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Objective: To present the diagnostic workup in an extremely low birth weight infant patient with signs of both sepsis and hemophagocytosis., Participants: A preterm infant presented with clinical and laboratory signs of early-onset sepsis including hepatosplenomegaly, thrombocytopenia, direct hyperbilirubinemia, and elevated liver enzymes., Methods: Despite extensive septic workup, no underlying infection was detected. Additional hyperferritinemia and other elevated inflammatory parameters raised the suspicion of a primary or secondary hemophagocytic lymphohistiocytosis (HLH)., Results: However, further metabolic analysis yielded a positive result for Gaucher disease (GD) type 2, a rare, but possible trigger of HLH., Conclusions: Our case shows that GD may lead to the picture of a secondary HLH and that a metabolic workup should always be performed in patients in whom primary HLH has been excluded.

Background: Intestinal ischemia plays a major role in the pathogenesis of necrotizing enterocolitis (NEC). The diagnosis of intestinal ischemia would be highly desirable, as it is impossible to achieve with the current diagnostic regimes. Preliminary data from an animal NEC model indicate a possible correlation between the plasma activity of the lysosomal enzyme beta-glucosidase and intestinal ischemia., Methods: In this case-control study the plasma activities of six different lysosomal enzymes were detected by high-performance liquid-chromatography tandem mass-spectrometry in 15 infants with NEC and compared to 18 controls., Results: The plasma activities of β-glucosidase (ABG), α-glucosidase (GAA), and galactocerebrosidase (GALC) were significantly higher in the NEC group compared with controls (ABG, p=0.009; GAA, p<0.001; GALC, p<0.001). GAA and GALC showed the highest diagnostic value with areas under the curve of 0.91 and 0.87., Conclusions: We identified GAA and GALC as new promising biomarkers for gut wall integrity in infants with NEC, and report first results on the plasma activity of ABG. The present study supports the hypothesis that the plasma activity of ABG might serve as a marker of intestinal ischemia in NEC. The identification of intestinal ischemia could facilitate early discrimination of infants at risk for NEC from infants with benign gastrointestinal disorders., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: The impact of growth restriction on perinatal morbidity is well known, but electroencephalographic (EEG) data on its influence are still scarce., Objectives: We aimed to analyze the influence of being born small for gestational age (SGA; defined as a birth weight <10th percentile) on the amplitude-integrated EEG (aEEG) score in the first 2 weeks of life in preterm infants born before 30 weeks of gestation, and its impact on later outcome., Methods: aEEG data obtained within the first 2 weeks of life on preterm infants born SGA and before 30 weeks of gestational age (GA) were analyzed retrospectively using a combined score [including background activity, occurrence of sleep-wake cycles (SWC) and suspected seizure activity]. Neurodevelopmental outcome was evaluated at 24 months by means of the Bayley Scales of Infant Development II and a standardized neurological examination., Results: One hundred and thirty-six patients were included (47 SGA and 89 controls). Infants with SGA had abnormal aEEG scores significantly more often (57 vs. 24%, p = 0.002) than infants born appropriate for gestational age (AGA). They also displayed SWC less frequently (65 vs. 96%, p = 0.001), were more likely to develop seizure activity (15 vs. 4%, p = 0.013) and had a normal neurodevelopmental outcome at the age of 2 years less frequently (36.2 vs. 59.6%, p = 0.02)., Conclusion: Preterm infants born SGA and before 30 weeks of GA had less optimal scores on early aEEG and a poorer neurodevelopmental outcome at 24 months than the AGA controls., (© 2015 S. Karger AG, Basel.)

