Search

Background: Two earthquakes on 6 February 2023 destroyed 10 cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death., Method: Web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were recorded., Results: A total of 903 injured children (median age 11.62 years) were evaluated. Mean TUR was 13 h (interquartile range 32.5, max 240 h). Thirty-one of 32 patients with a TUR of >120 h survived. The patient who was rescued after 10 days survived. Two-thirds of the patients were given 50 mEq/L sodium bicarbonate in 0.45% sodium chloride solution on admission day. Fifty-eight percent of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% at 3000-4000 mL/m2 BSA and only 2% at >4000 mL/m2 BSA. A total of 425 patients had surgeries, and 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively. Crush-AKI developed in 314 patients (36% of all patients). In all, 189 patients were dialyzed. Age >15 years, creatine phosphokinase (CK) ≥20 950 U/L, TUR ≥10 h and the first-day IVF volume <3000-4000 mL/m2 BSA were associated with Crush-AKI development. Twenty-two deaths were recorded, 20 of 22 occurring in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived., Conclusions: These are the most extensive pediatric kidney disaster data obtained after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also associated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days., (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Background: On February 6th, 2023, two consecutive earthquakes struck southeastern Türkiye with magnitudes of 7.7 and 7.6, respectively. This study aimed to analyze the clinical and laboratory findings, as well as management of pediatric victims with Crush Syndrome (CS) and Acute Kidney Injury (AKI)., Methods: The study included pediatric earthquake victims who were presented to Mersin University Hospital. Clinical and laboratory characteristics of the patients were collected retrospectively., Results: Among 649 patients, Crush injury (CI), CS and AKI was observed in 157, 59, and 17 patients, respectively. White blood cell count (12,870 [IQR: 9910-18700] vs. 10,545 [IQR: 8355-14057] /µL, P < 0.001), C-reactive protein (51.27 [IQR: 14.80-88.78] vs. 4.59 [1.04-18.25] mg/L, P < 0.001) and myoglobin levels (443.00 [IQR: 198.5-1759.35] vs. 17 [11.8-30.43] ng/ml) were higher in patients with CS, while their sodium (IQR: 134 [131-137] vs. 136 [134-138] mEq/L, P < 0.001) levels were lower compared to non-CS patients. An increase in myoglobin levels was identified as an independent risk factor for developing CS (OR = 1.017 [1.006-1.027]). Intravenous fluid replacement was administered to the patients with CS at a dose of 4000 cc/m 2 /day. Hypokalemia was observed in 51.9% of the CS patients on the third day. All patients with AKI showed improvement and no deaths were reported., Conclusions: Hyponatremia and increase in inflammation markers associated with CS may be observed. An increase in myoglobin levels was identified as a risk factor for CS. Hypokalemia may be seen as a complication of vigorous fluid therapy during hospitalization., (© 2024. The Author(s).)

Background: Hemolytic uremic syndrome (HUS) is a serious cause of acute kidney injury in children. There is a suggestion that coronavirus disease 2019 (COVID-19) may be a trigger for HUS. In this study, we present a pediatric case diagnosed with HUS associated with COVID-19, which progressed to end-stage kidney disease., Case: A previously healthy 13-year-old girl with fever and vomiting was referred to our hospital. Laboratory investigations revealed direct Coombs-negative hemolytic anemia, thrombocytopenia and renal impairment accompanied by COVID-19 infection. Although anemia and thrombocytopenia showed improvement on the seventh day after admission, the renal impairment persisted. The histopathological findings of a renal biopsy were compatible with both HUS and COVID-19. One month later, the patient had a recurrence of HUS, again testing positive for COVID-19. Kidney function improved with plasma exchange therapy. Eculizumab treatment was recommenced after COVID-19 PCR became negative. Anemia and thrombocytopenia did not recur with eculizumab, while renal impairment persisted. Eculizumab was discontinued after three months when genetic analysis for HUS was negative. Subsequently, the patient was diagnosed with end-stage kidney disease., Conclusions: COVID-19 can be associated with HUS relapses, leading to chronic kidney disease. Further studies should investigate the mechanism of HUS associated with COVID-19., Competing Interests: The authors declare that there is no conflict of interest.

