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Objective: Optimal care in the delivery room is important to decrease neonatal morbidity and mortality. We aimed to evaluate neonatal resuscitation practices in Turkish centers. Materials and Methods: A cross-sectional survey consisted of a 91-item questionnaire focused on delivery room practices in neonatal resuscitation and was sent to 50 Turkish centers. Hospitals with

Galen Veni anevrizmal malformasyonu nadir görülen konjenital bir malformasyondur. Tüm damarsal malformasyonların %1’ini oluşturmakla birlikte çocukluk çağında bu oran %30’a ulaşmaktadır. Yenidoğan döneminde genellikle yüksek debili kalp yetmezliği ile bulgu verir. Daha az oranda kitle etkisiyle ilerleyici nörolojik bozukluklara ve hidrosefaliye yol açabilir. Erken yenidoğan döneminde bulgu verdiğinde ölüm oranları yüksektir. Tedavi multidisipliner yaklaşım gerektirir. Günümüzdeki tedavi seçeneği endovasküler embolizasyon olup özellikle erken dönemde yapılırsa hayat kurtarıcı olabilir. Yazıda doğumdan hemen sonra kalp yetmezliği bulguları ile kliniğimizde tanı alan bir yenidoğan olgu sunuldu., The vein of Galen aneurysmal malformation is rare congenital vascular malformation. Abnormalities of the vein of Galen account for 1% of all vascular arteriovenous malformations and around 30% of all vascular malformations in the pediatric population. It usually manifests in the newborn period with high-output cardiac failure. It can also cause mass effect leading to progressive neurological impairment and can result in hydrocephalus. The death rate is higher in those who present early in the neonatal period. The management of this condition requires a multidisciplinary approach. The current treatment of choice is endovascular embolization and can be life-saving, especially when used in the early period. The management of this condition requires a multidisciplinary approach. We report a female newborn with a vein of Galen aneurysmal malformation who presented with cardiac failure immediately after birth

An infrequent reason of neonatal cholestasis is congenital pituitary hormone deficiency. Clinical manifestations of cholestasis and hypoglycaemia in the neonatal period. Gestational week 37, 3700 grams, girl baby born with cesarean sectioning. Hypoglicemia symptoms developed at postnatal first and cholestasis at postnatal third week. Multiple pituitary hormone deficiency was identified. Cholestasis symptoms recovered with growth hormone therapy. Congenital pituitary hormone deficiency should be kept in mind in the newborns with hypoglicemia and cholestasis., Konjenital hipofiz hormoneksikliği neonatal kolestazın sık olmayan nedenidir. Yenidoğan döneminde klinik bulguları kolestazve hipoglisemidir. Sezeryan ile, 37 haftalık, 3700 gr doğan kız bebekte postnatalbirinci hafta hipoglisemi, 3. haftada kolestaz bulguları gelişti. Çoklu hipofiz hormon eksikliği saptandı. Büyüme hormonu tedavisi ile kolestaz bulguları düzeldi. Konjenitalhipofiz hormon eksikliği, hipoglisemi vekolestazı olan yenidoğanlarda ayırıcı tanıda akılda tutulmalıdır.

Objective: While routine administration of rhesus (Rh) immunoglobulin has significantly reduced the incidence of Rh alloimmunization, maternal alloimmunization to other red cell antigens remains a contributor to perinatal morbidity and mortality. Although the Kell antigen is seen on the red cells of only 9% of the general population, attention to Kell antibodies continues to increase. Case Report: A case of fetal hydrops was sonographically detected at 30 weeks of gestation. Antenatal tests to evaluate the fetus’s condition clearly showed that the level of hemolytic disease was critical and the baby was delivered prematurely due to fetal distress. The combination of anemia, reticulocytopenia, hydrops fetalis, and a positive indirect Coombs test suggested Kell isoimmunization. The baby was successfully treated with exchange transfusion of Kellnegative packed red cells, and was discharged on postnatal d 30. Conclusion: The presented case of hydrops fetalis was due Kell alloimmunization that was detected during the postnatal period, and thus we plan to discuss the perinatal approach to Kell immunization.

