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Aim: The aim of this study was to determine the long-term results of children followed up with a diagnosis of nephrotic syndrome in a single center. Material and Methods: The medical data of 33 patients aged between 6 months and 10 years who were diagnosed with idiopathic nephrotic syndrome in our center between January 2000 and December 2012 and followed up for a period of 2-12 years were reviewed (Gulhane Military Medical Academy Ethics committee, 07.11.2012/10). Results: The mean age of disease onset was 3.2±2.04 years (range: 0.5-10 years) and the mean follow-up period was 6±3.4 years (range: 2-12 years). Thirteen (39.4%) of the study group (or the patients) were female and 20 (60.6%) were male. Twenty seven (1.8%) of the patients were sensitive to steroid and 6 (18.1%) were resistant to steroid. Four (12.1%) of the steroid-resistant patients had steroid-dependent nephrotic syndrome, 5 (15.2%) had frequently relapsing nephrotic syndrome and 18 (54.5%) had rarely relapsing nephrotic syndrome. Histopathological diagnoses of six patients who underwent biopsy because of resistance to steroid were as follows: focal segmental glomerulosclerosis (n=3), C1q nephropathy (n=1), diffuse mesangial proliferation (n=1) and membraneous nephropathy (n=1). Fifteen (45.5%) patients entered into full remission and 2 (6%) patients developed chronic renal failure. Treatment complications including decreased bone mineral density in three patients (9%), short stature in 2 patients (6%) and cataract in 2 patients (6%) developed. Conclusions: Children with nephrotic syndrome carry a risk in terms of short stature, osteoporosis, cataract and renal failure in the long-term follow-up. It was observed that our rates of response to steroid were similar to the literature and the most common histopathological diagnosis was focal segmental glomerulosclerosis in our patients who underwent biopsy because of resistance to steroid. It was thought that multi-center studies should be conducted to demonstrate regional or national differences related with long-term results of childhood nephrotic syndrome.

Pediatric nephrotic syndrome (NS) is a chronic glomeruslar disorder, and if untreated, is associated with increased risk of life-threatening infectious, thromboembolism, lipid abnormalities, and malnutrition. The aim of the managesment of NS in children is to induce and maintain comsplete remission with resolution of proteinuria and edema without encountering serious adverse effects of therapy. Over 90% of cases in children with idiopathic NS and a majority of them will respond to steroid therapy. However, a substantial number of patients relapse frequently and become steroid dependent. The occurrence of frequent relapses necessitates clear therapeutic strategies in orsder to maintain sustained remission and minimize steroid toxicity. Numerous therapeutic regimens have been prosposed utilizing steroid sparing agents such as alkylating agents, principally, cyclophosphamide and chlorambucil, calcineurin inhibitors namely cyclosporin A and an immusnomodulatory drug, levamisole, with variable success and associated side-effects. Recently, mycophenolate mofetil (MMF), tacrolimus, the anti-CD20 antibody, rituximab, have emerged as new therapeutic options for the mansagement of steroid dependent NS in a few uncontrolled clinical trials. It is therefore important that the benefits and risks of these agents are weighed before considering their use in the treatment of patients with NS.

This study evaluated the plasma levels of trace elements in children with chronic hepatitis B virus (HBV) infection and assessed whether they can be a factor that affects the response to interferon alpha (IFN-alpha) treatment. The study included 35 cases (ten girls, 25 boys) aged 3-13 years with chronic HBV infection and the control group. Plasma levels of copper (Cu), manganese (Mn), molybdenum (Mo), selenium (Se), and zinc (Zn) were measured before IFN-alpha treatment and biochemical, virological, and histopathologic response to treatment were assessed. Children were followed for at least 15 months. Although plasma Cu levels showed no difference between the groups, Mn, Mo, Se, and Zn levels were significantly lower in the study group before treatment. Fourteen cases (40%) showed biochemical response; 17 (48.6%) showed virological response; 16 (47.6%) showed histopathologic response, and ten (28.6%) showed response according to all three parameters. Plasma Cu and Mn levels of patients with triple response showed no difference; but Mo, Se, and Zn levels were significantly lower (p0.001) in the study group. No difference was observed between responders and nonresponders (p0.05). Plasma levels of Mn, Mo, Se, and Zn are lower in children with chronic HBV infection compared to healthy children. The pretreatment levels of these elements did not show difference between responders and nonresponders to IFN-alpha.

