Neonatal Lymphatic Flow Disorder.

OBJECTIVE: To examine and discuss patients diagnosed with acquired and congenital chylothorax in the neonatal period in the light of the literature. METHODS: The files of newborns followed-up in the neonatal intensive care unit (NICU) and diagnosed with congenital and acquired chylothorax were reviewed retrospectively. Patients with isolated chylothorax were classified as Group 1 and those with multiple lymphatic flow disorders were classified as Group 2. Antenatal and clinical features were recorded and compared between the groups. RESULTS: Thirteen infants were diagnosed with chylothorax; 92.3% (n = 12) of the patients were congenital. The rate of antenatal diagnosis was 61.5% (n = 8). Eight patients (61.5%) were diagnosed with hydrops fetalis. Among the cases in Group 1 and Group 2, receiving ocreotide and the incidence of sepsis (p = 0.05) were partially significant. Seven of the patients (66.6%) responded to medium chain triglycerides (MCT), and complete resolution was seen in 6 (85.7%) of the responders. Complete resolution of chylothorax fluid was observed in 7 (77.7%) of nine patients who responded to ocreotide treatment. CONCLUSIONS: In neonatal chylothorax, the postnatal period includes a multidisciplinary approach that requires drug therapy, dietary modifications, drainage of pleural fluid, and rarely, surgery.

The Effect of Breastfeeding Self-Efficacy on Infants' Weights and Breastfeeding Outcomes.

OBJECTIVE: The aim of this study was to investigate the effects of breastfeeding self-efficacy (BSE) and breastfeeding success (BFS) on infants' weight gain rates and breastfeeding outcomes. MATERIALS AND METHODS: Mothers were evaluated within the postpartum 72 hours with "Breastfeeding Self-Efficacy Scale-Short Form (BSES-SF)" and "LATCH Scale." After 6 months, the mothers were called to learn the exclusive breastfeeding (EB) duration and the weight of the infants. RESULTS: Two hundred mother-infant couples were enrolled, 176 (88%) of them could be reached in the sixth month. The BSES-SF scores were positively correlated with LATCH scores (P = .0001). The EB rate in the sixth month was positively correlated with BFS but not correlated with BSE (r, P = .218, .004; .79, .297, respectively). The percentage of weight gain and the rate of weight percentile change of babies according to birth weight at the sixth month of age were negatively correlated with BSE (r = -0.226, -0.148, P = .003, .049, respectively) but not correlated with BFS. CONCLUSION: Higher BFS was associated with increased duration of EB in the first 6 months of life, and higher BSE was related to lower increase in the percentage of weight gain and a lower rate of weight percentile change of babies at 6 months of age.

Vasoactive inotropic score as a predictor of mortality in neonatal septic shock.

BACKGROUND: Although many improvements in neonatal care have been achieved, mortality rates for sepsis and septic shock in newborns are still high. The vasoactive inotropic score (VIS) was designed and studied to predict mortality in different settings. There are currently no data on the predictive ability of the VIS for mortality in newborn patients with septic shock. METHODS: Patients with late-onset neonatal sepsis who required inotropes because of fluid-refractory septic shock during the study period were included in the study. Four distinct VIS values were calculated for each septic shock episode after inotropic treatment had begun, that is, at the initiation of inotropic treatment and at 24 and 48 h after inotropic treatment had begun, and the highest VIS (VISmax) at any time after initiation of inotropic agents. RESULTS: The 98 episodes studied were divided into two groups according to the outcomes of their sepsis episodes as survivors (n = 39) or nonsurvivors (n = 59). The areas under the curve of the VIS values for the prediction of mortality were the VISmax (0.819, p < 0.001), followed by the VIS48 (0.802, p < 0.001), VIS24 (0.762, p = 0.001) and VIS0 (0.699, p = 0.015). Patients with a VISmax of greater than 20 had significantly higher odds of mortality (p < 0.001, ß = 14.7, 95% confidence interval [4.7-45.9]). CONCLUSION: We found that the VISmax was an easy-to-use and helpful tool for predicting a poor outcome in neonatal sepsis. Physicians should be aware that the prognosis is poor for any newborn with a VIS of 20 or greater at any point after the onset of sepsis.

