Urine soluble TLR4 levels may contribute to predict urinary tract infection in children: the UTILISE Study.

BACKGROUND: One of the most common bacterial infections in childhood is urinary tract infection (UTI). Toll-like receptors (TLRs) contribute to immune response against UTI recognizing specific pathogenic agents. Our aim was to determine whether soluble TLR4 (sTLR4), soluble TLR5 (sTLR5) and interleukin 8 (IL-8) can be used as biomarkers to diagnose UTI. We also aimed to reveal the relationship between urine Heat Shock Protein 70 (uHSP70) and those biomarkers investigated in this study. METHODS: A total of 802 children from 37 centers participated in the study. The participants (n = 282) who did not meet the inclusion criteria were excluded from the study. The remaining 520 children, including 191 patients with UTI, 178 patients with non-UTI infections, 50 children with contaminated urine samples, 26 participants with asymptomatic bacteriuria and 75 healthy controls were included in the study. Urine and serum levels of sTLR4, sTLR5 and IL-8 were measured at presentation in all patients and after antibiotic treatment in patients with UTI. RESULTS: Urine sTLR4 was higher in the UTI group than in the other groups. UTI may be predicted using 1.28 ng/mL as cut-off for urine sTLR4 with 68% sensitivity and 65% specificity (AUC = 0.682). In the UTI group, urine sTLR4 levels were significantly higher in pyelonephritis than in cystitis (p < 0.0001). Post-treatment urine sTLR4 levels in the UTI group were significantly lower than pre-treatment values (p < 0.0001). CONCLUSIONS: Urine sTLR4 may be used as a useful biomarker in predicting UTI and subsequent pyelonephritis in children with UTI. A higher resolution version of the Graphical abstract is available as Supplementary information.

Complement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?

BACKGROUND: C3 glomerulopathy (C3G) is a complement-mediated disease. Although genetic studies are not required for diagnosis, they are valuable for treatment planning and prognosis prediction. The aim of this study is to investigate the clinical phenotypes, kidney survival, and response to mycophenolate mofetil (MMF) treatment in pediatric C3G patients with and without mutations in complement-related genes. METHODS: Sixty pediatric C3G patients were included, divided into two groups based on complement-related gene mutations. Demographic and clinical-pathological findings, treatment modalities, and outcome data were compared, and Kaplan-Meier analysis was performed for kidney survival. RESULTS: Out of the 60 patients, 17 had mutations. The most common mutation was in the CFH gene (47%). The mean age at diagnosis was higher in the group with mutation (12.9 ± 3.6 vs. 11.2 ± 4.1 years, p = 0.039). While the patients without mutation most frequently presented with nephritic syndrome (44.2%), the mutation group was most likely to have asymptomatic urinary abnormalities (47.1%, p = 0.043). Serum parameters and histopathological characteristics were similar, but hypoalbuminemia was more common in patients without mutation. During 45-month follow-up,10 patients progressed to chronic kidney disease stage 5 (CKD5), with 4 having genetic mutation. The time to develop CKD5 was longer in the mutation group but not significant. MMF treatment had no effect on progression in either group. CONCLUSIONS: This study is the largest pediatric C3G study examining the relationship between genotype and phenotype. We showed that the mutation group often presented with asymptomatic urinary abnormalities, was diagnosed relatively late but was not different from the without mutation group in terms of MMF treatment response and kidney survival.

Kidney Stones in Epileptic Children Receiving Ketogenic Diet: Frequency and Risk Factors.

