Gurkan Genc

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

BackgroundDense 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/treatmentA 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.ConclusionsOur 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.

Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient

Acikgoz Y, Ozkaya O, Bek K, Genc G, Sensoy SG, Hokelek M. Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient.

Kidney Injury Molecule-1 as a Promising Biomarker for Acute Kidney Injury in Premature Babies

OBJECTIVE To evaluate the role of urinary kidney injury molecule-1 (uKIM-1) in early determination of renal injury in premature infants with respiratory distress syndrome (RDS). STUDY DESIGN Forty-eight premature babies hospitalized in the neonatal intensive care unit were included in the study and divided into three groups: group I, healthy premature infants; group II, preterm infants with RDS without acute kidney injury (AKI); group III, preterm infants with RDS and AKI. uKIM-1 and creatinine along with serum creatinine levels were measured with enzyme-linked immunosorbent assay on days 1, 3, and 7 of life. RESULTS On day 1, uKIM-1 levels in babies with RDS and AKI were higher than the other two groups. In this group, a significant increase in uKIM-1 levels were detected on day 3 (p = 0.015). The sensitivity and specificity of uKIM-1 were calculated as 73.3% and 76.9%, respectively, along with the increase of 0.5 ng per milligram of creatinine of uKIM-1 in day 3, when compared with values on day 1. Elevated uKIM-1 on day 7 was found to increase the risk of death by 7.3 times. CONCLUSION Serial uKIM-1 measurements can be used as a noninvasive indicator of kidney injury and uKIM-1 can be an ideal biomarker in premature infants.

Acute renal failure with acyclovir treatment in a child with leukemia

Acyclovir is an effective, frequently used antiviral agent. Adverse effects of this drug are well known and are especially seen with high doses and/or dehydration. In this article, we report a 6-year-old boy with leukemia with nonoliguric acute renal failure in normal hydration status after using acyclovir treatment. He had no preexisting renal impairment, and there were no additional symptoms. Dimercaptosuccinic acid radionucleid scyntigraphy and other laboratory findings revealed impairment of proximal tubule function, in addition to distal tubule. We emphasize that renal functions should be monitored carefully during treatment with acyclovir, and asymptomatic nephrotoxicity must be kept in mind.

A case of acetaminophen (paracetamol) causing renal failure without liver damage in a child and review of literature

Acetaminophen (paracetamol) is a widely used drug and known as a safety antipyretic and analgesic drug in childhood. Acetaminophen-associated liver damage is more recognized than kidney damage. Nephrotoxicity and hepatotoxicity can be seen together after acetaminophen overdose, but renal damage without liver damage is a rarely seen entity in all age groups being reported more rarely in childhood. We present here a 16-year-old girl with renal failure without liver damage because of acetaminophen toxicity and a review of literature for pathophysiological mechanisms, clinical course, treatment, and outcome.

A rare cause of recurrent hematuria in children: Nutcracker syndrome.

Nutcracker syndrome (NCS), the left renal vein hypertension due to compression between aorta and superior mesenteric artery, may present with hematuria, flank pain and proteinuria. We report a 6-year-old girl with episodic macroscopic hematuria who was diagnosed as NCS with Doppler ultrasound and 3D computerized tomography angiography. She was managed conservatively with spontaneous resolution of macroscopic hematuria. With this case we would like to emphasize that in children with hematuria and orthostatic proteinuria without an apparent cause, a great index of suspicion and appropriate imaging is necessary for the diagnosis of NCS.

Temporary Peritoneal Dialysis in Newborns and Children: A Single-Center Experience over Five Years

Aim: To evaluate the indications, complications, and outcomes of temporary peritoneal dialysis (TPD) in children with acute renal failure (ARF). Patients and methods: All patients undergoing TPD between February 2006 and January 2011 in a children’s hospital were included in the study. Patient characteristics, indications, complications, and duration of TPD (DPD), requirement of re-operation, length of stay, presence of sepsis, and outcome were recorded. Results: There were 21 newborns (14 prematures), 9 infants, and 9 children. The main nephrotoxic agents were gentamicin (n = 7), netilmisin (n = 5), vancomycin (n = 3), and ibuprophen (n = 3). Patients with multiorgan failure (n = 9) had significantly higher blood urea nitrogen (BUN) and creatinine levels than those without multiorgan failure (n = 30) [BUN: 94 ± 27.3 vs. 34.3 ± 4.9) and creatinine: 4.1 ± 0.8 vs. 1.9 ± 0.2)]. The mean DPD was longer in mature patients than in prematures (newborn: 3.7; children: 7.1). Nine complications were observed (23%) (leakage in three and poor drainage in six patients). Twenty-five patients (64.1%) responded to TPD treatment and were discharged, and 14 patients (10 newborns and 7 of them were premature) died (35.9%). Mortality rate was higher in prematures (n = 7) and patients with a history of nephrotoxic agent (n = 10). Conclusion: TPD is effective especially in neonates with ARF and it is a reliable alternative to the hemodialysis or other continuous renal replacement therapies but it is not free of complications. It has limited effects, particularly in patients with multiorgan failure.

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.

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

Abstract 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-β (TGF-β), 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-β, PDGF-B, bFGF, NFκB and fibronectin, immunohistochemical methods were used. In the Epo and Simv groups, expression of TGF-β 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.

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 groups 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.