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© 2021, IPNA.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.

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., NA

Various molecular and cellular processes are involved in renal fibrosis, such as oxidative stress, inflammation, endothelial cell injury, and apoptosis. Heat shock proteins (HSPs) are implicated in the progression of chronic kidney disease (CKD). Our aim was to evaluate changes in urine and serum HSP levels over time and their relationships with the clinical parameters of CKD in children. In total, 117 children with CKD and 56 healthy children were examined. The CKD group was followed up prospectively for 24 months. Serum and urine HSP27, HSP40, HSP47, HSP60, HSP70, HSP72, and HSP90 levels and serum anti-HSP60 and anti-HSP70 levels were measured by ELISA at baseline, 12 months, and 24 months. The urine levels of all HSPs and the serum levels of HSP40, HSP47, HSP60, HSP70, anti-HSP60, and anti-HSP70 were higher at baseline in the CKD group than in the control group. Over the months, serum HSP47 and HSP60 levels steadily decreased, whereas HSP90 and anti-HSP60 levels steadily increased. Urine HSP levels were elevated in children with CKD; however, with the exception of HSP90, they decreased over time. In conclusion, our study demonstrates that CKD progression is a complicated process that involves HSPs, but they do not predict CKD progression. The protective role of HSPs against CKD may weaken over time, and HSP90 may have a detrimental effect on the disease course. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12192-021-01239-9.

The aim of the study is to present incidence of COVID-19 in children with renal replacement therapy (RRT) and compare the severity and outcomes of the disease between the groups undergoing dialysis and kidney transplantation (KTx). This multicenter observational study was based on data collected from children receiving RRT, either chronic dialysis or KTx, diagnosed with COVID-19. All members of the Istanbul branch of the Turkish Pediatric Nephrology Association were requested to submit all their confirmed COVID-19 cases between April 1 and December 31, 2020. They were also asked to report the number of their prevalent patients on RRT under the age of 20. A total of 46 RRT patients diagnosed with COVID-19 were reported from 12 centers. Of these, 29 were KTx recipients, and 17 were on dialysis. COVID-19 cases represented 9.3% of all prevalent dialysis patients and 9.2% of all prevalent KTx recipients. Twelve KTx recipients (41.4%) and three dialysis patients (17.6%) were asymptomatic (p = 0.12). The hospitalization rate was higher in dialysis patients than KTx recipients (82.4% vs. 27.6%, p = 0.001). Two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. Acute kidney injury developed in 36% of KTx recipients, but none required dialysis or lost their graft. In the entire cohort, one patient on hemodialysis with multiple comorbidities died.Conclusion: Although most of cases were asymptomatic or had a mild disease, children on RRT seem to have an increased risk for COVID-19.

Tam Metin / Full Text Q4 PMID: 34174796 WOS:000724833600003 SCI-Expanded 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. Materials and 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. Conclusion: 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. Keywords: Bartter syndrome, hypercalciuria, nephrocalcinosis, renal tubular acidosis

Background/aim: this study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or -resistant) and the dosing regimen. Materials and methods: this multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m(2)) or high (2-4 doses of 375 mg/m(2)) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9-17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 +/- 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: the current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined., NA

OBJECTIVE In this study, we examined the patients' characteristics, who underwent voiding cystourethrography (VCUG), in order to determine any selectivity for indication of this invasive method. MATERIAL AND METHODS After exclusion of indications of neurogenic bladder or antenatal hydronephrosis and control VCUGs, 159 VCUGs performed in our clinic within one year were evaluated. Patients are divided into three groups accoding to age. Clinical characteristic and findings of renal ultrasonography (US) and renal scintigraphy were examined. RESULTS Vesicoureteral reflux (VUR) was detected in 61 (38.3%) of 159 patients who underwent cystourethrographic examinations, in 45.8% of the patients with a history of recurrent urinary tract infection (UTI), in 22.0% of the patients with pathological urinary system US without history of recurrent UTI. High-grade reflux rate was significantly more frequent in renal units with pathological US findings. Severe scar was significantly more frequent in renal units with high-grade reflux when compared to renal units without reflux and those with low-grade reflux. Predictive values of recurrent UTI, scarring status and pathological US for VUR were separately analyzed and seen that likelihood of indicating VUR was increased when all 3 risk factors were assessed together. CONCLUSION Vesicoureteral reflux is a problem in which diagnostic process and management strategy should have to be considered in individualized manner for each patient. Before prescribing invasive VCUG, imaging urinary system by US and scintigraphy and determining whether there is recurrent UTI will improve selectivity and success of VCUG.

Endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to examine whether there is endothelial dysfunction in children with familial Mediterranean fever (FMF), hypothesizing that endothelial dysfunction would be present especially with acute-phase response in the active period of the disease.This cross-sectional study included 65 FMF patients (41 attack free, 24 attack period) and 35 healthy controls. Circulating EMPs, serum amyloid A (SAA), and other inflammation markers were measured in all groups. Circulating EMPs were measured using flow cytometry. Study groups were compared for circulating EMP and inflammatory markers. The relationship between EMPs and the activation of the disease was evaluated.The levels of CD144Our results suggest that endothelial damage is present especially in the active period of the disease in children with FMF. The endothelial dysfunction becomes an overt parallel with inflammation.

Objective: Nail fold capillary changes are accepted as early symptoms of several rheumatic disorders such as scleroderma and dermatomyositis. In adults, nail fold capillary abnormality (NCA) has been described in Familial Mediterranean Fever (FMF). However, there is not enough study in children with FMF. The aim of the present study was (1) to investigate whether children with FMF have NCA in both active and/or remission period or not and (2) to assess insulin resistance in children with FMF in both active and/or remission period of the disease compared with the control group.

©2011 Turkish League Against Rheumatism. All rights reserved. Amac: Bu calismada Turk cocuklarda juvenil sistemik lupus eritematozusa (JSLE) iliskin temel klinik ve laboratuvar ozellikler ve yani sira morbidite ve mortalitenin tanimlanmasi amaclandi. Hastalar ve yontemler: Otuz JSLE’li cocuk (27 kiz, 3 erkek; ort. tani yasi 12.3 yil; dagilim 3-16 yil) retrospektif olarak degerlendirildi. Hastalarin hepsi SLE tanisina iliskin revize edilmis 1982 Amerikan Romatoloji Birligi kriterlerini karsilamaktaydi ve 16 yasindan once hastaligin klinik bulgularini sergilemisti. Bulgular: Ortalama takip suresi 2.9 yil (dagilim 2 ay-10 yil), tani oncesi semptomlarin ortalama suresi ise 3.3 ay (dagilim 2 gun-2 yil) idi. Hastalarin klinik ve laboratuvar ozelliklerinin sikligi soyle idi: eritematoz malar dokuntu 17 (%56), discoid dokuntu bir (%3.3), fotosensitivite 16 (%53.3), oral ulser sekiz (%26.7), kas-iskelet tutulumu 26 (%87), serozit 13 (%43), hematolojik anormallikler 21 (%70), renal tutulum 17 (%56), antinukleer antikor pozitifligi 27 (%93), pulmoner tutulum sekiz (%27), kardiyovaskuler hastalik yedi (%23) ve merkezi sinir sistemi tutulumu yedi (%23) hastada goruldu. Izlem sirasinda ucu bobrek yetmezliginden, biri sepsisten olmak uzere dort hasta oldu (%13). Calisma sirasinda iki hasta tedavisiz remisyonda, 22 hasta ise tedavi ile remisyonda takip edilmekte idi. Iki hasta ise takipten cikmis idi. Sonuc: Juvenil sistemik lupus eritematozus ciddi, multisistemik bir organ hastaligidir. JSLE erken tani ve tedavi ile yuksek bir remisyon orani sergilemesine karsin, hala onemli bir mortalite oranina sahiptir.

