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© 2022, The Author(s), under exclusive licence to International Pediatric Nephrology Association.Background: Automated peritoneal dialysis (APD) is increasingly preferred worldwide. By using a software application (Homechoice with Claria sharesource system (CSS)) with a mod-M added to the APD device, details of the home dialysis treatment become visible for PD nurses and physicians, allowing for close supervision. We aimed to evaluate the perceptions of patients/caregivers, PD nurses, and physicians about the advantages and disadvantages of CSS. Methods: Three different web-based questionnaires for patients/caregivers, nurses, and physicians were sent to 15 pediatric nephrology centers with more than 1 year of experience with CSS. Results: Respective questionnaires were answered by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses. Most of the nurses and physicians (87% and 73%) reported that CSS improved patient monitoring. A total of 73% of nurses suggested that CCS is not well known by physicians, while half of them reported reviewing CSS data for all patients every morning. Sixty-eight percent of physicians thought that CSS helps save time for both patients/caregivers and healthcare providers by reducing visits. However, only 20% of patients/caregivers reported reduced hospital visits. A total of 90% of patients/caregivers reported that being under constant monitoring made them feel safe, and 83% stated that the patient’s sleep quality improved. Conclusions: A remote monitoring APD system, CSS, can be successfully applied with children for increased adherence to dialysis prescription by giving shared responsibility and may help increase the patient’s quality of life. This platform is more commonly used by nurses than physicians. Its potential benefits should be evaluated in further well-designed clinical studies with larger patient groups. Graphical abstract: A higher resolution version of the Graphical abstract is available as Supplementary information. [Figure not available: see fulltext.]

Objective: Vaccines are an important preventive health service due to the high risk of infection in children with chronic kidney failure (CKF). This study aimed to examine the vaccination rate in children with CKF and affecting factors. Methods: Parents of 81 patients who were followed up with a diagnosis of CKF between the ages of 0-18 were included. The questionnaire form, consisting of 29 questions was applied to the parents using a face-to-face interview technique. The parents’ demographic characteristics, the vaccination status of the patients with the vaccines in the national vaccination schedule, and vaccination status with meningococcal, influenza, and human papilloma virus that are not in the national vaccination schedule were recorded. Results: It was observed that 85.2% of the patients (n=69) had complete vaccinations in the national vaccination schedule, and 75.3% (n=61) of the parents received vaccination training. The immunization rate with vaccines in the national vaccination schedule of the children of parents who received vaccination training was statistically significantly higher than the children of parents who did not receive training (p

Objective: Acute tubulointerstitial nephritis (aTIN) is a rare cause of acute renal injury in children. The incidence of cases linked to insensible use of energy drinks has increased in recent years. In thıs study, we aimed to examine patients with aTIN connected to energy drink in detail in our own patients.Methods: In this study, the files of patients with acute tubulointerstitial nephritis (aTIN in the pediatric nephrology clinic were retrospectively screened.Results: 32 patients were included in the study (F/M: 22/10). Mean age was 12±4.3 years (9-17). The most common causes in etiology were drugs (18; 56.2%) and energy drinks (9; 28.1%). The most common attendance symptoms were vomiting (84.3%), fatigue (68.7%), hypertension (56.2%), abdominal pain (40.6%), joint pain (34.3%), fever (31.2%). The mean duration from onset of symptoms to first attendance at hospital was 3.8±2.1 days, with the mean duration between first intake of causative agent to onset of symptoms 9±4.1 days.Conclusion: The inclusion of energy drink use among candidates for causes of aTIN in recent years is an important health problem. Due to serious side effect profile beginning with acute kidney injury and resulting in CKD, it is a cause that should definitely be questioned in the anamnesis of young patients especially.