Background: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey., Methods: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey. Detailed patient data was collected which included demographic, clinical and laboratory features. Patients were classified according to their age and characteristics at presentation: patients presenting in the first year of life with nephrocalcinosis/nephrolithiasis (infantile oxalosis, Group 1), cases with recurrent nephrolithiasis diagnosed during childhood (childhood-onset PH1, Group 2), and asymptomatic children diagnosed with family screening (Group 3)., Results: Forty-eight patients had a mutation consistent with PH1. The most common mutation was c.971&#95;972delTG (25%). Infantile oxalosis patients had more advanced chronic kidney disease (CKD) or kidney failure necessitating dialysis (76.9% vs. 45.5%). These patients had much worse clinical course and mortality rates seemed to be higher (23.1% vs. 13.6%). Patients with fatal outcomes were the ones with significant comorbidities, especially with cardiovascular involvement. Patients in Group 3 were followed with better outcomes, with no kidney failure or mortality., Conclusion: PH1 is not an isolated kidney disease but a systemic disease. Family screening helps to preserve kidney function and prevent systemic complications. Despite all efforts made with traditional treatment methods including transplantation, our results show devastating outcomes or mortality., (© 2024 Asian Pacific Society of Nephrology.)

In pediatric patients with hemolytic uremic syndrome (HUS), cardiac involvement and autonomic nervous system function can be evaluated by a non-invasive method called heart rate variability (HRV). This study aims to evaluate heart rate variability and electrocardiography findings in patients with HUS by comparing a healthy group. Patients who are diagnosed with HUS at a university hospital from December 2020 to June 2022 are screened by electrocardiography (ECG), echocardiography, and 24-h Holter ECG. A healthy control group, compatible in age and gender with the patient group, was selected from healthy subjects. HRV parameters, laboratory values, and ECG findings were analyzed and compared with the healthy group and each other. There were 25 patients with HUS and 51 participants in the healthy control group. Statistically significant differences were found in some HRV parameters: standard deviation of normal to normal intervals, the mean of the 5-min RR interval standard deviations, the standard deviation of 5-min RR interval means, the triangular interpolation of normal to normal interval, and very-low-frequency power. HUS patients had impaired and declined HRV values compared to the healthy group. There was a significant decrease in the PR distance, while a significant increase in the corrected QT and QT dispersion values was detected in the electrocardiographic findings of the patient group. HRV values impaired as renal failure parameters increased.  Conclusion: Patients with HUS may have autonomic nervous system dysfunction. HRV measurement is a non-invasive method that can evaluate this. It can be thought that there may be an increased risk of cardiovascular events and arrhythmias in some patients with HUS. ECG should be also considered to detect arrhythmia. What is Known: • Hemolytic uremic syndrome (HUS) primarily effects the hematologic parameters and kidney. • Secondary cardiomyopathy with hypertension and renal failure could be observed in these patients. • Rhythm problems are not expected primarily in these patients. • There is very limited data in evaluating autonomic function and arrhythmia risk for these patients. What is New: • Patients with HUS may have autonomic nervous system dysfunction. • HRV measurement is a non-invasive method that can evaluate this. • Cardiovascular events and arrhythmias due to the deterioration of the balance between the sympathetic and parasympathetic systems could manifest in patients with HUS. • An ECG and screening patients for cardiac events, and monitoring them closely should be considered., (© 2024. The Author(s).)

Background: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey., Methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively., Results: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5-208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease., Discussion: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease.

Background: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors., Methods: Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated., Results: A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5-13.2) and 7.6 (IQR, 2.8-11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079-1.315, p = 0.001 and OR 1.580; 95% CI 0.660-0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%., Conclusions: Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely.

