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Öğe Arterial Wall Thickening and Stiffening in Children and Adolescents with Type 1 Diabetes(Elsevier Ireland Ltd, 2006) Atabek, Mehmet Emre; Kurtoğlu, Selim; Pirgon, Özgür; Baykara, MuratObjective: We evaluated structural and functional characteristics of the common carotid artery (CCA), a marker of early carotid atherosclerosis, and investigated their relation to metabolic and anthropometric parameters in children and adolescents with type 1 diabetes. Materials and methods: Non-invasive ultrasonographic measurements were made in 45 type I diabetic patients and 33 controls. Age, sex, and body mass index were matched between patients and controls. We investigated intima-media thickness (IMT), compliance, distensibility, diastolic wall stress (DWS) and incremental elastic modulus (IEM) of the CCA. Metabolic and anthropometric parameters such as serum lipids, plasma glycated haemoglobin, body mass index, waist-hip ratio and blood pressure were assessed. Results: The diabetic patients had significantly higher CCA-IMT than the controls (0.48 +/- 0.06 mm versus 0.33 +/- 0.07 mm; p < 0.001). The diabetic children had significantly higher values than the controls for DWS (1.18 +/- 0.29 mmHg x 102 versus 0.81 +/- 0.25 mmHg x 10(2); p < 0.001) and for IEM (1.26 +/- 0.57 mmHg x 10(3) versus 0.77 +/- 0.28 mmHg x 10(3); P < 0.001). The difference was not significant between patients and controls for arterial compliance and for distensibility (p > 0.05). In a multivariate regression model for all subjects, diabetic state was the best predictor of IMT (p < 0.001), DWS (p < 0.001) and IEM (p = 0.001). Conclusion: Our results suggested that children and adolescent patients with type I diabetes are associated with early impairment of CCA structure and function and that diabetic state may be the main risk factor for CCA wall stiffening and thickening, which are of considerable concern as possible early events in the genesis of atheroma.Öğe Assessment of Abnormal Glucose Homeostasis and İnsulin Resistance in Turkish Obese Children and Adolescents(Karger, 2007) Atabek, Mehmet Emre; Pirgon, Özgür; Kurtoğlu, SelimBackground: The worldwide increase in the prevalence of childhood obesity is reaching epidemic proportions and is associated with a dramatic rise in cases of type 2 diabetes. We determined the prevalence of impaired glucose regulation and insulin resistance in obese children and adolescents. Methods: A total of 196 obese children [SD score (SDS) of body mass index (BMI): 2.17 0.03], aged 7–18 years, including 86 male subjects, underwent an oral glucose tolerance test (1.75 g glucose/kg body weight). We used the modified WHO criteria adapted for children for abnormal glucose homeostasis. Homeostasis model assessment was used to estimate insulin resistance in all subjects. The insulin sensitivity index was also determined in subjects. Results: Of the total of 196 obese children, 15 (6.6%) had an abnormal fasting glucose level. Of the 196 obese children, 35 (18%) had impaired glucose tolerance (IGT) and 83 (43%) had insulin resistance. Of the 196 obese children were six (3%) diagnosed with type 2 diabetes. Insulin resistance indices were correlated well with the degree of abnormal glucose tolerance. Conclusions: IGT, insulin resistance and type 2 diabetes are far more common in obese Turkish children than previously thought. Early treatment in obese children and adolescents with IGT constitutes a strategy of reversing the progression to b-cell failure and preventing type 2 diabetes.