SUMMARY: Aim: Adrenomedullin (AM), a novel peptide recently isolated from pheochromocytoma, eliciting vasorelaxing activity, is the strongest among all known peptides. AM has been detected in the adrenal medulla, cardiac tissue, lung and kidney. Immunohistochemical studies have demonstrated the localization of AM in glomeruli, tubules and collecting cells of the kidney. Clinically, plasma and urinary AM levels are altered in patients with different renal disease. The present study aims to determine plasma and urinary AM levels in children with acute pyelonephritis (APN) and compare the results with a control group. Materials and Methods: The study group was comprised of 19 patients with APN aged 11.6 ± 3.7 months (range, 6–18 months) and the control group consisted of 16 cases aged 11.5 ± 3.2 months (range, 7–16 months). Acute pyelonephritis was diagnosed by clinical, laboratory and imaging methods. Plasma and urinary AM levels were measured by high performance liquid chromotography (HPLC). Results: The plasma AM levels were lower in APN patients (33.40 ± 2.27 pmol/mL) than in the control group (43.76 ± 4.27 pmol/mL) (P

Intrauterine growth retardation/restriction (IUGR) is associated with fetal malnutrition. It has consequences for later life including increased incidence of obesity, diabetes mellitus, cardiovascular disease (CVD), and metabolic syndrome. Adipokines (adiponectin and leptin), adropin, and endothelin-1 are associated with obesity and metabolic syndrome regulation. Intrauterine changes in these mediators could affect programming of later adult obesity and metabolic syndrome. Our objectives were to compare the levels of these mediators in both cord and maternal blood between IUGR pregnancies and control, healthy pregnancies, and to study the correlation of adipokines with adropin and endothelin-1 in maternal and cord blood in IUGR pregnancies as well as in healthy control pregnancies. Maternal and cord blood samples were taken from 16 women with IUGR pregnancies and 16 women with healthy pregnancies. Serum levels of leptin, adiponectin, adropin, and endothelin-1 were measured by ELISA. Maternal blood adropin levels were significantly lower in the IUGR group than in the control group; the other mediators did not differ significantly. There was a positive correlation between maternal blood adropin and endothelin levels. (r=0.731, P=0.001) in the control but not the IUGR group. Cord blood adropin and adiponectin levels were significantly lower in the IUGR group compared with the control group, while leptin or endothelin-1 did not differ significantly. There was a negative correlation between adropin and leptin (r=–0.704, P=0.001) in the IUGR but not the control group cord blood. There were also positive correlations between endothelin and adropin for both groups (r=0.594, P=0.006; r=0.560, P=0.010, respectively); to the best of our knowledge, this is the first report of such a correlation. Differences in fetal expression of adropin and adiponectin in IUGR could influence programming of obesity, metabolic syndrome, diabetes, and CVD in later life.

SUMMARY: Background: β-thalassemia minor is a common heterozygous haemoglobinopathy that is characterized by both microcytosis and hypochromia. It requires no treatment. It has been postulated that low-grade haemolysis, tubular iron deposition and toxins derived from erythrocytes might cause renal tubular damage in adult patients with β-thalassemia minor. Our aim was to investigate the renal tubular functions in children with β-thalassemia minor and to determine its possible harmful effects. Methods: The study was conducted on 32 children (14 female and 18 male) at the age of 5.8 ± 3.1 years (range 2–14 years) with β-thalassemia minor. The patients were classified as anaemic (haemoglobin (Hb) ≤ 11 g/dL) (Group 1, n = 14) and non-anaemic (Hb > 11 g/dL) (Group 2, n = 18). A control group was formed with 18 healthy children whose ages and sexes match those in other groups (Group 3, n = 18). Fractional excretion of sodium (FENa, %), fractional excretion of magnesium (FEMg, %), fractional excretion of uric acid (FEUA, %) and tubular phosphorus reabsorption (TPR,%) were calculated with standard formulas. Urinary calcium excretion (mg/kg per 24 h), zinc (Zn) (µg/dL), glucosuria (mg/dL), β-2 microglobulin (mg/dL) and N-acetyl-β–D-glycosaminidase (NAG, U/mmol creatinine) levels were measured through biochemical methods. Results: There was no statistically significant difference among the three groups in terms of the results of FENa (%), FEMg (%), FEUA (%), TPR (%), calciuria (mg/kg per 24 h), NAG, urine Zn, proteinuria, glucosuria or urine β- 2 microglobulin levels (P > 0.05). Conclusion: On the contrary of children with β-thalassemia major, renal tubular dysfunction has not been determined in children with β-thalassemia minor in the present study.