Background/aim The objective of this study is to determine the clinical features of unilateral multicystic dysplastic kidney (MCDK) patients. Materials and methods The demographic, clinical, laboratory, and radiologic features of MCDK patients at Diyarbakir Children’s Hospital and Diyarbakir Gazi Yasargil Training and Research Hospital between January 2008-June 2019 were retrospectively evaluated. Results A total of 111 [59 (53.2%) male and 52(46.8%) female] patients with MCDK were followed for a mean period of 41.89 ± 32.03 months. MCDK was located on the left and right sides in 46 (41.4%) and 65 (58.6%) of the children, respectively (p > 0.05). A total of 87 (78.4%) patients had antenatal diagnosis. The mean age at diagnosis was 13.7 ± 34.2 months. Of the 49 voiding cystourethrogram (VCUG)-performed patients, vesicoureteral reflux was detected in 11 patients (22.4%). Other associated urological anomalies in the patients were detected in 12 (10.8%) patients. On Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy which was performed in all patients showed scarring in four children. Eight patients had history of UTI (7.2%). Renal failure, hypertension, and proteinuria were diagnosed in three children (2.7%). Sixty-nine (62%) patients developed compensatory hypertrophy. Conclusion All cases should be followed up closely and VCUG should be reserved for patients with recurrent UTI and other urological problems indicated by ultrasonography and abnormal DMSA scan results.

Objectives: Ureteral stend use is a risk factor for BK nephropathy (BKVN). In 2015, we compared the patients with anti-reflux mechanism DJS (ARD-DJS) and those used standard DJS (st-DJS) in terms of BKV and BKVN frequency in 90 kidney transplant patients in two centers. With the increase in the number of our patients over time and lengthening of the follow-up duration, we needed to re-evaluate the data in one center. Materials and Methods: We retrospectively evaluated 211 patients who underwent kidney transplantation at Diyarbakır Gazi Yaşargil Training and Research Hospital between September 2012 and September 2019. The following parameters were recorded, demographic data, immunosuppression protocols, presence of rejection, graft loss, plasma BKV levels, and presence of BKVN. Median and IQR follow-up time for ARD-DJS and St-DJS patients was 72 months (62,5-80,3 months) and 27,8 months (17,4-39,6 months) respectively. Results: Thirteen patients (6,1%) had BKV viremia. BKVN was revealed by kidney biopsy in 3 of 13 patients. However, graft loss due to BKVN was observed in only one patient. ARD-DJS was used in 4 of these cases and standard DJS was used in 9 of these cases. Patients in whom BKV revealed in the first 3 months were compared in the aspect of DJS technique, BKV was significantly less observed in the ARD-DJS group (ARD-DJS: 2 patients; St-DJS:9 patients), (p=0,046). Conclusions: In our study, BKV was observed less in patients with ARD-DJS that were clinically significant but not statistically significant. Therefore, prospective randomized studies with high patient numbers are needed to determine the effectiveness of ARD-DJS.

Amac: Idiyopatik nefrotik sendrom (INS) cocuklarda en sik gorulen glomeruler hastalik olup, %80’i steroide duyarlidir. Steroide duyarli nefrotik sendrom (SDNS) hastalarinin yaklasik yarisi steroid bagimli nefrotik sendromudur (SBNS). Bu calismanin amaci ilk ataginda basvuran SDNS hastalarinda, steroid bagimliligini tedavi oncesi ongorebilecek risk faktorlerini belirlemektir. Gerec ve Yontem: Cocuk Nefroloji Klinigimize Eylul 2016-Eylul 2018 tarihleri arasinda ilk atak INS ile basvuran ve steroid tedavisine yanitli hastalar geriye donuk olarak degerlendirildi. Hastalarin demografik ozellikleri, poliklinik kan basinci olcumu, kan albumin ve kreatinin degeri, beyaz kure sayisi, lenfosit sayisi, lenfosit/beyaz kure orani, spot idrar protein/kreatinin orani, steroid tedavisi baslanmasini takiben remisyona girme suresi ve atak baslaticisi kaydedildi. Bulgular: Hastalarin 23’u SDNS, 16’si SBNS tanisi aldi. Basvuru yasi ortalamasi SDNS grubunda 77,34±32 ay, SBNS grubunda 73,62±27,32 ay idi (p=0,690). Iki grup arasinda kan albumin, kreatinin, lenfosit sayisi ve lenfosit/beyaz kure orani acisindan anlamli fark saptanmadi. Atak baslaticisi SBNS grubunda daha fazla goruldu (p=0,001) ve en sik tespit edilen atak baslaticisi ust solunum yolu enfeksiyonu idi. Spot idrar protein/kreatinin orani, kan beyaz kure seviyesi SBNS grubunda anlamli yuksek saptandi (p=0,014, p=0,004) ve remisyona girme suresi SBNS grubunda daha uzundu (17,5 gune 9 gun) (p=0,000). Sonuc: Idiyopatik NS’de, ust solunum yolu enfeksiyonuyla ilk atagin tetiklenmesi, basvuru aninda agir proteinuri saptanmasi ve gec remisyona girme suresi SBNS gelismesi acisindan risk faktorleri olabilir. Bu hastalarda steroid ayirici tedavilerin daha erken gundeme alinmasi, steroid tedavisinin istenmeyen uzun donem yan etkilerinden koruyacaktir.

