Mild X-linked Alport syndrome due to the COL4A5 G624D variant originating in the Middle Ages is predominant in Central/East Europe and causes kidney failure in midlife.

A study of 269 children enrolled into a National Registry for children with persistent glomerular hematuria identified 131 individuals with genetically confirmed X-linked Alport Syndrome. A single variant c.1871G>A p.

Acute tubulointerstitial nephritis following aciclovir treatment for chickenpox in children with nephrotic syndrome - a report of two cases.

Tubulointerstitial nephritis (TIN) is an inflammatory process primarily involving the renal interstitium and is the cause of acute kidney injury (AKI) in 3-7% of cases confirmed by renal biopsy in children. Aciclovir may have a nephrotoxic effect by crystallization in renal tubules or by inducing an immunologic process that leads to development of TIN. We report 2 male patients, aged 10 and 8 years, with nephrotic syndrome (NS), in whom disease relapse was triggered by varicella zoster infection.

Carotid intima-media thickness in children with idiopathic nephrotic syndrome: A single center cross-sectional study .

Aim: Our aim was to assess common carotid artery intima-media thickness (cIMT) in children with idiopathic nephrotic syndrome (INS) and to find relation between cIMT and clinical and biochemical parameters in these patients.

Materials And Methods: In 50 children with INS we retrospectively evaluated: cIMT ((mm) and Z-score) and selected clinical and biochemical parameters. The control group consisted of 20 healthy children aged 9.

[Zwiększenie częstości zachorowania na ostre popaciorkowcowe kłębuszkowe zapalenie nerek w pierwszej połowie roku 2018 - doświadczenie jednego ośrodka].

Unlabelled: Acute poststreptococcal glomerulonephritis (APSGN) is a complication of infection with group A beta-hemolytic streptococcus. The disease manifests as microscopic/gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly self-limiting course.

Aim: The aim of study was the analysis of clinical course of APSGN in period of increased incidence in the first half of 2018.

Asymmetric dimethylarginine is not a marker of arterial damage in children with glomerular kidney diseases.

Introduction: Asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide synthase, correlates with cardiovascular risk especially in patients with chronic kidney disease. The aim of our study was to establish significance of ADMA as a biomarker of arterial damage in children with glomerulopathies.

Material And Methods: In 80 children with glomerulopathies (mean age, 11.

Tuberculosis infection in children with proteinuria/nephrotic syndrome.

Children with nephrotic syndrome (NS) are at greater risk of infections than the general population, due to immunodeficiency in the course of the disease and the treatment. In this study we present 4 children (2 girls, 2 boys), mean age 7.6 ±5.

Effectiveness of rituximab in nephrotic syndrome treatment.

Idiopathic nephrotic syndrome (INS) is a common chronic illness characterized by massive proteinuria and hypo-albuminemia in children. Baseline treatment is 6 month-corticotherapy. In cases of steroid resistant/dependent INS several types of treatment are used, including course of methyloprednisolone "pulses", alkylating agents, cyclosporin A, levamisole and mycophenolate mofetil.

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models.

Renalase in Children with Glomerular Kidney Diseases.

Studies suggest that renalase, a renal catecholamine-inactivating enzyme, plays a major role in the pathogenesis of kidney and cardiovascular diseases in adults. This study seeks to determine the role of renalase in children with glomerular kidney diseases. We evaluated the serum renalase, arterial stiffness, intima-media thickness, blood pressure, and clinical and biochemical parameters in 78 children (11.

Lupus nephritis in children - 10 years' experience.

Systemic lupus erythematosus (SLE) in children is usually more severe than it is in adults and there is a higher incidence of renal involvement. We described 18 children (16 girls, 2 boys) with lupus nephritis (LN), whose average age was 14.4 ±1.

Levamisole therapy in children with frequently relapsing and steroid-dependent nephrotic syndrome: a single-center experience.

Introduction: Numerous studies suggest that levamisole, an antihelmintic agent with an immunomodulatory effect, reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome (FRNS/SDNS). The aim of the study was to present a single center's experience in treatment of FRNS and SDNS with levamisole.

Material And Methods: Among 72 children with FRNS/SDNS treated in our department with levamisole in the years 1984-2011 we studied in detail 53 patients (mean age: 6.

Body weight changes in children with idiopathic nephrotic syndrome.

Background: Aim of the study was to evaluate factors affecting body mass change in children with idiopathic nephrotic syndrome (INS) during 6-months treatment of initial disease bout with glucocorticoids (GC).

Material And Methods: We studied 31 children with INS (22♂, 9♀, 3.6±1.

Mycoplasma pneumoniae as a trigger for Henoch-Schönlein purpura in children.

Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis.

Hemodiafiltration efficacy in treatment of methanol and ethylene glycol poisoning in a 2-year-old girl.

Introduction: Every year about 2.4 million people in USA are exposed to toxic substances. Many of them are children below 6 years of age.

Continuous veno-venous hemodiafiltration in methotrexate intoxication.

Unlabelled: Methotrexate is a highly nephro- and hepatotoxic drug used in osteosarcoma treatment protocols, in children and adults. High dose methotrexate therapy may lead to kidney injury and decrease of methotrexate clearance, followed by an increase of its serum concentration. As a result, systemic intoxication may develop.

Mutational analysis in podocin-associated hereditary nephrotic syndrome in Polish patients: founder effect in the Kashubian population.

Hereditary nephrotic syndrome is caused by mutations in a number of different genes, the most common being NPHS2. The aim of the study was to identify the spectrum of NPHS2 mutations in Polish patients with the disease. A total of 141 children with steroid-resistant nephrotic syndrome (SRNS) were enrolled in the study.

Genetic screening in adolescents with steroid-resistant nephrotic syndrome.

Genetic screening paradigms for congenital and infantile nephrotic syndrome are well established; however, screening in adolescents has received only minor attention. To help rectify this, we analyzed an unselected adolescent cohort of the international PodoNet registry to develop a rational screening approach based on 227 patients with nonsyndromic steroid-resistant nephrotic syndrome aged 10-20 years. Of these, 21% had a positive family history.

[Neurological symptoms in patient with focal segmental glomerulosclerosis treated with cyclosporin A--case report].

Unlabelled: Cyclosporin A using in treatment glomerulonephritis can cause neurological side effects.

Case Report: Nephrotic syndrome (NS) with microscopic hematuria was recognized in a boy in the age of 9.7 years.