Congenital nephrogenic diabetes insipidus (CNDI) is a rare inherited disease characterized by renal tubular unresponsiveness to the antidiuretic effect of arginine-vasopressin due to the mutations of two molecules, the vasopressin V2 receptor (AVPR2) and the aquasporin-2 water channel. We report a novel AVPR2 mutation in a Turkish 18 month-old boy with skeletal anomalies.

Glue sniffing is a serious medical problem among teenagers. Various chemical substances such as toluene and benzene containing glues have been reported to be toxic. It has been demonstrated that some toxic metals such as lead are elevated in the blood of solvent-addicted patients. Whereas aluminium is an element that has toxic effects on neurological, hematopoetic system and bone metabolism. We want to determine the serum levels of aluminium in glue-sniffer adolescents in comparison with healthy subjects. In addition, we compared aluminium levels of different commercial glue preparations (i.e. metal and plastic containers), to determine which type of container is better for less aluminium toxicity. We measured serum levels of aluminium in 37 glue-sniffer and 37 healthy subjects using atomic absorption spectrophotometry. The average duration of glue-sniffer was 3.8 +/- 0.8 years. We also measured aluminium levels of 10 commercial glue preparations that seven of them with metal and three with plastic containers. We found that serum levels of aluminium were 63.29 +/- 13.20 ng/ml and 36.7 +/- 8.60 ng/ml in glue-sniffer and in control subjects, respectively (P < 0.001). The average aluminium level in the glues was 8.6 +/- 3.24 ng/g in the preparations with metal containers, whereas 3.03 +/- 0.76 ng/g with plastic containers (P < 0.001). Therefore, to decrease the incidence of aluminium toxicity in glue-sniffers, it may be a good step to market of glue preparations in plastic instead of metal containers.

A 17-year-old boy was admitted to the hospital twice in a year for 2 episodes of hemolytic uremic syndrome (HUS). During these 2 HUS episodes he had diarrhea, decreased serum complement, decreased total protein and decreased serum albumin concentrations. We suggest that protein-losing enteropathy and hypocomplementemia due to intestinal lymphangiectasia is may be a rare cause of atypical HUS.

OBJECTIVES It is well known that a relationship exists between vesicoureteral reflux (VUR) and dysfunctional voiding, and the spontaneous resolution rate in older children is lower than the rate in younger children. In this study, we analyzed our experience with biofeedback treatment in older children with confirmed voiding dysfunction and VUR and investigated the effect of this treatment on the reflux resolution rates in these children. METHODS A total of 78 children, 5 to 14 years old (mean age 9), with voiding dysfunction and VUR detected by voiding cystourethrography were treated with biofeedback therapy. Voiding cystourethrography was performed 6 months after completion of the biofeedback program to determine the reflux status. The treatment results were also documented as subjective and objective improvements. RESULTS The reflux in 98 units (20 bilateral) was grade 1 in 26, grade 2 in 32, grade 3 in 28, and grade 4 in 12. At 6 months of follow-up, VUR had resolved on voiding cystourethrography in 62 units (63%), the grade had improved in 28 units (29%), and the reflux had remained unchanged in 8 units (8%). Among the older children treated with biofeedback, we also observed improvements in nocturnal enuresis (82%), daytime wetting (70%), constipation (78%), frequency (76%), infrequency (64%), urgency (71%), staccato voiding (81%), flattened voiding (81%), bladder overactivity (82%), detrusor sphincter dyssynergia (77%), spinning top urethra (67%), and urinary tract infection (80%). CONCLUSIONS Biofeedback therapy is applicable in older children with dysfunctional voiding and VUR and yields greater resolution rates than the historical resolution rates.