Amaç: Bu çalışmanın amacı idiyopatik epilepsi tanılı çocuklarda anti epileptik tedavi kesimi sonrası nöbet tekrarına etki eden risk faktörlerini değerlendirmektir. Gereç ve Yöntem: En az 24 ay anti epileptik tedavi alan, kraniyal manyetik rezonans görüntüleme ve elektroensefalografi sonuçları normal olan hastaların ilaç tedavisi 4-6 aylık süreçte azaltılarak kesilmiştir. Tedavi kesimi sonrası nöbeti tekrarlayan 72 hasta relaps grubunu, nöbeti tekrar etmeyen 82 hasta ise remisyon grubunu oluşturmuştur.Sonuç: Hastaların tanı yaşı ve tedavi başlanması sonrasında nöbet kontrol sürelerinin relaps üzerine etki eden risk faktörleri olduğu görülmüştür. Relaps grubundaki hastaların %95,9’unda nöbet tekrarının ilk 2 yıl içerisinde geliştiği belirlenmiştir. Tartışma: Tedavi başlanmasından sonra nöbet kontrol süresi 3 ay ve üzerinde olan hastalarda ilaç kesim sonrası tekrarlama riski daha yüksektir. Ayrıca epilepsi tanı yaşı 12 ve üzerinde olan hastalarda da tekrarlama riskinin yüksek olduğu görülmüştür. Bununla birlikte anti epileptik tedavisi kesilen tüm hastalarda 2 yıl yakın takip önermekteyiz., Objective: The purpose of this study is to evaluate the risk factors on seizure recurrence after antiepileptic drugwithdrawal in children with idiopathic epilepsy.Material and Methods: The treatment is withdrawn within 4-6 months in all patients who had normal magneticresonance imaging and electroencephalogram results on at least 24 months of antiepileptic drug treatment. After drugwithdrawal 72 patients had seizure recurrence in whom labeled as relapse group. Eighty-two patients had no seizurerecurrence are labeled as remission group.Results: The age of diagnosis with epilepsy and remission time were observed as risk factors on seizure recurrence. Itis determined that after drug withdrawal seizure recurrence is occurred within the first 2 years of follow-up in 95,9 % ofthe relapse group’s patients.Conclusion: In patients, who had more than 3 months of seizure remission time have more seizure recurrence riskafter drug withdrawal. Furthermore, it is observed that in patients older than 12 years old the risk of recurrence

Targeting the vascular endothelial growth factor (VEGF) signaling pathway has become an important approach to current cancer therapy. Anti-VEGF therapy-related renal adverse effects may present as hypertension, non-nephrotic proteinuria, and rarely as nephrotic syndrome (NS) and acute kidney injury. In this report, we present a 15-year-old boy who had developed nephrotic syndrome and thrombotic microangiopathy 26 months after administration of anti-VEGF therapy. Treatment was discontinued and nephrotic syndrome remitted spontaneously within 3 months. Nephrologists should be aware of the side effects of anti-VEGF therapy. Early diagnosis and prompt management with withdrawal of the agents will result in spontaneous remission.

Kistik bobrek hastaliklari, kalitimsal bobrek hastaliklari icinde en sik gorulen grup olup, her biri farkli etiyoloji, patogenez, klinik prezentasyon ve prognoza sahiptir. Hastaligin tanisi aile oykusu, klinik bulgular ve goruntuleme yontemleriyle konulabilmektedir ancak bazi olgularda kesin tani icin genetik calisma gerekebilir. Son on yil icerisinde kistik bobrek hastaliklarinin etyopatogeneziyle ilgili bircok yeni bilgi kazanilmistir. Edinilen bilgiler ozellikle primer siliyanin bozulmus mekanik algilama fonksiyonu, artmis proliferasyon ve iletisim yolaklarinin kist gelisiminin temelini olusturdugunu isaret etmektedir. Bu derlemede kalitimsal kistik bobrek hastaliklarinin patogenezi, belirtileri ve tanida genetik incelemenin yeri tartisilmistir

