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Amaç: Erken membran rüptürü (EMR) preterm doğuma, neonatal morbiditelere ve mortaliteye sebep olabilir. Bu çalışmadaki amacımız EMR’nin

In this article, we aim to represent a neonatal fatal case of CHARGE syndrome and make a mini-review of the literature to underline the wide range of congenital abnormalities and clinical symptoms of individuals with CHARGE syndrome face early in life. This patient, with his characteristic dysmorphic features, congenital heart defects, and severe feeding intolerance, is a good example of how complex the syndrome could be. CHARGE syndrome might affect numerous organ systems and result in severe early lethal cases for some, while some of the cases show a very mild phenotypic spectrum and may go unrecognized.

Amaç: Bronkopulmoner displazi (BPD), çok düşük doğum ağırlıklı her üç bebekten birini etkileyen uzun dönem ciddi olumsuz sonuçları olan bir prematüre morbiditesidir. Bu çalışmada orta-ağır BPD için antenatal ve postnatal risk faktörlerini araştırmayı amaçladık. Gereç ve Yöntemler: Tek merkezli retrospektif kohort çalışmada Ocak 2014-Aralık 2018 tarihleri arasında doğan, Objective: Bronchopulmonary dysplasia (BPD) is the chronic lung disease of prematurity that affects a substantial proportion of extremely preterm infants. We aimed to find out the antenatal and postnatal risk factors for BPD in a large-scale cohort of preterm infants. Material and Methods: Records of preterm infants born

Amaç: Bu çalışmanın amacı çok düşük doğum ağırlıklı (ÇDDA) bebeklerde cinsiyetin prematüre morbiditeleri ve mortalite üzerine etkisini değerlendirmektir. Yöntem: Bu retrospektif çalışmaya, 2013-2017 yılları arasında Zekai Tahir Burak Kadın Sağlığı Eğitim ve Araştırma Hastanesi’nde doğarak yenidoğan yoğun bakım ünitesinde izleme alınan, gebelik haftası 30 haftadan küçük tüm prematüre bebekler alındı. Her iki cinsiyetteki bebekler; demografik özellikler, neonatal morbiditeler ve mortalite açısından karşılaştırıldı. Ardından bebekler gebelik haftalarına göre (

Fibular hemimeli (FH), fibulanın kısmi veya tam yokluğu ile karakterize konjenital alt ekstremite anomalisidir. Bildirilen vakaların çoğu sporadiktir. Etiyolojisi ve anomalinin gelişimsel biyolojisi tam olarak anlaşılamamıştır. Tam yokluğu kısmi yokluğa göre, unilateral tutulum bilateral tutuluma göre daha sık görülmekte , sağ taraf sola göre daha sık etkilenmektedir. Erkeklerde görülme sıklığı kızlara göre iki kat daha fazladır. Kardiyak anomaliler, ürogenital sistem anomalileri, trombositopeni, sağırlık, torakoabdominal şizis sağırlık gibi durumlarla birlikte olabildiği gibi en çok iskelet sistemi anomalilerine eşlik ettiği görülmüştür.

Objective: To determine the association between feeding intolerance and late onset sepsis (LOS) in very low birth weight (VLBW) infants.Material and Methods: In this retrospective study inborn infants with a gestational age less than 32 weeks or birth weight less then 1500 grams were enrolled. Demographic and neonatal characteristics of infants who had culture proven LOS were compared with the control infants, matched for birth weight and gestational age. Results: A total of 408 infants (LOS n=136, non LOS n=272) were analyzed. The mean (SD) gestational age and birth weight of the whole cohort were 27.8 weeks (±1.6) and 1016.92 grams (±200.39) Exclusively breast feeding was lower and feeding intolerance was more frequent in LOS group (p=0.028, p, Amaç: Çok düşük doğum ağırlıklı (ÇDDA) bebeklerdeki beslenme intoleransının geç neonatal sepsis (GNS) ile ilişkisinin değerlendirilmesi amaçlanmıştır.Gereç ve Yöntemler: Bu retrospektif çalışmaya doğum ağırlığı ≤1500 gram veya gestasyon haftası ≤32 hafta olan infantlar dahil edildi. Kültür ile kanıtlanmış GNS tanısıyla izlenen bebeklerin demografik ve klinik özellikleri, doğum ağırlığı ve gestasyon haftası benzer olan kontrol grubu ile karşılaştırıldı. Bulgular: Toplam 408 infant (GNS olan n=136, GNS olmayan=272) değerlendirildi. Çalışmaya dahil edilen hastaların ortalama (SD) gestasyon haftası ve doğum ağırlığı 27.8 hafta (±1.6) ve 1016.92 gram (±200.39) idi. GNS olan bebeklerde sadece anne sütü ile beslenme oranı daha düşük, beslenme intoleransı daha fazlaydı (sırasıyla p=0.028, p

To determine the effectiveness of ventilator-delivered nasal intermittent positive pressure ventilation and nasal biphasic positive airway pressure (n-BiPAP) after first extubation attempt in infants ≤1250-g birthweight.This randomized controlled study included mechanically ventilated preterm infants of ≤1250-g birthweight who were randomly assigned to ventilator-delivered NIPPV or n-BiPAP at first extubation within 2 weeks of age. The primary outcome (extubation failure within 96 h following extubation) and secondary outcomes were compared.Extubation failure occurred in 22 of 74 infants in n-BiPAP group and 34 of 75 infants in NIPPV group (OR 0.51, 95% CI: 0.26-1.002;Sustained extubation in infants ≤1250-g birthweight and2 weeks age did not differ between ventilator-delivered NIPPV and n-BiPAP.Clinical Trials.gov under identifier NCT02842190.

