Background Few previous studies indicated the role of oxidative stress in the pathogenesis of childhood idiopathic thrombocytopenic purpura (ITP), but there are little data regarding changes in redox ...balance in different forms of the disease, and changes after therapeutic procedures. We aimed to investigate the values of pro-oxidants and antioxidative capacity in various forms of ITP before and after the applying therapy.
Materials and Methods The research included 102 children, classified into the following groups: (1) newly diagnosed ITP (ndITP), (2) persistent ITP, (3) chronic ITP (chITP), and (4) control groups: (A) healthy control and (B) previously experienced ITP—healthy children who had been suffering from ITP earlier. During the clinical assessment, a blood sample was taken from the patients, from which the value of pro-oxidants (index of lipid peroxidation measured as TBARS, nitrites NO2
−, as measurement of nitric oxide NO production, superoxide anion radical O2
−, and hydrogen peroxide H2O2) and the capacity of antioxidant protection (activity of superoxide dismutase and catalase, and quantity of reduced glutathione) were determined spectrophotometrically.
Results Our results demonstrated that values of pro-oxidants, especially reflected through the TBARS and O2
−, were the highest in the ndITP and exacerbated chITP groups. Also, the activity of the endogenous antioxidative defense system was the lowest in these groups. Intravenous immunoglobulin therapy in the ndITP group exerted the most prominent effect on the redox balance.
Conclusion It can be concluded that severity and exacerbation of the ITP are closely related to the redox status.
This paper presents an innovative system for 3D reconstruction of a physical dental model. The innovative system is based on close-range photogrammetry and enables the projection of digital light ...texture on the objects surface. It is based on the application of mirrors that direct the digital light texture to the vertical surfaces of the physical model. In this way, high coverage of the object is achieved, and 3D reconstruction from one set of photographs is possible. 3D digitization, verification and comparison of the proposed methodology was performed on dental models that are characterized by extremely complex surfaces. It was performed by comparing the proposed approach with active stereovision, and the efficiency was evaluated in relation to the reference 3D model obtained by the structured light 3D scanner. The comparison of the results was performed on the basis of the mean deviation and standard deviation for the 3D model with combined teeth and for the 3D model with metal caps. The absolute mean deviations for the 3D model with combined teeth are 0.004-0.021 mm, with a standard deviation of 0.055-0.058 mm, and for the 3D model with metal caps absolute mean deviations are 0.015-0.033 mm, with a standard deviation of 0.095-0.113 mm, respectively. Absolute minimum values of mean deviation of 0.004 mm and standard deviations of 0.055 mm were obtained by 3D model with combined teeth, which was reconstructed by the proposed innovative approach. The obtained results indicate a higher accuracy of the innovative approach in relation to the use of a commercial 3D scanner that uses active stereovision principle.
The study concerns measurement and evaluation of the geometric characteristics of the inhalable fraction of particulate matter generated in the process of grinding dental restorations, which is a ...process that generates a large quantity of particulates. The research is based on measurements taken with a personal sampler, whereby the characteristics of particulates are determined based on the particle mass collected on filters. The collected filters were examined using scanning electron microscopy (SEM), and the resulting micrographs were processed through image analysis. The complex geometry of particles was examined through the analysis of 14 parameters, of which 6 define size and 8 describe morphological characteristics. Two software programs were used for the image analysis process to gather a wide range of parameters describing particle geometry. The relationship and dependence between the geometric parameters used to describe particle shape and size were investigated using multivariate analysis methods. Through correlation analysis, principal component analysis, and cluster analysis, parameter selection and reduction were performed to provide an understanding of the particles inhaled by exposed workers, which also influences the biological response of exposed organisms and the burden on the working environment.
Abstract Background Hemophagocytic lymphohistiocytosis is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central ...nervous system. PATIENTS and Methods Thirty children diagnosed with hemophagocytic lymphohistiocytosis between 1997 and 2010 were reviewed and analyzed. Central nervous system disease involvement was defined as the presence of neurological symptoms and signs or elevated values of cerebrospinal fluid cells and/or proteins. Results Among the 30 patients, 17 (56%) had central nervous system involvement. Fourteen patients (46%) presented with neurological symptoms including seizures, irritability, bulging fontanelle, cranial nerve palsy, or disturbance of consciousness, whereas the remaining three patients developed central nervous system symptoms during the course of the disease. Seventeen patients (56%) had cerebrospinal fluid abnormalities. Neuroradiological studies were performed in nine patients. The most common findings were edema, atrophy, subcortical necrosis, and high signal intensity on T2-weighted magnetic resonance imaging. All patients were treated according to the Hemophagocytic Lymphohistiocytosis-94 and Hemophagocytic Lymphohistiocytosis-2004 protocols. Patients with central nervous system involvement had greater mortality. In prediction of the outcome, the cutoff value for cerebrospinal fluid protein was 470 mg/L. The most common neurological sequela was psychomotor delay. Conclusion Central nervous system involvement in hemophagocytic lymphohistiocytosis is common and is associated with poor outcome.
