The use of cinacalcet after pediatric renal transplantation: an international CERTAIN Registry analysis.

BACKGROUND: Secondary hyperparathyroidism (SHPT) may persist after renal transplantation (RTx), inducing hypophosphatemia and hypercalcemia that precludes the use of vitamin D analogs. The calcimimetic cinacalcet improved plasma calcium and parathyroid hormone (PTH) levels in randomized controlled trials in adults after RTx, but pediatric data are scarce. METHODS: In this retrospective study, we analyzed 20 pediatric patients from the Cooperative European Paediatric Renal TransplAnt Initiative (CERTAIN) Registry who received cinacalcet after RTx. The results are presented as median and interquartile range (25th-75th percentile). RESULTS: At 13.7 (11.0-16.5) years of age, 20 pediatric patients received a renal allograft. Cinacalcet was introduced at 0.4 (0.3-2.7) years post-transplant at an estimated glomerular filtration rate (eGFR) of 50 (34-66) mL/min/1.73 m2, plasma calcium of 2.58 (2.39-2.71) mmol/L, age-standardized (z score) phosphate of - 1.7 (- 2.7-- 0.4), and PTH of 136 (95-236) ng/L. The starting dose of cinacalcet was 0.5 (0.3-0.8) mg/kg per day, with a maximum dose of 1.1 (0.5-1.3) mg/kg per day. With a follow-up of 3.0 (1.5-3.6) years on cinacalcet therapy, eGFR remained stable; PTH levels decreased to 66 (56-124) ng/L at the last follow-up (p = 0.015). One patient displayed hypocalcemia (1.8 mmol/L). Cinacalcet was withdrawn in three patients (hypocalcemia, parathyroidectomy, incompliance). Nephrocalcinosis of the graft was not reported. CONCLUSIONS: This pilot study suggests that cinacalcet as off-label therapy for SHPT after pediatric RTx is efficacious in controlling post-transplant SHPT with acceptable tolerability. Continuing cinacalcet even with normal PTH can lead to dangerous life-threatening hypocalcemia. Therefore, at each subsequent visit, the need to continue cinacalcet must be assessed.

Sex and age as determinants for high blood pressure in pediatric renal transplant recipients: a longitudinal analysis of the CERTAIN Registry.

BACKGROUND: High prevalence of arterial hypertension is known in pediatric renal transplant patients, but how blood pressure (BP) distribution and control differ between age groups and whether sex and age interact and potentially impact BP after transplantation have not been investigated. METHODS: This retrospective analysis included 336 pediatric renal transplant recipients (62% males) from the Cooperative European Pediatric Renal Transplant Initiative Registry (CERTAIN) with complete BP measurement at discharge and 1, 2 and 3 years post-transplant. RESULTS: At discharge and 3 years post-transplant, arterial hypertension was highly prevalent (84% and 77%); antihypertensive drugs were used in 73% and 68% of the patients. 27% suffered from uncontrolled and 9% from untreated hypertension at 3 years post-transplant. Children transplanted at age < 5 years showed sustained high systolic BP z-score and received consistently less antihypertensive treatment over time. Younger age, shorter time since transplantation, male sex, higher body mass index (BMI), high cyclosporine A (CSA) trough levels, and a primary renal disease other than congenital anomalies of the kidney and urinary tract (CAKUT) were significantly associated with higher systolic BP z-score. Sex-stratified analysis revealed a significant association between high CSA and higher systolic BP in older girls that likely had started puberty already. An association between BP and estimated glomerular filtration rate was not detected. CONCLUSIONS: BP control during the first 3 years was poor in this large European cohort. The description of age- and sex-specific risk profiles identified certain recipient groups that may benefit from more frequent BP monitoring (i.e. young children) or different choices of immunosuppression (i.e. older girls).

Nutcracker Syndrome in Children: Role of Doppler Ultrasonographic Indices in Detecting the Pattern of Symptoms.

