Natural coagulation inhibitors (protein C, protein S, antithrombin) in patients with sickle cell anemia in a steady state

PubMedID: 11737735Background: Patients with sickle cell anemia (SCA) run the risk of having decreased levels of natural coagulation inhibitors. This may be due to either hemostatic abnormalities or hepatic dysfunction. This study is designed to evaluate coagulation profiles of patients with SCA in a steady state and to determine whether hypercoagulable state is present or not. Methods: Seventeen children with SCA in a steady state were included in this study. The routine hematological evaluation was done with a coulter-counter. Reticulocyte percentage and blood coagulation tests were also determined. The coagulation inhibitors such as protein C (as activated partial thromboplastine time prolongation time), protein S (as Factor V inhibition) and antithrombin (colorimetric assay) were measured in all cases. Results: In the coagulation profile, mean euglobuline lysis time and mean fibrin degradation product levels were both significantly higher in the patient group than in the control group (P < 0.05), although other parameters were within normal limits. The values for aspartate aminotransferase (AST), alanine aminotransferase (ALT) and indirect reacting bilirubin were significantly higher in the patient group than in the control group (P < 0.001, P < 0.005, P < 0.0001, respectively). The serum protein levels were normal. Mean factor V level was significantly lower and mean factor VIII level was found significantly higher in the patient group than in the control group (P < 0.05 and P < 0.005, respectively). Protein C and AT levels were lower in patients with SCA than in control subjects (P < 0.001). Protein S levels were also lower in the patient group than in the control group, but the difference between the two groups was not significant (P < 0.05). Conclusion: It is indicated that antithrombotic functions of patients with SCA are handicapped even in a steady state; and both hemostatic abnormalities and hepatic dysfunction contribute to low levels of natural coagulation inhibitors

Retroperitoneoscopic nephroureterectomy via three trocars in pediatric patients with end-stage reflux nephropathy

PubMedID: 20879861Background and Purpose: In children with end-stage reflux nephropathy, nephroureterectomy can be performed either open or laparoscopically. It is not common to use a three-trocar retroperitoneal approach for nephroureterectomy with complete ureteral excision in the pediatric age group. We report the results of pediatric retroperitoneoscopic nephroureterectomies by using three trocars only. Patients and Methods: Between August 2003 and November 2009, 13 children-seven boys and six girls-with end-stage reflux nephropathy underwent retroperitoneoscopic nephroureterectomy. The operations were performed by using three trocars with the patient in a flank position under general anesthesia. Renal blood vessels were individually dissected and divided followed by the transection of the ureter at the level of the ureterovesical junction. Results: The mean age of the patients was 6.5±4.4 years (15 months-14 years). Eight of the operations were performed on the left and five on the right side. Mean operative time was 137±47 minutes (75-230min). Estimated blood losses were minimal. Mean hospitalization time was 2.2±0.9 days (2-5 d). No major intraoperative complication was seen. Overall postoperative analgesic requirement was minimal. Cosmetic results were excellent at the initial postoperative visit. Conclusion: Retroperitoneoscopic nephroureterectomy by using three trocars is feasible and safe with excellent outcomes in pediatric patients with end-stage reflux nephropathy. Copyright 2010, Mary Ann Liebert, Inc

Comparison of mycophenolate mofetil and azathioprine in obstructive nephropathy

PubMedID: 12579396The effect of mycophenolate mofetil (MMF) and azathioprine (AZA) in reducing renal interstitial fibrosis in rats with unilateral ureteral obstruction (UUO) was studied. Fifty-nine rats with surgically induced UUO received oral MMF (n=19), AZA (n=19), or no treatment (n=21). The obstructed kidneys were analyzed by histology and morphometry on days 21 and 60 post UUO. Fibronectin- and collagen-stained areas were significantly lower in both treatment groups when compared with the control group 21 days post surgery. Transforming growth factor (TGF)-ß expression was significantly lower in the MMF-treated group than in the AZA-treated (P0.05). No significant difference was found in TGF-ß and fibronectin expression between treated and untreated groups on the 60th day post surgery. However, collagen expression was significantly lower in both treated groups than in the untreated group on the 60th day (P<0.005). We observed that MMF is more effective in preventing fibrosis than AZA in the UUO model in the short term; however, there is a less significant anti-fibrotic effect of these drugs in long-term than in short-term obstruction

Interleukin-18, CRP and procalcitonin levels in vesicoureteral reflux and reflux nephropathy

