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Permanent junctional reciprocating tachycardia (PJRT) is most often observed in infants and children and can lead to incessant tachycardia. PJRT is usually refractory to medical treatment. Tachycardia may infrequently occur in the fetus in which case fetal tachycardia transplacental treatment should be started immediately. Term delivery is recommended for fetuses with tachycardia in the absence of significant clinical compromise to avoid complications of preterm birth. Herein, a 36-week preterm neonate presented with PJRT. He had tachycardia in the fetal period and was treated with digoxin, amiodaron, and esmolol therapy after birth without undergoing the catheter ablation procedure.

Muğla ilinde, prematür doğum öyküsüyle Prematüre Retinopatisi taraması için kliniğimize başvuran ya da yönlendirilen bebeklerdeki prematüre retinopatisi sonuçlarını değerlendirmek. 32 hafta altında gestasyonel yaşı olan bebeklerin tümü ile 32 hafta üstünde olan ve neonatolog ya da pediatrist tarafından klinik olarak riskli bebek olarak değerlendirilen tüm bebekler tarandı. 2014-2019 yılları arasında toplam 486 bebeğe, 949 mükerrer muayene yapıldı. Prematüre retinopatisi, herhangi bir evrede %8 olarak tespit edildi. Tedaviye ihtiyaç duyan bebek sayısı sadece 1 idi. Prematüre retinopatisine bağlı körlüklerin önüne geçebilmek için, en ideal yöntem, risk grubundaki bebeklerin, standart bir çerçeve içinde taranmasıdır. Bu şekilde, tedavi gerektirecek bebeklerin zaman kaybetmeden etkili şekilde tedavisi ile prematüre retinopatisine bağlı körlükler önlenebilir.

Townes–Brocks syndrome (TBS) is a rare syndrome characterized by triad of anal, ear, and thumb anomalies. Further malformations/anomalies include congenital heart diseases, foot malformations, sensorineural and/or conductive hearing impairment, genitourinary malformations, and anomalies of eye and nervous system. Definitive diagnosis for TBS is confirmed by molecular analysis for mutations in the SALL1 gene. Only one known case of TBS with absent pulmonary valve syndrome (APVS) has been previously described to our knowledge. Here, we report a newborn diagnosed with TBS with APVS and tetralogy of Fallot (TOF) who was found to carry the most common pathogenic SALL1 gene mutation c.826C > T (p.R276X), with its surgical repair and postoperative follow-up. To our knowledge, this is the first genotyped case of TBS from Turkey to date. TBS should be suspected in the presence of ear, anal, and thumb malformations in a neonate. If a patient with TBS and TOF-APVS needs preoperative ventilation within the first months of life, this implies prolonged postoperative intubation and increased risk of mortality. https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0041-1740371

Townes–Brocks syndrome (TBS) is a rare syndrome characterized by triad of anal, ear, and thumb anomalies. Further malformations/anomalies include congenital heart diseases, foot malformations, sensorineural and/or conductive hearing impairment, genitourinary malformations, and anomalies of eye and nervous system. Definitive diagnosis for TBS is confirmed by molecular analysis for mutations in the SALL1 gene. Only one known case of TBS with absent pulmonary valve syndrome (APVS) has been previously described to our knowledge. Here, we report a newborn diagnosed with TBS with APVS and tetralogy of Fallot (TOF) who was found to carry the most common pathogenic SALL1 gene mutation c.826C > T (p.R276X), with its surgical repair and postoperative follow-up. To our knowledge, this is the first genotyped case of TBS from Turkey to date. TBS should be suspected in the presence of ear, anal, and thumb malformations in a neonate. If a patient with TBS and TOF-APVS needs preoperative ventilation within the first months of life, this implies prolonged postoperative intubation and increased risk of mortality.

Aim The perfusion index (PI) is a noninvasive marker derived from photoelectric plethysmographic signals in pulse oximetry in the evaluation of peripheral perfusion. This study was aimed to determine the correlation between PI and left ventricular output (LVO) in healthy late preterm infants at 48th hour of life. Methods With new generation pulse oximeter (MASIMO Rad 7 Oximeter) pre- and post-ductal PI values were recorded from healthy late preterm babies at the 48th hour of life. PI was determined simultaneously with LVO as measured by transthoracic echocardiography. Results A total of 50 late preterm babies were included in the study. The mean gestational age of the cases was 35.4 ± 0.7 weeks and the birth weight was 2,586 ± 362 g. Mean pre- and post-ductal PI values at the postnatal 48th hour of babies' life were found to be 2.0 ± 0.9 and 1.7 ± 1.1. The mean LVO value was 438 ± 124, LVO/kg 175 ± 50. When the LVO value was normalized according to the babies' body weight, there was no statistically significant correlation between the pre- and post-ductal PI and the LVO/kg value (r 0.05 in both comparisons). Conclusion There was no correlation between pre- and post-ductal PI and LVO values in healthy late preterm infants. This may be due to the failure of the LVO, a systemic hemodynamic parameter, to accurately reflect microvascular blood flow due to incomplete maturation of the sympathetic nervous system involved in the regulation of peripheral tissue perfusion in preterm babies. Key points · No correlation found between PI and LOV in preterm babies.. · LVO cannot adequately reflect peripheral blood flow.. · Sympathetic nervous system is immature in preterm infants..