Abstract Complications caused by elevated white blood cell count in pediatric patients with CML could be a presenting feature of the disease. Here, we present two adolescents, aged 16 and 17 years, ...who were admitted for investigation of extremely elevated leukocytes and complications of leucostasis. Initial manifestations were priapism and blurred vision, respectively. Diagnosis of chronic phase of chronic myeloid leukemia is established, and conventional measures for leucoreduction began. However, since there were no improvements, a leukapheresis procedure was initiated. After undergoing 3 daily procedures the leukocyte count declined for each patient, with resolution of pripaism and ophtalmological disturbances. Leukapheresis is safe and effective therapeutic option for patients with complications of hyperleucocytosis. If started in a timely manner, permanent organ damage or death could be avoided.
Risk factors for thrombosis in Serbian children Serbic-Nonkovic, Olivera M; Kuzmanovic, Milos B; Rakicevic, Ljiljana B ...
Blood coagulation & fibrinolysis,
2014-January, 2014-Jan, 2014-01-00, 20140101, Letnik:
25, Številka:
1
Journal Article
Recenzirano
Venous and arterial thromboses are increasingly encountered in the pediatric population. We present results of a case–control study of inherited and acquired risk factors for thrombosis in 129 ...pediatric patients from the first day of life to 18 years. The aims of study were to determine the importance of thrombophilic risk factors and comorbidity as a cause of thrombosis in children. Single thrombophilic risk factor was found in 24.4% (n = 21), whereas combined thrombophilic factors were found in 15.1% (n = 13) patients. A total of 87.2% of the children had recognized thrombophilic risk factors for thrombosis and/or additional comorbid risk factors. The single independent risk factors for thrombosis were mutation of factor V Leiden (P = 0.021), lupus anticoagulant antibodies (P = 0.028), and comorbidity (P = 0.000). Mutation of factor V Leiden odds ratio (OR), 6.2 (95% confidence interval, CI 1.1–38.1, P = 0.048 was found to be a risk factor for venous thrombosis. Lupus anticoagulant antibodies were related to both venous (P = 0.008) and arterial thrombosis (P = 0.016). The frequency of inherited thrombophilic factors were the same in neonates and adolescents (23%). The prothrombotic gene mutations were present in 18.6% (n = 8) of asymptomatic children. Our study confirms that thrombosis in children is a multifactorial disorder, and associated most with the underlying medical disease (comorbidity) for vein thrombosis OR, 18.6 (95% CI 3.7–93.4), P = 0.000 and for arterial thrombosis OR, 10.5 (95% CI 2.2–49.9) P = 0.003. Inherited thrombophilic disorders contributed to the development of thrombosis in children.
The International Berlin-Frankfurt-Münster (BFM) study group conducted a study on pediatric acute lymphoblastic leukemia (ALL). Minimal residual disease (MRD) was assessed using flow cytometry (FCM), ...and the impact of early intensification and methotrexate (MTX) dose on survival was evaluated.
We included 6,187 patients younger than 19 years. MRD by FCM refined the risk group definition previously used in the ALL intercontinental-BFM 2002 study on the basis of age, WBC count, unfavorable genetic aberrations, and treatment response measured morphologically. Patients at intermediate risk (IR) and high risk (HR) were randomly assigned to protocol augmented protocol I phase B (IB) versus IB regimen. MTX doses of 2 versus 5 g/m
every 2 weeks, four times, were evaluated in precursor B-cell-ALL (pcB-ALL) IR.
The 5-year event-free survival (EFS ± SE) and overall survival (OS ± SE) rates were 75.2% ± 0.6% and 82.6% ± 0.5%, respectively. Their values in risk groups were standard risk (n = 624), 90.7% ± 1.4% and 94.7% ± 1.1%; IR (n = 4,111), 77.9% ± 0.7% and 85.7% ± 0.6%; and HR (n = 1,452), 60.8% ± 1.5% and 68.4% ± 1.4%, respectively. MRD by FCM was available in 82.6% of cases. The 5-year EFS rates in patients randomly assigned to protocol IB (n = 1,669) and augmented IB (n = 1,620) were 73.6% ± 1.2% and 72.8% ± 1.2%, respectively (
= .55), while those in patients receiving MTX doses of 2 g/m
(n = 1,056) and MTX 5 g/m
(n = 1,027) were 78.8% ± 1.4% and 78.9% ± 1.4%, respectively (
= .84).
