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Therapeutic plasma exchange (TPE) is a recognized treatment in pediatric immune-mediated and toxicological disorders, though evidence remains limited. This study assesses the safety and efficacy of TPE in children with non-neurological diseases treated at a tertiary hospital over 27 years. We focused on non-neurological indications to avoid excessive data heterogeneity. This retrospective analysis included 97 children (51 nephrological, 46 non-nephrological), who underwent 380 TPE procedures between 1997 and 2023. Indications were categorized according to ASFA 2023 guidelines. Effectiveness was evaluated semi-quantitatively based on clinical and laboratory parameters up to 3 months post-treatment. Safety was assessed by incidence of adverse events (AEs), allergic AEs, and mortality. The most common indication was thrombotic microangiopathy (TMA, 23.7%), followed by toxicological and hematological disorders. TPE was effective (complete or partial improvement) in 67% of patients; full recovery occurred in 25.8%. In the nephrology group, eGFR increased 3.8-fold by 3 months post-TPE. However, immunosuppressive therapy was used 7.7 times more often in this group. The non-nephrological group had a higher mortality (26.1% vs 9.8%), mostly due to multiple organ failure. Intensive Care Unit admission and ASFA III indications significantly decreased survival odds. Fresh-frozen plasma (FFP) - based TPE was associated with significantly more AEs and interventions compared to TPE without FFP. Over time, FFP use declined, with 100% of TPEs in 2023 performed without FFP. TPE is a safe and effective therapy in selected pediatric non-neurological conditions when performed in specialized centers.

The aim of the study was to investigate the relationship between the severity of typical clinical symptoms, severity of histopathological lesions in kidney biopsies in IgA vasculitis nephritis (IgAVN) and to propose indications for kidney biopsy in children. Material and methods This retrospective study enrolled 106 patients, included in the IgAVN registry of Polish children, diagnosed by kidney biopsy. Renal and extrarenal symptoms at onset of the disease were analyzed. Biopsy results were assessed using Oxford classifications (MEST-C). The patients were divided into 3 groups depending on the severity of proteinuria: A-nephrotic proteinuria with hematuria; B-non-nephrotic proteinuria with hematuria; C-isolated hematuria. Results The first symptoms of nephropathy were observed at the 0.7 (1–128.4) months from the onset of extrarenal symptoms. Kidney biopsy was performed on 39 (6–782) days after the onset of nephropathy symptoms. MEST-C score 4 or 5 was significantly more frequent in children from group A than in groups B and C. Significantly higher mean MEST-C score was found in patients with abdominal symptoms than without. In group A: S0 and T0 we found in significantly shorter time to kidney biopsy than in S1, T1–2 p  < 0.05) and in group B the significantly shorter time in T0 compare to T1–2 p < 0.05). The ROC analysis shows that S1 changes appear in kidney biopsies in group A with cut off 21 days (AUC 0,702, p  = 0.004, sensitivity 0.895 specificity 0.444) T1–2 changes after 35 days (AUC 0.685, p  = 0.022, sensitivity 0.750, specificity 0.615), and in goupn B T1–2 cut off is 74 days (AUC 0,738, p  = 0.002, sensitivity 0.667, specificity 0.833). Conclusions In childhood IgAVN, the severity of changes in the urine is clearly reflected in the result of a kidney biopsy. The biopsy should be performed in patients with nephrotic proteinuria no later than 3 weeks after the onset of this symptom in order to promptly apply appropriate treatment and prevent disease progression. Accompanying abdominal symptoms predispose to higher MESTC score.

Background/Aim: As continuous renal replacement therapy (CRRT) has emerged as a standard therapy in pediatric intensive care units (PICU), many related issues that may have an impact on circuit survival have gained in importance. Objective of the study was an evaluation of factors associated with circuit survival, including anticoagulation (ACG). Methods: Retrospective study that included 40 patients, who in total received 7636 hours of CRRT during 150 sessions (84 filters, 4260 hours with heparin anticoagulation (Hep-ACG); 66 filters, 3376 hours with regional citrate anticoagulation (RCA)). Results: The Kaplan-Meier analysis of the total circuit survival time depending on the type of ACG did not demonstrate a significant difference between Hep-ACG and RCA. The percentage of clotted filters was significantly higher in case of smaller filters (HF20: 58.8%; ST60: 29.5%; ST100: 15.8%), and their lifetime was significantly lower regardless of ACG (the mean and median lifetime for HF20: 38.7/27.0 h; for ST60: 54.1/72.0 h., for ST100: 62.1/72.0 h, respectively). Conclusions: Irrespectively of filter size, filter clotting occurs within the first 24 hours after the initiation of CRRT. Most commonly, clotting affects small filters, and their lifetime is significantly shorter as compared to larger filters regardless of the type of the ACG.