ozkaya, ozan/0000-0002-0198-1221 WOS: 000240807600018 PubMed: 16521052 The clinical course of Henoch-Schonlein Purpura (HSP) in children is variable, with some patients having a much more rapidly progressing course than others. We investigated whether polymorphisms of the renin-angiotensin system (RAS) genes are involved in HSP. Three RAS genotypes were examined in 114 children with HSP and in 164 healthy children: the angiotensin I converting enzyme (ACE) insertion/deletion polymorphism, the M235T mutation in the angiotensinogen gene (Agt), and the A1166C in the angiotensin II type I receptor (AT1R) gene. Significant differences were observed between HSP patients and control group in the frequency of ACE and Agt genotypes (p=0.004 and p=0.003, respectively). The TT genotype of Agt gene was associated with a 3.5-fold increased risk for Henoch-Schonlein nephritis (HSN) compared with the MM/MT genotype (odds ratio, 3.5; 95% confidence interval, 1.2-10.4). There was a trend to a higher prevalence of the TT genotype of the Agt gene among patients with nephrotic range proteinuria when compared to the patients with mild proteinuria, although the difference did not reach a statistical significance. The results of this study suggest that polymorphisms of ACE gene and Agt gene likely influence the risk of developing HSP. However, among the three genes of the RAS studies, only Agt gene was associated with the susceptibility to HSN. RAS gene polymorphisms studied are not associated with the presence of nephrotic range proteinuria. Additional studies are warranted to verify the correlation between RAS gene polymorphisms and susceptibility to HSP.

Adrenomedullin (AM) is a strong vasodilator peptide with proven antimitogenic and antiproliferative effects in renal mesangial cells, as well as diuretic and natriuretic actions. Its gene expression is stimulated by endotoxins (lipopolysacharides) and cytokines. Consequently, its plasma and urinary levels are known to deviate from normal levels in many renal diseases. The purpose of this study is to determine plasma and urinary AM levels in children with renal parenchymal scar (RPS) and vesicoureteral reflux (VUR). The study was carried out on 74 children with recurrent urinary tract infections, arranged in groups: 25 patients with RPS with VUR (group I), 16 patients with RPS without VUR (group II), 12 patients with VUR without RPS (group III) and 21 healthy children as the control group. Plasma and urinary AM concentrations were both determined by high performance liquid chromotography (HPLC). Plasma AM was measured as picomoles per milliliter (pM/ml) and urinary AM as pM/mg urinary creatinine. In addition, serum creatinine, creatinine clearance and fractional sodium excretion (FENa) were measured. All cases with RPS and VUR had normal blood pressure levels. The plasma AM levels were higher, although not significantly, in the control group (56.2 +/- 14.0 pM/ml) than in group I (50.6 +/- 4.2 pM/ml), group II (49.6 +/- 3.7 pM/ml) and group III (50.6 +/- 3.6 pM/ml) ( P =0.162). The urinary AM levels were higher in the control group (80.1 +/- 33.9 pM/mg) than in the three study groups (52 +/- 7.6 pM/mg, 58.6 +/- 7.5 pM/mg and 44.2 +/- 6.4 pM/mg; P =0.003, P =0.002 and P =0.002, respectively). There were no differences among the 4 groups (group I, group II, group III and the control group) in terms of FENa and creatinine clearance ( P > 0.05 and P > 0.05, respectively). The finding that diminished urinary AM levels in patients with RPS and VUR implies that AM can be a prognostic factor in the long-term follow-up of cases with these diseases.

We present an 11-year-old boy with Bracellae osteoarthritis on caput femoris. We therefore emphasize the importance of Brucellae osteoarthritis and in particular bone scintigraphy in the diagnosis of childhood monoarthritis.