Aim: Sepsis is an important cause of morbidity and mortality for newborn babies. Cardiac involvement plays an important role in the pathophysiology of sepsis and has a significant effect on prognosis. In this study, the relationship between cardiac biomarkers, such as troponin-I (cTnI) and creatine kinase-myocardial band (CK-MB) levels, and prognosis in neonatal sepsis were investigated.Methods: A total of 87 patients with culture positive sepsis (Group I, n = 26) or clinical sepsis (Group II, n =61) were included in the study. The control group (Group III) consisted of 42 non-septic otherwise healthy newborns. The demographic data of the cases (gestational age, postnatal age, gender, age at diagnosis) and laboratory examination results (complete blood count, C-reactive protein, cTnI, CK-MB, blood gases and lactate and culture-antibiogram results) were recorded in the previously prepared study forms. In addition, the outcome (survival or death), and short- and long-term prognosis of the cases (epilepsy, neuromotor disability, vision-hearing problems, etc.) were also recorded.Results: No statistically significant difference was found between the groups in terms of gender, gestational age and birth weight. CK-MB level was high in 77%, 74%, and 71% of cases for Group I, Group II and Group III, respectively. No statistically significant difference was found in the rates of CK-MB levels in the patients who died and survived (15.8% and 11.8%, respectively) (p > 0.05). The cTnI cut-off value for the newborns included in the study was found to be 0.13 ng/ml (sensitivity 89.5%, specificity 90.9%). It was observed that a 0.001 unit increase in cTnI value (p =0.012) increased the risk of death 1.4 times.Conclusion: There is a relationship between cTnI test abnormality and mortality in septic newborns. However, multi-center studies involving more cases are needed to determine the relationship between cTnI test abnormality and long-term prognosis of patients.

Gelenekler, kusaktan kusaga ve toplumdan topluma gecen kultur miraslari, aliskanliklar, bilgiler, toreler ve davranislardir. Bebek bakimiyla ilgili bazi geleneklerde kundaklama gibi guncel tibbi bilgiyle celisen uygulamalar bulunmaktadir. Bu tur geleneksel uygulamalarin egitim sonrasi buyuk olcude azaldigi gozlenmektedir. Buna karsilik, ulkemizde bebek tuzlama gibi bazi yan etkileri ciddi sorunlara neden olan geleneksel uygulamalarin pek cok yorede hala yaygin olarak uygulandigi bilinmektedir. Cocuk hekimlerinin gorev yaptiklari bolgelerdeki halkin geleneklerini tanimasi ve egitici rol ustlenmesi cocuk sagliginin gelistirilmesi acisindan onem arz etmektedir. Calismamizda Mugla ilimizde bebek bakimindaki geleneksel uygulamalarin arastirilmasi amaclanmistir. Hastanemiz Cocuk Sagligi ve Hastaliklari poliklinigine basvuran 305 anneye bebek bakimindaki geleneksel uygulamalara yonelik olarak anket uygulanmistir. En sik gozlenen geleneksel uygulama %59,9 ile bebek tuzlama olmustur. Annelerin egitim suresiyle geleneksel uygulamalar arasinda anlamli iliski oldugu gorulmustur. Sonuc olarak annelerin egitilmesinin bebek bakiminda zararli olma potansiyeli olan geleneksel uygulamalari azaltabilecegi kanisina varilmistir.

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p

BackgroundThe aim of this study was to evaluate if the cord blood nucleated red blood cell (nRBC) levels can predict the development of hyperbilirubinemia in healthy neonates.MethodsAll healthy newborn infants born after 35 or more weeks of gestation at our hospital between January 2016 and April 2017 were included. The levels of nRBC were counted in umbilical cord blood samples. Neonates were grouped into two study groups based on the presence or absence of neonatal jaundice.ResultsThe study included overall 149 eligible newborn infants. The levels of nRBC and absolute nRBC count showed statistically significant differences between newborns with or without jaundice (p=0.01 and 0.02, respectively).ConclusionsWe suggest that increased nRBC counts in cord blood may be a predictive marker for hyperbilirubinemia in healthy newborn infants.

