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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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Background: Reduced renal parenchymal thickness (PT) is a parameter used by clinicians to assess the degree of hydronephrosis. In patients with a congenital hydronephrotic solitary functioning kidney (SFK), PT is difficult to determine as there is no comparison with the contralateral kidney. The aim of this study was to obtain ultrasound measurements of PT in children with normal SFK and to compare these data with PT measurements in children with two functioning kidneys., Methods: This was a prospective multicenter study carried out between 2006 and 2011 in which 236 children aged 11 days to 18.96 years with healthy SFK were examined. The SFK etiologies were unilateral renal agenesis or a nonfunctioning contralateral kidney, mostly due to multicystic dysplasia. In addition to determining other parameters, we measured PT in the middle third of the kidney by ultrasound. Correlations between PT and age, height and weight were assessed., Results: Correlation analysis showed a positive correlation with renal PT for all parameters. The correlation coefficients for age, height and weight were 0.863, 0.873 and 0.874, respectively. In most age categories, the renal parenchyma was significantly thicker in the SFK than in two functioning kidneys., Conclusions: Based on our results, we suggest that PT in the SFK is correlated with height, weight and age of the patient. Consequently, measurements of PT may be used for monitoring the development of the healthy SFK and may contribute to a more accurate assessment of the severity of SFK anomalies.

The study was aimed at (1) the determination of the incidence of abnormalities of the urinary tract in newborn infants detected by postnatal ultrasound screening, and (2) the evaluation of the diagnostic accuracy of postnatal ultrasound screening for detecting surgical urinary tract abnormalities. The prospective study was of full-term neonates born in the University Hospital of Olomouc in 2005-2008 who underwent renal ultrasound screening after 72 h of life. Significant findings were recorded. Subsequent diagnostic and therapeutic procedures were recorded and evaluated in a group of children with detected renal pelvic dilatation (RPD). (1) A total of 6,088 newborn infants was examined. The absolute and relative RPD incidence rates (anteroposterior diameter, APD) were as follows: 5-7 mm, 146 (2.4%); 7-10 mm, 70 (1.15%); 10-15 mm, 13 (0.21%), and 15 mm or more, 5 (0.08%). Of those, 16 children were operated on for abnormalities of the urinary tract, of which nine (56%) had been detected by prenatal screening. Other findings: six cases of unilateral renal agenesis, four cases of multicystic renal dysplasia, four of renal dystopia, one of polycystic kidney disease and one of renal hypoplasia. (2) A group of 224 children with postnatally detected RPD was examined, of whom 40 (17.9%) underwent voiding cystourethrography and/or scintigraphy and 16 (7.1%) were treated surgically. The receiver operating characteristic curves were analyzed, and the areas under the curves were calculated. Postnatal renal ultrasound screening is probably a suitable test for detecting significant urinary tract abnormalities.

Background: Left-ventricular hypertrophy (LVH) is a risk factor for cardiovascular morbidity. Antihypertensive treatment with angiotensin-converting enzyme inhibitors (ACEI) is able to induce the regression of LVH in adults. However, there has been no study of the ability of ACEI to induce the regression of LVH in children. Our aim was to investigate the effect of ramipril on left-ventricular mass and blood pressure (BP) in hypertensive children., Methods: Twenty-one children (median age, 15 years) with renal (76%) or primary (24%) hypertension were prospectively treated with ramipril monotherapy for 6 months. Blood pressure was evaluated using ambulatory BP monitoring, with hypertension defined as mean BP >or=95th percentile. Left-ventricular hypertrophy was defined either as left-ventricular mass index (LVMI) >38.6 g/m(2.7) (pediatric definition) or as LVMI >51.0 g/m(2.7) (adult definition)., Results: Nineteen children completed the study. The median LVMI decreased from 36.8 g/m(2.7) (range, 18.9 to 55.8 g/m(2.7)) to 32.6 g/m(2.7) (range, 19.0 to 52.1 g/m(2.7); P < .05) after 6 months. The prevalence of LVH decreased from 42% to 11% using the pediatric definition (P < .05) and did not change using the adult definition (ie, it remained at 5%). The median ambulatory BP decreased by 11, 7, 8, and 7 mm Hg for daytime systolic, daytime diastolic, nighttime systolic, and nighttime diastolic BP (P < .05), respectively. A positive correlation was found between LVMI and nighttime systolic BP at the start of the study (r = 0.46, P < .05)., Conclusions: Ramipril is an effective drug in children with hypertension, for its ability to reduce not only BP but also left-ventricular mass and induce regression of LVH.

Background: Angiotensin-converting enzyme inhibitors are the drugs of choice in renal hypertension. The efficacy and safety of ramipril in adults has been proved; however, data on effectiveness of ramipril in children are few. The aim of the present study was to investigate the effect of ramipril on blood pressure (BP) and proteinuria in children with chronic kidney diseases., Methods: A total of 31 children (median age 11.3 years, range 1.9-19.8 years) with various chronic nephropathies and hypertension or proteinuria were prospectively treated with ramipril for 6 months. Blood pressure was evaluated using ambulatory BP monitoring and hypertension was defined as mean BP equal to or greater than the 95th percentile for healthy children. Proteinuria was defined as protein excretion > or =100 mg/m(2)/24 h. The starting dose of ramipril was 1.5 mg/m(2)/24 h once daily. In 27 children it was given as monotherapy., Results: The median decrease in ambulatory BP was 11 mm Hg for daytime systolic, 10 mm Hg for daytime and nighttime diastolic, and 8 mm Hg for nighttime systolic BP. Hypertension normalized in 55% of the children. Proteinuria decreased in 84% of the children with pathologic proteinuria; the median decrease was 51%. A positive correlation was found between initial proteinuria and change of proteinuria (r = 0.95, P <.001). Glomerular filtration rate and serum potassium level did not change significantly. One child developed a cough that was believed to be related to ramipril., Conclusions: Ramipril is an effective and safe drug in children with chronic kidney diseases associated with hypertension, proteinuria, or both.