Combined Therapy with Azathioprine, Prednisone, and Enalapril in Children with IgAN and IgAVN.

: The aim of this study was to evaluate the efficacy of 1-year treatment in children with IgAN and IgAVN using azathioprine, prednisone, and enalapril (AZA+PRED+E) combined with a control kidney biopsy. : This study consists of 68 children diagnosed via kidney biopsy with Oxford classification. The study group included 36 children (15 IgAN, 21 IgAVN) treated with AZA+PRED+E (according to the protocol with a control kidney biopsy); and the control group included 32 children (21 IgAN, 11 IgAVN) who were treated with enalapril alone during one year after kidney biopsy.

PL1 and PM1 versus Placebo as Prophylaxis for Recurrence of Urinary Tract Infections in Children.

Urinary tract infections (UTIs) rank among the most prevalent bacterial infections in children. Probiotics appear to reduce the risk of recurrence of UTIs. This study aimed to evaluate whether probiotics containing PL1 and PM1 therapy prevent UTIs in the pediatric population compared to a placebo.

Development of a tool for predicting HNF1B mutations in children and young adults with congenital anomalies of the kidneys and urinary tract.

Background: We aimed to develop a tool for predicting HNF1B mutations in children with congenital abnormalities of the kidneys and urinary tract (CAKUT).

Methods: The clinical and laboratory data from 234 children and young adults with known HNF1B mutation status were collected and analyzed retrospectively. All subjects were randomly divided into a training (70%) and a validation set (30%).

A rare cause of steroid-resistant nephrotic syndrome - a case report.

Steroid resistance is a common condition occurring in children with nephrotic syndrome. Until now, over 50 genes involved in steroid-resistant nephrotic syndrome (SRNS) pathogenesis have been identified, among which the most prevalent are NPHS1, NPHS2, CD2AP, and PTPRO. The patterns of inheritance of SRNS are autosomal recessive, autosomal dominant, or mitochondrial, and tissues of those patients show focal segmental glomerulosclerosis (FSGS) signs in histopathological image analysis.

Thrombotic complications in children with first-episode steroid-sensitive nephrotic syndrome: A single-center experience.

Objective: The aim of the study was to evaluate the clinical course and risk factors of venous thromboembolic complications (VTEC) in children with a first episode of steroid-sensitive nephrotic syndrome (SSNS).

Materials And Methods: We retrospectively analyzed the medical records of children hospitalized due to SSNS in one pediatric nephrology unit between 2012 and 2019. Demographic data, clinical symptoms at the onset of NS, and laboratory parameters were compared between patients with and without VTEC.

24-hour central blood pressure and immune system activation in adolescents with primary hypertension - a preliminary study.

Introduction: Adult and pediatric data suggest a positive relationship between the extent of subclinical inflammation, blood pressure, and hypertension-mediated organ damage (HMOD) in primary hypertension (PH). 24-hour (24-h) ambulatory blood pressure (ABPM) and central blood pressure (CBP) are strong predictors of HMOD. Our study aimed to analyze the relationship between 24-h central ABPM, subclinical inflammation, and clinical data in adolescents with PH.

Nephrological and urological symptoms in patients with Robinow syndrome - a report of two cases.

Unlabelled: Robinow syndrome is a rare congenital syndrome described in 1969 by Meinhard Robinow. The genetic background is heterogeneous - mutations of DVLI1, DVLI3, WNT5A genes (mild, autosomal dominant inheritance) or ROR2 gene (severe, autosomal recessive inheritance) are responsible for the syndrome. The syndrome is characterized by facial dysmorphism, skeletal defects, short stature, cardiovascular and urinary system abnormalities.

Circadian Blood Pressure Profile in Pediatric Patients with Primary Hypertension.

Our study aimed to evaluate factors affecting circadian BP profile and its association with hypertension-mediated organ damage (HMOD) in pediatric patients with primary hypertension (PH). The study included 112 children (14.7 ± 2.

Reduced Blood Pressure Dipping Is A Risk Factor for the Progression of Chronic Kidney Disease in Children.

Background: Elevated blood pressure and proteinuria are well-established risk factors for chronic kidney disease (CKD) progression in children. This study aimed to analyze risk factors for CKD progress, emphasizing detailed ambulatory blood pressure (ABPM) data. Methods: In 55 children with CKD II−V, observed for ≥1 year or until initiation of kidney replacement therapy, we analyzed ABPM, clinical, and biochemical parameters.

Evaluation of Active Renin Concentration in A Cohort of Adolescents with Primary Hypertension.

Our study aimed to assess active renin concentration in children with primary hypertension. Thus, we evaluated active renin concentration, clinical parameters, office and ambulatory blood pressure, and biochemical parameters in 51 untreated adolescents with primary hypertension (median: 14.4 [interquartile range-IQR: 13.

Serum and urine periostin and cytokeratin-18 in children with congenital obstructive nephropathy.

Congenital obstructive nephropathy (CON) is one of the most common causes of chronic kidney disease in children. The aim of the study was to investigate serum and urine periostin and cytokeratin-18 (CK-18) in children with CON in relation to CON etiology, treatment, and kidney injury. We evaluated 81 children with CON secondary to ureteropelvic junction obstruction (UPJO), ureterovesical junction obstruction (UVJO), posterior urethral valves (PUV) and 60 controls.

Burnout Syndrome among Pediatric Nephrologists-Report on Its Prevalence, Severity, and Predisposing Factors.