Aim: The study investigated early postnatal vital signs in very low birthweight (VLBW) infants who later developed patent ductus arteriosus (PDA). We hypothesised that the early postnatal course of vital signs and blood gas variables might differ between infants whose PDA closed spontaneously, those who responded to ibuprofen and those who later required PDA ligation., Methods: We analysed computerised records of VLBW infants born <28 weeks of gestational age, including vital signs, arterial pH values and echocardiographic data from the first postnatal days., Results: In total, 104 infants were included in the study. In the group of infants born <26 weeks of gestational age and requiring ibuprofen for PDA (n = 34), 12 infants ultimately required surgical ligation. Infants requiring ligation showed significantly lower oxygen saturation (p = 0.019), mean blood pressure (p = 0.034) and higher heart rate fluctuation ranges (p = 0.040) in the first five postnatal days than those who responded to ibuprofen. In multivariable logistic regression analysis, lower pH values in the first 48 h predicted the subsequent requirement for ligation independent of gestational age (p = 0.004)., Conclusion: Patients <26 weeks of gestational age requiring PDA ligation showed significant differences in the course of vital signs and pH during the first days of life., (©2014 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Aim: The aim of this study was to measure the brain activity of preterm infants treated with caffeine citrate and doxapram for preterm apnoea, using amplitude-integrated electroencephalography (aEEG), to identify any adverse effects on cerebral function., Methods: We analysed the aEEG tracings of 13 preterm infants <30 weeks of gestation before, during and after doxapram treatment, with regard to background activity (percentages of continuous and discontinuous patterns), occurrence of sleep-wake cycling and appearance of electrographic seizure activity. They were also compared with 61 controls without doxapram treatment., Results: During doxapram treatment, aEEG tracings showed an increase in continuous background activity (19 ± 30% before treatment, 38 ± 35% during treatment) and a decrease in discontinuous patterns. In addition, they showed more frequent electrographic seizure activity (0% before treatment, 15 ± 37% during treatment) and less frequent sleep-wake cycling (92 ± 27% before treatment, 85 ± 37% during treatment) could be observed. These results were confirmed when compared to the control group., Conclusion: Doxapram treatment influences aEEG in preterm infants, showing higher percentages of continuous activity as well as more electrographic seizure activity and less sleep-wake cycling. It should, therefore, be used with caution in very preterm infants., (©2014 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Background: Intravenous sildenafil treatment has recently shown promising results and good tolerability in the treatment of refractory pulmonary hypertension (PH) in term and near-term neonates, while comparable data in preterm infants are still lacking. However, for critically ill preterm infants suffering from PH refractory to conventional treatment, sildenafil may represent a last treatment resort., Patients and Methods: We reviewed the records of 6 critically ill extremely preterm infants who had suffered from PH refractory to conventional treatment and had obtained intravenous sildenafil after careful consideration as ultima ratio treatment., Aim: To describe the responses to sildenafil in terms of hemodynamic and respiratory changes during treatment and outcome., Results: 4/6 patients showed resolution of severe PH with full reversal of ductal shunt direction into pure left-to-right shunt within 82 ± 35 h after sildenafil start. Remarkably, 2/6 patients developed pulmonary hemorrhage at a time point when significant improvement of PH had already taken place, both of them survived. Overall 4/6 patients died, two deaths were related to treatment-refractory PH., Conclusion: Intravenous sildenafil treatment seems effective in improving severe PH and hemodynamic instability in extremely preterm infants with refractory PH. Pulmonary hemorrhage may represent a distinct adverse effect of sildenafil treatment in these patients, presumably due to sudden reversal of ductal shunt. Accordingly, sildenafil should be restricted to most severe and refractory cases in this population., (© Georg Thieme Verlag KG Stuttgart · New York.)

Aim: To investigate gender-related differences in amplitude-integrated electroencephalography (aEEG) associated with neurodevelopmental outcome at 3 years., Methods: Preterms born <30 weeks' gestational age between 2000 and 2002 were prospectively included. aEEGs obtained within the first 2 weeks of life were classified according to aEEG composite scores - including background pattern, sleep-wake cycling (SWC) and seizure activity. Neurodevelopmental outcome was assessed at 3 years of age., Results: Neurodevelopmental outcome data was available for 148 of 264 eligible infants - 64 showed a normal outcome and 84 an impaired outcome. A logistic regression model revealed a significant independent influence of IVH, analgetic/sedative/anticonvulsant medication, gestational age and gender on aEEG composite scores. Odds ratios for having an abnormal aEEG composite score within the first 2 weeks of life for 'female sex', 'no IVH', 'no medication' and 'gestational age' were calculated. aEEG did not differ between males and females with normal outcomes. In patients with abnormal outcome, however, male preterms showed more burst-suppression patterns and less SWC when compared with female preterms., Conclusion: Being male with an abnormal outcome at 3 years of age is reflected by a less mature early aEEG when compared with the one of females. This association is independent of IVH and medication and was less evident with increasing gestational age., (©2013 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Objective: Intraventricular haemorrhage is still the most common cause of brain lesion in preterm infants and development of a posthaemorrhagic ventricular dilatation (PHVD) can lead to additional neurological sequelae. Flash visual evoked potentials (fVEP) and amplitude-integrated electroencephalography (aEEG) are non-invasive neurophysiological monitoring tools. The aim of the study was to evaluate fVEPs and aEEGs in preterm infants with progressive PHVD prior to and after neurosurgical intervention for cerebrospinal fluid removal and to correlate the findings with severity of ventricular dilatation., Design: fVEPs and aEEGs were performed weekly in infants with developing PHVD. As soon as the ventricular index reached the 97th percentile recordings were performed twice a week., Methods: 17 patients admitted to the neonatal intensive care unit of the Medical University of Vienna who developed progressive PHVD were evaluated using fVEP and aEEG until and after reduction of intracranial pressure by placement of an external ventricular drainage., Results: In all 17 cases (100%) wave latencies of fVEP increased above normal range and aEEG showed increased suppression in 13 patients (76%) with increasing ventricular dilatation. Both methods showed normalisation of patterns mostly within a week of successful therapeutic intervention (mean 8.5 days). Both changes in fVEP latencies and aEEG background patterns were detected before clinical signs of elevated intracranial pressure occurred. In only 10 patients (58.8%) ventricular width exceeded the 97th percentile+4 mm., Conclusions: fVEP and aEEG are useful additional tools for the evaluation of preterm infants with progressive PHVD.

