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Papers by seçil conkar

Research paper thumbnail of Early hemodiafiltration HDF is life saving in cases with mushroom poisoning

Research paper thumbnail of Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Pamukkale Medical Journal, 2015

Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clini... more Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, also other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta. Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, it is an important cause of renovasculer hypertension in childhood. Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We here presented our two cases diagnosed with hypertension while being screened for NF1 to emphasize the importance of blood pressure measurement in children with NF1 screening. Also we suggest that hypertensive NF1 patients should be examined in terms of stenosis in the major branch vessels of the aorta and renal arteries.

Research paper thumbnail of Çocukluk Yaşlarında Nefrotik Sendrom

Research paper thumbnail of Takrolimusa bağlı posterior geri dönüşümlü Ensefalopati Sendromu (PRES)

Solid organ ve hematopoetik kök hücre nakli artan sayıda yapılmaktadır. Bu nedenle siklosporin ve... more Solid organ ve hematopoetik kök hücre nakli artan sayıda yapılmaktadır. Bu nedenle siklosporin ve takrolimus gibi immünsupresif ajanların kullanımında önemli artışlar olmuştur. Posterior geri dönüşümlü ensefalopati sendromu (PRES) solid organ veya kök hücre nakli sonrasında immünsupresif tedavi kullanımının ciddi bir komplikasyonudur. Baş ağrısı, mental durum değişiklikleri, fokal nörolojik ve/veya görme bozuklukları gibi klinik bulgular görülmektedir. Bilgisayarlı tomografi (BT) veya manyetik rezonans görüntüleme (MRG) subkortikal beyaz cevher lezyonlarına ait karakteristik görüntüleme bulguları vardır. Subkortikal beyaz cevher değişiklikleri, geri dönüşümlü vazojenik ödeme veya geri dönüşümü olmayan sitotoksik ödem ikincil olarak görülebilir. Bu görüntüleme bulguları posterior serebral arter kanlanma bölgelerinde görülür. Birçok çalışma, takrolimus nörotoksisite ile ilişkili terapötik seviyelerde ortaya olabileceğini göstermiştir. PRES septomları çoğu durumda, ilaç dozunun azaltıl...

Research paper thumbnail of Renal hasarlı çocuklarda ayaktan kan basıncı izlemi ile hipertansiyon sıklığı

Amaç: Skarlı böbrek olan çocuklarda hipertansiyon sıklığını saptamak.Yöntem: Bu çalışma, Şubat 20... more Amaç: Skarlı böbrek olan çocuklarda hipertansiyon sıklığını saptamak.Yöntem: Bu çalışma, Şubat 2013 - Nisan 2015 tarihleri arasında Diyarbakır Çocuk Hastalıkları Hastanesi Çocuk Nefroloji Biriminde yürütülmüştür. Çalışmaya, renal skar tanısı konan toplam 54 çocuk hasta dahil edildi. Böbrek skarlanması tanısında Dimercaptosuccinic acid kullanıldı. Tüm hastalarda Spacelabs 90217 kullanılarak ayaktan kan basıncı izlemi uygulandı, ayrıca üriner mikroalbuminüri incelendi.Bulgular: Ofis ölçümleri sırasında renal skar bulunan 7 hastada (%13) hipertansiyon belirlendi. Öte yandan, ayaktan kan basıncı izlemi ile 23 (%42,7) sıklıkta hipertansiyon belirlendi. Dimercaptosuccinic acid renal incelemede şiddetli ve hafif tutulum gösteren hastalar, hipertansiyon ve mikroalbüminürisi sıklığı açısından karşılaştırıldı. Bununla birlikte, istatistiksel olarak anlamlı fark bulunamadı.Sonuç: Böbrek hasarlanması, çocuklarda hipertansiyon riski oluşturmaktadır. Renal hasarlı çocuklarda ofis kan basıncı ölçü...

Research paper thumbnail of The Use of Haemodiafltration in conditions other than renal insuffciency

Sürekli renal replasman tedavisi (SRRT) son zamanlarda akut böbrek yetmezlikli yoğun bakım hastal... more Sürekli renal replasman tedavisi (SRRT) son zamanlarda akut böbrek yetmezlikli yoğun bakım hastalarında önemli tedavi yöntemi haline gelmiştir. SRRT ayrıca nedeni belirlenemeyen böbrek dışı hastalıklarda da uygulanmaktadır. Sistemik yangısal yanıt sendromu, sepsis, akut sıkıntılı solunum sendromu gibi hastalıklarda infamatuvar sitokinlerin temizlenmesi amaçlanırken, doğuştan metabolizma bozuklukları, ezilme sendromu, laktik asidoz ve tümör lizis sendromu gibi tablolarda kullanım nedeni endojen toksik solütlerin temizlenmesidir. Hemodiyofltrayonun son zamanlarda giderek artan kullanımı olmasına rağmen klinik çalışmalarda henüz etkinliği tam olarak gösterilmemiştir. Aşırı diyalizin faydalı etkisi yoktur. Sonuçta hastanın uygun diyaliz tedavisi için tedaviyi uygulayan doktorun, diyaliz hemşiresinin ilgisi, takibi diyaliz tipinden daha etkili olduğu düşünülmektedir. (Ha se ki Tıp Bül te ni 2015; 53: 110-15)Continuous renal replacement therapy (CRRT) has recently become an important trea...

