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BackgroundCongenital anomalies of the kidney and urinary tract (CAKUT) are the predominant cause for chronic kidney disease below 30 years of age. Many monogenic forms have been discovered mainly due to comprehensive genetic testing like exome sequencing (ES). However, disease-causing variants in known disease-associated genes still only explain a proportion of cases. Aim of this study was to unravel the underlying molecular mechanism of syndromic CAKUT in two multiplex families with presumed autosomal recessive inheritance.Methods and ResultsES in the index individuals revealed two different rare homozygous variants inFOXD2,a transcription factor not previously implicated in CAKUT in humans: a frameshift in family 1 and a missense variant in family 2 with family segregation patterns consistent with autosomal-recessive inheritance. CRISPR/Cas9-derivedFoxd2knock-out (KO) mice presented with bilateral dilated renal pelvis accompanied by renal papilla atrophy while extrarenal features included mandibular, ophthalmologic, and behavioral anomalies, recapitulating the phenotype of humans withFOXD2dysfunction. To study the pathomechanism ofFOXD2-dysfunction-mediated developmental renal defects, in a complementary approach, we generated CRISPR/Cas9-mediated KO ofFoxd2in ureteric-bud-induced mouse metanephric mesenchyme cells. Transcriptomic analyses revealed enrichment of numerous differentially expressed genes important in renal/urogenital development, includingPax2andWnt4as well as gene expression changes indicating a cell identity shift towards a stromal cell identity. Histology ofFoxd2KO mouse kidneys confirmed increased fibrosis. Further, GWAS data (genome-wide association studies) suggests thatFOXD2could play a role for maintenance of podocyte integrity during adulthood.ConclusionsIn summary, our data implicate thatFOXD2dysfunction is a very rare cause of autosomal recessive syndromic CAKUT and suggest disturbances of the PAX2-WNT4 cell signaling axis contribute to this phenotype.

The kidney is the second most commonly affected organ by severe acute respiratory syndrome coronavirus-2, characterized by hematuria, proteinuria, and acute kidney injury. There are few studies describing renal involvement in pediatric cases.This retrospective study evaluated the prevalence of hematuria, proteinuria, and acute kidney injury in severe acute respiratory syndrome coronavirus-2-positive pediatric cases (1-18 years old) who visited emergency department between March and November 2020. Patients with urinary tract infections were excluded. An age-specific upper limit of reference interval was used to define "elevated serum creatinine" (greater than upper limit of reference interval) and acute kidney injury (1.5 times upper limit of reference interval).A total of 228 patients were evaluated, median age was 12.7 years (interquartile range: 7.5; 16.1), and 51.3% were male. The prevalence of asymptomatic, mild, and moderate-to-severe disease was 12.7% (29/228), 77.2% (176/228), and 10.1% (23/228), respectively. The prevalence of hematuria, proteinuria, and elevated serum creatinine was 15.8% (36/228), 6% (14/228), and 3% (7/228), respectively. Kidney involvement (i.e., at least 1 of these findings) was 23.2% (53/228) and significantly higher in the moderate-to-severe disease (43.5%). None of the patients met the acute kidney injury criterion. In logistic regression analysis, female sex (odds ratio: 1.97, 95 CI%: 1.03; 3.70, P = .040) and fever (odds ratio: 2.28, 95% CI: 1.19; 4.36, P = .012) were independent predictors of kidney involvement. Three patients demonstrated a kidney presentation (macroscopic hematuria) on admission, and another patient was diagnosed with C3 glomerulonephritis during hospitalization.Kidney involvement was found about in 1 quarter of children with coronavirus disease 2019. Awareness and recognition of kidney involvement and follow-up are important in the management.

© 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.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.

