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Decision making to transfer a late preterm or term neonate with the diagnosis of transient tachypnea of the newborn (TTN) to an intensive care unit for respiratory support is a challenge for caregivers in level one and two NICUs. The aim of this study was to identify "practical bedside clinical clues" that may help to predict the severity of disease and need for respiratory support in patients with the diagnosis of TTN. Newborns having the diagnosis of TTN were classified into two groups according to the intensity of the respiratory support. Infants receiving only supplemental oxygen and infants requiring nasal continuous positive airway pressure or mechanical ventilation constituted group 1 (mild) and group 2 (severe), respectively. Demographic, clinical and laboratory characteristics were compared between the two groups. Patients in group 2 had lower gestational age, higher Silverman and Richardson scores, longer mean duration of oxygen support and hospitalization. A positive correlation was found between subcostal and xiphoid retractions, asynchrony in chest-abdomen movements, arterial pH < 7.30, ratio of PaO < sub > 2 < /sub > / % inspired O < sub > 2 < /sub > < 1.2 and need of respiratory support (p < 0.05). We suggest that simple scores can help physicians to get a good sense of a given baby's likelihood of deterioration.

Percutaneous treatment of supravalvular aortic stenosis (SVAS) by means of balloon dilation and stent deployment has been rarely reported in the literature. In this report, we present the case of a patient with mycotic aneurysms, disseminated peripheral and cerebral septic embolizations, and infected vegetations associated with a stent that had previously been deployed to treat restenosis of surgically corrected SVAS in the infancy., (© The Author(s) 2015.)

GRACILE Syndrome, is an autosomal recessive disease presenting with growth retardation, severe lactic acidosis, Fanconi type tubulopathy, cholestasis, iron overload and early death without any dysmorphological or neurological features. The BCSIL gene mutation is responsible for GRACILE syndrome, Bjornstad syndrome and complex III deficiency. Bjomstad syndrome is characterized by sensorineural hearing loss and abnormal flat twisted hair shafts. The case is GRACILE syndrome with Bjomstad phenotype in neonatal period due to BCSL1 gene mutation.

Our aim is to present the etiology and risk factors for cerebral sinovenous thrombosis (CSVT) and the radiological findings, anticoagulant therapy used, and treatment outcome of patients with CSVT. This study included 12 patients who were treated for CSVT at the Ankara University, School of Medicine, Department of Pediatric Neurology. This study included 5 girls (41.7%) and 7 boys (58.3%) with a mean age of symptom onset of 5.2 ± 6.29 years (range: 0-18 years), who were followed at our institution for a mean of 1.8 ± 1.73 years (range: 0-6.5 years). Among the patients, 3 had no risk factors, 2 had 1 risk factor, and 7 had multiple risk factors. Anticoagulant therapy was administered to 4 patients, of which 1 had neurological sequelae; neurological sequelae or exitus occurred in 4 of the 8 patients who did not receive anticoagulant therapy. The present findings showed that appropriate prophylaxis in appropriately selected patients reduced the rate of recurrence of CSVT., (© The Author(s) 2014.)

Despite recent advances in neonatal and perinatal medicine, extremely low birth weight infants (ELBW) are at high risk of developing anaemia of prematurity (AOP) requiring packed red blood cell (RBC) transfusions. The benefit of transfusing allogenic RBCs for AOP is a controversial issue, except for disturbances in tissue oxygenation. Although the role of erythropoietin (EPO) in the pathophysiology of AOP is well known, neither early nor late recombinant human EPO therapy alters the number or volume of RBC transfusions. It is also known that one-half of the feto-placental blood volume remains outside the newborn infant's circulation at 30 weeks of gestation if the umbilical cord is clamped immediately. Delayed cord clamping (DCC) and umbilical cord milking (UCM) are the main methods for enhancing placental transfusion. The basic principle of these approaches depends on providing high haemoglobin (Hb) levels to premature infants in the delivery room. The enhancement of placental transfusion clearly results in higher Hb levels at birth, reducing the need for RBC transfusions as well as creating a better haemodynamic status during the initial hours of life. To date, enhancement of placental transfusion in the delivery room by either DCC or UCM seems to be the best preventive measure for AOP. Yet, studies on the associated neurodevelopmental outcomes are insufficient to reach a conclusion. This review summarizes the pathophysiology, treatment and preventative strategies of anaemia of prematurity in light of the current literature.

Total anomalous pulmonary venous return (TAPVR) is a rare and frequently isolated defect identified in 1% to 3% of all congenital heart diseases. To the best of our knowledge, portal vein thrombosis (PVT) associated with TAPVR has not been reported in the literature. We report a successfully managed PVT in a newborn with infracardiac-type TAPVR and review the literature. Anticoagulation therapies were used during the neonatal period to prevent thrombus progression. PVT should be kept in mind in TAPVR patients who have open heart repair with total correction. The treatment in each neonate should be individualized with consideration of the risk/benefit ratio.