Background: A new mode of surfactant administration without intubation - less invasive surfactant administration (LISA) - has recently been described for premature infants., Objective: We report single-center outcome data of extremely premature infants who have been managed by LISA in our department. Mortality and morbidity rates of the cohort were compared to historical controls from our own center and to data of the Vermont-Oxford Neonatal Network (VONN)., Patients and Methods: All infants born at 23-27 weeks' gestational age during 01/2009 and 06/2011 (n = 224) were managed by LISA and included in the study group., Results: LISA was tolerated by 94% of all infants. 68% of infants stayed on continuous positive airway pressure on day 3. The rate of mechanical ventilation was 35% within the first week and 59% during the entire hospital stay. Compared to historical controls, we found significantly higher survival rates (75.8 vs. 64.1%) and significantly less intraventricular hemorrhage (IVH) (28.1 vs. 45.9%), severe IVH (13.1 vs. 23.9%) and cystic periventricular leukomalacia (1.2 vs. 5.6%); only persistent ductus arteriousus (PDA) (74.7 vs. 52.6%) and retinopathy of prematurity (ROP) (40.5 vs. 21.1%) occurred significantly more often. Compared to VONN data, we found significantly less chronic lung disease (20.6 vs. 46.4%), severe cerebral lesions (IVH 3/4 + cystic PVL; 9.4 vs. 16.1%) and ROP (all grades) (40.5 vs. 56.5%); only PDA (74.7 vs. 63.1%) and severe ROP (> grade 2) (24.1 vs. 14.1%) occurred significantly more often in our cohort., Conclusion: Surfactant can be effectively and safely delivered via LISA and this is associated with low rates of mechanical ventilation and various adverse outcomes in extremely premature infants., (Copyright © 2013 S. Karger AG, Basel.)

Objective: The purpose of this study was to determine the risk of poor perinatal outcome in normal karyotype second-trimester fetuses with the sonographic finding of isolated echogenic bowel., Method: Medical records, ultrasonographic findings and outcome details were reviewed for 97 cases of isolated fetal echogenic bowel, after excluding cases of aneuploidy and major congenital anomalies, and compared with a cohort of 400 fetuses without pathologic intra-abdominal findings., Results: The incidence of echogenic bowel during the 14-year study period was 0.8%. Eighty (82.5%) pregnancies resulted in healthy, live-born infants. Congenital infection and cystic fibrosis was reported in 6.2% and 4.4%, respectively. The incidence of intrauterine growth restriction and intrauterine fetal demise was significantly higher in the group of isolated echogenic bowels compared with the control group (9.9% versus 1.3%, p ≤ 0.001; 8.9% versus 0.5% p ≤ 0.001)., Conclusion: Echogenic bowel is a risk factor for an adverse pregnancy outcome, even in normal karyotype fetuses without congenital anomalies. This information should be considered when counseling patients after midtrimester echogenic bowel is diagnosed., (© 2012 John Wiley & Sons, Ltd.)

Objective: To determine whether enteral application of the osmotic contrast agent Gastrografin accelerates complete meconium excretion and improves feeding tolerance in very low birth weight infants., Methods: This study was a stratified, randomized, placebo-controlled trial in premature infants with a birth weight <1500 g and a gestational age <32 weeks who received 3 mL/kg Gastrografin diluted 1:3 with water within their first 24 hours of life, or placebo., Results: Passage of last meconium occurred after a median of 7 days (95% confidence interval: 6-9 days, n = 39) in the intervention group and after 8 days (95% confidence interval: 7-10 days, n = 39) in the control group (P = .61); however, Gastrografin application was associated with a 7.5-day shorter time to full enteral feedings, a 24-day shorter stay in the NICU, and a 17-day reduction in the overall hospital stay in the intervention group compared with the control group. A numerically higher incidence of necrotizing enterocolitis (21%) was observed in the intervention group, however., Conclusions: Gastrografin application did not accelerate meconium evacuation, but the higher stool frequency during the first week of life had a beneficial effect on the time to full enteral feedings and later hospital stay; however, it may increase the necrotizing enterocolitis risk. Further investigations are needed with modified protocols, and the prophylactic use of Gastrografin cannot currently be recommended without further clinical trials.