The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome (NCS). We also investigated the left renal vein Doppler ultrasonography (DUS) results, to determine whether or not there was an association between clinical symptoms and DUS findings among these patients. The clinical data from 44 pediatric patients who were diagnosed as having NCS from January 2008 to December 2015 were retrospectively reviewed. We grouped the patients according to the presenting symptoms as symptomatic (loin pain; macroscopic hematuria or both) and non-symptomatic (microscopic hematuria and proteinuria were detected incidentally) and evaluated the left renal vein DUS indices in these two groups separately. Asymptomatic NCS was found in 27 (61.4%) patients; 21 (47.7%) of whom were admitted for the evaluation of proteinuria. The most frequent presenting symptoms were left flank pain (20.5%) and macroscopic hematuria (13.6%); and 2 (4.5%) patients presented with a combination of left flank pain and macroscopic hematuria. The mean ratio of the diameter of the hilar portion of the left renal vein (LRV) to that of the aortomesenteric portion was 4.36 ± 1.55. The mean ratio of the peak velocity (PV) between the two sites of the LRV was 7.32 ± 2.68 (3.1⁻15.6). The differences in the ratio of the diameters were statistically significant between the two groups and significantly higher in children with asymptomatic NCS (p = 0.025). The PV ratios of the LRV (p = 0.035) were significantly higher in asymptomatic children with NCS than in the symptomatic group. Our study identifies that increased compression ratio of the LRV entrapment is most observed in orthostatic proteinuria and microscopic hematuria.

Disseminated fungal infection by Aureobasidium pullulans in a renal transplant recipient.

Renal transplant recipients are on long-term potent immunosuppressive therapy, which makes them highly vulnerable to opportunistic fungal infections. Dematiaceous, or dark-pigmented saprophytic fungi, are being increasingly seen as opportunistic pathogens of mycoses in immunosuppressed patients. One of these is Aureobasidium pullulans, which is a black yeast-like dematiaceous fungus found ubiquitously in the environment that can cause various opportunistic human infections. Most infections occur by traumatic inoculation, such as keratitis and cutaneous lesions; disseminated mycoses are very rare and occur only in severely immunocompromised patients. We report a case of disseminated fungal infection due to A. pullulans in a pediatric patient who underwent renal transplant. The use of voriconazole and vacuum-assisted closure along with surgical drainage most likely contributed to the patient's positive outcome.

The role of bioelectrical impedance analysis, NT-ProBNP and inferior vena cava sonography in the assessment of body fluid volume in children with nephrotic syndrome / El papel del análisis de impedancia bioeléctrica, la NT-proBNP y la ecografía de vena cava inferior en la evaluación del volumen de líquidos corporales en niños con síndrome nefrótico

Background: Assessment of volume status and differentiating ’underfill ’ and ’overfill ’ edema is essential in the management of patients with nephrotic syndrome (NS). Objectives: Our aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children with NS. Methods: The hydration status of 19 patients with NS (before treatment of NS and at remission) and 25 healthy controls was assessed by multifrequency BIA, serum N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels, inferior vena cava (IVC) diameter, left atrium diameter (LAD) and vasoactive hormones. Results: Renin, aldosterone levels, IVC diameter and LAD were not statistically different between the groups. NT-proBNP values were statistically higher in the attack period compared to remission and the control group (p=0.005 for each). Total body water (TBW), overhydration (OH) and extracellular water (ECW) estimated by the BIA measurement in the attack group was significantly higher than that of the remission group and controls. There were no significant correlations among volume indicators in group I and group II. However, significant correlations were observed between NT-proBNP and TBW/BSA (p=0.008), ECW/BSA (p=0.003) and ECW/ICW (p=0.023) in the healthy group. TBW was found to be higher in patients with NS in association with increased ECW but without any change in ICW. NT-proBNP values were higher in patients during acute attack than during remission. Conclusions: Our findings support the lack of hypovolaemia in NS during acute attack. In addition, BIA is an easy-to-perform method for use in routine clinical practice to determine hydration status in patients with NS (AU)