Background When the newborn brain is exposed to hypoxia, as in hypoxic ischemic encephalopathy (HIE), it causes an inflammatory response. A wide variety of inflammatory markers are therefore used in the diagnosis of HIE. Objective We aimed to determine the diagnostic role of systemic inflammatory indices in infants with moderate-to-severe HIE. We have also investigated the effect of hypothermia treatment over those indices. Study Design A retrospective cohort study of infants suffering from moderate-to-severe HIE was conducted in a tertiary-level neonatal intensive care unit between September 2019 and March 2021. Systemic inflammatory indices including systemic immune-inflammation index (SII), pan-immune-inflammation value (PIV), systemic inflammation response index (SIRI), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and monocyte-to-lymphocyte ratio (MLR) were calculated for infants with HIE and controls at baseline, and after therapeutic hypothermia in those with HIE. Results A total of 103 infants (53 in the HIE group and 50 in the control group) were included in the study. Median gestational ages (GA) were 39 (37–40) and 38 (37–39) weeks, and median birth weights (BW) were 3,165 (2,890–3,440) and 3,045 (2,850–3,460) g in the HIE and control groups, respectively. GA, BW, mode of delivery, and gender of infants were similar between the groups. Infants in the HIE group had significantly higher NLR (p = 0.001), SII (p = 0.001), PIV (p = 0.001), and SIRI (p = 0.004) values when compared with the control group. Those indices decreased significantly after hypothermia treatment in the HIE group. Areas under curve for NLR, PLR, MLR, SII, SIRI, and PIV to predict HIE were found to be 0.808, 0.597, 0.653, 0.763, 0.686, and 0.663, respectively. Cutoff values having a good ability to predict HIE for SII and NLR were 410 and 1.12. Elevated NLR level above 1.12 was found to be an independent predictor for HIE, as revealed by multivariate analyses. No associations were found between systemic inflammatory indices and amplitude-integrated electroencephalography (aEEG) patterns, presence of seizures, and death. Conclusion Systemic inflammatory indices may represent reliable and readily available predictors of HIE risk. NLR seems to be an independent factor in diagnosing moderate-to-severe HIE. Key Points

This study aimed to compare the effects of adjustable fortification (AF), targeted fortification (TF), and standard fortification (SF) methods on the early growth of very low birth weight infants.Sixty infants32 weeks of gestational age and weighing1,500 g were selected. These infants were exclusively fed with breast milk and were randomized into three fortification groups: SF, AF, and TF. SF consists of adding a fixed amount of fortifier to maternal milk. Blood urea nitrogen levels are used to adjust fortifier in AF. In TF, breast milk is analyzed and fortified accordingly based on the macronutrient content to achieve the targeted intake.A total of 76 preterm infants were eligible for the study. Sixty infants were randomized into three groups. At the end of the fourth week of fortification period, median daily weight gain of the infants was similar in the AF [23.5 g/(kg·d)] and TF groups [25.5 g/(kg·d)], and significantly higher in both groups than in SF group [12 g/(kg·d)] (AF versus SF group, p 0.001 and TF versus SF group, p 0.001). Weight, head circumference, and length were compared across the three groups regarding percentile. Significant improvement in the percentile groups of weight and head circumference was observed in the AF and TF groups. In the SF group, a significant decline in the percentile groups of weight, head circumference, and length was detected. Daily protein and energy intakes in AF and TF groups were significantly higher than those in SF group (SF versus AF, p 0.001 and SF versus TF, p 0.001).Fortifying breast milk with AF and TF methods was found to improve body weight, length, and head circumference percentiles of preterm infants, whereas SF method was found unsatisfactory. To provide optimum growth for preterm babies, breast milk that has been fortified by using AF and TF strategies should be recommended in neonatal intensive care units.

Objective To compare the effectiveness of lung lavage with surfactant vs. bolus surfactant treatment in meconium aspiration syndrome (MAS). Patients and methods This randomized controlled trial included newborns ventilated with MAS. In lavage group (n = 17) 30 ml/kg of diluted porcine surfactant was instilled into the lung. In bolus group (n = 16) porcine surfactant (100 mg/kg) was administered as bolus. Respiratory outcomes and mortality were compared between groups. Results Duration of respiratory support was found to be similar between lavage and bolus groups (3 vs. 3.5 days, p = 0.36). Death or requirement for extracorporeal membrane oxygenation (ECMO) was 12% vs. 6%; respectively (RR: 2, 95% CI 0.16–24.48; p = 1.0). Duration of oxygen therapy, high-frequency ventilation or inhaled nitric oxide requirement did not differ among the groups. Conclusion Lung lavage did not show any advantage over bolus therapy on duration of respiratory support. The incidence of pneumothorax and surfactant re-administration decreased nonsignificantly in lavage group. Clinical Trial Registration We registered the trial to ClinicalTrials.gov (http://clinicaltrials.gov) under identifier NCT02041546. Registry name Lung Lavage With Dilute Poractant Alfa for Meconium Aspiration Syndrome.

