Amyloidosis can be identified by the deposition of amyloid fibrils in biopsy specimens from multiple organs, including the heart, kidney, skin, and bowel. Systemic amyloid protein A amyloidosis (AA amyloidosis) is commonly associated with chronic inflammatory diseases or chronic infectious conditions. Cardiac involvement in AA amyloidosis is found in < 1% of reported cases. Here, we report a case of cardiac AA amyloidosis confirmed by heart biopsy in a 54-year-old-female with a medical history of rheumatoid arthritis and stage 4 chronic kidney disease due to renal amyloidosis. She had suffered from progressive aggravation of dyspnea for 2 years. Infiltrative disease involving the heart was suspected by echocardiography, and the patient was diagnosed with AA amyloidosis involving the heart by cardiac biopsy. This is a rare case of cardiac involvement in a patient with systemic AA amyloidosis associated with rheumatoid arthritis.
Amyloid* ; Amyloidosis* ; Arthritis, Rheumatoid* ; Biopsy ; Dyspnea ; Echocardiography ; Heart Failure ; Heart* ; Humans ; Kidney ; Renal Insufficiency, Chronic ; Rheumatic Fever ; Skin ; Staphylococcal Protein A*

This study was performed to compare the 2 different portable devices measuring fractional exhaled nitric oxide (FeNO) and to see the correlation between FeNO and induced sputum eosinophil count (ISE). Forty consecutive subjects clinically suspected to have asthma underwent FeNO measurement by NIOX-MINO(R) and NObreath(R) concurrently. All also had induced sputum analysis, methacholine provocation test or bronchodilator response test, and spin prick test. Agreement between the 2 devices was evaluated. The correlation between FeNO and ISE was assessed, as well as the cut-off level of FeNO to identify ISE > or =3%. The intraclass correlation coefficient (ICC) between FeNO levels measured by NIOX-MINO(R) (FeNO(NIOX-MINO)) and NObreath(R) (FeNO(NObreath)) was 0.972 with 95% confidence interval of 0.948-0.985. The 95% limits of agreement were -28.9 to 19.9 ppb. The correlation coefficient between ISE and FeNO(NIOX-MINO) was 0.733 (P<0.001), and 0.751 between ISE and FeNO(NObreath) (P<0.001). The ROC curve found that the FeNO(NIOXMINO) of 37.5 ppb and the FeNO(NObreath) of 36.5 ppb identified ISE > or =3% with 90% sensitivity and 81% specificity. Age, sex, body mass index, smoking history, atopy, and the presence of asthma did not affect the FeNO level and its correlation with ISE. The NIOX-MINO (R) and NObreath(R) agree with each other to a high degree. Both devices showed close correlation with ISE with similar cut-off value in identifying ISE > or =3%.
Asthma ; Body Mass Index ; Electrochemical Techniques ; Eosinophils* ; Methacholine Chloride ; Nitric Oxide* ; ROC Curve ; Smoke ; Smoking ; Sputum*

A 53-yr-old man underwent radiofrequency ablation to treat persistent atrial flutter. After the procedure, the chest pain was getting worse, and the electrocardiogram showed ST-segment elevation in inferior leads with reciprocal changes. Immediate coronary angiography showed total occlusion with thrombi at the distal portion of the right coronary artery, which was very close to the ablation site. Intervention with thrombus aspiration and balloon dilatation was successful, and the patient recovered without any kind of sequelae. Although the exact mechanism is obscure, the most likely explanation is a thermal injury to the vascular wall that ruptured into the lumen and formed thrombus. Vasospasm and thromboembolism can also be other possibilities. This case raise the alarm to cardiologists who perform radiofrequency ablation to treat various kinds of cardiac arrhythmias, in that myocardial infarction has been rarely considered one of the complications.
Acute Disease ; Angioplasty, Balloon, Coronary ; Atrial Flutter/*surgery ; Catheter Ablation/*adverse effects ; Chest Pain/etiology ; Coronary Occlusion/etiology ; Coronary Vessels/radiography ; Humans ; Male ; Middle Aged ; Myocardial Infarction/*diagnosis/etiology/therapy ; Thrombosis/surgery