Antecedentes: La evaluación del estado volumétrico y la diferenciación entre edema «por sobrellenado» y «por infrallenado» es fundamental en el manejo de los pacientes con síndrome nefrótico (SN). Objetivos: Nuestro objetivo fue evaluar el estado volumétrico de los pacientes con SN mediante el uso de diversos métodos y estudiar la utilidad del análisis de impedancia bioeléctrica (BIA) en niños con SN. Métodos: Se evaluó el estado de hidratación de 19 pacientes con SN (antes del tratamiento y en la remisión) y de 25 controles sanos mediante BIA multifrecuencia, valores plasmáticos de la fracción N-terminal del péptido natriurético cerebral (NT-proBNP), diámetro de la vena cava inferior, diámetro de la aurícula izquierda y hormonas vasoactivas. Resultados: La renina, los niveles de aldosterona, el diámetro de la vena cava inferior y el de la aurícula izquierda no fueron estadísticamente diferentes entre los grupos. Los valores de la NT-proBNP fueron estadísticamente más altos en el período de crisis que en el momento de remisión y que en el grupo de control (p=0,005 en cada uno). El agua total corporal (TBW), la hiperhidratación y el agua extracelular (ECW) estimada mediante la medición del BIA en el grupo de crisis fue considerablemente mayor que la del grupo de remisión y los controles. No hubo correlaciones importantes entre los indicadores de volumen en el grupo I y en el grupo II. Sin embargo, se observaron correlaciones considerables entre NT-proBNP y TBW/BSA (p=0,008), ECW/BSA (p=0,003) y ECW/ICW (p=0,023) en el grupo sano. Se encontró que TBW fue mayor en los pacientes con SN relacionado con el aumento de ECW, pero sin ningún cambio en ICW. Los valores de la NT-proBNP fueron más altos en los pacientes durante la crisis aguda que durante la remisión. Conclusiones: Nuestros hallazgos apoyan la falta de hipovolemia en el SN durante la crisis aguda. Además, BIA es un método fácil de utilizar en la práctica clínica habitual para determinar el estado de hidratación en pacientes con SN (AU)

Vaccination titres pre- and post-transplant in paediatric renal transplant recipients and the impact of immunosuppressive therapy.

BACKGROUND: Avoidance of vaccine-preventable infections in paediatric renal allograft recipients is of utmost importance. However, the development and maintenance of protective vaccination titres may be impaired in this patient population owing to their need for immunosuppressive medication. METHODS: In the framework of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN), we therefore performed a multi-centre, multi-national study and analysed vaccination titres pre- and post-transplant in 155 patients with serial titre measurements in comparison with published data in healthy children. RESULTS: The percentage of patients with positive vaccination titres before renal transplantation (RTx) was low, especially for diphtheria (38.5%, control 75%) and pertussis (21.3%, control 96.3%). As few as 58.1% of patients had a hepatitis B antibody (HBsAb) titre >100 IU/L before RTx. 38.1% of patients showed a vaccination titre loss post-transplant. Patients with an HBsAb titre between 10 and 100 IU/L before RTx experienced a significantly (p < 0.05) more frequent hepatitis B vaccination titre loss post-transplant than patients with an HBsAb titre >100 IU/L. The revaccination rate post-transplant was low and revaccination failed to induce positive titres in a considerable number of patients (27.3 to 83.3%). Treatment with rituximab was associated with a significantly increased risk of a vaccination titre loss post-transplant (odds ratio 4.26, p = 0.033). CONCLUSIONS: These data show a low percentage of patients with positive vaccination titres pre-transplant, a low revaccination rate post-transplant with limited antibody response, and a high rate of vaccination titre losses.

The role of bioelectrical impedance analysis, NT-ProBNP and inferior vena cava sonography in the assessment of body fluid volume in children with nephrotic syndrome.

BACKGROUND: Assessment of volume status and differentiating "underfill" and "overfill" edema is essential in the management of patients with nephrotic syndrome (NS). OBJECTIVES: Our aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children with NS. METHODS: The hydration status of 19 patients with NS (before treatment of NS and at remission) and 25 healthy controls was assessed by multifrequency BIA, serum N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels, inferior vena cava (IVC) diameter, left atrium diameter (LAD) and vasoactive hormones. RESULTS: Renin, aldosterone levels, IVC diameter and LAD were not statistically different between the groups. NT-proBNP values were statistically higher in the attack period compared to remission and the control group (p=0.005 for each). Total body water (TBW), overhydration (OH) and extracellular water (ECW) estimated by the BIA measurement in the attack group was significantly higher than that of the remission group and controls. There were no significant correlations among volume indicators in group I and group II. However, significant correlations were observed between NT-proBNP and TBW/BSA (p=0.008), ECW/BSA (p=0.003) and ECW/ICW (p=0.023) in the healthy group. TBW was found to be higher in patients with NS in association with increased ECW but without any change in ICW. NT-proBNP values were higher in patients during acute attack than during remission. CONCLUSIONS: Our findings support the lack of hypovolaemia in NS during acute attack. In addition, BIA is an easy-to-perform method for use in routine clinical practice to determine hydration status in patients with NS.