Amplitude-integrated electroencephalography (aEEG) is a method for continuous monitoring of brain activity that is increasingly used in the neonatal intensive care unit. aEEG is accessible by non-expert person, and offers continuous bedside cerebral function monitoring (CFM). In its simplest form, aEEG is a processed single-channel electroencephalogram that is filtered and time-compressed. Several classifications are currently in use to describe patient tracings, incorporating voltage criteria, pattern recognition, cyclicity, and the presence or absence of seizures. The main usage of the CFM currently is for monitoring term newborns after birth asphyxia. Another well-established indication for CFM is monitoring for seizure activity with an addition advantage of monitoring the treatment effect. Current evidences demonstrate that aEEG is useful to monitor cerebral background activity, diagnose and treat seizures and predict neurodevelopmental outcomes for newborns. Thus, the main advantages of this device are its simplicity for both application and interpreting on one hand and the possibility of continuous long-term monitoring with real time assessment of clinical events on the other. This review aims to explain the fundamentals behind aEEG and its clinical applications.

AMAÇ: Uzamış unkonjuge hiperbilirubinemi, bir neona-tal sarılık tipidir. Yenidoğanlarda, postnatal 14-21 günden daha uzun süren, yüksek bilirubin düzeyleri(, OBJECTIVE: Prolonged unconjugated hyperbilirubinemia is a type of neonatal jaundice, which occurs in newborns with high bilirubin levels (> 8 mg/dl) persisting beyond 14-21 postnatal days. This study was carried out to evaluate and determine the clinical and laboratory characteristics of infants with prolonged jaundice and underling causes.MATERIAL AND METHODS: This prospective study was conducted on newborn infants diagnosed with prolonged unconjugated hyperbilirubinemia during January 2018 and December 2018 at our pediatric outpatient clinic. The demographic, clinical and laboratory characteristics of these patients were recorded and then analyzed.RESULTS: In total, 91 infants diagnosed with prolonged jaundice and 65 healthy newborn infants as control group were enrolled in this study. Of the infants with prolonged jaundice, mean birth-weight was 3152 ± 504 g (1800-4300 g), mean gestational age was 38.2 ± 1.6 weeks (35-41 weeks), and mean total bilirubin level at the time of diagnosis was 10.98 ± 2.3 mg/dL (8-18.7 mg/dL). There was male sex dominance in prolonged jaundice group (61.5%) Time to reach birth-weight was longer in infants with prolonged jaundice than control group (p = 0.02). The underlying etiologies of prolonged jaundice were as follows: breast milk (45%), congenital hypothyroidism (9.9%) and urinary tract infection (4.4%).CONCLUSIONS: Determining the main causes of prolonged jaundice in neonates is of paramount importance. In the majority of cases, neonatal hyperbilirubinemia is associated with physiological factors like breastfeeding

Background:To investigate the feasibility of critical congenital heart disease (CCHD) screening test by pulse oximetry in four geographical regions of Turkey with different altitudes, before implementation of a nationwide screening program.Methods:It was a prospective multi-centre study performed in four centres, between December, 2015 and May, 2017. Pre- and post-ductal oxygen saturations and perfusion indices (PI) were measured using Masimo Radical-7 at early postnatal days. The results were evaluated according to the algorithm recommended by the American Academy of Pediatrics. Additionally, a PI value Results:In 4888 newborns, the mean screening time was 31.5 ± 12.1 hours. At first attempt, the mean values of pre- and post-ductal measurements were: saturation 97.3 ± 1.8%, PI 2.8 ± 2.0, versus saturation 97.7 ± 1.8%, PI 2.3±1.3, respectively. Pre-ductal saturations and PI and post-ductal saturations were the lowest in Centre 4 with the highest altitude. Overall test positivity rate was 0.85% (n = 42). CCHD was detected in six babies (0.12%). Of them, right hand (91 ± 6.3) and foot saturations (92.1 ± 4.3%) were lower compared to ones with non-CCHD and normal variants (p Conclusion:This study concluded that pulse oximetry screening is an effective screening tool for congenital heart disease in newborns at different altitudes. We support the implementation of a national screening program with consideration of altitude differences for our country.

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.