Introduction: Skeletal dysplasia occurs due to abnormalities in growth and development of bone. There are over 450 distinct types of skeletal dysplasias.Case: Fetal sonography is used for prenatal diagnosis and molecular diagnosis is used for classification. First patient was a 39 week-old boy. He had cleft palate and intrauterine growth retardation. Hypotonia, club foot, cleft palate, micrognathia and laryngomalacia, narrow thorax, underdeveloped scapula and dislocated ribs were determined in the postnatal period and the patient had 11th ribs bilaterally. The patient was considered to be a case of campomelic dysplasia (CMD). The patient requiring supportive ventilation dead of respiratory failure on the 21th Day. Second patient was a 39 week-old girl. Skeletal radiographs showed that she had shortness of the long bones and narrow thorax in the prenatal period. The patient was considered to be a case of achondrogenesis but the result of analysis of the FGFR3 gene was reported to be negative. Shortening of the proximal parts of the limbs, punctate calcifications in the paravertebral soft tissues and in the epiphyseal cartilages of shoulder and knee, coronal cleft in the vertebral bodies and cataract were determined in the postnatal period. The patient was considered to be a case of rhizomelic chondrodysplasia punctata (RCDP). Growth retardation and mental retardation were determined during follow-up of the patient.Conclusion: The aim of this case presentations is to evaluate the prenatal diagnostic criteria of skeletal dysplasias, lethal and non-lethal skeletal dysplasia., Giriş: İskelet displazileri kemik büyümesi ve gelişimindeki anormallikler sonucu oluşur. 450 farklı tipi vardır. Prenatal tanıda fetal ultrason, sınıflandırmada moleküler tanı kullanılmaktadır.Olgu: Birinci olgu 39 haftalık erkek idi. Prenatal yarık damak ve intrauterin gelişme geriliği mevcuttu. Postnatal dönemde hipotoni, yumru ayak, yarık damak, mikrognati ve laringomalazi, dar toraks, az gelişmiş skapula, disloke kaburgalar ve bilateral 11. kaburga saptandı. Kampomelik displazi düşünüldü. Yardımcı ventilasyon ihtiyacı olan hasta 21. Gününde solunum yetmezliğinden kaybedildi. İkinci olgu 39 haftalık kız idi. Prenatal uzun kemiklerde kısalık ve dar toraks mevcuttu. Akondrogenezis düşünülen hastanın FGFR3 gen analizi negatif bulunmuştu. Postnatal dönemde, ekstremite proksimallerinde kısalık, paravertebral, omuz ve diz epifizinde punktat kalsifikasyonlar, vertebra korpuslarında koronal kleft ve katarakt saptandı. Rizomelik kondrodisplazi düşünüldü. İzleminde büyüme geriliği ve mental retardasyon saptandı.Sonuç: Bu olgu sunumlarının amacı iskelet displazisinin, letal ve non letal iskelet displazisinin prenatal tanı kriterlerini değerlendirmektir.