Incomplete vaccination coverage in European children with end-stage kidney disease prior to renal transplantation.

BACKGROUND: Because infections constitute a major cause of morbidity and mortality in paediatric renal allograft recipients, avoidance of preventable systemic infections by vaccination before transplantation is of utmost importance. However, data on the completeness of vaccinations and factors associated with incomplete vaccination coverage are scarce. METHODS: Within the framework of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN), we therefore performed a multi-centre, multi-national, retrospective study investigating the vaccination coverage before transplantation of 254 European children with end-stage renal disease (mean age 10.0 ± 5.6 years). RESULTS: Only 22 out of 254 patients (8.7%) presented complete vaccination coverage. In particular, the respective vaccination coverage against human papillomavirus (27.3%), pneumococci (42.0%), and meningococci (47.9%) was low. Patients with complete pneumococcal vaccination coverage had numerically less lower respiratory tract infections during the first 3 years post-transplant than children without vaccination or with an incomplete status (16.4% vs 27.7%, p = 0.081). Vaccine-preventable diseases post-transplant were 4.0 times more frequently in unvaccinated than in vaccinated patients. Factors associated with an incomplete vaccination coverage were non-Caucasian ethnicity (OR 9.21, p = 0.004), chronic dialysis treatment before transplantation (OR 6.18, p = 0.001), and older age at transplantation (OR 1.33, p < 0.001). CONCLUSIONS: The vaccination coverage in paediatric kidney transplant candidates is incomplete. Paediatric nephrologists, together with primary-care staff and patients' families, should therefore make every effort to improve vaccination rates before kidney transplantation.

Efficacy of anakinra in a patient with systemic amyloidosis presenting as amyloidoma.

Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. We report a case of a soft tissue amyloidoma in the abdomen of a 16-year-old girl who was diagnosed as having systemic amyloidosis. A girl aged 16 years was referred to our hospital with a pre-diagnosis of a retroperitoneal mass documented with abdominal ultrasonography and tomography. A laboratory examination revealed nephrotic syndrome. She underwent surgery for a complete resection of the lesion. A histopathologic examination with Congo red and crystal violet dyes verified the diagnosis of amyloidoma. An immunohistochemical study for amyloid A protein was positive. A renal biopsy was also compatible with AA amyloidosis. A detailed search for the etiology of systemic amyloidosis revealed heterozygous mutation in the Mediterranean fever gene. Treatment with colchicine and anakinra were started with the diagnosis of familial Mediterranean fever because the other causes of secondary amyloidosis were ruled out. After 3 months of anakinra treatment, the laboratory findings returned to normal and excessive proteinuria disappeared. In countries where FMF and other autoinflammatory diseases are prevelant, systemic amyloidosis should be kept in mind in the differential diagnosis of children who present with nephrotic syndrome and abdominal mass. Taking previously reported cases and our case together, it appears that anti-interleukin-1 treatment represents a promising new approach in a subset of patients with systemic amyloidosis secondary to autoinflammatory diseases.

Acoustic Radiation Force Impulse Quantification in the Evaluation of Renal Parenchyma Elasticity in Pediatric Patients With Chronic Kidney Disease: Preliminary Results.

OBJECTIVES: To evaluate renal parenchymal elasticity with acoustic radiation force impulse imaging in pediatric patients with chronic kidney disease (CKD) and compare with healthy volunteers. METHODS: Thirty-eight healthy volunteers and 30 pediatric CKD patients were enrolled in this prospective study. The shear wave velocity (SW) values of both kidneys in CKD patients and healthy volunteers were evaluated. RESULTS: The mean SW in healthy volunteers was 2.21 ± 0.34 m/s, whereas the same value was 1.81 ± 0.49, 1.72 ± 0.63, 1.66 ± 0.29, 1.48 ± 0.37, and 1.23 ± 0.27 for stages 1, 2, 3, 4, and 5 in CKD patients, respectively. The SW was significantly lower for each stage in the CKD patients compared with healthy volunteers. Acoustic radiation force impulse could not predict the different stages of CKD, with the exception of stage 5. The cut-off value for predicting CKD was 1.81 m/s; at this threshold, sensitivity was 76.5% and specificity was 92.1% (area under the curve = 0.870 [95% confidence interval: 0.750-0.990]; P < .001). Interobserver agreement expressed as intraclass coefficient correlation was 0.65 (95% confidence interval: 0.34 to 0.83; P < .001). CONCLUSIONS: Acoustic radiation force impulse may be a potentially useful tool in detecting CKD in pediatric patients.