Neonatal sepsis is still one of the most important causes of mortality and morbidity in the neonatal period, and it is also a significant public health problem. Researchers have been looking for reliable biomarkers and scoring systems to detect neonatal sepsis and predict outcomes. The vasoactive inotropic score has been validated and found to be useful for predicting mortality in septic shock in adults and children and newborns who underwent cardiac surgeries. However, there are no neonatal sepsis data. In this retrospective study, we showed that a maximal vasoactive inotropic score of 20 or greater is an easy, noninvasive and useful tool to determine the poor outcome.

G-ROP criteria for predicting retinopathy of prematurity among neonates with different birth weight percentiles.

BACKGROUND: The Postnatal Growth and Retinopathy of Prematurity (G-ROP) Study showed that adding postnatal weight gain to birth weight and gestational age detected 100% of cases with type 1 retinopathy of prematurity (ROP) while reducing the ROP examinations by 30%. The purpose of this study was to investigate whether being small for gestational age (SGA) affects the sensitivity and specificity of the G-ROP model. METHODS: We applied the G-ROP criteria for premature infants. The infants were classified as three subgroups: SGA, appropriate for gestational age (AGA), and large for gestational age (LGA). The performance of G-ROP criteria was assessed for each group for ROP. RESULTS: There were 41 (10.5%) SGA, 312 (80%) AGA, and 37 (9.5%) LGA neonates. Twenty-six (6.7%) neonates were treated for ROP, and the G-ROP model identified all of them. The sensitivity of the model for treatment-requiring ROP (TR-ROP) was found to be 100% in the whole patient group and for each subgroup. The specificity for TR-ROP was 46.4% for the whole group, 50% for SGA, 44% for AGA, and 63.6% for LGA. By applying the G-ROP model, the number of ROP examinations could be reduced by 25% for the whole group, 27% for SGA, 24% for AGA, and 31% for LGA, without missing TR-ROP. CONCLUSIONS: The sensitivity and specificity of the G-ROP model for TR-ROP in SGA infants were similar to the whole group. The model did not miss any cases of TR-ROP.

Factors Associated with the Transition Time to Full Enteral Feeding in Newborns with Hypoxic Ischemic Encephalopathy.

BACKGROUND: We aimed to assess the factors associated with the transition time to full enteral feeding (FEF) in newborns with hypoxic ischemic encephalopathy (HIE) undergoing therapeutic hypothermia. METHODS: We obtained data retrospectively from medical records of the neonates diagnosed with HIE and treated by therapeutic hypothermia to evaluate the factors associated with transition time to FEF. RESULTS: Sixty-one neonates were included in the study. The median gestational age (GA) and birth weight were 39 (37-40) weeks and 3245 (2715-3575) grams, respectively. APGAR scores at the first and fifth minutes were 3 (1-5) and 6 (4-7), respectively. Fifty-seven (93.4%) of the newborns were diagnosed as having moderate HIE, and 4 (6.6%) of them had severe HIE. Transition time to FEF was found to be negatively correlated with gestational week (r, P: -0.280, 0.029) and birth weight (r, P: -0.315, 0.013); and positively correlated with lactate (r, P: 0.295, 0.044), BUN (r, P: 0.285, 0.026) and creatinine levels (r,P: 0.345, 0.007); duration of invasive (r, P: 0.565, 0.0001) and non-invasive mechanical ventilation (r, P: 0.261, 0.042), use of antibiotics (r, P: 0.556, 0.0001) and inotropic agents (r, P: 0.524, 0.0001) and hospitalization (r, P: 0.654, 0.0001). CONCLUSION: Clinicians should be more careful while starting to feed babies undergoing therapeutic hypothermia with higher lactate levels and impaired renal functions, and should be encouraged to feed clinically stable neonates with HIE as soon as possible, as the transition time to FEF could be related with better clinical outcomes.

The Associations Between Lung Ultrasonography Scores in the First Day of Life and Clinical Outcomes.