GİRİŞ: Prematürelerde postnatal CMV (sitomegalovirüs) enfeksiyonu en sık enfekte anne sütü alımı ile kazanılmaktadır. Diğer bulaş yolları perinatal dönemde genital sekresyonlar ve kan transfüzyonudur. Tanı postnatal 3. haftadan sonra idrar, kan ve sekresyonlarda PCR ya da kültür ile virüsün izole edilmesine dayanmaktadır. Term olgularda enfeksiyon çoğunlukla asemptomatiktir. Prematürelerde CMV ilişkili klinik bulgu ve sepsise neden olabilir. Prematüre olgularda CMV ilişkili sepsis az sayıda bildirilmiştir. OLGU SUNUMU: Olgularımızın her ikisi de 1000 gr altında prematüre idi. C/S ile doğmuşlardı. Premature formulası ile beslenmişler, lökosit filtresi kullanılarak çoklu eritrosit süspansiyonu transfüzyonu almışlardı. İzlemlerinin 2. ayında birinci olguda beslenme intoleransı ve splenomegali, ikinci olguda oksijen ihtiyacında artma ve invaziv ventilasyon ihtiyacı gelişti. Laboratuvar bulgularında her iki olguda karaciğer fonksiyon testlerinde yükselme, kolestaz, trombositopeni ve akut faz yüksekliği saptandı. Kan CMV IgM ve CMV DNA PCR pozitif bulundu. Gansiklovir başlandı. Birinci olgunun 2, ikinci olgunun altı hafta sonunda klinik ve laboratuvar bulguları düzeldi. İzlemlerinde nörolojik sekel ya da işitme kaybı gelişmedi. TARTIŞMA: Prematürelerde, 3. haftadan sonra gelişen sepsis nedeni olarak postnatal CMV enfeksiyonu akılda tutulmalıdır. INTRODUCTION: Postnatal CMV (cytomegalovirus) infection in the premature infants is most often transmitted with infected breast milk. The other routes of transmission are blood transfusion and genital secretions. Diagnosis is made by PCR or viral culture of urine, blood, or other body secretions after the first 3 weeks of life. It is mostly asymptomatic in term infants. CMV infection may cause sepsis and many clinical signs in premature infants. There are a few reports of premature infants with CMV sepsis in the literature. CASE REPORTS: Both of our cases were premature infants weighing less than 1000 g and born with ceaserean section. Since both of the mothers did not have breast milk, infants were fed with premature formula. They were transfused with erythrocyte suspension more than once. The first case has developed feeding intolerance and splenomegaly, the second case had increased oxygen demand and invasive mechanical ventilation support at the end of the second month of life. Laboratory findings showed cholestasis, thrombocytopenia, elevated transaminases and acute phase reactants. Blood CMV IgM and CMV DNA PCR were positive in both cases. Ganciclovir treatment was started. The first case had improvement in clinical and laboratory findings in 2 weeks, the second case had improvement in 6th week of the treatment. None of the patiens developed neurological sequelae or hearing loss. CONCLUSİON: We aimed to remind that postnatal CMV infection should be suspected in preterm infants who developed sepsis later than fist 3 weeks of life.

Yenidoğan bakım kalitesinin artması çok küçük prematüre bebeklerin yaşatılmasına olanak sağlamış olmakla birlikte bu bebeklerde “Yeni Bronkopulmoner Displazi” (BPD) olarak tanımlanan klinik, radyolojik ve histopatolojik bulguları daha hafif seyirli olan BPD nin yeni bir şekli ortaya çıkmıştır. Yeni BPD şekli, esas olarak akciğer gelişiminde duraksama ve pulmoner fonksiyonların bozulması ile ilişkilidir. Akciğer gelişiminin kanaliküler ve sakküler fazında doğan prematüre yenidoğanlarda çeşitli risk faktörlerinin etkisiyle alveol ve damar gelişimi sekteye uğramakta böylece alveolar ve kapiller hipoplazi ortaya çıkmaktadır. Surfaktan yetersizliği, yenidoğanın ventilasyonu, oksijen tedavisi, reaktif oksijen ürünleri, akciğer ödemi, fetal ve neonatal infeksiyonlar ve akciğer damar gelişiminde bozulma ile bazı antenatal etmenler akciğer hasarlanmasından sorumlu tutulmaktadır. Bu yazıda prematüre yenidoğanlardaki akciğer hasar mekanizmaları, yeni BPD tanımı, klasik BPD tablosundan farklılıkları, BPD den korunmaya yönelik güncel yaklaşımlar incelenmiştir., Improvements in antenatal and neonatal care have resulted in an increase in the survival of very preterm infants, whereas new form of Bronchopulmonary displasia (BPD) that has mild clinical course, radiological, and histopathological findings has occurred in these babies.. This new form of BPD is mainly related with a disruption of lung organogenesis and impaired pulmonary function. In premature infants born at the intersection of the canalicular and saccular phases of lung development, alveolar and capiller development are inhibited following exposure to the noxious effects of different risk factors. As a result, alveolar and capiller hypoplasia occur in these premature babies. These risk factors include surfactant deficiency, neonatal ventilation, oxygen therapy, reactive oxygen species, lung oedema, fetal and neonatal infections, disruption of vasculogenesis, and some antenatal factors that dysregulate subsequent lung development. In this article, mechanisms of lung injury in the preterm infants, the definition of new BPD, the difference between ‘‘old’’ and ‘’new’’ BPD, and the current approaches to prevent BPD have been reviewed