Research paper thumbnail of Comparison of Hemodialysis and Online Hemodiafiltration in Removal of Uremic Toxins

Hemodiyaliz (HD) tedavisi son yıllarda son dö- nem böbrek yetmezlikli hastaların yaşam süresini u... more Hemodiyaliz (HD) tedavisi son yıllarda son dö- nem böbrek yetmezlikli hastaların yaşam süresini uzatmıştır. Standart veya geleneksel HD normal böbrek fonksiyonlarını tam olarak karşılayamamaktadır. Üremik toksinlerin tam olarak temizlenememesi kardiovasküler mortaliteye ve morbititeye yol açmaktadır. Bu derlemede değişik çalışmalar ve yeniliklerin temelinde hastaların yaşam kalitesini yükseltecek ve mortalite, morbititeyi azaltacak değişik diyaliz tekniklerinden söz edilecektir.Hemodialysis (HD) treatment in recent years has extended the life span of patients with end stage renal disease. Standard or conventional HD is far from fully compensating normal renal functions. Inadequately cleaned uremic toxins lead to cardiovascular morbidity and mortality. in this review, different dialysis techniques improving the quality of life of patients and reducing morbidity and mortality will be discussed on the basis of various studies and scientific innovations

Research paper thumbnail of Yeni Tanımlanmış Bir Sendrom olan Shrunken Pore Sendromlu Çocuk Hasta

Journal of Contemporary Medicine, 2020

GİRİŞ: Sistatin C bazlı tahmini Glomerüler filtrasyon hızı (GFR) ölçümleri son dönem böbrek yetme... more GİRİŞ: Sistatin C bazlı tahmini Glomerüler filtrasyon hızı (GFR) ölçümleri son dönem böbrek yetmezliği, kardiyovasküler bulgular ve mortaliteyi öngörmede kreatinin bazlı ölçümlerden üstün olup; cinsiyet, yaş ve kas kitlesinden bağımsızdır. Bazı olgularda sistatin C'nin filtrasyonunun, kreatinine göre daha az olduğu gözlemlenmiş ve bunun porların daralmasından kaynaklandığı kanıtlanmıştır. Sistatin C bazlı tahmini GFR (eGFRsistatin C), kreatinin bazlı tahmini GFR'nin (eGFRkreatinin) %60'ına eşit veya bunun altında olması patofizyolojik olarak 'Shrunken Pore Sendromu' olarak tanımlanmıştır. Bu yazıda Shrunlen Pore sendromlu bir kız olgudan bahsedildi. OLGU: 16 aylık kız hastanın meningomiyelosel ve nörojenik mesane ile Ege Üniversitesi Çocuk Nefroloji polikliniğine başvurdu. Özgeçmişinde antenatal meningomyelosel ve hidrosefali nedeniyle Arnold Chiari tip 2 tanısı, postnatal 1. günde meningomyelosel kesesi eksizyonu ve ventriküloperitoneal şant operasyonu mevcuttu....

Research paper thumbnail of Genetics and Consequences of Atypical Hemolytic-uremic Syndrome in Turkish Children

WOS: 000492798700005Introduction. Atypical hemolytic uremic syndrome (aHUS) is associated with mu... more WOS: 000492798700005Introduction. Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. Several studies were published recently, describing these mutations. We present the initial clinical findings, treatments, and long-term follow-up results of 19 patients hospitalized with the diagnosis of aHUS. Methods. Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. the reasons for complement factor H (CFH) mutations were investigated. Results. CFH mutations were detected in 5 of the 19 aHUS cases. of these, one was novel, while four were previously reported. We reported here the clinical course of aHUS patients with CFH previously defined mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys), which caused previously defined aHUS. Two of the CFH mutation cases developed end-stage kidney disease that required hemodialysis, and one patient developed chronic kidney disease. Two cases were in remission; one of them under supportive therapy and the other one in remission with eculizumab treatment. Conclusions. Morbidity rates are higher in children with aHUS. However, renal prognosis and morbidity rates are higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS-children with CFH mutation depends on the genetic background

Research paper thumbnail of Özel Hasta Gruplarında Periton Diyalizi Uygulaması (Kolostomi, Polikistik Böbrek Hastalığı, Prune Belly, Mental Retarde, Down Sendromu)

Research paper thumbnail of Genetics and outcome of atypical hemolytic-uremic syndrome in Turkish children: a retrospective study between 2010 and 2017, a single-center experience

Iranian journal of kidney diseases, 2019

INTRODUCTION Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies... more INTRODUCTION Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. In the recent years several studies have been published describing these mutations. In this study, the initial clinical findings, treatments and long-term follow-up results of 19 patients who were hospitalized with the diagnosis of aHUS were presented. MATERIALS AND METHODS Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. Disease causing complement factor H (CFH) mutations were determined. Results. CFH mutations were detected in 5 of 19 aHUS cases. Of these, one was novel and 4 were previously reported. We reported here the clinical course of aHUS patients with CFH mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys) which caused previously defined aHUS. Two of the CFH mutation ...