Although Mycoplasma pneumoniae infections are common among school children and young adults, they have been rarely reported in renal transplant recipients. Herein, we report an 8-yr-old boy who had M. pneumoniae infection 1 yr after transplantation and showed liver dysfunction during the course of the disease. In children who underwent renal transplantation and receive immunosuppressive treatment, we suggest that symptoms of a simple upper respiratory tract infection may precede M. pneumoniae disease with potentially serious extrapulmonary complications.

Idiopathic hypercalciuria is a complex disease resulting from an interaction between environmental and genetic factors. Recently, the relationship between vitamin D receptor (VDR) alleles and calcium homeostasis has been investigated. This study was conducted to explore the association of VDR gene polymorphism with the risk of absorptive hypercalciuria (AH). We investigated the VDR gene polymorphisms, ApaI, BsmI, and TaqI, in relation to intact parathormone (PTH), osteocalcin, and 25-hydroxyvitamin D in 80 children (42 males, 38 girls) with AH and in 86 healthy children without hypercalciuria. A significant difference in the ApaI genotype was observed between the AH group and the control group (χ 2 =7.21, P=0.027). The AA genotype was associated with a 3.5-fold increased risk for idiopathic hypercalciuria compared with the Aa/aa genotype (odds ratio 3.5, 95% confidence interval 1.1–11). The BsmI and TaqI polymorphisms did not show any significant association with AH. Serum osteocalcin levels were significantly higher in the group with the AA genotype compared with those with the Aa or aa genotype (P=0.02, P=0.05, respectively). The results indicate that the ApaI AA genotype of the VDR gene is not only associated with AH but is also related to differences in serum osteocalcin.

Measles is a rare communicable disease which may be fatal in renal transplant patients. Herein we present a patient with renal transplantation who had measles and who subsequently developed subacute measles encephalitis 4 months later. We recommend all children with chronic renal failure be vaccinated against measles before transplantation.

Leptin may contribute to renal pathology in some situations by stimulating transforming growth factor-beta1 (TGF-beta1) synthesis. The soluble leptin receptor (sOb-R) is a transport protein contributing to binding and activation of circulating leptin. We investigated the interaction between serum and urinary leptin, TGF-beta1, and serum sOb-R levels in 38 patients with minimal change nephrotic syndrome (MCNS) aged between 6 and 12 years and 10 age- and sex-matched healthy controls (group III). Patients were divided into two groups: group I, proteinuria exceeding40 mg/m(2) per hour and group II, patients in remission. Serum leptin levels in group I were significantly lower than those in group II and group III ( P=0.011, P=0.007, respectively). There was a negative correlation between serum leptin levels and proteinuria ( r=-0.52, P=0.02) as well as between serum leptin and sOb-R levels ( r=-0.82, P=0.000) in group I. Urine leptin and sOb-R levels in group I were significantly higher than in group II ( P=0.0021, P=0.001, respectively) and group III ( P=0.07, P=0.009, respectively). Serum TGF-beta1 levels in healthy controls (406+/-424 pg/ml) were significantly lower than those in groups I and II ( P=0.004, P=0.000, respectively). However, no significant correlation was found between the serum TGF-beta1 and leptin levels in MCNS patients. In conclusion, low serum leptin, high serum TGF-beta1 and sOb-R levels, and elevated urine leptin concentrations were observed at the onset of MCNS. Since long-term proteinuria and leptinuria might be associated with the progression of renal damage, future in vivo and in vitro studies are needed to explain the interaction between these parameters in different types of nephrotic syndrome.

Cyclosporine (CsA)-associated neurotoxicity has been reported in recipients of solid organ and bone marrow transplants. These neurological side effects are usually mild and resolve with temporary reduction or withdrawal of CsA. We report a 16-year-old renal transplant recipient who developed tremor, tinnitus, and peripheral facial paralysis during oral CsA treatment. Her serum magnesium level was below the normal range and her peripheral facial paralysis did not improve with the cessation of the drug.

Henoch-Schönlein purpura (HSP) is one of the most common vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, arthritis, renal and gastrointestinal system (GIS) involvement. HSP is usually triggered by an antigenic stimulus including infectious agents, drugs, cold, insect bite or food. HSP is rarely triggered by Varicella zoster infection. We herein presented a case with HSP following varicella.