Ipek Kaplan Bulut

Success of continuous veno-venous hemodiafiltration treatment in children monitored in the intensive care units

Abstract Introduction: As intensive care units (ICU) have improved, presence of multiple-organ dysfunctions in majority of patients with acute renal failure (ARF) has become clearer. To facilitate multi-organ support, continuous renal replacement therapy (CRRT) techniques have been developed. This study is the one that reports the experience on children including newborns receiving CRRT monitored in ICU. Materials and Methods: The study was performed retrospectively in children who had Continuous Veno- Venous Hemodiafiltration (CVVHDF) as a CRRT modality in ICU. Clinical data, primary cause, consultation time, duration and initiation time of CVVHDF were recorded. Patients were classified as cardiac and non-cardiac in respect to primary dysfunction. Stage of renal failure was evaluated according to pRIFLE criteria. Outcome was identified as primary and secondary. Primary outcome was accepted as the composite correction of uremia and metabolic parameters, and regression of fluid overload, while secondary outcomes were assessed as improvement of hemodynamic instability and survival. Results: A total of 36 patients’ files were scanned. There were 10 cases in cardiac group and 26 cases in non-cardiac group. There were statistically better differences between primary and secondary outcome rates of cardiac cases. Although there was no difference between cardiac and non-cardiac cases in terms of primary outcome, secondary outcome was statistically significant. Timing of consultation and CVVHDF was not found to have an effect on the outcome. Conclusion: Our results indicated that CVVHDF treatment was successful even in cardiac patients with high mortality and in patients at their later stage of ARF.

Does NPHS1 polymorphism modulate P118l mutation in NPHS2

Nephrotic syndrome (NS) in the first year of life is uncommon and makes up a heterogeneous group of disorders. Subsequent studies have further defined the phenotype associated with mutations in the NPHS2 gene, revealing that patients usually develop NS from birth to 6 years of age. We report a child aged 4 months with steroid-resistant NS who had polymorphism of NPHS1 (E117K) and mutation of NPHS2 (P118L). Our patient was carrying a polymorphic NPHS1 mutation, while phenotypically she had a poor prognostic NPHS2 mutation. However, it must be questioned whether this polymorphic change (E117K) alters the signaling pathways of the podocytes and leads to P118L mutation, thus making it behave differently. Perhaps, this would be called a genetic modifier in future.

Outcome results in children with IgA nephropathy: a single center experience.

Background Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis. Patients manifest variable clinical symptoms (eg, microhematuria) with preserved or progressive deterioration of renal function resulting in end-stage renal disease. The aim of this study was to evaluate patients from a single center to describe the clinical features, treatments, and follow-up results of those with the disease. Methods This is a retrospective data study of all children with IgAN. Patients who had a histopathologically proven diagnosis of IgAN and were followed up for at least 5 years were included in the study. Renal biopsy, graded as Hass classification, was performed on all patients. A total of 39 patients were included in the study. Results The mean follow-up time (± standard deviation) was 10.4 ± 3.51 (range 5–16) years. Twenty-nine patients (74.4%) were male and ten (25.6%) were female. Nineteen (48.7%) patients presented with recurrent macroscopic hematuria, ten (25.6%) with microscopic hematuria ± proteinuria, six (15.4%) with nephritic syndrome, and four (10.3%) with nephrotic syndrome. All patients underwent a renal biopsy, which was graded according to the Hass classification. At the end of follow-up time, 18 (46.1%) patients were normal, 15 (38.5%) had minor urinary abnormalities, three (7.7%) had active renal disease, and three (7.7%) developed renal failure. Conclusion The results of the present study are better than those from most other series. The majority of children with IgAN in this study were admitted with recurrent macroscopic hematuria and found to have a good prognosis. We suggest that children with IgAN have a good prognosis in the first 5-year follow-up period.

The predictive value of urinary UPIb mRNA levels in VUR and recurrent urinary tract infections.