Atmosferde konsantrasyonu artan sera gazlarının neden olduğu küresel ısınma yirminci yüzyılın son çeyreğinden itibaren dünyanın en önemli çevre sorunu haline gelmiştir. Sıcaklık artışının sadece yağış toplamlarında değil aynı zamanda ekstrem yağışlarda değişimlere, daha sık ve şiddetli sel ve kuraklık hadiselerine yol açması beklenmektedir. Küresel ısınma bağlamında, ülkemizdeki yağış toplamlarındaki değişimler oldukça geniş çaplı araştırma konusu olmasına karşın yağış şiddetlerindeki değişimler yeterince incelenmemiştir. Bu çalışmada, ülkemizin batısında yer alan 13 ildeki 32 istasyon için Meteoroloji Genel Müdürlüğü’nden sağlanan günlük yağış verileri, hafif yağış, orta kuvvette yağış, kuvvetli yağış, çok kuvvetli yağış, şiddetli yağış ve aşırı yağış olmak üzere altı farklı kategoriye ayrılmış ve her bir kategori için üç farklı indisin (yağışlı gün sayısı, yağış miktarı, kategorideki yağışın toplam yağışa oranı) mevsimlik ve yıllık ölçekte 1966 ile 2011 arasındaki zamansal değişimleri ile çalışma alanındaki konumsal değişimleri incelenmiştir. Çalışma sonucunda, yaz mevsiminde çalışma alanının kuzeyinin, diğer mevsimlerde ise güneyinin daha fazla sel riskine sahip olduğu saptanmıştır. Zamansal değişim analizinde ise, hafif yağış kategorisinde yıllık ölçekte yağışlı gün sayısı, yağış miktarı ve kategorideki yağışın toplam yağışa oranı için saptanan yaygın azalış eğilimleri haricinde diğer tüm kategori-indis-ölçek kombinasyonlarında %95 düzeyinde istatistiksel olarak önemli bir değişim saptanmamıştır. Başka bir anlatımla, sıcaklık artışına rağmen ülkemizin batı kesimlerinde üst kategori yağışlarda önemli bir değişim meydana gelmediği saptanmıştır.

Predictors of early diagnosis and severe infection in children with coronavirus disease 2019 (COVID-19), which has killed more than 4 million people worldwide, have not been identified. However, some biomarkers, including cytokines and chemokines, are associated with the diagnosis, pathogenesis and severity of COVID-19 in adults. We examined whether such biomarkers can be used to predict the diagnosis and prognosis of COVID-19 in pediatric patients. Eighty-nine children were included in the study, comprising three patient groups of 69 patients (6 severe, 36 moderate and 27 mild) diagnosed with COVID-19 by real-time polymerase chain reaction observed for 2–216 months and clinical findings and 20 healthy children in the same age group. Hemogram, coagulation, inflammatory parameters and serum levels of 16 cytokines and chemokines were measured in blood samples and were analyzed and compared with clinical data. Interleukin 1-beta (IL-1β), interleukin-12 (IL-12) and interferon gamma-induced protein 10 (IP-10) levels were significantly higher in the COVID-19 patients (p = 0.035, p = 0.006 and p < 0.001). Additionally, D-dimer and IP-10 levels were higher in the severe group (p = 0.043 for D-dimer, area under the curve = 0.743, p = 0.027 for IP-10). Lymphocytes, C-reactive protein and procalcitonin levels were not diagnostic or prognostic factors in pediatric patients (p = 0.304, p = 0.144 and p = 0.67). Increased IL-1β, IL-12 and IP-10 levels in children with COVID-19 are indicators for early diagnosis, and D-dimer and IP-10 levels are predictive of disease severity. In children with COVID-19, these biomarkers can provide information on prognosis and enable early treatment.

Air cargo transport is a growing industry in parallel with world trade growth and increasing e-commerce. It is performed by the passenger aircraft belly and the pure cargo air crafts, i.e., the freighters. Assignment of Unit Load Devices (ULDs) to the specific positions on the freighter is performed by loadmasters by manual or semimanual methods. As the global air cargo transport traffic gets busier, the importance of timely loading with minimum error increases. Therefore, digitalization of the air cargo loading process is needed. This study aimed to write a program that automatically performs the ULD assignment by simulating the loading process performed by the experienced loadmasters and considering the weight and balance constraints. The SEMMA (sample, explore, modify, model, assess) model is used while analyzing the data. Fifty real-world setting data were used to evaluate the program. The ULD assignment by the program and the loadmasters by semi-manual loading were compared regarding time and center of gravity by the Mann Whitney U test. The results demonstrated that the program could load all the given sets of ULDs, as efficiently as a loadmaster with a similar center of gravity in a shorter period of time. In conclusion, digitalization of the ULD assignment is possible, which needs to be improved to cover all the constraints. Hava kargo taşımacılığı, dünya ticaretinin büyümesine ve artan e-ticarete paralel olarak büyüyen bir sektördür. Yolcu uçaklarının bagaj kısmı ve kargo uçakları ile gerçekleştirilir. Birim yük cihazlarının (unit load devices; ULDs) kargo uçağındaki belirli pozisyonlara atanması, manuel veya yarı manuel yöntemlerle loadmasterlar tarafından gerçekleştirilir. Küresel hava kargo taşımacılığı trafiği yoğunlaştıkça, minimum hata ile zamanında yükleme yapmanın önemi giderek artmaktadır. Bu nedenle hava kargo yükleme sürecinin dijitalleştirilmesine ihtiyaç duyulmaktadır. Bu çalışmada, ağırlık ve denge kısıtlamalarını gözeten ve deneyimli loadmasterlar tarafından gerçekleştirilen yükleme işlemini benzeten, ULD atamasını otomatik olarak gerçekleştiren bir program yazmayı amaçladık. Veriler analiz edilirken SEMMA (örnekle, araştır, değiştir, modelle, değerlendir) modeli kullanıldı. Programı değerlendirmek için elli tane gerçek veri kullanıldı. Program ve loadmaster tarafından yapılan (yarı manuel) ULD atanması işlemi, süre ve ağırlık merkezi açısından Mann Whitney U testi ile karşılaştırıldı. Programın verilen tüm ULD setlerini belirlenen güvenlik ve stabilite sınırları içinde yerleştirdiği görüldü. Program yüklemeleri daha kısa sürede gerçekleştirirken ağırlık merkezi load mastera göre benzerdi. Sonuç olarak, ULD atanma işleminin dijitallejmesi mümkündür ancak tüm kısıtlamaları kapsayacak şekilde geliştirilmesi gerekmektedir.

