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Purpose: Horseshoe kidney is the most common fusion anomaly that congenital systemic and urological anomalies accompany most of the patients. In this study, we aimed to investigate its association with vascular anomalies and especially nutcracker syndrome along with accompanying urological and other systemic anomalies in children with horseshoe kidney. Materials and methods: Twenty-six patients are diagnosed with horseshoe kidney in our clinic and 22 healthy children of the same age and sex were included in the study. All children were prospectively evaluated using Doppler ultrasonography in terms of renal artery and renal vein flow velocities, lumen diameters and vascular anomalies and presence of nutcracker syndrome. Results: Urological anomaly was found in 50% of the children with horseshoe kidneys, and systemic anomaly in 44% of them. In Doppler ultrasonographic evaluations performed on patients to detect vascular pathologies and nutcracker syndrome; findings of nutcracker syndrome were present in 2 patients in the horseshoe kidney group, while they were detected incidentally in 1 patient in the control group. An accessory renal artery originating from the left common iliac artery was found in a case with horseshoe kidney, and a circumaortic left renal vein in one case. Conclusions: In our study in which we investigated nutcracker syndrome based on the presence of vascular anomalies accompanying horseshoe kidneys in children, nutcracker syndrome findings were found in similar numbers in both groups. However, we think that these children should be followed for a long time in terms of vascular pathologies (aneurysm, rupture) and malignancies that may occur in adulthood, as well as congenital vascular anomalies.

Amaç: Bu çalışmada Pamukkale Üniversitesi Çocuk Nefroloji Kliniği’nde ‘spinal disrafizm' nedeniyle izlenen çocuk hastaların üriner sistem bulgularının retrospektif olarak değerlendirilerek, böbrek hasarı sıklığı ve olası risk faktörlerini değerlendirmek amaçlanmıştır.Gereç ve yöntem: Ocak 2014 ile Ağustos 2021 tarihleri arasında ‘spinal disrafizme bağlı nörojen mesane’ tanısı ile izlenen çocuk hastaların verileri retrospektif olarak değerlendirildi. Hastaların demografik ve klinik özellikleri, temiz aralıklı katater (TAK) tedavisi alıp almadığı, alıyorsa başlanma zamanı ve son kontroldeki üriner sistem ultrasanografi bulguları (hidronefroz ve mesane duvar kalınlığı), vezikoüreteral reflü (VUR) varlığı, Dimerkaptosüksinik asit (DMSA) sintigrafi bulguları, Shwartz’a göre tahimini glomeruler filtrasyon hızı (eGFR) düzeyleri kayıt edildi.Bulgular: Çalışmaya 17 erkek, 27 kız olmak üzere toplam 44 hasta dahil edildi. Ortalama yaş 7,2±4,2 yaş olarak bulundu. Çocukların 20’si (%45,5) 5 yıldan uzun süredir takip edilmekteydi. Olgular arasında meningomyelosel (%88,6) en çok saptanan defekt olarak bulundu. Spinal disrafizme yönelik operasyon geçirmeyen sadece 2 hasta vardı. Olguların yaklaşık %90’ı 1 yaşından önce opere edilmişti. Hastaların yaklaşık %40’ında DMSA sintigrafide böbrekte hasar, %52’sinde hidronefroz, yaklaşık %16’sında ise eGFR’nin düşük olduğunu saptandı. Ürodinami bulgularında 18 (%40,9) hastada yüksek basınçlı mesane (mesane basıncı >40 cmH2O), 11 (%25) hastada arefleks mesane, 13 (%29,5) hastada normal ürodinami bulguları vardı. Onbeş (%34,1) hastada VUR vardı. Operasyon zamanı, TAK başlama yaşı ve gergin kord gelişimi ile böbrekte hasarlanma arasında anlamlı ilişki saptanmadı.Sonuç: Spinal disrafizm hastalarının ürolojik problemlerinin yönetiminde temel hedef üst üriner sistemin korunmasıdır. Bu hastalarda ürolojik problemlerin erken tanınıp tedavilerin başlanması yönünde öneriler olsa da halen bu hastaların yönetimi tartışmalı ve zordur., Purpose: It was aimed to evaluate the frequency of kidney damage and possible risk factors by retrospectively evaluating the urinary system findings of pediatric patients followed up for 'spinal dysraphism' in Pamukkale University Pediatric Nephrology Clinic.Materials and methods: The data of pediatric patients who were followed up with the diagnosis of neurogenic bladder due to spinal dysraphism between January 2014 and August 2021 were evaluated retrospectively. Demographic and clinical characteristics of the patients, whether they received clean intermittent catheter (CIC) treatment, if so, when to start and at the last follow-up, urinary system ultrasound findings (hydronephrosis and bladder wall thickness), presence of vesicoureteral reflux (VUR), dimercaptosuccinic acid scintigraphy findings, according to Shwartz Glomerular filtration rate (eGFR) levels were recorded.Results: A total of 44 patients, 17 male and 27 female, were included in the study. The mean age was 7.2±4.2 years. Twenty (45.5%) of the children were followed for more than 5 years. Among the cases, meningomyelocele (88.6%) was found to be the most common defect. There were only 2 patients who did not undergo surgery for spinal dysraphism. Approximately 90% of the cases were operated before 1 year of age. Approximately 40% of the patients were found to have kidney damage on DMSA scintigraphy, 52% had hydronephrosis, and approximately 16% had low eGFR. In urodynamic findings, 18 (40.9%) patients had high pressure bladder (bladder pressure >40 cmH2O), 11 (25%) patients had areflex bladder, 13 (29.5%) patients had normal urodynamic findings. Fifteen (34.1%) patients had VUR. There was no significant relationship between operation time, age at onset of CIC, tethered cord development and kidney damage.Conclusion: The main goal in the management of urological problems in patients with spinal dysraphism is the protection of the upper urinary tract. Although there are suggestions for early diagnosis and treatment of urological problems in these patients, the management of these patients is still controversial and difficult.

