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BACKGROUND: This study aims to evaluate the experience of a tertiary health center on the timeliness of postnatal management in newborns with open neural tube defects (NTDs). METHODS: This is a retrospective review of 38 neonates with NTDs who were treated surgically at a tertiary health care center between January 2009 and January 2019. Five neonates with genetic syndromes were excluded. RESULTS: Twenty-six neonates with NTD underwent surgery on the first postnatal day while 12 neonates with NTD had surgery after the first postnatal day. The reasons for the latency in operative treatment were the delay in the referral of the affected newborn from other health care centers (n=8) and the transient abnormalities in coagulation tests (n=4). Rural residence was significantly more frequent, gestational age at delivery was significantly lower, preterm delivery was significantly more frequent and prenatal diagnosis was significantly less frequent in neonates that underwent surgery for NTD repair after the first postnatal day (p=0.001, p=0.048, p=0.024 and p=0.003 respectively). Postoperative motor dysfunction was significantly more severe (p=0.002), postoperative complications were significantly more frequent (p=0.008), the reoperation and postoperative mortality rates were significantly higher (p=0.009 and p=0.048 respectively) and the duration of hospital stay was significantly longer (p=0.033) for the neonates who underwent surgery after the first postnatal day. CONCLUSIONS: Our study appears to favor the early repair of NTD`s within the first 24 hours of life. Such an approach may reduce the risk of infectious and neurological complications significantly.

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AIM: For the small for gestational age (SGA) infants born at the normal birth time but whose birth weights were below the 10th percentile and for the appropriate for gestational age (AGA) infants born in normal birth weights according to gestational week, the diameters of right and left coronary arteries were echocardiographically measured in three separate time intervals with their body weights, heights and body surface area parameters. The aim of this study is to compare the progression of coronary artery diameters of SGA infants with AGA infants over time and the effects of coronary artery diameters on the clinical and hemodynamic parameters of SGA babies. MATERIAL AND METHODS: Term 55 SGA babies were compared with 200 AGA infants at birth, first and sixth months of ages for both growth parameters and coronary artery diameters measurements. RESULTS: In comparison of SGA and AGA groups at birth, first and sixth months of ages on body weight, height and body surface area, aortic annulus and left coronary artery and right coronary artery diameters, it was seen that SGA group could not catch up the AGA group in all time periods on both anthropometric and coronary artery diameters data even if they have an increased growth (p<.05). Although SGA infants appeared to be far away from SGA criteria in body weight measurements at 6th months. These infants maintained a significant deficit in height and coronary artery measurements according to the AGA group (p<.05). CONCLUSIONS: In the evaluation of growth indices and coronary artery diameters at birth, first and sixth months of ages in SGA babies, it is clear that impaired fetal growth of these babies has lasting-effects on coronary artery diameters and cardiac structure.

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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.

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Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy. The genetic basis of CHI includes a variety of defects in key genes regulating insulin secretion. Mutations in at least seven genes are found in 50% of cases. The most common forms of medically unresponsive CHI, which requires a near-total pancreatectomy are associated with autosomal recessive mutations in the ABCC8 and KCNJ11 genes encoding the two subunits of the pancreatic ß-cell ATP-sensitive potassium channel. We report a neonate with CHI and have a novel homozygous splicing mutation in the ABCC8 gene.

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Calcium metabolism disturbances are common in childhood. In infancy, hypercalcemia generally occurs due to hyperparathyroidism, familial hypocalciuric hypercalcemia, subcutaneous fat necrosis, total parenteral nutrition administration, hyperthyroidism, and adrenal insufficiency. Granulomatous disorders such as tuberculosis and sarcoidosis are rarer cause of hypercalcemia. Hypercalcemia outcomes including nephrocalcinosis, brain, eye, artery calcifications and encephalopathic features are life-threatening. We report a seven-month-old girl with miliary tuberculosis who presented with severe hypercalcemia.

