Objective Echocardiography is used for the detection of cardiac sarcoid involvement in patients with non-cardiac sarcoidosis. Little information is available regarding temporal changes in left ventricular ejection fraction (LVEF) and left ventricular end-diastolic dimension (LVDd) in non-cardiac sarcoidosis patients. Methods and Results Fifty-four sarcoidosis patients who received periodic follow-up with echocardiography at our institute were enrolled in this study. At the time of initial ultrasonography, 13 patients were diagnosed with cardiac sarcoid involvement. All of the remaining 41 patients with extra-cardiac sarcoidosis only had a LVEF of >50%. During the median follow-up period of 39 months, two (4.9%) of the non-cardiac sarcoidosis patients were diagnosed with cardiac sarcoid involvement; one patient showed a progressive decline in the LVEF over a short period of time. It was also found that two of 41 non-cardiac sarcoidosis patients showed declines in the LVEF of >10% per year; however, they were not diagnosed with cardiac sarcoidosis during the follow-up period. Conclusion Rapid deterioration of left ventricular function may increase the suspicion of sarcoid involvement of the heart in non-cardiac sarcoidosis patients; however, we must be aware that a certain subfraction of patients may not demonstrate significant abnormalities in LVEF or LVDd on periodic echocardiographic follow-up.
Objective To investigate the association between low free triiodothyronine (fT3) levels and the severity and prognosis of patients with acute myocardial infarction. Methods A total of 501 patients with acute myocardial infarctions were enrolled in our study. The circulating levels of thyroid hormones and clinical parameters were assayed. The patients were categorized into either the low fT3 group or the normal fT3 group according to the fT3 level on admission. All patients underwent a follow-up for 10±2 months for mortality from any cause and the occurrence of any adverse major cardiac events (MACE). Results There were 171 patients in the low fT3 group (fT3<3.5 pmol/L) and 330 patients in the normal fT3 group (≥3.5 pmol/L). During the follow-up period, 33 patients died (6.6%) and the overall survival rates were 86.0% and 97.3% in patients with a low fT3 level and a normal fT3 level, respectively. The rates of MACE were 66.7% and 45.5% in the patients with and those without low fT3 levels, respectively. Using a multivariable Cox proportional hazards model, the fT3 level was found to be the most important predictor of cumulative death and MACE (hazard ratio [HR] for death: 0.142, p<0.001 and HR for major adverse cardiac events: 0.748, p=0.007). A Kaplan-Meier analysis revealed that those patients with low fT3 levels had higher rates of MACE and death. Conclusion A low fT3 level, a common phenomenon in patients with acute myocardial infarctions, is a strong predictor of short-term and long-term poor prognoses in patients with acute myocardial infarctions.
Objective The aim of this study was to determine whether the absence of coronary artery calcium (CAC) can safely exclude obstructive coronary artery disease (CAD) in asymptomatic patients with type 2 diabetes. Methods We enrolled 478 consecutive asymptomatic patients with type 2 diabetes who visited the diabetes clinic of the Asan Medical Center between October 1, 2009 and December 31, 2010. All patients underwent 64-slice dual-source computed tomography (DSCT) for CAC scoring as well as computed tomography angiography (CTA). Patients with at least one significant coronary stenosis with >50% luminal narrowing were classified as having obstructive CAD. The findings were confirmed using conventional coronary angiography (CAG). Results Among the 478 patients, 157 (33%) had a CAC score of 0 (CAC=0). Of these, 17 (11%) had obstructive CAD confirmed on CAG. The presence of CAC had a negative predictive value for obstructive CAD on CAG of 89% and a sensitivity of 88%, a specificity of 42% and a positive predictive value of 38%. A multivariate logistic regression analysis showed that current smoking habits were significantly associated with the presence of obstructive CAD in patients with CAC=0 after adjusting for traditional cardiovascular risk factors (odds ratio 4.87, 95% confidence interval 1.65-14.42, p=0.004). Conclusion Our findings suggest that CAC=0 on 64-slice DSCT cannot safely exclude obstructive CAD on CAG in asymptomatic patients with type 2 diabetes, particularly in current smokers. CTA should be combined with CAC scoring in screening for CAD in asymptomatic patients with type 2 diabetes.
We herein report a rare case of hepatocellular carcinoma (HCC) with sarcomatous changes. A 66-year-old man was admitted to our hospital with a high fever and upper abdominal pain. Initially, he was diagnosed as having a liver abscess; however, antibiotic treatment and drainage were ineffective. Further imaging studies revealed the typical appearance of HCC: the tumor had invaded the hepatic and portal veins. Surgical resection of the tumor was performed. A pathological examination demonstrated the presence of a sarcomatous hepatocellular carcinoma. Sarcomatous hepatocellular carcinoma with remittent fever is a rare disease entity.
