We investigated plasma sphingomyelin (CerPCho) and ceramide (Cer) levels in pediatric patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Plasma samples were obtained from CF (n = 19) and PCD (n = 7) patients at exacerbation, discharge, and stable periods. Healthy children (n = 17) of similar age served as control. Levels of 16-24 Cer-PCho and 16-24 Cer were measured by LC-MS/MS. Concentrations of all CerPCho and Cer species measured at exacerbation were significantly lower in patients with CF than PCD. 16, 18, 24 CerPCho, and 22, 24 Cer in exacerbation; 18, 24 CerPCho, and 18, 20, 22, 24 Cer at discharge; 18, 24 CerPCho and 24 Cer at stable period were significantly lower in CF patients than healthy children (p < 0.001 and p < 0.05). All CerPCho and Cer levels of PCD patients were significantly higher except 24 CerPCho and 24 Cer during exacerbation, 24 CerPCho at discharge, and 18, 22 CerPCho levels at stable period (p < 0.001 and p < 0.05) compared with healthy children. There was no significant difference among exacerbation, discharge, and stable periods in each group for Cer and CerPCho levels. This is the first study measuring plasma Cer and CerPCho levels in PCD and third study in CF patients. The dramatic difference in plasma levels of most CerPCho and Cer species found between two diseases suggest that cilia pathology in PCD and CFTR mutation in CF seem to alter sphingolipid metabolism possibly in opposite directions.

Background. Sickle cell disease (SCD) is an inflammatory disease that can result in both chronic and acute inflammation. Immature granulocytes (IG) are not-yet-mature white blood cells that can be easily detected in complete blood count (CBC) tests. In recent studies it has been suggested that IG may play a role in determining the prognosis of inflammatory diseases. The aim of our study was to investigate the role of IG percentage on predicting acute chest syndrome (ACS) and the severity of vaso-occlusive crisis (VOC) in patients with SCD.Methods. The study cohort consisted of 49 SCD patients admitted to the emergency department for VOC. If symptoms did not regress despite appropriate treatment including hydration and analgesia, they were hospitalized. Patients whose symptoms regressed were discharged from the emergency department within 24 hours. Blood samples, including CBC and C-reactive protein (CRP), a marker of inflammation, were taken within the first hour of admission. Steady state laboratory parameters from the previous visit in the last three months were collected from patient files.Results. The mean age was 18±4 (range 8-25) years. Most were hospitalized (41/49; 83.7%) and 8 of 49 were discharged from the emergency department after their treatment for VOC. ACS developed in 13 of 49 (26.5%). White blood cell, neutrophil and nucleated red blood cell counts, percentage of IG (IG%) and CRP levels were significantly increased in patients with VOC. IG% of patients with ACS was significantly higher than patients without ACS. However, ROC analysis showed that IG% was not associated with the development of ACS or hospitalization for VOC.Conclusions. Despite a small SCD cohort, the significant increase in the IG% in patients with VOC compared to their baseline values has suggested a role for IG% in predicting VOC. Although IG% was higher in ACS, its utility in predicting ACS was poor.

BackgroundWe investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic brosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. MethodsPlasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and uorometric assay, respectively. Data were compared with healthy children and adults of similar age. ResultsYKL-40 levels of pediatric and adult CF patients at all periods were signi cantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were signi cantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls.YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with % FVC (r= -0.800, p = 0.014) and % FEV1 (r= -0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with % FVC (r= -0.697, p = 0.0082) and % FEV1 (r= -0.720, p = 0.006). ConclusionsCHIT1 activity may be a valuable marker of chronic in ammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology. Furthermore, as YKL-40 levels are correlated with FEV1 and FVC in patients, it may be useful for the monitoring of pulmonary function in CF patient.

The primary purpose of medical laboratories is to provide the most accurate results appropriate to the patient's medical condition. Therefore, the reliability of each laboratory must be scientifically tested. Approximately 10 % of laboratory errors occur in the analytical phase. In this study, we aimed to evaluate analytical process performances of 14 routinely assayed parameters according to Six Sigma methodology.Materials and Methods: Mean, standard deviation and coefficient of variation were calculated from internal quality control data for 3 months from 14 routinely assayed parameters (albumin, alanine aminotransferase, aspartate aminotransferase, chloride, creatinine, glucose, HDL cholesterol, lactate dehydrogenase, potassium, total cholesterol, total protein, sodium, triglyceride and urea) in the laboratory (Roche Cobas c501). Bias was calculated using external quality control values for same months. Total error was also calculated. Acceptable total error was determined according to the Clinical Laboratory Improvement Amendments and Turkey criteria. Sigma values were calculated and divided into four groups; as <3 unacceptable; 3-4 suited for purpose; 4-6 acceptable; >6 world-class performance.Results: According to the Clinical Laboratory Improvement Amendments and Turkey sigma assessment, first levels of chloride, total cholesterol, glucose and urea performance were unacceptable. Moreover according to the Clinical Laboratory Improvement Amendments sigma assessment, first levels of albumin, creatinine, total protein; and both levels of sodium, chloride and urea were unacceptable. Other tests were found to be suited for purpose, acceptable or world-class performance.Conclusions: Sigma measurements should be routinely performed in laboratories for evaluating the analytical period performance of the laboratory. That will increase its quality via regulatory preventive actions. Our study allowed us to see and improve our measurement quality by determining the three-month periodic performance of our laboratory tests.

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