Öğe The association of common atrium and smith-lemli-opitz syndrome in an infant(2008) Sert, Ahmet; Pirgon, Özgür; Atabek, Mehmet Emre; Doğan, MustafaSmith-Lemli-Opitz sendromu düşük plazma kolesterol düzeylerinin eşlik ettiği çoklu doğumsal anomali/mental gerilik ile kendini gösteren nadir bir sendromdur. Klinik spektrum holoprozonsefali ya da letal malformasyonlarla prenatal ölümden normal zeka ya da hafif zihinsel bozukluk ve hafif fiziksel anormalliği olan hastalara kadar değişmektedir. Doğumsal kalp hastalığı Smith-Lemli-Opitz sendromlu hastaların yarısında bulunmaktadır. Bilgilerimize göre, Smith-Lemli-Opitz sendromu ve ortak atrium birlikteliği tıp literatüründe daha önceden açıklanmamıştır. Böyle birlikteliğin olduğu 4 aylık infant olguyu sunuyoruz.Öğe A Case of Perlman Syndrome Presenting With Hemorrhagic Hemangioma(Lippincott Williams & Wilkins, 2006) Pirgon, Özgür; Atabek, Mehmet Emre; Akın, Fatih; Sert, AhmetThe presence of enlarged, echogenic kidneys in a newborn with generalized macrosomia points toward diagnosis of an overgrowth syndrome. These include Beckwith-Wiedemann, Perlman and Simpson-Golabi-Behmel syndromes. Perlman syndrome is an autosomal recessively inherited overgrowth syndrome characterized by fetal gigantism, visceromegaly, unusual face, bilateral renal hamartomas with nephroblastomatosis, and Wilms tumor. We report a male infant who exhibits typical features of Perlman syndrome with an unusual presentation. In this report, it is emphasized that hemangioma in neonatal period may be an unusual finding in Perlman syndrome.Öğe Effects of Phototherapy on the Growth Plate in Newborn Rats(Lippincott Williams & Wilkins, 2006) Atabek, Mehmet Emre; Pirgon, Özgür; Kurtoğlu, Selim; Tavlı, Lema; Esen, H. Hasan; Köylü, Öznur; Erkul, İbrahimThe aim of the present study was to evaluate the effect of phototherapy and oxidative stress on the growth plate of newborn rats. Forty newborn Sprague-Dawley rats were randomized into a phototherapy group and a control group. Twenty of the rats received phototherapy for 7 days. All zones of the growth plate were assessed with quantitative histomorphometric analysis. Individual zonal lengths were measured for the reserve zone (RZ), the proliferative zone (PZ), the hypertropbic zone (HZ), ossifying cartilage (OC), and total zone (TZ) of the growth plate. Levels of plasma malondialdehyde (MDA), an index of oxidative stress, were also evaluated. Compared with zonal lengths on day 7 after phototherapy between the two groups, the phototherapy group had significantly lower values than those of controls for RZ (5.13 +/- 0.36 vs. 6.4 +/- 0.85 mm X 10(-2); P < 0.001), PZ (20.6 +/- 3.0 vs. 29.25 +/- 1.68 mm X 10(-2); p < 0.001), HZ (15.4 +/- 1.44 vs. 20.87 +/- 1.12 mm X 10(-1); P < 0.001), OC (47.08 +/- 4.25 vs. 62.06 +/- 3.7 mm X 10(-2); p < 0.001), and TZ (88.15 +/- 6.56 vs. 118.48 +/- 4.50 mm X 10(-2); p < 0.001). Plasma MDA levels were correlated with the size of the PZ in the phototherapy group (r = -0.53, P = 0.01). In a multivariate regression model for all rats, being in the phototherapy group was the best predictor of the size of the TZ (beta = -0.94, P < 0.001), with the total variance explained being 88%. These results suggest that in newborn rats, receiving phototherapy is associated with early impairment of growth plate structure, and oxidative stress may be the main risk factor for growth plate injury.Öğe Genital Prolapse in a Newborn Following Resection of Sacrococcygeal Teratoma(2009) Pirgon, Özgür; Atabek, Mehmet Emre; Suleymanoğlu, SelamiNeonatal genital prolapse usually occurs during the first few days of life and presents as a tumor mass protruding from the vulva. We present a 15-day-old female neonate who developed neonatal genital prolapse following the operation of sacral teratoma. We suggest that sacral teratoma operation may be a significant predisposing factor for genital prolapse in neonates and the neonatal vaginal prolapse may be one of the differential diagnoses of interlabial mass during the late neonatal period.