Kucukozkan, Tuncay/0000-0003-4280-3883 WOS: 000418200900010 Objective: To evaluate the effectiveness of non-contact infrared thermometer (NCIT) by comparing with other thermometers and/or body temperature measurement methods. Methods: The patients were divided into 3 groups: 0-1 year-old babies, >16 year-old female patients, and 1-16 year-old children. The body temperature of all patients was measured by NCIT at the temporal region, and the digital thermometer through the axillary region. Moreover, body temperature of the children was measured with tympanic thermometer and of adults with digital thermometer through oral route. Results: When compared to other temperature measurement methods, in all patients, the temporal temperature was not statistically different to that of rectal temperature (p>0.05); but it was 0.6 degrees C higher than axillary (p= 38 degrees C has been taken as a reference value for fever; the sensitivity, specificity, positive and negative predictive values for average temporal temperature were 48.5, 99.5, 88.9, and 96.3%, respectively. Based on visual inspection of the Bland-Altman plots, NCIT underestimated the higher body temperatures which resulted in a low sensitivity. Conclusion: Our findings indicate that NCITs can be used as a rapid, hygienic and noninvasive method for excluding fever in children. However, this method has low sensitivity for detecting fever compared to that of rectal measurement and other noninvasive techniques. Although temporal temperature seems to be close to rectal temperature, this method is not ideal for detecting febrile patients yet.

Objective To evaluate the underlying causes and outcomes of neonates who underwent acute peritoneal dialysis (APD). Study Design This report describes a 7-year experience with APD in 77 neonates. Results Underlying causes requiring APD were acute tubular necrosis (ATN; n = 53), inborn error of metabolism ( n = 18), bilateral renal vein thrombosis ( n = 3), obstructive uropathy ( n = 2; posterior urethral valve and neurogenic bladder), and bilateral renal artery thrombosis ( n = 1). Fifteen of the 53 patients developed post–cardiac surgery ATN. The mean dialysis duration was 6.2 ± 10.7 days (range 1 to 90 days). Complications of procedure were hyperglycemia ( n = 35), leaking of dialysate ( n = 13), peritonitis ( n = 10), catheter obstruction ( n = 3), bleeding when inserting the catheter ( n = 3), exit site infection ( n = 2), and bowel perforation ( n = 1). There were 57 deaths (74%) in this high-risk group due to underling causes. Of the 20 survivors, 16 patients showed a full renal recovery, but mild chronic renal failure developed in 1 patient and proteinuria with/without hypertension in 3 patients. Conclusion Peritoneal dialysis is an effective means of renal replacement therapy in the neonatal period in the management of metabolic disturbances as well as renal failure. Although major complications of procedure are not so common, these patients have high mortality rates due to the serious nature of the primary causes.

Yenidogan diyabeti yasamin ilk alti ayi icinde gorulen pankreas beta; hucrelerinde islevsel ya da hucresel bozukluklarla birlikte olusan tek gen hastaligidir Sikligi 400 000 500 000 canli dogumda bir olarak bildirilmistir Olgularin yaklasik yarisindan KCNJ11 ABCC8 ve INS genlerinde mutasyonlar sorumludur Burada diyabet tanisi konulan bir yenidogan olgusu nadir gorulmesi nedeniyle literatur bilgileri isiginda sunuldu Otuz yedinci gebelik haftasinda 1 400 g agirliginda dogan 32 gunluk erkek bebek kan sekerlerinin yuksek seyretmesi nedeniyle sevk edildi Vucut agirligi 1 460 g olan hastanin cilt alti yag dokusunun azaldigi iki tarafli inmemis testisleri ve mikropenisi oldugu saptandi; mezokardiyak odakta III VI deg; sistolik ufurum duyuldu Laboratuvar tetkiklerinde kan sekerleri yuksek 340 431 mg dL serum ve idrar ketonu negatif; insulin ve C peptit duzeyleri dusuk sirasiyla lt;2 mU mL ve lt;0 1 ng mL ; insulin adacik hucre ve glutamik asit dekarboksilaza karsi antikorlari negatif bulundu Beslenmenin duzenlenmesi ile birlikte insulin tedavisi baslanan hastada iyi bir glisemik kontrol saglanamadi ve dogum sonrasi 83 gunde araya giren sepsis nedeniyle kaybedildi nbsp; Turk Ped Ars 2012; 47: 137 40

Burkholderia gladioli is a rare cause of bacteremia and sepsis in the absence of such predisposing factors as chronic granulomatous disease, cystic fibrosis, and immunosuppression. Little is known about B. gladioli infection in newborns. The aim of this study was to present the features of B. gladioli infection in newborns. Clinicopathological characteristics, patterns of antimicrobial susceptibility, predisposing factors, and outcomes of B. gladioli bloodstream infection were retrospectively analyzed in newborns treated between 2008 and 2011. During the 3-year study period, B. gladioli was isolated from the blood cultures of 14 patients (3.7 per 1,000 admissions). In all, 5 (35.7 %) of the 14 cases had a positive blood culture at the time of initial admission. Primary diagnoses in the neonates were severe major congenital anomalies, congenital leukemia, prematurity with respiratory distress syndrome, pneumonia, and parapneumonic pleural effusion. In total, 10 (71.4 %) of the patients underwent ≥2 invasive procedures. The overall in-hospital mortality rate was 21.4 %, whereas the mortality rate due to B. gladioli infection was 7 %. B. gladioli might be a causative microorganism of both early neonatal and nosocomial sepsis in newborns. To the best of our knowledge, this is the first study on B. gladioli infection in newborns. Invasive procedures and severe major congenital anomalies may be predisposing factors for B. gladioli bloodstream infection in neonates. Although it appears to have low pathogenic potential and an insidious clinical course in newborns, resistance to antibiotics may be a potential problem. Mortality was primarily associated with underlying diseases.