Research paper thumbnail of Hipertansiyon sebebi olarak Nörofibromatozis Tip 1(NF1)

Norofibromatozis tip 1 otozomal dominant gecis gosteren noroektodermal ve mesodermal dokularin di... more Norofibromatozis tip 1 otozomal dominant gecis gosteren noroektodermal ve mesodermal dokularin displazisi sonucu ortaya cikan klinik bulgular ile karekterizedir. Cafe-au-lait denilen karekteristik pigmente deri lezyonu, aksiller ve inguinal cillenme, deri norofibromalari, Lisch nodulleri denilen pigmente iris hamartomalari, merkezi sinir sistemi tumorleri, periferik sinir sistemi tumorleri ve diger sistemleri ilgilendiren tumorler gorulebilir. Norofibramatoz tip 1 de hipertansiyon renovaskuler hastaliklar, mid-aortik sendrom veya feokromasitomaya bagli olarak gelisebilir. Cocukluk yaslarinda norofibramatoza bagli hipertansiyonun nedeni genellikle renal arter stenozu olup genellikle damarin proksimalini veya orijini ilgilendirir. Hastalarin %25’inde aort koarktasyonu ile birliktedir. Norofibramatozis nadir gorulen bir hastalik olsada cocukluk caginda ki renovaskuler hipertansiyonun onemli nedenlerinden biridir. Norofibramatoz tip 1 nedeniyle izlenirken hipertansiyon tespit edilen iki...

Research paper thumbnail of The Pediatric Case of a Recently Defined Syndrome: Shrunken Pore Syndrome

Journal of contemporary medicine, 2020

GIRIŞ: Sistatin C bazli tahmini Glomeruler filtrasyon hizi (GFR) olcumleri son donem bobrek yetme... more GIRIŞ: Sistatin C bazli tahmini Glomeruler filtrasyon hizi (GFR) olcumleri son donem bobrek yetmezligi, kardiyovaskuler bulgular ve mortaliteyi ongormede kreatinin bazli olcumlerden ustun olup; cinsiyet, yas ve kas kitlesinden bagimsizdir. Bazi olgularda sistatin C’nin filtrasyonunun, kreatinine gore daha az oldugu gozlemlenmis ve bunun porlarin daralmasindan kaynaklandigi kanitlanmistir. Sistatin C bazli tahmini GFR (eGFRsistatin C), kreatinin bazli tahmini GFR’nin (eGFRkreatinin) %60’ina esit veya bunun altinda olmasi patofizyolojik olarak ‘Shrunken Pore Sendromu’ olarak tanimlanmistir. Bu yazida Shrunlen Pore sendromlu bir kiz olgudan bahsedildi. OLGU: 16 aylik kiz hastanin meningomiyelosel ve norojenik mesane ile Ege Universitesi Cocuk Nefroloji poliklinigine basvurdu. Ozgecmisinde antenatal meningomyelosel ve hidrosefali nedeniyle Arnold Chiari tip 2 tanisi, postnatal 1. gunde meningomyelosel kesesi eksizyonu ve ventrikuloperitoneal sant operasyonu mevcuttu. Piyelonefrit oykusu...

Research paper thumbnail of Şiddetli Hipertansiyon Ile Birlikte Olan Anjiomiyolipom Olgusu

Son zamanlarda adelosan yasta hipertansiyon gorulme sikligi artmaktadir. Bu artis obezite sikligi... more Son zamanlarda adelosan yasta hipertansiyon gorulme sikligi artmaktadir. Bu artis obezite sikliginda artis ile paralellik gostermektedir. Adelosan yas grubunda hipertansiyonun ana nedeni esansiyel hipertansiyondur. Ancak hipertansiyonun diger nadir nedenlerinin esansiyel hipertansiyon teshisi oncesinde ayirici tanisi yapilmalidir. Bobrek kitleleri cocuklarda sik gorulmeyen iyi veya kotu huylu olusumlardir. Bobrek kitleleri cocukluk caginda hipertansiyonun nadir nedenidir. Anjiyomiyolipom (AML), cocukluk caginda nadir bobrek mezenkimal tumorlerinden biridir. AML genellikle tubero skleroz (TS) ile bir arada olup TS klinik olarak on plandadir. Burada TSnin eslik etmedigi malign HT olarak ortaya cikan bir AML olgusunu sunduk. Adelosan donemde malign hipertansiyon nedeni olarak bobrek anjiyomiyolipomu olabilecegi unutulmamalidir.

Research paper thumbnail of A case of angiomyolipoma associated with severe hypertension

Hypertension incidence in adolescent age has been increasing recently. One of the causes of this ... more Hypertension incidence in adolescent age has been increasing recently. One of the causes of this increase is the increase in obesity incidence. The main cause of hypertension in adolescent age group is primary essential hypertension. However other rare causes of hypertension should be discriminated prior to diagnosing primary essential hypertension. Renal masses are benign or malignant formations that are not commonly seen in childhood. Renal masses are a rare cause of hypertension in childhood. Angiomyolipoma (AML) is one of the rare renal mesenchymal tumors in childhood. AML usually coexists with tuberous sclerosis (TS) and TS cases are clinically in the foreground. Here we present a case of AML case emerging as malignant HT without TS. In adolescence, it sholud be kept in mind that renal AML may cause malignant hypertension.