PubMedID: 23957657Background: Some patients with vesicoureteral reflux (VUR) develop reflux nephropathy (RN) and a number of them progress to chronic kidney disease (CKD). However, it is unclear to predict which patient will develop RN and/or CKD. The aim of this study is to evaluate the role of Interleukin-18 (IL-18), C-reactive protein (CRP) and procalcitonin (PCT) as an indicator of RN in VUR. Methods: Ninety-three children aged 3.5-16 years with primary VUR were enrolled. Patients were divided into two groups according to the presence of renal scarring (RS). CRP, PCT, blood urea nitrogen (BUN), serum creatinine (Scr), urinary protein (Up), creatinine (Ucr) and microalbumin (Umalb), serum and urine IL-18 levels were determined during urinary tract infection (UTI) free episode. Results: BUN, Scr, Up/Ucr and Umalb/Ucr concentrations were higher whereas calculated creatinine clearance (Ccr) values were lower in RS (+) group compared to RS (-) group. CRP, PCT, serum and urine IL-18 levels and mean urine IL-18/Cr concentrations were similar in both groups. Serum and urine IL-18 levels did not differ according to the grade of VUR. No significant correlation was found between CRP, PCT and IL-18. Conclusions: Proteinuria and microalbuminuria are valuable hallmarks of RN. CRP and PCT seem not to be reliable indicators of RN in VUR patients. Moreover, serum and urine IL-18 might not predict RN. © 2013 Informa Healthcare USA, Inc

Ambulatory blood pressure monitoring in children with vesicoureteral reflux

PubMedID: 24365089Objective To assess the value of ambulatory blood pressure monitoring (ABPM) for identifying the risk of hypertension (HT) in children with vesicoureteral reflux (VUR). Methods Seventy-six children with primary VUR were enrolled. Patients were divided into 2 groups according to renal scarring (RS). Serum creatinine, urine protein, and urine creatinine were measured. All patients underwent ABPM and casual blood pressure (BP) examination. ABPM measurements were standardized to age, gender, and height by Lambda-Mu-Sigma method. Results Urine protein and creatinine concentrations were statistically higher in RS (+) group than RS (-) group (P =.05). Casual systolic and diastolic BP standard deviation score (SDS) values were not statistically different between RS (+) and RS (-) groups. Day, night and 24-hour systolic BP SDS, day and 24-hour mean arterial blood pressure SDS values were significantly higher in RS (+) group than that of RS (-) group (P =.015, P =.031, P =.013, P =.07, and P =.021, respectively). All ABPM values significantly increased in patients with severe RS compared with ones with mild and moderate RS (P .05). Conclusion ABPM is more sensitive than casual BP measurements and might be used for early identification of HT in VUR patients. The severity of RS increases the risk of HT in children with VUR. Controlling HT with proteinuria would decrease the progression of renal damage in VUR. © 2014 Elsevier Inc. All Rights Reserved

Assessment of cystatin C and cystatin C-based GFR formulas in reflux nephropathy

PubMedID: 24128877Objective: Early identification of reflux nephropathy (RN) could reduce the frequency of chronic kidney disease (CKD) caused by vesicoureteral reflux (VUR). We aimed to assess whether cystatin C has value for determining RN in children with VUR. Materials and methods: Ninety-three children with VUR were classified into two groups according to the presence of renal parenchymal scarring (RS). Patients with RS were divided into three subgroups according to scar grade. Serum cystatin C, serum creatinine (Scr) and urine creatinine were measured. eGFR values of the patients were calculated with Scr-based, cystatin C-based and combined formulas. Results: Cystatin C was significantly higher in patients with RS than patients without RS and declined in parallel with grade of RS (p = 0.01). Scr was not significant in patients with and without RS. It was only significant between mild and severe scar subgroups (p < 0.05). All eGFR values were lower in RS (+) patients compared with RS (-) patients. All eGFR equations were negatively correlated with grade of RS (p < 0.05). Conclusion: Cystatin C could be a useful marker for identifying the risk and severity of RN in patients with VUR. Renal functions could be more accurately determined with Scr-cystatin C combined eGFR equations. © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved

