Helena Ziółkowska

Determinants of the intima–media thickness in children and adolescents with chronic kidney disease

The aim of the study was to evaluate changes in the arterial wall in children with chronic kidney disease (CKD). We studied 60 patients: 32 with stages 2–4 CKD [chronic renal failure (CRF)], 28 with stage 5 CKD [end-stage renal disease (ESRD)], and 43 controls (C). The evaluated parameters included intima–media thickness (IMT) of the carotid arteries, bone mineral density (BMD), serum lipid levels, and parameters of the calcium–phosphorus metabolism. Patients were divided into two groups: group 1 with normal arteries, and group 2 with arterial changes. The highest serum fetuin A level was found in group 1 compared with groups 2 and C. A negative correlation between IMT and fetuin A level was found. In patients with ESRD, a positive correlation of IMT with phosphorus level and age and a negative correlation with cyclase-activating parathyroid hormone and cyclase inhibiting parathyroid hormone (CAP/CIP) ratio was observed. Multiple linear regression showed that lower fetuin-A and alkaline phosphatase (AP) levels and higher lumbar spine BMD independently predicted higher IMT. Arterial wall changes in children with CKD were related to lower fetuin A and AP level and higher BMD. Low CAP/CIP and high phosphorus level may also be significant factors for arterial changes in patients with ESRD.

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.

Anaemia treatment in chronically dialysed children: a multicentre nationwide observational study

Abstract Objective.Erythropoiesis-stimulating agents (ESAs) are applied as a standard therapy in children with anaemia in chronic kidney disease. The aim of this study was to describe the efficacy and details of ESA treatment in a population of dialysed children in Poland. Material and methods. The study had a prospective observational design and was performed in 12 dialysis centres. The study group comprised 117 dialysed children with a mean age at enrolment of 165.33 (97.18–196.45) months. Results.Dialysed children were treated mostly with epoietin beta and darbepoietin. The mean dose of ESA was 99 (68–147) U/kg/week with a significant difference between patients on peritoneal dialysis [83 (54–115)] and haemodialysis [134 (103–186)] (p < 0.0001). The mean haemoglobin of all the time-point tests during 6 months was 10.91 ± 1.18 g/dl. The efficacy of anaemia treatment was unsatisfactory in 52% of subjects. In multivariate analysis, initial haemoglobin level <10 g/l, any infection, younger age at first dialysis, malnutrition and inadequate ESA dosage remained significant predictors of anaemia. Conclusions.The study revealed that anaemia treatment in Polish children is unsatisfactory. Late commencement of the treatment, inadequate dosing, malnutrition and infections could constitute risk factors for therapy failure.

Do children with end-stage renal disease live shorter? Analysis of mortality on the basis of data from the Polish Registry of Renal Replacement Therapy in Children

PURPOSE The mortality of patients with end-stage renal disease (ESRD) is much higher than that of the general population. To date no data has been published on the mortality of children with ESRD in Poland. The aim of this study was to compare the risk of death for pediatric patients on renal replacement therapy (RRT) with that of the general pediatric population and to identify the risk factors of death. MATERIAL/METHODS Data of 779 children with ESRD registered in the Polish Registry of Children on RRT was analyzed. The relative risk of death was calculated as the ratio of the mortality rate in ESRD patients to the mortality rate in age-adjusted general population. RESULTS The mortality rate of children with ESRD was 74-fold higher than that of the age- and gender-adjusted general pediatric population (4.05 vs. 0.05/100 person-years). The highest mortality rate (4.53/100 patient-years) was found in the youngest age group. Younger age and duration of dialysis therapy were identified as mortality risk factors. The major causes of death in ESRD patients were infections and cardiovascular complications, whereas deaths in general child population were mainly due to accidents or congenital defects. CONCLUSIONS The mortality in Polish children with ESRD is 74-fold higher than that of the general pediatric population. Infections, followed by cardiovascular complications, constitute the main causes of mortality in children subjected to RRT. The risk of death is the highest among children who started RRT at a younger age and in those subjected to long-term dialysis treatment.

