Beata Leszczyńska

Perception of health-related quality of life in children with chronic kidney disease by the patients and their caregivers: Multicentre national study results

ObjectiveThe aim of the study was to analyse the health-related quality of life (HRQoL) in Polish children with chronic kidney disease (CKD) dependant on the CKD stage, treatment modality and selected social life elements in families of the patients. Furthermore, potential differences between self-report and parent/proxy reports and the factors influencing them were assessed.MethodsA total of 203 CKD children (on haemodialysis (HD), peritoneal dialysis (PD) and conservative treatment (CT)) and their 388 parent/proxies were enrolled into a cross-sectional national study. The demographic and social data were evaluated. We used the Paediatric Quality of Life Inventory 4.0 Generic Core Scales to assess the HRQoL in children.ResultsHealth-related quality of life scores for all CKD groups were significantly lower in all domains compared with population norms, the lowest one being in the HD group. In CT children, HRQoL did not depend on the CKD stage. Both parents assessed the HRQoL of their children differently depending on their involvement in the care. There are differences between the HRQoL scores of the children and their parents.ConclusionThe HRQoL in children with CKD is lower than in healthy children. This is already observed in the early stages of the disease. The disease itself influences the child’s mental state. Children on HD require special support on account of the lowest demonstrated overall HRQoL. Children’s lower rating of the quality of life observed by their parents may render the patients unmotivated and adversely affect their adjustment to life in later years. It may also create conflicts between the parents and the children.

Molecular epidemiology of a household outbreak of Shiga-toxin-producing Escherichia coli in Poland due to secondary transmission of STEC O104:H4 from Germany

We characterized two STEC O104 : H4 clinical isolates collected in Poland from a 7-year-old boy with haemolytic uraemic syndrome (HUS) and his nanny. This household outbreak began on 29 May 2011. Because of its time-frame, the outbreak was assumed to be part of the international STEC O104 : H4 outbreak that arose in Germany in May 2011. The two Polish isolates were Shiga-toxin-producing Escherichia coli (stx2 lpf) with enteroaggregative E. coli pathotype (aggR aap aggA), thereby sharing the unique virulence properties of the epidemic STEC O104 : H4 strain from the international outbreak. The Polish isolates were multi-drug resistant and carried bla(TEM), strA, strB, tetA, sul1 and sul2 markers together with the bla(CTX-M-15) gene for CTX-M-15 extended-spectrum β-lactamase. PFGE patterns and plasmid profiles of the Polish isolates and the epidemic STEC O104 : H4 strain corresponded closely. This finding suggested an epidemiological link between the Polish STEC O104 : H4 isolates and the international outbreak. Retrospective serological investigations proved person-to-person transmission of the epidemic STEC O104 : H4 strain from a father who had visited Dortmund, Germany, to his 7-year-old son in Giżycko, Poland. To the best of our knowledge, this is the first report of household transmission of Shiga-toxin-producing E. coli in Poland.

Diagnostic Value of Serological Tests Against Verotoxigenic Escherichia coli in Hemolytic Uremic Syndrome in Children

BACKGROUND Diarrhea-associated hemolytic uremic syndrome (HUS D+) caused by verotoxigenic E. coli strains (VTEC) is a major cause of acute kidney injury in children between 1 and 5 years of age. Because of the short presence of VTEC in the gastrointestinal tract as well as difficulties with the detection of the verotoxigenic strain, identification of HUS etiology might be challenging. OBJECTIVES The aim of the study was to assess the clinical and diagnostic value of serological tests for specific antibodies against verotoxigenic strains of E. coli in patients with HUS. MATERIAL AND METHODS Eight children aged 8 months - 7.1 years (mean 40 ± 29 months) with symptoms of acute kidney injury, hemolytic anemia and thrombocytopenia observed after hemorrhagic diarrhea were included to the study. VTEC presence was detected in a stool culture with subsequent analysis of the ability to produce verotoxin and the presence of VT1 and VT2 as well as intimin and enterohemolysin genes. In addition, the presence of specific IgA, IgM and IgG antibodies against E. coli serogroups O26, O103, O104, O111, O121, O145 and O157 was measured using ELISA. RESULTS In 3 subjects, VTEC O26, O157 and O104 serogroups were cultured in the stool and the specific IgA, IgM and IgG antibodies were detected. In 4 subjects, no VTEC strains were cultured, however, high titers of IgA, IgM and IgG antibodies against E. coli O26, O157 and O111 were detected. In a single patient, the negative results of bacteriological and serological analyses excluded VTEC etiology of HUS. CONCLUSIONS A serological analysis of VTEC can confirm the result of stool culture for verotoxigenic E. coli strains and help to find the cause of HUS in case of negative results of a stool culture.