Objective To determine the clinical spectrum of hemolytic disease due to irregular blood subgroup incompatibility in hospitalized neonates. Study Design The medical records of the all hospitalized newborn patients diagnosed with indirect hyperbilirubinemia due to subgroup incompatibility in Kell, C, c, E, and e systems were included in the study. Data from 106 newborns with hemolytic jaundice due to irregular blood subgroups were retrospectively evaluated, and clinical and laboratory findings were compared between patients . The treatment modalities given to the patients of each subgroup types and the laboratory findings and treatment modalities of the cases according to Coombs tests results were also analyzed. Fetal affection of the hemolysis and also fetal losses due to irregular red-cell alloimmunization were not detected in prenatal course, as there was no follow-up of these pregnancies. Results The mean postnatal hospitalizing age was 6.1 ± 5.2 days after birth. The mean total bilirubin level and the mean hemoglobin value on hospitalization were 343.7 ± 63.3 µmol/L (=20.1 ± 3.7 mg/dL) and 14.9 ± 3.4 g/dL, respectively. Of 106 patients identified with irregular subgroup incompatibility, 40 infants (37.7%) were associated with C, 22 (20.8%) with c, 30 (28.3%) with E, 9 (8.5%) with e, and 5 (4.7%) with Kell subgroup system. Positive Coombs tests (either direct and/or indirect) occurred in 28.3% of the study cases. Hydrops fetalis was determined in 5 of 106 neonates (4.7%). Twenty-two of 106 (20.8%) patients required total exchange transfusion. Positive Coombs test in cases required total exchange transfusion was 63.6%. Conclusion Our data expose the magnitude and spectrum of the potential developing severe hemolytic disease and immune hydrops due to irregular subgroup incompatibility. Minor group antibody screening is recommended both in the mother and the high-risk infants with hyperbilirubinemia and hemolytic disease of the newborn.

Double outlet left ventricle is very rare congenital cardiac anomaly with numerous anatomical subtypes. Here, we presented and discussed a 2-day-old male baby with aorta and pulmonary artery arising from the left ventricle. Congenital heart disease anomalies observed in our patient included a well-developed pulmonary artery being posterior and to the left of the aorta, the aorta being anterior and to the right of the pulmonary artery (D-transposition of the great vessels), ostium secundum atrial septal defect, tricuspid artesia, severe hypoplastic right ventricle, subaortic ventricular septal defect, increased pulmonary artery blood flow with normal pulmonary artery pressure and mitral-pulmonary fibrous continuity.

Crisponi syndrome is an infrequently described disorder with autosomal recessive trait. It is characterized by extensive muscular contractions in the face after even minimal stimuli or crying, hypertonia, opisthotonus, camptodactyly, and typical facial features. Muscle contractions attenuate during rest or when the infant calms down. As a recently described new disease, Crisponi syndrome may be confused with epileptic manifestations. Most of the patients die in the first months of life due to hyperthermia and feeding problems. Recently, it has been demonstrated that mutations of the CRLF1 gene ‘cytokine receptor-like factor 1’ are associated with Crisponi syndrome. Here, we present a newborn diagnosed with Crisponi syndrome and report a novel homozygous CFRL1 gene mutation.

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Their study included 67 term newborns with perinatal asphyxia who were randomized into three groups: supportive treatment; high-dose phenobarbital; or erythropoietin. In their study, longterm neurologic follow up results were better in the phenobarbital group than the erythropoietin group with respect to motor and cognitive functions at 3 and 6 months, and expressive language skills. At 18 months of age, however, the differences between these two groups were insignificant. Therefore, they suggested that high-dose phenobarbital or erythropoietin along with supportive treatment has a positive influence on the outcome of newborns with perinatal asphyxia. 1 There are some controversies, however, about the safety of phenobarbitalevenattheactualdosageusedforneonatalseizures, which is an initial loading dose of 15‐20 mg/kg followed by a maintenance dose of 3‐4 mg/kg per day. 2 It has significant side