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.

Background: Patients on dialysis have a high burden of bone-related comorbidities, including fractures. We report a post hoc analysis of the prospective cohort study HDF, Hearts and Heights (3H) to determine the prevalence and risk factors for chronic kidney disease-related bone disease in children on hemodiafiltration (HDF) and conventional hemodialysis (HD). Methods: The baseline cross-sectional analysis included 144 children, of which 103 (61 HD, 42 HDF) completed 12-month follow-up. Circulating biomarkers of bone formation and resorption, inflammatory markers, fibroblast growth factor-23, and klotho were measured. Results: Inflammatory markers interleukin-6, tumor necrosis factor-alpha, and high-sensitivity C-reactive protein were lower in HDF than in HD cohorts at baseline and at 12 months (P < .001). Concentrations of bone formation (bone-specific alkaline phosphatase) and resorption (tartrate-resistant acid phosphatase 5b) markers were comparable between cohorts at baseline, but after 12-months the bone-specific alkaline phosphatase/tartrate-resistant acid phosphatase 5b ratio increased in HDF (P = .004) and was unchanged in HD (P = .44). On adjusted analysis, the bone-specific alkaline phosphatase/tartrate-resistant acid phosphatase 5b ratio was 2.66-fold lower (95% confidence interval, -3.91 to -1.41; P < .0001) in HD compared with HDF. Fibroblast growth factor-23 was comparable between groups at baseline (P = .52) but increased in HD (P < .0001) and remained unchanged in HDF (P = .34) at 12 months. Klotho levels were similar between groups and unchanged during follow-up. The fibroblast growth factor-23/klotho ratio was 3.86-fold higher (95% confidence interval, 2.15-6.93; P < .0001) after 12 months of HD compared with HDF. Conclusion: Children on HDF have an attenuated inflammatory profile, increased bone formation, and lower fibroblast growth factor-23/klotho ratios compared with those on HD. Long-term studies are required to determine the effects of an improved bone biomarker profile on fracture risk and cardiovascular health., Kidney Research UK; National Institute for Health Research, Career Development Fellowship; National Institute for Health Research; Fresenius Medical Care, The 3H study was sponsored by Kidney Research UK. Part sponsorship was obtained from Fresenius Medical Care, who approved the study protocol, but had no role in data collection, data analysis, or drafting the manuscript. RS is funded by a National Institute for Health Research, Career Development Fellowship for this research project. This publication presents independent research funded by the National Institute for Health Research. The views expressed are those of the author (s) and not necessarily those of the National Health Service, the National Institute for Health Research, or the Department of Health and So-cial Care. A part of the work took place in the Biomedical Research Centre at Great Ormond Street Hospital for Children National Health Service Foundation Trust and University College London.

Background Hypertension is prevalent in children on dialysis and associated with cardiovascular disease. We studied the blood pressure (BP) trends and the evolution of BP over 1 year in children on conventional hemodialysis (HD) vs. hemodiafiltration (HDF). Methods This is a post hoc analysis of the "3H - HDF-Hearts-Height" dataset, a multicenter, parallel-arm observational study. Seventy-eight children on HD and 55 on HDF who had three 24-h ambulatory BP monitoring (ABPM) measures over 1 year were included. Mean arterial pressure (MAP) was calculated and hypertension defined as 24-h MAP standard deviation score (SDS) >= 95th percentile. Results Poor agreement between pre-dialysis systolic BP-SDS and 24-h MAP was found (mean difference - 0.6; 95% limits of agreement -4.9-3.8). At baseline, 82% on HD and 44% on HDF were hypertensive, with uncontrolled hypertension in 88% vs. 25% respectively; p < 0.001. At 12 months, children on HDF had consistently lower MAP-SDS compared to those on HD (p < 0.001). Over 1-year follow-up, the HD group had mean MAP-SDS increase of +0.98 (95%CI 0.77-1.20; p < 0.0001), whereas the HDF group had a non-significant increase of +0.15 (95%CI -0.10-0.40; p = 0.23). Significant predictors of MAP-SDS were dialysis modality (beta = +0.83 [95%CI +0.51 - +1.15] HD vs. HDF, p < 0.0001) and higher inter-dialytic-weight-gain (IDWG)% (beta = 0.13 [95%CI 0.06-0.19]; p = 0.0003). Conclusions Children on HD had a significant and sustained increase in BP over 1 year compared to a stable BP in those on HDF, despite an equivalent dialysis dose. Higher IDWG% was associated with higher 24-h MAP-SDS in both groups., Kidney Research UKKidney Research UK (KRUK); Fresenius Medical Care, The 3H study was sponsored by Kidney Research UK. Part sponsorship was obtained from Fresenius Medical Care, who approved the study protocol, but had no role in data collection, data analysis or drafting the manuscript.