BACKGROUND Vesicoureteral reflux (VUR) is a risk factor for progressive kidney damage especially when it is accompanied by urinary tract infections (UTIs). Uroplakins (UPs) are integral proteins found in the structure of urothelium. In the present study, we evaluated the usefulness of urinary UPIb messenger ribonucleic acid (mRNA) levels as an early and noninvasive diagnostic tool for VUR and as an indicator for predisposition to UTI. METHODS Urinary UPIb mRNA levels were determined in patients experiencing their first UTI episode (n = 28) or recurrent UTI (n = 31) as well as patients having UTI with VUR (n = 30). These results were compared to a control group (n = 26). RESULTS The UPIb mRNA values among patients diagnosed with their first UTI were lower, but not statistically different, than those in the control group. The UPIb mRNA levels of patients with recurrent UTI and UTI with VUR were significantly lower than those observed in control individuals. CONCLUSION Urine UPIb levels may be useful for predicting the risk of recurrent UTI in patients diagnosed with their first UTI and may also be considered as a noninvasive screening test for VUR.

Clinical Everolimus Experience in Pediatric Renal Transplant Patients

OBJECTIVE Everolimus is a potent immunosuppressive agent that has antiproliferative activities. This study sought to share our experience among renal transplanted children who required conversion from calcineurin inhibitors (CNIs) to the mammalian target of rapamycin inhibitor everolimus. PATIENTS AND METHODS Exclusion criteria were multiple organ transplantations, loss of a previous graft due to immunologic reasons, receipt of an organ donated after cardiac death, donor age <5 years or >65 years, panel reactive antibodies >25%, platelets <75,000/mm(3), absolute neutrophil count of <1,500/mm(3), leucocytes <2,500/mm(3), hemoglobin <6 g/dL, severe liver disease, cold ischemia time >40 hours or anti-HLA panel-reactive antibodies >50%. RESULTS Eighteen renal transplant patients (10 male, 8 female) underwent conversion to everolimus from CNI: 8 from cyclosporine (CsA) and 10 from tacrolimus. The mean age was 12.6 ± 0.9 years and the mean body mass index 21.8 ± 1.7 kg/m(2). The mean 2-hour postdose level of CsA before conversion was 671 ± 142 ng/mL; the patients on tacrolimus showed a mean trough concentration of 4.5 ng/mL. Six (33,3%) were taking mycophenolate mofetil and 12 (66.6%) enteric-coated mycophenolate sodium. No significant changes were observed in either hepatic functions, serum lipids, or hemograms. There was no mortality or graft loss. The mean level of serum creatinine was 1.3 ± 0.7 mg/dL before and 1.09 ± 0.6 mg/dL after conversion. Proteinuria observed in only 1 patient was well controlled with angiotensin-converting enzyme inhibitor therapy. All patients responded to statin therapy. One patient developed unilateral lower extremity edema and 1 a lymphocele. Although there were 3 cases (14%) of biopsy-confirmed acute rejection, there was no mortality or graft loss. CONCLUSIONS Everolimus conversion has become an excellent choice, offering safety and efficacy with good outcomes.

Effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism in children with chronic kidney disease

Background We investigated the effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism, i.e. serum levels of fibroblast growth factor 23 (FGF23), Klotho, bone alkaline phosphatase (BAP) and sclerostin, in two cohorts with chronic kidney disease (CKD). Methods In all, 80 vitamin D-deficient children were selected: 40 with mild to moderate CKD from the ERGO study, a randomized trial of ergocalciferol supplementation [estimated glomerular filtration rate (eGFR) 55 mL/min/1.73 m2], and 40 with advanced CKD from the observational Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study (eGFR 24 mL/min/1.73 m2). In each study, vitamin D supplementation was started in 20 children and 20 matched children not receiving vitamin D served as controls. Measures were taken at baseline and after a median period of 8 months. Age- and gender-related standard deviation scores (SDSs) were calculated. Results Before vitamin D supplementation, children in the ERGO study had normal FGF23 (median 0.31 SDS) and BAP (-0.10 SDS) but decreased Klotho and sclerostin (-0.77 and -1.04 SDS, respectively), whereas 4C patients had increased FGF23 (3.87 SDS), BAP (0.78 SDS) and sclerostin (0.76 SDS) but normal Klotho (-0.27 SDS) levels. Vitamin D supplementation further increased FGF23 in 4C but not in ERGO patients. Serum Klotho and sclerostin normalized with vitamin D supplementation in ERGO but remained unchanged in 4C patients. BAP levels were unchanged in all patients. In the total cohort, significant effects of vitamin D supplementation were noted for Klotho at eGFR 40-70 mL/min/1.73 m2. Conclusions Vitamin D supplementation normalized Klotho and sclerostin in children with mild to moderate CKD but further increased FGF23 in advanced CKD.