Background: Soluble urokinase plasminogen activator receptor (suPAR) has been studied in a variety of diseases. The aim of the study is to investigate the levels of suPAR in neonates with sepsis., Methods: The infants enrolled to this prospective study were classified into four groups. Group 1, 2, and 3 were referred as the patient groups (40 infants), and group 4 was referred as control group (26 infants). Blood samples for whole blood count, C-reactive protein (CRP), suPAR and blood culture were obtained before initiating antimicrobial therapy, and two further samples were obtained on day 3 and at the end of the treatment for CRP and suPAR., Results: The mean gestational ages of patient and control groups was similar. The median level of initial suPAR was 18.8 ng/mL (range 6.8-30.1 ng/mL) in the patient groups, and 6.0 ng/mL (range 3.7-10.8 ng/mL) in the control group (P < 0.001). A significant decrease in suPAR level was observed from the inclusion to the third day and end of the treatment (P < 0.001). The area under the curve (AUC) for suPAR is 0.959 (95% Cl: 0.919-0.999) and for CRP is 0.782 (95% Cl: 0.669-0.895). At a cut-off value of 11.3 ng/mL for suPAR the specificity was 100%, and the sensitivity was 82.5%. There was a positive correlation between laboratory values of CRP and suPAR (r: 0.359, P = 0.003)., Conclusion: This is the first study that investigated the levels of suPAR in neonates and our results demonstrate that suPAR is a powerful marker of inflammation in infants with sepsis., (© 2014 Wiley Periodicals, Inc.)

Objective: To investigate the association of perinatal risk factors including delivery mode with mortality in very low birthweight (VLBW) in a tertiary hospital setting., Methods: Medical records of 241 live-born VLBW infants (≤1500 g) were retrospectively reviewed. Details of maternal, obstetrical, perinatal risk factors and their associations with infant mortality were evaluated., Results: The overall infant mortality rate was 23.2%. Mortality was significantly higher for infants born at ≤27 gestational weeks and with a birthweight of ≤750 g (p = 0.000 and p = 0.000, respectively), showing a steep decrease thereafter. On ROC analysis, a cut off of 26.5 weeks was determined for mortality with a sensitivity of 57.1% and a specificity of 90.3% (area under the curve = 0.792, 95% CI: 0.719-0.866). On multivariate regression analysis, gestational week at birth, birthweight, antenatal steroid treatment and pathologic Doppler ultrasound findings were found as independent risk factors for mortality., Conclusions: Gestational week at birth, birthweight and antenatal steroid treatment remain the most important perinatal risk factors for infant mortality in VLBW infants. Mode of delivery does not seem to be associated with mortality when adjusted for other perinatal risk factors.

Background: Genetic predisposition is a suggested risk factor in the etiology of varicose veins. The matrix metalloproteinase (MMP) family degrades extracellular matrix (ECM) and may lead to disturbances in vein wall structure. The activity of MMPs in the ECM are controlled by specific tissue inhibitors of MMPs (TIMP). The present study aimed to investigate the relationship between MMP9 and TIMP2 gene polymorphisms and varicose vein risk., Materials and Methods: Genotyping of the polymorphisms of MMP9 (1562 C/T) and TIMP2 (418G/C) was performed using polymerase chain reaction and restriction-fragment length polymorphism assays in a group of patients with varicose veins (n=63) and healthy controls (n=70)., Results: The frequencies of MMP9 alleles and genotypes did not differ significantly between patient and control groups. However, TIMP2 -418 C allele was associated with increased risk for varicose vein formation (p=0.007). It was also shown that the frequency of the GG genotype was significantly higher in the control group than in the patient group (odds ratio=0.333, 95% confidence interval=0.14-0.78, p=0.012)., Conclusion: TIMP2 -418 C allele is associated with susceptibility for varicose vein formation and individuals with GG genotype may have a lower risk for varicose vein formation., (Copyright © 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.)

Chylothorax is a relatively uncommon condition defined as an abnormal collection of lymphatic fluid within the pleural space. Morbidity of congenital chylothorax (CC) is high, and prognosis is very poor if CC is associated with hydrops fetalis. Although the optimal treatment of CC has not been determined, conservative treatment and surgical intervention are employed. However, there is still little experience with the use of octreotide therapy for this condition, and optimal duration of the treatment for response evaluation is not known. We report a newborn with CC who presented with intrauterine bilateral pleural effusion and was resistant to conservative treatments. Octreotide (6 μg/kg/h) infusion was started on the 10th postnatal day due to ongoing pleural drainage. Although the patient improved rapidly with continuous administration of octreotide, we had to continue the drug for 151 days, even subcutaneously on outpatient follow-up. To the best of our knowledge, this patient is unique in receiving octreotide treatment for such a long time, with a successful outcome and a safe profile.

We aimed to determine whether an early prophylactic INSURE strategy combined with early nasal continuous positive airway pressure (nCPAP) treatment could decrease the subsequent need for mechanical ventilation (MV) compared to the administration of surfactant prophylaxis later, at the 15th minute after birth, combined with early nCPAP. Infants born at <30 weeks' gestation were randomized to receive surfactant prophylaxis immediately or at the 15th minute after birth. All infants received 100 mg/kg poractant alpha, were ventilated with a T-piece resuscitator and were extubated to nCPAP if they had sufficient respiratory drive. Forty infants were analyzed in each group. Ten (25%) infants in the early prophylactic INSURE group, and 13 (32.5%) infants in the later prophylactic INSURE group could not be extubated after surfactant administration in the delivery room. Among the infants who were initially extubated to nCPAP after surfactant administration, 6 in the early prophylactic INSURE group and 4 in the later prophylactic INSURE group needed intubation for MV within the first 3 days of life (20% vs. 16.7%; P=0.73). The duration of total respiratory support (CPAP plus MV) of infants who were intubated within the first 3 days of life was shorter in the early prophylactic INSURE group than in the later prophylactic INSURE group (median: 96 h vs. 309 h; P=0.038). The incidence of all neonatal morbidities and mortality and the duration of hospitalization were similar between the groups. Our study did not demonstrate superiority of early surfactant prophylaxis combined with early nCPAP to the later administration of prophylactic surfactant with early nCPAP; however, it did detect an absolute difference in the primary outcome: need for MV within the first 3 days of life.