OBJECTIVES: To investigate the role of lung ultrasonography (LU) in predicting noninvasive ventilation (NIV) failure and the relationship between lung ultrasonography scores (LUS) with clinical outcomes in neonatal respiratory failure (NRF). METHODS: A prospective, cross-sectional study was conducted in newborns with NRF who needed NIV and were evaluated by LU. The first LUS (LUS1) was calculated at 2-6 hours and the second (LUS2) at 12-24 hours of life. The patients were divided into NIV failure and NIV non-failure groups. The relationship between LUS and clinical outcomes was evaluated. RESULTS: Among 157 neonates, the median (interquartile range) of gestational week and birth weight were 37 weeks (34-39), and 2890 grams (2045-3435), respectively. The reasons for NRF were transient tachypnea of the newborn (n = 92, 58.6%), congenital pneumonia (n = 58, 36.9%), and respiratory distress syndrome (n = 7, 4.5%). The rate of NIV failure was 17.8% (n = 28). Both LUS1 and LUS2 were significantly higher in neonates with NIV failure compared to neonates with NIV non-failure (P = .001). A cutoff value of LUS1 ≥ 4 predicted NIV failure with 96% sensitivity and 63% specificity. There were positive correlations between LUS and PEEP values, IMV and total MV days, carbon dioxide values, length of hospital stay, and antibiotic days (ρLUS1 , P = .843, <.001; .474, <.001; .444, <.001; .258, .001; .212, .008; .270, <.001, respectively). CONCLUSIONS: Lung ultrasound scores were higher in neonates with NIV failure than with NIV non-failure group, and strongly correlated with end-expiratory pressure values. Lung ultrasound scores were found to be related with some of the clinical outcomes of the NRF, and this suggested that LUS could provide information about the prognosis of NRF.

Acute liver failure in newborns.

Acute liver failure is a condition that is defined as a sudden, complete, or nearly complete loss of liver functions without any previous liver disease, usually accompanied by encephalopathy, which can be reversible, but with a mortality rate of 55-70%. Acute liver failure newborns is an acute liver failure in the first 28 days of life. The Pediatric Acute Liver Failure Working Group identified the presence of coagulopathy as the main finding for the identification of acute liver failure in childhood following vitamin K administration. Although the incidence of acute liver failure is reported to be 17/100 000 in all ages, its incidence is not known exactly in newborn and childhood. The most common cause of acute liver failure in the newborn period is the gestational alloimmune liver disease that was previously known as neonatal hemochromatosis. This is followed by viral infections, metabolic diseases, hemophagocytic lymphohistiocytosis, and other rare causes. In the neonatal period, acute liver failure is a rare condition with a high mortality rate. For this reason, the vital signs of the patients should be closely monitored and supportive treatments should be planned according to the follow-up and the etiology of the disease should be clarified urgently. In this process, acyclovir treatment until herpes simplex virus infection is excluded and lactose-free feeding until galactosemia is excluded are recommended as life-saving treatments. In the literature, since there is a limited number of studies related to neonatal acute liver failure, prospective studies investigating the factors affecting treatment and prognosis are needed.

Effects of COVID-19 pandemic on breastfeeding rates in a neonatal intensive care unit.

OBJECTIVES: To find out if the expressed breast milk delivery rate to neonatal intensive care unit (NICU) for babies who were hospitalized for any reason other than COVID-19, and exclusive breastfeeding (EB) rates between discharge date and 30th day of life of those babies were affected by COVID-19 pandemic. METHODS: Babies who were hospitalized before the date first coronavirus case was detected in our country were included as control group (CG). The study group was divided into two groups; study group 1 (SG1): the mothers whose babies were hospitalized in the period when mother were asked not to bring breast milk to NICU, study group 2 (SG2): the mothers whose babies were hospitalized after the date we started to use the informed consent form for feeding options. The breast milk delivery rates to NICU during hospitalization and EB rates between discharge and 30th day of life were compared between groups. RESULTS: Among 154 mother-baby dyads (CG, n=50; SG1, n=46; SG2, n=58), the percentage of breast milk delivery to NICU was 100%, 79% for CG, SG2, respectively (p<0.001). The EB rate between discharge and 30th day of life did not change between groups (CG:90%, SG1:89%, SG2:75.9; p=0.075). CONCLUSIONS: If the mothers are informed about the importance of breast milk, the EB rates are not affected by the COVID-19 pandemic in short term, even if the mothers are obligatorily separated from their babies. The breast milk intake rate of the babies was lowest while our NICU protocol was uncertain, and after we prepared a protocol this rate increased.

The Role of Lung Ultrasound as an Early Diagnostic Tool for Need of Surfactant Therapy in Preterm Infants with Respiratory Distress Syndrome.