[No abstract available]

Cardiac involvement is very rare in patients with Henoch-Schonlein purpura (HSP). In this case study, we present an 8-year-old girl presenting with HSP-induced myocarditis and thrombus in the right atrium and HSP nephritis. To date, 15 cases of HSP-related cardiac involvement have been reported in the PubMed/MEDLINE, Scopus, and Google Scholar databases. These cases, together with our case, are included in this review. We excluded those patients with other rheumatologic diseases (acute rheumatic fever, acute post-streptococcal glomerulonephritis, Kawasaki disease) accompanied by HSP. Three were children and 13 were adults and all were male except our case. This review revealed tachyarrhythmia, chest pain, dyspnea, murmur, and heart failure as the major signs. Cardiac tests, electrocardiogram (ECG), and imaging methods (echocardiography in all patients, cardiac magnetic resonance imaging (MRI) in three, cardiac biopsy in one, and post-mortem necropsy in three) showed that the cardiac involvements were pericardial effusion, intra-atrial thrombus, myocarditis, coronary artery changes, myocardial ischemia, infarction and necrosis, subendocardial hemorrhage, and left ventricular dilatation. Kidney involvement was not observed in three patients. As the treatment, high-dose prednisolone and cyclophosphamide, oral corticosteroid, azathioprine, nadroparin calcium, ACE inhibitors, calcium antagonists, beta-blockers, and diuretics were used. Eleven patients (all three children and eight of the adults) had a complete cardiac recovery. Cardiac involvement in adults was more likely to be fatal. Death (three patients), ischemia, and infarct have been reported only in adults. We suggested that early and aggressive treatment can be life-saving. MRI examination is effective at identifying cardiac involvement.

Aim: Primary hypertension is the most important risk factor for chronic kidney disease in adulthood. The genesis of essential hypertension is likely to be multifactorial. The aim of this study is to investigate the causing factors of primary hypertension detected by ambulatory blood pressure monitoring in children.Materials and Methods: Fifty-six patients who had blood pressure higher than 90 percentile during the healthy children follow-up and 27 healthy children with the normal blood pressure were included in the study. Twenty-four hour blood pressure measurements with the blood pressure monitor were recorded as the day-night mean blood pressure, the blood pressure loads and the dipper-non-dipper characteristics. Plasma renin levels, serum aldosterone, nitric oxide and endothelin levels and amounts of sodium and potassium excretion in the 24 hour urine and were investigated in all patients and the control group.Results: Laboratory evaluations of the patients showed that the patient group has higher mean levels of plasma renin, lower mean levels of blood endothelin and mean levels of urinary sodium excretion than the control group.Conclusion: In the pathogenesis of childhood primary hypertension and white coat hypertension, which were seen in the half of the children diagnosed as primary hypertension, plasma renin and urinary sodium excretion had important roles.

Background. Kidney involvement related to infective endocarditis (IE) may present with different clinical findings. The most common histopathological finding of renal involvement is a combination of proliferative and exudative glomerulonephritis. However, severe acute kidney injury (AKI) induced by crescentic glomerulonephritis (CGN) is extremely rare in children with IE. To date, only 4 pediatric cases with IE-induced CGN had been reported. We present a 14-year old girl with IE-induced CGN. Case. A 14-year old girl with fever, macroscopic hematuria, oliguria, and acute kidney injury (AKI) was admitted to our clinic. The medical history revealed that the patient had undergone several cardiac interventions due to truncus arteriosus type 1, and she recovered from IE-induced glomerulonephritis following antibiotherapy six months ago. During admission, the patient was diagnosed with IE according to one major (positive imaging finding) and three minor (fever, predisposing cardiac disease, and immunological criterion) criteria. Immediate antibiotic treatment was initiated. A kidney biopsy was performed, which showed crescentic glomerulonephritis (CGN with crescents, >50%). Daily pulse steroid (3 days), monthly pulse cyclophosphamide (6 doses), and oral steroid (2 mg/kg/day) therapy were initiated with gradual dose tapering. The patient underwent 12 hemodialysis sessions until the 38th day of the treatment. She was discharged on the 45th day of treatment with normal kidney function tests and negative acute phase reactants. Treatment was maintained with mycophenolate mofetil (MMF) after a 6-month course of cyclophosphamide. MMF was discontinued in the 12th month. At the 18th -month follow-up visit the patient had mild proteinuria, and was on ramipril therapy. Conclusions. The occurrence of CGN should be considered in children with predisposing cardiac disease, who develop hematuria, proteinuria, and severe AKI. Although antibiotic therapy alone is often sufficient in this immune complex GN induced by infection, early initiation of additional immunosuppressive therapy in the presence of CGN may be beneficial for long term preservation of kidney functions.

