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Pediatric patients on kidney replacement therapy (KRT) are among the most vulnerable during large-scale disasters, either natural or man-made. Hemodialysis (HD) treatments may be impossible because of structural damage and/or shortage of medical supplies, clean water, electricity, and healthcare professionals. Lack of peritoneal dialysis (PD) solutions and increased risk of infectious/non-infectious complications may make PD therapy challenging. Non-availability of immunosuppressants and increased risk of infections may result in graft loss and deaths of kidney transplant recipients. Measures to mitigate these risks must be considered before, during, and after the disaster including training of staff and patients/caregivers to cope with medical and logistic problems. Soon after a disaster, if the possibility of performing HD or PD is uncertain, patients should be directed to other centers, or the duration and/or number of HD sessions or the PD prescription adapted. In kidney transplant recipients, switching among immunosuppressants should be considered in case of non-availability of the medications. Post-disaster interventions target treating neglected physical and mental problems and also improving social challenges. All problems experienced by pediatric KRT patients living in the affected area are applicable to displaced patients who may also face extra risks during their travel and also at their destination. The need for additional local, national, and international help and support of non-governmental organizations must be anticipated and sought in a timely manner.

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.

© 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 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.

Introduction: The objectives of this study were to determine the incidence of congenital anomalies of the kidney and urinary tract (CAKUT) in asymptomatic first-degree relatives of patients with a CAKUT diagnosis and to evaluate the benefits of such screening. Methods: Files of patients who were followed up at Cerrahpaşa Faculty of Medicine, Pediatric Nephrology Outpatient Clinic, Istanbul University-Cerrahpaşa between 1998 and 2016 were examined retrospectively and those with CAKUT were identified. These patients, and their asymptomatic first-degree relatives, were invited to participate in this study. Ultrasonography of the urinary tract was performed in siblings and parents of 145 CAKUT patients (index cases) who could be reached by phone and agreed to participate. Results: A total of 412 asymptomatic first-degree relatives of 145 index patients were screened by ultrasound. CAKUT was diagnosed in 23 individuals among the family members of 21 index subjects. Anomalies detected in asymptomatic first-degree relatives were renal agenesis (RA) and grade 3 hydronephrosis (n = 1), RA (n = 7), renal hypodysplasia (n = 7), grade 2 hydronephrosis (n = 1), and grade 1 hydronephrosis (n = 7). The frequency of familial CAKUT was 14.4%. Familial RA was found in 3 of the 5 families of index cases with RA. Conclusion: The ratio of familial CAKUT was 14.4%. The findings of the present study could not support a systematic family screening to all asymptomatic first-degree relatives; however, family screening with ultrasonography can be considered for children with RA.

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.