Background: Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone., Methods: The outcomes of seven genetically confirmed aHUS patients (2 girls, 5 males) were evaluated by means of clinical presentation, response to plasma therapy, course of the disease during the follow-up period and last status., Results: The median age of the patients at admission was 6.7 years (IQR 0.7-7.8). Three patients received plasma exchange therapy and the other four patients were treated with plasma infusions. One patient was lost to follow-up after one year; the median duration of follow-up for other patients was 3.7 years (IQR 2.7-6.5). During the follow up, two patients from our historical records when complement blocking therapies had not been in clinical use yet in Turkey, underwent kidney transplantation. One transplant patient experienced an acute rejection episode without graft loss. The remaining five patients had a glomerular filtration rate of more than 90 ml/min./1.73 m < sup > 2 < /sup > at the last visit., Conclusion: Although we had a relatively small patient population, our findings indicate that PT might still be considered in selected patients particularly in countries where complement blocking therapies are difficult to reach due to their unavailability or costs that are not covered by the health care systems.

Introduction: Cystinuria is an autosomal recessive disorder due to intestinal and renal transport defects in cystine and dibasic amino acids, which result in recurrent urolithiasis and surgical interventions. This study aimed to assess the impact of surgical interventions on renal function by analyzing estimated glomerular filtration rates., Methods: Thirteen pediatric patients with cystinuria, who were followed-up in a single tertiary institution between 2004 and 2016, were included in the study. Medical records were reviewed to collect data on clinical presentation of patients, urine parameters, stone formation, medical treatment, surgical intervention, stone recurrence after surgical procedure, stone analysis, ultrasonography, 99m-technetium dimercaptosuccinic acid (99mTc-DMSA) radionuclide imaging results, and follow-up time. Creatinine clearances estimated by modified Schwartz (eGFR) formula before and after surgery were used to assess renal function and compared statistically., Results: Nine patients (69.2%) had renal scarring which were detected with 99mTc-DMSA radionuclide imaging. In ten patients (76.9%), open surgical intervention for stones were needed during follow-up. Significant difference was not detected between eGFR before and after surgical intervention (mean 92 versus 106, p = 0.36). Nine of the patients (69.2%) were stone free in the last ultrasonographic examination. Relapses of stone after surgery were seen in 66.6% of patients who underwent surgical intervention., Conclusions: Surgical interventions for urinary stones are commonly required in patients with cystinuria. Renal scarring is a prevalent finding in cystinuric patients. Surgical interventions have no negative impact on eGFR in patients with cystinuria according to the present study.

Sürmeli-Döven S, Delibaş A, Gürses İ, Kayacan UR, Coşkun-Yılmaz B, Esen K, Korkmaz E, Özaltın F. Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not? Turk J Pediatr 2018; 60: 81-85. A previously healthy 18-month old boy, presenting with diarrhea, anemia, thrombocytopenia and acute renal failure was admitted to our hospital. Hemolytic uremic syndrome (HUS) was diagnosed with his clinical and laboratory findings. His stool was negative for Shiga toxin producing E. coli (STEC). During follow-up he developed respiratory distress, hypertrophic cardiomyopathy and seizure. His genetic tests for atypical HUS (aHUS) were negative. His clinical and histological findings indicated hemolytic uremic syndrome and immunglobulin A nephropathy (IgAN). The patient responded to steroid treatment and plasma exchange therapy with peritoneal dialysis. We discuss the probable connection between HUS and IgAN.