Öğe Herediter multiple eksostoz(2005) Pirgon, Özgür; Atabek, M. Emre; Sert, AhmetHerediter multiple eksostoz, çok sayıda uzun kemiğin metafizinden dışarı büyüyen kıkırdakla kaplı benign kemik tümörü ile karakterize genetik bir hastalıktır. Prevalansının 1/50.000 olduğu tahmin edilmektedir. Eksostozlar iskelet büyümesinde duraklama, kemiklerde deformite, eklem hareketlerinde kısıtlanma, kısa boy, erken osteoartroz ve periferik sinir basısı gibi semptomlara neden olabilir. Malign dejenerasyon riski düşük (yaklaşık %1) olmasına rağmen kondrosarkoma dönüşüm riski yaşla artar. Herediter multiple eksostozun nadir görülen bir kemik displazisi olması nedeniyle üç vaka sunulmaktadır. Vakalarımızda olduğu gibi herediter multiple eksostozlu birey ve aileler için genetik konsültasyonun gerektiğini vurguladık.Öğe The Importance of Karyotyping in Girls With Inguinal Hernia (Case Report)(2002) Atabek, Emre; Pirgon, Özgür; Erkul, İbrahimKomplet androgen insensitivite sendromu, tek gen bozukluğuyla X'e bağlı geçiş gösteren nadir bir hastalıktır. Fenotipik olarak normal kız görünümüyle birlikte erken yetişkin çağda primer amenore ile farkedilmektedir fakat süt çocukluğu döneminde anlamlı olarak inguinal herni bulunmakadır. Bu çalışmada, tanı konulmadan önce inguinal herni nedeniyle öpere edilen 2.5 yaşında komplet androgen insensitivite sendromu olan bir kız hasta sunulmaktadır. Cerrahi sırasında fıtık kesesi içinde bulunan gonadlar över sanılarak abdomen içine yerleştirilmişti. Hastanemize geldiğinde komplet androgen insensitivite sendromu tanısı konuldu. Bu vaka; inguinal herni ile gelen kız hastaların dikkatli değerlendirilmesi sonucunda .komplet androjen insensitivite sendromu tanısının erken konulabileceğini vurgulamak amacıyla sunulmuştur.Öğe Klediokranyal displazili olgularımızda endokrinolojik bulgular(2007) Sert, Ahmet; Pirgon, Özgür; Atabek, Mehmet EmreAmaç: Klediokranyal displazi iskelet ossifikasyonu ve diş gelişimini etkileyen, otozomal dominant kalıtılan bir hastalıktır. Klediokranyal displazinin başlıca semptomları açık fontanel, klavikulaların hipoplazisi veya yokluğu, dar pelvis, geniş simfizis ve kısa boydur. Olgu sunumu: Bu yazıda klediokranyal displazili olgularımızdaki endokrinolojik bulguları sunduk. Sonuç: Klediokranial displazili olgularda endokrinolojik bozukluklar diğer iskelet displazilerine göre muhtemelen daha sık görülmektedir.Öğe Metabolic syndrome features presenting in early childhood in Alström syndrome: A case report(2009) Pirgon, Özgür; Atabek. Mehmet Emre; Tanju, İlhan AsyaAlström syndrome is a rare autosomal recessive disorder characterized by retinal degeneration, sensorineural hearing loss, early-onset obesity, and non-insulin-dependent diabetes mellitus. Affected individuals have normal birth weight, but growth deceleration starts at about 8-10 years of age. In patients with the disorder linked to chromosome 2, the increase in body mass index is very high in childhood and continues high thereafter. In this paper, we report a patient who had the proposed diagnostic criteria for Alström syndrome associated with metabolic syndrome starting at age 7, a relatively early age. © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing. All rights reserved.Öğe Otopalatodigital sendrom tip I: Bir olgu sunumu(2006) Sert, Ahmert; Atabek, M. Emre; Pirgon, Özgür; Silahlı, MusaAmaç: Otopalatodigital sendrom, iskelet displazisi, işitme kaybı ve damak anomalileri, atipik yüz görünümü, hafif derecede mental retardasyon ile seyreden X’e bağlı nadir bir hastalıktır. 