The role of urinary N-acetyl-β-D-glucosaminidase in early detection of acute kidney injury among pediatric patients with neoplastic disorders in a retrospective study.

Background: The 1-year cumulative incidence of AKI reportedly is high (52%) in pediatric neoplastic disorders. About half of these events occur within 2 weeks. However, subclinical AKI episodes may remain unrecognized by the conventional creatinine-based approaches.

Transglutaminase 2 Has Metabolic and Vascular Regulatory Functions Revealed by In Vivo Activation of Alpha1-Adrenergic Receptor.

The multifunctional tissue transglutaminase has been demonstrated to act as α1-adrenergic receptor-coupled G protein with GTPase activity in several cell types. To explore further the pathophysiological significance of this function we investigated the in vivo effects of the α1-adrenergic receptor agonist phenylephrine comparing responses in wild type and TG2 mice. Injection of phenylephrine, but not a beta3-adrenergic agonist (CL-316,243), resulted in the long-term decline of the respiratory exchange ratio and lower lactate concentration in TG2 mice indicating they preferred to utilize fatty acids instead of glucose as fuels.

Detection of haemolysis, a frequent preanalytical problem in the serum of newborns and adults.

Background: Preanalytical problems can be more frequent in case of preterm and term newborns as compared to the general patient population. Here we present the leading preanalytical errors in our laboratory, the prevalence of haemolysis and its impact on laboratory test results, and our efforts to improve the diagnostic workup of newborns' samples.

Methods: Preanalytical quality indicators were analysed in all samples in 2018.

Intracardiac Fibrinolysis and Endothelium Activation Related to Atrial Fibrillation Ablation with Different Techniques.

Objective: The effect of pulmonary vein isolation (PVI) on fibrinolytic and endothelial activation with currently applied periprocedural anticoagulation has not been explored. We measured markers of fibrinolysis and endothelium activation before and after PVI with the second-generation cryoballoon (Cryo), pulmonary vein ablation catheter (PVAC-Gold), and irrigated radiofrequency (IRF).

Methods: Markers of fibrinolysis and endothelium activation in left atrial (LA) blood samples were measured in 31 patients before and after PVI (Cryo:10, PVAC-Gold: 7, IRF: 14).

Comparative erythrocyte deformability investigations by filtrometry, slit-flow and rotational ektacytometry in a long-term follow-up animal study on splenectomy and different spleen preserving operative techniques: Partial or subtotal spleen resection and spleen autotransplantation.

Background: Partial or subtotal spleen resection or spleen autotransplantation can partly preserve/restore the splenic filtration function, as previous studies demonstrated.

Objective: For better evaluation and follow-up of the various spleen-preserving operative techniques' effectiveness versus splenectomy, a composite methodological approach was applied in a canine experimental model.

Methods: Beagle dogs were subjected to control (n = 6), splenectomy (SE, n = 4), partial and subtotal spleen resection (n = 4/each) or spleen autotransplantation groups (AU, Furka's spleen-chip method, n = 8).

[Inborn error of cholesterol biosynthesis: Smith-Lemli-Opitz syndrome].

Smith-Lemli-Opitz syndrome is an autosomal recessive mental retardation and multiple malformation syndrome caused by deficiency of the 7-dehydrocholesterol reductase, the enzyme catalyzing the last step in cholesterol biosynthesis. The authors summarize the pathophysiology, epidemiology, clinical picture, diagnostics and therapy of the disease based on a review of the international literature. Since 2004, fourteen patients have been diagnosed with Smith-Lemli-Opitz syndrome in Hungary, which suggests an underdiagnosis of the disease based upon estimated incidence data.

Altered lipid subfraction profile and impaired antioxidant defense of high-density lipoprotein in Smith-Lemli-Opitz syndrome.

Background: Smith-Lemli-Opitz syndrome (SLOS) is a rare disease caused by biallelic mutation in the 7-dehydrocholesterol (7DHC) reductase gene. High oxidizability of 7DHC and the appearance of small-sized low-density lipoprotein (LDL) subfractions indicate increased endogenous oxidative stress that is counterbalanced by natural antioxidant defense mechanisms including the high-density lipoprotein (HDL)-associated paraoxonase-1 (PON1) enzyme. PON1 prevents lipoproteins from oxidative modifications; however, PON1 activity and the distribution of lipoprotein subfractions have not been studied in SLOS.