Background: Cystinosis is a rare metabolic genetic disorder caused by a mutation in cystinosin lysosomal cystine transporter (CTNS). The diagnosis of nephropathic cystinosis (NC) is made by observing corneal cystine crystals and/or measuring the cystine content of leukocytes. CTNS mutation analysis confirms the diagnosis of cystinosis, but leukocyte cystine measurement and CTNS analysis have not been widely available, and cystine crystals in the cornea may not be apparent in the first months of life. Cystine crystal deposition can be seen in the bone marrow earlier than corneal deposition, in patients with NC., Methods: Ten patients with cystinosis diagnosis were enrolled in the study. Medical records were reviewed retrospectively to collect demographic and clinical data such as age at diagnosis, disease presentation, parental consanguinity, family history, corneal cystine deposition, leukocyte cystine level, bone marrow cystine deposition, presence of renal failure, follow-up time and prognosis., Results: Cystine crystals were seen in all of the patients' fresh bone marrow aspiration samples. Eight patients had corneal cystine deposition. Leukocyte cystine measurement could have been performed in four patients who had come from another center. Complications such as pulmonary hypertension and idiopathic intracranial hypertension (IIH) were observed in two patients., Conclusions: Bone marrow aspiration might be an easy and short-cut diagnostic tool for NC especially when it is not possible to measure fibroblast cystine content. Additionally some rare complications such as pulmonary hypertension and IIH can be encountered during the course of NC., (© 2017 Japan Pediatric Society.)

We present a 7-year-old patient who was diagnosed with asymptomatic nephropathic cystinosis following the detection of the pathognomonic corneal white crystalline opacities during a routine eye examination., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Objective: The majority of antenatal hydronephrosis disappears spontaneously. In our study, we have investigated the effective predictors for surgical decision in antenatal hydronephrosis., Material and Methods: Forty-four patients found to have renal pelvic anteroposterior (AP) diameter more than 10-mm on antenatal ultrasonograpy were followed after birth. The study groups were divided into two groups: follow-up and surgery group. On follow-up, longitudinal diameter of the kidney, parenchymal thickness of the kidney, AP diameter of renal pelvis, AP diameter of middle calyces on ultrasonograpy, and differential renal function, 20th minute clearance, half-life of radionuclide tracer (T1/2), Tmax, Tmax-T1/2, normalised residual activity (NORA) on diuretic renography were evaluated. Ultrasonograpy and diuretic renography measurements were compared in patients whose hydronephrosis resolve or proceeded to surgery., Results: Forty-four patients were diagnosed as antenatal hydronephrosis, spontaneous resolution occurred in 23 (52%), and surgery was performed in 21 patients (48%). Mean age at operation was 8.5±6.5 months (3-24 months). Mean AP diameter of renal pelvis was 29.5±14.2 mm for surgery group, 13.6±4.2 mm for follow-up group (p<0.001). Univariate analyses showed significance for all ultrasonographic measurements and only the differential renal function by diuretic renography. Multivariate logistic regression analysis showed significance for AP diameter of renal pelvis (odds ratio 1.37; 95% Cl 1.13-1.66), and differential renal function (odds ratio 1.14; 95% Cl 1.01-1.29)., Conclusion: AP diameter of renal pelvis and differential renal function were the most effective parameters for surgical decision. These parameters can be used for appropriate management of antenatal hydronephrosis., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Purpose: Subureteral injection of bulking agents in the endoscopic treatment of vesicoureteral reflux is widely accepted therapy with high success rates. Although the grade of vesicoureteric reflux and experience of surgeon is the mainstay of this success, the characteristics of augmenting substances may have an effect particularly in the long term. In this retrospective study, we aimed to evaluate the clinical outcomes of the endoscopic treatment of vesicoureteric reflux (VUR) with two different bulking agents: Dextranomer/hyaluronic acid copolymer (Dx/HA) and Polyacrylate polyalcohol copolymer (PPC)., Materials and Methods: A total 80 patients (49 girls and 31 boys) aged 1-12 years (mean age 5.3 years) underwent endoscopic subureteral injection for correction of VUR last six years. The patients were assigned to two groups: subureteral injections of Dx/HA (45 patients and 57 ureters) and PPC (35 patients and 45 ureters). VUR was grade II in 27 ureters, grade III in 35, grade IV in 22 and grade V in 18 ureters., Results: VUR was resolved in 38 (66.6%) of 57 ureters and this equates to VUR correction in 33 (73.3%) of the 45 patients in Dx/HA group. In PPC group, overall success rate was 88.8% (of 40 in 45 ureters). Thus, Thus, this equates to VUR correction in 31 (88.5%) of the 35 patients., Conclusions: Our short term data show that two different bulking agent injections provide a high level of reflux resolution and this study revealed that success rate of PPC was significantly higher than Dx/HA with less material.