Giriş: Son yıllarda dünyada ve ülkemizde sezaryen ile doğum oranları giderek artmaktadır. Bu durum, yenidoğan ve sonraki çocukluk döneminde olumsuz bazı etkilere sebep olmaktadır. Bu çalışmada, doğum şeklinin, çok küçük prematüre bebeklerde neonatal sonuçlar üzerine olan etkisinin incelenmesi amaçlanmıştır. Metot: 2015-2019 yılları arasında III. düzey yenidoğan yoğun bakım ünitesinde izlenen, gebelik haftası, Introduction: In recent years, cesarean delivery rates have been increasing in the world and in our country. This situation causes some negative effects on infant and child health in the early neonatal period and later childhood. In this study, we aimed to examine the effect of delivery method on neonatal outcomes in very premature infants.Methods: Premature infants with a gestational age of

Background: Preterm infants are at risk of extrauterine growth restriction (EUGR) and associated complications in the long term. Growth curves are important in assessing postnatal growth in these infants. The aim of this study was to determine the prevalence of EUGR in preterm infants and the factors associated with EUGR using two different growth curves. Methods: We retrospectively evaluated 596 preterm infants with birth weight ≤1500 g. Small for gestational age (SGA) was defined as birth weight 16 days to full enteral feeding (OR: 1.8, 95% CI: 1.22–2.68). Similarly, independent risk factors for EUGR according to the IG-21 curve were SGA (OR: 16.3, 95% CI: 7.23–36.9), RDS (OR: 1.81, 95% CI: 1.16–2.83), LNS (OR: 2.29, 95% CI: 1.43–3.68), and >16 days to full enteral feeding (OR: 2.11, 95% CI: 1.38–3.23). Conclusion: The growth curves used for diagnosis may lead to differences in EUGR rates in intensive care units and the factors identified as associated with EUGR. At-risk infants should be evaluated for EUGR and their weight and nutritional support should be monitored carefully. Comparisons of long-term outcomes are needed to assess the suitability of growth curves used for EUGR follow-up.

Background: Breast milk (BM) is considered the ideal and natural way of feeding for all infants. Although previous studies evaluated the factors influencing the content of BM, data concerning the e...

Objective Structured light plethysmography (SLP) is a novel and noncontact respiratory assessment technique. It provides tidal breathing measurement in patients difficult to cooperate. In this study, we aimed to determine data for tidal breathing parameters measured by SLP in newborns. Study Design Infants between 2 and 5 days of life without having any respiratory symptoms were eligible for this observational study. In total, 5 minutes of tidal breathing was recorded using SLP (Thora-3Di, PneumaCare Ltd, Cambridge, U.K.) in each infant. Various tidal breathing parameters including timing indices, flow-based parameters, and regional parameters were obtained from SLP data. Results A total of 57 infants underwent measurements in the study. Evaluable recordings from 42 term and 11 late preterm infants were analyzed. Median gestational age and birthweight of the infants were 38 (37–39) weeks and 3,195 (2,790–3,585) g, respectively. In terms of flow-based parameters, “tidal inspiratory flow at 50% of inspiratory volume divided by tidal expiratory flow at 50% of expiratory volume” was 1.29 (1.13–1.53). Relative contribution of the thorax to each breath in percentage was measured as 38.67 (28.21–43.60). Median values of left–right hemithoracic asynchrony and thoraco-abdominal asynchrony were 6.92 (5.35–9.04) and 17.96 (12.98–36.44) degrees in the study population, respectively. There were no differences in tidal breathing parameters except “hemithoracic asynchrony” between term and late preterm infants. Hemithoracic asynchrony was significantly lower in term neonates than late preterms. Conclusion SLP was found to be feasible to obtain measures of tidal breathing parameters in newborns and it could be performed successfully even in the first days of life.