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OBJECTIVE: Fetal calprotectin levels increase in the early stages of necrotizing enterocolitis. Although the effects of several factors on fetal calprotectin have been studied, the effect of phototherapy is not known. In this study, we analyzed the effect of phototherapy on fetal calprotectin levels. METHODS: Ninety breast-fed newborns (46 male, 44 female) who were hospitalized for indirect hyperbilirubinemia and treated with phototherapy were included to the study. Forty-two of them were term and 44 of them were preterm. Newborns treated with phototherapy (n = 53) constituted the phototherapy group (29 preterm, 24 term) and 37 newborns who did not receive phototherapy (19 preterm, 18 term) constituted the control group. Fecal samples were collected 24 hours after phototherapy had been started. Fecal samples (100 mg) were weighed with sensitive scales and preserved at -80°C after buffering with a special solution. All samples were studied at the same time with a fecal calprotectin kit by using enzyme-linked immunosorbent assay. RESULTS: There were no statistically significant difference between fecal calprotectin levels of term and preterm babies who received phototherapy and babies who did not receive phototherapy. CONCLUSION: There was no effect of 24-hour phototherapy on fecal calprotectin levels in preterm and term newborns.

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OBJECTIVE: To analyze non benign neonatal arrhythmias (NA) observed in a tertiary neonatal intensive care unit (NICU). METHODS: From June 2006 through July 2011, newborns admitted to the NICU for NA or diagnosed as NA after hospitalization were evaluated retrospectively. The newborns with non benign NA were included in the study. RESULTS: During the study period, the incidence of non-benign NA was 0.7 % (n = 55/7880). The mean age at diagnosis was 16.7 ± 1.8 d ranging from 1 d to 90 d. The most common type was supraventricular arrhythmia (SVT) with an incidence of 0.3 %. Univariate analyses showed that there were significant differences between the survived and died infants according types of congenital heart disease (CHD), electrolyte imbalance, and arrhythmias. The mortality rates were higher among infants with obstructive type left-to right shunt and common mixing type CHD. The most dangerous type of electrolyte imbalance was hyperkalemia. CONCLUSIONS: Many arrhythmias could not be noticed at neonatal period even in NICU, implying that it is increasingly important for the physician to be aware of the etiology, development, and natural history of these arrhythmias.

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Chitotriosidase (ChT) is an enzyme secreted by activated macrophages and involved in defense against, and in degradation of chitin-containing pathogens, such as fungi, nematodes, and insects. In addition, it plays an important role in the development of atherosclerosis related with systemic low-grade inflammation. To this effect of activity of ChT, we aimed to investigate serum ChT activity in obese subjects and to determine to relation with insulin resistance and high-sensitive C-reactive protein (hsCRP). A total of 73 obese subjects (10.9 +/- 2.6 years of age, 44 male patients) and 41 age and gender-matched healthy lean subjects (11.6 +/- 2.9 years of age, 18 male patients) were included in this study, between 2007 and 2008. The criterion for diagnosing obesity was defined as the body mass index (BMI) being over 97th percentile of the same gender and age. Fasting serum glucose, insulin, hsCRP and ChT levels were measured. We compared the differences in variables between obese and lean subjects with Student's t-test compared after ascertaining that the data were normally distributed. All data were expressed as mean +/- standard deviation. There was statistically significant increase in serum ChT activity of obese subjects, while there was statistically significant difference in serum hsCRP levels when compared to healthy lean subjects (30.0 +/- 17.9 and 23.0 +/- 17.8, p=0.045; 2.3 +/- 3.1 and 0.7 +/- 1.2, p=0.001). Obese subjects had significantly higher BMI-SDS, TG and HOMA-IR and lower HDL-C levels when compared with the healthy lean subjects (p<0.05). Correlation analysis showed no significant correlation between serum ChT activity and hsCRP, HOMA-IR and BMI-SDS (p>0.05). Although the data need to be validated by further investigation, the observations made in this study seem to indicate that serum ChT activity may not be a useful marker for monitoring systemic low-grade inflammation and insulin resistance in obese subjects.