Aim: We aimed to evaluate the efficacy of rituximab therapy in childrenwith nephrotic syndromes and to share our experiences.Material and Methods: Twelve children with nephrotic syndrome (fourwith steroid-dependent, eight with steroid-resistant nephrotic syndrome) who were treated with rituximab were retrospectively evaluatedin terms of clinical and laboratory data and CD19-20 levels. All patientsreceived rituximab (375 mg/m2) once weekly for 4 weeks. A proteinuria-free period under steroid therapy was not sought prior to initiatingrituximab therapy.Results: The overall remission rates in patients with steroid-dependent and steroid-resistant nephrotic syndrome were 100% and 27%.Focal segmental glomerulosclerosis was diagnosed in six patients andthe remission rate was 33% in this population. CD19 cell depletion wasobserved in 10 of the 12 children. Seven of the 10 patients with CD19depletion achieved remission, whereas the other three had persistentnephrotic proteinuria despite CD19 depletion. Two patients withoutCD19 depletion never achieved remission. Relapse occurred in three ofthe seven patients associated with increased CD19.Conclusion: We observed that rituximab could be given without waitingfor a proteinuria-free period under steroid therapy. Our result suggestthat administering four weekly doses of rituximab increases the likelihood of remission, considering the amount of drug lost in the urineof children with nephrotic proteinuria. However, our findings must beconfirmed with dose-comparison studies conducted with larger populations and an evaluation of long-term adverse effects. Some patientsdid not achieve remission despite B cell depletion, which suggests thatB cell depletion is necessary but insufficient for remission in nephroticsyndromes.

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Objective: Mineral bone disorders due to chronic kidney disease (CKD-MBD) in children occur as a result of decreased glomerular filtration rate and abnormalities in phosphorus, calcium, parathyroid hormone, and vitamin D metabolism rates. Increased parathormone synthesis may result in the development of adenomas in the parathyroid gland. The aim of this study was to evaluate parathyroid lesions in children with CKD-MBD using ultrasound (US) elastography.Materials and Methods: Fifteen patients with a diagnosis of CKD-MBD (average age, 15.5±2.4 years; seven girls) and 15 healthy children (average age, 13.8±2.5 years; six girls) were included in the study. The patients were evaluated clinically and in terms of laboratory findings (calcium, phosphorus, alkaline phosphatase, vitamin D, and parathormone levels), and all patients were evaluated using strain US elastography. The parathyroid strain ratio indices of the study group were compared with the thyroid strain ratio indices of the control group.Results: Among 11 patients with stage 5 CKD who underwent parathyroid US, nodules were observed in 8 patients, whereas parathyroid nodules were not observed in 3 patients with stage 3-4 CKD. The parathyroid lesions average strain ratio index (1.1±0.5) was significantly higher than that of the control group (0.46±0.16).Conclusion: The stiffness of parathyroid lesions in children with US elastography was evaluated for the first time in this study to the best of our knowledge, and it is believed that a high strain ratio index could be a useful indicator of the presence of adenomas. We propose that the findings be supported by future larger studies.

Amaç: İdrar yolu enfeksiyonu, çocuklarda en sık görülen bakteriyel enfeksiyonlardan biridir. Ampirik antibiyotik seçimi hastanın özelliklerine ve bölgesel antibiyotik dirençlerine göre değişir. Uygunsuz, geniş spektrumlu antibiyotik kullanımının neden olduğu antibiyotik direnci günümüzde önemli sorunlardan biridir. Bu çalışmanın amacı, akut piyelonefrite neden olan mikroorganizmaların ve bunların antibiyotik direnç ve duyarlılıklarının 5 yıl ara ile iki zamanlı dönemde karşılaştırılmasıdır.Yöntem: Akut piyelonefritli çocukların klinik özellikleri, tedavi modalitesi, idrar kültür antibiyogramlarında mikroorganizma türleri ve antibiyotik dirençleri, iki zaman dilimi arasında karşılaştırıldı.Bulgular: Grup 1; 2012-2013 yılları arasında piyelonefrit nedeni ile yatırılarak tedavi edilen 86 çocuktan (yaş ortalaması 3,52±0,4 yıl, 32 erkek), grup 2; 2017-2018 yılları arasında tedavi edilen 72 çocuktan (yaş ortalaması 3,78±0,7 yıl, 25 erkek) oluşturuldu. E. coli her iki grupta en sık üreyen mikroorganizmaydı.Piyelonefrit tedavisi için en sık uygulanan antibiyotikler sırasıyla hem grup 1 hem Grup 2’de amikasin (%55 ve %51), seftriakson (%33 ve %37), gentamisin (%5 ve %2,2) olduğu belirlendi. Grup 1’deki çocukların %77’si profilaktik antibiyotik kullanırken, bu oran Grup 2’de %23 olup anlamlı olarak düşüktü (p=0,00).Grup 2’de ampisilin, sefepim, seftriakson dirençlerinde istatistiksel olarak anlamlı düşüş saptandı. Özellikle son yıllarda sorun olan seftriakson direncinin %60’tan % 37’ye gerilediği izlendi.Sonuç: Bu çalışmada, yıllar arasında başta seftriakson olmak üzere birçok antibiyotik direnç oranlarında düşüş olduğu gözlenmiştir. Bu sonucun nedenlerinin piyelonefritli çocuklarda ampirik tedavide seftriakson tedavisinden ziyade aminoglikozidlerin daha sık tercih edilmesinin, profilaksi tedavisinin seçili olgularda kısıtlı şekilde verilmesinin ve profilakside sefalosporinlerin kullanılmamasının olduğunu düşünmekteyiz. Akılcı ampirik antibiyotik seçiminin idrar yolu enfeksiyonlarında direnç gelişimini de engelleyeceğini düşünmekteyiz.

