine-membered macrolide with lactone and lactam moieties. To investigate its applicability in skin whitening cosmetics, its anti-melanogenic activity in B16F10 murine melanoma cells was examined. As a result, antaroide displayed strong inhibitory activities against melanin synthesis and also attenuated the dendrite formation induced by the α-melanocyte stimulating hormone (α-MSH). Antaroide suppressed the mRNA expression of the melanogenic enzymes such as tyrosinase, TRP-1 and TRP-2. This suggests that it may serve as a transcriptional regulator of melanogenesis. Collectively, the discovery of this novel natural nine-membered macrolide and its anti-melanogenic activity could give new insights for the development of skin whitening agents.

PURPOSE: We developed a new workflow design which included results from both biochemical and targeted gene sequencing analysis interpreted comprehensively. We then conducted a pilot study to evaluate the benefit of this new approach in newborn screening (NBS) and demonstrated the efficiency of this workflow in detecting causative genetic variants. MATERIALS AND METHODS: Ten patients in Group 1 were diagnosed clinically using biochemical assays only, and 10 newborns in Group 2 were diagnosed with suspected inherited metabolic disease (IMD) in NBS. We applied NewbornDiscovery (SD Genomics), an integrated workflow design that encompasses analyte-phenotype-gene, single nucleotide variant/small insertion and deletion/copy number variation analyses along with clinical interpretation of genetic variants related to each participant's condition. RESULTS: A molecular genetic diagnosis was established in 95% (19/20) of individuals. In Group 1, 13 and 7 of 20 alleles were classified as pathogenic and likely pathogenic, respectively. In Group 2, 11 and 6 of 17 alleles with identified causative variants were pathogenic and likely pathogenic, respectively. There were no variants of uncertain significance. For each individual, the NewbornDiscovery and biochemical analysis results reached 100% concordance, since the single newborn testing negative for causative genetic variant in Group 2 showed a benign clinical course. CONCLUSION: This integrated diagnostic workflow resulted in a high yield. This approach not only enabled early confirmation of specific IMD, but also detected conditions not included in the current NBS.
Alleles ; Diagnosis ; Diagnosis, Differential* ; Humans ; Infant, Newborn* ; Mass Screening* ; Metabolic Diseases* ; Molecular Biology ; Pilot Projects

Yolk sac tumors are rare malignant germ cell neoplasms that usually arise from the gonads. Extragonadal yolk sac tumors (EGYSTs) frequently occur in the mediastinum in post-pubertal females. EGYSTs in the pelvis are extremely rare, and to date, only thirteen cases have been reported in the English literature. Among them, the primary EGYST of the pelvic peritoneum in post-pubertal females has only been reported in ten cases. The present case describes a 26-year-old female diagnosed with primary peritoneal yolk sac tumor located in the rectouterine pouch. We report clinical and tumor imaging features, including ultrasound, computed tomography (CT), magnetic resonance images (MRI), positron emission tomography-computed tomography (PET-CT), and present a review of the literature.
Adult ; Douglas' Pouch ; Electrons ; Endodermal Sinus Tumor ; Female ; Gonads ; Humans ; Magnetic Resonance Imaging ; Mediastinum ; Neoplasms, Germ Cell and Embryonal ; Pelvis ; Peritoneum ; Ultrasonography ; Yolk Sac

PURPOSE: An intestinal perforation is a rare condition, but has a high mortality rate, even after immediate surgical intervention. The clinical predictors of postoperative morbidity and mortality are still not well established, so this study attempted to identify risk factors for postoperative morbidity and mortality after surgery for an intestinal perforation. METHODS: We retrospectively analyzed the cases of 117 patients who underwent surgery for an intestinal perforation at a single institution in Korea from November 2008 to June 2014. Factors related with postoperative mortality at 1 month and other postoperative complications were investigated. RESULTS: The mean age of enrolled patients was 66.0 ± 15.8 years and 66% of the patients were male. Fifteen patients (13%) died within 1 month after surgical treatment. Univariate analysis indicated that patient-related factors associated with mortality were low systolic and diastolic blood pressure, low serum albumin, low serum protein, low total cholesterol, and high blood urea nitrogen; the surgery-related factor associated with mortality was feculent ascites. Multivariate analysis using a logistic regression indicated that low systolic blood pressure and feculent ascites independently increased the risk for mortality; postoperative complications were more likely in both females and those with low estimated glomerular filtration rates and elevated serum C-reactive protein levels. CONCLUSION: Various factors were associated with postoperative clinical outcomes of patients with an intestinal perforation. Morbidity and mortality following an intestinal perforation were greater in patients with unstable initial vital signs, poor nutritional status, and feculent ascites.
Ascites ; Blood Pressure ; Blood Urea Nitrogen ; C-Reactive Protein ; Cholesterol ; Female ; Glomerular Filtration Rate ; Humans ; Hypotension ; Intestinal Perforation* ; Korea ; Logistic Models ; Male ; Mortality* ; Multivariate Analysis ; Nutritional Status ; Postoperative Complications ; Retrospective Studies ; Risk Factors ; Serum Albumin ; Vital Signs

BACKGROUND/AIMS: The first edition of the Korean treatment guidelines for chronic myelogenous leukemia (CML) was published in 2006. We intend to update those guidelines to include the use of next-generation tyrosine kinase inhibitors (TKIs). METHODS: New guidelines were developed in 2012 based on the results of a survey and a consensus meeting of various Korean experts, the reports of recent clinical studies, and updated guidelines from external study groups. RESULTS: An assessment of risk factors is strongly recommended before treating newly diagnosed chronic phase CML. Imatinib, dasatinib, and nilotinib are reimbursable in Korea as first-line treatments, and the patient's age, comorbidities, and possible adverse events should be considered in the choice of treatment. Molecular studies are recommended for assessing treatment efficacy instead of invasive cytogenetic response evaluations, and an early response is believed to correlate with a good prognosis. Second-line TKIs can be considered for patients who fail or are intolerant of first-line therapy, pending analysis of ABL tyrosine kinase mutation status. For treating advanced stages, a combination of TKIs with cytotoxic agents and hematopoietic cell transplantation is recommended. The adverse effects of TKI therapy can be managed via dose reduction and supportive care, or switching to an alternate TKI. CONCLUSIONS: The use of TKIs has improved the outcome of CML treatment. Treatment-free remission after discontinuing TKIs might be possible in select patients who achieve sufficient response, indicating that curative treatment for CML can be expected in the future.
Cell Transplantation ; Comorbidity ; Consensus ; Cytogenetics ; Cytotoxins ; Hematology* ; Humans ; Korea ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Prognosis ; Protein-Tyrosine Kinases ; Risk Factors ; Transplants ; Treatment Outcome ; Dasatinib ; Imatinib Mesylate