Objectives: To describe the efficacy of intravenous colistin on clinical and microbiological outcomes in preterm infants with nosocomial sepsis in neonatal intensive care unit (NICU) and define adverse events observed with this treatment., Methods: The records of preterm infants who received colistin with or without positive cultures in the NICU were retrospectively reviewed. Patients were evaluated for response to therapy and side effects., Results: A total of 21 preterm infants with medians of 28 weeks (23-36) gestational age and 870 g (620-2,650) birth weight were included. The median duration and dose of colistin therapy were 9 days (3-26) and 3 mg/kg/d (2-5). Recovery rate in patients including all with/without positive culture was 81% (17/21). Microbiological clearance by colistin was 69% (9/13). The major side effect observed was acute kidney injury (19%). At least 24% of infants required electrolyte supplementation during the colistin therapy. Magnesium levels were significantly lower at the end of the colistin therapy (p < 0.001). Acute kidney injury and electrolyte disturbances including hypomagnesemia were reversible in all surviving patients., Conclusion: We suggest that renal function tests and serum electrolytes should be monitored closely and replaced in case of any need during the colistin therapy in preterm infants., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Objective: Lactoferrin (LF) is effective in the prevention of sepsis in very low birth weight (VLBW) neonates. T-regulatory cells (Tregs) are important subsets of T lymphocytes that control pathogen-specific immune responses and are essential for intestinal immune homoeostasis. The aim of the present study is to determine whether oral LF at a dosage of 200 mg/d reduces nosocomial sepsis episodes and necrotizing enterocolitis (NEC) in premature infants and to evaluate the possible effects of LF on Treg levels., Study Design: In this prospective, placebo-controlled, double-blind, randomized trial, infants either VLBW or born before 32 weeks were assigned to receive either placebo (n = 25), or 200 mg LF (n = 25) daily throughout hospitalization. Episodes of culture proven nosocomial sepsis and NEC were recorded. The level of FOXP3 + CD4 + CD25hi lymphocytes was studied by flow cytometry at birth and discharge. A third comparison was made with healthy term neonates (n = 16)., Results: Fewer sepsis episodes were observed in LF-treated infants (4.4 vs. 17.3/1,000 patient days, p = 0.007) with none developing NEC, without statistical significance. Treg levels at birth and discharge were similar, while preterm infants showed significantly lower levels than term controls. However, individual increases in Treg levels were higher in the LF group., Conclusion: LF prophylaxis reduced nosocomial sepsis episodes. Treg levels in preterm infants were lower than in term infants and an increase of Treg levels under LF prophylaxis was observed. Increase in Treg levels can be the mechanism for protective effects of LF on nosocomial sepsis., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Objective: The aim of this study was to evaluate the effects of umbilical cord milking (UCM) on the need for packed red blood cell (PRBC) transfusion and hematologic and hemodynamic parameters in very-low-birth-weight infants., Methods: The infants were randomized into 2 groups: group 1 (UCM) and group 2 (control). The primary outcome was the number of PRBC transfusions during the first 35 days of life. The secondary outcome measures were the hemodynamic variables during the first 24 hours of life., Results: A total of 44 infants were included with 22 infants in each group. Two of 21 infants in group 1 and 4 of 21 infants in group 2 received transfusion in the first 3 days of life (P=0.384). The number and volume of PRBC transfusions were similar in both groups. However, the levels of hemoglobin (Hb) at the first and 24th hour of life were significantly higher in group 1. Phlebotomy volume was found as a statistically significant risk factor for the need for PRBC transfusion (P=0.005)., Conclusions: UCM in delivery room results in a higher Hb level in the first day of life. In these groups of infants, phlebotomy losses may impact the transfusion need.

Introduction: Almost 95% of the platelet transfusions (PTs) conducted in the neonatal intensive care unit (NICU) are prophylactic transfusions. Guidelines for prophylactic PTs are based on platelet counts, but not on platelet functions. Nowadays, in order to reduce unnecessary transfusions, utilizing platelet mass index (PMI) was investigated. The aim of study is to find out whether PTs performed in our NICU during last 2 years were in accordance with the current guideline and to evaluate whether the frequency of PTs should be reduced if PMI was considered., Methods: Forty-three infants who received 96 prophylactic PTs were enrolled in the study. The guideline utilized in our NICU advocate keeping the platelet count: (a) >100 000 in pre/post-operative, (b) >50 000 in unstable and (c) >20 000 in stable patients. According to PMI criteria, PT should be performed if PMI: (a) <800 in pre/post-operative, (b)<400 in unstable and (c) <160 in stable patients., Results: In all, 53.2% of PTs should not be given if the decision was in accordance with the current guideline. If decision for every PT was made according to the current guideline and taking PMI into consideration, an additional 11.5% reduction in total PTs could be achived., Conclusion: We suggest that better compliance with the new guidelines which take platelet functions into account may yield lower transfusion rate, lower costs and better conservation of blood bank resources.