Amaç: Kliniğimizde izlem ve tedavi edilen jüvenil idiyopatik artrit tanılıhastaların demografik verilerini, alt gruplarına dağılımını, tedaviye yanıtlarını ve uzun dönem izlemlerini ortaya koymak, ulusal ve uluslararası veriler ile karşılaştırmaktır.Gereç ve Yöntemler: Ocak 2012 ile Ocak 2018 tarihleri arasında jüvenilidiyopatik artrit tanısı alarak tedavi başlanan, düzenli kontrole gelen 116hastanın dosyası incelendi. Demografik bulgular, aldığı tedaviler, aktif/inaktif hastalık (ilaçlı ve ilaçsız) ve tedaviye yanıt durumları değerlendirildi.Bulgular: Uluslararası Romatoloji Derneği Birliği ölçütlerine göre jüvenilidiyopatik artrit alt tipleri 38 entezit ilişkili artrit, 37 oligoartiküler, 17 romatoid faktör (-) poliartiküler, 15 sistemik, 5 romatoid faktör (+) poliartiküler, 4 psöriatik jüvenil idiyopatik artrit olarak değerlendirildi. Toplamdakız erkek oranı 1,5 idi. İlk yakınma ile tanı konulması arasındaki gecikmesüresi ortalama 5,7±5,2 aydı. En erken tanı alan sistemik tipte hastalar, engeç tanı alan hastalar ise poliartiküler ve entezit ilişkili alt tipte olanlardı. Hastaların %32’sine yalnızca metotreksat tedavisi verilirken, %38’ineek biyolojik ilaçlar verildi. Her iki tedavi grubunda inaktif zamana geçişsüresi en kısa oligoartiküler, en uzun entezit ilişkili artrit grubundaydı.Çalışma döneminde 38 hasta ilaçsız iyilik hali (en yüksek oran %53,3 ilesistemikte), 71 hasta ilaçlı iyilik hali (en yüksek oran %70,2 ile oligoartikülerde) olarak izlenmekteydi. Hastaların %94’ünde iyilik hali sağlandı.Çıkarımlar: Entezit ilişkili artritin dikkat çekici bulgusu olan entezitin rutin fizik muayenede göz ardı edilmemesi gerekmektedir. Entezit farkındalığı, entezit ilişkili artritli çocuklarda tanı gecikmesinin önlenmesinekatkıda bulunabilir.

Objective: Urinary tract infections (UTIs) are among the most common bacterial infections in children. Selection of empirical antibiotic therapy is based on patient characteristics and regional antibiotic resistance patterns. Antibiotic resistance driven by inappropriate antibiotic use remains currently one of the major public health concerns. The aim of this study was to compare the microbiological spectrum of pediatric acute pyelonephritis and antimicrobial resistance patterns in two time periods 5 years apart., Method: Clinical characteristics, treatment modalities, causative uropathogens in urine cultures, antibiotic susceptibility and resistance patterns of the patients with acute acute pyelonephritis were compared between the two time periods., Results: Group 1 consisted of 86 children (mean age 3.52 +/- 0.4 years, 32 boys) hospitalized, and treated for acute pyelonephritis between 2012-2013; Group 2 included 72 children (mean age 3.78 +/- 0.7 years, 25 boys) between 2017-2018. Escherichia coli was the most common microorganism in both groups. The most frequently used antibiotics for pyelonephritis treatment in both groups were amikacin (55% vs 51%) and ceftriaxone (33% vs 37%), gentamicin (5% vs 22%) While 77% of the children in Group 1 used prophylactic antibiotics, this rate was significantly lower with 23% in Group 2. Resistance to ampicillin, cefepime and ceftriaxone were significantly lower in Group 2. Ceftriaxone resistance which created concerns in recent years regressed from 60% to 37%., Conclusion: Our study revealed significant reductions in rates of resistance to several antibiotics, particularly ceftriaxone within 5 year-period. Possible explanations for these results may be that aminoglycosides are preferred more frequently than ceftriaxone therapy, prophylactic treatment is limited in selected cases, and cephalosporins are not used for prophylaxis. We believe that rational empirical antibiotic selection will prevent the development of resistance in urinary tract infections.

