Particle enhanced turbidimetric immunoassay for the determination of urine cystatin C on Cobas c501

ObjectiveUrinary cystatin C has been reported to be a good marker for tubular damage and acute kidney injury. The aim of this study was to develop a high throughput assay for the quantification of urine cystatin C.MethodsAntigen-excess, imprecision, interference, linearity, recovery, sample stability and reference values were evaluated on Cobas c501.ResultsThe assay was linear over the dynamic range of the study (R² = 0.9994). The total assay imprecision was below 5%. The assay recovery was estimated at 87–100%. No tendency to antigen-excess (up to 35 mg/L), nor interference with haemoglobin (1.25–10 g/L) was observed. Cystatin C was stable for 1 day at ambient temperature (19–23 °C) but for 2 days at + 4 °C. The reference interval for cystatin C in urine was < 0.414 mg/L.ConclusionsThe urinary cystatin C assay verified to be a reliable assay with convenient performance characteristics, enabling routine testing on clinical chemistry platforms.     

HDL/LDL ratio: a useful parameter for separation of pleural transudates from exudates

The first diagnostic step in pleural effusions is the separation of transudates from exudates. We aimed in present study to investigate the value of HDL/LDL ratio for distinguishing between pleural exudates and transudates. Pleural fluids (PF)from 121 patients, including 28 transudates and 93 exudates were analyzed. The levels of cholesterol, HDL cholesterol and LDL cholesterol in PF were measured. The HDL/LDL ratio was calculated. HDL/LDL ratio found significantly higher in transudates than exudates (p= 0.001). Receiver operating characteristic (ROC) curves were generated and the cut off points determined to the highest level of accuracy and precision. The HDL/LDL ratio was to maximize sensitivity over specificity in the diagnosis of a transudative effusion. The usefulness of HDL/LDL ratio for identifying transudates was evaluated in terms of sensitivity and specificity. The value of pleural HDL/LDL ratio that best differentiated between transudates and exudates was 0.6 (sensitivity 89%, and specificity of 79%). Measurement of HDL and LDL in PF and calculating of HDL/LDL ratio can be proposed to aid for differentiation between pleural exudates and transudates with advantage of not requiring serum levels.     

A rare cause of sudden vision loss in a young male: papillary fibroelastoma of the aortic valve

Cardiac papillary fibroelastoma (CPF) is a rare cardiac neoplasm predominantly affecting the cardiac valves. Although it is most commonly an incidental finding, it can result in life-threatening complications, such as coronary and cerebral embolism, acute valvular dysfunction, and sudden death. In this report, we describe a case of a patient with aortic valve papillary fibroelastoma, which presented with sudden vision loss due to branch retinal artery occlusion. To the best of our knowledge, this is the third case of CPF-related retinal artery embolism in English literature.     

Evaluation of silicosis in ceramic workers

This study is aimed to evaluate the incidence of silicosis and the relation of it with personal and work-related factors among workers exposed to silica in ceramic factory. Workers were evaluated by respiratory symptoms, physical examination, pulmonary function and radiological findings. Occupational and Enviromental Pulmonary Disease Evaluation Questionnaire of the Turkish Thoracic Society Enviromental and Occupational Pulmonary Diseases Working Group was used. 365 of 626 workers had exposure to silica and the rest 261 were concerned as control group. There was no difference between mean age, duration of work and smoking pack year among the groups (p> 0.05). Cough and sputum rates were higher in silicosis group FEV1 and FVC values were lower in silica group but this was not statistically significant. When the two subgroups of silica group (the workers in high dust concentration and the ones in low concentration) were compared, the high concentrated group had significantly more sputum but the other symptoms and pulmonary functional parameters were not different significantly. 24 workers had parenchymal densities adjusted with pneumoconiosis. The workers with the pneumoconistic finding, had a higher mean age and longer duration of work. As a conclusion, ceramic industry has risk for silicosis. And the risk increase by time and age.     

Pulmonary haemorrhage in a 6-year-old boy with Henoch-Schönlein purpura

Henoch–Scho¨nlein purpura (HSP) is the most common vasculitis in children. It is a multisystemic disease but pulmonary haemorrhage is extremely rare. We present the case of a 6-year-old boy with Henoch–Scho¨nlein purpura, pulmonary haemorrhage and severe renal involvement. The patient responded to a combination of intravenous methylprednisolone and cyclophosphamide. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment. Renal biopsy is helpful in the differential diagnosis of HSP-mimicking pulmonary vasculitic syndromes. Combining cyclophosphamide with glucocorticoids may improve the outcome in severe HSP cases with pulmonary haemorrhage. Received: 5 July 2000 / Accepted: 2 February 2001     