1976’da sendromun daha letal bir varyantı olan tip II Fitch ve ark tarafından tanımlanmıştır. Sendromdan sorumlu olan filamin A genindeki mutasyonlardır. Son zamanlarda genetik araştırmalar sorumlu mutant genin X kromozomunun distal bölgesinde olduğunu göstermiştir. Klinik ve prognoz bu genin ekspresyonuna göre değişmektedir. Otopalatodigital sendrom teşhis edilen bir olgunun, nadir görülmesi nedeniyle tartışılması amaçlandı. Olgu sunumu: Bu makalede otopalatodigital sendromun klinik özelliklerini taşıyan bir kız vaka takdim edilmektedir. Sonuç: Literatürde kız vakaların tanımlanması gen ekspresyonu ve X genindeki inaktivasyonla açıklanmaktadır. İşitme kaybı ve dismorfik özellikleri olan vakaların otopalatodigital sendrom açısından değerlendirilmesi önemlidir.Öğe Papillon-Lefevre Sendromu(2007) Pirgon, Özgür; Atabek, Mehmet Emre; Sert, AhmetPapillon-Lefevre Sendromu, tipik olarak 1-5 yaş civarında ortaya çıkan son derece nadir genetik bir bozukluktur. Papillon-Lefevre Sendromu, palmoplantar hiperkeratoz ile başlayan ve hızla gelişen periodontopatiye bağlı olarak süt dişleri ve kalıcı dişlerin erken kaybıyla karakterizedir. Sütdişleri serbest hale gelir ve bunlar 5 yaş civarında kaybedilir. Genel populasyonda bozukluğun görülme sıklığı, yaklaşık milyonda 1-4 civarındadır. Nadir görülmesi nedeniyle Papillon-Lefevre Sendrom’lu bir olgu ilgili literatür bilgileri ile birlikte sunuyoruz.Öğe Plasma Homocysteine Concentrations in Adolescents With Subclinical Hypothyroidism(Freund Publishing House Ltd, 2003) Atabek, Mehmet Emre; Pirgon, Özgür; Erkul, İbrahimObjective: Hyperhomocysteinemia is a risk factor for premature atherosclerotic vascular disease and venous thrombosis. The aim of the present study was to assess plasma total homocysteine (tHCys) concentrations in adolescent patients with subclinical hypothyroidism. Patients and Methods: Nineteen patients with subclinical hypothyroidism and 19 healthy children were studied. Fasting plasma concentrations of tHCys and its putative determinants (plasma concentrations of free thyroxine [FT4], folate, vitamin B-12 and renal function) were measured. Results: tHCys concentrations showed no statistical difference between patients and controls (p >0.05). Moreover, the difference in tHCys and total cholesterol concentrations was not significant between patients with mild TSH elevations (less than or equal to10 mIU/l) and patients with prominent TSH elevations (>10 mIU/l). No correlation was found between tHCys concentrations and its putative determinants. Conclusions: We concluded that plasma tHCys concentrations were not increased in adolescent patients with subclinical hypothyroidism.Öğe The Role of Umbilical Cord Blood Insulin Like Growth Factor-1 Levels in the Assessment of Bone Maturation in Term Neonates(2009) Kurtoğlu, Selim; Atabek, Mehmet Emre; Pirgon, Özgür; Hatipoğlu, NihalPurpose: In this study, we aimed to investigate the relation of anthropometric data and knee epiphyseal area with cord blood Insulin Like Growth Factor-1 (IGF-1) levels at birth in term neonates. Material and Method: Twenty-six term neonates were recruited. Anthropometric measures and the knee epiphyseal area were recorded and umbilical cord blood samples were collected at birth. The knee epiphyseal area was calculated by the addition of [width x height x 3.1415/4] of the distal femoral epiphysis and proximal tibia epiphysis. Results: Mean concentrations of IGF-1 levels of cord blood and the knee epiphyseal area were 45.2 ± 36.8 ng/ml and 29.0 ± 18.4 mm2 in term neonates, respectively. IGF-1 levels correlated significantly with knee epiphyseal area (r: 0.45, p: 0.018). In a multiple regression model, IGF-1 emerged as independent correlates for mean knee epiphyseal area in term neonates with the total variance being 18 %. Although IGF-1 level had significantly positive correlations with body surface area, birth weight, midarm circumference did not correlate with gestational age, birth length, head circumference, ponderal index and skinfold thickness. Conclusion: Cord blood IGF-1 level is useful for assessing the bone maturation in neonates, because a changes in IGF-1 correlates with the change in epiphyseal area.