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Objective: Pediatric urolithiasis is a globally growing problem. The composition and frequency of urinary tract stones vary not only among different countries, but across various regions in a country. Hence, we aimed to identify the types and frequencies of urinary tract stones in children from our region (Inner Western Anatolian part of Turkey), and to compare our findings with the results from other regions in our country. Material and methods: In this retrospective analysis of 53 pediatric urolithiasis cases that were treated in our hospital between 2009 and 2019, the demographic data, clinical course, radiological and metabolic find-ings, the recurrence rate, and the composition of the stones were evaluated. Results: The mean age of the patients was 5.9±4.6 (0.5–18) years, and there were 30 (56.6%) girls and 23 (43.4%) boys. An analysis of the composition of the stones revealed that the majority (85%) consisted of calcium oxalate. The highest risk of recurrence and the need for multiple shockwave lithotripsy (SWL) sessions or surgical intervention appeared to be related with the presence of whewellite stones, which are the most challenging stones in childhood. Conclusion: According the study results, the urinary stone types vary across different regions in our coun-try, and the frequency of uric acid stones decreased going westward, while the frequency of oxalate stones increased. We conclude that this difference in the frequency of the type of urinary stones might reflect the regional dietary habits. Regional frequency and etiology studies for the types of urolithiasis may facilitate the approach to the treatment of urolithiasis. © 2020 by Turkish Association of Urology.

Amac: Hemolitik uremik sendrom (HUS), mikroanjiopatik hemolitik anemi, trombositopeni, akut bobrek hasari ile karakterize bir tablodur. Cocukluk caginda en sik diyare sonrasi gelisen HUS gorulur, bunun da en sik nedeni shigatoksin ureten Escherichia coli (STEC) dir. Yerel STEC gastroenterit salginlari, STEC ile iliskili HUS sikliginin artmasina neden olur. STEC ile iliskili HUS'un sikliginin 2018'de arttigini gozlemledik. Amacimiz bolgemizde 2018 yilinda artmis sayidaki HUS olgularimizin ozelliklerini, tedavilerini ve klinik deneyimlerimizi diger epidemilerle karsilastirarak sunmaktir. Gerec Yontem: Klinigimizde 2018 yilinda tedavi edilen toplam 10 diyare sonrasi gelisen HUS tanili hasta verileri geriye donuk tarandi. Hastalarin hemogram, periferik yayma, bobrek fonksiyon testleri, LDH, haptoglobulin, kompleman duzeyleri, tam idrar tetkiki, idrar protein/kreatinin orani, gayta kulturleri ve gaytada shigatoksin bakildi. Bulgular: Hastalarin median yas degeri 17 (10-108) ay olup dordu kiz, altisi erkek cinsiyetten olusmakta idi. Hastalarin gelis zamani mevsimsel ozellik gostermiyordu. Alti hastada iyi pisirilmemis et yeme suphesi var idi. Bes hastada shigatoksin ya da verotoksin ureten E. Coli saptandi. Alti hastaya diyaliz, dort hastaya norolojik tutulum ve siddetli HUS bulgulari nedeniyle eculizumab tedavileri uygulandi. On hastanin yedisi tam olarak iyilesti, uc tanesi proteinuri nedeni ile ACE (anjiotensin donusturucu enzim) inhibitoru tedavisi almaktadir. Sonuc: Calismamizda bir yil icinde toplam 10 diyare sonrasi gelisen HUS’lu hasta basari ile tedavi edilmistir. Erken renal replasman tedavisinin hastaligin prognozunda etkili oldugunu, anuri gelisen hastalarda proteinurinin komplikasyon olarak gelisebilecegini, norolojik tutulumu olan ve secilmis siddetli HUS bulgulari olan diyare iliskili HUS vakalarinda da eculizumab tedavisinin basarili sonuclarinin oldugunu dusunmekteyiz. 0000-0003-2617-4466

Akut infantil hemorajik odem (AIHO); ates, odem, genis purpurik deri lezyonlari ile karakterize genellikle sistemik tutulum bulgusu olmadan birkac gun veya hafta icerisinde kendiliginden gerileyen ve sut cocuklarinda gorulen derinin lokositoklastik bir vaskulitidir. Hastalik baslangicta oldukca agir bir klinik tablo ile ortaya cikmasina ragmen, klinik gidisat selim bir seyirde olup kisa surede tamamen iyilesmektedir. Etiyoloji tam olarak bilinmemekle birlikte, enfeksiyon, asilama ve ilac tedavilerinin AIHO’ye neden oldugu dusunulmektedir. Bu yazida literaturde nadir olarak bildirilmis Adenovirus enfeksiyonun neden oldugu AIHO’lu 9 aylik erkek bir hasta sunulmustur.