Tufting enteropathy is an autosomal recessive congenital enteropathy presenting with early-onset severe intractable diarrhea. It presents with watery diarrhea that develops in the first days after birth and persists despite bowel rest. Growth is impaired, and most patients require total parenteral nutrition. The histological characteristic of tufting enteropathy is the presence of epithelial tufts. We hereby present a patient who was referred to our neonatal intensive care unit because of chronic diarrhea and diagnosed with tufting enteropathy according to histological examination. To the best of our knowledge, the newborn case presented here is the first one reported from Turkey. As TE is a very rare disease, it should be considered in patients with continuing diarrhea beginning in the first days of life.

We present the case of a 58-year-old woman who had large lipomatous hypertrophy of the interventricular septum, a condition that is reported very infrequently. Preoperative cardiac magnetic resonance images revealed an inhomogeneous, infiltrating mass that was suppressed in fat-suppression mode. The extensive mass was causing right ventricular dysfunction, so we excised it through a right ventricular approach. The findings on histologic analysis of the mass were consistent with lipomatous hypertrophy. The patient died of septic shock on the 28th postoperative day. In addition to the patient's case, we discuss the characteristics and diagnosis of this rare entity.

In this paper, we report an alternative approach for implantation of ventricular pacing lead for complete atrioventricular block after tricuspid valve replacement., (© 2014 Wiley Periodicals, Inc.)

Introduction: Congenital analbuminemia is a rare autosomal recessive disorder manifested by the presence of a very low amount of circulating serum albumin. It is an allelic heterogeneous defect, caused by variety of mutations within the albumin gene in homozygous or compound heterozygous state. Herein we report the clinical and molecular characterization of a new case of congenital analbuminemia diagnosed in a female newborn of consanguineous (first degree cousins) parents from Ankara, Turkey, who presented with a low albumin concentration (< 8 g/L) and severe clinical symptoms., Materials and Methods: The albumin gene of the index case was screened by single-strand conformation polymorphism, heteroduplex analysis, and direct DNA sequencing. The effect of the splicing mutation was evaluated by examining the cDNA obtained by reverse transcriptase - polymerase chain reaction (RT-PCR) from the albumin mRNA extracted from proband's leukocytes., Results: DNA sequencing revealed that the proband is homozygous, and both parents are heterozygous, for a novel G>A transition at position c.1652+1, the first base of intron 12, which inactivates the strongly conserved GT dinucleotide at the 5' splice site consensus sequence of this intron. The splicing defect results in the complete skipping of the preceding exon (exon 12) and in a frame-shift within exon 13 with a premature stop codon after the translation of three mutant amino acid residues., Conclusions: Our results confirm the clinical diagnosis of congenital analbuminemia in the proband and the inheritance of the trait and contribute to shed light on the molecular genetics of analbuminemia.

Objective: The objective of this study was to find out the percentage of preterm infants that needed treatment for patent ductus arteriosus (PDA), when treatment decision was based on clinical signs and symptoms, besides echocardiographic findings., Methods: Daily echocardiographic evaluation was conducted in 39 preterms ≤29(6/7) weeks' gestation. Patients with ductus arteriosus were closely followed-up for clinical symptoms of PDA for treatment decision until ductus arteriosus was closed either spontaneously or by treatment., Results: PDA was found in 25 (64%) infants. Mean gestational age and birth weight (BW) of the patients with PDA were 27.8 ± 1.2 and 998 ± 221 g, respectively. PDA closed spontaneously or had minimal ductal shunting before any signs and symptoms attributable to PDA were observed in 16 (41%) infants. Mean ductus size/BW ratio and mean left atrial/aortic root ratio were significantly higher in 9 (23%) symptomatic patients (2.06 ± 0.75 versus 1.32 ± 0.75 mm, p = 0.012 and 1.31 ± 0.52 versus 1.19 ± 0.2 mm, p = 0.043, respectively). PDA closure was observed after the first dose of ibuprofen in six of nine patients., Conclusion: Correlation of clinical signs with echocardiographic findings for the decision of PDA treatment can be appropriate to prevent unnecessary medical treatments.

Objective: We assessed the effect of human milk (HM) fortification with extra protein supplement by an adjustable protein fortification method according to the weekly blood urea nitrogen (BUN) levels on growth in hospitalized preterm infants., Method: A prospective observational intervention study in 58 preterms born ≤32 weeks of gestation and fed with breast milk was conducted. Preterms who were given a commercial HM fortifier which provides an additional protein of 0.8 g/3 scales according to the standard feeding strategy served as a historical control group. Infants who were given extra protein in addition to the HM fortifier with another commercial protein supplement which provides an additional protein of 2.2g/1 scale comprised the intervention group. Additional protein supplementation was adjusted according to BUN levels weekly in the intervention group. Weight gain velocities (g/kg/day), length, head circumferences (HC) gain velocities (mm/day) and daily growth indexes for weight, height and HC (percentage per day) were calculated., Results: The median amount of daily enteral protein intake [4 (3.4-4.6) vs. 2.78 (2.1-3.1) g/kg/day, p < 0.0001] was significantly higher in the interventional group. Length (p = 0.008) and HC (p < 0.0001) gain velocities were significantly higher in the intervention group. Daily growth indexes for weight (2.2% vs. 1.8%, p = 0.026), for length (0.4% vs. 0.3%, p = 0.027) and for HC (0.48% vs. 0.36% per day, p = 0.003) were significantly higher in the intervention group., Conclusion: A higher protein intake by adjustable protein fortification method without energy or volume change leads to improved postnatal in-hospital-growth in very low birth weight infants., (© 2013 Elsevier Ireland Ltd. All rights reserved.)