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

Vitamin D has an immunomodulating property that regulates the inflammatory response. In this study, the aim was to evaluate the relationship between vitamin D levels and clinical severity and inflammation markers in children and adolescents with COVID-19. The clinical and laboratory records of 103 pediatric cases with COVID-19, whose vitamin D levels had been measured, were retrospectively reviewed. The cases were divided into groups according to their clinical severity (asymptomatic, mild, and moderate-to-severe) and vitamin D levels. The moderate-to-severe clinical group had significantly higher inflammation markers (CRP, procalcitonin, fibrinogen, d-dimer) and a lower lymphocyte count compared to both the mild and asymptomatic groups. The 25 OH vitamin D levels were also significantly lower (p < 0.001), and the ratio of vitamin D deficiency was 70.6% in the moderate-to-severe group. The vitamin D–deficient group had a significantly higher age and fibrinogen levels while also having a lower lymphocyte count compared to the insufficient and normal groups. The 25 OH vitamin D level was correlated positively with the lymphocyte count (r = 0.375, p =

Background and Aims Alport syndrome (AS) is an inherited glomerular basement membrane disease caused by mutations in COL4A3, COL4A4 or COL4A5 genes. Recently, it has been reported that focal segmental glomerulosclerosis (FSGS) can be seen in AS and COL4A mutations can be detected in FSGS. In this study, we aimed to define the clinical characteristics of patients with genetically confirmed AS, in order to establish genotype-phenotype correlation and investigate the effects of different treatment regimes. Method A total of 87 pediatric AS patients included in this multicenter study. We retrospectively collected data from medical records and requested other centers to fill out a questionnaire, which included data regarding patient demographic features, family history, clinical and laboratory characteristics at first presentation, histopathological (if available) and genetic tests results, treatments and yearly follow-up results. Results A total of 87 (41 female, 46 male) genetically confirmed AS patients (COL4A5, n=43; COL4A3, n=25; COL4A4, n=19) were studied. Mean age at first presentation was 7.6±4.1 years and the median follow-up duration was 4.3 years (IQR 1.9–7.3). 14 (16.1%) of 87 patients presented with nephrotic syndrome (NS); renal biopsy findings showed FSGS in 11 (79%) of 14 patients with NS, and COL4A3 mutations were the most common (n=7, 50%) in this group. Of 14 NS patients, 12 received steroid, 11 received cyclosporine (CsA) and 4 received other immunosuppressives prior to genetic diagnosis. The inheritance pattern of the patients with NS was consistent with ARAS in 10 patients (71.4%), XLAS in 3 patients (21.4%), and ADAS in 1 patient (7.2%). During follow up, glomerular filtration rate (GFR) decreased below 90 ml/min/1.73 m2 in 24 of 87 patients (27.5%). COL4A3 mutations (n=14, 58.3%) were the leading genetic abnormality in patients who progressed to chronic kidney disease (CKD). At the last visit, GFR loss was significantly higher in patients with COL4A3 mutations when compared to patients with COL4A4 and COL4A5 mutations (p=0.04). Among patients with NS, 9 of 14 (64.2%) progressed to CKD. Genetic results of patients with NS who progressed to CKD were COL4A3 in 6 (66.7%), COL4A4 in 2 (22.2%) and COL4A5 in 1 (11.1%) patients. In survival analysis, renal survival rate without CKD was 12.1 years (95% CI: 6.7-17.5). After the first presentation, the 5-year cumulative risk of CKD was 51.8%, 12.6%, and 12.9% in patients with COL4A3, COL4A4 and COL4A5 mutations, respectively (p=0.001). We observed that patients with COL4A3 mutations, ARAS inheritance pattern, histopathology of FSGS or NS presentation progressed to CKD earlier (p Conclusion Detailed analyses of data from genetically confirmed Turkish patients with AS provided important clues regarding to the presentation, course and outcomes of the disease. COL4A3 mutations, ARAS inheritance pattern, NS presentation and FSGS finding in renal biopsy are major risk factors for progression to CKD. We recommend genetic testing for patients suspected of having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid resistant NS. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications.