Amaç: Tam enteral beslenmeye erken geçiş protein katabolizmasını önleyerek bebeklerin büyüme ve gelişimine olumlu katkı sağlar. Bu çalışmada çok düşük doğum ağırlıklı preterm bebeklerde tam enteral beslenmeye geçiş süresine etki eden faktörlerin belirlenmesi amaçlandı.Gereç ve Yöntemler: Doğum ağırlığı ≤1500 gr ve gestasyon yaşı ≤32 hafta olan preterm bebeklerin demografik verileri, tam enteral beslenmeye geçiş süreleri, hastanede yatış süreleri, taburculuktaki vücut ağırlıkları ve baş çevreleri, günlük kilo alımları, bronkopulmoner displazi (BPD), nekrotizan enterokolit (NEK), intraventriküler kanama (IVK), respiratuar distres sendromu (RDS), geç neonatal sepsis (GNS) ve mortalite oranları kaydedildi. Veriler tam enteral beslenmeye geçiş zamanına göre ilk 10 günde geçenler erken, 10 günden uzun sürede geçenler ise geç şeklinde gruplandırılarak karşılaştırıldı.Bulgular: Çalışmaya 474 bebek dahil edildi. Bebeklerin 91’i, ilk 10 günde, 383’ü ise 10 günden sonra tam enteral beslenmeye geçen bebeklerdi. Ortanca (IQR) gestasyon haftası sırasıyla 29 (28-30) ve 28 (27-29) hafta, doğum ağırlığı 1240 (1080-1350) gr ve 1030 (850-1190) gr’dı. Tam enteral beslenmeye erken geçen bebeklerde hastanede yatış süresi daha kısa olarak saptandı (p, Objective: Early transition to full enteral nutrition prevents protein catabolism and has a positive impact on the growth and development of infants. The aim of this study was to determine the factors affecting the time to full enteral feeding in very low birth weight infants.Material and Methods: Demographic data of infants with birth weight ≤1500 g and gestational age ≤32 weeks, time to full enteral feeding, weights and head circumference at discharge, daily weight gain, duration of hospital stay, bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), intraventricular hemorrhage (IVH), respiratory distress syndrome (RDS), late neonatal sepsis (LNS) and mortality rates were recorded. Data were compared by grouping according to the time of transition to full enteral nutrition as early in the first 10 days and delayed in more than 10 days.Results: Totally 474 infants were included in the study. Of those 91 infants achieved full enteral feeding in the first 10 days and 383 of them after 10 days. Median gestational age were 29 and 28 weeks and birth weights were 1240 g and 1030 g respectively. While the duration of hospital stay was shorter in the early enteral feeding group (p

To determine the effect of two different feeding strategies on time to achieve full enteral feeding and the incidence of feeding intolerance in preterm infants with birth weight ≤1250 g.A prospective randomized trial (NCT02913677) conducted at a tertiary level neonatal intensive care unit. Preterm infants with birth weight ≤1250 g were randomly allocated to either prolonged minimal enteral nutrition (MEN) in which feed volumes were not increased for five days or early feeding advancement groups in which feed volumes were advanced by 20-25 ml/kg/d until 150 ml/kg/d feed volume was achieved. The primary outcomes were time to reach full enteral feeding sustained for 72 h and incidence of feeding intolerance.A total of 199 infants (99 in prolonged MEN and 100 in early feeding advancement groups) were involved in the study. No statistically significant differences were observed in time to achieve full enteral feeding and feeding intolerance. Daily weight gain (19 versus 16 g;Prolonged MEN is not associated with a delay in time to achieve full enteral feedings. It may even provide an advantage for development of NEC in extremely low birth weight infants.Clinical Trials.gov: NCT02913677.

Background Preterm newborns are born with lower vitamin D stores. Although vitamin D supplementation is recommended there is no consensus regarding the adequate dose of supplementation for preterm infants. Aims To assess the effect of three different doses of vitamin D supplementation (400, 800 and 1000 IU/d) in preterm infants ≤ 32 weeks gestation on the prevalence of vitamin D deficiency and 25(OH) D levels at 36 weeks postmenstrual age (PMA). Study design Prospective randomized trial. Subjects 121 preterm infants with gestational age of 24–32 weeks were randomly allocated to receive 400, 800 or 1000 IU/d vitamin D. Outcome measures Serum concentration of 25(OH) D and the prevalence of vitamin D deficiency at 36 weeks PMA. Vitamin D deficiency was defined as serum 25(OH) D concentrations Results Of the 121 infants 72% had deficient vitamin D levels before supplementation. The average 25(OH) vitamin D concentrations at 36 weeks PMA were significantly higher in 800 IU (40 ± 21.4 ng/ml) and 1000 IU group (43 ± 18.9 ng/ml) when compared to 400 IU group (29.4 ± 13 ng/ml). The prevalence of vitamin D deficiency (2.5 vs 22.5; RR: 0.09; CI:0.01–0.74) and insufficiency (30 vs 57.5; RR:0.32; CI:0.13–0.80) was significantly lower in 1000 IU group when compared to 400 IU group at 36 weeks PMA. Conclusion 1000 IU/d of vitamin D supplementation in preterm infants ≤ 32 weeks gestation age effectively decreases the prevalence of vitamin D deficiency and leads to higher concentrations of 25(OH) vitamin D at 36 weeks PMA Trial registration Clinical Trials.gov : NCT02941185 .

Objective: We aimed to compare the effect of the combined therapy, sildenafil and inhaled nitric oxide with inhaled nitric oxide monotherapy for the treatment of Pulmonary Hypertension of the Newborn.Study Design: Newborn infants (gestational age greater than 34 weeks) who were diagnosed with pulmonary hypertension between December 2008 and 2010 were retrospectively evaluated. Group I (n=14) received monotherapy with inhaled nitric oxide and Group II (n=9) received combined therapy with inhaled nitric oxide and oral sildenafil. Primary outcome was to compare the duration of inhaled nitric oxide therapy between groups.Results: Demographic characteristics were similar between the groups. Combination therapy was associated with early weaning of inhaled nitric oxide (4.8±1.5 vs. 13.5±7.6 hours). The duration of inhaled nitric oxide therapy was slightly shorter in combined therapy group (75[24-125] vs. 109[24-210] hours), however, the difference was insignificant (p=0.2). The incidence of mortality and neonatal outcomes were similar between the groups (p>0.05).Conclusion: Combined therapy did not result in shorter duration of inhaled nitric oxide therapy. Further well designed and larger studies that will elucidate the benefits of combination therapies and optimal therapy whereinhaled nitric oxide is not available are warranted.

