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Amaç: İdrar yolu enfeksiyonu, çocukluk çağının en sık görülen enfeksiyonlarından biridir. Piyelonefritin uzun dönemde görülen sekelleri nedeniyle sistitten ayırımı önemlidir. Bu çalışmanın amacı, çocuklarda 99mTc-Dimerkaptosüksinik Asid ile sintigrafik görüntüleme yapılmaksızın piyelonefrit ayırımını yapmakta prokalsitonin ve C-Reaktif Protein gibi belirteçlerin tanısal değerini saptamak, 99mTc-Dimerkaptosüksinik Asid ile sintigrafik görüntüleme yapılacak hastalar konusunda yol gösterici olup olmayacağını belirlemektir. Materyal ve Metod: Bu çalışmaya, 3 ay ile 16 yaş arasında, ilk kez idrar yolu enfeksiyonu geçiren 50 çocuk dahil edilmiştir. Retrospektif gözleme dayalı bir çalışmadır. Piyelonefrit tanısı için serum C-Reaktif Protein ve prokalsitonin düzeylerinin ortanca değeri, duyarlılık, özgüllük ve cut-off değerleri saptandı. Bulgular: Hastaların 32’si (%64) piyelonefrit tanısı alırken, 18 (%36) hasta sistit tanısı aldı. Piyelonefrit tanısı için CReaktif Protein cut-off değeri %69 duyarlılık ve %61 özgüllük ile 34 mg/L iken, prokalsitonin için bu değer %69 duyarlılık ve %66 özgüllük ile 0,23 ng/mL saptandı. Her iki belirteçin birlikte kullanılması ise %63 duyarlılık ve %78 özgüllük ile piyelonefrit tanısını destekledi. Sonuç: Çocuklarda piyelonefrit tanısını koymakta serum prokalsitonin ve C-Reaktif Proteinin birlikte değerlendirilmesi, 99mTc-Dimerkaptosüksinik Asid ile sintigrafik görüntüleme yapılacak hastalarda yol gösterici olabilir. Böylece çocuklar 99mTc-Dimerkaptosüksinik Asid ile sintigrafik görüntülemenin dezavantajlarından uzak tutulabilir.

Çocuklarda akut serebral venöz sinüs trombozu nadir görülen durum olmasına karşın, sıklıkla enfeksiyonlara sekonder görülmektedir. Serebral venöz sinüs tromboz kliniği, etkilenen damarın lokalizasyonuna, damarın büyüklüğüne ve hastanın yaşına göre değişiklik gösterir. Bu yazıda 2 farklı presentasyonla karşımıza çıkan akut serebral venöz sinüs trombozlu, 8 ve 12 yaşında 2 çocuk olgu sunulmuştur.

Nazal polipler, nazal mukozanın kronik enflamasyonundan kaynaklanan oluşumlardır. Çocuklarda nadir görülmekle birlikte saptandığında eşlik edebilecek kistik fibrozis ve primer silier diskinezi açısından dikkat edilmelidir. Primer silier diskinezili olguların %50"sinde situs inversus gösterilebilir; böyle olgular Kartagener sendromu olarak adlandırılır. Kartagener sendromu, otozomal resesif geçişli, sinüzit, bronşektazi ve situs inversus ile karakterize, nadir görülen bir durumdur. Klinik olarak en sık nazal tıkanıklık ile Kulak Burun Boğaz uzmanlarına başvururlar. Pediatristler olarak biz de, çocukluk çağında nazal tıkanıklığın nadir bir sebebi olarak nazal polipleri akla getirmeli; poliplere eşlik eden tekrarlayan üst ve alt solunum yolu enfeksiyonlarının varlığında ise nadir olarak görülen Kartagener sendromu akılda tutulmalıdır.