To the Editor: We read with great interest the article by Kumar et al. that reported the clinical course, complications and laboratory profile of Hepatitis A in children [1]. Although, they emphasized the hyperendemicity, fatal complications and cost effectiveness of Hepatitis A infection; and importance of improved sanitation and health education for its prevention, the importance of vaccination for preventing and control of Hepatitis A virus infection was not mentioned in their article. We think that vaccination against Hepatitis A would be significant part of planning for the prevention and control of viral hepatitis. Therefore, we want to make some comments on their article. Hepatitis A infection is a widespread disease, accounting for 1.4 million cases annually worldwide. In high endemic areas, the reported incidence of Hepatitis A infection may reach 150 per 100,000 per year [2]. The clinical spectrum of Hepatitis A infection ranges from asymptomatic infection to fulminant hepatitis. Clinical manifestations depend on the age of host: less than 30 % of infected young children are symptomatic, while approximately 80 % of adults manifest severe hepatitis with remarkably elevated serum aminotransferases [3]. Acute liver failure occurs in less than 1 % of cases. Hepatitis A infection is the most common detected cause of fulminant hepatitis among children in our country as well as worldwide [4]. The application of a routine Hepatitis A virus vaccine among children will reduce the potential for the development of severe complications. Hepatitis A incidence has declined 92 % with universal vaccination of young children in United States [5]. Universal Hepatitis Avirus immunization program has begun in Turkey since October 2012. Nearly 100 % of people develop protective levels of antibodies to the virus within one month after a single dose of the vaccine. Even thougha single dose of the vaccine has protectiveeffects; still, manufacturers recommend two vaccine doses to ensure a longer-term protection of about 5 to 8 y after vaccination. Hepatitis A infection is an important public health problem indevelopingcounties.Vaccinationagainst hepatitisAvirusis necessary for children older than one year. We believe that in addition to improved sanitation and food safety, the universal Hepatitis Avirus immunization programs are the most important means in effort to reduce Hepatitis A virus infection and related severe complications.

Is the use of prophylactic theophylline safe for the prevention of severe renal dysfunction in term and post-term neonates with perinatal asphyxia?

Introduction: Respiratory syncytial virus (RSV) is a major cause of pneumonia in infants worldwide. We aimed to evaluate the clinical course of community-acquired RSV pneumonia in newborns hospitalized in neonatal intensive care unit. Materials and Methods: All the newborns diagnosed as pneumonia were prospectively evaluated for RSV infection between November 2010 and April 2011. Fifty-four specimens of nasopharyngeal secretions were tested in parallel with the RAT and the multiplex real time polymerase chain reaction (RT- PCR). Downes’ score was used to assess the disease severity in patients with pneumonia. Results: RAT has a sensitivity of 90% and a specificity of 78.5%, as the PCR technique target assay. Four of the patients with RSV pneumonia had secundum atrial septal defect (ASD) and all of four needed mechanical ventilation support. The first day Downes’ score was positively correlated with time of intravenous fluid requirement (p= 0.001, r= 0.48), total oxygen need (p= 0.000, r= 0.63), and re-enteral feeding (p= 0.001, r= 0.46). Blood pH (p= 0.031, r= 0.46) were negatively correlated with Downes’ score. The second day Downes’ score was higher in patients with ASD than those of without ASD (3.8 ± 2.6 vs. 2 ± 1.1, p= 0.01). The most possible risk factor for longer hospital stay was the higher second day Downes’ score (p= 0.02 OR: 1.9, CI 95% (1.1-3.2). All infants were discharged from hospital in a good health. Conclusion: RAT is sensitive and specific in detecting RSV infections in newborns. Physicians may use Downes’ score for evaluation of disease severity in infants with RSV pneumonia. In these patients, ASD has increased the disease severity.

Background The aim of this study was to evaluate the features and outcome of classic galactosemia diagnosed in the neonatal period. Methods A retrospective study was carried out on 22 newborns with classic galactosemia who were followed-up in a tertiary neonatal intensive care unit from January 2005 to January 2011. Results During the study period, 22 (18 boys, 4 girls) newborns were diagnosed with classic galactosemia. The median gestational age was 38 weeks (31 - 42) with a median age of 13 (3 - 23) days on admission. Major presenting symptoms were hepatomegaly (n = 22, 100%), jaundice [n = 19, 86%; including (n = 14, 63%) indirect and (n = 8, 36%) direct hyperbilirubinemia], vomiting (n = 17, 77%), and nuclear cataract (n = 15, 68%). Liver dysfunction (n = 22, 100%), Escherichia coli sepsis (n = 10), purpura fulminans (n = 1), hemophagocytosis (n = 1), and long QT syndrome (n = 1) were also noted. Cataract resolved in 11 (73%) patients with galactose-restricted diet in the first months. Four patients were operated for cataracts. Neurodevelopmental evaluation showed mild psychomotor retardation in one patient, learning disabilities in five, and developmental delay in three. None died from galactosemia or its complications. Patients who were diagnosed before 17 days did not require cataract operation. Conclusions Early diagnosis of galactosemia and treatment with a galactose-restricted diet could partially prevent and recover complications of the disease, but not all of them. Cataracts can develop even in the first few weeks of life. Early diagnosis seems important in the prevention of severe cataracts. Therefore, newborn screening for galactosemia should improve morbidity.