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.

Minimizing bone abnormalities in children with renal failure.

Renal osteodystrophy (ROD), a metabolic bone disease accompanying chronic renal failure (CRF), is a major clinical problem in pediatric nephrology. Growing and rapidly remodeling skeletal systems are particularly susceptible to the metabolic and endocrine disturbances in CRF. The pathogenesis of ROD is complex and multifactorial. Hypocalcemia, phosphate retention, and low levels of 1,25 dihydroxyvitamin D3 related to CRF result in disturbances of bone metabolism and ROD. Delayed diagnosis and treatment of bone lesions might result in severe disability.Based on microscopic findings, renal bone disease is classified into two main categories: high- and low-turnover bone disease. High-turnover bone disease is associated with moderate and severe hyperparathyroidism. Low-turnover bone disease includes osteomalacia and adynamic bone disease.The treatment of ROD involves controlling serum calcium and phosphate levels, and preventing parathyroid gland hyperplasia and extraskeletal calcifications. Serum calcium and phosphorus levels should be kept within the normal range. The calcium-phosphorus product has to be <5 mmol2/L2 (60 mg2/dL2). Parathyroid hormone (PTH) levels in children with CRF should be within the normal range, but in children with end-stage renal disease PTH levels should be two to three times the upper limit of the normal range. Drug treatment includes intestinal phosphate binding agents and active vitamin D metabolites. Phosphate binders should be administered with each meal. Calcium carbonate is the most widely used intestinal phosphate binder. In children with hypercalcemic episodes, sevelamer, a synthetic phosphate binder, should be introduced. In children with CRF, ergocalciferol (vitamin D2), colecalciferol (vitamin D3), and calcifediol (25-hydroxyvitamin D3) should be used as vitamin D analogs. In children undergoing dialysis, active vitamin D metabolites alfacalcidol (1α-hydroxyvitamin D3) and calcitriol (1,25 dihydroxyvitamin D3) are applied. In recent years, a number of new drugs have emerged that hold promise for a more effective treatment of bone lesions in CRF. This review describes the current approach to the diagnosis and treament of ROD.

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.

Antihypertensive treatment prescription in pediatric dialysis patients in Poland: A comparison between two nationwide studies 2003/2004–2013

BACKGROUND Blood pressure in pediatric dialyzed patients is under poor control. OBJECTIVES The aim of the study was to assess the strategy and efficacy of antihypertensive drugs used for the treatment of hypertension in pediatric dialyzed patients in 2013 in comparison with the data collected in 2003/2004. The results have been viewed against present strategies of antihypertensive treatment in children. There is still limited data concerning the treatment of hypertension in dialyzed pediatric patients. MATERIAL AND METHODS The study embraced 10 of 12 pediatric dialysis units in Poland treating 59 pediatric patients (mean age - 132 months). Collected information included present antihypertensive treatment with regard to drug classes and the dose of antihypertensive agent. The treatment was regarded as effective if both systolic and diastolic values of blood pressure were below 1.64 SDS. The results from 2013 were juxtaposed with previously analyzed data from a similar study on hypertension in dialyzed children conducted in 2003/2004. RESULTS Forty subjects have been provided with antihypertensive treatment. In monotherapy and polytherapy 50% of the subjects were treated with ACEI (enalapril and ramipril), 67.5% with amlodipine, 50% with beta-blockers. Only 10% of the subjects were treated with angiotensin II receptor blocker (losartan). Thirty percent of the subjects received furosemide, whereas 5% were given doxazosin. Antihypertensive drugs regarded as the 2nd and 3rd choice in treating high blood pressure (doxazosin, beta-blockers and furosemide) were applied as monotherapy in 46% of the patients. Satisfactory control of treated blood pressure was reached in 45% of them. CONCLUSIONS Antihypertensive treatment in dialyzed children did not change significantly during the last decade with regard to the groups of drugs being used. Despite a wider feasibility of antihypertensive substances, the effectiveness of this therapy was still unsatisfactory.

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.