Evaluation of Renal Parenchyma Elasticity With Acoustic Radiation Force Impulse Quantification in Nutcracker Syndrome and Comparisons With Grayscale Doppler Sonography and Laboratory Findings.

OBJECTIVES: We sought to evaluate renal parenchymal elasticity with Virtual Touch quantification of acoustic radiation force impulse imaging in nutcracker syndrome and to compare shear-wave velocity (SWV) values with grayscale Doppler sonography and laboratory findings. METHODS: Thirty-eight healthy volunteers and forty-three nutcracker syndrome patients were enrolled in this prospective study. SWV values for both kidneys in nutcracker syndrome patients and healthy volunteers were evaluated. Grayscale Doppler ultrasound and laboratory findings were obtained and compared with SWV values in both nutcracker syndrome patients and healthy volunteers. RESULTS: In nutcracker syndrome patients, SWV values for the left kidney were significantly lower than those for the right kidney (n = 43; 1.93 ± 0.43 m/s vs 2.53 ± 0.45 m/s [P < .001]). Healthy volunteers' SWV values for both kidneys had no statistically significant differences. There was a statistically significant difference between nutcracker syndrome patients and healthy volunteers for the SWV values and body mass index values. There was no statistically significant correlation between SWV values of nutcracker syndrome patients and age, gender, glomerular filtration rate, body mass index, vein diameter ratio, peak velocity ratio, or resistive indices. CONCLUSIONS: Acoustic radiation force impulse imaging offers new, additional information on the affected left kidney parenchymal changes in nutcracker syndrome patients.

Single port laparoscopic and open surgical accesses of chronic peritoneal dialysis in children: A single center experience over 12 years.

INTRODUCTION: The aim of this study was to evaluate patients with end stage renal failure (ESRD) who underwent chronic peritoneal dialysis (CPD). The clinical outcomes of laparoscopic and open placements of catheters were compared. MATERIALS AND METHODS: We reviewed 49 (18 male and 31 female) children with CPD according to age, sex, cause of ESRD, catheter insertion method, kt/V rate, complications, presence of peritonitis, catheter survival rate between January 2002 and February 2014. RESULTS: Thirty-three patients were with open placement and 16 patients were with laparoscopic placement. The rate of the peritonitis is significantly less in patients with laparoscopic access than open access (n = 4 vs n = 25) (P <0.01). Patients with peritonitis were younger than those who had no attack of peritonitis (10.95 ± 0.8 years vs 13.4 ± 0.85 years). According to the development of complications, significant difference has not been found between the open (n = 9) and laparoscopic (n = 3) approaches except the peritonitis. Catheter survival rate for the first year was 95%, and for five years was 87.5%. There was no difference between open and laparoscopic group according to catheter survival rate. The mean kt/V which indicates the effectiveness of peritoneal dialysis was mean 2.26 ± 0.08. No difference was found between laparoscopic and open methods according to kt/V. CONCLUSION: Laparoscopic placement of CPD results in lower peritonitis rate. Catheter survival rate was excellent in both groups. Single port laparoscopic access for CPD catheter insertion is an effective and safe method.

Kidney injury molecule-1 and neutrophil gelatinase associated lipocalin in normoalbuminuric diabetic children.

BACKGROUND: We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gelatinase associated lipocalin (NGAL), and the relationship between these markers and clinical and laboratory variables in normoalbuminuric children with type 1 diabetes (T1D). METHODS: The study group consisted of 60 (F/M: 28/32) children with T1D with a median age of 13 (min: 7.1-max: 17.9) years and a mean HbA1c of 8.6%. The average period of treatment was 6.8±2.2 years. The control group consisted of 60 healthy children [(F/M: 32/28); median age: 13.6 (min: 6.9-max: 17.9) years]. RESULTS: Urinary KIM-1 and NGAL levels were significantly elevated in the diabetic group (KIM-1: 0.50±0.34 ng/mg-cr; NGAL: 33±31 ng/mg-cr) compared with the nondiabetic control subjects (KIM-1: 0.26±0.25 ng/mg-cr, NGAL 13.3±14.5 ng/mg-cr) (p<0.001). No significant associations were observed between NGAL or KIM-1 and the duration of diabetes and HbA1c levels. NGAL was found to be weakly correlated with KIM-1 (p<0.005, r=0.289). CONCLUSIONS: NGAL and KIM are high in normoalbuminuric diabetic children before reduction in glomerular filtration rate. High NGAL and KIM-1 levels may indicate early diabetic kidney injury; however, we did not observe any relationship between these markers and diabetic indices. For clinical usefulness of these markers, long-term studies are required.

