Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35 months (0.4-231 months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3 mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12 months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year's follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF ( - ) stones than in MRF ( +) stones. However, remission rate with medical treatment was higher in cases with MRF ( +) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.

COVID-19 in pediatric patients undergoing chronic dialysis and kidney transplantation.

The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20. A total of 46 confirmed cases of COVID-19 were reported from 12 centers, of which 17 were dialysis patients, and 29 were KTx recipients. Thus, the incidence rate of COVID-19 was 9.3% among dialysis patients and 9.2% among KTx recipients over a 9-month period in Istanbul. Twelve KTx recipients and three dialysis patients were asymptomatic (p = 0.12). Most of the symptomatic patients in both the dialysis and KTx groups had a mild respiratory illness. Only two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. In the entire cohort, one hemodialysis patient with multiple comorbidities died.Conclusion: While most cases are asymptomatic or have a mild disease course, pediatric patients undergoing dialysis and a kidney transplant are at increased risk for COVID-19. What is Known: • In adult population, both dialysis patients and kidney transplant recipients are at increased risk for severe illness of COVID-19 and have higher mortality rate. • Children with kidney transplantation are not at increased risk for COVID-19 and most have mild disease course. • Data on children on dialysis are scarce. What is New: • Pediatric patients undergoing dialysis and kidney transplantation have an increased risk for COVID-19. • Most patients undergoing renal replacement therapy either on dialysis or transplanted develop asymptomatic or mild COVID-19 disease with a favorable outcome.

A nationwide retrospective study in Turkish children with nephrocalcinosis.

BACKGROUND: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. METHODS: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. RESULTS: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5-208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. DISCUSSION: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease.

Scientific publications in pediatrics over the last ten years in Turkey and worldwide.

In keeping with the large area of interest comprised within the field of pediatrics, the number and diversity of publications in this field has gradually increased over the last ten years in our country as well as worldwide. The objective of this paper is to offer an analysis of the status of pediatrics publications during those ten years. The publications analyzed were listed in the Thomson Reuters Web of Science and/or published in journals included in the Science Citation Index, the Science Citation Index Expanded and the Social Sciences Citation Index. All of these publications appeared in journals in which at least one pediatrics specialist published between the years 2004 and 2013. It may be seen that Turkey is in fifth place worldwide in terms of the number of studies published in these journals, with 6,134 such publications. The conclusion is that it is necessary for more Turkish studies to be multicenter, to involve international participation and to receive more citations; importance must be attached to the objective of transforming of the knowledge produced by Turkish researchers into a greater number of publications of increased quality.

Circulating fibroblast growth factor 23 in children on peritoneal dialysis is associated with effective dialysis.

Our purpose was to assess the relationship between serum fibroblast growth factor 23 (FGF23) and left ventricular function, carotid intima-media thickness (CIMT) and laboratory features in children on peritoneal dialysis (PD). The study population consisted of 17 patients (11 female; median age 7.83 years, range 0.66-17.75) undergoing PD for 22 months (range 2-98). Serum FGF23, serum phosphorus, calcium, intact parathyroid hormone (iPTH), 25(OH) vitamin D, 1,25(OH)2 vitamin D and Kt/V urea, left ventricular mass (LVM) and LVM index (LVMI) were assessed. Median FGF23 level was 29.92 pg/ml (22.7-74.76), phosphorus was 5.2 mg/dl (3.1-9.9), iPTH was 438 pg/ml (16-1446), 25(OH) vitamin D was 11 ng/ml (5-35), 1,25(OH)2 vitamin D was 11 pg/ml (2-106), Kt/V urea was 2.33 (1.01-3.84). FGF23 level was independently associated with Kt/V urea (p<0.001). We found that effective dialysis may be the leading determinant of FGF level, independent from the calcium-phosphorus-PTH axis, in pediatric PD patients.

Long-term results of children diagnosed with idiopathic nephrotic syndrome; single center experience.

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. MATERIALS AND METHOD: 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.

Malondialdehyde, antioxidant enzymes, and renal tubular functions in children with iron deficiency or iron-deficiency anemia.

We aimed to investigate the effects of iron deficiency (ID) or iron-deficiency anemia (IDA) on oxidative stress and renal tubular functions before and after treatment of children. A total of 30 children with a diagnosis of IDA constituted the IDA group and 32 children with a diagnosis of ID constituted the ID group. Control group consisted 38 age-matched children. Serum ferritin, soluble transferrin receptor (sTfR), serum, and urinary sodium (Na), potassium (K), calcium (Ca), phosphorus (P), creatinine (Cr), uric acid (UA), urinary N-acetyl-ß-D-glucosaminidase (NAG) levels, and intra-erythrocyte malondialdehyde (MDA), catalase (CAT), superoxide dismutase (SOD), and glutathione peroxidase (GSH-Px) levels were measured before and after iron therapy in the IDA and ID groups, whereas it was studied once in the control group. We have divided the study group in groups according to age (infants <2 years, children 3-9 years, and adolescents 10-15 years). Patients with IDA (infant, adolescent) and ID (infant, children, and adolescent) had a significantly high level of MDA in post-treatment period in comparison to those of healthy control. Patients with IDA (children, adolescent) and ID (infant, children) had a significantly high level of pre-treatment GSH-Px than controls. Post-treatment SOD was lower in IDA (children and adolescent) groups than control and post-treatment CAT was lower in IDA and ID (adolescent) groups than control. These findings show that ferrous sulfate used in the treatment of ID or IDA could lead to oxidative stress; however, a marked deterioration of in proximal renal tubular functions was not seen.