Bu çalışmada glukoz oksidaz (E.C.1.1.3.4) (GOD) enzimi poli(glisidil metakrilat) (PGMA) ve karboksilli asit uçlu poli(glisidil metakrilat) (PGMA-COOH) mikrokürelerine kovalent bağlanma ile immobilize edilmiştir. İmmobilize enzimler üzerine pH, sıcaklık ve depolama kararlılığı incelenmiştir. İmmobilize enzimler için optimum pH 7.0 olarak bulunmuştur. PGMA ve PGMA-COOH kürelerine immobilize enzimler için optimum sıcaklık sırasıyla 60 ve 50 °C olarak bulunmuştur. Maksimum reaksiyon hızı (Vmax) ve Michaelis-Menten sabiti (Km) immobilize enzimler için hesaplanmıştır. Michaelis-Menten sabiti (Km) PGMA kürelerine immobilize ve PGMA-COOH kürelerine immobilize enzimler için sırasıyla 2.44x10-2 mM ve 3.22x10-2 mM olarak bulunmuştur. Aynı şekilde maksimum reaksiyon hızı (Vmax) immobilize enzimler için sırasıyla 4,44x10-2 x M dak-1 ve 4,59x10-2 Mx dak-1 olarak bulunmuştur. Glukoz oksidazın tekrar kullanım sayısı ve depolama kararlılığı immobilizasyonu ile arttırılmıştır.Anahtar Kelimeler : Glukoz oksidaz, enzim immobilizasyonu, kovalent bağlanma In this study, glucose oxidase (E.C.1.1.3.4) (GOD) enzyme was covalently immobilized onto poly(glycıdyl methacrylate) (PGMA) and carboxylic acid ended poly(glycıdyl methacrylate) (PGMA-COOH) mıcrospheres. The effect of pH, temperature, storage stability on the immobilized enzyme was investigated. Optimum pHs for immobilized enzymes were determined as 7.0. Optimum temperatures for PGMA and PGMA-COOH spheres immobilized enzymes were found to be 60.0 and 50.0 oC, respectively. The maximum reaction rate (Vmax) and the Michaelis-Menten constant (Km) was calculated for the immobilized enzymes. Michaelis-Menten constant (Km) for immobilized onto PGMA and immobilized onto PGMA-COOH enzymes were found to be 2.44x10-2 mM, and 3.22x10-2 mM, respectively. In the same way, the maximum reaction rate (Vmax) for immobilized enzymes were found to be 4,44x10-2 mM x min-1, and 4,59x10-2 mM x min-1, respectively. Reuse number and storage stability of glucose oxidase were increased with immobilization.Key Words : Glucose oxidase, enzyme immobilization, covalent binding 63