Amaç: Çalışmamızda, bulgu veren ilk idrar yolu enfeksiyonu olan çocuklarda akut piyelonefrit ve böbrek parankim zedelenmesinde MAG3 sintigrafinin tanısal değeri ve DMSA sintigrafi ile karşılaştırılması amaçlanmıştır. Gereç ve Yöntem: İdrar yolu enfeksiyonu düşündüren şikayetlerle başvuran, daha önce idrar yolu enfeksiyonu tanısı almamış, 70 hasta çalışma kapsamına alındı. Çalışmaya alınan tüm hastaların tedavisi yapılmadan önce, kan ve idrar örnekleri alınarak, lökosit sayısı, sedimentasyon hızı, C-reaktif protein konsantrasyonu saptandı. Böbrek parankim zedelenmesinin saptanmasında DMSA sintigrafi 'altın standart' tanı yöntemi kabul edildi. Bulgu veren ilk idrar yolu enfeksiyonu tanısı alan çocuklarda, böbrekte parankim zedelenmesini saptamada MAG3 sintigrafinin duyarlılık, seçicilik, pozitif ve negatif kestirim değeri saptandı. Bulgular: Çalışmamızın sonucunda, piyelonefrit ve sistitli olgularımızın ayrımında ateş, lökosit, C-reaktif protein ve sedimentasyon yüksekliği istatistiksel olarak oldukça anlamlı bulundu; ancak, bu değerlerin, DMSA sintigrafide saptanan parankim zedelenmesinin şiddetini göstermede istatistiksel olarak anlamlılık taşımadığı saptandı. Böbrek parankim zedelenmesini göstermede, MAG3 sintigrafinin duyarlılığı % 32,5, seçiciliği ise % 98,1 saptandı. Sonuç: Çocuklarda, MAG3 sintigrafinin böbrek parankim zedelenmesini belirlemede DMSA sintigrafinin yerini alamayacağı kanısına varıldı.

Purpose: Thrombosis in children is a multifactorial disorder associated with both genetic and acquired factors. Idiopathic thrombosis is a rare condition. This study aimed to examine the etiology and risk factors of thrombosis in pediatric patients followed at Baskent University Adana Teaching and Research Center, a reference hospital in the southern part of Turkey.Material and Methods: This study included a sample of 49 pediatric patients who were retrospectively evaluated. The patient records were examined retrospectively in terms of age at diagnosis with thrombosis, gender, family history of thrombosis, localization of thrombosis, underlying primary disease and presence of catheter, as well as indicators of genetic risk factors. Results: The number of children presenting to our clinic during the study period was 10358. The incidence of thrombosis was calculated as 49 cases in 10358 children. The median age of the patients was 5.1 years (ranging from 0 to 17.2 years), and there were 32 (65.3%) males. In the analysis of the genetic factors causing susceptibility to primary thrombosis, only a portion of the patients were studied for genetic mutations. Among these, 5 patients showed factor V Leiden mutation, 13 patients MTHFR 1298C mutation, and 18 patients MTHFR 677CT mutation. Seven patients were found have a combination of multiple mutations. When we investigated any possible correlation between high lipoprotein (a) levels and the distribution of mutations, we found that 7 patients with mutations had normal levels of high lipoprotein (a), while other 10 patients with mutations were found to have elevated levels of lipoprotein (a).Conclusion: We conclude and strongly emphasize that lipoprotein (a) elevation is one of the important genetic risk factors, which might prove a major risk indicator, as valuable as other mutation screening tests performed by molecular genetics

Purpose: Thrombosis in children is a multifactorial disorder associated with both genetic and acquired factors. Idiopathic thrombosis is a rare condition. This study aimed to examine the etiology and risk factors of thrombosis in pediatric patients followed at Baskent University Adana Teaching and Research Center, a reference hospital in the southern part of Turkey. Material and Methods: This study included a sample of 49 pediatric patients who were retrospectively evaluated. The patient records were examined retrospectively in terms of age at diagnosis with thrombosis, gender, family history of thrombosis, localization of thrombosis, underlying primary disease and presence of catheter, as well as indicators of genetic risk factors. Results: The number of children presenting to our clinic during the study period was 10358. The incidence of thrombosis was calculated as 49 cases in 10358 children. The median age of the patients was 5.1 years (ranging from 0 to 17.2 years), and there were 32 (65.3%) males. In the analysis of the genetic factors causing susceptibility to primary thrombosis, only a portion of the patients were studied for genetic mutations. Among these, 5 patients showed factor V Leiden mutation, 13 patients MTHFR 1298C mutation, and 18 patients MTHFR 677CT mutation. Seven patients were found have a combination of multiple mutations. When we investigated any possible correlation between high lipoprotein (a) levels and the distribution of mutations, we found that 7 patients with mutations had normal levels of high lipoprotein (a), while other 10 patients with mutations were found to have elevated levels of lipoprotein (a). Conclusion: We conclude and strongly emphasize that lipoprotein (a) elevation is one of the important genetic risk factors, which might prove a major risk indicator, as valuable as other mutation screening tests performed by molecular genetics [Cukurova Med J 2016; 41(2.000): 285-290]