Background Obesity in children increases the risk of atherosclerosis. Endothelial dysfunction is an important factor in the pathogenesis of atherosclerosis, and endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to evaluate circulating EMPs in obese and overweight children and to disclose the measure of obesity with the strongest relation with circulating microparticles and carotid atherosclerosis. Methods This prospective study included 55 obese and overweight children and 23 healthy controls. Insulin resistance was studied. Both in vivo and in vitro human umbilical vein endothelial cell evaluations were used for the study. Circulating EMPs (CD144 and CD146) were measured by flow cytometry. The carotid artery intima-media thickness (cIMT) and left ventricular mass index (LVMI) were measured using ultrasound and echocardiography, respectively. Study groups were compared for anthropometric measurement, insulin resistance, circulating EMP, cIMT, and LVMI. The relationship among overweight, obesity, and circulating EMPs were investigated. Results Blood pressure, CD144+EMP levels, and LVMI were statistically higher in the patients group than in the control group. The multiple logistic regression analysis and the backward elimination method showed that CD144+EMP and systolic blood pressure had a linear relationship with overweight and obesity. Conclusion Our results suggest that endothelial damage starts in the early stage of childhood obesity and that obese and overweight children have increased circulating CD144+EMPs, showing that endothelial dysfunction and increased CD144+EMPs may be related to obesity.

Patients with type 1 diabetes (T1D) are at increased risk for developing other autoimmune diseases, most commonly autoimmune thyroiditis and celiac disease. Few reports have described the association of systemic lupus erythematosus and T1D in the literature. To the best of our knowledge, this is the first report of lupus nephritis in a child with T1D.

Congenital nephrotic syndrome is a rare clinical entity defined as massive proteinuria leading to symptoms within the infant’s first 3 months of life. Although the association between congenital nephrotic syndrome and cytomegalovirus infection has been identified, association with haemophagocytosis has not been reported in the literature. In this case report we describe concomitant cytomegalovirus infection and haemophagocytosis in a 3-month-old girl with congenital nephrotic syndrome.

Primary cutaneous mucormycosis (MM) is a rare fungal infection of childhood and is most often encountered in immunocompromised patients. It is a potentially lethal opportunistic fungal infection with rapid progression and high mortality. A report of cutaneous MM involving the head region is very rare. We herein report a case of primary cutaneous MM in a malnourished patient. The infection progressed rapidly, and the infant died from infection. The diagnosis was made at postmortem examination. Early diagnosis and surgery should be undertaken to prevent fatal outcome, and complete study of the etiologic agent must be carried out in all cases.

Hypertensive crisis is thought to be initiated by an abrupt increase in systemic vascular resistance, likely related to humoral vasoconstrictors. Left ventricular dilatation and failure may develop as a result of increased afterload due to hypertension. Left heart failure may result in secondary pulmonary hypertension by increasing pulmonary venous pressure. Here, we report the effects of sodium nitroprusside for treatment of hypertensive crisis complicated with acute left ventricular dilatation, heart failure, and pulmonary hypertension secondary to vesicoureteral reflux in a 10-year-old female patient. This article presents a complex clinical case treated by simple medication. Key Words: Hypertensive crisis, dilated cardiomyopathy, pulmonary hypertension, sodium nitroprusside, heart failure, vesicoureteral reflux

Case A-7 year old boy was admitted with complaints of edema on his eyelid and lower extremity. On admission, he had edema, hepatosplenomegaly, proteinuria and hypoalbuminemia. So his diagnosis was accepted as nephrotic syndrome and steroid therapy was started. Since he did not respond steroid therapy, kidney biopsy was performed. Biopsy findings were consistent with amyloidosis. Steroid therapy was ceased and colchicine was started. The patient was reevaluated for autoinflammatory diseases. His parent told that he had slightly periodic fever and he had no abdominal pain, arthritis, pleuritis and erysipelas-like erythema. MEFV mutation was normal. Other autoinflammatory syndromes were investigated and he had elevated serum IgD concentration. Mevalonate kinase gene mutation was positive for G326R/V377I. His diagnosis was Hyperimmunoglobulin D syndrome (HIDS). Having a poor response to colchium therapy, anti-TNF therapy (etanercept) was planned.

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system, and may lead to significant morbidity and even mortality. Childhood-onset SLE (cSLE) is a rare disease with an incidence of 0.3-0.9 per 100.000 children-years and a prevalence of 3.3-8.8 per 100.000 children.