Jeune Syndrome is a rareautosomal recessive inherited skeletal chondrodystrophy. Patients usually dieof respiratory failure during early infancy. The disease has a wide clinical pi cture including renal dysplasia, hepatic and pancreatic fibrosis. Here,we present a case of Jeune Syndrome diagnosed by narrowing of thoracic anteriorposterior and transverse diameter and flattening of acetabular ceiling. Weaimed to emphasize the necessity of early diagnosis and close monitoringbecause of the risk of sudden death., Jeune Sendromu nadir görülen otozomal resesif kalıtım gösteren bir iskelet kondrodistrofisidir. Hastalar genellikle solunum yetmezliğinden erken süt çocukluğu döneminde kaybedilirler. Hastalık renal displazi, hepatik ve pankreatik fibrozisi içeren geniş bir klinik tabloya sahiptir. Burada toraks ön arka ve transvers çapta daralma, asetabular tavanda düzleşme saptanarak tanı konulan Jeune Sendromlu bir olguyu sunduk. Ani ölüm riski taşımaları nedeniyle erken tanınıp yakından takip edilebilmesi gerekliliğini vurgulamayı amaçladık.

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.

Objective To determine the within-day variation of fat, protein, and carbohydrate content of breast milk. Methods The study was conducted at Zekai Tahir Burak Maternity Teaching Hospital between April 2013 and January 2014. We obtained milk samples from lactating mothers of hospitalized infants through hand expression after breast-feeding or pumping three times a day. A mid-infrared human milk analyzer was used for measuring the macronutrient contents of breast milk samples. Results Lactating mothers of 52 infants (30 preterm, 22 term) were recruited to the study. No significant difference was found in protein, fat, and carbohydrate content of milk samples throughout the day. We compared within-day variation of macronutrients of transitional and mature milk, milk samples from the mothers of preterm and term infants, and samples collected by either hand expression or pumping. We did not find a significant difference between the groups. Conclusion Absence of circadian variations in lipid, carbohydrate, and protein content of breast milk in our study may be related to ethnic differences, maternal nutritional status, different milk content measurement technique, and population characteristics.

Nemalin miyopatisi degisik derecelerde ve genellikle ilerleyici olmayan kas gucsuzlugu hipotoni ve derin tendon reflekslerinin azalmasi veya kaybi ile karakterize nadir gorulen konjenital, herediter bir noromuskuler hastaliktir. Kas gucsuzlugu ve hipotoni en belirgin ozellik olup en sik yuz kaslari, boyun ve govde fleksor kaslari, ayak dorsifleksor ve ayak parmak fleksor kaslari etkilenir. Bazi hastalarda ince yuz gorunumu, yuksek damak, pektus ekskavatum gibi minor bulgular da gorulebilmektedir. Diger atipik bulgular arasinda artrogripozis, santral sinir sistemi tutulumu ve konjenital frakturler sayilabilir. Burada hipotoni ve bilateral femur frakturu ile prezente olan bir nemalin miyopatisi olgusu sunulmaktadir. Bu olgu hipotonisi olan olgularda diger klinik bulgularla birlikte nemalin miyopatisinin ayirici tanida dusunulmesi ve tani konmasinda kas biyopsisinin oneminin vurgulanmasi amaciyla sunulmustur.

Lamotrigine (LTG) is a widely used second-generation antiepileptic drug for long-term therapy of epileptic patients. Although LTG monotherapy during pregnancy is assumed to be relatively safe, teratogenic effects related to LTG has been reported previously. The presence of fetal malformations and maternal drug-induced lupus erythematosus concurrently in a pregnant women using LTG have not been reported before. We herein report a term infant with coarctation of aorta and ventricular septal defect, who was born to a mother treated with LTG for epilepsy before conception and throughout pregnancy. The mother was diagnosed with drug-induced lupus erythematosus at the 36th gestational week, and the symptoms resolved after discontinuation of the drug. Fetal cardiac anomalies should be searched in mothers who were exposed to LTG during pregnancy.

Aims Mastitis in lactating mothers reduces milk production and alters the cellular composition of milk. Changes occurring in the mammary gland during the inflammatory response are believed to increase the permeability of the blood–milk barrier. This study examined the effect of mastitis during lactation on the macronutrient content of breast milk. Methods The study was conducted at Zekai Tahir Burak Maternity Teaching Hospital. Transitional breast milk samples were obtained from term lactating mothers with or without mastitis. Milk protein, fat, carbohydrate, and energy levels were measured using a mid-infrared human milk analyzer. Results The study recruited 30 term lactating mothers: 15 mothers diagnosed with mastitis and 15 healthy mothers. The characteristics of the mothers in both groups were similar. Fat, carbohydrate, and energy levels were statistically lower in the milk samples of mothers with mastitis compared with the mothers without mastitis. Conclusion Lactational mastitis was associated with lower breast milk fat, carbohydrate, and energy levels. The local inflammatory response induced by cytokines and increased blood–milk barrier permeability might account for the changes in the fat, carbohydrate, and energy levels of human milk.