Background: We present the case of an adolescent girl with prominent clitoral swelling as the first symptom when she presented to the emergency department, and who was subsequently diagnosed with nephrotic syndrome. Case: A 14-year-old adolescent girl was admitted with painless clitoral swelling. She denied recent masturbation, itching, or discharge. She was within the last few days of menstruation. Physical examination revealed clitoral edema without erythema or genital edema. Urine dipstick test and microscopic evaluation revealed protein 2+, blood 3+, abundant erythrocytes and 9-10 leukocytes. A few days later, additional clinical findings, such as pretibial and facial edema, were diagnosed as nephrotic syndrome. Summary and Conclusion: This case is a reminder that clitoral swelling is to be considered a sign in the diagnosis of nephrotic syndrome, even when it occurs alone. C1 [Girisgen, Ilknur; Yuksel, Selcuk] Pamukkale Univ, Sch Med, Dept Pediat Nephrol, Denizli, Turkey. [Pekal, Yucel; Pekal, Busra] Pamukkale Univ, Sch Med, Dept Pediat, Denizli, Turkey.

Aim We aimed to evaluate the efficacy of rituximab therapy in children with nephrotic syndromes and to share our experiences. Material and methods Twelve children with nephrotic syndrome (four with steroid-dependent, eight with steroid-resistant nephrotic syndrome) who were treated with rituximab were retrospectively evaluated in terms of clinical and laboratory data and CD19-20 levels. All patients received rituximab (375 mg/m2) once weekly for 4 weeks. A proteinuria-free period under steroid therapy was not sought prior to initiating rituximab therapy. Results The overall remission rates in patients with steroid-dependent and steroid-resistant nephrotic syndrome were 100% and 27%. Focal segmental glomerulosclerosis was diagnosed in six patients and the remission rate was 33% in this population. CD19 cell depletion was observed in 10 of the 12 children. Seven of the 10 patients with CD19 depletion achieved remission, whereas the other three had persistent nephrotic proteinuria despite CD19 depletion. Two patients without CD19 depletion never achieved remission. Relapse occurred in three of the seven patients associated with increased CD19. Conclusion We observed that rituximab could be given without waiting for a proteinuria-free period under steroid therapy. Our result suggest that administering four weekly doses of rituximab increases the likelihood of remission, considering the amount of drug lost in the urine of children with nephrotic proteinuria. However, our findings must be confirmed with dose-comparison studies conducted with larger populations and an evaluation of long-term adverse effects. Some patients did not achieve remission despite B cell depletion, which suggests that B cell depletion is necessary but insufficient for remission in nephrotic syndromes.

Background: Nocturnal enuresis is defined as bed-wetting in children from the age of five years that occurs during sleep; if untreated, the condition can result in social and psychological problems both for the children and their parents. Nocturnal enuresis is a complicated disease that includes multiple pathogenetic factors. Nocturnal enuresis is divided into two subgroups: monosymptomatic and non-monosymptomatic. The role of some biomarkers in patients with monosymptomatic enuresis has been reported in a small number of the studies. Objective: The aim of this research was to evaluate the serum levels of copeptin and corticotropin-releasing factor (CRF) in monosymptomatic and non-monosymptomatic nocturnal enuresis cases. Although these markers were previously examined in children with monosymptomatic enuresis, there is no study that has evaluated these markers in non-monosymptomatic children. Study design: One hundred nineteen children with nocturnal enuresis (5–16 years) and forty healthy children (5–17 years) were enrolled to the study. Of the nocturnal enuresis group, forty-nine were monosymptomatic and seventy were non-monosymptomatic. Copeptin and CRF were measured by a competitive inhibition method with enzyme-linked immunosorbent assay. Results: The serum copeptin levels were significantly lower in children with monosymptomatic and non-monosymptomatic nocturnal enuresis than in the controls.(median, 34.7 [interquartile range (IQR): 34 pg/ml], 39.8 [IQR: 29 pg/ml] vs 52.1 [IQR: 14 pg/ml], respectively, P < 0.05). The serum CRF levels were significantly lower in children with monosymptomatic and non-monosymptomatic nocturnal enuresis than in the controls (median, 35.1 [IQR: 19 pg/ml], 34.05 [IQR: 24 pg/ml] vs 78.3 [IQR: 39 pg/ml], respectively, P < 0.05). There was no significant difference in copeptin and CRF levels between the children with monosymptomatic and non-monosymptomatic nocturnal enuresis. Discussion: Copeptin is presumed to be a sensitive surrogate biomarker for arginine vasopressin release. To date, there are only two studies in the literature that assess the relationship between copeptin and monosymptomatic enuresis. The only study in the literature demonstrated significantly decreased levels of CRF in monosymptomatic enuretic children. It was demonstrated that the levels of copeptin and CRF differ in both children with monosymptomatic and non-monosymptomatic nocturnal enuresis from the control groups. It was also demonstrated that copeptin and CRF levels were not different between the children in monosymptomatic and non-monosymptomatic groups. Conclusion: Those changes in both copeptin and CRF which were shown in this study in monosymptomatic and non-monosymptomatic enuretic children may contribute to the pathogenesis of nocturnal enuresis. Further case–control studies can evaluate the copeptin and CRF levels before treatments in monosymptomatic and non-monosymptomatic patients to decide potential effectiveness of treatment.[Figure presented] © 2019 Journal of Pediatric Urology Company