Percutaneous nephrolithotomy in the management of pediatric renal calculi

PubMedID: 11962558Background and Purposes: In the era of extracorporeal shockwave lithotripsy (SWL), there are still some patients who will require percutaneous nephrolithotomy (PCNL). Our experience with this procedure is reviewed and discussed. Patients and Methods: Fifty five patients with a mean age of 7.9 years (10 months-14 years) underwent 67 PCNL procedures on 62 renal units between September 1997 and April 2001. Of the patients, 13 had previous open renal surgery, 4 had a solitary kidney, 4 were SWL failures, 2 had osteogenesis imperfecta, one had anuria secondary to bilateral calculi, 2 had poorly functioning kidneys, and I had cystinuria (complete staghorn calculus). One disabled patient with neurologic disorders who had multiple stones underwent PCNL in order to decrease the stone burden and to improve the kidney function. Operations were performed under the guidance of monoplane fluoroscopy. Pneumatic or ultrasonic lithotripsy and forceps extraction were used with a rigid nephroscope or ureteroscope (as an alternative instrument in small-caliber tracts). Results: Excluding the patient with neurologic disorders, 53 of the renal units (86.9%) were stone free at the time of discharge, and the success rate was 96.7%, with six patients having insignificant residual fragments after the procedure. In one patient, open surgery was required, and the other patient was sent for SWL treatment. There was no contiguous organ injury, but in 16 procedures (23.9%), intraoperative hemorrhage was seen, and blood transfusions were required. Conclusion: Pediatric urolithiasis is usually a result of metabolic abnormalities and urinary tract infection, and there is always a risk of recurrence that may necessitate multiple additional intervention. Therefore, PCNL must be considered in selected cases by urologists who are experienced in adult percutaneous procedures

Urinary nephrocalcin excretion in children with urolithiasis

PubMedID: 12972707The aim of this study was to investigate the role of nephrocalcin in childhood urolithiasis. Forty-one patients with urinary stones and 25 age- and sex-matched healthy controls were admitted to the study. Blood and timed urine samples were taken from both patient and control groups for biochemical analysis. Serum and urine creatinine (Cr) and urinary nephrocalcin (NC) were measured. NC excretion was expressed as a NC/Cr (mg/g) ratio. NC-PreA/Cr and NC-D/Cr ratios were found to be significantly higher in patients than in the control group. No statistically significant differences were found in NC-A/Cr, NC-B/Cr, NC-C/Cr ratios between the patient and control groups. The high NC-PreA/Cr ratio (p = 0.012) observed in stone-forming patients indicates that this ratio may also be an important stimulatory factor for urinary stone disease. Copyright © 2003 S. Karger AG, Basel

An infant with hyponatremia, hyperkalemia, and metabolic acidosis associated with urinary tract infection: Answers

PubMedID: 31053900[No abstract available

Labial adhesion and urinary tract problems: The importance of genital examination

PubMedID: 26590736Background Urinary tract infection (UTI) is a common bacterial illness in children. Delay in the treatment of UTI may lead to acute renal parenchymal damage and subsequent renal scarring. It is well established that several risk factors increase the tendency for UTI - one being labial adhesion (LA). Objective The purpose of this study was to emphasize the importance of genital examination in girls with nephrourologic symptoms, particularly UTIs, in order to detect LA. Material and methods Data were collected from the files of 46 girls with LA, including: the girl's age, thickness of LA, any recurrence and treatment options of LA, and the reason for admission to hospital. The LAs were grouped in terms of thickness as thin, moderate and dense, and also partial or complete. Results The average age of the girls at the first visit was 51.9 ± 37.57 months (min-max: 3.5-157 months). Twenty-seven (58.7%) of the girls had history of recurrent UTI. There was a marked association between the presence of UTI and the type of adhesions. The percentages of UTIs in girls with complete and partial LA were 84.0% and 28.6%, respectively (P &lt; 0.05). The percentages of UTIs in girls with thick and thin LA were 100% and 44.1%, respectively (P &lt; 0.05). None of the girls' primary care physicians or pediatricians recognized LA at the time of a periodic health examination. Discussion In the present study, girls with complete and thick LA had a greater tendency towards having UTIs than those with partial and thin LA, respectively. After treatment of LA, the UTIs did not recur in any girls. None of the girls in this study had undergone a previous genital examination. Therefore, this study suggests that physicians do not often perform genital examinations. Limitations of this study were the small sample size and the short follow-up period. In addition, although all of the girls were examined by the same physician, the thickness of the membrane is subjective and solely depends on the physician's experience. Conclusions This study showed that although genital examination is a routine part of a physical examination, it is not always performed. Therefore, it is recommend that genital examination should be performed in girls with nephrourologic complaints, particularly for UTI. By timeously determining the presence of LA, many unnecessary and invasive investigations could be avoided in these children. Reason for admission to the Pediatric Nephrology department. Reason for admissionNumber of patients% of patientsUTIa27a58.7Hematuria24.3Hydronephrosisb7b15.2Urinary frequency24.3Urinary incontinence12.2Pseudoincontinence12.2Abdominal and back pain12.2Others510.9Total46100.0Five patients with UTI had additional complaints (hematuria, antenatal hydronephrosis, urinary incontinence, stains on underclothes, and recurrent convulsions).Five patients with hydronephrosis had the diagnosis of antenatal hydronephrosis. © 2016 Journal of Pediatric Urology Company