Anna Medyńska

Serum Concentration of IL-2, IL-6, TNF-Alpha and Their Soluble Receptors in Children on Maintenance Hemodialysis

In chronic renal failure patients a state of immunodeficiency paradoxically coexists with the activation of immune effector cells, including monocytes and lymphocytes. The activation of these cells leads to the release of cytokines. The aim of this study was to estimate the serum concentrations of IL-2, IL-6, TNF-α and their soluble receptors: IL-2 sRα, IL-6 sR, sTNF RI in children with chronic renal failure and young adults on maintenance hemodialysis (HD). The study included 16 HD patients (11 females, 5 males) aged 11–22 (mean 16.1 ± 3.1) years and a control group of 15 age-matched healthy children. Only the mean concentration of IL-6 was similar in HD patients and the control group. The levels of the other cytokines were significantly higher in patients undergoing HD compared to the healthy subjects. No significant differences were observed between the pre- and post-dialysis values or between the values obtained using various dialyzer membranes. These data suggest that immune cells in HD children are in an activated state and that neither a single dialysis session nor the type of dialyzer membrane has an influence on the cytokines examined.

Interleukin-18 in Urine and Serum of Children with Idiopathic Nephrotic Syndrome

Background/Aims: Interleukin (IL)-18, a member of the IL-1 cytokine superfamily, is recognized as an important regulator of immune responses. The aim of our study was to investigate the IL-18 levels in serum and urine from children with idiopathic nephrotic syndrome (INS) during relapse and remission, and to evaluate the role of IL-18 in this disease. Methods: 67 children with INS, aged 3–16 years, and 15 normal controls were included in the study. The patients were divided into two groups according to activity of the disease: I (n = 37) – INS in relapse, II (n = 30) – INS in remission. Serum and urinary IL-18 were determined by ELISA and in urine related to the urinary creatinine (Cr) concentration. Serum creatinine, protein, albumin and 24-hour proteinuria were measured in children with INS. Results: Urinary IL-18 concentration was significantly higher in group I (213.51 ± 162.15 pg/mg Cr) compared to group II (64.74 ± 10.95 pg/mg Cr) and to normal controls (37.03 ± 4.1 pg/mg Cr, p < 0.001). Serum IL-18 concentration was significantly higher in group I than in the controls (146.4 ± 30.2 and 113 ± 10 pg/ml, respectively; p < 0.05); the differences between either groups I and II or group II and controls were not significant. Urinary IL-18 correlated positively with serum IL-18 and with urinary protein excretion, but no correlations were found with other laboratory data. Conclusion: Increased serum and urine IL-18 levels were observed during relapse of INS. These findings indicate the association between the active phase of INS and the levels of IL-18 and can suggest the role of this cytokine in the INS development. The changes in urinary IL-18 excretion in the course of INS are connected with the disease activity.

Lymphoepithelioma-like thymic carcinoma in a 16-year-old boy with nephrotic syndrome—a case report

Nephrotic syndrome can occur as a consequence of, among others, malignancy. In this report we describe a 16-year-old boy with secondary nephrotic syndrome associated with lymphoepithelioma-like thymic carcinoma, an extremely rare subtype of thymic carcinoma with poor prognosis.

Multicenter analysis of the efficacy and safety of a non-standard immunosuppressive therapy with rituximab in children with steroid-resistant nephrotic syndrome

The aim of the study was a multicenter analysis of the efficacy and safety of a non‐standard immunosuppressive therapy with rituximab (Rtx) in children with steroid‐resistant nephrotic syndrome (SRNS) with particular emphasis on the possibility of permanent discontinuation or dose reduction of other immunosuppressive drugs such as glucocorticoids and cyclosporine A after 6 months of observation. The study group consisted of 30 children with idiopathic nephrotic syndrome, who were unresponsive to standard immunosuppressive treatment, and hospitalized in the years 2010–2017 in eight paediatric nephrology centres in Poland. The children were administered a single initial infusion of rituximab at the dose of 375 mg/m2 of the body surface area. Proteinuria, the daily supply of glucocorticoids, and cyclosporine were assessed at the moment of the start of the treatment and after 6 months since its commencement. Before Rtx therapy, complete remission was found in 13 patients (43%) and partial remission was found in 8 patients (26%). These numbers increased to 16 (53%) and 12 (40%), respectively. At the start of the treatment 23 patients (76.6%) were treated with cyclosporine A. After 6 months, this number decreased to 15 patients (35%). At the start of the treatment, 18 patients (60%) were treated with prednisone. After 6 months, this number decreased to 8 patients (44%). Children with SRNS may potentially benefit from Rtx treatment despite relative risk of side effects. The benefits may include reduction of proteinuria or reduction of other immunosuppressants.

