Histiocytic sarcoma is a rare hematologic malignancy, with very few cases of primary histiocytic sarcoma of the breast described in English scientific literature. Herein, we describe a case of primary histiocytic sarcoma of the breast in a 75-year-old woman, with no clinical history of malignant tumors, who presented with a palpable solitary breast mass. Microscopically, the resected breast mass showed large pleomorphic cells, some multinucleated giant cells, and admixed inflammatory components. The pleomorphic tumor cells further showed a diffuse, noncohesive growth pattern, an abundant eosinophilic cytoplasm, and strong and diffuse immunoreactivity for cluster of differentiation (CD) 68 and CD163. Furthermore, a whole-body positron-emission tomography/computed tomography using deoxy-2-[¹⁸F]fluoro-D-glucose performed after surgery showed no other masses or lesions. After surgical excision, the patient was followed up, and no evidence of tumor recurrence or metastasis was noted.
Aged ; Breast ; Cytoplasm ; Eosinophils ; Female ; Giant Cells ; Hematologic Neoplasms ; Histiocytes ; Histiocytic Sarcoma ; Humans ; Neoplasm Metastasis ; Recurrence

BACKGROUND: Vitamin K antagonist (VKA) to prevent thromboembolism in non-valvular atrial fibrillation (NVAF) patients has limitations such as drug interaction. This study investigated the clinical characteristics of Korean patients treated with VKA for stroke prevention and assessed quality of VKA therapy and treatment satisfaction. METHODS: We conducted a multicenter, prospective, non-interventional study. Patients with CHADS2 ≥ 1 and treated with VKA (started within the last 3 months) were enrolled from April 2013 to March 2014. Demographic and clinical features including risk factors of stroke and VKA treatment information was collected at baseline. Treatment patterns and international normalized ratio (INR) level were evaluated during follow-up. Time in therapeutic range (TTR) > 60% indicated well-controlled INR. Treatment satisfaction on the VKA use was measured by Treatment Satisfaction Questionnaire for Medication (TSQM) after 3 months of follow-up. RESULTS: A total of 877 patients (age, 67; male, 60%) were enrolled and followed up for one year. More than half of patients (56%) had CHADS2 ≥ 2 and 83.6% had CHA2DS2-VASc ≥ 2. A total of 852 patients had one or more INR measurement during their follow-up period. Among those patients, 25.5% discontinued VKA treatment during follow-up. Of all patients, 626 patients (73%) had poor-controlled INR (TTR < 60%) measure. Patients' treatment satisfaction measured with TSQM was 55.6 in global satisfaction domain. CONCLUSION: INR was poorly controlled in Korean NVAF patients treated with VKA. VKA users also showed low treatment satisfaction.
Atrial Fibrillation* ; Drug Interactions ; Follow-Up Studies ; Humans ; International Normalized Ratio ; Male ; Prospective Studies ; Risk Factors ; Stroke ; Thromboembolism ; Vitamin K* ; Vitamins*

BACKGROUND: Although it is rare for the fracture itself to become a life threatening injury in patients suffering from rib fracture, the lives of these patients are occasionally threatened by other associated injuries. Especially, early discovery of patients with rib fracture and intra-abdominal organ injury is extremely important to the prognosis. This study analyzed the link between rib fracture and intra-abdominal injury to achieve improved treatment. MATERIALS AND METHODS: Among trauma patients that had visited the hospital emergency room from January 2007 to December 2009, a retrospective study was conducted on 453 patients suffering from rib fracture due to blunt trauma. Rib fracture was classified according to location (left, right, and bilateral), and according to level (upper rib fracture [1-2nd rib], middle rib fracture [3-8th rib], and lower rib fracture [9-12th rib]). The researched data was statistically compared and analyzed to investigate the correlation between the location, level, and number of rib fracture and intra-abdominal organ injury. RESULTS: Motor vehicle injury was found to be the most common mechanism of injury with 208 cases (46%). Associated injuries accompanied with rib fracture were generated in 276 cases (61%). Intra-abdominal organ injury was discovered in 97 cases (21%). Liver injury was the most common intra-abdominal injury associated with rib fracture with 39 cases (40%), followed by spleen injury, with 23 cases (23%). Intra-abdominal injury according to level of rib fracture was presented as upper rib fracture in 11 cases (11%), middle rib fracture in 31 cases (32%), and lower rib fracture in 55 cases (57%), thus verifying that intra-abdominal injuries were commonly accompanied in lower rib fractures (p=0.03). In particular, significant increase of intra-abdominal injury was presented in fractures below the 8th rib (p=0.03). The number of intra-abdominal injuries requiring emergency operations was significantly higher in patients with more than 6 rib fractures (p=0.04). CONCLUSION: Intra-abdominal organ injury is more common in patients with lower rib fracture, especially fractures below the 8th rib. Intra-abdominal organ injuries generated in multiple rib fracture patients with more than 6 fractures significantly higher severity. These cases must be thoroughly inspected and carefully observed as there is possibility of emergency operation.
Abdomen ; Aluminum Hydroxide ; Carbonates ; Emergencies ; Humans ; Liver ; Motor Vehicles ; Prognosis ; Retrospective Studies ; Rib Fractures ; Ribs ; Spleen ; Stress, Psychological

