[Show abstract][Hide abstract] ABSTRACT: Background:
Hemodialysis (HD) in children with a concomitant ventriculoperitoneal shunt (VPS) is rare. Registry data suggest that peritoneal dialysis with a VPS is safe, but little is known about HD in the presence of a VPS.
We performed a 10-year survey to determine the prevalence of a VPS, complications and outcome in children with a VPS on HD in 15 dialysis units from the 13 countries participating in the European Pediatric Dialysis Working Group.
Eleven cases of HD with a VPS were reported (prevalence 1.33 %; 328 patient-months) and compared with prospective Registry data. The median age at start of dialysis was 9.6 [inter-quartile range (IQR) 1.0-15.0] years and median HD vintage was 2.4 (IQR 1.7-3.0) years. Dialysis was performed through a central venous line (CVL) and through an arteriovenous fistula in six and five children, respectively. Three CVL infections occurred in two children, but these children did not develop VPS infections or meningitis. Symptoms of hemodynamic instability were reported in six (55 %) children at least once per week, with hypotension or hypertension occurring in four of these children and nausea, vomiting and headaches occurring in two; four other children reported less frequent symptoms. Seizures on dialysis occurred in two children, at a frequency of less than once per month, with one child also experiencing visual disturbances. During follow-up (median 4.0; IQR 0.38-7.63 years), three children remained on HD and eight had a functioning transplant. No patients were switched to PD.
Hemodialysis in children with a VPS is safe, but associated with frequent symptoms of hemodynamic instability. No episodes of VPS infection or meningitis were seen among the children in the survey, not even in those with CVL sepsis.
[Show abstract][Hide abstract] ABSTRACT: Pleural or pericardial effusions secondary to pleuro-peritoneal fistula (PPF) and pericardio-peritoneal fistula (PcPF) are rare but serious complications of peritoneal dialysis (PD).
We conducted a 10-year survey across all participating centres in the European Paediatric Dialysis Working Group to review the incidence, diagnostic techniques, therapeutic options and outcome of children on chronic PD with PPF and/or PcPF.
Of 1506 children on PD there were ten cases (8 of PPF, 1 each of PcPF and PPF + PcPF), with a prevalence of 0.66 %. The median age at presentation was 1.5 [inter-quartile range (IQR) 0.4-2.4] years, and nine children were <3 years. The time on PD before onset of symptoms was 4.3 (IQR 1.3-19.8) months. Eight children had herniae and seven had abdominal surgery in the preceding 4 weeks. Symptoms at presentation were respiratory distress, reduced ultrafiltration and tachycardia. PD was stopped in all children; three were managed conservatively and thoracocentesis was performed in seven (with pleurodesis in 3). PD was restarted in only three children, in two of them with success.
In conclusion, PPF and PcPF are rare in children on chronic PD, but are associated with significant morbidity, requiring a change of dialysis modality in all cases. Risk factors for PPF development include age of <3 years, herniae and recent abdominal surgery.
Full-text · Article · Jun 2015 · Pediatric Nephrology
[Show abstract][Hide abstract] ABSTRACT: Background
Hypertension (HT) is a common and serious complication following renal transplantation in children and it is an important risk factor for cardiovascular morbidity and mortality. This study evaluated the clinical characteristics of HT in children after renal transplantation.Methods
Twenty-four children who were followed-up at least six months after renal transplantation were enrolled the study. From the clinical records the demographic and laboratory data, casual blood pressure (BP) measurements, ambulatory blood pressure monitoring (ABPM), medications, left ventricular mass index (LVMI) at echocardiogram were documented.ResultsMean age of the patients at the time of transplantation was 12.6 ± 3.0 years and mean follow-up period was 19.6 ± 15.8 months. Hypertension was detected in 21 (87.5%) children after renal transplantation. Twelve (50%) patients had HT both before and after transplantation and nine (38%) had only after transplantation. Hypertension was developed in 67% within the first week and 95% within the first month. All hypertensive children had nighttime HT and no child had isolated daytime HT. The efficacy of hypertensive control was 42%. Median LVMI level of patients who had HT after renal transplantation was 42.3 g/m2.7.Conclusions
Severe HT which is an important complication was frequently seen in patients in early period after renal transplantation. Predominance of nocturnal HT and the lack of isolated daytime HT after transplantation were underlined the importance of ABPM. We suggest that ABPM should be performed regularly in the first year after transplantation not only for diagnosis but also evaluation of the control of HT.
