45,X/47,XYY mosaicism is a rare sex chromosomal disorder with clinical information limited to 25 cases in the literature. We report an unusual mosaic Turner syndrome case in a 35-year old Korean woman with a phenotypic female, primary amenorrhea, short stature, immature secondary sexual characteristics. Cytogenetic analysis including G- and Q-banding revealed 45,X/47,XYY mosaicism, and SRY gene was demonstrated by polymerase chain reaction(PCR). Prophylactic bilateral gonadectomy was performed because the presence of Y-chromosomal sequences in Turner stigmata may predispose this patient to gonadoblastoma formation.
Adult ; Amenorrhea ; Christianity ; Chromosome Disorders ; Cytogenetic Analysis ; Female ; Genes, sry ; Gonadoblastoma ; Humans ; Mosaicism ; Turner Syndrome*

No abstract available.
Aneurysm* ; Coronary Aneurysm ; Coronary Vessels* ; Drug-Eluting Stents ; Stents* ; Ultrasonography, Interventional

Tumor lysis syndrome is one of the major oncological emergency. It causes complications such as acute kidney injury, cardiac arrhythmia, and death. However, it is rare in solid tumors. We report on a case of a patient with myoma of uterus who developed tumor lysis syndrome and acute kidney injury after high intensity focused ultrasound (HIFU) treatment. The potential complications of tumor lysis syndrome should be considered in HIFU treatment.
Acute Kidney Injury ; Arrhythmias, Cardiac ; Emergencies ; Humans ; Leiomyoma* ; Myoma ; Tumor Lysis Syndrome* ; Ultrasonography* ; Uterus

Treatment strategies for patients with coronary artery disease (CAD) should be based on objective evidence of inducible ischemia in the subtended myocardium to improve clinical outcomes, symptoms, and cost-effectiveness. Fractional flow reserve (FFR) is the most verified index to-date for invasively evaluating lesion-specific myocardial ischemia. Favorable results from large clinical trials that applied FFR-guided percutaneous coronary intervention (PCI) prompted changes in coronary revascularization guidelines to emphasize the importance of this ischemia-based strategy using invasive coronary physiology. However, the frequency of functional evaluations is lacking in daily practice, and visual assessment still dominates treatment decisions in CAD patients. Despite recent efforts to integrate functional and anatomical assessments for coronary stenosis, there is considerable discordance between the 2 modalities, and the diagnostic accuracy of simple parameters obtained from current imaging tools is not satisfactory to determine functional significance. Although evidence that supports or justifies anatomy-guided PCI is more limited, and FFR-guided PCI is currently recommended, it is important to be aware of conditions and factors that influence FFR for accurate interpretation and application. In this article, we review the limitations of the current anatomy-derived evaluation of the functional significance of coronary stenosis, detail considerations for the clinical utility of FFR, and discuss the importance of an integrated physiologic approach to determine treatment strategies for CAD patients.
Coronary Angiography ; Coronary Artery Disease ; Coronary Stenosis ; Humans ; Ischemia ; Myocardial Ischemia ; Myocardium ; Percutaneous Coronary Intervention ; Physiology ; Ultrasonography, Interventional

Treatment strategies for patients with coronary artery disease (CAD) should be based on objective evidence of inducible ischemia in the subtended myocardium to improve clinical outcomes, symptoms, and cost-effectiveness. Fractional flow reserve (FFR) is the most verified index to-date for invasively evaluating lesion-specific myocardial ischemia. Favorable results from large clinical trials that applied FFR-guided percutaneous coronary intervention (PCI) prompted changes in coronary revascularization guidelines to emphasize the importance of this ischemia-based strategy using invasive coronary physiology. However, the frequency of functional evaluations is lacking in daily practice, and visual assessment still dominates treatment decisions in CAD patients. Despite recent efforts to integrate functional and anatomical assessments for coronary stenosis, there is considerable discordance between the 2 modalities, and the diagnostic accuracy of simple parameters obtained from current imaging tools is not satisfactory to determine functional significance. Although evidence that supports or justifies anatomy-guided PCI is more limited, and FFR-guided PCI is currently recommended, it is important to be aware of conditions and factors that influence FFR for accurate interpretation and application. In this article, we review the limitations of the current anatomy-derived evaluation of the functional significance of coronary stenosis, detail considerations for the clinical utility of FFR, and discuss the importance of an integrated physiologic approach to determine treatment strategies for CAD patients.

Fistula formation between the upper urinary tract and cutaneous tissue is an uncommon complication in urogenital disease. Xanthogranulomatous pyelonephritis(XGP) is a severe, chronic infection of the renal parenchyma that is often associated with calculi and obstruction. We present a rare case of XGP with a nephrocutaneous fistula.
Calculi ; Fistula ; Pyelonephritis, Xanthogranulomatous ; Urinary Tract

PURPOSE: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. METHODS: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients. RESULTS: All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma. CONCLUSION: All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.
Adult ; Child* ; Cholangiocarcinoma ; Cholangitis, Sclerosing* ; Chungcheongnam-do ; Colitis, Ulcerative ; Colorectal Neoplasms ; Crohn Disease ; Female ; Follow-Up Studies ; Hematologic Tests ; Humans ; Incidence ; Inflammatory Bowel Diseases* ; Liver Cirrhosis ; Liver Transplantation ; Retrospective Studies

This article was inadvertently omitted Acknowledgments section for grant support.

Mycobacterium massiliense which is recognized as a separate species from M. abscessus is little known regarding its clinical patterns and the response to treatment. We present a case of a localized cutaneous infection due to M. massiliense of the sole associated with acupuncture. M. massiliense was identified via polymerase chain reaction-hybridization analysis. We treated the patient with single-drug therapy consisting of clarithromycin for 4 months and the patient showed a significant response to this treatment.
Acupuncture ; Clarithromycin ; Foot* ; Humans ; Mycobacterium*

Episodic angioedema with eosinophilia is characterized by recurrent angioedema, urticaria, fever, weight gain and hypereosinophilia. Although this syndrome shows similarities to idiopathic hypereosinophilic syndrome, it differs in that it has good prognosis and has no organ involvement. The pathogenesis is unclear, but IL-5 is thought to be major cytokine of pathogenesis. It has benign course with spontaneous remission or low-dose prednisolone treatment. Here, we describe a patient whose clinical and laboratory features are similar to those observed in episodic angioedema with eosinophilia.
Angioedema* ; Eosinophilia* ; Fever ; Humans ; Hypereosinophilic Syndrome ; Interleukin-5 ; Prednisolone ; Prognosis ; Remission, Spontaneous ; Urticaria ; Weight Gain