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UNLABELLED: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here we present a newborn galactosemia case presented with HLH. Close monitoring of the diagnostic criteria of HLH during the course of galactosemia-associated hemophagocytosis, both before and after dietary treatment, should be performed in order to fully determine if the triggering mechanism is infection or accumulation of metabolites. CONFLICT OF INTEREST: None declared.

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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.

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We sought to determine the effect of neonatal transport on the incidence of adverse events of transient tachypnea of the newborn (TTN) in term neonates. A retrospective study was performed of neonates who had TTN and were admitted to Neonatal Intensive Care Unit (NICU) of Sami Ulus Maternity, Children's Education and Research Hospital by land-based transport. Data from 208 newborns with TTN were evaluated, and clinical and laboratory findings were compared between patients who were transported from within the city (group 1) or from outside of the city (group 2). In the present study, long-distance land-based neonatal transport increased the adverse effects of TTN in newborns. Arterial blood gas parameters of the neonates in both groups before transport were similar, and these parameters and Downes' scores were comparable in both groups, implying that patients from outside the city had greater respiratory insufficiency than those from inside the city at admission to NICU. Respiratory support in the NICU and pulmonary air leak syndrome ratios were found to be significantly higher in the group from outside the city. Long-distance land-based transport in neonates with TTN increases the severity of illness. Furthermore, adverse events and the outcome of such infants depend on the effectiveness of the neonatal transport system.

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UNLABELLED: Congenital chylous ascites is a rare condition seen in the neonatal period and the data on pathogenesis and treatment modalities are limited. In this article, we report a case of neonate with chylous ascites and review the therapeutic management procedures on chylous ascites in childhood. We present our experience in the diagnosis and treatment of this condition. CONCLUSION: Medium-chain triglycerides (MCT)-based diet can be tried as a first option in chylous ascites treatment. In resistant or unresponsive cases, somatostatin along with TPN can have use in closing the lymphatic leakage or relieving the symptoms effectively and rapidly. Conventional regimens including enteral feeding with MCT-based formula can then be re-administered as a maintenance treatment after reduction of lymph flow with the use of total parenteral nutrition (TPN) and somatostatin infusion combination. Patient-specific approach should be attempted for chylous ascites caused by various disorders and started as soon as possible.

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OBJECTIVE: The aim of this study was to investigate the relations between the P-wave dispersion and diastolic functions in type 1 diabetic children. PATIENTS: A total of 33 diabetic patients without any cardiovascular disease, with a mean age of 12.3 plus or minus 4.2 years, and 29 healthy controls, with a mean age of 10.4 plus or minus 3.9 years were enrolled for this study. Left and right ventricular functions were assessed by using standard pulsed-wave Doppler echocardiography. P-wave dispersion was calculated by measuring minimum and maximum P-wave duration values on the surface electrocardiogram. RESULTS: For the diabetic patients, P-wave maximum duration and dispersion was found to be significantly increased compared with healthy controls. Likewise, mitral A velocity and A velocity time integral was significantly increased while the isovolumic contraction time was significantly higher in the diabetics. In tricuspid valve measurements, however, A velocity time integral was found to be significantly higher, whereas the deceleration time was significantly lower in the diabetics. No relation was found between the left ventricle diastolic functions and duration of diabetes, HbA1c levels and P-wave dispersion in the diabetic children. No correlation was found between the diastolic functions and P-wave minimum, maximum duration, and dispersion for all the participants. CONCLUSION: In type-1 diabetic children, the diastolic functions of both the ventricles were observed to be affected negatively together. Diabetes might be causing the prolongation of P-wave dispersion, but there was no relationship between the diastolic functions and P-wave dispersion in the diabetic children.

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