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Background Although acute coronary syndrome is rare in children, it is the most important cause of mortality in children with end-stage renal disease. Case presentation Here, a 16-year-old pediatric patient, who has been on dialysis since the age of 3, and who was diagnosed with acute coronary syndrome and placed an emergency percutaneous transcatheter stent in the left anterior descending branch of the left coronary artery is presented. It is important that the present patient does not have any electrocardiography findings in favor of cardiovascular disease and that he cannot fully explain the complaint of chest pain due to his mental retardation. Conclusions Early detection of acute coronary syndrome is life-saving, especially in children with chronic kidney disease.

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.

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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.

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.