BACKGROUND: Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney stones and risk factors for their development in epileptic children receiving KD is unclear. The aim of this study was to determine the frequency and risk factors for the development of renal stones in children receiving KD therapy. METHODS: A total of 95 patients receiving KD were identified. Of these, seven patients were excluded from the study due to the duration of KD being less than 12 months. The remaining 88 children were enrolled in the study. RESULTS: Renal stones were detected in 15 patients (17%), of which 12 (73.3%) received potassium citrate treatment. Two (13.3%) patients needed lithotripsy despite receiving potassium citrate treatment, and one of these, who received potassium citrate treatment for 5 months, developed acute vesicourethral reflux and underwent surgery. No patient discontinued KD due to renal stone development. The serum uric acid concentrations and urine calcium/creatinine ratio did not change significantly over the 24-month follow-up period. Age, gender, etiology, age at seizure onset, duration of KD, mobility status, use of topiramate or zonisamide, and the number of antiepileptic drugs used were not significantly different between patients with and without kidney stones. CONCLUSION: Renal stone appears to be a common adverse effect of KD therapy. Although adequate hydration and potassium citrate treatment are effective in most patients, lithotripsy and surgery may be required in a minority of patients.

Urinary HSP70 improves diagnostic accuracy for urinary tract infection in children: UTILISE study.

BACKGROUND: The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation. We explored the added value of uHSP70 in discriminating UTI from other infections and conditions confused with UTI. METHODS: A total of 802 children from 37 pediatric centers in seven countries participated in the study. Patients diagnosed with UTI (n = 191), non-UTI infections (n = 178), contaminated urine samples (n = 50), asymptomatic bacteriuria (n = 26), and healthy controls (n = 75) were enrolled. Urine and serum levels of HSP70 were measured at presentation in all patients and after resolution of the infection in patients with confirmed UTI. RESULTS: Urinary (u)HSP70 was selectively elevated in children with UTI as compared to all other conditions (p < 0.0001). uHSP70 predicted UTI with 89% sensitivity and 82% specificity (AUC = 0.934). Among the 265 patients with suspected UTI, the uHSP70 > 48 ng/mL criterion identified the 172 children with subsequently confirmed UTI with 90% sensitivity and 82% specificity (AUC = 0.862), exceeding the individual diagnostic accuracy of leukocyturia, nitrite, and leukocyte esterase positivity. uHSP70 had completely normalized by the end of antibiotic therapy in the UTI patients. Serum HSP70 was not predictive. CONCLUSIONS: Urine HSP70 is a novel non-invasive marker of UTI that improves the diagnostic accuracy of conventional urinalysis. We estimate that rapid urine HSP70 screening could spare empiric antibiotic administration in up to 80% of children with suspected UTI. A higher resolution version of the Graphical abstract is available as Supplementary information.

Automated complete blood counter, urine analyzer and urine dipstick test results are correlated with thoma cell counting chamber counts in the diagnosis of dialysis related peritonitis in children.

INTRODUCTION: Peritoneal dialysis is the treatment of choice for end-stage renal disease. Peritoneal dialysis related peritonitis is of great importance for patient and technical survival. The aim of our study was to evaluate the accuracy and the correlation between the three methods (complete blood count, urinalysis device, urine dipstick test) and with the reference manual method (Thoma Cell Counter Chamber). MATERIALS AND METHODS: We retrospectively analyzed 167 peritoneal fluid samples taken from 25 patients receiving peritoneal dialysis treatment. Leukocyte counts were evaluated with Thoma Cell Counter Chamber, complete blood count, urinalysis device and urine dipstick test. RESULTS: There was a significant positive correlation between Thoma Cell Counter Chamber and complete blood count results (Spearman's rho=0.70), between Thoma Cell Counter Chamber and urinalysis device (Spearman's rho=0.73), and between Thoma Cell Counter Chamber and urine dipstick test (Spearman's rho=0.71). Area under curve for complete blood count, urinalysis device and urine dipstick test were 0.93, 0.94 and 0.89 respectively, indicating good accuracy. Sensitivity and specificity were 89.7% and 86.7% in the complete blood count analysis (associated criterion: 130 cells/mm3). Sensitivity and specificity were 89.7% and 86.7% in the urinalysis device (associated criterion: 10 cells/HPF). Sensitivity and specificity were 79.6% and 91.4% when in the urine dipstick test analysis (associated criterion: +1 positivity). The Bland-Altman plot showed good agreement. CONCLUSION: Automatic complete blood count and urinalysis devices have good correlation and agreement with manual method in the diagnosis of peritonitis in the pediatric age group. Urine dipstick test in the home setting can be useful for screening patients with suspected peritonitis.