Methcillin-resistant Staphylococcus aureus (MRSA) has emerged as an important cause of community-acquired infections, which has been recently designated as community-associated (CA) MRSA. Panton-Valentine leukocidin (PVL)-negative multilocus sequence type 72 (ST72)-staphylococcal cassette chromosome mec (SCCmec) type IV has been reported as the predominat CA-MRSA strain in Korea and is commonly associated with skin and soft tissue infections in addition to healthcare-associated pneumonia. However, community-acquired pneumonia (CAP) for this strain has not yet been reported. We hereby report two cases of CAP caused by PVL-negative ST72-SCCmec type IV strain in patients who had no risk factors for MRSA acquisition. While CA-MRSA infections are not yet prevalent in Korea, our cases suggest that CA-MRSA should be considered in cases of severe CAP, especially for cases associated with necrotizing pneumonia.
Bacterial Toxins ; Community-Acquired Infections ; Exotoxins ; Humans ; Korea ; Leukocidins ; Methicillin-Resistant Staphylococcus aureus ; Pneumonia ; Risk Factors ; Skin ; Soft Tissue Infections ; Sprains and Strains ; Staphylococcus ; Staphylococcus aureus

Drug-induced anaphylaxis is a big pitfall in patients receiving antineoplastic chemotherapy. We report a case of lung cancer patient who experienced two near-fatal anaphylactic reactions that resulted from paclitaxel and multivitamin, seperately. Recurrent severe reactions to different agents led to further investigation to which material the patient was hypersensitive. The skin prick test revealed sensitization to cremophor, which is a commonly used emulsifying agent. This case emphasizes the importance of correctly identifying the culprit drug of anaphylaxis to avoid potentially fatal reaction.
Anaphylaxis* ; Drug Therapy ; Humans ; Hypersensitivity ; Lung Neoplasms ; Paclitaxel ; Skin

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.
Aged ; Aorta, Abdominal/pathology ; Diagnosis, Differential ; Echocardiography ; Female ; Humans ; Immunoglobulin G/*blood/immunology ; Magnetic Resonance Imaging ; Mitral Valve Stenosis/diagnosis ; Myocardium/*pathology ; Peritoneum/surgery ; Positron-Emission Tomography ; Retroperitoneal Fibrosis/*congenital/diagnosis/drug therapy/ultrasonography ; Steroids/therapeutic use ; Tomography, X-Ray Computed

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. It is characterized by peripheral eosinophilia, asthma, neuropathy, skin manifestation, and less frequently gastrointestinal tract symptoms. Here we report a case of CSS which is initially manifested as acute acalculous cholecystitis. A 67-year-old asthmatic woman visited Emergency Room because of acute abdominal pain. Computed tomography showed acalculous cholecystitis and laparoscopic cholecystectomy was done. Pathology of gallbladder showed marked eosinophilic infiltration with necrotizing vasculitis and granuloma. On the basis of asthma, sinusitis, lung infiltration, combined peripheral eosinophilia and neuropathy, the patient was diagnosed as CSS. To our knowledge, this is the first case of pathologically proven eosinophilic cholecystitis in Korea.
Abdominal Pain ; Acalculous Cholecystitis* ; Aged ; Asthma ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Churg-Strauss Syndrome* ; Emergencies ; Eosinophilia ; Eosinophils ; Female ; Gallbladder ; Gastrointestinal Tract ; Granuloma ; Humans ; Korea ; Lung ; Pathology ; Sinusitis ; Skin Manifestations ; Systemic Vasculitis ; Vasculitis

BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed. RESULTS: Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2–18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p<0.001), respectively. The peripheral neuropathy relapsed in one patient. CONCLUSIONS: The long-term clinical outcome of peripheral neuropathy in patients with EGPA receiving initial corticosteroid and cyclophosphamide combination therapy was favorable with a very low relapse rate.
Asthma ; Cyclophosphamide ; Eosinophilia ; Eosinophils* ; Follow-Up Studies ; Granulomatosis with Polyangiitis* ; Humans ; Inflammation ; Medical Records ; Peripheral Nerves ; Peripheral Nervous System Diseases* ; Prognosis ; Recurrence ; Vasculitis