The role of apoptosis in childhood Henoch–Schonlein purpura

The pathogenesis of vasculitis is complex and is yet to be fully elucidated, although it is known that inflammatory cells play a major role. Dysregulation of apoptosis and defective clearance of inflammatory cells could lead to the persistence of inflammation and excessive tissue injury. In this study we aimed to investigate Fas (CD95) and apoptosis on peripheral blood (PB) neutrophil and lymphocytes in Henoch–Schonlein purpura, both in the acute phase and after resolution to determine the role of apoptosis in this self-limited vasculitis. Leukocytoclastic vasculitis presenting with Henoch–Schonlein purpura (HSP) was diagnosed according to ACR 1990 criteria and confirmed by skin biopsy. Thirty-seven patients (22 boys, 15 girls) aged 2.5–17 years (9 ± 3.3) were enrolled in the study. Expression of CD95 and apoptosis were investigated by the annexin/PI method on peripheral blood neutrophils and lymphocytes in both the acute and the resolution phases of the disease. The mean neutrophil and lymphocyte CD95 expression was 65.4 ± 37.6% and 33.3 ± 7.3%, respectively, in the acute stage and 62.8 ± 44.2% and 41 ± 20%, respectively, in the resolution (P > 0.05). The percentage of apoptotic peripheral blood neutrophils and lymphocytes as determined by annexin positivity was 13.3 ± 11.31% and 8.6 ± 9.5%, respectively, during the acute phase and 4.6 ± 3.4% and 3.1 ± 3.1%, respectively, in the resolution (P = 0.002, P = 0.008). These results suggest that increased apoptotic process in the immune effector cells in the acute phase of the disease may play an important role in the early control of inflammatory response and repair in leukocytoclastic vasculitis, thereby contributing to the self-limited nature of the disease.     

Increased myocardial collagen turnover in patients with progressive cardiac conduction disease

Background: Histopathologically, progressive cardiac conduction disease (PCCD) is characterized by progressive fibrosis and sclerodegenerative changes in the proximal and distal conduction system of the heart. Therefore, we sought to determine the serum levels of myocardial collagen turnover markers, extracellular matrix components, transforming growth factor β₁ (TGFβ₁), and bone morphogenic protein‐7 (BMP‐7) in this population. Methods: Study population included 20 patients (6 M/14 F, mean age 76 ± 8 years) with acquired, permanent 2:1, or complete atrioventricular block and compared with age‐ and sex‐matched, asymptomatic, healthy control subjects (n = 18, 6 M/12 F, mean age 75 ± 7 years). Serum myocardial collagen turnover markers:matrix metalloproteinases (MMP‐1, 2, 9), tissue inhibitor of matrix metalloproteinase (TIMP‐1), amino‐terminal propeptide of procollagen type I (PINP) and type III (PIIINP), carboxy‐terminal telopeptide of collagen type I (CITP), and carboxy‐terminal propeptide of procollagen type I (PICP), serum extracellular matrix components (laminin and fibronectin), TGFβ₁, and BMP‐7 levels were measured in both groups. Results: Serum PICP (849 ± 396 vs 631 ± 294 ng/mL, P = 0.04), PIIINP (3.7 ± 1.3 vs 3 ± 1 μg/L, P = 0.03), CITP (0.68 ± 0.35 vs 0.48 ± 0.25 ng/mL, P = 0.037), and plasma MMP‐9 (58.8 ± 56 vs 25.9 ± 17.3 ng/mL, P = 0.006) levels were higher in patient population compared to control subjects. Serum MMP‐1 (24.1 ± 20.5 vs 13.6 ± 7.5 ng/mL, P = 0.045) and MMP‐2 (1310 ± 139 vs 1186 ± 163 ng/mL, P = 0.01) levels were higher in control subjects compared to patient population. There was no difference in serum TIMP‐1, PINP, laminin, fibronectin, TGFβ₁, and BMP‐7 levels between two groups. Conclusion: Our findings demonstrate the presence of increased myocardial collagen turnover and active fibrotic process in patients with PCCD compared to control subjects.     

Relation between sympathetic overactivity and left atrial spontaneous echo contrast in patients with mitral stenosis and sinus rhythm