We herein report the case of a 39-year-old woman with a pulmonary embolism caused by intimal sarcoma of the pulmonary artery. She presented with shortness of breath and leg edema. Computed tomography showed a low density area that extended from the main pulmonary artery to the bilateral pulmonary arteries. We diagnosed her to have a pulmonary thromboembolism. The thrombosis did not decrease after the administration of anti-coagulant therapy, and she underwent resection of the thrombotic tissue. Histopathologically, the surgical specimen was not found to be thrombotic tissue but rather an intimal sarcoma of the pulmonary artery. After undergoing surgery, she received radiation therapy and chemotherapy; however, she died 31 months after being diagnosed.
A 43-year-old man was admitted to our hospital presenting with seizures and syncope. He had a history of a cold with a fever of 39°C occurring three days earlier. Electrocardiography (ECG) showed complete atrioventricular block (AV block) with a maximum pause of 32 seconds, for which temporary pacing was performed. Echocardiography showed mild hypertrophy of the left ventricle (LV) with a normal ejection fraction of 61%. Coronary angiography showed normal coronary arteries. Then, an endomyocardial biopsy was performed, the results of which indicated a diagnosis of acute myocarditis. After admission, the complete atrioventricular block disappeared together with normalization of the LV wall thickness.
Inherited hypercoagulopathies such as protein C and S deficiency usually lead to the formation of venous thrombi in clinical practice; however, they rarely lead to arterial thrombosis. It has been demonstrated that both protein C and S deficiency may lead to myocardial infarctions. However, our literature review revealed no reports of left main coronary artery thrombi caused by protein C and S deficiency. This paper presents a case of a left main coronary artery thrombus resulting from protein C and S deficiency in a young patient with normal coronary arteries.
We herein report two cases of pancreatitis associated with incretin-based therapies in end-stage renal disease (ESRD) patients undergoing dialysis. A 75-year-old woman with a history of liraglutide use and a 68-year-old man with a history of vildagliptin use both presented with nausea. They showed elevated levels of pancreatic enzymes and pancreatic tail swelling on CT. Their symptoms improved after discontinuing the drugs. In the absence of any obvious secondary causes of pancreatitis, we believe that the pancreatitis observed in these cases was associated with the incretin-based therapies. Few reports have been published on the safety and efficacy of incretin-based therapies in ESRD patients, and it remains uncertain whether the changes in the pancreas observed in the present cases are characteristic of ESRD patients.
We herein present a case of serial opportunistic infections that included disseminated nocardiosis and cryptococcal meningitis in a 67-year-old man who was diagnosed with ANCA-associated vasculitis and treated with corticosteroids. Upon admission, the initial manifestations of the disease included subcutaneous tumors and multiple lesions in the brain and lungs. Nocardia farcinica was identified in a culture of the aspirated pus. The patient was successfully treated for disseminated nocardiosis with antibiotics. However, three months after discharge, he was hospitalized with complaints of nuchal pain. Cryptococcus neoformans was identified on a culture of the cerebrospinal fluid. Anti-fungal treatment resulted in the remission of cryptococcal meningitis.
A 42-year-old woman who underwent renal transplantation from her mother at the age of 26 due to IgA nephropathy had since been treated with immunosuppressive agents, including prednisolone (PSL), azathioprine (AZA) and cyclosporine (CsA). The patient had remained clinically stable for 15 years. However, in the middle of May 2010, she developed bronchial asthma for the first time after performing house-cleaning activities and was treated with corticosteroids and antiasthmatic agents. The use of immunosuppressive agents as a treatment for severe bronchial asthma might have been related to the manifestation of bronchial asthma in this case.
Multicentric Castleman's Disease (MCD) is a systemic disease characterized by generalized lymphadenopathy and the proliferation of plasma cells. The development of MCD in a patient with preexisting sarcoidosis has not been previously reported. We herein describe a case of MCD developing in a 78-year-old woman with a 17-year history of sarcoidosis. The patient's serum interleukin-6 (IL-6) levels were only slightly elevated; however, the IL-6 levels in the fluid of both pleural effusion and ascites were markedly elevated. The administration of steroid-pulse therapy and prednisolone was ineffective in treating the MCD, although treatment with tocilizumab proved highly effective.