Prostaglandin E1 (PGE1) is the drug of choice for providing ductal patency in cyanotic congenital heart disease (CCHD) for a short period of time until essential surgical management. Occasionally, prolonged use of PGE1 is required when the surgical procedure is delayed due to certain clinical conditions. Prolonged use of PGE1 may lead to bone and tissue changes such as pretibial and soft tissue swelling of the extremities and reversible cortical proliferation of the bones. Venous stasis as an additional risk factor can result in generalized periosteal reaction that initially can cause the separation of the periosteum from the cortex. We report an infant with CCHD who developed severe tissue swelling and generalized periosteal reaction due to coexistence of prolonged use of PGE1 and venous stasis. To the best of our knowledge, this is the first case report with both of these risk factors.

The topsy-turvy heart is characterized by a global 90°clockwise rotation around the heart's long axis. This rotation displaces all basal great arteries inferiorly and posteriorly, resulting in elongation and stretching of the brachiocephalic arteries and the bronchi. To date, reports of only four living cases have been published in the literature. We report here three new cases, with additional aortopulmonary window defects, and present their morphological details, clinical presentations, and our management.

Objective: Failure of adequate and timely clearance of fetal lung fluid has been implicated in transient tachypnea of the newborn (TTN). There has been lack of human data on the association between endocrinological adaptation and fetal lung fluid clearance. Although TTN development in term or late preterm newborns delivered by cesarean section (CS) is well known, whether stress hormones levels at birth contribute to it or not is not known. The aim of the study was to assess the possible association between low adrenocorticothrophic hormone (ACTH), cortisol and free triiodothyronin (fT3) levels at birth and TTN in late preterm and term infants., Study Design: We compared cord blood concentrations of epinephrine, cortisol, ACTH, fT4, fT3 and thyroid stimulating hormone in two groups of term and late pretrem infants born by CS: those who developed TTN and a comparison group without respiratory distress., Results: While there were no significant demographic differences between patient and control groups, cord ACTH, cortisol and fT3 were significantly lower and epinephrine was higher in infants developing TTN (p < 0.05)., Conclusions: Lower cord levels of cortisol, ACTH and fT3 in patients with TTN may indicate the possible relation of these hormones in fetal lung fluid clearance and postnatal pulmonary adaptation through their modulatory effect on epithelial sodium channel and Na-K-ATPase.

Streptococcus pneumoniae infections which are relatively unusual in the neonates, are associated with substantial morbidity and mortality. Although S.pneumoniae has more than 90 serotypes, invasive disease is more common with certain serotypes. Pneumococcal polysaccharide and conjugate vaccines are developed against the serotypes that cause invasive disease. Until now invasive pneumococcal diseases have been defined due to various serotypes in the neonatal period. Here we present a neonate with an invasive pneumococcal disease due to S.pneumoniae serotype 7F which has not yet been reported in the literature. A 3180 g infant was born to a healthy 31 year old primigravida by normal vaginal delivery at 39 weeks gestation. Since the infant developed uneasiness, decreased sucking reflex and respiratory difficulty at postnatal 10th hour, he was admitted to the neonatal intensive care unit. Upon admission to intensive care unit mechanical ventilation, fluid replacement and empirical penicillin G and netilmycin had been initiated since the patient had hypotension, hypothermia and tachycardia. The infant later developed severe metabolic acidosis and had anemia, leukocytosis and high CRP values. CSF culture revealed no bacterial growth, however, S.pneumoniae was grown in the blood culture. The isolate was identified as serotype 7F by capsule swelling reaction. The vaginal specimen taken from the mother for the determination of the infectious foci revealed no pathogen growth. The patient's general condition improved after the second day of supportive treatment and antibiotherapy. Since the isolate was penicillin susceptible, the treatment continued with penicillin G and netilmycin for 14 days and the case was discharged with cure. This case was presented to emphasize that S.pneumoniae serotype 7F might lead to invasive disease not only during childhood but also during early neonatal period.

Acquired Bartter-like syndrome (BLS), characterized by hypokalemic metabolic alkalosis, hypomagnesemia, hypocalcemia, and normal kidney function, can be induced by diuretics or antibiotics. It is a very rare condition and only anecdotal cases mostly in adults were reported. Although tubulopathy associated with colistin was reported in adults, to the best of our knowledge, colistin-associated BLS neither in adults nor in children has been reported in the literature. We here report a-28-week, 740 g female preterm infant who developed BLS just after colistin treatment for Acinetobacter baumannii infection and recovered few days after the drug cessation, and discuss the possible association of colistin and tubulopathy. More research on colistin pharmacokinetics and pharmacodynamics in critically ill patients and preterm infants is needed to guide adequate colistin dosing at the least toxicity.

Crigler-Najjar syndrome (CNS), caused by deficiency of bilirubin uridine diphosphate glucuronosyltransferase (UGT) 1A1, is a rare and autosomal recessive inherited disorder characterized by severe unconjugated nonhemolytic hyperbilirubinemia since birth. We present a girl with CNS type I caused by a novel mutation and Gilbert type genetic defect. Gilbert's Syndrome (GS) and CNS type I both involve abnormalities in bilirubin conjugation secondary to deficiency of bilirubin UGT. The combined defects even in benign genetic forms were shown to cause more serious clinical disease. The patient has been treated with daily home-based phototherapy for more than nine months and considered as a candidate for liver transplantation.