The aim of this study was to compare the efficacy of orally administered Lactobacillus reuteri (L. reuteri) versus nystatin in prevention of fungal colonization and invasive candidiasis in very low birth weight infants.A prospective, randomized comparative study was conducted in preterm infants with a gestational age of ≤32 weeks and birth weight of ≤1500 g. Patients were randomized into two groups, to receive L. reuteri or nystatin. Skin and stool cultures were performed once a week for colonization and blood cultures for invasive infections. The trial was registered to ClinicalTrials.gov under identifier NCT01531192.A total of 300 preterm infants were enrolled (n = 150, for each group). Gastrointestinal colonization and skin colonization rates were not significantly different between the groups (18.7% versus 16%, p = 0.54 and 14% versus 12%, p = 0.6, respectively). Invasive candidiasis was detected in two patients of the probiotic group and one patient of the antifungal group. Proven sepsis, feeding intolerance, and duration of hospitalization were significantly lower in the probiotics group than in the antifungal group.Prophylactic L. reuteri supplementation is as effective as nystatin, and more effective in reducing the incidence of proven sepsis in addition to its favorable effect on feeding intolerance.

Background and Objective: It is essential to establish optimum parameters for maintaining the quality of stored milk until the moment of consumption with minimal deterioration of its properties. The aim of the study was to evaluate total antioxidant capacity (TAC) and total oxidation status (TOS) of fresh and freeze-stored samples (at −80 ◦ C) of preterm human milk (HM). Methods: Samples of colostrum were collected from 98 healthy women within the first 4 days after delivery. The total milk volume collected (6 ml) was divided in two aliquot parts: 3 ml for the fresh analysis which was done immediately after the extraction and 3 ml for storage under freezing conditions at −80 ◦ C for three months. The

Summary Objectives/Hypothesis The date of last menstruation period and ultrasonography are the most commonly used methods to determine gestational age (GA). However, if these data are not clear, some scoring systems performed after birth can be used. New Ballard Score (NBS) is a commonly used method in estimation of GA. Cry sound may reflect the developmental integrity of the infant. The aim of this study was to evaluate the connection between the infants' GA and some acoustic parameters of the infant cry. Study Design A prospective single-blind study was carried out. Methods In this prospective study, medically stable infants without any congenital craniofacial anomalies were evaluated. During routine blood sampling, cry sounds were recorded and acoustic analysis was performed. Step-by-step multiple linear regression analysis was performed. Results The data of 116 infants (57 female, 59 male) with the known GA (34.6 ± 3.8 weeks) were evaluated and with Apgar score of higher than 5. The real GA was significantly and well correlated with the estimated GA according to the NBS, F0, Int, Jitt, and latency parameters. The obtained stepwise linear regression analysis model was formulized as GA = (31.169) − (0.020 × F0) + (0.286 × GA according to NBS) − (0.003 × Latency) + (0.108 × Int) − (0.367 × Jitt). The real GA could be determined with a ratio of 91.7% using this model. Conclusions We have determined that after addition of F0, Int, Jitt, and latency to NBS, the power of GA estimation would be increased. This simple formula can be used to determine GA in clinical practice but validity of such prediction formulas needs to be further tested.

There is some evidence that maternal psychological status in the prenatal and postnatal periods is associated with infants' cognitive, behavioural, and emotional functions.The aim of this study was to examine the relationships of maternal depression and anxiety with neurodevelopmental outcomes of preterm infants with a gestational age of ≤32weeks, examined at a corrected age of 18 to 22months.Cross-sectional study.In total, 220 preterm infants with a gestational age of ≤32weeks who were born from January 2008 to September 2011 and admitted to the neonatal intensive care unit were prospectively examined.Neurodevelopmental evaluation was performed at a corrected age of 18 to 22months by a developmental paediatrician using the Bayley Scales of Infant Development II (BSID-II). The Beck Depression Inventory and Beck Anxiety Inventory were used to assess maternal depression and anxiety at the same visit as the neurodevelopmental evaluation.The depression scores of mothers of infants with a Mental Development Index (MDI) score of70 were significantly higher than those of mothers of infants with an MDI score of70 (16.3±12.8 vs 8.8±7.0, p0.001). The depression scores of mothers of infants with neurodevelopmental impairment were also significantly higher than those without neurodevelopmental impairment (12.8±10.5 vs 8.8±7.3, p=0.003). There was no relationship between the presence of cerebral palsy or a Psychomotor Developmental Index (PDI) score of70 and the mothers' depression scores. Multiple regression analysis revealed that maternal depression and the occurrence of more than two sepsis attacks were associated with an MDI score of70, and grade III to IV intraventricular haemorrhage was associated with neurodevelopmental impairment and a PDI score of70.Maternal depression is negatively associated with the neurodevelopment of preterm infants at a gestational age of ≤32weeks. Maternal psychological well-being should be taken into consideration during the long-term follow-up of preterm infants.