Research paper thumbnail of Frequency of Vitamin D Deficiencies in Children Who Living in the South-East of Turkey Region with Plenty of Sunshine

Annals of Clinical and Laboratory Research, 2017

Objectives: The present study was designed to evaluate the relationship between vitamin D, vitami... more Objectives: The present study was designed to evaluate the relationship between vitamin D, vitamin B12 and folic acid levels and nutritional status among 24 to 60 months children. Methods: In this cross-sectional study were performed 116 children at Diyarbakır children hospital. Serum vitamin B12, folic acid and vitamin D levels were measured in all children. Anthropometric indices of weight-forage and BMI-forage were used to estimate the children's nutritional status. Z-scores for weight-forage (WAZ) were derived from Epi info and WHO Anthro Plus softwares. The nutritional status was classified as normal (± 2SD), moderate (<-2SD ≥-3SD) and severe (<-3SD). Results: The mean serum levels of Vitamin B12, vitamin D, Folic acid and haemoglobinin the study population were 223.1 ± 8.89 pg/mL, 23.5 ± 8.97 ng/mL, 26.5 ± 18.4 ng/mL and 11.6 ± 3.24 g/dL, respectively. Vitamin D deficiency, Folate deficiency and vitamin B12 deficiency occurred in 41.4%, 3.4%, and 24.1% of the 24 to 60 months children, respectively. The mean levels of the biochemical indices of vitamin D, vitamin B12 and folic acid levels and haemoglobin levels were not statistically significantly different among different levels of nutritional status normal (p>0.05) and moderate (p>0.05). Conclusion: The present study shows that, despite the sunny environment, vitamin D deficiency is highly prevalent among of 24 to 60 months children. These findings suggest that much more efficient vitamin D prophylaxis programs should be implemented for risk groups such as infants and 24 to 60 months children.

Research paper thumbnail of The Role of Neonatal Sepsis in the Development of Allergic Diseases in Childhood

The Journal of Pediatric Research, 2016

We aimed to demonstrate the effect of neonatal sepsis on the development of allergic diseases at ... more We aimed to demonstrate the effect of neonatal sepsis on the development of allergic diseases at later ages. Materials and Methods: For the study, 126 children who were diagnosed with sepsis in the neonatal period, and hospitalized between 2001 and 2003 were recruited, and divided into two groups. Group one consisted of children who had sepsis in the neonatal period. Group two children were chosen from among group one's siblings as controls in whom genetic and environmental factors leading to allergic diseases were similar. The prevalence of allergic diseases were compared between the two groups. The Turkish version of the International Study of Asthma and Allergies in Children questionnaire was used via the face-to-face methodology. Additionally, total blood count was studied for potential eosinophilia, total immunoglobulin E (IgE) levels were measured, and skin prick tests were performed on each subject. Results: Total IgE levels and sensitivity to Dermatophagoides pteronyssinus, Dermatophagoides farinea were significantly lower (p<0.05) in group one. Also the prevalence of asthma and allergic symptoms were significantly less common (p<0.05) in the first group. Interestingly, we found no significant difference in the prevalence of allergic rhinitis and atopic dermatitis between the two groups. Conclusion: The present study highlighted that contact with severe infections such as sepsis in the neonatal period can be a cause of decreased sensitivity to environmental allergens and the prevalence of asthma in childhood.

Research paper thumbnail of Renal abscess in a previously healthy 4-year-old girl: A case report

Pediatric Urology Case Reports, 2016

Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abd... more Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abdominal pain and occasional flank mass. Renal ultrasonography enables us to achieve an early diagnosis, however; it may still be insufficient to distinguish it from pyelonephritis. Renal abscesses are usually associated with different predisposing conditions. In the present report, we aim to describe a case of a previously healthy child who developed a renal abscess.

Research paper thumbnail of Outcome of Antenatal Hydronephrosis

Annals of Clinical and Laboratory Research, 2016

Objective: The aim of this study was to report the outcome of infants with antenatal hydronephros... more Objective: The aim of this study was to report the outcome of infants with antenatal hydronephrosis. Methods: All patients diagnosed with isolated fetal renal pelvic dilatation (RPD) were prospectively followed between January 2009 and December 2010. The events of interest were the presence of uropathy, requiring surgical intervention, RPD resolution and urinary tract infection (UTI). The diagnosis was established by ultrasound in 49 neonates with antenatal hydronephrosis (ANH) and they were submitted to a specific post-natal evaluative protocol with a follow-up period of 2 years. RPD was classified as mild (5-9.9 mm), moderate (10-14.9 mm) or severe (≥15 mm). Results: A total of 49 patients were included in the analysis; 32 were allocated to the group of non-significant findings (65.3%) and 17 to the group of significant uropathy (34.6%). It was detected that 8 (16.3%) of the group of significant uropathy had ureteropelvic junction obstruction (UPJO), 3 (6.1%) had vesicoureteral reflux (VUR), and 3 (6.1%) had posterior urethral valves (PUV). 9 patients (18.3%) required surgical intervention because of obstructive uropathy. Of the 49 infants with RPD, 20 (40.8%) presented UTI and 32 (65.3%) presented RPD resolution during followup. In our prospective study, it was shown that 81.9% of infants with moderate RPD had UTI during follow-up. Conclusion: Our findings suggested that, in contrast to patients with mild/severe RPD, infants with moderate RPD required a strict clinical surveillance for UTI.

Research paper thumbnail of Evaluation and therapy in four patients with Takayasu′s arteritis

Saudi Journal of Kidney Diseases and Transplantation, 2016

Takayasu&#39;s arteritis (TA) is a large-vessel vasculitis primarily affecting the aorta and ... more Takayasu&#39;s arteritis (TA) is a large-vessel vasculitis primarily affecting the aorta and its branches. It ranks third among other types of childhood vasculitis, while it is the most common large vessel vasculitis observed in childhood. The diagnosis of TA should be made on the basis of clinical criteria and supported with laboratory findings, while confirming it with the imaging methods. Angioplasty, stent and bypass grafts may be necessary in the case of an irreversible arterial stenosis. Small-vessel involvement in TA and acute phase reactants should be taken into account for the diagnosis of an attack. In this report, treatment choices for four patients with the diagnosis of pediatric TA, their clinical and laboratory findings and their responses to treatment will be discussed.