PURPOSE: Toxocariasis is the most common cause of peripheral blood eosinophilia in Korea and produces eosinophilic infiltration in various organs, including the lung. However, the prevalence of toxocariasis in the general population is rarely reported. METHODS: We investigated the seroprevalence of Toxocara larval antibody among asymptomatic people who attended Samsung Medical Center for a health checkup, including low-dose chest computed tomography (CT) between March 2012 and December 2013. A total of 633 people (400 men and 233 women) were prospectively recruited. RESULTS: The Toxocara-seropositive rate was 51.2% using the current cutoff value based on Toxocara enzyme-linked immunosorbent assay (ELISA) (67.0% for men and 24.0% for women). In the multivariate-adjusted model, age (odds ratio [OR], 1.08; 95% confidence intervals [CI], 1.04-1.11), male sex (OR, 3.47; 95% CI, 2.26-5.33), rural residence (OR, 1.55; 95% CI, 1.05-2.30), and history of raw liver intake (OR, 8.52; 95% CI, 3.61-20.11) were significantly associated with Toxocara seropositivity. When subjects were divided into 3 groups using cutoff values base on weak positive and strong positive control optical densities (ODs), the ORs for peripheral blood eosinophilia and serum hyperIgEaemia were 0.31 (95% CI, 0.02-2.89) in the weakpositive group and 36.64 (95% CI, 11.73-111.42) in the strong positive group compared to the seronegative group. Similarly, ORs for the solid nodule with surrounding halo were 2.54 (95% CI, 0.60-10.84) in the weak positive group and 15.08 (95 CI 4.09-55.56) in the strong positive group compared to the seronegative group. CONCLUSIONS: The study indicated that the Toxocara-seropositive rate obtained by using the current cutoff value based on ELISA was high in the asymptomatic population in Korea. The results of this study suggest that active toxocariasis may be more frequently seen in the Toxocara-strong positive group than in the Toxocara-weak positive group.
Adult* ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Eosinophilia ; Eosinophils ; Humans ; Korea ; Liver ; Lung ; Male ; Prevalence* ; Prospective Studies ; Seroepidemiologic Studies ; Serologic Tests* ; Thorax ; Toxocara ; Toxocara canis ; Toxocariasis*

BACKGROUND/AIMS: Crohn's disease is a chronic inflammatory bowel disease. Stricture is a very important indication for surgical intervention as strictures can lead to intestinal obstruction. Strictures can be divided into inflammatory and fibrous strictures. Intestinal obstruction due to inflammatory stricture is expected to be resolved with medical treatment. However, factors that can predict the response to medical treatments are unknown. In the present study, we aimed to identify the factors that can predict the response to medical treatments in Crohn's disease patients with intestinal obstruction. METHODS: Data were collected by retrospectively reviewing the medical records of patients with Crohn's disease who visited the emergency department at Samsung Medical Center in Seoul from January 1, 2000 to December 31, 2010 because of intestinal obstruction. Based on the response to medical treatments, we classified the patients as responders and non-responders and compared the clinical, biochemical, and radiological findings of the two groups. RESULTS: A total of 39 patients were enrolled. Twenty-nine patients responded to medical treatments whereas 10 patients did not. Significant differences were observed between the two groups in terms of vomiting and duration of disease before the development of obstruction. CONCLUSIONS: Patients who responded to the medical treatments exhibited a higher incidence of vomiting and longer duration of disease before the development of obstruction. However, further prospective studies are needed to identify the factors that can predict the response to medical treatments.
Adolescent ; Adult ; Anti-Inflammatory Agents, Non-Steroidal/*therapeutic use ; C-Reactive Protein/analysis ; Crohn Disease/*complications/*drug therapy/radiography ; Female ; Follow-Up Studies ; Humans ; Intestinal Obstruction/*etiology/surgery ; Leukocytes/cytology ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome ; Young Adult