Nida Dinçel

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.

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.

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.

Urine Matrix Metalloproteinase-3 Level as a Biomarker for Monitoring in Familial Mediterranean Fever Attacks

Objective: Matrix metalloproteinase-3 (MMP-3) has been implicated in experimental and clinical models of human inflammatory conditions. Increased MMPs levels have been shown in serum and body fluids in inflammatory conditions. Familial Mediterranean Fever (FMF) is an inherited, auto inflammatory disease characterized by recurrent self-limited bouts of fever and localized inflammation. We aimed to investigate whether urine MMP-3 level can serve as a biomarker for monitoring attack in FMF patients in daily practice. Methods: We studied 50 patients diagnosed with FMF according to Tel Hashomer criteria and 32 age-matched healthy controls. We determined all subjects both in attack (FMF-AP) and attack free period (FMF-AFP) groups. Serum and urine samples were obtained within the first 6-24 h of the AP, and 10 days later after the attack (AFP). The serum samples were measured on the same day while urine samples were frozen immediately and stored at -80°C. Results: The mean age at onset was 57.26 ± 33.5 months. The most common symptoms seen during the attacks were: fever (80%) abdominal pain (72%), arthritis (40%). In genotype distribution, homozygous M694V mutation was seen mostly (28%). During AP, urine MMP-3 levels of patients were higher as well as during AFP and controls (2.32 ± 0.51, 0.89 ± 2.29 ng/mL and 1.24 ± 0.17 ng/mL, respectively, p=0.00). In AP, urinary MMP levels were higher in patients with arthritis than others (p<0.05). Urinary MMP-3 levels were also significantly higher in males (2.29 ± 0.45 versus 2.24 ± 0.57, p=0, 00). The patients with M694V allele (n=29) had statistically significant high urine MMP-3 levels than others (2.37 ± 0.56 versus 1.99 ± 0.31, p=0.04, respectively). Also, acute phase reactants were higher in patients with M694V allele without statistically significant difference (p=0.89, 0.75, 0.86, 0.85, 0.7, respectively). Conclusion: In this study we focused on presence of MMPs in urine and showed inflammation-specific MMP patterns may provide clinicians valuable information in FMF patients.

A Case With Congenital Nephrotic Syndrome Having E117K Mutation: Is This a Polymorphism? or Mutation?

Ebru Yılmaz1*, Nida Dinçel2, Gözde Gözüolu3, Kadriye Özdemir4, Afig Berdeli4 and Sevgi Mir4 1Behcet Uz Children Disease and Surgery Training and Research Hospital, Izmir, Turkey 2Ankara Children Health and Research Hematology Oncology Education Research Hospital, Ankara, Turkey 3Department of Pediatrics, Faculty of Medicine, Ege University, Turkey 4Department of Pediatric Nephrology, Faculty of Medicine, Ege University, Turkey

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

PW01-017 – Urine MMP-3 level as a biomarker for in FMF attack

Matrix metalloproteinase-3 (MMP-3) has been implicated in experimental and clinical models of human inflammatory conditions. Increased levels of MMPs have been shown in serum and other body fluids such as synovial fluid in inflammatory conditions including ankylosing spondylitis, rheumatoid arthritis and juvenile idiopathic arthritis (JIA). Familial Mediterranean fever (FMF) is an autosomal recessive, inherited, autoinflammatory disease characterized by recurrent, self-limited bouts of fever and localized inflammation, usually involving the peritoneum, pleura, joints or skin.