Research paper thumbnail of Early hemodiafiltration HDF is life saving in cases with mushroom poisoning

Research paper thumbnail of Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Pamukkale Medical Journal, 2015

Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clini... more Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, also other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta. Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, it is an important cause of renovasculer hypertension in childhood. Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We here presented our two cases diagnosed with hypertension while being screened for NF1 to emphasize the importance of blood pressure measurement in children with NF1 screening. Also we suggest that hypertensive NF1 patients should be examined in terms of stenosis in the major branch vessels of the aorta and renal arteries.

Research paper thumbnail of Çocukluk Yaşlarında Nefrotik Sendrom

Research paper thumbnail of Takrolimusa bağlı posterior geri dönüşümlü Ensefalopati Sendromu (PRES)

Solid organ ve hematopoetik kök hücre nakli artan sayıda yapılmaktadır. Bu nedenle siklosporin ve... more Solid organ ve hematopoetik kök hücre nakli artan sayıda yapılmaktadır. Bu nedenle siklosporin ve takrolimus gibi immünsupresif ajanların kullanımında önemli artışlar olmuştur. Posterior geri dönüşümlü ensefalopati sendromu (PRES) solid organ veya kök hücre nakli sonrasında immünsupresif tedavi kullanımının ciddi bir komplikasyonudur. Baş ağrısı, mental durum değişiklikleri, fokal nörolojik ve/veya görme bozuklukları gibi klinik bulgular görülmektedir. Bilgisayarlı tomografi (BT) veya manyetik rezonans görüntüleme (MRG) subkortikal beyaz cevher lezyonlarına ait karakteristik görüntüleme bulguları vardır. Subkortikal beyaz cevher değişiklikleri, geri dönüşümlü vazojenik ödeme veya geri dönüşümü olmayan sitotoksik ödem ikincil olarak görülebilir. Bu görüntüleme bulguları posterior serebral arter kanlanma bölgelerinde görülür. Birçok çalışma, takrolimus nörotoksisite ile ilişkili terapötik seviyelerde ortaya olabileceğini göstermiştir. PRES septomları çoğu durumda, ilaç dozunun azaltıl...

Research paper thumbnail of Renal hasarlı çocuklarda ayaktan kan basıncı izlemi ile hipertansiyon sıklığı

Amaç: Skarlı böbrek olan çocuklarda hipertansiyon sıklığını saptamak.Yöntem: Bu çalışma, Şubat 20... more Amaç: Skarlı böbrek olan çocuklarda hipertansiyon sıklığını saptamak.Yöntem: Bu çalışma, Şubat 2013 - Nisan 2015 tarihleri arasında Diyarbakır Çocuk Hastalıkları Hastanesi Çocuk Nefroloji Biriminde yürütülmüştür. Çalışmaya, renal skar tanısı konan toplam 54 çocuk hasta dahil edildi. Böbrek skarlanması tanısında Dimercaptosuccinic acid kullanıldı. Tüm hastalarda Spacelabs 90217 kullanılarak ayaktan kan basıncı izlemi uygulandı, ayrıca üriner mikroalbuminüri incelendi.Bulgular: Ofis ölçümleri sırasında renal skar bulunan 7 hastada (%13) hipertansiyon belirlendi. Öte yandan, ayaktan kan basıncı izlemi ile 23 (%42,7) sıklıkta hipertansiyon belirlendi. Dimercaptosuccinic acid renal incelemede şiddetli ve hafif tutulum gösteren hastalar, hipertansiyon ve mikroalbüminürisi sıklığı açısından karşılaştırıldı. Bununla birlikte, istatistiksel olarak anlamlı fark bulunamadı.Sonuç: Böbrek hasarlanması, çocuklarda hipertansiyon riski oluşturmaktadır. Renal hasarlı çocuklarda ofis kan basıncı ölçü...

Research paper thumbnail of The Use of Haemodiafltration in conditions other than renal insuffciency

Sürekli renal replasman tedavisi (SRRT) son zamanlarda akut böbrek yetmezlikli yoğun bakım hastal... more Sürekli renal replasman tedavisi (SRRT) son zamanlarda akut böbrek yetmezlikli yoğun bakım hastalarında önemli tedavi yöntemi haline gelmiştir. SRRT ayrıca nedeni belirlenemeyen böbrek dışı hastalıklarda da uygulanmaktadır. Sistemik yangısal yanıt sendromu, sepsis, akut sıkıntılı solunum sendromu gibi hastalıklarda infamatuvar sitokinlerin temizlenmesi amaçlanırken, doğuştan metabolizma bozuklukları, ezilme sendromu, laktik asidoz ve tümör lizis sendromu gibi tablolarda kullanım nedeni endojen toksik solütlerin temizlenmesidir. Hemodiyofltrayonun son zamanlarda giderek artan kullanımı olmasına rağmen klinik çalışmalarda henüz etkinliği tam olarak gösterilmemiştir. Aşırı diyalizin faydalı etkisi yoktur. Sonuçta hastanın uygun diyaliz tedavisi için tedaviyi uygulayan doktorun, diyaliz hemşiresinin ilgisi, takibi diyaliz tipinden daha etkili olduğu düşünülmektedir. (Ha se ki Tıp Bül te ni 2015; 53: 110-15)Continuous renal replacement therapy (CRRT) has recently become an important trea...