Cerebral vein thrombosis in a four year old with Behçet's disease.

Behçet's disease (BD) is a multisystem disorder. The main pathology in BD is vasculitis that involves arteries and veins of all calibers. Central nervous system involvement occurs in 5-10% of patients. Increased morbidity and mortality is rarely observed in children. The mean age at onset in pediatric BD is approximately 7 years. Neurologic involvement in BD is usually observed after 3-6 years. We report the case of a four-year-old Turkish boy with BD with sagittal sinus thrombosis treated with infliximab. The patient presented papilledema without neurologic signs. Although long-term efficacy evaluations are needed in this case, infliximab therapy may be a good option in childhood BD with refractory sinus thrombosis. This is the youngest case of BD with sagittal sinus thrombosis reported so far.

QT and JT dispersion and cardiac performance in children with neonatal Bartter syndrome: a pilot study.

BACKGROUND: QT dispersion and JT dispersion are simple noninvasive arrhythmogenic markers that can be used to assess the homogeneity of cardiac repolarization. The aim of this study was to assess QT and JT dispersion and their relation with left ventricular systolic and diastolic functions in children with Bartter syndrome (BS). METHODS: Nine neonatal patients with BS (median age 9.7 years) and 20 controls (median age 8 years) were investigated at rest. Both study and control subjects underwent electrocardiography (ECG) in which the interval between two R waves and QT intervals, corrected QT, QT dispersion, corrected QT dispersion, JT, corrected JT, JT dispersion and corrected JT dispersion were measured with 12-lead ECG. Two-dimensional, Doppler echocardiographic examinations were performed. RESULTS: Patients and controls did not differ for gender and for serum levels of potassium, magnesium, and calcium (p > 0.05). Both study and control subjects had normal echocardiographic examination and baseline myocardial performance indexes. The QT dispersion and JT dispersion were significantly prolonged in patients with BS compared to those of the controls {37.5 ms [interquartile range (IQR) 32.5-40] vs. 25.5 ms (IQR 20-30), respectively, p = 0.014 and 37.5 ms (IQR 27.5-40) vs. 22.5 ms (IQR 20-30), respectively, p = 0.003}. CONCLUSIONS: Elevated QT and JT dispersion during asymptomatic and normokalemic periods may be risk factors for the development of cardiac complications and arrhythmias in children with BS. In these patients the need for systematic cardiac screening and management protocol is extremely important for effective prevention.

Evaluation of lipid peroxidation and antioxidant system in healthy iron-replete infants receiving iron prophylaxis.

OBJECTIVE: Iron supplementation is commonly recommended for infants; however, there are some reports that it causes oxidative damage. The aim of this study was to investigate the potential effects of iron supplementation at 4 mo of age, for a period of 2 mo, on lipid peroxidation and free radical scavenging enzymes. METHODS: Twenty-seven healthy 4-mo-old infants chosen randomly and given iron supplementation (ferrous sulfate, 10 mg of elemental iron per day) constituted the study group and 26 healthy 4-mo-old infants who were chosen randomly and not given iron supplementation constituted the control group. Weight, height, head circumference, complete blood cell count, serum ferritin level and intraerythrocytic zinc, iron, copper, malondialdehyde, catalase, superoxide dismutase, and glutathione peroxidase levels were measured in the two groups at 4 and 6 mo of life. RESULTS: Compared with controls at 6 mo of age, no significant differences were observed for intraerythrocytic zinc (0.5 ± 0.1 versus 0.6 ± 0.2 µg/mL, P > 0.05), copper (0.2 ± 0.1 versus 0.2 ± 0.2 µg/mL, P > 0.05), iron (130.8 ± 10.9 versus 127.4 ± 11.1 µg/mL, P > 0.05), malondialdehyde (21.4 ± 3.5 versus 22.4 ± 2.3 nmol/g of hemoglobin, P > 0.05), catalase (135.4 ± 23.9 versus 135.1 ± 23.3 MU/g of hemoglobin, P > 0.05), superoxide dismutase (1736.4 ± 141.1 versus 1701.3 ± 103.9 U/g of hemoglobin, P > 0.05), and glutathione peroxidase (8.9 ± 1.6 versus 8.4 ± 1.6 U/g of hemoglobin, P > 0.05) levels. CONCLUSION: Our study indicates that the supplemental use of elemental iron 10 mg/d for a period of 2 mo in healthy iron-replete infants did not cause lipid peroxidation or an impairment of antioxidant status.