Evaluation of proximal tubule functions in children with COVID-19: a prospective analytical study.

BACKGROUND: There are limited numbers of studies focusing on renal effects of coronavirus disease 2019 (COVID-19) infection and proximal tubular dysfunction in children with COVID-19 infections. The purpose of this study was to evaluate the functions of the proximal tubule in hospitalized children with confirmed acute COVID-19. METHODS: The children who were hospitalized for confirmed COVID-19 were included in this prospective descriptive analysis. The presence of at least two of the following four abnormalities was used to diagnose proximal tubule injury: abnormal tubular reabsorption of phosphate, normoglycemic glycosuria, hyperuricosuria, and proteinuria. RESULTS: A total of 115 patients were included in the study. About a third of the individuals had elevated blood creatinine levels or proteinuria. In addition, abnormal renal tubular phosphate loss measured by renal tubular phosphate loss was found in 10 (8.7%) patients, as was hyperuricosuria in 28.6%. As a result, total proximal tubular dysfunction was found in 24 (20.9%) patients. CONCLUSIONS: One in every five children with acute COVID-19 infections had proximal tubular dysfunction, according to our data. Although, the rate of proximal tubular dysfunction was lower than in adults, it should be noted. The recovery of proximal tubular function in children with COVID-19 should be followed.

Early endoscopic injection may prevent new scarring in idiopathic detrusor overactivity disorder with vesicoureteral reflux in children.

OBJECTIVE: To present baseline characteristics and the long-term treatment results of three groups of patients with idiopathic detrusor overactivity (IDOD) and vesicoureteral reflux (VUR) according to different treatment regimens, which underwent endoscopic subureteric injection (STING) in the early phase of targeted treatment (TT) (ES group), underwent STING in the late phase of TT (LS group) and with TT only (TT group). PATIENTS AND METHODS: A total of 49 IDOD cases with VUR which were divided into three groups according to treatment regimens were evaluated in terms of age, symptoms, bladder capacities, involuntary contraction pressures (ICP), presence and degree of renal scar, differential renal functions (DF), new scar formation and STING success. RESULTS: There was no significant difference between the groups in terms of age, side, symptoms, presence of urinary tract infection (UTI), DF, ICP and bladder capacity at diagnosis. A high grade of reflux was found to be significantly lower in the TT group (p = 0.037). There was no significant difference in terms of ICP, DF, bladder capacity and reflux grade between ES and LS Group. But new scar formation was more in LS Group (p = 0.003). CONCLUSION: The STING success is satisfactory in IDOD cases with VUR, waiting a long period of time for diminishing symptoms may cost new scar formation.

Evaluation of renal effects of liposomal amphotericin B in children with malignancies with KDIGO and RIFLE criteria.

BACKGROUND: Amphotericin B is a broad-spectrum antifungal agent and is the backbone of the treatment for medically important opportunistic fungal pathogens in children. This study aimed to compare the nephrotoxicity associated with L-AmB in children with acute lymphoblastic leukemia and acute myeloid leukemia. MATERIALS AND METHODS: A total of 112 pediatric acute lymphoblastic leukemia or acute myeloid leukemia patients who received treatment with L-AmB (Ambisome®) at the University of Health Sciences Dr Behcet Uz Children's Hospital over 7 years were included. The incidence of hypokalemia, decreased estimated glomerular filtration rate and presence of acute kidney injury was recorded. RESULTS: The average L-AmB treatment duration was 17.1±15.0 days. Five patients (4.4%) of the patients had grade I acute renal injury according to KDIGO criteria and 16 patients (14.2%) had increased risk for kidney injury according to RIFLE criteria. There were no patients with eGFR decrease above 50% and no renal injury and failure were observed during L-AmB treatment. The rate of patients with hypokalemia in the pre-treatment was 17.9% and the post-L-AmB group was 50.0%. The rate of hypokalemia was higher in the post-treatment group (P=0.0015). Among the 112 patients, only two patients (1.7%) required cessation of L-AmB treatment due to resistant hypokalemia despite supplementation. CONCLUSIONS: Hypokalemia was more common compared to glomerulotoxicity and acute renal injury (according to KDIGO and RIFLE criteria) in pediatric leukemia patients treated with L-AmB. Hypokalemia developed in nearly half of the patients and the study shows the need for randomized controlled trials and strategies for hypokalemia associated with L-AmB treatment.