Goldenhar syndrome also known as Oculo-auriculo-vertebral spectrum is a complex developmental disorder characterised mainly by anomalies of the ear and eye, hemi facial microsomia and vertebral anomalies. It is associated with anomalous development of the first branchial arch and second branchial arch. The aetiology of this rare sydrome is largely unknown and seems to be heterogeneous. The malformations affect usually one side of the body, however 4-8 percent of affected individuals have bilateral involvement. The management of this condition requires a multi-diciplinary approach.We report a pretem neonate of Goldenhar sydrome who presented bilateral cataract in addition to all classical signs of the condition., Goldenhar sendromu veya okuloaurikulovertebral spektrum, göz-kulak anomalileri, hemifasyal mikrozomi vevertebral anomaliler ile karakterize gelişimsel bir bozukluktur. Sendrom, birinci ve ikinci brakial yarıktan köken alan yapıların doğumsal defektlerinden oluşur. Sendromun nedeni tam olarak bilinmemektedir ancak heterojen olduğu düşünülmektedir. Olguların çoğunda tek taraflı tutulum görülürken %4-8 inde iki taraflı olabilmektedir. Bu sendromun yönetimi multidisipliner yakalaşım gerektirir. Burada sendromun tüm klasik bulgularına çift taraflı kataraktın eşlik ettiği prematüre doğan bir olgu sunulmuştur.

Beken, Serdar/0000-0002-8609-2684; BAS, AHMET YAGMUR/0000-0002-1329-2167; Bulbul, Ali/0000-0002-3510-3056; Karatekin, Guner/0000-0001-7112-0323, WOS: 000534242500056, PubMed: 31851725, Objective To investigate the early neonatal outcomes of very-low-birth-weight (VLBW) infants discharged home from neonatal intensive care units (NICUs) in Turkey. Material and methods A prospective cohort study was performed between April 1, 2016 and April 30, 2017. the study included VLBW infants admitted to level III NICUs. Perinatal and neonatal data of all infants born with a birth weight of.1500 g were collected for infants who survived. Results Data from 69 NICUs were obtained. the mean birth weight and gestational age were 1137 +/- 245 g and 29 +/- 2.4 weeks, respectively. During the study period, 78% of VLBW infants survived to discharge and 48% of survived infants had no major neonatal morbidity. VLBW infants who survived were evaluated in terms of major morbidities: bronchopulmonary dysplasia was detected in 23.7% of infants, necrotizing enterocolitis in 9.1%, blood culture proven late-onset sepsis (LOS) in 21.1%, blood culture negative LOS in 21.3%, severe intraventricular hemorrhage in 5.4% and severe retinopathy of prematurity in 11.1%. Hemodynamically significant patent ductus arteriosus was diagnosed in 24.8% of infants. Antenatal steroids were administered to 42.9% of mothers. Conclusion the present investigation is the first multicenter study to include epidemiological information on VLBW infants in Turkey. Morbidity rate in VLBW infants is a serious concern and higher than those in developed countries. Implementation of oxygen therapy with appropriate monitoring, better antenatal and neonatal care and control of sepsis may reduce the prevalence of neonatal morbidities. Therefore, monitoring standards of neonatal care and implementing quality improvement projects across the country are essential for improving neonatal outcomes in Turkish NICUs., Turkish Neonatal Society [2-2016]; Turkish Neonatal Society, This study was supported by the Turkish Neonatal Society, http://www.neonatology.org.tr, number 2-2016, received by AYB. Turkish Neonatal Society funded the study's online registry system. the funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Background To evaluate the prevalence, risk factors and treatment of retinopathy of prematurity (ROP) in Turkey and to establish screening criteria for this condition., Methods A prospective cohort study (TR-ROP) was performed between 1 April 2016 and 30 April 2017 in 69 neonatal intensive care units (NICUs). Infants with a birth weight (BW)=1500 g or gestational age (GA) 1500 g or GA> 32 weeks with an unstable clinical course were included in the study. Predictors for the development of ROP were determined by logistic regression analyses., Results The TR-ROP study included 6115 infants: 4964 (81%) with a GA 32 weeks. Overall, 27% had any stage of ROP and 6.7% had severe ROP. A lower BW, smaller GA, total days on oxygen, late-onset sepsis, frequency of red blood cell transfusions and relative weight gain were identified as independent risk factors for severe ROP in infants with a BW=1500 g. Of all infants, 414 needed treatment and 395 (95.4%) of the treated infants had a BW, Conclusions Screening of infants with a GA