Jolanta Sołtysiak

Efficacy and safety of rituximab treatment in children with primary glomerulonephritis.

BACKGROUND The aim of our study was to analyze the efficacy and safety of rituximab, a chimeric monoclonal antibody against CD20 lymphocytes, as a nonstandard immunosuppressive therapy in children with different types of primary glomerulonephritis who were not eligible for routine treatment. METHODS The study group was composed of 16 children with proteinuric glomerulopathies, not responding to standard immunosuppressive therapy. The indications included steroid-resistant nephrotic syndrome (n=14) and steroid-dependent nephrotic syndrome (n=2). The dose of rituximab was established as 375 mg/m2 of body surface area, administered by intravenous infusion once weekly for 1 to 4 weeks, depending on the CD19 lymphocyte count. We evaluated proteinuria and plasma concentration of CD19 lymphocytes at intervals of 1, 3 and 6 months, after which patients received a single repeat dose. RESULTS Remission, defined as proteinuria less than 150 mg per 24 hours, was observed in 7 of the 16 children. There were no statistically significant differences in leukocyte counts between single and multiple rituximab doses. We also did not observe any clinical or biochemical side effects. CONCLUSIONS In conclusion, we postulate that alternative rituximab therapy should be taken into consideration in nephrotic patients not responding to standard therapy.

Hypertensive nephropathy in children – do we diagnose early enough?

Background/Aims. The aim was to evaluate the level of neutrophil gelatinase-associated lipocalin (NGAL), interleukin 18 (IL-18) and retinol binding protein (RBP) in children with primary hypertension and no features of hypertensive nephropathy. Methods. The study group consisted of 19 children (15 males) aged 14.8 ± 2.18 years with primary hypertension. Estimated glomerular filtration rate (eGFR) and albumin/creatinine ratio (ACR) were within the normal range. Mean blood pressure (BP) was 141/79 mmHg (mean systolic BP percentile was 98, mean diastolic BP percentile was 80). Ambulatory BP measurement (ABPM), blood and urine biochemical measurements and features of end organ damage were assessed. The control group consisted of 20 healthy children. Results. Hypertensive children showed significantly increased serum and urine NGAL concentration vs controls. Urine RBP was significantly higher in the study group vs controls. A positive correlation was found between urine NGAL and the index of mean systolic BP measured in ABPM, between urine IL-18 and the index of office diastolic BP, between serum NGAL and ACR, and between urine NGAL concentration and serum HDL. Conclusion. In children with primary hypertension, increased serum and urine NGAL may reflect kidney injury earlier than typical markers of hypertensive nephropathy.

Neutrophil gelatinase-associated lipocalin and Cathepsin L as early predictors of kidney dysfunction in children with type 1 diabetes

INTRODUCTION The aim of this study was to evaluate serum levels and urinary excretion of neutrophil-gelatinase associated lipocalin (respectively sNGAL and uNGAL) and urinary excretion of Cathepsin L (uCathL) in children with type 1 diabetes mellitus (DM1) who presented normoalbuminuria and the estimated glomerular filtration rate (eGFR) above 90 mL/min/1.73 m2. MATERIAL AND METHODS The study group consisted of 63 children with a diabetes duration of 5.16 ± 3.39 years. The degree of albuminuria was based on urine albumin-to-creatinine ratio (ACR), while eGFR was based on serum cystatin C. Glomerular hyperfiltration (GH) was defined as an eGFR value above 135 mL/min/1.73 m2. RESULTS Children with DM1 showed significantly higher concentrations of uNGAL, and lower sNGAL and uCathL. Significant changes of uNGAL and uCathL levels were even found in children without GH and with optimal glycaemic control (HbA1c < 7.5%). Positive correlations between uNGAL, ACR and eGFR were shown, as well as between uCathL and eGFR. CONCLUSIONS Significant changes in the concentration of markers of early kidney injury: sNGAL, uNGAL, and uCathL, can occur in children with DM1 and normoalbuminuria. The changes of uNGAL and uCathL can be even found in children without GH and with optimal glycaemic control. The earliest signs of diabetic kidney dysfunction seem to result from tubular damage.