Research paper thumbnail of Comparison of Hemodialysis and Online Hemodiafiltration in Removal of Uremic Toxins

Hemodiyaliz (HD) tedavisi son yıllarda son dö- nem böbrek yetmezlikli hastaların yaşam süresini u... more Hemodiyaliz (HD) tedavisi son yıllarda son dö- nem böbrek yetmezlikli hastaların yaşam süresini uzatmıştır. Standart veya geleneksel HD normal böbrek fonksiyonlarını tam olarak karşılayamamaktadır. Üremik toksinlerin tam olarak temizlenememesi kardiovasküler mortaliteye ve morbititeye yol açmaktadır. Bu derlemede değişik çalışmalar ve yeniliklerin temelinde hastaların yaşam kalitesini yükseltecek ve mortalite, morbititeyi azaltacak değişik diyaliz tekniklerinden söz edilecektir.Hemodialysis (HD) treatment in recent years has extended the life span of patients with end stage renal disease. Standard or conventional HD is far from fully compensating normal renal functions. Inadequately cleaned uremic toxins lead to cardiovascular morbidity and mortality. in this review, different dialysis techniques improving the quality of life of patients and reducing morbidity and mortality will be discussed on the basis of various studies and scientific innovations

Research paper thumbnail of Yeni Tanımlanmış Bir Sendrom olan Shrunken Pore Sendromlu Çocuk Hasta

Journal of Contemporary Medicine, 2020

GİRİŞ: Sistatin C bazlı tahmini Glomerüler filtrasyon hızı (GFR) ölçümleri son dönem böbrek yetme... more GİRİŞ: Sistatin C bazlı tahmini Glomerüler filtrasyon hızı (GFR) ölçümleri son dönem böbrek yetmezliği, kardiyovasküler bulgular ve mortaliteyi öngörmede kreatinin bazlı ölçümlerden üstün olup; cinsiyet, yaş ve kas kitlesinden bağımsızdır. Bazı olgularda sistatin C'nin filtrasyonunun, kreatinine göre daha az olduğu gözlemlenmiş ve bunun porların daralmasından kaynaklandığı kanıtlanmıştır. Sistatin C bazlı tahmini GFR (eGFRsistatin C), kreatinin bazlı tahmini GFR'nin (eGFRkreatinin) %60'ına eşit veya bunun altında olması patofizyolojik olarak 'Shrunken Pore Sendromu' olarak tanımlanmıştır. Bu yazıda Shrunlen Pore sendromlu bir kız olgudan bahsedildi. OLGU: 16 aylık kız hastanın meningomiyelosel ve nörojenik mesane ile Ege Üniversitesi Çocuk Nefroloji polikliniğine başvurdu. Özgeçmişinde antenatal meningomyelosel ve hidrosefali nedeniyle Arnold Chiari tip 2 tanısı, postnatal 1. günde meningomyelosel kesesi eksizyonu ve ventriküloperitoneal şant operasyonu mevcuttu....

Research paper thumbnail of Genetics and Consequences of Atypical Hemolytic-uremic Syndrome in Turkish Children

WOS: 000492798700005Introduction. Atypical hemolytic uremic syndrome (aHUS) is associated with mu... more WOS: 000492798700005Introduction. Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. Several studies were published recently, describing these mutations. We present the initial clinical findings, treatments, and long-term follow-up results of 19 patients hospitalized with the diagnosis of aHUS. Methods. Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. the reasons for complement factor H (CFH) mutations were investigated. Results. CFH mutations were detected in 5 of the 19 aHUS cases. of these, one was novel, while four were previously reported. We reported here the clinical course of aHUS patients with CFH previously defined mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys), which caused previously defined aHUS. Two of the CFH mutation cases developed end-stage kidney disease that required hemodialysis, and one patient developed chronic kidney disease. Two cases were in remission; one of them under supportive therapy and the other one in remission with eculizumab treatment. Conclusions. Morbidity rates are higher in children with aHUS. However, renal prognosis and morbidity rates are higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS-children with CFH mutation depends on the genetic background

Research paper thumbnail of Özel Hasta Gruplarında Periton Diyalizi Uygulaması (Kolostomi, Polikistik Böbrek Hastalığı, Prune Belly, Mental Retarde, Down Sendromu)

Research paper thumbnail of Genetics and outcome of atypical hemolytic-uremic syndrome in Turkish children: a retrospective study between 2010 and 2017, a single-center experience

Iranian journal of kidney diseases, 2019

INTRODUCTION Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies... more INTRODUCTION Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. In the recent years several studies have been published describing these mutations. In this study, the initial clinical findings, treatments and long-term follow-up results of 19 patients who were hospitalized with the diagnosis of aHUS were presented. MATERIALS AND METHODS Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. Disease causing complement factor H (CFH) mutations were determined. Results. CFH mutations were detected in 5 of 19 aHUS cases. Of these, one was novel and 4 were previously reported. We reported here the clinical course of aHUS patients with CFH mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys) which caused previously defined aHUS. Two of the CFH mutation ...

