Association of clearance of middle- and large-molecular-weight substance with arterial stiffness and left ventricular mass in children receiving renal replacement therapy.

BACKGROUND: The prominent cause of mortality in children receiving dialysis treatment is cardiovascular diseases. Risk factors related to chronic renal disease, are effective in the development of cardiovascular diseases. The aim of study was to investigate cardiovascular system (CVS) involvement for functional and structural alterations in children receiving dialysis, and display any association between cardiovascular morbidity and uremic toxins. METHODS: 20 dialysis patients and 20 healthy controls were included to the study. Clearance of small, middle and large molecular-weight uremic toxins was evaluated in blood samples collected 30 minutes before (D0) and 2 hour after dialysis (D2), and change value was calculated as D0-D2/D0. Cardiovascular involvement was determined by comparing arterial stiffness, carotid intima-media thickness (CIMT) and Left Ventricular Mass Index (LVMI) with the control group. RESULTS: Four patients receiving hemodialysis and two patients in continuous ambulatory peritoneal dialysis (CAPD) group who have significant differences in all functional and structural parameters were detected. Four dialysis patients with detected cardiovascular disease have distinctively lower beta-2 microglobulin and homocysteine clearances compared to the patients with no CVS involvement. CONCLUSIONS: The clearance of middle and large molecular-weight substances should be closely monitored in children receiving dialysis.

Incidence and causes of urolithiasis in children between 0-2 years.

BACKGROUND: Urolithiasis (UL) is a common problem in pediatric nephrology practice. About 9-23% of all pediatric UL cases consist of patients under 1 years old. The aim of the present study was to determine etiologic causes, clinical features and short term prognosis of urolithiasis in the first two years of life. METHODS: Two-hundred children between 0-2 years of age (mean age 10.3±6.1 months) with urolithiasis were included to the study. All children were completely evaluated for urolithiasis etiology. Patients were followed for at least 1 year with 3-month follow-up intervals. RESULTS: The mean follow-up duration was 36.2 months. Family history was positive for urolithiasis in 99 (49.5%) patients. The cause of admission were urinary tract infection and related symptoms in 101 (50.5%), incidentally during imaging for other causes in 40 (20%), stone passage in 21 (10.5%), hematuria in 18 (9%), voiding difficulty in 14 (7%) and antenatal detected urinary anomaly in 6 (3%) patients. Accompanying urinary anomalies (anatomical defects) in 51 (25.5%) patients were detected primarily including vesiculoureteral reflux (VUR) in 32 (62.9%) of them. Development of new stones was determined in 45 (22.5%) patients in the first year, in 15 (22%) patients in the second year and in 8 (36.3%) patients in the third year. CONCLUSIONS: In result, among pediatric urinary stone diseases, infantile UL appears to be a seperate clinical entity in terms of both the etiological characteristics and the clinical course of the disease. Furthermore, when an infant is presented with nonspecific symptoms for kidney stone, a renal ultrasonography could be performed for detecting nephrolithiasis.

Can Urinary Nerve Growth Factor and Brain-Derived Neurotrophic Factor be used in the Diagnosis and Follow-Up of Voiding Dysfunction in Children?

PURPOSE: We investigated the utility of urinary nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) levels as non-invasive markers for diagnosis and evaluation of treatment efficacy in children with overactive bladder (OAB). MATERIALS AND METHODS: This prospective study included 24 children with OAB and 30 healthy controls. At the time of diagnosis, micturition disorder symptom scores (MDSS) were determined, blood and urine samples were collected, and anticholinergic therapy was initiated. Clinical responses were evaluated, at the third and sixth month of treatment, by MDSS and urinary NGF, BDNF, and creatinine levels. RESULTS: The patient group had significantly higher urine NGF/Cr ratio (975 ± 827 and 159 ± 84, respectively, P < .001) and BDNF/Cr ratio (5.98 ± 5.78 and 0.81 ± 0.70, respectively, P < .001) before treatment. Significantly decreased BDNF/Cr ratio was found at the sixth month (5.98 ± 5.78 and 2.24 ± 0.98, respectively, P = .004). NGF/Cr > 360 was found to have 87.5% sensitivity and 100% specificity, and BDNF/Cr > 1.288 was found to have 87.5% sensitivity and 83.3% specificity for OAB diagnosis. CONCLUSION: In conclusion, urine NGF/Cr and BDNF/Cr ratios may be useful markers for diagnosis of OAB. The BDNF/Cr ratio was found to be more significant in monitoring treatment response. .