Amac: Enurezisli cocuklar ve annelerindeki anksiyete durumunu degerlendirmek Gerec ve Yontem: Arastirma 96 cocuk (52 enuretik cocuk, 44 saglikli cocuk) ve annelerinden olusmaktadir. Cocuklarda Cocukluk Cagi Anksiyete Tarama Olcegi, annelerde Anlik ve Surekli Anksiyete Olcekleri kullanilarak anksiyete duzeyleri degerlendirildi. Bulgular: Enuretik hastalarin cocukluk cagi anksiyete tarama olcegi (CATO) puani ortalamasi kontrol grubundan daha yuksekti (32,5±10,7 ve 26,7±10,6 p=0,009). Enuretik cocuklarin annelerinin Surekli Anksiyete Olcek puani ortalamasi kontrol grubundaki annelerin puanindan daha yuksek bulundu (49,4±8,8 ve 42,4±6,8 p=0,001). Sonuc: Arastirmamizda PEN’li cocuklar ve annelerinde anksiyete duzeyleri kontrol grubuna gore yuksek bulundu. Gece idrar kacirma, cocuklari ve anneleri etkileyen onemli bir sorundur. Enuretik cocuklarda ve annelerinde anksiyetenin taninmasi ve yonetimi basarili bir tedavi icin oldukca onemlidir.

What is Known? • Vitamin D has multiple roles in the immune system that can modulate the body reaction to an infection • Vitamin D binding protein (DBP) is the key transport protein which, along with albumin, binds over 99% of the circulating vitamin D metabolites What is New? • Lower 25 OH vitamin D levels were associated with higher inflammation markers, suggesting an important role of vitamin D in the clinical course of COVID-19 in children and adolescents probably by regulating the systemic inflammatory response • Further studies are warranted to investigate the possible causal association of DBP levels and polymorphism with vitamin D status (total and bioavailable vitamin D) in COVID-19 patients.

Atmosferdekonsantrasyonu artan sera gazlarının neden olduğu küresel ısınma yirminciyüzyılın son çeyreğinden itibaren dünyanın en önemli çevre sorunu halinegelmiştir. Sıcaklık artışının sadece yağış toplamlarında değil aynı zamandaekstrem yağışlarda değişimlere, daha sık ve şiddetli sel ve kuraklıkhadiselerine yol açması beklenmektedir. Küresel ısınma bağlamında, ülkemizdekiyağış toplamlarındaki değişimler oldukça geniş çaplı araştırma konusu olmasınakarşın yağış şiddetlerindeki değişimler yeterince incelenmemiştir. Buçalışmada, ülkemizin batısında yer alan 13 ildeki 32 istasyon için MeteorolojiGenel Müdürlüğü’nden sağlanan günlük yağış verileri, hafif yağış, orta kuvvette yağış, kuvvetliyağış, çok kuvvetli yağış, şiddetli yağış ve aşırı yağış olmak üzere altı farklı kategoriye ayrılmış ve her bir kategori için üçfarklı indisin (yağışlı gün sayısı, yağış miktarı, kategorideki yağışın toplamyağışa oranı) mevsimlik ve yıllık ölçekte 1966 ile 2011 arasındaki zamansaldeğişimleri ile çalışma alanındaki konumsal değişimleri incelenmiştir.Çalışma sonucunda, yaz mevsiminde çalışma alanının kuzeyinin, diğer mevsimlerdeise güneyinin daha fazla sel riskine sahip olduğu saptanmıştır. Zamansaldeğişim analizinde ise, hafif yağış kategorisinde yıllık ölçekte yağışlı gün sayısı, yağış miktarı ve kategorideki yağışın toplamyağışa oranı için saptanan yaygın azalış eğilimleri haricinde diğer tümkategori-indis-ölçek kombinasyonlarında %95 düzeyinde istatistiksel olarakönemli bir değişim saptanmamıştır. Başka bir anlatımla, sıcaklık artışınarağmen ülkemizin batı kesimlerinde üst kategori yağışlarda önemli bir değişimmeydana gelmediği saptanmıştır., Global warmingassociated with the increase of greenhouse gases in the atmosphere has becomethe most important environmental problem of the World since the last quarter of20th century. Temperature increase is expected to result in changes not only intotal rainfall amounts but also in extreme rainfall events, more frequent andintense flood and drought events. Under changing climate although how totalrainfall amounts have changed in Turkey are extensively investigated, changesin rainfall intensities are not adequately examined. In this study, dailyrainfall data recorded between 1966 and 2011 at 32 rainfall gaging stationslocated in 13 provinces of western Anatolia, Turkey, were supplied from theState Meteorological Service (MGM) and categorized into six classes, namelylight, light-moderate,moderate-heavy, heavy, heavy-torrential and torrential. Then, for eachcategory, spatial and monotonic temporal changes of three indices (number ofrainy days, rainfall amount and the ratio of rainfall amount to total rainfall)were analyzed in seasonal and annual scales. Results showed that flood risk ismore likely in northern part in summer and in southern part in other seasons. Temporalanalysis showed that there is no statistically significant (at 95% level)monotonic trend for all combinations of category-index-temporal scale, except anumber of decreasing trends in light rainfall. It can be concluded that there hasbeen no statistically significant change in daily rainfall intensities overwestern Anatolia (Turkey) over the period 1966–2011 althoughaverage temperature has increased.