Ultrasonographic evaluation of the children with candiduria for the fungal ball: Is It necessary?

INTRODUCTION: Imaging of the urinary tract by ultrasonography (USG) or computerized tomography scanning is recommended for detecting structural abnormalities, hydronephrosis, abscesses, emphysematous pyelonephritis, or fungus ball formation. Limited studies on the epidemiology and the imaging results of candiduria were present in the children. AIM: This study aimed to evaluate the results of renal ultrasonography imaging in hospitalized pediatric patients with candiduria. STUDY DESIGN: In this descriptive study, we reviewed our ultrasonography findings with hospitalized children and infants who with candiduria. The study included the period between January 2012 and December 2019. Demographic data, the previous medical history, the clinical features of the patients, ultrasonographic findings of the urinary tract system, presence of an indwelling urinary catheter, type of urinary samplings, type of candida species were retrospectively recorded. The study was approved by Institutional Review Board with the registration number 2019/366. RESULTS: During the study period, 220 children with nosocomial candiduria were evaluated. The most common isolated candida species was Candida albicans (68.2%) and followed by C. tropicalis (9.1%). Among all patients, 2 (0.9%) had renal fungal balls associated with C. Albicans. Twenty-five patients (11.4%) had findings including internal echogenicity in the bladder (n = 12), uroepithelial thickening of the kidney (n = 10), and sediments in the renal pelvis (n = 3). DISCUSSION: Candida albicans was the most prominent candida isolated from the patients. The fungal ball is an uncommon infection especially in children and predominantly caused by Candida species. Fungal ball in the kidney was reported as case reports especially in neonates, in immunosupressed patients, and in patients who had undergone surgical procedures. In our study, none of the patients with the fungal ball were in the neonatal period, however, the patients with fungus ball had underlying disease or condition. Our study has several limitations including a retrospective study, and the USG were not performed by a single radiologist. Besides these limitations, our findings are important to give information about the place of USG for diagnosis of the renal fungal ball in children adding valuable information to a topic in which data came from mainly case reports. CONCLUSIONS: Despite the low incidence of fungal balls reported, considering the high consequences of missing a fungal ball and elimination of it, a non-invasive method such as the renal bladder USG is still necessary for detection of fungal ball especially. More prospective studies are required for high risk groups to establish the diagnostic value of renal USG.

The Emerging Resistance in Nosocomial Urinary Tract Infections: From the Pediatrics Perspective.

BACKGROUND: Healthcare-associated infections results in increased health care costs and mortality. There are limited studies concerning the distribution of the etiologic agents and the resistance patterns of the microorganisms causing healthcare-associated urinary tract infections (HA-UTI) in pediatric settings. OBJECTIVES: The aim of this study was to evaluate the distribution and antibiotic susceptibility patterns of pathogens causing HA-UTI in children. MATERIAL AND METHODS: Isolates from 138 children with UTI who were hospitalized in pediatric, neonatal and pediatric surgery intensive care units were reviewed. RESULTS: Most common isolated organism was Klebsiella pneumoniae (34.1%) and Escherichia coli (26.8%). Among the Pseudomonas aeruginosa, Meropenem and imipenem resistance rates were 46.2% and 38.5%. Extended-spectrum beta-lactamase (ESBL) production was present in 48 Klebsiella species (82.8%). Among ESBL positive Klebsiella species, the rate of meropenem and imipenem resistance was 18.8%, and ertapenem resistance was 45.9%. Extended spectrum beta-lactamase production was present in 27 (72.9%) Escherichia coli species. Among ESBL positive E. coli, the rate of meropenem and imipenem resistance was 7.4%, and ertapenem resistance was 14.8. CONCLUSIONS: Emerging meropenem resistance in P. aeruginosa, higher rates of ertapenem resistance in ESBL positive ones in E. coli and Klebsiella species in pediatric nosocomial UTI are important notifying signs for superbug infections.