Assessment of vascular function in systemic onset juvenile idiopathic arthritis.

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 these patients to an early onset of atherosclerosis. We aimed to assess the presence of early vascular dysfunction in patients with systemic onset juvenile idiopathic arthritis (sJIA) and investigate the role of therapy sJIA in vascular health. Thirty-three patients (22 males, 11 females) diagnosed with sJIA according to the International League of Associations for Rheumatology criteria were compared to 72 age- and sex-matched controls. None of the participants was overweight, obese, or had a history of hypertension, dyslipidemia, diabetes mellitus, or cardiovascular disease. Arterial stiffness (As) was evaluated by measurement of carotid-femoral pulse wave velocity (PWV) and augmentation index (AIx) with a Vicorder. The mean age of patients in this study was 9.96 ± 3.71 years (range 4-16 years) and the mean age of controls was 10.9 ± 3.52 years (range 4-19 years). These two groups were well matched for age, sex, and BMI. The mean age of patients at the onset of disease was 7.06 ± 3.9 years (range 3-15 years). The mean duration of disease and active disease was 79 ± 45 months (range 6-162 months) and 58 ± 49 months (range 1-101 months), respectively. The highest levels of PWV and AIx were found in the patient group. Seven patients had had macrophage activation syndrome at presentation. In these patients, vascular changes were higher than other patients (6.30 ± 0.42 m/s vs 5.17 ± 0.55 m/s, p = 0.01, respectively). The corticosteroid therapy was found associated with higher PWV, (p < 0.05), while there was no difference between vascular parameters and use of non-steroid therapies (methotrexate (MTX), anti-TNF alfa agents). We also find statistically significant correlation between PWV and disease duration (p = 0.003, r = 0.45). Vascular function is impaired in patients with sJIA at a very young age. Vascular dysfunction may be partly attributed to the effects of disease-related characteristics (inflammation, disease activity, and medications).

Cardiovascular functional and structural changes in children with primary hypertension.

BACKGROUND: Primary hypertension in children is not a benign condition because of end-organ damage. Early investigations of cardiovascular effects rely on vascular structural and functional changes. We aimed to determine presence of early cardiovascular involvement in children with primary hypertension. For this purposes, we investigated functional and morphological changes in vascular system. METHODS: Seventy-five consecutive patients with primary hypertension and 35 healthy children comparable for age and gender were studied. Patients were classified according to the stages of blood pressure. Intima-media thickness of the carotid arteries (cIMT), carotid-femoral pulse wave velocity (PWVcf) and Pulse Wave Augmentation Index (AIx) and Left Ventricular Mass Index (LVMi) were investigated. RESULTS: PWVcf and AIx were higher in patients (5.87±0.87 m/s vs. 5.29±0.67 m/s, P=0.02; 9.41±8.54% vs. 8.36±3.59%, P=0.04, respectively) than in controls. cIMT was evaluated significantly higher in study group than controls (0.46±0.06 mm vs. 0.35±0.12 mm, respectively, P=0.01). The mean LVMi was higher in patients (32.9±11.5 vs. 28.8±1.55, P=0.01). CONCLUSION: Our results demonstrated that vascular findings were more sensitive for target organ changes than the more frequently utilized LVMi in patients with pre-hypertension.

Are we late for the diagnosis of acute kidney injury in the intensive care units in pediatric patients? A preliminary, retrospective observational study among 66 patients.