Purpose: In this study, it was aimed tocompare the frequency of significant bacteriuria and antibiotic resistancecharacteristics in children with myelodysplasia-neurogenic bladder in whomclean intermittent catheterization (CIC) and children with normal bladder and recurrent urinary tractinfection.Material-Methods: Children with neurogenic bladder in whom CICgroup were divided into two subgroups as taking antibiotic prophylaxis and not.Children with normal bladder and recurrent urinary tract infection group weredivided in to two subgroups as with vesicoureteral reflux and without. Renal scar, vesicoureteral reflux,microorganism types, antibiotic resistance in urine culture antibiogram was compared in allgroups.Results: The neurogenic bladder group wascomposed 99 cultures of 26 patients (mean age:6.01±4.00 years 18 girls) who werefound to have significant bacteriuria and the normal bladder group was composed of 352 cultures of 75 children(mean age:7.2±4.34 years 56 girls). Growth of Escherichia coli wasfound with the highest rate in children with neurogenic bladder and children with normal bladder. However ESBL producingmicroorganisms were found highestrate in children with neurogenic bladder than children with normal bladder. Asignificant increase resistance to ampicillin and piperacillintazobactam was found inchildren with neurogenic bladder than children with normal bladder. Non E. Coli microorganisms such as Klebsiella were found highest rate inchildren with neurogenic bladder who were using prophylactic antibiotic. Asignificant increase resistance to ampicillin, ceftriaxone and gentamicinresistance was also found in the patients who received prophylactic antibioticcompared to the patients who did not receive prophylactic antibiotic inchildren with neurogenic bladder. Asignificant increase resistanceto ampicillin, gentamicin, trimethoprim-sulfamethoxazole,aztreonam,cefepime, ceftazidime, fosfomycin was also found in the patients with vesicoureteral reflux than without.Conclusion: Early and correct treatment as CIC may easilyprevent renal damage in children with myelodysplasia-neurogenic bladder. Theuse of prophylactic antibiotics may lead to the development of drug-resistancein patients with neurogenic bladder. Therefore use of prophylacticantibiotics this group may not be necessary. Highest antibiotic resistance wasfound in the patients with normal bladder and vesicoureteral reflux. Also asignificant increase renal scar incidence in same group. Although, it was concluded that prophylactic antibiotic useshould be continued as the scar ratio is high in patients with normal bladderand vesicoureteral reflux., Giriş: Bu çalışmada spinaldisrafizme bağlı nörojen mesane nedeni ile temiz aralıklı kateterizasyon (TAK) uygulayanhastalar ile nörojen mesanesi olmayan ve tekrarlayan idrar yolu enfeksiyonuolan hastaların idrar kültürlerindeki mikroorganizmaların tipleri, sıklıkları,antibiyotik dirençleri ve direnç gelişimine etki eden faktörler ayrımlanmayaçalışıldı.Gereç-Yöntem: Spinaldisrafizm nedeni ile nörojen mesanesi olan ve TAK uygulamakta olan hastalargrup 1, nörojen mesanesi olmayıp tekrarlayan idrar yolu enfeksiyonu ile takipedilen hastalar grup 2 olarak adlandırıldı. Ayrıca grup 1 antibiyotikproflaksisi alan ve almayan olmak üzere, grup 2‘deki hastalar isevezikoüreteral reflü olup olmamasına göre iki alt gruba ayrıldı vemikroorganizma türleri ve antibiyotik dirençlerine göre karşılaştırıldı. Bulgular: Nörojen mesane nedeni ile temiz aralıklıkataterizasyon yapılan 26 (ortalama yaş 6,01±4 yıl, 18 kız) hastanın 1 yıliçinde alınan idrar kültürlerinin 99’unda, nörojen mesane olmayan tekrarlayanidrar yolu enfeksiyonu tanısı ile izlenen 76 (ortalama yaş 7,2±4,34 yıl, 56 kız)hastanın 352 idrar kültüründe anlamlı üreme saptandı. Her iki hasta grubunda ensık görülen mikroorganizma türü E. Coliolup görülme sıklıkları açısından fark saptanmadı. Nörojen mesane grubundagenişletilmiş spektrumlu beta laktamaz mikroorganizma (ESBL+) normal mesanegrubuna göre daha yüksekti. İki grup arasında antibiyotik direnç oranlarıkarşılaştırıldığında nörojen mesane grubunda piperasilin tazobaktam veampisilin dirençleri anlamlı yüksek saptandı. Nörojen mesanesi olup antibiyotikproflaksisi alan grupta (16 hasta, % 61) E.Coli dışı mikroorganizma ile Klebsiellaüreme sıklığı ve ampisilin, seftriakson, gentamisin dirençleri anlamlı yükseksaptandı. Normal mesaneli grupta; VUR olan hastalarda üreyen mikroorganizmalarve ESBL+ liği açısından anlamlı fark saptanmazken, ampisilin, trimetoprim/sulfametoksazol,aztreonam, sefepim, seftazidim, gentamisin, fosfomisin dirençleri anlamlıyüksek saptandı. Gruplar arasında en yüksek skar oranı normal mesaneli VUR+hastalarda saptandı.Sonuç: TAKyapılan hastalarda ESBL+ dirençli mikroorganizmaların yüksek görülmesi TAKyapılmasının dirençte risk faktörü olduğunu göstermektedir. Çalışmada hem en yüksekantibiyotik direnci hem de en yüksek skar oranı normal mesaneli VUR+ hastalardasaptandı. TAK yapılan nörojen mesaneli hastalarda, mikroorganizmalardaantibiyotik direnci gelişmesi nedeni profilaksi kullanılmamalı iken normalmesaneli VUR+ hastalarda skar oranı yüksek olduğu için profilaktik antibiyotikkullanımına devam edilmesi gerektiği sonucuna varılmıştır.

