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Introduction and aim of the study: During their lives, people with a solitary kidney develop hyperfiltration damaging the kidney and results in decreased renal function. Reduced Schwartz-estimated GFR is a relatively late marker of renal damage. According to some authors, an earlier marker of subclinical damage to the solitary kidney is serum cystatin C. The study aimed to determine whether in children with a congenital solitary/solitary functioning kidney (SFK), cystatin C-estimated GFR is significantly lower than creatinine-estimated GFR. Methods: A prospective study assessed GFRs in 70 children aged 2–18 years (mean 9.1 years; 49 boys) with a SFK due to unilateral agenesis or multicystic dysplasia. Excluded were children with SFK and additional CAKUT or a history of febrile UTI. Serum creatinine (μmol/L) was measured using the enzymatic method. Cystatin C (mg/L) was measured with an immunoturbidimetric assay. A difference was determined between GFRs estimated with the following formulae: Schwarz-estimated GFR: height in cm x 0.6/creatinine Cystatin C-estimated GFR: 1.178 x (Scyst)-0.931 For statistical analyses, the paired t-test, non-parametric Wilcoxon test and Bland-Altman plot were used. Results: The mean Schwartz- and cystatin C-estimated GFRs were 1.639 (SD 0.295) and 1.268 (SD 0.196) mL/s/1.73 m2, respectively; a statistically significant difference (p<0.0001). The Bland-Altman plot suggested that the difference between the two methods depended on the GFR; the higher the GFR, the greater the difference. Conclusions: In children with an intact solitary kidney (normal GFR to CKD II), cystatin C-estimated GFR was significantly lower than Schwartz-estimated GFR. Since the difference was GFR-dependent, it cannot be concluded that in children with a solitary kidney, reduced cystatin C-estimated GFR is an adequate early marker of subclinical renal damage. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a severe highly heterogenous multisystemic inflammatory disease, characterized by a combination of clinical and immunological criteria. The aim of a paper is to show variability of clinical and hematological findings, that could be first symptoms of developing SLE and might precede diagnosis by different time ranging from weeks to many years. These findings encompasses immune thrombocytopenic purpura (ITP), autoimmune hemolytic anemia, and asymptomatic strong positivity of anticardiolipine antibodies, which lasted from one month to 7 years in our patients. We highlight an importance of early assessment of autoantibodies in patients with these hematologic diseases and need for a deep collaboration between hematologist and rheumatologist, which is a quarantee of early SLE diagnosis and therapy initiation. [ABSTRACT FROM AUTHOR]

Here we report spontaneous recovery of 2 episodes of atypical hemolytic-uremic syndrome (aHUS) in a 17-year-old male. In both cases, the condition was caused by seasonal influenza (first, influenza A/H3; second, after 1 year, influenza B). Already at the first onset of aHUS, decreased expression of CD46 (membrane cofactor protein, MCP) on granulocytes was found, suggesting the diagnosis of complement-mediated HUS. Because the patient's clinical condition was stable and laboratory results were improving since day 3, we decided not to administer eculizumab, a drug whose administration is recommended within the first 48 hours in children, but to continue symptomatic therapy only. However, eculizumab was available in case that the patient's clinical condition deteriorated. Spontaneous remission of HUS was achieved in both episodes of aHUS. Considering the presumed risk of aHUS relapse, genetic examinations were done following the first episode. The patient was confirmed to carry a mutation in MCP (CD46) gene (heterozygote for the p.C35Y mutation) and also a high-risk MCPggaac haplotype of the same gene. We assume that both mentioned genetic lesions played a pathogenic role in the development of complement-mediated aHUS, with influenza being the triggering condition. Molecular genetic tests were also performed in the patient's parents and found accordingly, that the father is a heterozygote for the p.C35Y mutation and the mother is heterozygous for the high-risk MCPggaac haplotype of the MCP (CD46) gene. We confirmed that in an individual with a mutation in MCP, seasonal influenza may be a trigger of aHUS and spontaneous remission is possible. [ABSTRACT FROM AUTHOR]

The risk factors for recurrent urinary tract infection vary by age and thus, the diagnostic and treatment approach should vary accordingly. Whereas the main risk factor in children under 2 years of age is vesicoureteral reflux, in girls able to control their voiding (older than 3 years of age), recurrent urinary tract infection is more frequently caused by lower urinary tract dysfunction. At this age, noninvasive urodynamic testing is preferred to voiding cystourethrography. The former comprises filling in an intake and voiding diary for 2-4 days, measuring bladder wall thickness and post-void residual volume, and uroflowmetry. Based on noninvasive urodynamic testing results, daily measures and/or medication therapy are recommended. [ABSTRACT FROM AUTHOR]

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