Hemophagocytic lymphohistocytosis (HLH) is commonly associated with infectious diseases or T/NK cell-lymphoma; however that with Hodgkin lymphoma (HL) was rarely reported. Herein, we describe a young male diagnosed with lymphocyte-depleted HL (LD-HL) complicated by HLH as an initial manifestation. He was given high-dose steroid therapy plus recombinant thrombomodulin, and subsequent ABVd (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment. In spite of the achievement of a partial remission treated with one cycle of ABVd, he relapsed after 3 cycles. To our knowledge, the present case is very rare, and more intensive treatment might be needed for the long-term control of HLH-complicated HL.
Viridans streptococcus, an indigenous bacterial species of the mouth and gastrointestinal tract, is thought to be a rare cause of bacterial meningitis. The type of streptococcus involved is important because each type causes a different kind of meningitis and is associated with a different outcome. A 39-year-old previously healthy man was admitted due to the onset of acute purulent meningitis. A cerebrospinal fluid culture grew Streptococcus sanguis (S. sanguis). Although the patient was asymptomatic for dental caries, odontogenic maxillary sinusitis was found to be the cause of the meningitis. Treatment with intravenous antibiotics was successful. Following a review of the pertinent literature, we discuss the characteristics of S. sanguis meningitis.
We herein report a case of ocular myasthenia gravis (MG) that was highly positive for anti-muscle-specific tyrosine kinase (MuSK) antibodies. The examined patient exhibited bilateral ptosis and lateral gaze palsy without any generalized symptoms and was diagnosed with ocular MG with anti-MuSK antibodies. She responded to treatment with prednisolone and immunosuppressants and experienced only ocular symptoms for four years and eight months after onset. Ocular MG with anti-MuSK antibodies lasting for a long term has rarely been described. Our findings suggest that it may be reasonable to test for the presence of anti-MuSK antibodies in patients who present with external ophthalmoplegia.
We herein report the case of a patient who presented with an acute decrease of visual acuity, hypertension, focal seizures and transient mental dysfunction while undergoing desmopressin treatment. Neuroimaging revealed bilateral occipital-parietal lesions that presented with vasogenic edema. After controlling the hypertension and discontinuing the desmopressin treatment, the patient's condition improved. A follow-up imaging examination performed six months later showed complete resolution of the lesions. It is important to recognize posterior reversible encephalopathy syndrome (PRES) as a rare and serious complication of desmopressin administration. Both the blood pressure and water electrolyte balance should be carefully monitored in patients receiving desmopressin therapy.
We herein report a case of toxic shock syndrome (TSS) associated with the 2009 pandemic H1N1 (pH1N1) influenza virus and a community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infection in a 16-year-old Vietnamese girl. Staphylococcal enterotoxin B (SEB) was detected in the patient's serum, and the level of anti-SEB antibodies was found to be elevated. A flow cytometric analysis showed evidence of activated SEB-reactive Vβ3+ and Vβ12+ T cells. These data suggest that the CA-MRSA-induced activation of SEB-reactive T cells may cause TSS in patients with pH1N1 virus infection. Moreover, this is the first report describing immunological confirmation of SEB contributing directly to TSS in a patient fulfilling the diagnostic criteria of TSS.
A 66-year-old man with human immunodeficiency virus (HIV) infection was admitted for treatment of Pneumocystis pneumonia. Upon admission, a tumor mass adjacent to the thoracic descending aorta was revealed on computed tomography. Histology revealed an exudative granuloma with histiocytes packed with numerous acid-fast bacilli. Mycobacterium avium was isolated from the tissue. A genetic examination of the isolates demonstrated this strain to be located in the cluster consisting of strains that cause systemic infection. The patient's baseline CD4+ cell count was 9 /μL and the HIV-RNA viral load was 43,800 copies/mL. This case suggests the possibility of a localized onset of disseminated M. avium infection.
A 70-year-old woman with an increased uptake of 18-Fluorodeoxyglucose (FDG) in whole liver on positron emission tomography (PET) was referred to our hospital. Laboratory examinations showed increased serum levels of total immunoglobulin G (IgG) and IgG4. Gallium scintigraphy showed a remarkable uptake in the liver but not in any other organs. On computed tomography (CT) and magnetic resonance imaging (MRI), multiple foci of abnormal density were observed in the liver, but the pancreas and bile duct lacked any indications of IgG4-related sclerosing disease. A liver biopsy specimen revealed multiple non-necrotizing granulomas. This is the first report of hepatic sarocidosis in a patient with an elevated serum level of IgG4.