Caliskan, Salim/0000-0002-3316-8032; Canpolat, Nur/0000-0002-3420-9756; Agbas, Ayse/0000-0002-3658-8622 WOS:000488496400007 PubMed ID: 31246513 Background: This study aimed to evaluate the cardiometabolic risk factors in normotensive obese and hypertensive obese (HT-obese) children by comparison of anthropomorphic measurements, fat distribution, carotid artery intima-media thickness (CIMT), and inflammatory markers. Methods: Fifty-three obese patients 10-18 years of age with a BMI-for-age/gender >95th percentile and 20 age- and gender-matched healthy volunteers enrolled in the study. Obese patients were divided into two groups according to the presence of hypertension (HT), as follows: HT-obese subgroup (n = 30) and nonhypertensive obese (non-HT-obese) subgroup (n = 23). Results: Weight standard deviation score (SDS), BMI-SDS, waist circumference (WC) SDS, and the fat tissue z-score were significantly higher (p < 0.001 for all) in the obese patients than the control groups. Obese patients had higher 24-hour systolic blood pressure (SBP) SDS and leptin, high-sensitivity C-reactive protein, tumor necrosis factor-alpha, and interleukin-6 levels. Furthermore, CIMT and CIMT-SDS were significantly higher in them. HT-obese patients (n = 30) had significantly higher WC-SDS and lower serum leptin and adiponectin levels than those of non-HT-obese group (n = 23). Finally, an association between increased CIMT-SDS and WC-SDS (beta = 0.399, p = 0.002) and 24-hour SBP-SDS (beta = 0.272, p = 0.009) was shown. Conclusions: Association between increased WC and HT implies the importance of central obesity in atherosclerosis. We concluded that WC measurement could be used to define risk groups since it is related to cardiometabolic complications. scientific research unit of Istanbul University [5308, 24263] The authors sincerely thank all dialysis nurses for their help during the data collection. The study was funded by scientific research unit of Istanbul University, (Project ID: 5308, project code: 24263).

Atmosferde konsantrasyonu artan sera gazlarının neden olduğu küresel ısınma yirminci yüzyılın son çeyreğinden itibaren dünyanın en önemli çevre sorunu haline gelmiştir. Sıcaklık artışının sadece yağış toplamlarında değil aynı zamanda ekstrem yağışlarda değişimlere, daha sık ve şiddetli sel ve kuraklık hadiselerine yol açması beklenmektedir. Küresel ısınma bağlamında, ülkemizdeki yağış toplamlarındaki değişimler oldukça geniş çaplı araştırma konusu olmuş olmasına karşın yağış şiddetlerindeki değişimler yeterince incelenmemiştir. Bu eksikliğin giderilmesine katkıda bulunmak amacıyla, bu çalışmada, ülkemizin batısındaki 13 ildeki 32 istasyon için Meteoroloji Genel Müdürlüğü'nden sağlanan günlük yağış verileri, hafif yağış (0,1 - 3,9 mm), orta kuvvette yağış (4,0 - 15,9 mm), kuvvetli yağış (16,0 - 31,9 mm), çok kuvvetli yağış (32,0 - 63,9 mm), şiddetli yağış (64,0 - 127,9 mm) ve aşırı yağış (≥128 m) olmak üzere altı farklı kategoriye ayrılmış ve her bir kategori için üç farklı indisin (yağışlı gün sayısı, yağış miktarı, kategorideki yağışın toplam yağışa oranı) mevsimlik ve yıllık ölçekte 1966 ile 2011 arasındaki zamansal değişimleri ile çalışma alanındaki konumsal değişimleri incelenmiştir. Çalışma sonucunda, mekansal değişimin kategori-indis-zamansal ölçek bazında farklılık gösterdiği saptanmıştır. Zamansal değişim analizinde ise, hafif yağış kategorisinde yıllık ölçekte yağışlı gün sayısı, yağış miktarı ve kategorideki yağışın toplam yağışa oranı için saptanan yaygın azalış eğilimleri haricinde diğer tüm kategori-indis-ölçek kombinasyonlarında %95 düzeyinde istatistiksel olarak önemli bir değişim saptanmamıştır. Başka bir anlatımla, sıcaklık artışının ülkemizin batı kesimlerinde günlük yağış şiddetinde önemli bir değişime neden olmadığı söylenebilir. Global warming associated with the increase of greenhouse gases in the atmosphere has become the most important environmental porblem of the World since the last quarter of 20th century. Temperature increase is expected to result in changes not only in total rainfall amounts but also in extreme rainfall events, more frequent and intense flood and drought events. Under changing climate although how total rainfall amounts have changed in Turkey are extensively investigated, changes in rainfall intensities are not adequately examined. To fill this gap, in this study, daily rainfall data recorded between 1966 and 2011 at 32 rainfall gaging stations located in 13 provinces of western Anatolia, Turkey, were supplied from State Metetorological Service (MGM) and categorized into six classes, namely light (0,1-3,9 mm), light-moderate (4-15,9 mm), moderate-heavy (16-31,9 mm), heavy (32-63,9 mm), heavy-torrential (64-127,9 mm), and torrential (128-up mm). Then, for each category, spatial and monotonic temporal changes of three indices (number of rainy days, rainfall amount and the ratio of rainfall amount to total rainfall) were analysed in seasonal and annual scales. Results showed that spatial patterns vary depending on the combination category-index-temporal scale. Temporal analysis showed that there is no statistically significant (at 95% level) monotonic trend for all combinations of category-index-temporal scale, except a number of decreaing trends in light rainfall. It can be concluded that temperature increase has not caused any significant change in daily rainfall intensisities over western Anatola, Turkey over the period 1966-2011. 135