To determine whether there is an association between platelet counts and patent ductus arteriosus (PDA) incidence and/or closure in preterm newborns.Premature infants with hemodynamically significant PDA (n=154) and a control group without PDA (n=207) who were hospitalized in the NICU were retrospectively evaluated. Platelet counts and other platelet indices including mean platelet volume (MPV) and platelet distribution width (PDW) of the infants in both groups during the first 3 days of life were recorded. Ibuprofen was started in infants with hemodynamically significant PDA and echocardiography was repeated 48 h thereafter to assess the closure of ductus.Median gestational age and birth weight of the infants with PDA were 28 (range 26-29) weeks and 1060 (range 892-1250) g respectively. Platelet counts were significantly lower in the patient group than in the control group (p0.001). Multivariate analysis including gestational age, presence of RDS, presence of thrombocytopenia and PDW showed that hemodynamically significant PDA was independently associated with platelet count150,000 (OR=2.13, 95% CI 1.26-3.61; p=0.005), high PDW (17) (OR=2.68, 95% CI 1.41-5.09; p=0.003) and the presence of RDS (OR=2.25, 95% CI 1.41-3.59; p=0.001). Baseline platelet counts of the infants in whom ductus closed or persisted after ibuprofen treatment were similar.PDA was associated with low platelet count and high PDW but not with other platelet indices in preterm infants. We could not show an association between platelet counts and persistence or closure after medical treatment.

Objective To evaluate the growth and neurodevelopment outcomes of very low-birth-weight (VLBW) preterm infants supplemented with oral probiotics for the prevention of necrotizing enterocolitis (NEC). Study Design This prospective follow-up study was conducted in a cohort of VLBW preterm infants enrolled in a randomized controlled clinical trial to evaluate the efficacy of oral probiotics for the prevention of NEC. Growth outcomes included weight, length, and head circumference. Cognitive and neuromotor development were assessed by using the Bayley Scales of Infant Development II. Sensory and neurological performance was evaluated by standard techniques. The primary outcome was neurodevelopmental impairment at 18 to 22 months' corrected age. Results A total of 221 infants completed the trial protocol. Of the 208 infants eligible for follow-up, 174 infants (86 in the probiotics group and 88 in the control group) were evaluated. There was no significant difference in growth and neurodevelopmental outcomes between the two groups. Conclusion Oral probiotic administered to VLBW infants to reduce the incidence and severity of NEC started with the first feed did not affect growth, neuromotor, neurosensory, and cognitive outcomes at 18 to 22 months' corrected age.

Background/Aim: To evaluate the association between the cortisol levels and clinical outcomes in preterm infants. Methods: Baseline adrenocorticotropic hormone (ACTH) and cortisol levels and ACTH-stimulated cortisol concentrations after low-dose synthetic ACTH (Synacthen®, 1 µg/kg) were measured in preterm infants Results: Ninety-eight preterm infants were enrolled. Median baseline cortisol and ACTH levels were 13.7 µg/dl (25th-75th percentile, 9.7-21.1 µg/dl) and 11.5 pg/ml (25th-75th percentile, 6.9-22.6 pg/ml), respectively. Median peak cortisol level after ACTH stimulation was 33.6 µg/dl (25th-75th percentile, 27.2-40.2 µg/dl). The prevalence of relative AI, defined as baseline cortisol Conclusion: Neither baseline nor ACTH-stimulated cortisol levels were associated with clinical outcomes in preterm infants. A significant proportion of preterm infants hospitalized in the neonatal unit fulfilled the criteria for relative AI; however, relative AI did not affect outcome.

Background Oxidative damage is important in the pathogenesis of respiratory distress syndrome (RDS). However, data on the effect of surfactant therapy on oxidative stress in vivo are limited. We aimed to evaluate the oxidant/antioxidant status in preterm infants with RDS via measurement of total antioxidant capacity (TAC) and total oxidant status (TOS), to determine the effect of surfactant on oxidant/antioxidant balance and to assess the association between TAC, TOS and clinical outcomes of the patients. Methods Sixty-nine infants with RDS were included. Blood samples for determining TAC and TOS were collected before and 48 h after surfactant treatment. TAC and TOS levels were analysed in serum. Patients were followed up until discharge or death. Results Post-surfactant TAC levels were significantly higher than pre-surfactant TAC levels ( P = 0.029). TAC/TOS ratio significantly increased after surfactant treatment ( P = 0.018). Infants Conclusions Oxidant–antioxidant balance shifts in favour of the antioxidant system after surfactant treatment. Lower TAC/TOS ratio in preterm infants may be associated with increased mortality.

Neonatal adrenal hemorrhage is uncommon. It is present in 0,2% of newborns. Ten percent of the cases occur bilaterally. It can be associated with birth trauma, large birth weight, or neonatal course complicated by hypoxia and asphyxia, hypotension, or coagulopathy. Scrotal hematoma is an extremely rare manifestation of NAH. Most patients present scrotal swelling with bluish discolouration. Scrotal swelling with/without bluish discoloration in newborns may result from different causes. We report an unusual case of neonatal adrenal hemorrhage secondary to perinatal asphyxia, associated with SH. Neonatal adrenal hemorrhage and scrotal hematoma were diagnosed by ultrasonography and treated by conservative treatment, avoiding unnecessary surgical exploration.