What has changed in the prevalence of hypertension in dialyzed children during the last decade

Abstract 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.

End-stage renal disease in the course of urinary tract defects in Wolf–Hirschhorn syndrome – case report

Katarzyna Jungiewicz, Irena Makulska, Anna Medyńska, Danuta Zwolińska Received: 12.09.2014 Accepted: 26.09.2014 Published: 31.10.2014

Rituximab w leczeniu wielolekoopornego zespołu nerczycowego – opis przypadku

Streszczenie Wstep Wielolekooporne zespoly nerczycowe (ZN) wystepują u dzieci stosunkowo rzadko, stanowią one jednak trudny problem terapeutyczny. W ciągu ostatnich 5 lat pojawily sie doniesienia o skuteczności leczenia rituximabem (przeciwcialo monoklonalne ludzko-mysie anty-CD20) ZN, opornych na dotychczasową terapie. Cel pracy Celem pracy jest opis wykorzystania rituximbu w terapii wielolekoopornego zespolu nerczycowego u pacjentki, u ktorej mimo zastosowania standardowej terapii, a nastepnie pulsow metyloprednizolonu, lekow alkilujących, cyklosporyny, tacrolimusa, mykofenolanu mofetilu nie uzyskano remisji choroby. Zdecydowano o wykonaniu 5 zabiegow plazmaferez i podaniu rituximabu (4 wlewy dozylne 375 mg/m 2 /dawke). Uzyskano poprawe i kilkumiesieczną stabilizacje stanu klinicznego. W nastepnym okresie, niestety, doszlo do rozwoju schylkowej niewydolności nerek. Wyniki Rituximab wydaje sie byc bezpiecznym lekiem, nie zawsze pozwalającym na uzyskanie remisji zespolu nerczycowego. Ustalenie efektywności i wskazan do jego podawania wymaga dalszych badan.

Wydalanie alfa1-mikroglobuliny w moczu dzieci z idiopatycznym zespołem nerczycowym a postęp przewlekłej choroby nerek

Streszczenie Wstep W pierwotnych glomerulopatiach zmiany chorobowe mogą obejmowac nie tylko klebuszki, ale takze cewki nerkowe i tkanke środmiązszową, prowadząc do progresji przewleklej choroby nerek (PChN). Cel pracy Analiza wydalania z moczem bialka drobnocząsteczkowego alfa1- mikroglobuliny (alfa1M) u dzieci z idiopatycznym zespolem nerczycowym (IZN) w odniesieniu do oceny funkcji nerek w oparciu o stezenie cystatyny C w surowicy. Material i metody Badaniami objeto 80 dzieci z IZN w wieku 2–18 lat oraz 20 dzieci zdrowych. U wszystkich chorych, ktorych obserwowano przez okres 22–24 miesiecy, stwierdzono 1. stadium PChN. Podzielono ich na dwie grupy, zaleznie od progresji PChN na podstawie stezenia cystatyny C w surowicy: grupa I (n=37) – chorzy, u ktorych filtracja klebuszkowa nie ulegla zmianie w okresie obserwacji, grupa II (n=43) – dzieci, u ktorych doszlo do pogorszenia funkcji nerek. Wydalanie alfa1M w moczu oznaczono immunoenzymatycznie i wyrazono w g/g kreatyniny. Wyniki Wydalanie alfa1M w moczu dzieci z IZN bylo znamiennie wyzsze niz u osob zdrowych, natomiast nie roznilo sie istotnie pomiedzy wyodrebnionymi grupami chorych. Wnioski Zwiekszone wydalanie alfa1M w moczu dzieci z IZN w okresie remisji wskazuje na utrzymujące sie zmiany cewkowo-środmiązszowe w przebiegu glomerulopatii, lezących u podloza zespolu nerczycowego. Wydalanie alfa1M z moczem nie jest czulym wskaźnikiem upośledzenia funkcji nerek u dzieci z IZN.