The HACEK group of microorganisms is responsible for approximately 3-6% of endocarditis cases and is a major cause of culture-negative endocarditis. Here, we report a case of Haemophilus parainfluenzae infective endocarditis that was diagnosed by direct PCR sequencing of 16S rRNA from resected vegetation. A healthy 26-yr-old man was admitted to the emergency room (ER) on March 27, 2011 because of intermittent high fever. The patient was prescribed cefpodoxime for 5 days at the ER. Six and 11 sets of blood cultures were performed at the ER and in a general ward, respectively, using BACTEC Plus Aerobic/F (Becton-Dickinson, USA) and Lytic Anaerobic/F Plus (BD) together. Echocardiography revealed a large vegetation at the posterior mitral valve leaflet. After performing mitral valvoplasty on hospital day (HD) 11, the vegetation tissue was cultured in thioglycolate broth, blood agar, Brucella agar, and MacConkey agar for 7 days, but no organism was grown. Direct PCR sequencing of 16S rRNA of the tissue revealed the presence of H. parainfluenzae. In the 17 sets of blood cultures, bacterial growth was detected in only 2 aerobic bottles of 5 sets taken at HD 9 after 10-day and 14-day incubation. The organism was identified as H. parainfluenzae by using the VITEK NHI card (bioMerieux, France). Direct PCR sequencing of vegetation could be useful in diagnosing bacterial pathogens in infective endocarditis patients, especially in culture-negative cases.
Agar ; Brucella ; Ceftizoxime ; Echocardiography ; Emergencies ; Endocarditis ; Fever ; Haemophilus ; Haemophilus parainfluenzae ; Humans ; Mitral Valve ; Paramyxoviridae Infections ; Patients' Rooms ; Polymerase Chain Reaction ; Sequence Analysis, RNA

Bacteria belonging to the genus Tsukamurella are aerobic, gram-positive rods that are weakly acid-fast with no apparent branching. Infections of the Tsukamurella spp. are generally caused by the use of infected medical devices such as central venous catheters. The underreporting of these infections might be attributable to the frequent misdiagnosis of Tsukamurella infections as Corynebacterium or atypical Mycobacterium spp. infections. Therefore, when gram-positive aerobic rods are observed in the blood culture of a patient with a central venous catheter, it is important to consider Tsukamurella as one of the causative organisms. Here, we report the first case of a catheter-related blood stream infection caused by Tsukamurella inchonensis in a 3-yr-old Korean girl with underlying biliary atresia who underwent hepatoportoenterostomy.
Bacteria ; Biliary Atresia ; Catheters ; Central Venous Catheters ; Corynebacterium ; Diagnostic Errors ; Gram-Positive Rods ; Humans ; Nontuberculous Mycobacteria ; Rivers ; RNA, Ribosomal, 16S

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN) characterized by persistent peripheral blood neutorphilia, bone marrow hypercellularity of neutrophilic granulocyte proliferation and hepatosplenomegaly. Acquired somatic mutation, JAK2 V617F, is the only molecular marker known to have association with classic BCR-ABL1 negative MPNs. However, JAK2 V617F has been detected occasionally in other MPNs such as CNL or other disease entities. We experienced a case of CNL with JAK2 V617F mutation. The patient was diagnosed according to the 2008 WHO classification criteria, and developed AML 9 months after the diagnosis of CNL. The JAK2 V617F was detected in the bone marrow throughout the clinical course. More cases are needed to establish the role of JAK2 V617F in the pathogenesis, prognosis and disease course of CNL.
Bone Marrow ; Granulocytes ; Humans ; Leukemia, Myeloid, Acute ; Leukemia, Neutrophilic, Chronic ; Neutrophils ; Prognosis

BACKGROUND: Magnetic resonance imaging (MRI) findings of white-matter lesions are different from those of lacunar infarcts; however, both these conditions are related to cardiovascular risk factors. This study was performed to investigate the differences in the relationships of white-matter lesions and lacunar infarcts with cardiovascular risk factors and differences between the metabolic characteristics of patients with these conditions. METHODS: We included 4,255 patients who showed neurological deficits during health checkups. These individuals were classified into the following 3 groups on the basis of MRI findings: normal, white-matter lesion, and lacunar infarct. The groups were compared for age; weights; prevalence of metabolic syndrome; and levels of blood pressure, blood glucose, lipid, high sensitivity C-reactive protein, and HbA1c. RESULTS: Age, body mass index (BMI); waist circumference; levels of blood pressure, blood glucose, triglycerides and HbA1c; and prevalence of metabolic syndrome and its components were the highest in lacunar infarct group, followed by white matter lesion group, and normal group. Age and diastolic blood pressure level were related to white matter lesions, and age, systolic blood pressure level, and blood glucose level were related to lacunar infarcts. Further, the prevalence of the above-mentioned lesions increased with increase of the number of the components of metabolic syndrome. CONCLUSIONS: This study suggests that lacunar infarct is more advanced lesion than white matter lesion. Among all the cardiovascular risk factors, high blood pressure and impaired fasting blood glucose levels were significantly related to white-matter lesions and lacunar infarct.
Blood Glucose ; Blood Pressure ; Body Mass Index ; C-Reactive Protein ; Fasting ; Humans ; Hypertension ; Magnetic Resonance Imaging ; Prevalence ; Risk Factors ; Stroke, Lacunar ; Triglycerides