No preview · Article · May 2015 · Pediatrics International
[Show abstract][Hide abstract] ABSTRACT: Colchicine is the standard treatment in familial Mediterranean fever (FMF) patients. New treatment strategies are needed in FMF patients who were unresponsive to colchicine therapy or who had developed amyloidosis. The aim of this study was to present clinical-laboratory features and treatment responses of pediatric FMF patients that were treated with anti-IL-1 therapies. Files of patients who had been followed in our department with diagnosis of FMF were retrospectively evaluated. Patients that have been receiving anti-IL-1 therapies (anakinra or canakinumab) were included to the study. All patients were interpreted with respect to the demographic data, clinical and laboratory features of the disease, genetic analysis of MEFV mutations and treatment responses. Among 330 currently registered FMF patients, 13 patients were included to the study. Seven of them received anti-IL-1 therapy due to colchicine resistance and 6 due to FMF-related amyloidosis (1 of them with nephrotic syndrome, 2 with chronic kidney disease, 3 with renal transplantation). In all treated patients, attacks completely disappeared or decreased in frequency; partial remission occured in nephrotic syndrome patient; and their life quality improved. Anti-IL-1 therapies can be successfully used in colchicine-resistant FMF patients and patients with amyloidosis during childhood and adolescent period without major side effects.
No preview · Article · Sep 2014 · Clinical Rheumatology
[Show abstract][Hide abstract] ABSTRACT: Systemic AA amyloidosis is a serious complication of many chronic inflammatory disorders and chronic infections. Renal involvement is seen in the majority of the patients and can lead to end-stage renal disease. Renal transplantation can be performed in these patients; however, amyloidosis can recur in the transplanted kidneys. On the other hand, de novo AA amyloidosis in renal transplant patients has been rarely reported. We report a 17-yr-old patient with end-stage renal disease due to genitourinary anomalies who developed recurrent pyelonephritis after transplantation. Three yr after transplantation, renal biopsy was performed for proteinuria and AA amyloidosis was identified in the renal allograft. Although rare, chronic infections might cause de novo amyloidosis in renal transplant patients. Therefore, amyloidosis should be kept in mind in those types of patients who present with proteinuria.
No preview · Article · Sep 2014 · Pediatric Transplantation
[Show abstract][Hide abstract] ABSTRACT: AimPrevious studies suggest that 6-46% of children suffer from lower urinary tract dysfunction (LUTD). This study evaluated the prevalence of LUTD in children with a urinary tract infection (UTI) and assessed the impact of standard urotherapy on patients with LUTD. Methods
We enrolled 228 patients who were 4years of age or older with at least one episode of UTI, together with a control group of 100 children. All the children were evaluated using the Pediatric Lower Urinary Tract Symptom Score (PLUTSS), and the intervention group were re-assessed after therapy to gauge their response. ResultsLower urinary tract dysfunction was detected in 134 (59%) patients. Their mean PLUTSS was 15.95.3, and 78% of these patients had a reduced quality of life (QOL). After 5 +/- 2.7months of behavioural therapy, 105 (78%) patients with LUTD were evaluated for the second time. This showed that LUTD was ameliorated in 69% of the patients and improved in 26%, with a mean post-treatment PLUTSS of 6.6 +/- 5.6. Two control group children had LUTD. Conclusion
Lower urinary tract dysfunction was frequently seen in patients with UTIs, but standard urotherapy was usually successful. Most of the patients in our study with LUTD also had an impaired QOL.
No preview · Article · Jun 2014 · Acta paediatrica (Oslo, Norway: 1992). Supplement
[Show abstract][Hide abstract] ABSTRACT: There is increasing focus on the problems involved in the transition and transfer of young adult patients from paediatric to adult renal units. This situation was addressed by the 2011 International Pediatric Nephrology Association/International Society of Nephrology (IPNA/ISN) Consensus Statement on transition.
We performed a survey of transition practices of 15 paediatric nephrology units across Europe 2 years after publication of the consensus statement.
Two thirds of units were aware of the guidelines, and one third had integrated them into their transition practice. Forty-seven per cent of units transfer five or fewer patients with chronic kidney disease (CKD) stage 5 per year to a median of five adult centres, with higher numbers of CKD stages 2-4 patients. Seventy-three per cent of units were required by the hospital or government to transfer patients by a certain age. Eighty per cent of units commenced transition planning after the patient turned 15 years of age and usually within 1-2 years of the compulsory transfer age. Forty-seven per cent of units used a transition or transfer clinic. Prominent barriers to effective transition were patient and parent attachment to the paediatric unit and difficulty in allowing the young person to perform self-care.
Whereas awareness of the consensus statement is suboptimal, it has had some impact on practice. Adult nephrologists receive transferred patients infrequently, and the process of transition is introduced too late by paediatricians. Government- and hospital-driven age-based transfer policies distract focus from the achievement of competencies in self care. Variable use of transition clinics, written patient information and support groups is probably due to economic and human-resource limitations. The consensus statement provides a standard for evolving and evaluating transition policies jointly agreed upon by paediatric and adult units.