Renal abscess in a previously healthy 4-year-old girl: A case report

Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abdominal pain and occasional flank mass. Renal ultrasonography enables us to achieve an early diagnosis, however; it may still be insufficient to distinguish it from pyelonephritis. Renal abscesses are usually associated with different predisposing conditions. In the present report, we aim to describe a case of a previously healthy child who developed a renal abscess.

The Prevalence and Risk Factors of Anemia in Children with Renal Transplant

Objective: Anemia is commonly observed during the follow-ups of patients with renal transplantation. There is not enough information on anemia observed in children with renal transplantation. The aim of this study was to evaluate the prevalence and risk factors of anemia in children with renal transplantation on the short and long-term posttransplantation period. Material and Methods: This study was performed in children who underwent renal transplant at Ege University. Anemia was defi ned as having a value less than 2 Standard Deviation for hematocrit levels according to age. Results: In the early post-transplant period, the incidence of anemia was found as 18%. Late post transplant anemia rate was detected as 27.5% at 60 months. The incidence of anemia was 18.4%, 23.3%, 23% and 27.5%, at 24, 36, 48 and 60 months after transplantation, respectively. Anemia was detected at least once in 71 (67.6%) patients at any point of the follow-up period. There was no correlation between Angiotensin Converting Enzyme inhibitor therapy and anemia at any post-transplant time. Donor type and donor age were not signifi cantly associated with late anemia. On the other hand, late anemia was signifi cantly associated with a history of rejection. Conclusion: Low estimated Glomerular Filtration Rate, longer duration of post-transplantation and rejection attacks are the risk factors for anemia in pediatric renal transplant recipients.

Kronik diyaliz tedavisi alan çocuklarda eritropoetin kullanımının ortalama trombosit hacmine etkisi

Objective: In this study, it was aimed to determine the relationship between erythropoietin (EPO) use and mean platelet volume (MPV) in the children undergoing dialysis. Methods: MPV values before and after EPO use in 36 patients (16 hemodialysis - HD, 20 peritoneal dialysis PD) were retrospectively evaluated. Patients were divided into two groups according to weekly EPO need as; given less than 150 U/kg defined as low EPO and more than 150 U/kg as high EPO groups. The age, weight, primary cause of chronic renal failure, dialysis methods and EPO dosages of patients were recorded. Blood samples were taken before and 4 weeks after EPO usage and MPV values were noted from complete blood counts. Results: While significant increase was seen in the MPV values after EPO in comparison to MPV values before EPO in the HD group (8.18±1.52 fL vs. 9.20±1.46 fL; p=0.046); near significant difference was found in the MPV levels after EPO (8.28±1.80 fL vs. 9.39±1.50 fL; p=0,051) in PD group. In HD patients when high dose of EPO was given, MPV levels were found to be significantly elevated (7.81 ± 1.04 vs 9.61 ± 1.05; p=0.06) after EPO. However, no difference was seen with the lowe dose EPO subgroup (8.64 ± 1.97 ve 8.67 ± 1.81; p>0,05). No effect of EPO dose was found on MPV values in PD patients (9.57 ± 1.58 ve 9.1 ± 1.42; p>0.05). Conclusion: It was observed that EPO influenced MPV values in children undergoing HD, while no effect of erythropoietin was found on MPV values of PD patients. Physicians should be careful while using high dose erythropoietin in children with high thrombosis risk. J Clin Exp Invest 2014; 5 (3): 415-419

Risk factors for vesicoureteral reflux in children with upper and lower urinary tract infections.

Objective: Urinary tract infections (UTIs) are common in children and may signal vesicoureteralreflux (VUR). This study aimed to identify the risk factors associated with VUR and to emphasize value of diagnostic imaging studies in children. Methods: This study was assessed 173 medical records of children who had first -time