Efficacy of plasma exchange in septic shock: a case report

The mortality rate for severe sepsis and septic shock remains high. Additionally, this life-threatening state poses serious difficulties for the treatment of patients. Unfortunately, the mechanism of sepsis is complex and not well understood. In this paper, we present the case of a 2.5-year-old female with septic shock treated with plasma exchange (PE) as a nonstandard therapy. We analysed the medical history of disease, including patient data, physical examination, laboratory tests and treatment. Unexpectedly, we achieved clinical improvement after the first PE. During PE, the dose of catecholamine was reduced. In addition, the level of C-reactive protein seemed to be a better predictor of the efficacy of PE in septic shock compared to procalcitonin. We conclude that PE may improve the survival rate for patients with septic shock. These data could be useful in the search and introduction of new or alternative methods of treatment for critically ill children.

Atypical thymic carcinoid manifesting with nephrotic-range proteinuria in a 7-year-old boy

BackgroundNephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria.Case-Diagnosis/TreatmentWe present the case of a 7-year-old boy with an extremely rare atypical thymic carcinoid accompanied by nephrotic-range proteinuria as PNS. The kidney biopsy was consistent with minimal change disease (MCD). Tests for a neuroendocrine tumor were performed due to symptoms of hypercortisolemia and an elevated concentration of chromogranin A in the serum. The chest computed tomography revealed a tumor in the anterior mediastinum, which was diagnosed as an atypical thymic carcinoid. A complete resolution of the nephrotic-range proteinuria was observed within 1 week after the first thoracoscopic surgery, with almost complete reduction of the tumor mass.ConclusionsThis extremely rare case shows that MCD can occur as a PNS even in children. Nephrotic-range proteinuria can be a symptom of malignant solid tumor. This case highlights the possibility of secondary causes of MCD in children.

Erratum to: Normal-range albuminuria does not exclude nephropathy in diabetic children

Erratum to: Pediatr NephrolDOI 10.1007/s00467-010-1443-zIt has come to our attention that the reported NGAL levelsare 10-fold higher than those reported by other researchers.This is best seen from the normal values reported inTable 1. Inaccurate data is due to a decimal mistake. Allreported serum and urine values for NGAL have to bedivided by 10.

Ocena przebiegu oraz skuteczności stosowanego leczenia toczniowego zapalenia nerek u dzieci

Streszczenie Toczen rumieniowaty ukladowy (SLE) jest autoimmunologiczną chorobą tkanki lącznej o szerokim zakresie objawow klinicznych. U dzieci choroba wystepuje znacznie rzadziej niz u doroslych, ale az u 70–80% z nich wystepuje toczniowe zapalenie nerek (NT), ktore jest jednym z najpowazniejszych czynnikow rokowniczych SLE. W celu indukcji, a nastepnie podtrzymania remisji choroby stosowane jest intensywne leczenie immunosupresyjne obarczone mozliwością wystąpienia powaznych skutkow ubocznych. Cel pracy Celem pracy byla retrospektywna analiza przebiegu i wynikow leczenia toczniowego zapalenia nerek u dzieci. Material i metody Grupe badaną stanowilo 10 dzieci hospitalizowanych w Klinice Nefrologii Dzieciecej UM w Poznaniu. Analizowano objawy kliniczne, parametry laboratoryjne oraz wyniki leczenia immunosupresyjnego w okresie 24 miesiecy. Wyniki Średnia wieku w chwili zachorowania wynosila 14 lat i 3 miesiące. Wśrod pacjentow przewazaly dziewczynki. U 7 dzieci nefropatia toczniowa wystąpila w postaci zespolu nerczycowego, a u 6 jako ostra niewydolnośc nerek. U 7 pacjentow stwierdzono IV klase NT wg klasyfikacji WHO. W leczeniu stosowano steroidy (GK), cyklofosfamid (CF), cyklosporyne (CsA), plazmaferezy oraz u 3/10 mykofenolan mofetylu (MMF). U 2 chorych stosowano CsA w indukcji remisji. Wszyscy pacjenci uzyskali remisje kliniczną i biochemiczną w okresie indukcji. U 3 pacjentow leczonych MMF remisja wystąpila w pierwszych trzech miesiącach leczenia. Wnioski NT u dzieci przebiega z duzą aktywnością kliniczną oraz postepującą glomerulopatią, ktora wymaga intensywnego leczenia immunosupresyjnego. Zastosowanie MMF w okresie indukcji remisji moze przyspieszyc osiągniecie remisji oraz prowadzic do ograniczenia liczby pulsow cyklofosfamidowych i wydluzenia okresu remisji.