BACKGROUND: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. METHODS: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. RESULTS: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate. CONCLUSION: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.

Objective: To evaluate the anxiety levels in children and their mothers with enuresis. Material and Method: The study population is consisted of 96 children (52 enuretic children, and 44 healthy children) and their mothers. Anxiety levels of the study population were evaluated by using the Screen for Child Anxiety and Related Disorders in children, the and State-Trait Anxiety Inventory in the mothers. Results: The mean score of SCARED was significantly higher in the Primary monosymptomatic nocturnal enuresis (PMNE) group compared to the healthy control group (32.5±10.7 vs 26.7±10.6 respectively, p=0.009). The STAI-2 score was significantly higher in the enuretic children’s mothers compared to mothers of the healthy control (49.4±8.8, 42.4±6.8 respectively, p=0.001). Conclusion: We demonstrated high anxiety levels in children with PMNE and in their mothers. Nocturnal enuresis is an important problem affecting the children and mothers. Recognition and management of anxiety in enuretic children and their mothers is crucial for a successful treatment., Amaç: Enürezisli çocuklar ve annelerindeki anksiyete durumunu değerlendirmek Gereç ve Yöntem: Araştırma 96 çocuk (52 enüretik çocuk, 44 sağlıklı çocuk) ve annelerinden oluşmaktadır. Çocuklarda Çocukluk Çağı Anksiyete Tarama Ölçeği, annelerde Anlık ve Sürekli Anksiyete Ölçekleri kullanılarak anksiyete düzeyleri değerlendirildi. Bulgular: Enüretik hastaların çocukluk çağı anksiyete tarama ölçeği (ÇATÖ) puanı ortalaması kontrol grubundan daha yüksekti (32,5±10,7 ve 26,7±10,6 p=0,009). Enüretik çocukların annelerinin Sürekli Anksiyete Ölçek puanı ortalaması kontrol grubundaki annelerin puanından daha yüksek bulundu (49,4±8,8 ve 42,4±6,8 p=0,001). Sonuç: Araştırmamızda PEN’li çocuklar ve annelerinde anksiyete düzeyleri kontrol grubuna göre yüksek bulundu. Gece idrar kaçırma, çocukları ve anneleri etkileyen önemli bir sorundur. Enüretik çocuklarda ve annelerinde anksiyetenin tanınması ve yönetimi başarılı bir tedavi için oldukça önemlidir.

