Kadriye Özdemir

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.

Treatment of Steroid and Cyclophosphamide-Resistant Nephrotic Syndrome with Mycophenolate Mofetil and High Dose Dexamethasone (DEX)

Background: Clinicians often face with therapeutic challenges during the treatment of children with steroidresistant nephrotic syndrome (SRNS). The management of SRNS is primarily aimed at decreasing proteinuria and inducing remission. Mycophenolate mofetil (MMF) in combination with steroids is known to have some efficacy in the management of SRNS. The aim of this study is to evaluate the outcome of MMF/DEX therapy regimen. Material and methods: We reported a prospectively longitudinal clinical series of patients with SRNS, all of whom have been treated with Mycophenolate mofetil and oral dexamethasone (DEX). We enrolled 29 children who were previously treated with steroid and cyclophosphamide at Ege University, Children’s Hospital. Treatment with MMF/DEX was administered for up to 52 weeks. These children were followed for a period of 2 years. Complete remission was defined as negative or trace proteinuria on urinalysis with a serum albumin level of >2.5 g/dl. On the other hand, partial remission was defined as a serum albumin level of >2.5 g/dl, but developing persistent proteinuria at non-nephrotic levels. Results: Following the course of MMF/DEX, 68.9% (20/29) of children achieved a complete remission and 33.4% (1/29) remained at partial remission. After 24 months of follow- up, 55.1% (16/29) of children on MMF/DEX reached a complete remission and 13.7% (4/29) remained at partial remission. Conclusion: MMF/DEX can be an effective and safe maintenance therapy among children with SRNS.

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

P01-024 – Vascular risk assessment and MMP-3 gene in FMF

The patients characterized with chronic subclinical inflammation even during attack-free periods, are now considered to have an increased risk of atherosclerotic complications as well as other autoinflammatory disease. Damage to the arterial wall due to atherosclerosis causes increased arterial stiffness. Pulse wave velocity (PWV), a noninvasive measure of arterial stiffness, is accepted to be an indicator of subclinical atherosclerosis. Cardiovascular disease included various risk markers; blood biomarkers and genetic markers. Matrix metalloproteinases (MMPs) are closely related proteinases that together are able to degrade all macromolecules of the extracellular matrix. MMPs are potentially implicated in atherogenesis, progression of atherosclerosis. The gene encoding MMP-3 is polymorphic and an insertion (6A)/deletion (5A) polymorphism (5A/6A polymorphism) in the MMP-3 gene may have functional significance in the regulation of its expression. The 5A allele was associated with higher and the 6A allele with lower transcriptional activity. Up to date, the 6A/6A and 5A/6A genotypes were associated with coronary artery disease and carotid atherosclerosis in adults.

PReS-FINAL-2205: Vascular risk assesment and MMP-3 gene in FMF

The patients characterized with chronic subclinical inflammation even during attack-free periods, are now considered to have an increased risk of atherosclerotic complications as well as other autoinflammatory disease. Damage to the arterial wall due to atherosclerosis causes increased arterial stiffness. Pulse wave velocity (PWV), a noninvasive measure of arterial stiffness, is accepted to be an indicator of subclinical atherosclerosis. Cardiovascular disease included various risk markers; blood biomarkers and genetic markers. Matrix metalloproteinases (MMPs) are closely related proteinases that together are able to degrade all macromolecules of the extracellular matrix. MMPs are potentially implicated in atherogenesis, progression of atherosclerosis. The gene encoding MMP-3 is polymorphic and an insertion (6A)/deletion (5A) polymorphism (5A/6A polymorphism) in the MMP-3 gene may have functional significance in the regulation of its expression. The 5A allele was associated with higher and the 6A allele with lower transcriptional activity. Up to date, the 6A/6A and 5A/6A genotypes were associated with coronary artery disease and carotid atherosclerosis in adults.

P01-049 – Assessment of vascular function in systemic JIA.

An increased incidence of cardiovascular disease has been found in rheumatic disorders. Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Prolonged immunological inflammatory process leads in these patients to an early onset of atherosclerosis.