Acute tubulointerstitial nephritis (TIN) is a common cause of acute renal impairment, characterized by the infiltration of inflammatory cells in the interstitium of the kidney. We retrospectively reviewed the medical records of 19 acute TIN patients attended to our Pediatric Nephrology department between April 1999 and April 2014. Nineteen patients (7 boys and 12 girls) were evaluated. The median age was 14 years (range 7-19). Five were diagnosed as TIN histopathologically, fourteen patients were diagnosed as clinically. Six patients were treated with steroids, thirteen patients were treated symptomatically. All patients showed a rapid recovery at longest in one month. TIN is a common cause of acute renal impairment. Renal biopsy is recommended for persistent cases. Renal outcome is mostly good with symptomatic treatment but steroids could be preferred in severe nephritis however long-term follow up showed no differences between the treated and non-treated group.

Henoch-Schonlein purpura (HSP) is the most common vasculitis in children. Vasculitic processes can involve the lung. Although diffuse alveolar hemorrhage may be seen as one of the manifestation of HSP, it is not a frequent presentation. Here we reported the case of a 10-year-old girl with HSP nephritis who developed pulmonary hemorrhage. The patient was treated successfully with intravenous methylprednisolone. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment.

Henoch-Schonlein purpura (HSP) is the most common vasculitis in children. Vasculitic processes can involve the lung. Although diffuse alveolar hemorrhage may be seen as one of the manifestation of HSP, it is not a frequent presentation. Here we reported the case of a 10-year-old girl with HSP nephritis who developed pulmonary hemorrhage. The patient was treated successfully with intravenous methylprednisolone. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment.La púrpura de Schonlein-Henoch (PSH) es la vasculitis más frecuente en los niños. Los procesos vasculíticos pueden afectar el pulmón. Si bien la hemorragia alveolar difusa puede considerarse una de las manifestaciones de la PSH, no es un cuadro frecuente. En este artículo presentamos el caso de una niña de 10 años con nefritis por PSH que sufrió hemorragia pulmonar. La paciente recibió un tratamiento satisfactorio con metilprednisolona intravenosa. La revisión de las publicaciones reveló que la edad temprana puede influir de manera positiva en el pronóstico, y que los inmunosupresores y el tratamiento complementario son fundamentales.

The aim of this report was to evaluate and discuss treatment of pediatric familial Mediterranean fever (FMF) patients with anti-interleukin1 (IL-1) agents.Refractory or colchicine unresponsive FMF was described as severe and frequent attacks and/or having high acute phase reactance levels despite having a maximum dose of colchicine (2 mg/day). Disease course, adverse effects, duration of follow-up, treatment protocols, responses to the therapies were discussed.Eight patients (6 male, 2 female) having refractory FMF were identified. Mediterranean fever (MEFV) gene analyses revealed homozygous M694V mutations in six patients and heterozygote M694V mutations in one patient and no mutation in one patient. They were all treated with anakinra and/or canakinumab. The use of anti-IL-1 drugs was beneficial to all patients. None of them had any severe adverse effects due to the therapy.Anakinra and canakinumab were effective in patient refractory to colchicine treatment as shown both in our series and in the literature. Therefore, controlled trials are needed to evaluate the safety and long-term efficacy of IL-1 targeting agents in colchicine resistant patients.

Proteinuria has been shown to be an important and potentially treatable risk factor for graft loss. The aim of this study was to evaluate prevalence, etiology, and outcome of proteinuria during the follow-up of children with renal transplantation. We retrospectively reviewed the files of renal transplanted children between 2006 and 2016 in our center. All patients were interpreted with respect to the demographic data and clinical and laboratory features including information about proteinuria. Chi-square test and Mann-Whitney U test were used for analysis. Fifty-two children were eligible for the study. Proteinuria was observed in 34 (65%) and nephrotic range proteinuria was detected in 5 (9.6%) patients. Etiology of proteinuria could be identified in 21 patients. Acute rejection and uncontrolled hypertension were the most frequent causes of proteinuria. Proteinuria had resolved during the follow-up in 59% of the patients. We found that children with and without proteinuria had similar glomerular filtration rate at the end of 50 months of follow-up period. Proteinuria seems to be a common complication in renal transplant recipients. Graft functions can be preserved by immediate evaluation of increasing proteinuria, and by fixing treatable causes rapidly and efficiently during the follow-up in majority of the patients.