Zespół hemolityczno-mocznicowy u dzieci w latach 1971–2010; doświadczenia jednego ośrodka dializ dla dzieci☆

Streszczenie Zespol hemolityczno-mocznicowy (ZHM) jest to mikroangiopatia zakrzepowa z charakterystyczną triadą objawow: niedokrwistością hemolityczną, maloplytkowością i ostrą niewydolnością nerek. U 90% dzieci ZHM rozwija sie najcześciej po infekcji jelitowej wywolanej przez E. coli produkującą werotoksyne. U pozostalych 10%, bez poprzedzającej biegunki, rozpoznaje sie atypowy ZHM. Cel Celem pracy byla ocena czestości wystepowania i przebiegu klinicznego ZHM u 140 dzieci hospitalizowanych w latach 1971–2010 w Katedrze i Klinice Pediatrii i Nefrologii WUM. Material i Metody Przeanalizowano 2 grupy chorych: hospitalizowanych w latach 1971–1992 (grupa I; n=94) i 1993–2010 (grupa II; n=46). Podzial uwzglednial zmiane sposobu terapii, gdyz od roku 1993 stosowano plazmaferezy w leczeniu atypowych i ciezko przebiegających ZHM. Wyniki Dzieci z grupy I byly istotnie mlodsze (1,43 ± 1,97 roku) niz z grupy II (4,48 ± 4,46 roku); p Wniosek Leczenie plazmaferezami i wlewami osocza poprawilo rokowanie u chorych i zmniejszylo koniecznośc stosowania leczenia nerkozastepczego u dzieci z ZHM.

Successes and pitfalls of chronic peritoneal dialysis in infants – a Polish nationwide outcome study

Introduction Peritoneal dialysis (PD) is a preferred method of renal replacement therapy for end-stage renal disease in children. Recent advances have allowed chronic PD to be provided to children of all ages and sizes. Material and methods The study was designed as a national (10 dialysis centres), multicentre retrospective analysis of the medical history of 33 children who started chronic peritoneal dialysis in their infancy between 1993 and 2005, with a follow-up period of at least 24 months. Results The nutritional status of the infants was unsatisfactory. The mean SDS of body weight at the start was –2.0, at 1 year of age –1.7. Only 40% of infants were adequately nourished at 1 year of age. Long-term follow-up analysis showed that 12 children received a kidney transplant, 13 were still on dialysis (4 changed method) and 6 died (mortality rate in the first year of life of 9%). In 2 children we observed an improvement of renal function. We observed a relatively high (1/8.8 patient-months) peritonitis rate in the analysed children when compared to 1 : 22 patient-months in all children undergoing PD in Poland. Conclusions The results of our survey have shown that the management of dialysed infants is still a challenge for the medical team and families, but long-term results of the therapy are encouraging.

Serum GDIgA1 levels in children with IgA nephropathy and Henoch-Schönlein nephritis

Introduction GDIgA1 (galactose deficient IgA1) plays a significant role in the pathogenesis of IgA nephropathy (IgAN) and Henoch-Schönlein nephritis (HSN). Aim of the study The aim of this study was to assess the relevance of serum GDIgA1 level as a prognostic marker in children with IgAN and HSN. Material and methods 41 children were included to the study group (15 IgAN, 26 HSN) and 22 to the control group. The following parameters were evaluated at baseline and endpoint: proteinuria, erythrocyturia, serum creatinine, serum IgA, GFR. A kidney biopsy was performed in all patients and evaluated according to the Oxford Classification (1 – present, 0 – absent: M – mesangial hypercellularity; E– endocapillary hypercellularity; S – segmental sclerosis/adhesion; T – tubular atrophy/interstitial fibrosis), and was calculated as the total score (sum of M, E, S, T). At the end of follow-up, the serum GDIgA1 concentration was measured. Results The serum GDIgA1 concentration in patients with IgAN and HSN was significantly higher than in the control group. No significant differences in mean proteinuria, erythrocyturia, GFR, MEST score, or GDIgA1 in serum, as well as the duration of follow-up between IgAN and HSN were observed. Baseline serum IgA concentration and time to kidney biopsy were significantly higher in children with IgAN than in children with HSN. We observed a positive correlation between GDIgA1 and IgA levels (r = 0.53), and GDIgA1 and serum creatinine levels (r = 0.5), as well as negative correlation between GDIgA1 and GFR (r = –0.37). Conclusions Serum GDIgA1 level may have a prognostic value in children with IgAN and HSN; however, to fully elucidate its clinical potential further studies performed in larger patient cohorts are required.

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.

Growth and nutritional status in children with chronic kidney disease on maintenance dialysis in Poland

PURPOSE Despite vast availability of modern methods of treatment of chronic kidney disease and its complications, the short stature still is a major point of concern in adolescents with chronic kidney disease. The aim of the study was to assess changes in growth and nutritional status of Polish children on renal replacement therapy in the decade, 2004-2013. MATERIAL AND METHODS The study was designed as a cross-sectional analysis of anthropometric values and selected indices of growth status amongst children receiving dialysis in Poland between the years 2004 and 2013. Data were acquired during two different multicentre studies on hypertension in dialyzed children in Poland. Basic anthropometric parameters (body weight, body height/length, body mass index - BMI), dialysis adequacy and duration of RRT were assessed. RESULTS The study showed that anthropometric parameters of children undergoing renal replacement therapy had not significantly changed in the last 10 years of observation. Children on RRT were still of short stature despite availability of modern methods of hormonal therapy and nutrition. Median of height z-score was -2.10 in 2004 and -2.19 in 2013. Expected clinical improvement in these measures was not proven. CONCLUSIONS The cause of chronic kidney disease, method of dialysis, time on dialysis or dialysis adequacy did not influence the anthropometric parameters significantly in dialyzed children in Poland.