Effect of creatine and pioglitazone on Hk-2 cell line cisplatin nephrotoxicity.

Cisplatin is a chemotherapeutic agent, which is used in the treatment of various solid organ cancers, and its main dose limiting side effect of cisplatin is nephrotoxicity. The aim of this study is to investigate the role of pioglitazone and creatine on cisplatin nephrotoxicity in vitro. Real-time cell analyzer system (RTCA) was used for real-time and time-dependent analysis of the cellular response of HK-2 cells following incubation with cisplatin and combination with creatine or pioglitazone hydrochloride. First, half-maximal inhibitory concentrations (IC50) of cisplatin, creatine and pioglitazone were calculated by RTCA system. Afterwards creatine and pioglitazone was administered with serial dilutions under RTCA system. IC50 dose for cisplatin was 7.69 M × 10(-5) at 24th hour and 3.93 M × 10(-6) at 48th hour. IC50 dose for pioglitazone was 1.61 M × 10(-3) at 24th hour and 2.85 M × 10(-4) at 48th hour. Although cells were treated the dose of 40,225 mM creatine, IC50 dose could not been reached. Neither pioglitazone nor creatine had additional protective effect in any dose. Consequently, beneficial effect of creatine and pioglitazone on cisplatin-induced cell death could not be found. Further studies and clinical trials are needed to evaluate the effect of different doses of these drugs in cisplatin-induced nephrotoxicity.

Effect of free creatine therapy on cisplatin-induced renal damage.

Abstract Cisplatin is one of the commonly used anticancer drugs and nephrotoxicity limits its use. The aim of this study is to investigate the possible protective effect of creatine supplementation on cisplatin-induced nephrotoxicity. Sixty male Sprague-Dawley rats were divided into three groups: Group I: Cisplatin (n=20) (7 mg/kg cisplatin intraperitoneal (i.p.) single dose), group II: Cisplatin+creatine monohydrate (n=20) (7 mg/kg cisplatin i.p. single dose and 300 mg/kg creatine p.o. daily for 30 days starting on first day of cisplatin injection), group III: Control group (n=20) (Serum physiologic, 2.5 mL/kg i.p.). Sacrifications were performed at first week and 30th day. Blood urea nitrogen (BUN) and serum creatinine levels, histopathological evaluation, mitochondrial deoxyribonucleic acid (mtDNA) common deletion rates, and body weights of rats were evaluated. A significant decrease in body weight, higher values of kidney function tests, histopathological scores, and mtDNA deletion ratios were observed in group I compared to control group at days 7 and 30 (p<0.05). In group II, there was a slight decrease in body weight at same days (p=0.931 and 0.084, respectively). Kidney function tests, histopathological scores, and mtDNA common deletion ratios were statistically better in group II than group I at 7th and 30th day (p<0.05). Although creatine significantly reversed kidney functions and pathological findings, this improvement was not sufficient to reach normal control group's results at days 7 and 30. In conclusion, the present study demonstrates that creatine administration is a promising adjuvant protective drug for reducing nephrotoxic effect of cisplatin.

Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy.

BACKGROUND: Dense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. CASE-DIAGNOSIS/TREATMENT: A 14-year-old girl presented with edema and nephrotic range proteinuria. Blood tests showed hypoalbuminemia, nephrotic range proteinuria, normal renal function, and a low C3 level. Renal biopsy confirmed the diagnosis of crescentic DDD. Complement analysis revealed strong AP activation (low C3), positive C3 nephritic factor (C3NeF), and a decreased complement factor H (CFH) levels with CFH polymorphisms. Therapy with eculizumab was considered after the failure of corticosteroid and plasmapheresis to modulate the ongoing massive proteinuria and persistence of low serum C3 levels. There was a marked clinical and biochemical response following the administration of eculizumab. CONCLUSIONS: Our case emphasizes the efficacy of eculizumab in the management of crescentic DDD in a patient with a normal renal function, in a short follow-up period. Considering previously reported cases, it appears that eculizumab represents a promising new approach which may prevent progression to ESRD in a subset of patients with DDD.