Background: Innovative cardiopulmonary bypass (CPB) settings have been developed in order to integrate the concepts of "surface-coating," "blood-filtration," and "miniaturization.", Objectives: To compare integrated and nonintegrated arterial line filters in terms of peri- and postoperative clinical variables, inflammatory response, and transfusion needs., Material and Methods: Thirty-six patients who underwent coronary bypass surgery were randomized into integrated (Group In) and nonintegrated arterial line filter (Group NIn) groups. Arterial blood samples for the assessments of complete hemogram, biochemical screening, interleukin-6, interleukin-2R, and C-reactive protein were analyzed before and after surgery. Need for postoperative dialysis, inotropic therapy and transfusion, in addition to extubation time, total amount of drainage (mL), length of intensive care unit, and hospital stay, and mortality rates was also recorded for each patient., Results: Prime volume was significantly higher and mean intraoperative hematocrit value was lower in Group NIn, but need for erythrocyte transfusion was significantly higher in Group NIn. C-reactive protein values did not differ significantly except for postoperative second day's results, which were found significantly lower in Group In than in Group NIn., Conclusion: Intraoperative hematocrit levels were higher and need for postoperative erythrocyte transfusion was decreased in Group In.

Objectives: Obesity is a risk factor for morbidity after isolated coronary artery bypass grafting. This study aimed to analyze the sole effect of body mass index (BMI) on early morbidity and mortality in patients after isolated coronary artery bypass grafting., Design: This study was retrospective and used an electronic database of anesthesia information management., Setting: A single community hospital., Participants: The data of 803 consecutive patients after isolated on-pump coronary artery bypass grafting were analyzed retrospectively; off-pump cases were excluded., Intervention: According to measured BMI, patients were divided into 5 groups: underweight (BMI <20 kg/m(2)), normal weight (BMI 20.0-24.9 kg/m(2)), overweight (BMI 25.0-29.9 kg/m(2)), obese (BMI 30.0-34.9 kg/m(2)), and morbidly obese (BMI >34.9 kg/m(2)). Early postoperative morbidity and mortality were evaluated using logistic regression analysis., Measurements and Main Results: Early cumulative postoperative mortality was 3.9% (32 of 803 patients). Mortality was recorded in 3 underweight (n = 15, 20%, odds ratio [OR] 6.54, p = 0.001), 9 normal-weight (n = 159, 5.7%, OR 1.62, p = 0.228), 12 overweight (n = 371, 3.2%, OR 0.68, p = 0.314), 6 obese (n = 199, 3.0%, OR 0.69, p = 0.421), and 2 morbidly obese (n = 59, 3.4%, OR 0.83, p = 0.808) patients. Prolonged intensive care unit stay (p < 0.001), prolonged hospital stay (p < 0.001), and mortality (p = 0.01) were significantly more common in patients in the underweight group than in the other groups. Univariate and multivariate logistic regression analyses showed that underweight, hypertension, and chronic renal failure were independent risk factors for mortality., Conclusions: Underweight patients with a BMI <20 kg/m(2) are at increased risk of postoperative complications and mortality compared with normal-weight or overweight subjects., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: This study aimed to compare the storage properties of red blood cell (RBC) concentrates of umbilical cordblood (UCB) and adult donor blood (ADB), and to evaluate the feasibility of UCB-RBC concentrate as an autologoussource for blood transfusion in very low birth weight (VLBW) preterm neonates., Material and Methods: In all, 30 newborn (10 preterm, 20 full term) UCB and 31 ADB units were collected.RBC concentrates were stored and compared with regard to pH, potassium (K(+)), 2,3-biphosphoglycerate (2-3-BPG),adenosine tri-phosphate (ATP), plasma Hb, and bacterial contamination on d 1, 21, and 35 of storage., Results: The K(+) level increased with time and differed significantly between storage d 1 and 21, and between storaged 1 and 35 in both the UCB and ADB units. Initial and d 21 K(+) levels were higher in the UCB units than in the ADBunits. The 2,3-BPG level did not differ significantly between the UCB-PRC and ADB-PRC samples. After 35 d of storageboth UCB-PRC and ADB-PRC samples exhibited significant differences from the initial free Hb, intracellular ATP, andpH values. Significant differences in intracellular ATP and pH were also observed between the UCB-PRC and ADB-PRCsamples., Conclusion: The volume of harvested and prepared UCB-PRC can be used for some of the blood transfusions requiredduring the neonatal period and thus may decrease the number of allogeneic transfusions, especially in preterm newborns.The hematological and biochemical changes that occurred in UCB during storage were comparable with those observedin ADB, and do not pose a risk to the immature metabolism of neonates. UCB-RPC prepared and stored under standardconditions can be a safe alternative RBC source for transfusions in VLBW newborns.

Purpose: Protective effect of pulsatile flow cardiopulmonary bypass (CPB) on the occurrence of acute renal injury is still a matter of debate. The objective of this study was to compare the effects of pulsatile and non-pulsatile cardiopulmonary bypass on kidneys using Urinary neutrophil gelatinase-associated lipocalin (NGAL) and interleukin-18 (IL-18) as the markers of renal injury., Methods: 85 consecutive patients with normal preoperative renal function were prospectively enrolled in the study. Pulsatile perfusion (Group P) and non-pulsatile perfusion (Group NP) was used in 42 and 43 of the patients, respectively, during aortic cross-clamping period. NGAL and IL-18 were analyzed using ELISA in urine samples obtained preoperatively, and at 2, 12, and 24 h after CPB., Results: There was no significant difference between the groups in terms of perioperative renal function tests. IL-18 levels measured at 12 h after CPB were significantly lower in Group P, compared to Group NP (p<0.05). Urinary NGAL levels measured at 2 and 12 h were higher in Group NP; however, the difference was insignificant. In the subgroup of patients with a cross clamp time ≥45 minutes (pulsatile CPB, group P1, n = 33; non-pulsatile CPB, group NP1, n = 33), IL-18 levels measured at 12 hours after CPB were significantly lower in Group P1. Urinary NGAL concentrations measured at 2 and 12 hours in Group P1 were also significantly lower than that in Group NP1 (p = 0.048 and 0.043, respectively)., Conclusions: Low IL-18 and NGAL levels found in the pulsatile perfusion group might suggest the use of pulsatile flow resulted in better kidney protection.