We aimed to determine reference values of perfusion index (PI) in healthy newborns during the early neonatal period. Preductal (right hand) and postductal (foot) PI values were assessed during the first 5 days of life by using a new generation pulse oximetry. A total of 241 newborn infants (196 [81.3 %] term and 45 [18.7 %] preterm) were enrolled to the study. On the first day, in term infants, the median (interquartile range [IQR]) preductal and postductal PI were 1.35 (1.02–1.91) and 0.88 (0.62–1.22), respectively (p = 0.001). These values were 0.88 (0.60–1.26) and 0.61 (0.35–0.92) in preterm infants, with the same respect (p = 0.001). From the first to third days, preductal PI remained significantly higher than the postductal PI (p

Congenital anomalies are one of the important reasons of mortality and morbidity in newborns. The aim of this study is to determine the incidence, distribution and the mortality of the congenital anomalies in a single neonatal intensive care unit (NICU) from Turkey.A retrospective analysis was performed between 2005 and 2012 in NICU using a computerized database. Variables including the type of anomaly, antenatal and postnatal history, gestational age, birth weight, consanguinity and other demographic, clinical and related laboratory variables were extracted from the computerized database using ICD-10 codes. Congenital anomalies were classified according to involved organ systems and also classified as single and multiple anomalies.A total of 1024 newborns with congenital anomaly (CA) (13.7%) were identified among the 7450 hospitalized newborns in NICU. The most affected system was the cardiovascular system (68.8%). Most of the anomalies (67.1%) were single anomalies. Of all, 59.4% had single major, 7.7% had single minor, 9% had single major plus single minor, 18.4% had multiple major and 2% had multiple minor anomalies. On the other hand, 96.3, 1.9, 0.1 and 1.7% of the newborns had malformation, deformation, disruption and dysplasia, respectively. Chromosomal analysis was only performed 24.8% of the newborns with CA and among them, 65.3% of these were in normal limits. The most frequently detected chromosomal abnormality was trisomy 21. Overall, mortality rate was 15.5% among the newborns with CA.In conclusion, the most common and mortal CA was cardio-vascular malformations in our hospital. The overall prevalence of cardio-vascular malformations among the newborn was higher than previously reported studies in Turkey. Further, studies with larger sample size are needed to determine CA in Turkey.

Crisponi syndrome is an infrequently described disorder with autosomal recessive trait. It is characterized by extensive muscular contractions in the face after even minimal stimuli or crying, hypertonia, opisthotonus, camptodactyly, and typical facial features. Muscle contractions attenuate during rest or when the infant calms down. As a recently described new disease, Crisponi syndrome may be confused with epileptic manifestations. Most of the patients die in the first months of life due to hyperthermia and feeding problems. Recently, it has been demonstrated that mutations of the CRLF1 gene 'cytokine receptor-like factor 1' are associated with Crisponi syndrome. Here, we present a newborn diagnosed with Crisponi syndrome and report a novel homozygous CFRL1 gene mutation.

To evaluate the role of some antenatal factors on bone status of newborn infants.Tibial bone speed of sound (SOS) value was measured by quantitative ultrasound within 5 days after birth in 205 neonates whose gestational ages ranged between 31 and 40 weeks. The cohort was divided into two groups according to intrauterine growth curves, as small- (SGA) and appropriate-for-gestational age (AGA). All cases were also been evaluated by antenatal ultrasonography for oligohydroamniosis.The mean SOS value was found significantly higher in SGA (n = 43) than AGA infants (n = 162) (p0.001). The mean tibial SOS value of infants with a history of oligohydroamniosis (n = 28) was also higher than those without oligohydroamniosis (n = 177) (p0.001). But, in SGA infants with a history of oligohydroamniosis (n = 17), the mean tibial SOS value was similar to those SGA infants without oligohydroamniosis (n = 26) (p0.05). Infants whose mothers had smoked during pregnancy (n = 18) had significantly higher tibial SOS values compared to those of whose mothers had not (n = 187) (p = 0.006). In addition, mean tibial SOS values were determined higher in male infants (n = 116) compared to female infants (n = 89) (p = 0.036). There was a significant correlation between tibial SOS values and gestational age (r = 0.178, p = 0.011).While creating reference curves of SOS values at birth, smallness for gestational age, maternal smoking and gender as well as gestational age should be taken into account.