Sen, Sait/0000-0002-1100-6657, WOS: 000522854900020, PubMed: 31696856, Rapidly progressive glomerulonephritis (RPGN) is rare syndrome in children, characterized by clinical features of glomerulonephritis and rapid loss of renal function, and is associated with crescentic glomerulonephritis. Primary membranous nephropathy (MN) is an immune-complex-mediated cause of the adult nephrotic syndrome but occurs less frequently in children. RPGN is rarely observed in adults with primary MN. in this article, we report a case of MN, which developed during long-term follow-up of previously treated RPGN. Our case may be the first to demonstrate primary MN and crescentic glomerulonephritis in a child. We would like to underline the importance of not dropping the long-term follow-up of cases with primary RPGN (not accompanied by other glomerulonephritis and vasculitis symptoms) who had improved with treatment.

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch-Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch-Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch-Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.

Pertussis or whooping cough is a vaccine-preventable disease that still remains a serious infection in neonates and young infants. The disease is particularly severe in infants less than three months old, who are often infected by their parents. Congenital nephrotic syndrome is a rare entity presenting within the first three months. It encompasses a heterogeneous group of entities with genetic, infectious and idiopathic etiologies. In this report we describe a newborn infant who presented with congenital nephrotic syndrome secondary to Bordetella pertussis infection.

Primary hypertension is the most important risk factor for chronic kidney disease in adulthood. However, the role of hypertension in kidney damage in childhood is not known exactly. The aim of this study was to evaluate the ambulatory blood pressure measurements of healthy children diagnosed as hypertensive with routine office blood pressure monitoring and to investigate the effects of primary hypertension on the kidney. Fifty-six patients who had blood pressure higher than 90th percentile during their well-child follow-up and 27 healthy children with normal blood pressure were included in the study. Twenty-four hour blood pressure measurements were recorded for all the patients. Microalbumin and N-acetyl-β-D-glucosaminidase (NAG) levels in the 24-hour urine were determined in the study groups. According to the results of ambulatory blood pressure measurements, 52% of the patients were diagnosed as white coat hypertension. The patients and the white coat hypertensive group had higher levels of urinary NAG than the control group. No significant difference was found in the levels of urinary microalbumin excretion between the primary hypertension and control groups. It was thought that ambulatory blood pressure measurement was necessary for the true diagnosis of hypertension in children, and further, that primary and white coat hypertension had effects on kidney damage in childhood. It is suggested that urine NAG excretion might be used as an early sign of hypertension-induced renal damage.

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiologic entity characterized by headache, seizures, visual changes, altered mental status, and focal neurologic signs. Typically, PRES involves the parietooccipital lobes; however, it can involve atypical localizations such as frontal lobe, basal ganglia, thalamus, brainstem, and gray matter. Sudden increases in blood pressure and associated renal failure are probably the most frequently encountered etiologies in the literature. Recurrence of PRES is not common. In this article, we present recurrent atypical PRES in a hypertensive child with end-stage renal disease on a peritoneal dialysis program as a rare case and we discuss recurrence. Infections and sudden increase in blood pressure were observed as the causes of recurrent PRES in our patient. The reversibility of PRES depends on immediate diagnosis and therapy; therefore, it should be kept in mind in the differential diagnosis of seizures or coma in chronic kidney disease patients.

Objectives: HSP is a leucocytoclastic form of vasculitis and immunfluorescence staining reveals the presence of dominantly IgA, besides C3, fibrin/fibrinogen in vessels and perivascular IgG or/and IgM deposition. The aim of this study was to investigate the correlation between immune deposits on dermis and clinical findings in children with HSP. Methods: The children who were diagnosed as HSP were included to the study. The EULAR/PReS endorsed consensus criteria for HSP proposed by Ozen et al. was used for the diagnosis of HSP. The signs and symptoms of patients were evaluated and frequency of skin, joint, gastrointestinal and renal manifestations were determined. All cases were submitted to punch skin biopsy to verify the diagnosis of HSP by eliminating other forms of immune complex mediated small vessel vasculitides. Sections were stained with fluorescent antihuman immunoglobulin IgA, IgM, IgG, fibrin/fibrinogen and complement C3 component. Results: The relationship between immune deposits as C3, IgM, IgG and fibrin and the characteristics of systemic involvement was investigated. No significant relationship was found between immune deposits on skin and systemic involvement. Conclusion: C3, fibrinogen/fibrin deposition in vessels and IgM or and IgG deposition in derma were not found as prognostic and pathogonomic factors for HSP in children.