Background And Objectives: Burnout is an occupation-related syndrome comprising emotional exhaustion, depersonalization, and reduced feelings of work-related personal accomplishments. There are reports on burnout among adult nephrologists and general pediatricians, but little is known about burnout among pediatric nephrologists. The aim of our study was to assess the prevalence and severity of burnout syndrome among Polish pediatric nephrologists.

Developmental Abnormalities of Teeth in Children With Nephrotic Syndrome.

Introduction: Treatment with glucocorticoids in children with nephrotic syndrome can be the cause of developmental disorders of the masticatory organ and bone or teeth abnormalities. The aim was to assess the frequency and type of dental abnormalities and the correlation of their occurrence with a dosage of glucocorticoids and treatment time in children with idiopathic nephrotic syndrome.

Methods: The study group consisted of 31 patients aged 5 to 17 diagnosed with idiopathic steroid-sensitive nephrotic syndrome and 33 overall healthy children.

The Usefulness of Vanin-1 and Periostin as Markers of an Active Autoimmune Process or Renal Fibrosis in Children with IgA Nephropathy and IgA Vasculitis with Nephritis-A Pilot Study.

This study aimed to evaluate the usefulness of vanin-1 and periostin in urine as markers of the autoimmune process in kidneys and renal fibrosis in IgA nephropathy (IgAN) and IgA vasculitis with nephritis (IgAVN). From a group of 194 patients from the Department of Pediatrics and Nephrology, who were included in the Polish Pediatric Registry of IgAN and IgAVN, we qualified 51 patients (20 with IgAN and 31 with IgAVN) between the ages of 3 and 17, diagnosed based on kidney biopsy, for inclusion in the study. All of the patients received glucocorticosteroids, immunosuppressive drugs, or renoprotective therapy.

Acute post-streptococcal glomerulonephritis - immune-mediated acute kidney injury - case report and literature review.

Acute post-streptococcal glomerulonephritis (APSGN) is an immunological complication of infection with group A -hemolytic streptococcus (GAS). The disease manifests as microscopic or gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly a self-limiting course. We report a very severe case of APSGN in a 5-year-old girl with superimposed generalized infection.

The role of periostin in kidney diseases.

Chronic kidney disease (CKD) is a multi-symptomatic condition resulting from irreversible functional and structural damage to the kidneys. Therefore, finding a specific and sensitive marker to predict the development and progression of CKD is of great interest. Periostin is a matricellular protein involved in tissue remodeling and wound healing.

Serum neutrophil gelatinase-associated lipocalin for predicting anemia of inflammation in children with urinary tract infection.

Anemia of inflammation (IA), the second most common cause of childhood anemia, results from macrophage iron sequestration and impaired erythropoiesis. Neutrophil gelatinase-associated lipocalin (NGAL) plays an important role in innate microbial immunity through its influence on intracellular iron homeostasis and inhibition of erythropoiesis. The predictive value of NGAL in IA was assessed in 74 children (age 6.

Early Vascular Aging in Children With Tuberous Sclerosis Complex.

Experimental data indicate that activating mutations in the mTOR (mammalian target of rapamycin) pathway may lead to abnormal arterial wall structure. Vascular anomalies like arterial stenoses are reported in pediatric patients with tuberous sclerosis complex (TSC). In addition, large renal lesions (angiomyolipoma-AML and cysts) are risk factors for arterial hypertension in adult patients with TSC.

The Usefulness of Urinary Periostin, Cytokeratin-18, and Endoglin for Diagnosing Renal Fibrosis in Children with Congenital Obstructive Nephropathy.

Congenital obstructive nephropathy (CON) leads to renal fibrosis and chronic kidney disease. The aim of the study was to investigate the predictive value of urinary endoglin, periostin, cytokeratin-18, and transforming growth factor-β1 (TGF-β1) for assessing the severity of renal fibrosis in 81 children with CON and 60 controls. Children were divided into three subgroups: severe, moderate scars, and borderline lesions based on 99mTc-ethylenedicysteine scintigraphy results.

Treatment of idiopathic nephrotic syndrome with two steroid dosing regimens - one-year observational study.

Introduction: The aim of the study was to compare the first year of disease in children with idiopathic nephrotic syndrome (INS) treated according to two prednisone dosing regimens: a weight-based schedule (2 mg/kg/24 h in the 1 month, 2 mg/kg/48 h in the 2 month, with dose tapering during the following 4 months), and a body surface area (BSA)-based schedule (60 mg/m/24 h in the 1 month, 40 mg/m/48 h in the 2 month, with dose tapering during the following 4 months).

Material And Methods: In 2 groups of children treated with weight- and BSA-based regimens (20 patients, 3.13 ±1.

Arterial stiffness in children with primary hypertension is related to subclinical inflammation.

Introduction: The immune system can trigger an inflammatory process leading to blood pressure elevation and arterial damage. The aim of the study was to assess the relation between subclinical inflammation and arterial damage in pediatric patients with primary hypertension (PH) and to establish the usefulness of neutrophil-to-lymphocyte (NLR) and platelet-to-lymphocyte (PLR) ratios, and mean platelet volume (MPV) as markers of arterial damage in these subjects.

Material And Methods: In 119 children with PH (14.

Relationship between Gd-IgA1 and TNFR1 in IgA nephropathy and IgA vasculitis nephritis in children - multicenter study.

Aim Of The Study: To evaluate the relationship between serum Gd-IgA1 (sGd-IgA1) and serum and urine TNFR1 (sTNFR1, uTNFR1) levels as possible prognostic factors in IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN).

Material And Methods: From 299 patients from the Polish Registry of Pediatric IgAN and IgAVN, 60 children (24 IgAN and 36 IgAVN) were included in the study. The control group consisted of 20 healthy children.