EgeUn###, Background Hypertension and cardiovascular disease are common in children undergoing dialysis. Studies suggest that hemodiafiltration (HDF) may reduce cardiovascular mortality in adults, but data for children are scarce. Methods The HDF, Heart and Height study is a nonrandomized observational study comparing outcomes on conventional hemodialysis (HD) versus postdilution online HDF in children. Primary outcome measures were annualized changes in carotid intima-media thickness (cIMT) SD score and height SD score. Results We enrolled 190 children from 28 centers; 78 on HD and 55 on HDF completed 1-year follow-up. The groups were comparable for age, dialysis vintage, access type, dialysis frequency, blood flow, and residual renal function. At 1 year, cIMT SD score increased significantly in children on HD but remained static in the HDF cohort. On propensity score analysis, HD was associated with a +0.47 higher annualized cIMT SD score compared with HDF. Height SD score increased in HDF but remained static in HD. Mean arterial pressure SD score increased with HD only. Factors associated with higher cIMT and mean arterial pressure SD-scores were HD group, higher ultrafiltration rate, and higher 2-microglobulin. The HDF cohort had lower 2-microglobulin, parathyroid hormone, and high-sensitivity C-reactive protein at 1 year; fewer headaches, dizziness, or cramps; and shorter postdialysis recovery time. Conclusions HDF is associated with a lack of progression in vascular measures versus progression with HD, as well as an increase in height not seen in the HD cohort. Patient-related outcomes improved among children on HDF correlating with improved BP control and clearances. Confirmation through randomized trials is required., National Institute for Health ResearchNational Institute for Health Research (NIHR); Kidney Research UKKidney Research UK (KRUK); Fresenius Medical Care, R.S. holds a Career Development Fellowship with the National Institute for Health Research. The 3H study was sponsored by Kidney Research UK. Part sponsorship was obtained from Fresenius Medical Care.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic kidney disease may develop signs or symptoms during childhood, in particular hypertension. Although ambulatory BP monitoring is the preferred method to diagnose hypertension in pediatrics, data in children with autosomal dominant polycystic kidney disease are limited. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Our retrospective multicenter study was conducted to collect ambulatory BP monitoring recordings from patients with autosomal dominant polycystic kidney disease age 1 cm per kidney and daytime hypertension (odds ratio, 1.70; 95% confidence interval, 1.21 to 2.4; P=0.002), nighttime hypertension (odds ratio, 1.31; 95% confidence interval, 1.05 to 1.63; P=0.02), or 24-hour hypertension (odds ratio, 1.39; 95% confidence interval, 1.08 to 1.81; P=0.01). Kidney length, expressed as SD score, was also significantly associated with nighttime hypertension (odds ratio, 1.23; 95% confidence interval, 1.06 to 1.42; P=0.10). CONCLUSIONS: These data indicate high prevalence of hypertension in children with autosomal dominant polycystic kidney disease starting at young ages. ispartof: CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY vol:13 issue:6 pages:874-883 ispartof: location:United States status: published

WOS: 000441271500001, PubMed ID: 30097064, Background: Cardiovascular disease is prevalent in children on dialysis and accounts for almost 30% of all deaths. Randomised trials in adults suggest that haemodiafiltration (HDF) with high convection volumes is associated with reduced cardiovascular mortality compared to high-flux haemodialysis (HD); however paediatric data are scarce. We designed the haemodiafiltration, heart and height (3H) study to test the hypothesis that children on HDF have an improved cardiovascular risk profile, growth and nutritional status and quality of life, compared to those on conventional HD. We performed a non-randomised parallel-arm intervention study within the International Paediatric Haemodialysis Network Registry comparing children on HDF and conventional HD to determine annualised change in cardiovascular end-points and growth. Here we present the 3H study design and baseline characteristics of the study population. Methods: 190 children were screened and 177 (106 on HD and 71 on HDF) recruited from 28 centres in 10 countries. There was no difference in age, underlying diagnosis, comorbidities, previous dialysis therapy, dialysis vintage, residual renal function, type of vascular access or blood flow between HD and HDF groups. High flux dialysers were used in 63% of HD patients and ultra-pure water was available in 52%. HDF patients achieved a median convection volume of 13.3 L/m(2); this was associated with the blood flow rate only ((p = 0.0004, r = 0.42) and independent of access type (p = 038). Discussion: This is the largest study on dialysis outcomes in children that involves deep phenotyping across a wide range of cardiovascular, anthropometric, nutritional and health-related quality of life measures, to test the hypothesis that HDF leads to improved cardiovascular and growth outcomes compared to conventional HD., Kidney Research UKKidney Research UK (KRUK); Fresenius Medical Care; European Renal Association-European Dialysis and Transplant Association; Kuratorium fur Dialyse und Nierentransplantation (KfH) Foundation for Preventive Medicine; German Federal. Ministry of Education and ResearchFederal Ministry of Education & Research (BMBF); Baxter, This study was made possible by grants from Kidney Research UK and Fresenius Medical Care. 4C study has been funded by grants from the European Renal Association-European Dialysis and Transplant Association, the Kuratorium fur Dialyse und Nierentransplantation (KfH) Foundation for Preventive Medicine and the German Federal. Ministry of Education and Research.; The IPHN registry has been funded by Fresenius Medical Care and Baxter.