History of Pediatric Nephrology in Türkiye.

This article analyzes the foundation, institutiona-lization, and development of pediatric nephrology in Türkiye. Erich Frank is the pioneer and inspiration for Turkish nephrologists. He pioneered the training of a new generation of modern physicians, the first generation of Turkish nephrologists, and the establishment of the discipline of nephrology. Turkish pediatricians closely followed these developments and tried to improve their knowledge, making an effort to study abroad. These physicians established pediatric nephrology departments in their universities, trained young pediatric nephrologists, initiated pediatric hemodialysis-peritoneal dialysis units, and realized renal transplantation in children. Another pioneer of pediatric nephrology is Prof. Ihsan Dogramaci, who established the first pediatric nephrology department at the Ankara-Hacettepe Medical Center in the 1960s. He directed many physicians to pediatric nephrology and provided them education and research opportunities abroad. Pediatric nephrology became a subspecialty in pediatrics in 1983 in Türkiye. Over the years, the number of Turkish pediatric nephrologists has increased; in 1990, they established the Pediatric Nephrology Association. This gave momentum to the standardization of education, better patient care, and scientific studies. Renal biopsies were initiated in 1964, the first pediatric hemodialysis application was conducted in 1974, the first renal transplantation from the living donor was in 1975, and the first continuous peritoneal dialysis was in 1989. With regard to international collaborations, since 2000, a council member from Türkiye has continuously been elected to the executive board of the European Society for Pediatric Nephrology; currently, the European Society for Pediatric Nephrology is led by President Rezan Topaloglu. Several Turkish pediatric nephrologists have worked in international associations. Today, Türkiye has 107 pediatric nephrology centers, 265 well-trained specialists, 28 hemodialysis units, 39 peritoneal dialysis units, and 26 pediatric transplant centers.

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

The 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.

Etiology and outcome of acute kidney injury in children.

The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.6% were newborns (median age 3 days, range 1-24 days), and 67.4% were children aged >1 month (median 2.99 years, range 1 month-18 years). The most common medical conditions were prematurity (42.2%) and congenital heart disease (CHD, 11.7%) in newborns, and malignancy (12.9%) and CHD (12.3%) in children aged >1 month. Hypoxic/ischemic injury and sepsis were the leading causes of AKI in both age groups. Dialysis was performed in 30.3% of newborns and 33.6% of children aged >1 month. Mortality was higher in the newborns (42.6 vs. 27.9%; p < 0.005). Stepwise multiple regression analysis revealed the major independent risk factors to be mechanical ventilation [relative risk (RR) 17.31, 95% confidence interval (95% CI) 4.88-61.42], hypervolemia (RR 12.90, 95% CI 1.97-84.37), CHD (RR 9.85, 95% CI 2.08-46.60), and metabolic acidosis (RR 7.64, 95% CI 2.90-20.15) in newborns and mechanical ventilation (RR 8.73, 95% CI 3.95-19.29), hypoxia (RR 5.35, 95% CI 2.26-12.67), and intrinsic AKI (RR 4.91, 95% CI 2.04-11.78) in children aged >1 month.

Primary peritonitis in children with nephrotic syndrome: results of a 5-year multicenter study.

Primary peritonitis is a well-described infectious complication of nephrotic syndrome. Current data on the true incidence of peritonitis and efficacy of preventive pneumococcal vaccination are not clear in this group of children. In this nationwide study, among a total of 268 patients with an initial diagnosis of steroid sensitive nephrotic syndrome, eight episodes of primary peritonitis were detected in seven patients during 5 years. All eight attacks of peritonitis occurred in the relapse period. Seven of these peritonitis episodes occurred in the first 2 years of nephrotic syndrome, three of them during the first attack. One patient had two attacks with a 6-month interval. Only two of the patients were steroid sensitive, while four of them were steroid dependent, and one was steroid resistant at the diagnosis of peritonitis. The causing microorganism was identified in three patients (Streptococcus hemolyticus, Streptococcus pneumoniae, and alpha-hemolytic Streptococcus). Incidence of peritonitis (2.6%) in our series was not high when compared with previous reports. None of the patients had been immunized against pneumococcus before or after the peritonitis attack. It raises the question if the vaccine is necessary for every child with steroid sensitive nephrotic syndrome. However, we suggest that immunization against pneumococcus is not indicated in children with steroid-responsive nephrotic syndrome (NS) and should be reserved for the small number of children who have steroid-dependent or steroid-resistant NS.

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-Schönlein 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 Behçet 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.