Rheumatoid arthritis (RA) is an autoimmune disease that results in a chronic inflammatory disorder, which principally attacks the small joints. Several autoantibodies, such as rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibody, are known to be associated with RA. Anti-proliferating cell nuclear antigen (PCNA) antibodies are mainly observed in patients with systemic lupus erythematosus (SLE). Indeed, a high titer of these antibodies is considered highly suggestive of SLE; however, anti-PCNA antibodies also appear in other autoimmune diseases. Two previous reports described RA patients with low titers of anti-PCNA antibodies, respectively. In this report, we describe a case of an RA patient exhibiting a high titer (>1:2,560) of anti-PCNA antibodies. The 56-yr-old female patient, with no underlying disease or medication history, presented with multiple joint pain and morning stiffness that had begun 6 months prior. The erythrocyte sedimentation rate (ESR) and RF were elevated (102 mm/hr and 77 IU/mL, respectively), and C-reactive protein (CRP) was 0.8 mg/dL. While the test for anti-CCP antibodies was negative, an anti-PCNA pattern (>1:2,560) and a homogeneous pattern (1:320) were detected by autoimmune target (AIT) test. The presence of anti-PCNA antibodies was subsequently confirmed using the double immunodiffusion method. The anti-dsDNA test was also positive (1:160). X-ray imaging showed soft tissue swelling of multiple joints of both hands and wrists. According to the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria, the patient was classified as having RA. This is the first case to describe high titers anti-PCNA antibodies associated with RA.
Antibodies* ; Arthralgia ; Arthritis, Rheumatoid* ; Autoantibodies ; Autoimmune Diseases ; Blood Sedimentation ; C-Reactive Protein ; Classification ; Female ; Hand ; Humans ; Immunodiffusion ; Joints ; Lupus Erythematosus, Systemic ; Proliferating Cell Nuclear Antigen ; Rheumatic Diseases ; Rheumatoid Factor ; Wrist

Wegener's granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Arthritis, Rheumatoid* ; Biopsy ; Blood Sedimentation ; C-Reactive Protein ; Classification ; Cytoplasm ; Diagnosis ; Female ; Hematologic Tests ; Humans ; Korea ; Lung ; Rheumatic Diseases ; Rheumatic Nodule ; Rheumatoid Factor ; Skeleton ; Thorax ; Vasculitis ; Wegener Granulomatosis*

Human brucellosis is an important zoonotic disease and has a wide clinical spectrum. Nonspecific hematologic abnormalities related to brucellosis are frequently found, but pancytopenia is uncommon. Malignant diseases have been infrequently reported as a rare cause of pancytopenia in patients with brucellosis. We describe a patient with brucellosis and pancytopenia who was later diagnosed with acute myeloid leukemia. A 71-yr-old man was admitted to a hospital with fever and pancytopenia. Brucella was cultured from blood, and the bone marrow findings were in accordance with brucellosis. The patient's clinical symptoms improved; however, he still showed pancytopenia after completion of medical treatment. After approximately 6 months, he was readmitted with pneumonia and pancytopenia. The second bone marrow examination revealed hypercellular marrow with increased number of blasts. The chromosome analysis showed 46,XY,trp(8)(q11.2q22)[8]/46,idem,del(7)(q22)[12]. The patient was diagnosed with acute myeloid leukemia with myelodysplasia-related changes. He refused further evaluation and therapy, and subsequently died while receiving conservative treatment.
Bone Marrow ; Bone Marrow Examination ; Brucella ; Brucellosis* ; Fever ; Humans ; Leukemia, Myeloid, Acute* ; Pancytopenia* ; Pneumonia ; Zoonoses