Although lower urinary tract dysfunction (LUTD) in patients with cerebral palsy (CP) has been previously documented by clinical observations and urodynamic tests, its correlation with upper urinary tract deterioration (UUTD) has not been demonstrated. This paper documents symptoms and urodynamic findings of LUTD and their relationship with UUTD in 33 children with CP. By sonography, 4 of these children were found to have UUTD. Age was found to correlate with UUTD, but gender difference and mental or motor functions did not. When comparing urinary symptoms with UUTD, incontinence (n = 31) did not correlate, but on the other hand symptoms of detrusor sphincter dyssynergia (interrupted voiding, urinary retention, hesitancy; n = 5) and culture proven febrile urinary tract infections (n = 4) did. Abnormal urodynamics findings were not diagnostic. We conclude that, apart from incontinence, dysfunctional voiding symptoms and febrile urinary tract infections are valuable indicators of UUTD., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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.

Objective: To determine the relationship between vesicoureteric reflux (VUR), urinary tract infection (UTI), renal damage and the pattern of non-neurogenic lower urinary tract dysfunction (LUTD), and to reveal the possible risk factors for renal damage in children with LUTD., Methods: For the years 2004-2010, demographic, clinical, laboratory and urodynamic study reports of children with LUTD were retrospectively reviewed., Results: Of 96 patients, there were diagnosed 70 with overactive bladder (OAB), 8 pure dysfunctional voiding (DV) and 18 OAB plus DV. The rate of VUR, UTI and renal damage in patients with OAB plus DV and pure DV was higher than in patients with OAB alone. VUR was significantly higher among the patients who had UTI. Renal scarring was detected in 25 patients, of whom 78% had OAB plus DV and 75% DV. The presence of VUR was associated with a significant increase in the rate of renal damage, and dilating reflux caused significantly greater damage compared to non-dilating reflux., Conclusion: OAB plus DV and DV are major risk factors for VUR, UTI and renal damage. The presence of VUR in children with LUTD plays an important role with regard to UTI and renal damage, with dilating VUR a major risk factor associated with renal damage., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: To report our experience with the pyeloureterostomy (PU) for the treatment of the lower pole PUJO in incomplete duplex systems. The combination of the duplicated collecting system and pelviureteric junction obstruction (PUJO) is a rare association and infrequently reported. Surgical treatment can be challenging in such cases., Methods: We retrospectively reviewed the medical data of the patients who had surgery from 2001 to 2009, with a diagnosis of PUJO of the lower pole moiety in incomplete duplex system. Demographic, diagnostic, and procedural data were recorded., Results: Seven patients were identified with the lower pole PUJO associated with incomplete duplex systems. Their median age was 49 months (range 2-108 months). Prenatal hydronephrosis was detected in 3 patients, and 4 had a febrile urinary tract infection. PU was performed in 6 patients because of short ureteral length between the ureteropelvic junction and junction of lower and upper pole ureters. One patient was treated with the dismembered pyeloplasty because of sufficient ureteral length of the lower pole. No complications were detected during 14 months of follow-up., Conclusions: The management of the lower pole PUJO in incomplete duplex systems should be individualized for every patient. PU is a good surgical option in the management of the lower pole PUJO associated with incomplete ureteral duplication., (Copyright © 2010 Elsevier Inc. All rights reserved.)

