Clinical outcome in children with Henoch-Schonlein nephritis

WOS: 000242513200012PubMed ID: 17024391Henoch-Schonlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aim of this study was to evaluate both clinical features of the children with HSP and the prognoses of short- and long-term outcome of patients diagnosed as HSP nephritis (HSN). This is a retrospective data study of all children with HSP hospitalized from January 1991 to December 2005. The patients with HSN were classified according to their initial presentation, histologic findings, type of treatment and clinical outcome. All patients have been evaluated once every 2 months. Fifty-three of the patients had kidney biopsies. The patient population consisted of 141 children included 78 boys (55.3%) and 63 girls (44.7%) ranging in age at disease onset from 2 to 17 (8.9 +/- 3.29) years. Renal involvement was determined in 58.1%. Nephrotic and/or nephritic syndrome were found to be an unfavorable predictor both for short and long-term outcome (P < 0.05). However, 35% of these patients and 62% of them showed complete remission after 6 months and long-term course. Overall prognosis of HSN is relatively good and long-term morbidity is predominantly associated with initial presentation and renal involvement

Podocin mutations in a patient with congenital nephrotic syndrome and cardiac malformation

WOS: 000261080400010PubMed ID: 1906790

Effects of Aloe vera leaf gel extract on rat peritonitis model

WOS: 000336409800016PubMed ID: 24987181Objectives: The aim of this study was to investigate the antibacterial, anti-inflammatory, and antioxidant activities and probable toxic effects of Aloe vera (AV) in a rat peritonitis model. Materials and Methods: Rats were divided into five groups: (1) Control group, (2) AV group, (3) peritonitis group (P), (4) peritonitis + AV group (P + AV), and (5) peritonitis + antibiotherapy group (P + Ab). Ultrafiltration (UF) rates were determined and colony and leukocyte counts were calculated in the dialysate. Glucose, blood urea nitrogen (BUN), creatinine levels, and alanine transaminase (ALT) activities were studied in blood. Glucose, interleukins (IL-1 beta, IL-6), and prostaglandin E2 (PGE2) were studied in dialysate and peritoneal tissue for the assessment of the anti-inflammatory effect. Copper/zinc superoxide dismutase (Cu, Zn-SOD), malondialdehyde (MDA), and nitric oxide (NO) were also investigated in peritoneal tissue. Results: Aloe vera increased the UF rate and lowered leukocyte numbers in the peritonitis group. There was no significant difference in blood and dialysate glucose, BUN, creatinine levels and ALT activity among control and AV groups. AV decreased IL-1 beta, IL-6 and PGE2 in peritonitis, showing good anti-inflammatory effect. AV showed antioxidant effect on the chosen antioxidant parameters Cu, Zn-SOD, MDA, and NO. Conclusion: It was concluded that, AV might be used in peritonitis for its probable UF increasing, anti-inflammatory, and antioxidant effects.Adnan Menderes University Scientific Research Projects FundAdnan Menderes University [TPF-06015]Source of Support: This work was supported by Adnan Menderes University Scientific Research Projects Fund (Project no: TPF-06015). Conflict of Interest: None declared

NPHS2 (podicin) mutations in Turkish children with idiopathic nephrotic syndrome

WOS: 000250755700006PubMed ID: 17899208dThe podocin (NPHS2) gene encodes podocin protein, which has an important role in glomerular ultrafiltration and controlling slit membrane permeability. The detection of an NPHS2 mutation affects the treatment plan for children with nephritic syndrome (NS). The frequency and spectrum of podocin mutations in the Turkish population have remained largely unknown. The aim of this study was to screen for podocin mutations in Turkish patients with steroid-resistant NS (SRNS) and to compare it with other published series. There were 295 children with SRNS, originating from Turkey, included in this study. Forty-one patients (13.8%) had familial NS and 254 patients (86.2%) had sporadic NS. Mutation analysis was performed in all eight exons of the NPHS2 gene with the direct DNA sequencing method. There were 53 different pathogenetic NPHS2 mutations detected, including 37 novel mutations. The mutation detection rate was 24.7% for all patients, 29.2% for familial, and 24% for sporadic SRNS. The most common mutated exon was exon 5 ( 52 allele). The presence of mutations in exon 4 was found to increase the risk of end-stage renal disease ( ESRD). Among patients with mutations, the rates of renal failure and/or ESRD (26%) were significantly higher than in those without mutations (12.6%). The mean time of progression to renal failure and ESRD in patients with mutations (1.8 +/- 2.5 years) was significantly shorter than in patients without mutations (3.7 +/- 4.0 years). Additionally, in patients with heterozygote mutations, fewer cases (13.6%) progressed to renal failure and/ or ESRD than in with patients who had homozygote/ compound heterozygote mutations (31.3%). In conclusion, podocin mutations are responsible for some of both familial and sporadic SRNS cases in Turkey. The mutations in this gene should be searched for in every child after presentation with the first episode of NS

Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group

WOS: 000281020000004PubMed ID: 20718182The clinical course of focal segmental glomerulosclerosis (FSGS) is heterogeneous in children. To evaluate the clinical course and the predictors of outcome in Turkish children with primary FSGS, a retrospective study was conducted by the Turkish Pediatric Nephrology Study Group in 14 pediatric nephrology centers. Two hundred twenty-two patients (92 boys, 130 girls, aged 1-16 years) with biopsy-proven primary FSGS were included. One hundred forty-eight patients were followed-up for a median of 51 months (range: 0.26-270). The clinical course was characterized by complete remission in 50 (33.8%), persistent proteinuria in 50 (33.8%) and progression to renal failure in 48 (32.4%) patients. Progression to end-stage renal disease (ESRD) was significantly higher in patients who did not attain remission. Complete remission, partial remission and progress to renal failure were recorded in 37%, 32% and 28%, respectively, of the patients (n=73) treated with prednisone combined cyclophosphamide/cyclosporine A. However, in patients (n=33) treated with pulse methyl prednisolone plus oral prednisone (up to 20 months) combined with cyclophosphamide, complete remission in 51.5% and partial remission in 27.3% of the patients were noted. Progression to renal failure was observed in 9.1% of this group of patients. Multivariate analysis showed that only plasma creatinine at presentation was an independent predictive value for outcome. Patients with serum creatinine level higher than 1.5 mg/dl had 6.6 times increased rate of progression to renal failure. Failure to achieve remission is a predictor of renal failure in children with primary FSGS. The use of immunosuppressive treatment in conjunction with prolonged steroid seems beneficial in primary FSGS in children

Childhood vasculitides in Turkey: a nationwide survey

Aim: The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population. Patients and methods: A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schonlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed. Results: Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener's granulomatosis 0.4%, and Beh et disease 1.9%. There was no clear gender dominance. The mean age was 11.05 +/- 4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series. Conclusion: This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well

Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study.

Background Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. Methods Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. Results Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 +/- 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. Conclusions CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures

CLINICAL, EPIDEMIOLOGICAL AND PROGNOSTIC FEATURES OF CHILDREN WITH HEMOLYTIC UREMIC SYNDROME: MULTICENTER EXPERIENCE OF TURKEY

WOS: 00044399840014