Purpose: Urinary tract infection is one of the most common infections in childhood. Because of the long term sequelae, differentiation of pyelonephritis from cystitis is important. The aim of this study is to determine the value of biomarkers such as C-reactive protein and procalcitonin and whether preferred to predict pyelonephritis in children without 99mTc-Dimercaptosuccinic Acid scan. Material and Methods: Fifty children aged 3 months to 16 years with a first urinary tract infection were included in this retrospective observational study. The medians, sensitivity, specificity, and cut-off values of serum C - reactive protein and procalcitonin to predict pyelonephritis were determined. Results: Thirty-two (64%) patients were diagnosed with pyelonephritis and 18 (36%) were diagnosed with cystitis. The cut-off value for C - reactive protein was 34 mg/L to predict pyelonephritis, with 69% sensitivity and 61% specificity. The cut-off value for procalcitonin was 0.23 ng/mL to predict pyehlonephritis, with 69% sensitivity and 66% specificity. In combination, these biomarkers were 63% sensitive and 78% specific to predict pyelonephritis. Conclusion: Using a combination of procalcitonin and C-Reactive Protein is preferred to predict pyelonephritis in children, instead of the 99mTc-Dimercaptosuccinic Acid scan. Because of its disadvantages, the 99mTc-Dimercaptosuccinic Acid scan should be avoided in children. [Cukurova Med J 2015; 40(3.000): 495-503]

Acute cerebral venous sinus thrombosis is a rare disorder in children, but is often secondary to infections. The clinical features of cerebral venous sinus thrombosis are based on the localization and size of the affected vessel and age of the patient. In this article we encountered two different presentations of acute cerebral venous sinus thrombosis in two children aged 8 and 12 years old. [Cukurova Med J 2014; 39(4.000): 922-926]

The nasal polyposis is a chronic inflammatory process of the nasal mucosa. Although it is rare in children, there may be also association with cystic fibrosis and primary ciliary dyskinesia. About 50% of primary ciliary dyskinesia patients develop situs inversus and it is known as Kartagener's syndrome. The Kartagener's sydrome is a rare autosomal recessive disorder characterized by sinusitis, bronchiectasis, situs inversus. Clinically, patients present to the otolaryngologist with nasal obstruction. We as pediatricians, should consider nasal polyposis as a rare cause of nasal obstruction in children. In the presence of recurrent upper and lower respiratory tract infections accompanying nasal polyposis, Kartagener's syndrome must be kept in mind as a rare reason. [Cukurova Med J 2014; 39(4.000): 942-945]

Jeune syndrome, or asphyxiating thoracic dystrophy, is an autosomal recessive skeletal dysplasia with multiorgan involvement. Skeletal dysplasia is characterized by small, narrow thorax, short ribs, short squared iliac wings and short limbed dwarfism. Multiorgan involvement including renal, hepatic, pancreatic, and retinal complications may also occur. About 60% to 70% of children die from respiratory failure as infants or young children. Patients with Jeune syndrome who survive often develop problems with their kidneys, another serious feature of Jeune syndrome. We, herein, report a case of Jeune syndrome with proteinuria, due to its rarity. A 7-year-old girl presented to our hospital with poor growth for a year and urinary incontinence for 3 months with urgency and frequency. Skeletal survey showed findings consistent with Jeune syndrome. Laboratory examination demonstrated normal urinalysis and renal functions. Two years after her initial diagnosis with Jeune syndrome, the patient developed proteinuria. She was referred to pediatric nephrology department when proteinuria was noted. Because of persistent proteinuria, renal biopsy was performed. The histological diagnosis was consistent with chronic sclerosing glomerulonephritis. We wish to point out the progression of renal involvement of this uncommon syndrome over several years. The patients with Jeune syndrome should be followed closely for renal involvement and subsequently renal failure in corporation with pediatric nephrologists. (Journal of Current Pediatrics 2013;11:88-91)