BACKGROUND: Hepcidin, a key regulator of iron homeostasis, is associated with iron metabolism imbalance in patients with chronic kidney disease (CKD). However, serum hepcidin level in anemic patients with CKD presents a contradictory picture. We investigated the relationship between serum hepcidin-25 level and iron parameters in patients with CKD. METHODS: We defined and categorized patients with CKD according to the Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines. We analyzed the relationship between serum hepcidin-25 level and iron parameters [serum iron, total iron-binding capacity (TIBC), unbound iron-binding capacity (UIBC), transferrin saturation, and ferritin levels] according to the CKD stage and clinical and laboratory characteristics. RESULTS: Hb level, TIBC, and UIBC decreased and ferritin level increased (Ptrend<0.001) (stage 1-2, 28; stage 3, 40; stage 4, 36; stage 5, 42) as the CKD stage progressed. Serum hepcidin-25 level showed no significant trend with the progressing CKD stage [stage 1-2, 13.7 (3.7-25.0) ng/mL; stage 3, 14.0 (0.8-26.5) ng/mL; stage 4, 13.9 (2.0-32.1) ng/mL; stage 5, 13.8 (0.5-42.4) ng/mL; Ptrend=0.618]. No significant relationship was noted between serum hepcidin-25 level and kidney function parameters, Hb levels, or iron parameters (P>0.05). CONCLUSIONS: Serum hepcidin-25 level was not found to be associated with iron parameters or clinical status of CKD patients in our study. Determination of hepcidin-25 levels may not provide more information than conventional iron parameters in monitoring iron metabolism in CKD patients. However, further studies are needed to establish the clinical utility of hepcidin measurement in CKD patients.
Anemia ; Ferritins ; Hepcidins* ; Homeostasis ; Humans ; Iron* ; Kidney ; Kidney Diseases ; Metabolism ; Renal Insufficiency, Chronic* ; Transferrin

BACKGROUND: Lung cancer is the most lethal malignant neoplasm in the world. Serum cytokeratin fragment 21-1 (Cyfra 21-1) is a valuable tumor marker for detection of lung cancer, and it has good sensitivity and specificity. The aim of this study was to investigate the diagnostic value of Cyfra 21-1 levels in patients with lung cancer. METHODS: We retrospectively reviewed 814 samples from 169 patients with lung cancer, 124 patients with benign pulmonary diseases, and 521 normal controls from health check-up clinic. Serum Cyfra 21-1 levels were determined with Architect CYFRA 21-1 kit (Abbott, USA) using Architect i2000 analyzer. RESULTS: Median levels and interquartile ranges for Cyfra 21-1 in patients with lung cancer (non-small cell lung cancer: 3.16 [1.98, 9.00] ng/mL, small cell lung cancer: 3.32 [2.07, 5.20] ng/mL) were higher than those in patients with benign pulmonary diseases (1.50 [1.17, 2.17] ng/mL; P<0.01) and in normal controls (1.26 [0.93, 1.75] ng/mL; P<0.01). Sensitivity, specificity, positive predictive value, and negative predictive value for Cyfra 21-1 were 70.4%, 81.2%, 49.6%, and 91.3%, respectively. The area under the curve for Cyfra 21-1 was 0.839 (95% confidence interval, 0.802-0.877). CONCLUSIONS: We concluded that Cyfra 21-1 may be useful in the diagnosis of lung cancer.
Diagnosis ; Humans ; Keratins* ; Lung Diseases ; Lung Neoplasms* ; Retrospective Studies ; Sensitivity and Specificity ; Small Cell Lung Carcinoma

BACKGROUND: Dual antiplatelet therapy (aspirin and clopidogrel) is used to prevent adverse cardiac events in patients undergoing percutaneous coronary intervention (PCI). Some patients do not respond adequately to clopidogrel. Beta-thromboglobulin (beta-TG) and platelet factor 4 (PF-4) can act as markers to detect platelet activation. We investigated the relationship between clopidogrel response and the dynamics of beta-TG and PF4 concentrations. METHODS: This study included 36 myocardial infarction (MI) patients, who underwent PCI and was indicated for dual antiplatelet therapy. Platelet reactivity, using the VerifyNow P2Y12 assay, was measured on the 3rd day of PCI. At the time of admission, and on the 3rd and 10th day of PCI, the plasma beta-TG and PF4 concentrations were quantified. RESULTS: Ten patients (27.8%) were clopidogrel non-responders displaying >208 P2Y12 reaction units. At the time of admission, levels of beta-TG in patients were elevated than that in the healthy controls (P<0.001). A similar trend was observed on the 3rd and 10th day of PCI (P<0.001). The beta-TG levels on the 10th day were reduced than those at the time of admission and on the 3rd day of PCI. PF4 levels were not different between patients and controls, and were not significantly reduced after PCI. Higher beta-TG levels were observed in clopidogrel non-responders on the 10th day, but not significant. CONCLUSIONS: Clopidogrel therapy in MI reduce beta-TG concentration, but the beta-TG and PF4 levels before and after therapy are not associated with the response to clopidogrel. Platelet-derived markers may not be suitable for distinguishing clopidogrel non-responders.
beta-Thromboglobulin ; Blood Platelets* ; Humans ; Infarction* ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Plasma ; Platelet Activation* ; Platelet Factor 4