Objective To compare the effectiveness of nasal continuous positive airway pressure (NCPAP) and nasal intermittent positive-pressure ventilation (NIPPV) as the initial respiratory support within the minimally invasive surfactant therapy (MIST) approach in preterm infants with respiratory distress syndrome. Design Prospective, randomised controlled study. Setting Tertiary neonatal intensive care unit. Patients and interventions This study enrolled 200 preterm infants with a gestational age of 26–32 weeks who showed signs of respiratory distress but did not require intubation in the delivery room. Surfactant therapy was performed using the MIST approach in the patients who met the criteria for surfactant administration. Main outcome measures The primary outcomes were a need for intubation within the first 72 h of life and a surfactant requirement. Results The infants in the study displayed similar characteristics at birth. Fewer infants in the NIPPV group required surfactant therapy (38% vs 60%; p=0.002) or invasive ventilation during the first 72 h of life (13% vs 29%; p=0.005), and NIPPV reduced the rate of moderate-to-severe bronchopulmonary dysplasia (BPD) (7% vs 16%; p=0.046). Multivariate logistic regression analysis showed that NIPPV support (OR: 0.36, 95% CI 0.17 to 0.76; p=0.008) and higher gestational age (OR: 0.76, 95% CI 0.59 to 0.98; p=0.041) reduced the need for invasive ventilation within the first 72 h of life. Surfactant requirement was also decreased with NIPPV support (OR: 0.39, 95% CI 0.22 to 0.71; p=0.002). However, there was no impact on BPD, based on the multivariate analysis. Conclusions In infants born at 26–32 weeks’ gestation, NIPPV reduced the need for invasive ventilation and the surfactant requirement within the MIST approach. Trial registration number ClinicalTrials.gov under identifier NCT01741129.

Neonatal adrenal hemorrhage is uncommon. It is present in 0,2% of newborns. Ten percent of the cases occur bilaterally. It can be associated with birth trauma, large birth weight, or neonatal course complicated by hypoxia and asphyxia, hypotension, or coagulopathy. Scrotal hematoma is an extremely rare manifestation of NAH. Most patients present scrotal swelling with bluish discolouration. Scrotal swelling with/without bluish discoloration in newborns may result from different causes. We report an unusual case of neonatal adrenal hemorrhage secondary to perinatal asphyxia, associated with SH. Neonatal adrenal hemorrhage and scrotal hematoma were diagnosed by ultrasonography and treated by conservative treatment, avoiding unnecessary surgical exploration.

Enterocutaneous fistula in newborns and preterms is a well-recognized complication after necrotizing enterocolitis and abdominal surgical procedures/percutaneous interventions. However, to our knowledge, enterocutaneous fistula associated with purpura fulminans has not been reported before. Herein we report a preterm infant with purpura fulminans who developed cutaneous necrotic lesions on anterior abdominal wall. Adherence of necrotic abdominal skin to the adjacent intestinal wall resulted in enterocutaneous fistula. The patient was treated conservatively with bowel rest and antibiotics. The fistula was surgically closed 2 months later.

Objective To evaluate the effect of oral Lactobacillus reuteri ( L reuteri ) first on the incidence and severity of Necrotising enterocolitis (NEC) and second on sepsis. Design Prospective randomised controlled study. Setting Tertiary neonatal intensive care unit. Patients and interventions Preterm infants with a gestational age of ≤32 weeks and a birth weight of ≤1500 g were included (n=400). Infants in the first group were given 100 million CFU/day (5 drops) of lyophilised L reuteri (DSM 17938) mixed in breast milk or formula, starting from first feeding until discharge. Participants in the control group were given a placebo. Main outcome measures To determine and compare the frequency of NEC and/or death after 7 days, frequency of proven sepsis, rates of feeding intolerance and duration of hospital stay. Results There was no statistically significant difference between groups in terms of frequency of NEC stage ≥2 (4% vs 5%; p=0.63) or overall NEC or mortality rates (10% vs 13.5%; p=0.27). Frequency of proven sepsis was significantly lower in the probiotic group compared to the control group (6.5% vs 12.5%; p=0.041). A significant difference was also observed with regard to rates of feeding intolerance (28% vs 39.5%; p=0.015) and duration of hospital stay (38 (10–131) vs 46 (10–180) days; p=0.022). Conclusions Our results show that oral L reuteri does not seem to affect the overall rates of NEC and/or death in preterm infants followed up in the neonatal intensive care unit, and significant reductions were observed in the frequency of proven sepsis, rates of feeding intolerance and duration of hospital stay. Trial registration number NCT01531179.

Persistent pulmonary hypertension of the newborn is a source of considerable mortality and morbidity. Anomalous origin of one pulmonary artery, an uncommon congenital cardiac malformation, is a rare cause of persistent pulmonary hypertension. Here, we report the case of a patient with an anomalous origin of one pulmonary artery from the innominate artery who presented with persistent pulmonary hypertension refractory to treatment.