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.

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. .

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.

The long-term outlook to final outcome and steroid treatment results in children with idiopathic nephrotic syndrome.

Idiopathic Nephrotic Syndrome (INS) was defined as combination of a nephrotic syndrome and non-specific histological abnormalities of the kidney. Among these abnormalities, minimal change nephrotic syndrome (MCNS) is the most common. We report our experience with MCNS; its clinical course, treatments and outcomes. One-hundred twenty children (66 male, 54 female) with MCNS, admitted to Nephrology Department between 1987-2009 was assessed. Their clinical presentations, treatment and disease courses were reviewed. The mean duration of follow-up was 11.5 ± 1.9 years. Initially, all patients given prednisone 2 mg/kg/ day single dose per four weeks a followed by eight weeks of the same daily dose given every other day. After week 12, prednisone was progressively tapered off at the rate of 0.5 mg/kg per 15 daily intervals until complete discontinuation had been achieved by week 16. Steroid resistance was accepted as no achievement of remission following four weeks of prednisone 2 mg/kg/day followed by three intravenous pulses of corticosteroids. At the end of the initial steroid treatment, 106 (88.3%) patients were determinate steroid responsive while 14 (11.7%) patients were steroid resistance. Thirty-eight patients underwent biopsy. At the end of study recovery rate was increased from 88.3% to 94.1%. In conclusion, most of patients entered remission by our therapy end of follow up time. With the support of our satisfactory results among the whole study group, long-term prednisolone treatment still remains valid.

The long-term outlook to final outcome and steroid treatment results in children with idiopathic nephrotic syndrome.

Idiopathic Nephrotic Syndrome (INS) was defined as combination of a nephrotic syndrome and non-specific histological abnormalities of the kidney. Among these abnormalities, minimal change nephrotic syndrome (MCNS) is the most common. We report our experience with MCNS; its clinical course, treatments and outcomes. One-hundred twenty children (66 male, 54 female) with MCNS, admitted to Nephrology Department between 1987-2009 was assessed. Their clinical presentations, treatment and disease courses were reviewed. The mean duration of follow-up was 11.5 ± 1.9 years. Initially, all patients given prednisone 2 mg/kg/ day single dose per four weeks a followed by eight weeks of the same daily dose given every other day. After week 12, prednisone was progressively tapered off at the rate of 0.5 mg/kg per 15 daily intervals until complete discontinuation had been achieved by week 16. Steroid resistance was accepted as no achievement of remission following four weeks of prednisone 2 mg/kg/day followed by three intravenous pulses of corticosteroids. At the end of the initial steroid treatment, 106 (88.3%) patients were determinate steroid responsive while 14 (11.7%) patients were steroid resistance. Thirty-eight patients underwent biopsy. At the end of study recovery rate was increased from 88.3% to 94.1%. In conclusion, most of patients entered remission by our therapy end of follow up time. With the support of our satisfactory results among the whole study group, long-term prednisolone treatment still remains valid.

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.

Hemolytic uremic syndrome outbreak in Turkey in 2011.

The aim of this retrospective multicenter study was to define the epidemiological and clinical features and prognostic factors of the first diarrhea-related hemolytic uremic syndrome (D+HUS) outbreak in Turkey in 2011. All pediatric nephrology centers in Turkey were asked about D+HUS patients via e-mail. Seventy D+HUS patients (median age: 5.7 years) participated. The seasonal peak was around the 7th, 8th and 9th months with 44 cases, centered in the east Marmara region. No causative agent could be identified. The rate of neurological complications and mortality was 21.4% and 4.2%, respectively. Eculizumab was used in four cases. Two of them had severe neurological complications despite plasma exchange. Elevated polymorphonuclear leukocyte count during hospital admission was the predictor of both severe disease and poor outcome. Duration of prodrome was the predictor of poor outcome (p<0.05). In conclusion, the median age of the affected children was greater than in the previous reports, while clinical features and outcome were similar.