In this study we evaluated the indications, complications, and the spectrum of histopathological results of percutaneous renal needle biopsy (PRNB) performed in our clinic. Between June 1990 and December 2006, 679 PRNBs were performed on native kidneys of 614 children (304 boys, 310 girls) with a mean age of 10.4 years. Most frequent indications for PRNB were nephrotic syndrome (47%), hematuria, and/or proteinuria (15.9%), acute renal failure (14.6%) and complex renal manifestations (18.9%). The overall complication rate was 15.2%. The most common complications were perirenal hematoma (12.4%) and macroscopic hematuria (2.6%). The most frequent histopathological group of diseases were glomerulopathies; these were diagnosed in 376 patients (61.2%) and included membranoproliferative glomerulonephritis (11.1%), mesangial proliferation (10.7%), diffuse proliferative glomerulonephritis (7.7%), and focal segmental glomerulosclerosis (7.3%) as the most frequent. The second most frequent group of histopathology was manifestations secondary to systemic diseases; these were shown in 195 patients (31.8%). Amyloidosis (11.4%) and Henoch-Schönlein nephritis (9.9%) made the majority of this group. In conclusion, our study demonstrated that PRNB is a safe procedure with usually transient complications showing the most frequent renal diseases that cause diagnostic and therapeutic difficulties for pediatric nephrologists.

A new chelating resin, poly[N-(4-bromophenyl)-2-methacrylamide-co-2-acrylamido-2-methyl-1-propanesulfonic acid-co-divinylbenzene], was synthesized and characterized. The resin was used for selective separation, preconcentration and determination of Cu(II), Ni(II), Co(II), Cd(II), Pb(II), Mn(II) and Fe(III) ions in water samples by flame atomic absorption spectrometry. Effects of pH, concentration and volume of elution solution, sample flow rate, sample volume and interfering ions (Na(+), K(+), Ca(2+), Mg(2+), Fe(3+), Mn(2+), Al(3+), Zn(2+), Pb(2+), Cu(2+), Ni(2+), Cd(2+), Cl(-) and SO(4)(2-)) on the recovery of the analytes were investigated. The sorption capacity of the resin was 25.6, 19.8, 32.1, 41.3, 38.9, 13.9 and 18.3 mg g(-1) for Cu(II), Ni(II), Co(II), Cd(II), Pb(II), Mn(II) and Fe(III), respectively. A high preconcentration factor, 100, and low relative standard deviation,

A new solid phase extraction method for the speciation, separation and preconcentration of Cr(III) and Cr(VI) was developed. As solid phase material, a new chelating resin, poly N-(4-bromophenyl)-2-methacrylamide-co-2-acrylamido-2-methyl-1-propanesulfonic acid-co-divinylbenzene was synthesized. Cr(III) was separated from Cr(VI) and preconcentrated by using a column containing chelating resin. Total chromium was determined after the reduction of Cr(VI) to Cr(III) by hydroxylamine hydrochloride as a reducing agent. Then Cr(VI) was calculated by substracting Cr(III) concentration from the total chromium concentration. The parameters such as the effect of pH, eluent type, volume and concentration, flow rate of sample solution, sample volume and effect of interfering ions for the preconcentration of Cr(III) were investigated. The optimum pH was found to be 2. Eluent for quantitative elution was 10 mL of 1 mol L(-1) HCl. The preconcentration factor of the method was 100. At the optimum conditions determined experimentally, the recovery for Cr(III) was found to be 95 +/- 3%. The limit of detection (3s/b) of the method was 1.58 microg L(-1). In order to determine the adsorption behaviour of chelating resin, the adsorption isotherm of Cr(III) was studied. Adsorption capacity and binding equilibrium constant were calculated to be 21.8 mg g(-1) and 1.32 x 10(-2) L mg(-1), respectively. The method was validated by analysing certified reference material (TMDA-54.4 a trace element fortified calibration standard). The proposed method was applied to the determination and speciation of chromium in tap water, lake water, spring water and wastewater samples with satisfactory results.

Rhabdomyolysis induced acute renal failure (ARF) is relatively rare in children. We report an 8-year-old boy with McArdle disease and rhabdomyolysis induced ARF after heavy muscle work. Physical examination revealed generalized tenderness on his extremities. Laboratory examinations showed acute renal failure due to myoglobinuria and revealed alanine transaminase 428 U/l, aspartate transaminase 1,400 U/l, blood urea nitrogen 119 mg/dl, creatinin 3.6 mg/dl, uric acid 13 mg/dl, and serum creatinine kinase (CK) 33,766 U/l. Hemodialysis was carried out for ARF. His clinical and laboratory findings improved and became normal in 2 weeks. Enzymatic analysis of the muscle biopsy showed a phosphorylase A level of 129 nmol/s/mg protein (normal: 200-600) and a phosphorylase A+B level of 385 nmol/s/mg protein (normal: 500-1500), which was compatible with glycogenosis type V. As McArdle disease rarely becomes symptomatic and ARF secondary to this condition is very rare, our case represents a rare clinical presentation.