We present a case of persistent left superior vena cava, anomalous right pulmonary venous connection to the right-sided superior vena cava and sinus venosus atrial septal defect detected by computed tomography (CT) pulmonary angiography and echocardiography. These defects were surgically corrected using a double-patch technique. In fact, CT can provide anatomical information about a complex anomaly in the systemic and pulmonary vasculatures. Though modern imaging techniques are useful for detecting complex cardiovascular disease, careful echocardiographic examination should be performed to diagnose complex cardiac anomalies.
Angiography ; Cardiovascular Diseases ; Echocardiography ; Heart Septal Defects, Atrial ; Vascular Diseases ; Vena Cava, Superior

We present a case of persistent left superior vena cava, anomalous right pulmonary venous connection to the right-sided superior vena cava and sinus venosus atrial septal defect detected by computed tomography (CT) pulmonary angiography and echocardiography. These defects were surgically corrected using a double-patch technique. In fact, CT can provide anatomical information about a complex anomaly in the systemic and pulmonary vasculatures. Though modern imaging techniques are useful for detecting complex cardiovascular disease, careful echocardiographic examination should be performed to diagnose complex cardiac anomalies.
Angiography ; Cardiovascular Diseases ; Echocardiography ; Heart Septal Defects, Atrial ; Vascular Diseases ; Vena Cava, Superior

OBJECTIVES: Autism spectrum disorder (ASD) is a neurodevelopmental disorder that is characterized by abnormalities of social functioning, communication and behavior. The association of the 7q21-34 region with ASD has been reported. The DLX6 gene, which is located at the 7q22 region, is one of the positional and functional candidate genes for ASD. We found that there is no association between DLX6 polymorphisms and ASD in the Korean male population. METHODS: We selected three single nucleotide polymorphisms (SNPs) that might be implicated in the change of the DLX6 gene expression. The genomic DNA was collected from the venous blood of 147 male controls and 179 male patients with ASD. The genotypes of the selected SNPs were determined using the Illumina GoldenGate assay, and the statistical analyses were performed using HapAnalyzer software and SAS Enterprise. RESULTS: We found no association of the three SNPs in the DLX6 gene with ASD in the Korean male population. CONCLUSION: Our study suggests that the three SNPs in the DLX6 gene are not associated with ASD, and we need to analyze the previously reported regions for their associations with ASD.
Autistic Disorder ; Child ; Autism Spectrum Disorder ; DNA ; Gene Expression ; Genotype ; Humans ; Male ; Phenothiazines ; Polymorphism, Single Nucleotide

Systemic infection with Aspergillus is an opportunistic disease that affects mainly immunocompromised hosts, and is associated with a high mortality rate. It typically occurs in patients with several predisposing factors, but Aspergillus endocarditis of native valves is rare and experience in diagnosis and treatment is limited. We report a case of native valve endocarditis caused by Aspergillus. A 35-yr-old male patient who underwent pericardiocentesis four months previously for pericardial effusion of unknown etiology presented with right leg pain and absence of the right femoral artery pulse. Cardiac echocardiography revealed severe mitral insufficiency with large mobile vegetations, and computed tomographic angiography showed embolic occlusion of both common iliac arteries. We performed mitral valve replacement and thromoembolectomy, and Aspergillus was identified as the vegetation. We started intravenous amphotericin B and oral itraconazole, but systemic complications developed including superior mesenteric artery aneurysm and gastrointestinal bleeding. After aggressive management, the patient was discharged 78 days post surgery on oral itraconazole. He was well at 12 months post discharge but died in a traffic accident 13 months after discharge.
Administration, Oral ; Adult ; Amphotericin B/administration & dosage ; Antifungal Agents/administration & dosage ; Aspergillosis/complications/*diagnosis/drug therapy ; Aspergillus/isolation & purification ; Endocarditis/*diagnosis/surgery/ultrasonography ; Heart Valve Diseases/*diagnosis/microbiology/surgery ; Humans ; Itraconazole/administration & dosage ; Male ; Postoperative Complications/microbiology ; Tomography, X-Ray Computed

BACKGROUND: Recently, percutaneous cardiopulmonary support (PCPS) has been widely used to rescue patients in cardiogenic shock or cardiac arrest. However, patients with cardiopulmonary bypass (CPB) weaning failure during open heart surgery still have very poor outcomes after PCPS. We investigated clinical results and prognostic factors for patients who underwent PCPS during open heart surgery. MATERIAL AND METHOD: From January 2005 to December 2008, 10 patients with CPB weaning failure during open heart surgery underwent PCPS using the CAPIOX emergency bypass system (EBS(R), Terumo Inc, Tokyo, Japan). We retrospectively reviewed the medical records of those 10 patients. RESULT: The average age of the patients was 60.2+/-16.5 years (range, 19~77 years). The mean supporting time was 48.7+/-64.7 hours (range, 4~210 hours). Of the 10 patients, 6 (60%) were successfully weaned from the PCPS While 5 (50%) were able to be discharged from the hospital. Complications were noted in 5 patients (50%). In univariate analysis, long aortic cross clamp time during surgery, mediastinal bleeding during PCPS and high level of Troponin-I before PCPS were significant risk factors. All of the discharged patients are still surviving 34+/-8.6 months (range, 23~48 months) post-operatively. CONCLUSION: The use of PCPS for CPB weaning failure during open heart surgery can improve the prognosis. More experience and additional clinical studies are necessary to improve survival and decrease complications.
Cardiopulmonary Bypass ; Emergencies ; Extracorporeal Circulation ; Heart ; Heart Arrest ; Hemorrhage ; Humans ; Medical Records ; Prognosis ; Retrospective Studies ; Risk Factors ; Shock, Cardiogenic ; Thoracic Surgery ; Tokyo ; Troponin I ; Weaning

