Involvement of Hemopexin in the Pathogenesis of Proteinuria in Children with Idiopathic Nephrotic Syndrome

Hemopexin (Hpx) is considered a factor in the pathogenesis of idiopathic nephrotic syndrome (INS). The aim of the study was to evaluate the serum and urine values of Hpx (sHpx and uHpx) in children with INS, analyze the role of Hpx, and assess its usefulness as a marker of the disease course. 51 children with INS and 18 age-matched controls were examined. Patients were divided into subgroups depending on the number of relapses (group IA—the first episode of INS, group IB—with relapses) and according to method of treatment (group IIA treated with gluco-corticosteroids (GCS), group IIB treated with GCS and other immunosuppressants). Hpx concentrations were determined by enzyme-linked immunosorbent assay (ELISA). sHpx and uHpx values in relapse were elevated in the whole INS group versus controls (p < 0.000). In remission their levels decreased, but still remained higher than in the control group (p < 0.000). In group IB uHpx levels were increased during remission as compared to group IA (p < 0.006). No significant impact of immuno-suppressants on sHpx was observed, but uHpx excretion in group IIA was higher in relapse (p < 0.026) and lower in remission (p < 0.0017) as compared to group IIB. The results suggest the role of Hpx in the pathogenesis of INS. Hpx may be a useful indicator for continuation of treatment, but it requires confirmation by further controlled studies

The Role of TNF-α in the Pathogenesis of Idiopathic Nephrotic Syndrome and Its Usefulness as a Marker of the Disease Course

Background: The pathogenesis of idiopathic nephrotic syndrome (INS) has not been fully explained. Among the likely factors, tumor necrosis factor - alpha (TNF-α) is considered. We aimed to evaluate the TNF-α (sTNF-α, uTNF-α) levels in the serum and urine of INS children, with the aim of determining its association with proteinuria, and of determining its usefulness as a marker of the disease severity. Methods: Fifty-one examined patients were divided into subgroups depending on the number of relapses as follows: group IA—first episode; group IB—more than two relapses, and according to treatment modality; group IIA—glucocorticosteroids (GS) alone; and group IIB—GS with immunosuppressants. Healthy age-matched children served as the control group. Results: sTNF-α and uTNF-α levels were significantly increased in active phases in the whole INS group compared to the control group. They decreased in remission, but remained significantly higher when compared to the control group. During remission in the IB group, sTNF-α levels were significantly higher than in IA, whereas, in the relapse phase, these values were similar. In the IA group, a positive correlation between proteinuria and sTNF-α was demonstrated. Conclusions: Our findings suggest that TNF-α plays a role in the development of INS, and may be used as a prognostic marker, as well as an indicator for the continuation of therapy. Additional research is required to verify this statement

The role of IL-13 in the pathogenesis of idiopathic nephrotic syndrome (INS) in children

Background. INS pathogenesis has been still unclear. Among many factors Th2-derived IL -13 cytokine is taken into account. Objectives. Assessment of serum and urine level of IL -13 in children with INS as a pathogenic factor and a marker of the course of the disease. Material and methods. 51 children with INS and 18 healthy children were examined. Patients were divided into groups depending on the number of relapses of the disease, medical treatment and morphological types. The serum and urinary concentration of IL -13 were determined by ELIS A. Results . The significant increase in IL -13 in serum and urine of patients in the acute phase of the disease, compared to healthy subjects, was shown. In remission its level was significantly reduced, but still remained higher than in the control group. Regardless of the stage of the disease, the concentration of IL -13 in the urine of patients with the first relapse of INS was significantly lower than in children with recurrent INS or requiring immunosuppressive therapy. Serum level of IL -13 was not differen between these groups. In all histopathological types of INS , IL -13 in serum and urine were similar. Conclusions . Increased concentration of IL -13 in serum and urine of children with INS in the acute phase of the disease, and significant decrease in remission, indicate its role in the pathogenesis. Higher levels of IL -13 in the urine of patients with recurrent INS and requiring immunosuppressive therapy suggest the usefulness of this parameter as a prognostic marker. IL -13 in serum and urine does not allow one to distinguish between the morphological types of INS

Pelvicalyceal system duplication with ectopic ureter – diagnostic difficulties associated with the imaging procedure. Two cases report

Urinary tract abnormalities are the most frequently occurring developmental anomaly in children. Pelvicalyceal system duplication is one of them and signifies the existence of two urine diverting separate systems. This anomaly occurs in 10% of population, usually in girls, and is associated with complete or partial ureter duplication. The frequency of total ureter duplication, which in 20–40% of patients is found as bilateral, is 1:125 children (0.8% of the population). The most frequent malformation is asymptomatic, diagnosed coincidentally casually and does not require any treatment. In some patients with pelvicalyceal system duplication, vesicoureteral reflux (VUR) and ectopic ureter may coexist. Malposition of ureter’s orifice into the bladder predispose to urinary retention, development of hydronephrosis and urinary tract infection (UTI). Ectopic ureterocele is recognized in 6–20% of children with recurrent UTI. The reason why children are referred to the hospital is UTI or hydronephrosis revealed by ultrasound imaging. When the ultrasound image of pelvicalyceal system duplication is ambiguous, micturating cystourethrography (MCU) and scintigraphy become the primary diagnostic procedure, or urography – in more complicated cases. In the case of ectopic ureter, the danger of inappropriate catheterization, i.e. directly into its lumen, may occur. Though very rare, this may cause some diagnostic difficulties and lead to injury of the urinary tract. For this reason, we want to further discuss this complication following a diagnostic procedure on the example of two cases of pelvicalyceal system duplication with ectopic ureter

Spontaneous Rupture of Kidney Due to Posterior Urethral Valve– Diagnostic Difficulties

Background: Spontaneous kidney rupture could develop in the course of posterior urethral valve (PUV), the most common cause of outflow urinary tract obstruction in male infants. However, urinary extravasation is a rare complication among this group of children. Case Presentation: Our case report presents diagnostic difficulties connected with spontaneous kidney rupture due to PUV in a 6 week-old infant. Due to not equivocal images, thundery course of disease and rapid deterioration in the infant`s condition, the patient required an urgent laparatomy. Conclusion: This case showed that the investigation of renal abnormalities during early neonatal period, is very important specifically in PUV that can lead to kidney rupture

The role of complement component C3 activation in the clinical presentation and prognosis of IgA nephropathy - a national study in children

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children