Neonatal gastric perforation is an uncommon but life-threatening condition, which is mainly encountered in premature infants. Primary surgical repair is the principal mode of the treatment. Gastric perforation in neonates improving with percutaneous peritoneal drainage alone has not been described previously. Therefore, an extremely low birth weight infant is presented herein in order to emphasize that gastric perforation may improve with percutaneous peritoneal drainage alone. Isolated gastric perforations in newborn infants may be improved with percutaneous peritoneal drainage alone without need for primary surgical repair.

Hypocalcemia is a rare condition that causes dilated cardiomyopathy and can result in heart failure. Patients with hypocalcemia have been reported to recover in 3 to 12 months after calcium and vitamin D replacement therapy as well as treatment of heart failure. A 6-month-old male patient who presented with dyspnea was admitted to the intensive care unit with severe heart failure and dilated cardiomyopathy. Blood biochemistry revealed hypocalcemia and vitamin D deficiency. After administration of anticongestive treatment, positive inotropic support, as well as vitamin D and calcium supplementation, cardiac function returned to normal in a week. Our case is the first report of such a rapid improvement in cardiac morphology and function in a patient with hypocalcemic dilated cardiomyopathy and heart failure.

There are few reports on pandemic swine influenza A (H1N1) virus infection in very young infants. We aimed to discuss the clinical characteristics and management of the H1N1 influenza infection in very young infants. Clinical characteristics of ten infants diagnosed with H1N1 influenza virus infection during the 2009 outbreak season in a tertiary neonatal intensive care unit were evaluated. The diagnosis was confirmed by testing of respiratory samples with pandemic H1N1 influenza specific real-time PCR assay. Of the 46 patients with fever or respiratory problems, ten (22%) were diagnosed with H1N1 influenza virus infection during the 2009 (October–December) peak outbreak season. All infants including the preterms were admitted from home, seven (70%) were full-term and three (30%) were preterm. Median age of the patients at admission was 24.5 days. Fever and cough were the most common symptoms. Apnea was the initial symptoms in three patients. Two patients required oxygen support, one of which, a preterm baby, had been mechanically ventilated for 2 days. Mean duration of hospitalization was 7.8 ± 4.9 days. Chest radiography revealed radio-opacities on both lung fields in six patients. In addition, two patients had co-infection. All patients with proven infection were given oseltamivir medication. Recovery was achieved in all patients with no residual deficits or side effects from the antiviral oseltamivir treatment. The H1N1 influenza virus infection in very young infants appears to be mild to moderate in severity. The outcomes of the infants may be influenced by antiviral therapy. Treatment with antiviral oseltamivir appears to have no major adverse effects.

Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. To our knowledge, it had not been diagnosed in a premature newborn before the case we report have.A female baby weighing 1164 grams, who was born prematurely at the 29th week of gestation by Cesarean section was referred to our newborn intensive care unit due to prematurity and respiratory distress with no prenatal pathological findings. Physical examination revealed tachypnea and hepatosplenomegaly. Routine laboratory measurements showed significant leukocytosis (85,000/mm3) and anemia (Hb: 9.6 g/dL and Hct: 27.6%). The platelet count was normal. The peripheral blood smear suggested leukoerythroblastosis with the presence of nucleated erythrocytes, monocytosis, and 4% blasts. Bone marrow cytogenetic examination was normal. Parvovirus B19 Ig G and M serology were detected to be positive. The etiological factors observed in leukoerythroblastosis occurring during neonatal and early childhood period are congenital-postnatal viral infections, juvenile myelomonocytic leukemia and osteopetrosis. To our knowledge, no case of leukoerythroblastosis in such an early phase has been reported in the in literature. As a result, premature delivery and leukoerythroblastosis were thought to have developed secondary to intrauterine parvovirus B19 infection. Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. It is reported that it can be observed following hematologic malignancies especially juvenile myelomonocytic leukemia, acute infections, hemolytic anemia, osteopetrosis, myelofibrosis, neuroblastoma and taking certain medicines. To our knowledge, it has not been diagnosed in a premature newborn before. Here we the case of a newborn who was referred to our intensive care unit due to being born prematurely at the 29th week of gestation and diagnosed with leukoerythroblastosis.

We read with great interest the paper by Austin et al (1) that evaluated the impact of in utero exposure to antidepressant drugs on infants' neurodevelopment. Using the Bayley Scales of Infant Development (BSID-III) to assess neurodevelopmental at 18-months-of-age, they reported that in utero exposure to antidepressants - mostly serotonin reuptake inhibitors (SRIs) - had no impact on cognitive, motor, and/or language development outcomes. This article is protected by copyright. All rights reserved. Language: en