Research paper thumbnail of Hipertansiyon sebebi olarak Nörofibromatozis Tip 1(NF1)

Norofibromatozis tip 1 otozomal dominant gecis gosteren noroektodermal ve mesodermal dokularin di... more Norofibromatozis tip 1 otozomal dominant gecis gosteren noroektodermal ve mesodermal dokularin displazisi sonucu ortaya cikan klinik bulgular ile karekterizedir. Cafe-au-lait denilen karekteristik pigmente deri lezyonu, aksiller ve inguinal cillenme, deri norofibromalari, Lisch nodulleri denilen pigmente iris hamartomalari, merkezi sinir sistemi tumorleri, periferik sinir sistemi tumorleri ve diger sistemleri ilgilendiren tumorler gorulebilir. Norofibramatoz tip 1 de hipertansiyon renovaskuler hastaliklar, mid-aortik sendrom veya feokromasitomaya bagli olarak gelisebilir. Cocukluk yaslarinda norofibramatoza bagli hipertansiyonun nedeni genellikle renal arter stenozu olup genellikle damarin proksimalini veya orijini ilgilendirir. Hastalarin %25’inde aort koarktasyonu ile birliktedir. Norofibramatozis nadir gorulen bir hastalik olsada cocukluk caginda ki renovaskuler hipertansiyonun onemli nedenlerinden biridir. Norofibramatoz tip 1 nedeniyle izlenirken hipertansiyon tespit edilen iki...

Research paper thumbnail of The Pediatric Case of a Recently Defined Syndrome: Shrunken Pore Syndrome

Journal of contemporary medicine, 2020

GIRIŞ: Sistatin C bazli tahmini Glomeruler filtrasyon hizi (GFR) olcumleri son donem bobrek yetme... more GIRIŞ: Sistatin C bazli tahmini Glomeruler filtrasyon hizi (GFR) olcumleri son donem bobrek yetmezligi, kardiyovaskuler bulgular ve mortaliteyi ongormede kreatinin bazli olcumlerden ustun olup; cinsiyet, yas ve kas kitlesinden bagimsizdir. Bazi olgularda sistatin C’nin filtrasyonunun, kreatinine gore daha az oldugu gozlemlenmis ve bunun porlarin daralmasindan kaynaklandigi kanitlanmistir. Sistatin C bazli tahmini GFR (eGFRsistatin C), kreatinin bazli tahmini GFR’nin (eGFRkreatinin) %60’ina esit veya bunun altinda olmasi patofizyolojik olarak ‘Shrunken Pore Sendromu’ olarak tanimlanmistir. Bu yazida Shrunlen Pore sendromlu bir kiz olgudan bahsedildi. OLGU: 16 aylik kiz hastanin meningomiyelosel ve norojenik mesane ile Ege Universitesi Cocuk Nefroloji poliklinigine basvurdu. Ozgecmisinde antenatal meningomyelosel ve hidrosefali nedeniyle Arnold Chiari tip 2 tanisi, postnatal 1. gunde meningomyelosel kesesi eksizyonu ve ventrikuloperitoneal sant operasyonu mevcuttu. Piyelonefrit oykusu...

Research paper thumbnail of Şiddetli Hipertansiyon Ile Birlikte Olan Anjiomiyolipom Olgusu

Son zamanlarda adelosan yasta hipertansiyon gorulme sikligi artmaktadir. Bu artis obezite sikligi... more Son zamanlarda adelosan yasta hipertansiyon gorulme sikligi artmaktadir. Bu artis obezite sikliginda artis ile paralellik gostermektedir. Adelosan yas grubunda hipertansiyonun ana nedeni esansiyel hipertansiyondur. Ancak hipertansiyonun diger nadir nedenlerinin esansiyel hipertansiyon teshisi oncesinde ayirici tanisi yapilmalidir. Bobrek kitleleri cocuklarda sik gorulmeyen iyi veya kotu huylu olusumlardir. Bobrek kitleleri cocukluk caginda hipertansiyonun nadir nedenidir. Anjiyomiyolipom (AML), cocukluk caginda nadir bobrek mezenkimal tumorlerinden biridir. AML genellikle tubero skleroz (TS) ile bir arada olup TS klinik olarak on plandadir. Burada TSnin eslik etmedigi malign HT olarak ortaya cikan bir AML olgusunu sunduk. Adelosan donemde malign hipertansiyon nedeni olarak bobrek anjiyomiyolipomu olabilecegi unutulmamalidir.

Research paper thumbnail of A case of angiomyolipoma associated with severe hypertension

Hypertension incidence in adolescent age has been increasing recently. One of the causes of this ... more Hypertension incidence in adolescent age has been increasing recently. One of the causes of this increase is the increase in obesity incidence. The main cause of hypertension in adolescent age group is primary essential hypertension. However other rare causes of hypertension should be discriminated prior to diagnosing primary essential hypertension. Renal masses are benign or malignant formations that are not commonly seen in childhood. Renal masses are a rare cause of hypertension in childhood. Angiomyolipoma (AML) is one of the rare renal mesenchymal tumors in childhood. AML usually coexists with tuberous sclerosis (TS) and TS cases are clinically in the foreground. Here we present a case of AML case emerging as malignant HT without TS. In adolescence, it sholud be kept in mind that renal AML may cause malignant hypertension.

