Disparities in treatment and outcome of kidney replacement therapy in children with comorbidities: an ESPN/ERA Registry study.

Background: Data on comorbidities in children on kidney replacement therapy (KRT) are scarce. Considering their high relevance for prognosis and treatment, this study aims to analyse the prevalence and implications of comorbidities in European children on KRT.

Methods: We included data from patients <20 years of age when commencing KRT from 2007 to 2017 from 22 European countries within the European Society of Paediatric Nephrology/European Renal Association Registry.

Therapeutic Plasma Exchange in Pediatric Renal Transplantation Experience of One Decade and 389 Sessions.

Objective: There are no specific recommendations for therapeutic plasma exchange (TPE) in children after renal transplantation. The purpose of this study was to report the experience with TPE in a pediatric transplant setting.

Materials And Methods: 59 patients (mean age 12.

Eltrombopag (thrombopoietin-receptor agonist) and plasmapheresis as rescue therapy of acute post-renal transplant immune thrombocytopenia in a child with Schimke immuno-osseous dysplasia-case report.

SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T-cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11-year-old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL.

Rituximab is not a "magic drug" in post-transplant recurrence of nephrotic syndrome.

Unlabelled: Pediatric patients with end-stage renal failure due to severe drug-resistant nephrotic syndrome are at risk of rapid recurrence after renal transplantation. Treatment options include plasmapheresis, high-dose of cyclosporine A/methylprednisolone and more recently-rituximab (anti-B CD20 monoclonal depleting antibody). We report five patients with immediate (1-2 days) post-transplant recurrence of nephrotic syndrome, treated with this kind of combined therapy including 2-4 weekly doses of 375 mg/m(2) of rituximab.

Favorable four-yr outcome after renal transplantation in a patient with complement factor H antibody and CFHR1/CFHR3 gene mutation-associated HUS.

Unlabelled: aHUS is a clinical challenge for successful renal transplantation.

Case Report: A 14-yr-old girl lost her kidneys at the age of 7, due to CFH antibodies and CFH-related protein (CFHR1/CFHR3) homozygous deletion-associated aHUS. CFH, CFI, and MCP gene mutations were excluded.

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.

Liver transplantation with ABO incompatible graft under immunoadsorption protocol--case report.

Background: ABO incompatible liver transplantation is still controversial, but accepted in selected cases. Recently several authors reported use of the new technology aimed at elimination anti-donor ABO specific hemagglutinins to assist immunosuppression in preventing acute rejection after transplantation.

Case Report: We report two cases of liver transplantation in children with ABO incompatible graft under immunoadsorption protocol.

Evaluation of the genetic background of standard-immunosuppressant-related toxicity in a cohort of 200 paediatric renal allograft recipients--a retrospective study.

Background: Immunosuppressant toxicity is a limiting factor for the efficacy and safety of long-term therapy. Whether it stems solely from drug exposure, remains unclear.

Material/methods: Overall, 207 children and adolescents at the mean age of 11+/-4.

Multicentre prospective randomised trial of tacrolimus, azathioprine and prednisolone with or without basiliximab: two-year follow-up data.

A total of 192 children and adolescents undergoing renal transplantation were randomly chosen to receive tacrolimus, azathioprine and corticosteroids (TAS, n = 93) or tacrolimus, azathioprine, corticosteroids and two doses of basiliximab (TAS + B, n = 99). Six-month outcome data have previously been reported; this manuscript reports the 2-year data. Complete 2-year data were available on 164 (85.

Liver transplantation for fulminant Wilson's disease in children.

Background: Fulminant Wilson's disease (FWD) is rare and fatal condition in children unless liver transplantation is performed, however introduction of new technologies could change this poor prognosis. The aim of our study was retrospective analysis of clinical course, treatment and outcome of children with FWD treated in our institution.

Material/methods: Between 1999-2007 we've treated in our hospital 13 patients with mean age of 15.

Clinical course of homozygous familial hypercholesterolemia during childhood: report on 4 unrelated patients with homozygous or compound heterozygous mutations in the LDLR gene.

Natural history of the disease in 4 unrelated Polish children with homozygous familial hypercholesterolemia (FH) is described. Their phenotypic homozygosity was established by identification of known LDLR gene mutations on both alleles, respectively: p.G592E & p.

[Evaluation of pharmacokinetics and safety of valganciclovir oral suspension treatment in pediatric kidney graft recipients--preliminary report].

Background: Invasive CMV disease in transplanted organ recipients is a life threatening condition. CMV infection is also known risk factor for acute rejection and chronic allograft nephropathy. The risk of CMV infection in pediatric population is high, especially in terms of primary infection.

[Continuous renal replacement therapy in children with acute renal failure--5 years of experience].