[Pneumatic tube system for transport of laboratory samples: preanalytical aspects].

Introduction: A considerable proportion of laboratory errors occurs in the preanalytical phase.

Aim: The aims of the authors were to study preanalytical errors in routine and emergency laboratory diagnostics in a regional clinical laboratory and evaluate the effect of the pneumatic tube system on turnaround time and laboratory results.

Method: The ratio of preanalytical errors and reasons of test rejection were analysed.

Rosuvastatin improves impaired endothelial function, lowers high sensitivity CRP, complement and immuncomplex production in patients with systemic sclerosis--a prospective case-series study.

Introduction: We studied the effect of rosuvastatin on endothelial and macrovascular function, cardiovascular risk factors and the complement pathway in patients with systemic sclerosis (SSc).

Methods: Altogether 28 patients with SSc underwent laboratory and complex vascular assessments before and after six months of 20 mg rosuvastatin treatment. Flow-mediated dilation (FMD) of the brachial artery, as well as carotid artery intima-media thickness (ccIMT), carotid-femoral and aorto-femoral pulse wave-velocity (PWV) were analyzed by ECG-synchronized ultrasound.

Characterization of a novel high-density lipoprotein antioxidant capacity assay and its application to high-density lipoprotein fractions.

Objectives: High-density lipoprotein (HDL) inhibits low-density lipoprotein (LDL) oxidation therefore it is involved in the prevention of atherogenesis. HDL particles originating from different persons possess different antioxidant activities. Our aim was to establish a method for the measurement of HDL antioxidant capacity, which is suitable for testing the antioxidant activity of HDL samples in a wide range and produces data relevant to in vivo HDL-LDL interactions.

Relation between biomarkers and clinical severity in patients with Smith-Lemli-Opitz syndrome.

Unlabelled: Smith-Lemli-Opitz syndrome (SLOS), a multiple congenital anomaly with severe mental retardation, is caused by decreased activity of 7-dehydrocholesterol reductase. Fifteen Hungarian patients were diagnosed with SLOS on the basis of clinical symptoms, serum cholesterol, 7-dehydrocholesterol, and molecular genetic testing. Their age at the time of diagnosis in mild SLOS (n = 4, clinical score <20) was 0.

[Interpretation of highly sensitive troponin assays: acute or chronic myocardial damage?].

Unlabelled: Troponin is the first choice in the diagnosis of acute myocardial infarction. Correct interpretation is challenging, because high sensitive troponin tests used today detect even the smallest cardiac damage.

Methods: High sensitive troponin T (Roche) and troponin I (Mitsubishi Pathfast) and creatine-kinase activity were measured in 20 patients, each having two samples with the time lapse 3-9 hours.

Monitoring of mycophenolic acid and kidney function during combined immunosuppressive therapy.

Background: Mycophenolic acid (MPA), a selective inhibitor of lymphocyte proliferation, has lately been used to improve renal function and prolong graft survival in renal transplanted patients. Still, there is no consensus considering the recommended dosing and the therapeutic range of MPA.

Methods: To estimate the safe therapeutic range of MPA, its plasma level and indicators of kidney function were measured in 216 patients (138 male, 78 female, age 46 ± 12 years) 67 ± 46 months after transplantation.

Hepatobiliary response in postoperative lipid therapy in gastrointestinal surgery.

Background/aims: Intravenous lipid emulsions may contribute to the development of total parenteral nutrition (TPN)--induced hepatobiliary complications.

Methods: In a prospective, randomised setting the authors compared the short-term hepatic effects of medium-chain triglycerides/short-chain triglycerides (MCT/LCT) physical mixture with a four-component intravenous (i.v.

[New trends in the laboratory diagnostics of proteinuria and albuminuria].

According to current clinical trials, albumin excretion is an early indicator of cardiovascular damage. While proteinuria is considered as a marker of kidney function, albuminuria indicates cardiovascular risk first of all. Sensitivity of the previous laboratory tests does not meet the clinical requirements, and the error of urine collection makes the results misleading.