[Show abstract][Hide abstract] ABSTRACT: The pathogenesis of Henoch-Schönlein Purpura (HSP) has not been clearly defined. Inflammatory cytokines have been associated with HSP but there are only a few reports that have focused on coagulation. The endothelial protein C receptor (EPCR), which has anticoagulant and antiinflammatory activity, is the key component of the protein C pathway. Recent studies have implicated the soluble form of EPCR (sEPCR) in Wegener's granulomatosis, Behçet's disease, and systemic lupus erythematosus. The aim of this study was to evaluate the levels of sEPCR in HSP children. Twenty-two children with HSP and 17 healthy children were included. We found no significant differences (P > .05) between patient and control groups in the levels of von Willebrand factor and thrombomodulin. The median sEPCR values in the HSP group were lower than the control group (79 vs. 102 ng/mL, respectively) (P > .05). The mean sEPCR value in HSP patients with severe abdominal pain was lower than without (88.8 ± 54.9 vs. 108.2 ± 66.3 ng/mL, respectively) (P > .05). In addition, the mean IL-6 serum levels were significantly elevated in HSP patients during the acute stage of HSP (2.1 ± 1.7 vs. 1.5 ± 1.2 pg/mL, P = .038). We also observed a slight negative correlation between the levels of sEPCR and IL-6 (R = -.135, P > .05). To our knowledge, this was the first study to analyze sEPCR levels in HSP. Our results did not conclusively identify a direct role of sEPCR in HSP, but our findings warrant further investigations, especially in severe HSP cases characterized by gastrointestinal bleeding or renal involvement.
No preview · Article · Dec 2013 · Pediatric Hematology and Oncology
[Show abstract][Hide abstract] ABSTRACT: Many factors may impact upon choice of renal replacement therapy (RRT) for children and adolescents, including patient and family choice, patient size and distance from the renal centre as well as logistic issues such as facilities and staffing at the unit. We report a survey of factors influencing treatment choice in 14 European paediatric nephrology units.
A questionnaire was developed by consensus and completed by 14 members of the European Paediatric Dialysis Working Group on facilities, staffing and family assessments impacting on choice of therapy as well as choice of therapy for 97 patients commencing initial RRT in 2011.
All units offered all modalities of RRT, but there were limitations for pre-emptive transplantation (PET) and largely adult surgical dependence for creation of arteriovenous fistulae and transplantation. The average waiting time for a deceased donor kidney was 18.5 (range 3-36) months. Full time dietetic support was available in six of the 14 units. There was no social worker, psychology, play therapy or teaching support in three, two, seven and four units, respectively. Assessment by other members of the multidisciplinary team and home visits before choice of therapy was carried out in 50 % of units, and although all patients were discussed at team meetings, the medical opinion predominated. In terms of types of RRT, 50 % of patients were commenced on chronic peritoneal dialysis (PD), 34 % on haemodialysis (HD) and 16 % underwent pre-emptive transplantation (PET). Chronic PD predominated in patients aged <5 years and HD predominated in those aged >10 years. Patient and family choice and age or size of patient were predominant factors in choice of therapy with a predictable decline in renal function favouring PET and social factors HD.
Chronic peritoneal dialysis predominated as primary choice of RRT, especially in younger children. The PET rates remain low. The influence of surgeons predominanted, and national transplant rules may be significant. Most units had insufficient multiprofessional support, which may impact upon initial choice of therapy as well as sustaining families through RRT.
Full-text · Article · Dec 2013 · Pediatric Nephrology
[Show abstract][Hide abstract] ABSTRACT: Renal transplantation is the treatment of choice for children with end-stage renal disease. The aim of this study was to evaluate retrospectively of our 37 pediatric renal allograft recipients, including 20 boys and 17 girls from July 2007 to August 2012. The overall mean age at transplantation was 12.16 ± 4.25 years. Three patients (8.1%) were transplanted preemptively; two were ABO-incompatible transplantations. The majority of recipients received living donor grafts (81%). The mean duration of follow-up was 25.10 ± 14.95 months. Seven acute rejection episodes were observed in 6 patients (16.2%). Eleven recipients developed serious viral infections: cytomegalovirus (n = 8), parvovirus (n = 2), BK virus (polyoma hominis 1) (n = 2), or Ebstein-Barr virus (n = 1). Three patients died; one from posttransplant lymphoproliferative disease, one from primary disease recurrence with infection, and one from sepsis. In conclusion, kidney transplantation is the treatment of choice for end-stage renal disease. Infection was the major concern after this procedure.
No preview · Article · Apr 2013 · Transplantation Proceedings