Objective: To evaluate whether the mode of delivery (vaginal versus C-section) influences the levels of CD4+CD25+FOXP3+ Treg cells in cord blood and maternal peripheral blood and also to examine its relationship with plasma cortisol levels., Methods: Newborns either born vaginally (n = 19) or via elective C- section (n = 20) and their mothers, as well as 20 healthy but not pregnant women, were included in the study. CD4+CD25+FOXP3 (Treg) cells were examined by flow cytometry. Total lymphocyte counts (TLC) and serum cortisol levels were also determined for all the groups., Results: The percentages of CD4+CD25+FOXP3 cells and the serum cortisol levels of infants born vaginally (p < 0.004 and p < 0.0001) and their mothers (p < 0.0001 for both) were found to be significantly higher than those of newborns born by C-section and their mothers. Positive correlations were seen between CD4+CD25+FOXP3+ cells (r = 0.741) and serum cortisol levels (r = 0.468). No relationship was observed between newborns delivered by C-section and their mothers (r = 0.022 for both)., Conclusions: This study suggests that mode of delivery affects cord blood Treg cells. Higher CD4+CD25+FOXP3+ Treg cells of newborns and their mothers in vaginal delivery group and their relationship with serum cortisol levels suggest a stress phenomenon related to vaginal delivery.

Left pulmonary artery (PA) originating from patent ductus arteriosus is an exceptionally rare variant of tetralogy of Fallot with absent pulmonary valve. We described an alternative technique for the repair of discontinuous left PA with the use of the redundant pulmonary artery tissue as material for the conduit in a 3-year-old boy.

A total of 25 patients undergoing coronary artery bypass grafting (CABG) were included in the study. Patients received statin (20 mg daily) postoperatively for 2 weeks. All analyses were performed at 2 different time points: preoperatively (group 1) and 2 weeks after operation (group 2). Interleukin (IL)-6, IL-8, plasminogen activator inhibitor 1 (PAI-1), tumor necrosis factor α (TNF-α), tissue plasminogen activator (t-PA) levels, and tissue factor pathway inhibitor (TFPI) were evaluated. Statin treatment caused a significant reduction in the plasma level of PAI-1 (preop: 15.04 ± 0.13 ng/mL vs postop: 13.89 ± 2.14 ng/mL; P < .05) and increased t-PA levels (preop: 109.74 ± 0.13 vs postop: 231.40 ± 1.22 ng/mL; P < .001). Plasma TNF-α and IL-6 levels did not change with treatment. Statin treatment caused a significant reduction in plasma IL-8 level (279.70 ± 3.42 ng/mL vs postop: 207.18 ± 3.63 ng/mL, P < .05), and TFPI (4.87 ± 2.05 ng/mL vs postop: 6.27 ± 1.25 ng/mL; P < .05). The results demonstrate that atorvastatin attenuates systemic inflammatory reaction after cardiac surgery.

We describe a right atrial metastasis of a testicular germ cell tumor, which was successfully removed from the tricuspid valve and subvalvular apparatus without the need for valve replacement. , (© 2011 Wiley Periodicals, Inc.)

Neonatal diabetes mellitus (NDM) is a rare condition that can be either transient or permanent. K(ATP) channel (Kir6.2 or SUR1) mutation, chromosome 6 abnormalities, insulin, or glucokinase gene mutations can lead to isolated NDM. Cases caused by Kir6.2 mutation usually result in permanent NDM (PNDM) rather than transient NDM (TNDM). The majority of patients with the Kir6.2 or SUR1 mutation can be successfully managed with a sulfonylurea agent, without the need for insulin. We report a preterm male with NDM having two novel missense mutations, E322A and D352H, in the KCNJ11 gene. At 2 months of age, successful transition from insulin to glibenclamide (glyburide) therapy of the patient was managed. At 5 months of age, his diabetes went in to remission.

Background: Recessive mutations in ABCC8/KCNJ11 of beta-cell K(ATP) channel generally cause severe medically unresponsive hyperinsulinemic hypoglycemia (HH). Rarer dominant mutations in these genes have been described that mostly cause milder, medically responsive congenital hyperinsulinism. Rarer dominant mutations in these genes have been described that mostly cause milder, medically responsive congenital hyperinsulinism. To date the phenotype of patients with dominant mutations seems to be different from those with recessive mutations as the majority of patients are responsive to diazoxide therapy. Controversy exists on whether these dominant ABCC8 or KCNJ11 genes mutations predispose to diabetes mellitus in adulthood or not., Subjects: We report the clinical and genetic characteristics of five patients with neonatal HH, three had recessively inherited K(ATP) channel mutations and two with a dominantly acting mutation. As a result of failure to medical therapy, patients with recessive K(ATP) channel mutations underwent a near total pancreatectomy. Two siblings with a novel dominant mutation showed good response to medical treatment. Although the HH remitted in early infancy, they became diabetic at the prepubertal age. Their mother, maternal aunt and maternal grandfather had the same mutation without any medical history of neonatal HH., Conclusion: The clinical presentation of our two patients with a dominant ABCC8 mutation was milder than that of patients with the resessive form of the disease as they responded well to medical management.