Öğe Selçuk Üniversitesi 2000 Yılı Perinatal Mortalitesi(2001) Atabek, M. Emre; Koç, Hasan; Pirgon, Özgür; Erkul, İbrahimAMAÇ: Selçuk Üniversitesi 2000 yılı perinatal mortalite hızını araştırmak. GEREÇ VE YÖNTEM : Bu çalışmada, 1 Ocak 2000 ile 31 Aralık 2000 tarihleri arasında hastanemizdeki Perinatal mortalite hızı (PNMH), ölü doğum hızı ve erken neonatal mortalite hızı prospektif olarak araştırıldı. En sık gözlenen ölüm nedenleri saptandı ve sonuçlarımız daha önceki çalışmaların sonuçları ile karşılaştırıldı. BULGULAR: Çalışmamızda toplam doğum sayısı 2406 olarak belirlendi. PNMH %0 52.7, ölü doğum hızı %0 31.58, erken neonatal mortalite hızı %0 21.19 olarak hesaplandı. Genişletilmiş Wigglesworth Sınıflamasına göre ölüm nedenlerinden ilk üç sırayı % 41.5, % 23.3 ve % 13.3 gibi değerlerle sırasıyla ölü doğumlar, prematürelik ve sorunları ve letal konjenital malformasyonlara bağlı ölümler aldı. Perinatal mortalite hızı 1997 ve 1999 'da yapılan çalışmalardan daha düşük bulundu. SONUÇ: Bu bulgularla yüksek riskli gebelerin büyük oranda kabul edildiği Selçuk Üniversitesi Tıp Fakültesi hastanesinde; perinatal bakımın iyileştirilmesi, prematüreliğin önlenmesi, doğumların uygun şekilde yapılması, optimal solunum desteği sağlanması, enfeksiyonlardan koruma, ölümlerin otopsi ile değerlendirilmesi, prenatal genetik çalışmaların artırılması, yenidoğan servisinin teknik donanım ve personel yönünden iyileştirilmesi, prenatal ve neonatal mortalite hızının düşürülmesi hedefler olarak görülmektedir.Öğe Stevens-Johnson Syndrome-Like Exanthema Secondary to Methotrexate(2002) Atabek, Mehmet Emre; Çalışkan, Ümran; Pirgon, Özgür; Tol, Hüseyin; Erkul, İbrahimMethotrexate is an antineoplastic drug used commonly in leukemia treatment. Because of becoming resistant to standard doses after 1970s, it is used intermediate or high doses. The complications of high doses are mucositis, vomiting, dermatitis exfoliativa, B-cell dysfunction, hepatotoxicity, nephrotoxicity and bone marrow depression. There were only two studies in literature about Stevens-Johnson syndrome occuring in two patients with acute lymphocytic leukemia and non-Hodgkin lymphoma after receiving high doses methotrexate and leukoverin. We have reported a two-year-old boy patient suffering from acute lymphocytic leukemia (ALL) developed a severe skin reaction two days after administration of high dose methotrexate. The skin lesions simulated Stevens-Johnson syndrome with ulceration of the oral mucosa and erythema multiforme-like target lesions.Öğe Weismann-Netter-Stuhl Syndrome: A Family Report(2009) Alp, Hayrullah; Atabek, Mehmet Emre; Pirgon, ÖzgürWeismann-Netter-Stuhl (WNS) syndrome is a rare skeletal anomaly that affects the diaphyseal part of both the tibiae and fibulae with posterior cortical thickening and anteroposterior bowing. This anomaly is usually bilateral and symmetrical. The patients are generally of short stature. In some cases, a family history suggesting genetic transmission of a mutation with an unknown locus has been reported. In this paper we present an infant with WNS syndrome with bilateral involvement of the femur. Similar clinical findings were defined in three other family members.