Continuous renal replacement therapy (CRRT) has became a modality of choice in chidren with acute renal failure (ARF), especially in cases of multiorgan failure (MOF) and in hemodynamically unstable patients in whom regular hemodialysis is difficult to reform. Newborns and infants with contraindications to peritoneal dialysis are another group of patients treated with CRRT. Retrospective analysis of CRRT therapy in 112 patients treated with (CVVHD, CVVH, CVVHDF, SCUF-continuous veno-venous hemodialysis/hemofiltration/ hemodiafiltration/ultrafiltration) between 2000-2005 is presented.

Successful treatment of a child with fulminant liver failure and coma caused by Amanita phalloides intoxication with albumin dialysis without liver transplantation.

FLF is a life-threatening disease. Hepatic coma exerts dramatic impact on patient survival. At present, LTx is the treatment modality of choice that provides significant improvement in outcome of most patients with FLF.

[Atypical osteitis in hemodialysed child with uncontrolled secondary hyperparathyroidism: a case report].

Ostitis in the course of secondary hyperparathyroidism can lead to heavy atypical skeletal changes. In 4-year old girl on hemodialysis, with hyperparathyroidism (iPTH level > 1500 pg/ml), despite specific pharmacological treatment, very pronounced skull and maxillary bone malformations developed within two months, which were the cause of problems with breathing. We observed also growing bones malformations concerning arms, legs, vertebral column and pelvis.

[Clinical aspects of plasmapheresis therapy in children: single center experience].

Unlabelled: Data concerning 576 plasmapheresis sessions (indications, anticoagulation applied, supplement type and acute complications) in children (mean body weight = 35 kg +/- 15; min 5 kg, max 75 kg) performed between 1990 and 2001 were analysed.

Indications: Glomerulonephritis (GN)--185 (32%) (including recurrence after kidney transplantation--108, rapidly progressive GN--63, other GN--14), other immunological diseases--110 (19%) (systemic lupus erythematosus, Wegener's granulomatosis, myasthenia, Guillain-Barré syndrome, other), haemolytic-uraemic syndrome--104 (18%) (after kidney transplantation--50, atypical--54), Amanita poisoning 100 (17%), acute hepatic encephalopathy--41 (7%) (after liver transplantation--9), poisoning with drugs bound by plasma albumin--22 (4%) and complications in kidney graft recipients--14 (2%) (acute vascular rejection, parathormone toxicity).

Anticoagulation: Until the end of 1999--unfractionated heparin (in divided doses every 30 min--100 IU/kg/session on the average), from 2000 on--single dose of Fraxiparine (mean 70 IU anty-Xa/kg/session).

MARS procedure as a bridge to combined liver-kidney transplantation in severe chromium-copper acute intoxication: a paediatric case report.

We describe a case of multi-organ failure (liver-kidney insufficiency and brain oedema) caused by accidental, acute intoxication with a chromium and copper-containing substance, as an example of the introduction of the new extracorporeal procedure MARS (molecular adsorbents recirculating system) in a girl 3.5 years old.

Patient survival and causes of death on hemodialysis and peritoneal dialysis--single-center study.

Survival and causes of death in children dialyzed in a single center were analyzed. During the last 12 years a chronic dialysis program was introduced in 146 children in our center and 125 of them, eligible for observation, were included in this analysis; 58 patients were on hemodialysis (HD) and 67 on peritoneal dialysis [continuous ambulatory peritoneal dialysis/automated peritoneal dialysis (CAPD/APD)]. Mean age at the start of dialysis was 13.

[Different effect of angiotensin convertase inhibitors and AT1 receptor blockers on renal function in patients with chronic renal failure: report of two cases].

In two patients with chronic renal failure (CRF) different effects of angiotensin convertase inhibitors (ACEI) and angiotensin receptor 1 blockers (AT1B) were observed. In patient 1 with CRF due to hemolytic-uremic syndrome (HUS), with arterial hypertension (HT) and proteinuria, a switch from AT1B to ACEI led to significant deterioration of renal function. After restitution of AT1B, renal function returned to previous range.

[Analysis of causes of death in children and adolescents on chronic peritoneal dialysis and hemodialysis in 10 year period: single center experience].

Causes of death in children and adolescents treated with chronic peritoneal dialysis or hemodialysis in 1990-1999 in single centre were analysed. Overall 131 patients were treated, including 55 on peritoneal dialysis (PD) and 76 on hemodialysis (HD). Overall mortality in a 10-year period was 12% (16 patients).

[Critical evaluation of preparing pediatric recipients for renal transplantation: central waiting list analysis].

Transplantation is optimal modality of the renal replacement therapy (RRT), particularly in children. The number of transplants and improvement of tissue-typing may depend not only on overall number of procured organs, but also on the number of properly prepared recipients. The cases of pediatric recipients on central waiting list were analyzed.