We present a patient with aortic root aneurysm and severe aortic regurgitation who had a previous off-pump reduction ascending aortoplasty and external wrapping with concomitant coronary bypass grafting. Preliminary aortic dissection and erosion of the aortic intima were detected during the operation. This complication warrants the re-evaluation of the indications for reduction ascending aortoplasty and emphasizes the necessity for close follow-up., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The objective of this study was to evaluate the effect of the antireflux barrier formed by Na alginate on pH-metry-defined acid reflux events in preterms. Four times/day, 1 mL/kg of Na alginate was administered as an intervention to the preterm infants whose gastroesophageal reflux disease was confirmed by 24-hour pH monitoring, and the measurement was repeated after 48 hours. The major outcomes were improvement in the number of reflux events per 24 hours, the duration of the longest episode, the number of episodes >5 minutes per 24 hours, and reflux index on pH-metry. Thirty-four (83%) of 41 preterm infants who completed the study had pathologic measurement on 24-hour pH monitoring; 27 (83%) of the patients responded to treatment, whereas 7 patients (17%) did not. Na alginate treatment significantly improved the number of episodes with pH <4 per 24 hours, the reflux index, the number of episodes >5 minutes with pH <4, and the duration of longest episode with pH <4. Patients showed significant improvement in 2 evaluated clinical symptoms, which were vomiting and weight gain after the treatment. No side effect except thickening of stool in 3 patients was observed. Na alginate treatment for gastroesophageal reflux disease in preterm infants seems to be safe and effective.

Objective: We aimed to compare the effects of 2 different antiplatelet agents on platelet activity in patients receiv- ing atorvastatin after coronary artery bypass grafting (CABG)., Methods: We prospectively randomized 50 patients undergoing CABG into 2 groups; group 1 started to receive atorvastatin (10 mg) plus clopidogrel (75 mg; C + A, n = 25) and group 2 atorvastatin (10 mg) and acetylsalicylic acid (ASA; 300 mg, ASA + A, n = 25) daily on postoperative day 1 and continued for 6 months after operation. Adenosine diphosphate (ADP)-induced platelet aggregation and the expressions of glycoprotein (Gp) IIb, GpIIIa, P-selectin, and fibrinogen (Fg) and low-density lipoprotein (LDL) binding to platelets were assessed preoperatively and at postoperative days 7, 90, and 180., Results: The mean age of the patients was 59.6 +/- 7.6 years, and 82% of the patients were males. The combination of C + A markedly inhibited ADP-induced platelet aggregation compared with ASA + A at postoperative days 90 and 180 (52% +/- 6.0% vs 56% +/- 7.25% and 19.6% +/- 3.2% vs 37% +/- 4.1%, P = .039 and P = .0001, respectively). The therapy of C + A significantly suppressed the expressions of GpIIIa at postoperative days 7, 90, and 180 (P = .0001, P = .0001, and P = .0001, respectively) and P-selectin at postoperative days 90 and 180 (P = .035 and P = .002, respectively) when compared to ASA + A. The expression of GpIIb was also significantly depressed at postoperative day 180 in group 1 when compared to group 2 (P = .0001). Low-density lipoprotein binding was significantly increased at day 180 postoperatively in both the groups (basal: 42.9% +/- 5.6% vs 45.3% +/- 4.4% and day 180: 60.3% +/- 4.6% vs 61.8% +/- 5.7%, P = .0001)., Conclusions: Our results demonstrate that the combination of C + A is more effective than that of ASA + A in inhibiting ADP-mediated platelet aggregation and expression of major platelet receptors after CABG.

Hypoxic-ischemic cerebral injury that occurs during the perinatal period is one of the most commonly recognized cause of long-term neurological deficit in children, often referred to as cerebral palsy. We describe a case with capillary leak syndrome and seizures to co-morbid status epilepticus related to perinatal hypoxic-ischemic encephalopathy in newborn period.

Pregnant women with low B12 levels are unable to provide the necessary amount of this vitamin to their fetuses. The mothers are usually not anemic, and failure to thrive and neurologic deficits are more common in their infants than in megaloblastic anemia. We report the case of a newborn that was born to a vegetarian mother and presented with the symptoms of megaloblastic anemia at birth; we also discuss vitamin B12 metabolism during pregnancy and lactation. An interesting feature of the present case is that it is the only reported case of a newborn that was treated by supplementation of the mother.

Minimal invasive approaches, including partial J- or C-shaped sternotomies, have gained a worldwide acceptance in cardiac surgical practice. To increase the cosmetic benefit and improve the surgical exposure, the length of underlying partial sternotomy is usually 3 to 5 cm longer than the length of skin incision in most of these approaches. Using a standard sternal saw or a rotary craniotome, it is quite difficult to make sternal incision under subcutaneous tissue at both ends of skin incision. Moreover, standard sternal saws do not allow doing fine manipulations on sternum that may cause inadvertent sternal cutting, especially at the curved parts of C- or J-type partial sternotomies. We removed the blade protector part of a standard sternal saw. This simple maneuver enables surgeons to perform several challenging mini-sternotomies easily for adult cardiac procedures.