The MRDs were successfully assessed using FCM. An MTX dose of 2 g/m
was effective in preventing relapse in non-HR pcB-ALL. Augmented IB showed no advantages over the standard IB.Media: see text.
Inherited antithrombin (AT) deficiency is a rare autosomal dominant disorder, caused by mutations in the AT gene (
SERPINC1
). Considering that the genotype phenotype relationship in AT deficiency ...patients remains unclear, especially in pediatric patients, the aim of our study was to evaluate genotype phenotype correlation in a Serbian pediatric population. A retrospective cohort study included 19 children younger than 18 years, from 15 Serbian families, with newly diagnosed AT deficiency. In 21% of the recruited families, mutations affecting exon 4, 5, and 6 of the
SERPINC1
gene that causes type I AT deficiency were detected. In the remaining families, the mutation in exon 2 causing type II HBS (AT Budapest 3) was found. Thrombosis events were observed in 1 (33%) of those with type I, 11 (85%) of those with AT Budapest 3 in the homozygous respectively, and 1(33%) in the heterozygous form. Recurrent thrombosis was observed only in AT Budapest 3 in the homozygous form, in 27% during initial treatment of the first thrombotic event. Abdominal venous thrombosis and arterial ischemic stroke, observed in almost half of the children from the group with AT Budapest 3 in the homozygous form, were unprovoked in all cases.
Conclusion
: Type II HBS (AT Budapest 3) in the homozygous form is a strong risk factor for arterial and venous thrombosis in pediatric patients.
What is Known:
•
Inherited AT deficiency is a rare autosomal dominant disorder, caused by mutations in the SERPINC1gene.
•
The genotype phenotype correlation in AT deficiency patients remains unclear, especially in pediatric patients.
What is New:
•
The genetic results for our paediatric population predominantly showed the presence of a single specific mutation in exon 2, that causes type II HBS deficiency (AT Budapest 3).
•
In this group thrombosis mostly occurred as unprovoked, in almost half of them as abdominal thrombosis or stroke with high incidence of recurrent thrombosis, in 27% during initial treatment.
We report the successful use of rituximab as single treatment modality in a five-month-old boy with fulminant warm autoantibody autoimmune hemolytic anemia, resistant to standard treatment. On ...admission, laboratory tests showed a profound anemia with a hemoglobin of 2.6 g/dL. Indirect and direct antiglobulin tests were strongly positive, and nonspecific IgG autoantibodies were detected. Two days of intravenous corticosteroids (methylprednisolone 4mg/kg) and immunoglobulins (1g/kg) did not halt the hemolysis and the infant was severely transfusion-dependent. Rituximab 375mg/sq m weekly was given for 4 weeks, the hepatosplenomegaly gradually regressed, the lymphocytes normalized and he is free from hemolysis two years after treatment.
•Investigation of radiation dose and its impact on accuracy was performed.•New workflow for 2D and 3D analysis is proposed.•Insignificant differences were noticed between reconstructed surface 3D ...models.•Reduced radiation dose doesn’t have significant impact on dimensional accuracy.
The aim of this study is to investigate whether the radiation dose level, during 3D cone beam computed tomography scanning of the patient, influences the dimensional accuracy of the reconstructed surface 3D models, and if it does, to analyse the accuracy improvement in relations to radiation dose levels. In this study is proposed a workflow where two datasets of images acquired with different radiation dose levels of the lower jaw were used for analysis, with the focus on region where the tooth was extracted on the patient’s mandible. Statistical evaluators used for analysis are Jaccard index, Dice coefficient and Tanimotto coefficient, while accuracy and precision were also used as a statistical measure. The results of evaluation of six sample images for two datasets by Jaccard index are ranging from 0.684 to 0.873 and 0.579 to 0.849, respectively, by Dice are 0.812 to 0.932, and 0.733 to 0.918, while by Tanimoto are ranging from 0.763 to 0.895, and 0.626 to 0.867, respectively. Regarding the accuracy and precision, the average difference for those six images is a little over 1% and 2%, respectively. Reconstructed tooth cavities were also obtained where volume analysis showed insignificant difference between two reconstructed surface 3D models of cavities. This was also confirmed with performed dimensional measurements obtained for the two tooth cavities, as well as bone density evaluation inside those cavities. Computer-aided inspection was also performed which shows similar results of two datasets. It can be concluded, on bases of the obtained results, that the reduced level of radiation dose during image acquisition doesn’t have significant impact on dimensional accuracy compared to the higher dosage.