BACKGROUND: Spontaneous echo contrast (SEC) is common in patients with mitral stenosis (MS) and presence of SEC in left atrium (LA) is associated with a higher risk of thromboembolism. Recently, an increase in activation of platelets was demonstrated in patients with SEC raising the hypothesis that platelets are involved in the pathogenesis of SEC. In this study, we evaluated effects of autonomic nervous system activity on SEC formation in patients with rheumatic MS and sinus rhythm by heart rate variability analysis. METHODS AND RESULTS: Twenty-six patients with LASEC were compared with 28 patients without LASEC. Mean heart rate, low frequency (LF) and low frequency/high frequency (LF/HF) ratio were significantly higher, standard deviation of all NN (SDNN), root mean square of successive differences (RMSSD), number of NN intervals that differed by more than 50 ms from adjacent interval divided by the total number of all NN intervals (PNN50) and high frequency (HF) values were lower in the patients with LASEC. A standard deviation of all NN intervals <90ms separated the patients with LASEC from control subjects with a sensitivity of 77% and specificity of 90%; a low frequency >79.5 with a sensitivity of 92% and specificity of 90; a low frequency/high frequency ratio >3.7 with a sensitivity of 96% and specificity of 90%. A left atrial diameter >4.3 cm increased the LASEC formation by 3.0 folds, HR >78 beats/min by 6.4 folds, standard deviation of all NN intervals <90 ms by 9.2 folds, a low frequency/high frequency ratio >3.7 by 6.4 folds, sP-selectin>142 by 5.8 folds. Variables affecting sP-selectin levels were LA diameter, mitral valve area, transmitral mean gradient, left ventricular ejection fraction, the presence of mitral regurgitation, HR, standard deviation of all NN intervals, low frequency, high frequency and low frequency/high frequency ratio. CONCLUSION: Sympathetic overactivity and reduced heart rate variability are important determinants for LASEC formation and increased s-P selectin levels. Therefore, platelet activation via increased sympathetic activity may play an important role in pathogenesis of LASEC.     

Serum level of lipoprotein (a) is inversely associated with the development of coronary collateral circulation

BACKGROUND: This study sought to determine the relationship between serum lipoprotein (a) levels and angiographically visible coronary collateral circulation and to evaluate whether lipoprotein (a) exerts any effect on vascular endothelial cell growth factor. METHODS: The study population included 60 patients (39 men, mean age 59+/-13 years) with angiographically documented total occlusion in one of the major coronary arteries. Development of collaterals was classified by Rentrop's method. Patients were defined as having poorly developed collaterals for grades 0 and 1 (group 1), or well-developed collaterals for grades 2 and 3 (group 2). Serum lipoprotein (a) and vascular endothelial cell growth factor levels were determined by enzyme-linked immunosorbent assay. RESULTS: In group 1, lipoprotein (a) levels were significantly higher and vascular endothelial cell growth factor levels were significantly lower than in group 2 (34+/-19 vs. 20+/-12 mg/dl, P<0.001, and 2.5+/-0.7 vs. 3.4+/-0.8 ng/dl, P<0.001, respectively). Poorly developed collaterals were significantly more frequent in patients with lipoprotein (a) levels >or=30 mg/dl than in patients with levels <30 mg/dl (72 vs. 37%, P=0.008). A strong negative correlation was observed between lipoprotein (a) and vascular endothelial cell growth, factor (r=-0.708, P<0.0001). Multivariate analysis revealed that a high level of lipoprotein (a) negatively affected the development of collaterals, whereas the duration of angina had a positive effect. CONCLUSION: This study demonstrated for the first time that the high level of lipoprotein (a) negatively affects the formation of coronary collateral vessels in human beings. Reduced production or bioactivity of vascular endothelial cell growth factor caused by high levels of lipoprotein (a) may be the possible responsible mechanisms of hyperlipoprotein (a)-related poor collateral formation.     

E148Q is a disease-causing MEFV mutation: a phenotypic evaluation in patients with familial Mediterranean fever

BACKGROUND: Familial Mediterranean fever (FMF) is one of the periodic fever syndromes. It is common among Turks, Jews, Arabs, and Armenians. Several mutations in the MEFV gene, including E148Q, have been identified as causing this disease. It has been suggested that the E148Q mutation is the mildest mutation and some reports have questioned its disease association. OBJECTIVE: To evaluate the phenotypic features of the patients with E148Q mutation. SUBJECTS: 26 patients homozygous for E148Q, 10 compound heterozygous for E148Q, and eight complex cases were assessed. RESULTS: Although four of the 26 patients with E148Q/E148Q were asymptomatic at the time of evaluation, abdominal pain was seen in 77% of the patients, fever in 66%, arthralgia in 50%, arthritis in 15.4%, and vomiting in 23.8%. Compound heterozygotes and complex cases had a higher frequency of abdominal pain, fever, arthralgia, arthritis, myalgia, and chest pain than subjects who were homozygous for E148Q, but none of these symptoms reached statistical significance. None of our patients had amyloidosis but two with E148Q/E148Q had a family history of amyloidosis and one had rapidly progressive glomerulonephritis secondary to vasculitis, which progressed to chronic renal failure. CONCLUSIONS: Patients homozygous for E148Q have a heterogeneous clinical presentation. Most are symptomatic and colchicine treatment is required in these patients.