Background The role of idiopathic nephrotic syndrome (INS) in the pathogenesis of atherosclerosis in childhood has not been clearly elucidated. However, antioxidative defense in INS is thought to be imbalanced. This study aimed to assess the changes of plasma concentration of selected aminothiols in the blood of children with INS at various stages of the disease. Methods This cross-sectional study was conducted in 125 children aged 2-18 years. The children were divided into 4 groups: group A, early relapse ( n =37); group B, early remission for 4-6 weeks from the onset ( n =37); group C, late steroid-free remission ( n =31); and group D, long-term remission for 2-5 years ( n =20). Control group (E) consisted of 30 age- and gender-matched healthy children. The study protocol comprised an analysis of plasma concentrations of glutathione, homocysteine, cysteine and cysteinylglycine by high-performance liquid chromatography. Fractions of protein-bound and free aminothiols were measured. Endothelial injury was assessed by thrombomodulin, PAI-1 concentration, and von Willebrand factor activity. Results The children with INS had unbalanced aminothiol metabolism only in relapse and early remission, that shifted towards increased oxidative processes. Administration of cyclosporine A caused a significant increase in homocysteine and cysteine concentration. Changes in aminothiol metabolism were significantly related to endothelial injury. Conclusions The findings of this study may be helpful in elucidating the pathogenesis of premature atherosclerosis in patients with INS refractory to the treatment or in the case of frequent relapse.

Background: Hypertension very often accompanies progression of chronic kidney disease (CKD) in children. A cross-sectional analysis of hypertension prevalence in dialyzed children in Poland was designed with a comparison with the data previously recorded 10 years earlier. Methods: Two cohorts of children were analyzed: 59 subjects dialyzed in 2013, and 134 children from the previous study performed in 2003 that were reevaluated according to the current methodology. The incidence of hypertension (defined by SDS of sBP or dBP >1.64), clinical data, medical history, dialysis modalities and selected biochemical parameters of dialysis adequacy were analyzed. Results: The prevalence of hypertension increased from 64% in 2003 to 78% in 2013. The efficacy of antihypertensive treatment remained unsatisfactory (61% proper BP control). Preservation of residual urine output and strict fluid balance may prevent development of hypertension in children on dialysis. Conclusions: Despite the higher awareness of hypertension and its complications in dialyzed children, the incidence of this entity has increased during the last decade, with the percentage of undertreated patients comparable to that observed 10 years ago. Thus, more attention should be paid to therapy efficacy in this population to prevent further damage to the cardiovascular system and to decrease morbidity.

Background/Aims: Chronic medical illness is a significant risk factor for the development of psychiatric disorders. The aims of the study were: to investigate the level of anxiety in children with chronic kidney disease (CKD) and to identify factors associated with the presence of that emotional problem. Methods: CKD children on hemodialysis (HD, n=22), peritoneal dialysis (PD, n=20,) and on conservative treatment (CT, n=95) were enrolled in the study. We used State-Trait Anxiety Inventory (STAI) for adolescents and STAI-C for children. Socio-demographic and physical factors were assessed. Results: There was a significantly higher level of anxiety-state among HD children (8-12 years) compared with other groups of participants of the same age and Polish population norms. The level of anxiety among adolescents (13-18 years), both anxiety-state and anxiety-trait, was significantly higher in the HD group compared with other groups, which did not differ among themselves. In the HD adolescents, there was a correlation between the anxiety-state and the duration of the disease as well as with the number of hospitalizations. PD adolescents in the mainstream education had higher levels of anxiety-state and anxiety-trait compared with home schooled patients. Conclusions: Even though children and adolescents with CKD are at risk of developing a variety of emotional disorders, the level of anxiety among the researched group, with the exception of HD patients, was not significantly different than the level of anxiety among healthy subjects. Adolescents on HD who present a high level of anxiety should undergo long-term psychological treatment.