The effect of simvastatin and erythropoietin on renal fibrosis in rats with unilateral ureteral obstruction.

Prevention of fibrosis is a very important therapeutic strategy in the treatment of obstructive nephropathy (ON). The aim of this study is to show and compare the actions of Simvastatin (Simv) and Erythropoietin (Epo) in renal expression of nuclear factor kappa B (NFκB), transforming growth factor-ß (TGF-ß), basic fibroblast growth factor (bFGF), platelet-derived growth factor B (PDGF-B), fibronectin and development of interstitial fibrosis in rats with unilateral ureteral obstruction (UUO). A total of 48 Sprague-Dawley rats were allocated to 4 groups of sham, Epo, Simv and control. Unilateral ureteral ligation was performed on all rats except the Sham group. For interstitial fibrosis Masson's trichrome stain and for the expression of TGF-ß, PDGF-B, bFGF, NFκB and fibronectin, immunohistochemical methods were used. In the Epo and Simv groups, expression of TGF-ß and fibronectin and staining with Masson's trichrome were less compared to the control group. In addition, fibronectin expression in the Epo group was less than the Simv group. Unlike the Simv group, NFκB and bFGF expression in the Epo group were less when compared to the control group. Consequently, it was seen that both Epo and Simv prevented fibrosis in ON. Epo was superior in this effect by suppressing the expressions of NFκB and bFGF more effectively than Simv. Based on this finding, Epo might be a better agent than Simv in the prevention of fibrosis in ON.

The late effects of anticancer therapy after childhood Wilm's tumor: the role of diastolic function and ambulatory blood pressure monitoring.

OBJECTIVE: Wilms' tumor, or nephroblastoma, is the most common primary malignant renal tumor of childhood. The excellent outcome now expected for most children with this tumor is attributed to the combination of effective adjuvant chemotherapy, improved surgical and anesthetic techniques and also the radiosensitivity of the tumor. The numerous organ systems are subject to the late effects of anticancer therapy. The aim of this study was to investigate the blood pressure profile and ambulatory blood pressure monitoring, and also cardiac diastolic functions and pulmonary venous flow in 25 children with unilateral Wilms' tumor in remission. METHODS: The patient group consists of 25 patients who successfully completed anticancer treatment for unilateral Wilms' tumor. Thirty-three age-, weight- and height-matched healthy children were considered as a control group for an echocardiographic study. Also, 20 age-, weight- and height-matched healthy children were considered as a control group for the ambulatory blood pressure monitoring study. RESULTS: In our study, 24 h, daytime and night-time systolic blood pressure and night-time diastolic blood pressure measurements were found to be significantly increased in the patient group compared with healthy children. We detected diastolic filling pattern abnormalities. We also found increase in pulmonary venous flow (systolic and diastolic) in Wilms' tumor group. CONCLUSIONS: We suggest the regular follow-up of survivors of Wilms' tumor for care and prevention of cardiovascular diseases.

Urinary neutrophil gelatinase-associated lipocalin in septic preterm babies: a preliminary study.

OBJECTIVE: This study was conducted to evaluate the predictive value of urinary neutrophil gelatinase-associated lipocalin (uNGAL) for acute kidney injury (AKI) among septic preterm infants. METHODS: Twenty-six very low-birth-weight (VLBW) babies were separated into three groups: group I, healthy preterms; group II, preterms with sepsis but without AKI; group III, preterms with sepsis and AKI. Demographic, clinical, and laboratory data of the babies were recorded. uNGAL and creatinine values were obtained on days 1, 3, and 7 of life. RESULTS: uNGAL levels differed statistically among three groups for all 3 days. Levels in group I (days 1, 3, and 7) were significant lower than levels in both groups II and III [median (interquartile range): 4.5 (10.8) µ/L, 8.7 (18.5) µ/L, and 4.3 (1.1) µ/L, respectively]. In group III, uNGAL levels on days 1 and 3 were significantly higher than levels in group II (p = 0.001, 0.016, respectively). CONCLUSION: First-day uNGAL levels were higher in VLBW preterm infants who later developed sepsis; whether the baby had AKI or not; but uNGAL levels were higher in septic babies with AKI compared with the infants without AKI. uNGAL is a promising early biomarker of AKI in VLBW infants with sepsis.