In this study, we evaluated the frequency, clinical presentation, treatment protocols, prognostic factors, and outcome in children with diffuse proliferative lupus nephritis (DPLN). Between June 1990 and December 2004, 46 patients were diagnosed to have systemic lupus erythematosus (SLE), and 26 of them (56.5%) were found to have DPLN. Renal manifestations were present in 25 patients, and the majority of them presented with severe renal findings, such as nephrotic syndrome and renal failure. All patients were given a quadruple therapy protocol including 6-12 monthly courses of methyl prednisolone pulse therapy combined with oral prednisolone, oral cyclophosphamide, azathioprine, and dipyridamole. Nineteen of these patients were regularly followed up with a mean follow-up period of 5.9 years. Complete remission was achieved in 15 of 19 patients, and chronic renal failure developed in four patients. Renal survival rate was calculated to be 78.9% at the end of 5, 10, and 14 years. Although nephrotic range proteinuria, hypoalbuminemia, renal failure, and activity index above 12/24 at presentation seemed to be associated with poor prognosis, no significant difference could be found. Hypertension and chronicity index greater than 6/12 were found to be bad prognostic predictors. We concluded that satisfactory results were achieved with our quadruple therapy protocol; thus, more aggressive and expensive therapies can be avoided and preserved for more serious and persistent diseases.

Background/aims: Evaluation of the risk factors, and phenotype-genotype correlation of familial Mediterranean fever (FMF) gene (MEFV) and serum amyloid A1 (SAA1) gene polymorphisms in renal amyloidosis., Methods: We investigated MEFV and SAA1 genotypes (alpha, beta, and gamma isoforms) in 50 FMF patients and 50 healthy children. Tel-Hashomer criteria were used for the diagnosis and severity scoring of FMF., Results: The most common MEFV mutation and SAA1 genotype were M694V/M694V (n = 26/50) and SAA1 alpha/alpha (n = 26/50), respectively. Positive family history for amyloidosis was significantly higher (p < 0.001) with more severe clinical course (p = 0.006) in the amyloidosis group than the non-amyloid group. In M694V/M694V mutation, erysipelas-like skin erythema (p = 0.029), arthritis (p = 0.004), arthralgia (p < 0.001) were significantly more frequent with higher severity scores (p = 0.008) than the patients with other mutations. Comparison of the SAA1 alpha/alpha genotype with other genotypes revealed more frequent arthritis (p = 0.003) in the SAA1 alpha/alpha genotype. In amyloidosis group patients having both M694V/M694V and SAA1 alpha/alpha genotypes were the largest subgroup (n = 14, p < 0.001). Logistic regression analysis for amyloidosis corrected risk revealed a 1.2 times increase in M694V/M694V, a 2.4 times increase in SAA1 alpha/alpha genotypes and a 2.5 times increase when both are together., Conclusion: Positive family history for amyloidosis and presence of SAA1 alpha/alpha genotype in M694V/M694V mutation may predispose to amyloidosis by increasing the clinical severity. Therefore, in such children early colchicine treatment might be recommended even if they are asymptomatic., (Copyright (c) 2005 S. Karger AG, Basel.)

In nephrotic syndrome there is an increased tendency for bacterial infections due to immunological changes secondary to proteinuria, treatment (including steroids), and other as yet unknown causes. However, necrotizing fasciitis (NF) is an uncommon complication of the disease and has rarely been reported in nephrotic children. We report a 14-month-old boy with nephrotic syndrome who developed sepsis and NF as a complication. He was treated successfully with intensive medical and surgical treatment.