Background/Aims: The recent improvements of management of patients in pediatric intensive care units (PICU) are associated with improved outcome. However, this decrease in mortality is associated with an increased number of children with acute kidney injury (AKI), especially in patients with multiorgan failure. Methods: The report presents a retrospective analysis of 25 cases of AKI (assessed based on the pRIFLE criteria) in PICU within 7 years. Results: AKI was diagnosed in 1.24% of all hospitalized children. AKI percentage duration (as compared to the total hospitalization time) in the children who died vs. the survivors was 79.55% vs. 46.19%, respectively (p<0.05). The mortality rate of AKI patients was 40% which was 4.4-times higher as compared to the total mortality rate in PICU. The final cumulative survival ratio (FCSR) of patients meeting the oliguria criterion (which was met in 48% of AKI patients) was 37% vs. 49% in non-oliguric children. Averaged urine output values in the first week of hospitalization in the deceased vs. survivors were 1.49 vs. 2.57 ml/kg/h, respectively (p<0.05). Conclusions: Oliguria should not be considered as a sensitive parameter for AKI diagnosing in children below one year of age. A decreased mean urine output in the first week of PICU hospitalization (less than 1.4 ml/kg/h) should be considered as a poor prognostic factor. In many cases AKI was diagnosed too infrequently and too late.

Congenital anomalies of the kidney and urinary tract (CAKUT) are a major cause of pediatric kidney failure. We performed a genome-wide analysis of copy number variants (CNVs) in 2,824 cases and 21,498 controls. Affected individuals carried a significant burden of rare exonic (that is, affecting coding regions) CNVs and were enriched for known genomic disorders (GD). Kidney anomaly (KA) cases were most enriched for exonic CNVs, encompassing GD-CNVs and novel deletions; obstructive uropathy (OU) had a lower CNV burden and an intermediate prevalence of GD-CNVs; and vesicoureteral reflux (VUR) had the fewest GD-CNVs but was enriched for novel exonic CNVs, particularly duplications. Six loci (1q21, 4p16.1-p16.3, 16p11.2, 16p13.11, 17q12 and 22q11.2) accounted for 65% of patients with GD-CNVs. Deletions at 17q12, 4p16.1-p16.3 and 22q11.2 were specific for KA; the 16p11.2 locus showed extensive pleiotropy. Using a multidisciplinary approach, we identified TBX6 as a driver for the CAKUT subphenotypes in the 16p11.2 microdeletion syndrome. Genome-wide analysis of copy number variants in 2,824 cases across the phenotypic spectrum of CAKUT sheds light on the genomic architecture of disease and identifies TBX6 as a driver for CAKUT subphenotypes in the 16p11.2 microdeletion syndrome.

Cardiovascular diseases are substantial causes of mortality among patients with chronic kidney disease (CKD). The aim of the study was an assessment of the impact of cardiovascular risk factors on left ventricular hypertrophy (LVH) in children with CKD. Material and methods The study was conducted in a group of 71 children with mean age 11 years and CKD stage 1 to 5. Serum cystatin C, albumin levels, and lipids profile were measured. Ambulatory blood pressure measurements and echocardiography were performed. Results LVH was detected in 34 out of 71 children. In children with LVH, significantly higher values of BP were observed in 24-hour measurements: systolic (119 vs. 109 mm Hg; p = 0.002), diastolic BP (73 vs. 65 mm Hg; p = 0.009) and MAP (89 vs. 81 mm Hg, p = 0.004). These significantly higher BP values were observed within day and night. Increased cholesterol level was found in 25, LDL in 12, TGL in 28, and a decreased HDL in 20 children. In children with LVH higher BMI (18.6 vs. 16.7 kg/m 2 ;p = 0.039) and lower albumin (41.5 vs. 45.4 g/l; p = 0.013), HDL (1.14 vs. 1.5 mmol/l; p = 0.001) and Ca levels (2.36 vs. 2.47 mmol/l; p = 0.03) were found. Obesity and low HDL level were independent LVH risk factors. The results indicate a 3-fold increase in the risk of LVH in children with hypertension (OR 3.18, p = 0.045), rising up when 2–3 risk factors were present (OR 6, p = 0.015). Conclusions Hypertension, a decreased HDL cholesterol level and overhydration have significant impact on the development of LVH in CKD children.