A patient with Smith-Lemli-Opitz syndrome: novel mutation of the DHCR7 gene and effects of therapy with simvastatin and cholesterol supplement.

Background: The Smith-Lemli-Opitz (SLO) syndrome is a multiple congenital anomaly with mental retardation due to a decreased or lack of activity of 7-dehydrocholesterol reductase as a consequence of mutations of the DHCR7 gene. This paper describes a special patient with SLO syndrome. Laboratory examination showed low cholesterol (2.

[Biochemical alterations in patients with Down syndrome].

Unlabelled: Down syndrome is a chromosome abnormality with specific clinical symptoms and mental retardation caused by trisomy of chromosome 21. The basic genetic change cannot be cured, the control of the associated symptoms, however, may improve the patients' quality of life.

Aims: Authors studied the possible correlations between the Down-specific genes and the related biochemical changes.

[Challenge and limitations in determination of serum creatinine].

Since 2006 clinical guidelines have recommended that the estimated glomerular filtration rate should be calculated from serum creatinine for the early detection of chronic kidney disease. These brought into the limelight the limitations of the Jaffe method and for comparability of test results the different routine creatinine methods need to be harmonized. The disadvantage of the kinetic Jaffe creatinine determination is its low specificity.

[The epidemic of chronic kidney disease requires the estimation of glomerular filtration rate].

Nowadays chronic kidney disease has become a major public health problem due to the great increase in atherogenic nephropathies. In the absence of classic renal symptoms, chronic kidney disease is mostly diagnosed when renal failure is already advanced, although it can be revealed by laboratory tests in the earlier stages. When diagnosis is late, the progression to end-stage renal failure is unavoidable and renal replacement therapy is needed.

Anthracycline antibiotics induce acute renal tubular toxicity in children with cancer.

Experimental evidence suggests that anthracyclines, widely used in cancer chemotherapy, may impair kidney function. We assessed kidney function by serum creatinine, urinary N-acetyl-beta-D-glucosaminidase activity indices (NAGi) and microalbuminuria (MA) in 160 serum and urine samples obtained from 66 children with cancer. The effect of dexrazoxane was analyzed in 6 children on dexrazoxane supportive therapy in conjunction with daunorubicin (DNR) treatment, as compared with 6 children not receiving this agent.

["Turnaround time": a new parameter for the characterization of the overall efficacy of laboratory diagnostic processes].

Introduction: The authors developed a special computer-aided routine in their laboratory for the calculation of "turnaround time" which parameter is suitable for the characterization of the overall efficacy of laboratory diagnostic processes. The turnaround time is defined as the interval between the arrival time of a sample in the laboratory and the time of clinical validation. It characterizes the efficacy of the result generation process very well, and therefore, is considered as an important parameter of laboratory quality control.

Identification of sulfhemoglobinemia after surgical polypectomy.

Sulfhemoglobinemia (SHb) is an uncommon cause of cyanosis that is predominantly drug-induced in adults. We report an unusual case of sodium sulfate-induced sulfhemoglobinemia in a 61-year-old woman after surgical polypectomy. Fractional hemoglobin derivates were assayed by spectrophotometry and high-performance liquid chromatography.

Late effects on renal glomerular and tubular function in childhood cancer survivors.

Background: Late nephrotoxicity among childhood cancer survivors is poorly documented.

Methods: We investigated 115 patients and 86 controls assessing serum cystatin C concentration (CysC), urinary N-acetyl-beta-D-glucosaminidase activity (NAG), and microalbuminuria. Proteinuria was quantified and electrophoresis performed.

Cystatin C is a suitable marker of glomerular function in children with cancer.

Antineoplastic chemotherapy is associated with nephrotoxic side effects. Data on nephrotoxicity in childhood cancer are scanty, in part because of the difficulties in obtaining reliable markers of glomerular function. We used serum cystatin C (cysC) to assess glomerular function.

Age dependence of serum beta-N-acetylhexosaminidase (NAG) activity.

Serum N-acetyl-beta-D-glucosaminidase (NAG; EC 3.2.1.