Objectives The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schonlein purpura (HSP) when refractory to conventional therapies. Methods We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted. Results We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment. Conclusions Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.

Cytomegalovirus (CMV) infections are mostly seen in immunocompromised patients. However, unusual manifestations or complications of acquired CMV infections in immunocompetent patients are rarely reported. CMV-related hemorrhagic cystitis is extremely rare but should be considered even in immunocompetent patients. We present a case of a 3-year-old immunocompetent boy with intermittent, terminal gross hematuria lasting for 1 month. There was no history of genitourinary trauma or stone disease. Urine analysis revealed hematuria with eumorphic red blood cells and no proteinuria. Urine culture was negative. Ultrasonography showed increased bladder wall thickness and irregularity at inferior of bladder. Cystoscopy revealed hyperemia and edema. Histopathological examination was consistent with CMV infection, viral DNA by polymerase chain reaction in peripheral blood and urine were positive. Clinical, laboratory, and imaging features pointed towards hemorrhagic cystitis due to CMV. He was followed-up with no treatment. After 1 month, repeated investigations showed complete resolution of finding. This is a rare description of an immunocompetent child with CMV-induced cystitis.

To investigate the demographic and neurological features and treatment modalities of neuro-Behçet's disease (NBD) in children, to share our experiences and to summarise the literature.We retrospectively reviewed the medical records of Behçet's disease (BD) patients who attended our paediatric rheumatology department between December 2005 and October 2013. Five patients had the diagnosis of NBD. Initial neurological presentation, clinical BD presentation, magnetic resonance imaging pictures of those five patients was recorded.A total of 18 patients were diagnosed with BD. Among BD patients five of them were identified with NBD (27.8%). The mean age of NBD patients at the time of diagnosis was 12.4 years (range 5.5-15 years). The mean follow-up time after the neurological involvement was 5.2 years (range 0.5-14). In two cases neurological involvement occurred at the same time with the onset of other clinical findings of BD (40%). Both of these patients had parenchymal involvement. Three patients were admitted with headache as the initial neurological symptom. They revealed benign intracranial hypertension. One of them had cerebral venous sinus thrombosis (CVST). The other two had normal cranial magnetic resonance imagines. All patients received colchicine and steroid, two of them who had parenchymal involvement received also cytotoxic drugs.This study has shown that neurological symptoms can be the first manifestations of BD in children. Clinicians should be aware of this possibility and when a patient presents with neurological manifestations, it would be valuable to query the patient for the clinical features of Behçet's disease.

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, hyper-inflammatory syndrome that is characterized by untimely activation of macrophages, and manifests as cytopenia, organ dysfunction, and coagulopathy. Secondary HLH can be associated with infection, drugs, malignancy, and transplantation, and is mostly triggered by infection. Herein, we report the case of a patient with Henoch-Schonlein purpura (HSP) who developed severe HLH secondary to Varicella zoster infection.

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, hyper-inflammatory syndrome that is characterized by untimely activation of macrophages, and manifests as cytopenia, organ dysfunction, and coagulopathy. Secondary HLH can be associated with infection, drugs, malignancy, and transplantation, and is mostly triggered by infection. Herein, we report the case of a patient with Henoch-Schönlein purpura (HSP) who developed severe HLH secondary to Varicella zoster infection.

Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures. In addition, his symptoms fulfilled the 2012 revised criteria for the classification of SLE. He was treated with plasma infusions and methylprednisolone/prednisone. At follow up his laboratory findings and general condition improved and no relapse was seen.

Sjogren’s syndrome (SS) is a systemic autoimmune disease that presents with inflammation of lacrimal and salivary glands and accompanied by extraglanduler complications. The spectrum of disease extends from sicca symptoms to systemic involvement.In childhood, SS is an extremely rare autoimmune disorder (1).Here we present a girl with SS who was first presented with cutaneous lesions.