OBJECTIVE: This study aimed to determine the accuracy of neonatal lung ultrasound (LUS) in predicting the need for surfactant therapy compared with chest X-ray (CXR) in preterm infants. STUDY DESIGN: A prospective double-blind study was conducted in infants with a gestational age <34 weeks with respiratory distress syndrome (RDS) by evaluation with LUS and CXR on admission. RESULTS: Among 45 preterm infants, the median (interquartile range [IQR]) LUS score was 4 (2-8) in the mild RDS group, whereas it was 10 (IQR: 9-12) in the severe RDS group (p < 0.01). The LUS score showed a significant correlation with the need for total surfactant doses (ρ = 0.855; 95% confidence interval [CI]: 0.801-0.902; p < 0.001). A cut-off LUS score of four predicted the need for surfactant with 96% sensitivity and 100% specificity (area under the curve [AUC]: 1.00; 95% CI: 0.97-1.00; p < 0.01). LUS scores predicted continuous positive airway pressure (CPAP) failure accurately (AUC: 0.804; 95% CI: 0.673-0.935; p = 0.001). A significant correlation was observed between LUS scores and positive end-expiratory pressure levels (ρ = 0.782; p < 0.001). During the study period, the CXR number per infant with RDS decreased significantly when compared with preceding months (p < 0.001). The LUS score in the first day of life did not predict the development of bronchopulmonary dysplasia (AUC: 0.274; 95% CI: 0.053-0.495; p = 0.065). CONCLUSION: The LUS score in preterm infants accurately predicts the severity of RDS, the need for surfactant and CPAP failure. The routine use of LUS can decrease the frequency of CXRs in the neonatal intensive care units. KEY POINTS: · LUS is a nonhazardous bedside technique.. · LUS predicts the need for surfactant in preterm infants.. · LUS predicts the severity of RDS better than CXR..

Association of vitamin D receptor gene FokI and TaqI polymorphisms and risk of RDS.

Background: Vitamin D and its receptor (VDR) have important roles in perinatal lung development. The objective of this study was to investigate the possible association between VDR FokI and TaqI polymorphism and development of respiratory distress syndrome (RDS) in preterm infants.Method: A total of 173 premature infants <34 weeks: 82 with RDS and 91 without RDS were enrolled. Genotyping of VDR polymorphisms was assayed by real-time PCR. Serum 25-hydroxyvitamin D (25-OHD) levels were measured by ELISA in blood samples that were obtained at the time of admission to the neonatal intensive care unit.Results: Gestational age (GA) was significantly lower in the RDS group compared with the controls. In univariate analysis, VDR TaqI CT and CC genotypes were associated with the increased risk of RDS (OR = 3.264, p = .001, 95% CI = 1.597-6.672 and OR = 5.222, p < .001, 95% CI = 2.165-12.597, respectively); while VDR FokI showed no association with RDS. In multivariate logistic regression analysis, variant TaqI genotype increased risk of RDS (p = 0.001, OR = 3.464, 95% CI = 1.655-7.251) independent of gestational age, birth weight and gender. 25-OHD levels in the RDS group were significantly lower compared with those in without the RDS group (p = .002). Serum 25-OHD levels were not significantly different among the different FokI and TaqI genotypes in RDS group.Conclusions: This is the first report of association of VDR polymorphism with RDS development in preterm neonates. Current study suggests that VDR TaqI polymorphism may be involved in predisposition to RDS in premature neonates. Further studies are needed to assess the contribution of vitamin D and VDR signaling to the pathogenesis RDS.

Does platelet mass influence the effectiveness of ibuprofen treatment for patent ductus arteriosus in preterm infants?

OBJECTIVE: The aim of this study is to evaluate whether the platelet mass in the first 24 h of life is effective on closure of patent ductus arteriosus (PDA) or not. STUDY DESIGN: Preterm infants with a gestational age of < 32 weeks, hospitalized at a tertiary neonatal intensive care unit (NICU) and requiring medical treatment (intravenous or oral ibuprofen) for hemodinamically significant PDA (hsPDA) were enrolled in this study. The patients were divided into two groups after first course of pharmacologic treatment according to closure of PDA (Group 1: PDA closure, Group 2: PDA without closure). Groups were compared in terms of demographics findings, morbidities, platelet measurements like counts, mean platelet volume (MPV) and platelet mass (platelet count × mean platelet volume). RESULTS: The study included 77 preterm newborns in Group 1, and 30 preterms in Group 2. There were no differences in birth weight, gestational age, gender and maternal risk factors between the study groups. The mean platelet count in the first postnatal blood count was in Group 1: 211.3 ± 89.2 × 10(3)/mm(3) and in Group 2: 216.5 ± 26 × 10(3)/mm(3), respectively (p = 0.783). The mean platelet volumes (MPV) were similar in both groups (p = 0.535). No statistically significant difference between platelet mass values was detected (Group 1: 1811 ± 884 fl/nl, Group 2: 1868 ± 717 fl/nl) (p = 0.753). CONCLUSION: Our data suggest that platelet count, MPV and platelet mass did not affect the closure of hsPDA with ibuprofen.