BACKGROUND: The purposes of this study were to emphasize the importance of Risk, Injury, Failure, Loss, and End-Stage (RIFLE) classification in early diagnose and prognosis of acute kidney injury (AKI), and to evaluate the practicability of the RIFLE criteria in intensive care units. METHODS: Sixty-six patients applied acute peritoneal dialysis were included into the study. Patients having acute peritoneal dialysis within the first 24 hours of intensive care unit admission were named group 1, between 24-48 hours group 2, and those who had acute peritoneal dialysis 48 hours or more after admission to the intensive care unit were named group 3. Retrospectively, we evaluated patients by RIFLE criteria at the consultation time, and patients who had been just in AKI were called late referral patients. The mean interval time between the onset of AKI and the consultation time was defined as delay time in late referral patients. RESULTS: There were 20 patients in group 1, 15 were in group 2 and 31 in group 3. In total there were 18/66 patients in risk, 13/66 in injury while 35/66 in failure. There was statistically difference between delay times of in risk and failure class in group 3 (P<0.05). Also delayed patient numbers of both risk and failure class were found statistically highly significant (P<0.001). CONCLUSIONS: Most of the patients were in failure class at the time of consultation. We guess that if they would have been diagnosed earlier, prognosis might have been better. Therefore early diagnosis of AKI with RIFLE criteria and early initiation of acute peritoneal dialysis would probably improve prognosis.

Bioimpedance for assessing volume status in children with nephrotic syndrome.

BACKGROUND/AIM: The effectiveness of assessing volume load via bioimpedance in pediatric patients with nephrotic syndrome (NS) was investigated. MATERIALS AND METHODS: Patients with NS (n = 34) were compared with healthy controls (n = 20). The inferior vena cava index and inferior vena cava collapsibility index (IVCCI) scores were determined for all subjects. Bioimpedance measurements were used to directly determine volume load. Clinical findings, relative fluid load, and echocardiographic measurements were determined and compared with bioimpedance in calculating volume load. RESULTS: The sensitivity and specificity of bioimpedance in determining volume load were found to be 65% and 90%, respectively. IVCCI was lower in NS patients than in controls. There were also significant differences in IVCCI values between patients with localized and generalized edema. Relative fluid load was higher in NS patients with generalized edema as opposed to patients with localized edema. For calculating volume load, the sensitivity of bioimpedance, clinical findings, and echocardiographic measurements was 87%, 95%, and 83%, respectively, whereas the specificity of determining volume load by bioimpedance, clinical findings, and echocardiographic measurements was 50%, 10%, and 80%, respectively. CONCLUSION: Our data suggest that bioimpedance may be superior to echocardiography in determining volume load in children with NS, because it is cost-effective, reliable, and relatively simple to perform.

Familial Mediterranean fever in children from the Aegean region of Turkey: gene mutation frequencies and phenotype-genotype correlation.

BACKGROUND/AIM: Familial Mediterranean fever (FMF) is diagnosed by fever episodes with sterile peritonitis, arthritis, pleurisy, and erysipelas-like erythema. The relationship between phenotype and genotype in FMF has not been adequately explained. The aim of this study was to characterize the phenotype and genotype correlation in FMF. MATERIALS AND METHODS: Clinical diagnosis of FMF was conducted according to the Tel Hashomer criteria. Pras scoring was used to determine clinical severity. FMF strip assay analysis was used, and the hotspot regions were observed with PCR amplification and automatic DNA sequence analysis method. RESULTS: We showed commonly seen mutations (most frequently M694V) in a study group of 191 patients. The disease severity score of patients with M694V mutation was high on the Pras scoring system. Patients with M694V mutation needed high colchicine dosages to control disease activity. R202Q was the most commonly seen polymorphism in 70 patients. The coexpression of R314R single nucleotide polymorphism on third exon was shown in our study. Moreover, D102D, G138G, and A165A subhaplotypes and E474E, Q476Q, and D510D subhaplotypes were also shown. CONCLUSION: DNA sequence analysis should be a commonly used method for progress in the field of molecular genetics and for the better understanding of the FMF phenotype and genotype relationships in all populations.

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

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.