It has been reported that the immune response due to alpha-Gal epitopes is an important factor in tissue valve failure. The elimination of the interaction between the natural anti-Gal antibodies and alpha-gal epitopes on the xenografts is a prerequisite to the success of xenografts in humans. Previously, we reported that the green coffee bean alpha-galactosidase could remove all alpha-Gal epitopes from cell surface of porcine aortic valve and pericardial tissue, but it has limitations on cost effectiveness. In this study we wanted to know whether the recently produced recombinant human alpha-galactosidase A has the same effective enzymatic activity as green coffee bean alpha-galactosidase in removing alpha-Gal epitopes from the same tissues. After treating fresh porcine aortic valve and pericardial tissue with recombinant alpha-galactosidase A, each sample was stained with Griffonia simplicifolia type I isolectin B4 indirect immunoperoxidase avidin-biotin technique. We then examined whether the alpha-Gal epitopes were reduced or abolished in each consecutive concentration of recombinant alpha-galactosidase A by comparing the degree of the Griffonia simplicifolia isolectin B4 staining. As a result, the recombinant alpha-galactosidase A could remove cell surface alpha-Gals on porcine aortic valve and pericardial tissue as effectively as green coffee bean alpha-galactosidase.
Adolescent ; Animals ; Aortic Valve/chemistry/cytology/*immunology ; Child ; Coffea/enzymology ; Epitopes/*immunology ; Heart Valve Prosthesis Implantation ; Humans ; Pericardium/chemistry/cytology/*immunology ; Recombinant Proteins/genetics/*immunology ; Swine ; Transplantation, Heterologous/immunology ; alpha-Galactosidase/genetics/*immunology

BACKGROUND: Percutaneous cardiopulmonary support (PCPS) has the potential to rescue patients in cardiogenic shock who might otherwise die. PCPS has been a therapeutic option in a variety of the clinical settings such as for patients with myocardial infarction, high-risk coronary intervention and postcardiotomy cardiogenic shock, and the PCPS device is easy to install. We report our early experience with PCPS as a life saving procedure in cardiogenic shock patients due to acute myocardial infarction. MATERIAL AND METHOD: From January 2005 to December 2006, eight patients in cardiogenic shock with acute myocardial infarction underwent PCPS using the CAPIOX emergency bypass system (EBS(R), Terumo, Tokyo, Japan). Uptake cannulae were inserted deep into the femoral vein up to the right atrium and return cannulae were inserted into the femoral artery with Seldinger techniques using 20 and 16-French cannulae, respectively. Simultaneously, autopriming was performed at the EBS(R) circuit. The EBS? flow rate was maintained between 2.5~3.0 L/min/m2 and anticoagulation was performed using intravenous heparin with an ACT level above 200 seconds. RESULT: The mean age of patients was 61.1+/-14.2 years (range, 39 to 77 years). Three patients were under control of the EBS? before percutaneous coronary intervention (PCI), three patients were under control of the EBS? during PCI, one patient was under control of the EBS after PCI, and one patient was under control of the EBS(R) after coronary bypass surgery. The mean support time was 47.5+/-27.9 hours (range, 8 to 76 hours). Five patients (62.5%) could be weaned from the EBS(R) after 53.6+/-27.2 hours (range, 12 to 68 hours) of support. All of the patients who could successfully be weaned from support were discharged from the hospital. There were three complications: one case of gastrointestinal bleeding and two cases of acute renal failure. Two of the three mortality cases were under cardiac arrest before EBS(R) support, and one patient had an intractable ventricular arrhythmia during the support. All of the discharged patients are still surviving at 16.8+/-3.1 months (range, 12 to 20 months) of follow-up. CONCLUSION: The use of EBS(R) for cardiogenic shock caused by an acute myocardial infarction could rescue patients who might otherwise have died. Successfully recovered patients after EBS(R) treatment have survived without severe complications. More experience and additional clinical investigations are necessary to elucidate the proper installation timing and management protocol of the EBS? in the future.
Acute Kidney Injury ; Arrhythmias, Cardiac ; Catheters ; Emergencies ; Femoral Artery ; Femoral Vein ; Follow-Up Studies ; Heart Arrest ; Heart Atria ; Hemorrhage ; Heparin ; Humans ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Shock, Cardiogenic ; Tokyo