Mean platelet volume and uric acid levels in neonatal sepsis.

OBJECTIVES: To determine the role of mean platelet volume (MPV) and uric acid levels in the diagnosis of neonatal sepsis (NS). METHODS: A total of 146 newborns with suspected NS were prospectively included in the study and infants without NS (n = 142) were assigned as controls. The patients were divided into three groups: Group I (n = 64): clinical NS, Group II (n = 82): culture-proven NS, and Group III (n = 142): healthy controls. RESULTS: The patients in Group II had the highest C-reactive protein (CRP) levels (54.6 ± 5.4 mg/L), lowest platelet counts (199,329 ± 135,952/mm(3)) and lowest uric acid levels (2.6 ± 1.8 mg/dL) when compared to Groups I and III (p < 0.05, for all comparisons). MPV values were higher in Group I (10.6 ± 1.1 fL) and Group II (10.4 ± 0.9 fL) when compared to Group III (9.2 ± 1.2 fL) (p = 0.001), although there was no difference between Groups I and II. Area under curve (AUC) values for CRP, MPV, and uric acid were 0.92 (p = 0.001), 0.76 (p = 0.001) and 0.28 (p = 0.001), respectively. The diagnostic cut-off values for CRP and MPV were 9.5 mg/dL and 10.4 fL. Sensitivity and specificity of MPV in NS were 54 % and 82 % respectively. When combined with CRP its sensitivity and specificity increased to 89 % and 79 % respectively. CONCLUSIONS: The combined use of CRP and MPV should be considered in the early diagnosis of NS, but uric acid levels may only be utilized as an additional tool to support diagnosis. CRP is shown to be more sensitive and specific than MPV and uric acid in diagnosing neonatal sepsis.

Perfusion index variability in preterm infants treated with two different natural surfactants for respiratory distress syndrome.

OBJECTIVE: The objective of this study was to compare the perfusion index (PI) variability in premature infants with respiratory distress syndrome (RDS) following administration of two different natural surfactant preparations. STUDY DESIGN: This was a prospective study on 92 preterm infants with RDS. Patients were randomized into two groups. Group 1 (n = 46) received beractant; Group 2 (n = 46) received poractant alfa. Oxygen saturation, PI, and heart rate were measured by Masimo Rainbow SET Radical-7 pulse oximeter (Masimo Corp., Irvine, CA) before and after surfactant. The effects of the two treatment regimens on PI and oxygenation were compared. RESULTS: Repeated doses were more needed in beractant group (p = 0.04). Median oxygenation index (OI) before surfactant were similar, but improvement in OI was more prominent at 6th hour of surfactant in Group 2 (p = 0.001). Both groups had similar preductal PI values before surfactant. PI was higher at 6th hour of surfactant in Group 2 (p = 0.001). Pulmonary hemorrhage, intraventricular hemorrhage, patent ductus arteriosus, necrotizing enterocolitis, and mortality were more frequent in infants whose PI values lower than 0.7 within the first 5 days of life (p = 0.001). CONCLUSION: Poractant alfa resulted in more prominent improvement in PI and OI. Low PI values measured at early postnatal period may predict poor clinical outcome in preterm infants with RDS.

Successful hypothermia treatment of hypoxic-ischemic encephalopathy in a neonate with epidermolysis bullosa.

Despite advances in the neonatal care, hypoxic ischemic encephalopathy in late preterm and term infants remains an important cause of morbidity and mortality. There is lack of data on the application of therapeutic hypothermia in the existence of severe skin lesions. Epidermolysis bullosa is a rare group of inherited conditions which causes blisters in skin and mucosal membranes. In this report, the authors describe a successful whole-body hypothermia treatment of severe hypoxic ischemic encephalopathy in a term newborn with dystrophic epidermolysis bullosa. They observed that therapeutic hypothermia may also be given in newborns with dystrophic epidermolysis bullosa without any complications.

Distribution of congenital anomalies in a neonatal intensive care unit in Turkey.

BACKGROUND: 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. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

Clinical course of community-acquired respiratory syncytial virus pneumonia in newborns hospitalized in neonatal intensive care unit.

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.

Literature review and outcome of classic galactosemia diagnosed in the neonatal period.

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.

Non benign neonatal arrhythmias observed in a tertiary neonatal intensive care unit.

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.