Research paper thumbnail of Frequency of Vitamin D Deficiencies in Children Who Living in the South-East of Turkey Region with Plenty of Sunshine

Annals of Clinical and Laboratory Research, 2017

Objectives: The present study was designed to evaluate the relationship between vitamin D, vitami... more Objectives: The present study was designed to evaluate the relationship between vitamin D, vitamin B12 and folic acid levels and nutritional status among 24 to 60 months children. Methods: In this cross-sectional study were performed 116 children at Diyarbakır children hospital. Serum vitamin B12, folic acid and vitamin D levels were measured in all children. Anthropometric indices of weight-forage and BMI-forage were used to estimate the children's nutritional status. Z-scores for weight-forage (WAZ) were derived from Epi info and WHO Anthro Plus softwares. The nutritional status was classified as normal (± 2SD), moderate (<-2SD ≥-3SD) and severe (<-3SD). Results: The mean serum levels of Vitamin B12, vitamin D, Folic acid and haemoglobinin the study population were 223.1 ± 8.89 pg/mL, 23.5 ± 8.97 ng/mL, 26.5 ± 18.4 ng/mL and 11.6 ± 3.24 g/dL, respectively. Vitamin D deficiency, Folate deficiency and vitamin B12 deficiency occurred in 41.4%, 3.4%, and 24.1% of the 24 to 60 months children, respectively. The mean levels of the biochemical indices of vitamin D, vitamin B12 and folic acid levels and haemoglobin levels were not statistically significantly different among different levels of nutritional status normal (p>0.05) and moderate (p>0.05). Conclusion: The present study shows that, despite the sunny environment, vitamin D deficiency is highly prevalent among of 24 to 60 months children. These findings suggest that much more efficient vitamin D prophylaxis programs should be implemented for risk groups such as infants and 24 to 60 months children.

Research paper thumbnail of The Role of Neonatal Sepsis in the Development of Allergic Diseases in Childhood

The Journal of Pediatric Research, 2016

We aimed to demonstrate the effect of neonatal sepsis on the development of allergic diseases at ... more We aimed to demonstrate the effect of neonatal sepsis on the development of allergic diseases at later ages. Materials and Methods: For the study, 126 children who were diagnosed with sepsis in the neonatal period, and hospitalized between 2001 and 2003 were recruited, and divided into two groups. Group one consisted of children who had sepsis in the neonatal period. Group two children were chosen from among group one's siblings as controls in whom genetic and environmental factors leading to allergic diseases were similar. The prevalence of allergic diseases were compared between the two groups. The Turkish version of the International Study of Asthma and Allergies in Children questionnaire was used via the face-to-face methodology. Additionally, total blood count was studied for potential eosinophilia, total immunoglobulin E (IgE) levels were measured, and skin prick tests were performed on each subject. Results: Total IgE levels and sensitivity to Dermatophagoides pteronyssinus, Dermatophagoides farinea were significantly lower (p<0.05) in group one. Also the prevalence of asthma and allergic symptoms were significantly less common (p<0.05) in the first group. Interestingly, we found no significant difference in the prevalence of allergic rhinitis and atopic dermatitis between the two groups. Conclusion: The present study highlighted that contact with severe infections such as sepsis in the neonatal period can be a cause of decreased sensitivity to environmental allergens and the prevalence of asthma in childhood.

Research paper thumbnail of Renal abscess in a previously healthy 4-year-old girl: A case report

Pediatric Urology Case Reports, 2016

Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abd... more Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abdominal pain and occasional flank mass. Renal ultrasonography enables us to achieve an early diagnosis, however; it may still be insufficient to distinguish it from pyelonephritis. Renal abscesses are usually associated with different predisposing conditions. In the present report, we aim to describe a case of a previously healthy child who developed a renal abscess.

Research paper thumbnail of Outcome of Antenatal Hydronephrosis

Annals of Clinical and Laboratory Research, 2016

Objective: The aim of this study was to report the outcome of infants with antenatal hydronephros... more Objective: The aim of this study was to report the outcome of infants with antenatal hydronephrosis. Methods: All patients diagnosed with isolated fetal renal pelvic dilatation (RPD) were prospectively followed between January 2009 and December 2010. The events of interest were the presence of uropathy, requiring surgical intervention, RPD resolution and urinary tract infection (UTI). The diagnosis was established by ultrasound in 49 neonates with antenatal hydronephrosis (ANH) and they were submitted to a specific post-natal evaluative protocol with a follow-up period of 2 years. RPD was classified as mild (5-9.9 mm), moderate (10-14.9 mm) or severe (≥15 mm). Results: A total of 49 patients were included in the analysis; 32 were allocated to the group of non-significant findings (65.3%) and 17 to the group of significant uropathy (34.6%). It was detected that 8 (16.3%) of the group of significant uropathy had ureteropelvic junction obstruction (UPJO), 3 (6.1%) had vesicoureteral reflux (VUR), and 3 (6.1%) had posterior urethral valves (PUV). 9 patients (18.3%) required surgical intervention because of obstructive uropathy. Of the 49 infants with RPD, 20 (40.8%) presented UTI and 32 (65.3%) presented RPD resolution during followup. In our prospective study, it was shown that 81.9% of infants with moderate RPD had UTI during follow-up. Conclusion: Our findings suggested that, in contrast to patients with mild/severe RPD, infants with moderate RPD required a strict clinical surveillance for UTI.

Research paper thumbnail of Evaluation and therapy in four patients with Takayasu′s arteritis

Saudi Journal of Kidney Diseases and Transplantation, 2016

Takayasu&#39;s arteritis (TA) is a large-vessel vasculitis primarily affecting the aorta and ... more Takayasu&#39;s arteritis (TA) is a large-vessel vasculitis primarily affecting the aorta and its branches. It ranks third among other types of childhood vasculitis, while it is the most common large vessel vasculitis observed in childhood. The diagnosis of TA should be made on the basis of clinical criteria and supported with laboratory findings, while confirming it with the imaging methods. Angioplasty, stent and bypass grafts may be necessary in the case of an irreversible arterial stenosis. Small-vessel involvement in TA and acute phase reactants should be taken into account for the diagnosis of an attack. In this report, treatment choices for four patients with the diagnosis of pediatric TA, their clinical and laboratory findings and their responses to treatment will be discussed.