Cardiac biomarkers and left ventricular systolic function in former very preterm infants and term controls at preschool age.

Introduction: Due to improvements in perinatal care, survival rates of preterm infants have improved during the last decades. However, these infants remain at risk of developing cardiovascular sequelae later in life. This study aimed to investigate the cardiac biomarkers and left ventricular systolic function in former preterm infants in comparison with term controls at preschool age. Methods: The study included children aged 5-7 years old born below 32 weeks of gestational age. The control group consisted of same-age children born at term. Basic data of study participants were collected using questionnaires and follow-up databases. During the study visit, we recorded anthropometric data and blood pressure readings, determined high-sensitive cardiac troponin T (hs-cTnT) and N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) concentrations, and calculated fractional shortening (FS) and left ventricular mass (LVM). Results: Term-born (n = 25; median gestational age, 40.1 weeks) compared with preterm-born infants (n = 80; median gestational age 29.6 weeks) showed no significant differences in the median concentration of hs-cTnT [median, 3.5 (IQR 3.5; 3.5) vs. 3.5 (3.5; 3.5) ng/L, p = 0.328] and the median concentration of NT-pro-BNP [median, 91.0 (IQR 40.8; 150.3) vs. 87.5 (50.1; 189.5) ng/L, p = 0.087]. FS and LVM/LVMI were not significantly different between the two groups. Conclusion: At preschool age, we observed no significant differences in cardiac biomarkers and left ventricular systolic function in preterm infants. Further studies are warranted to explore the potential of cardiac biomarkers as a prognostic tool for subclinical cardiac alterations after preterm birth.

Umbilical venous catheter- and peripherally inserted central catheter-associated complications in preterm infants with birth weight < 1250 g : Results from a survey in Austria and Germany.

BACKGROUND AND OBJECTIVE: Umbilical venous catheters (UVC) and peripherally inserted central catheters (PICC) are commonly used in preterm infants but have been associated with a number of serious complications. We performed a survey in Austria and Germany to assess the use of UVCs and PICCs in preterm infants with a birth weight < 1250 g and associated rates of catheter-related adverse events. METHODS: Electronic survey of participating centers of the NeoVitaA trial. Main outcome parameter was the reported rates of UVC- and PICC-associated complications (infection, thrombosis, emboli, organ injury, arrhythmia, dislocation, miscellaneous). RESULTS: In total, 20 neonatal intensive care units (NICU) providing maximal intensive care in Austria and Germany (level I) were contacted, with a senior neonatologist response rate of 12/20 (60%). The reported rates for UVC with a dwell time of 1-10 days were bacterial infection: 4.2 ± 3.4% (range 0-10%); thrombosis: 7.3 ± 7.1% (0-20%); emboli: 0.9 ± 2.0% (0-5%); organ injury: 1.1 ± 1.9% (0-5%); cardiac arrhythmia: 2.2 ± 2.5% (0-5%); and dislocation: 5.4 ± 8.7% (0-30%); and for PICCs with a dwell time of 1-14 days bacterial infection: 15.0 ± 3.4% (range 2.5-30%); thrombosis; 4.3 ± 3.5% (0-10%); emboli: 0.8 ± 1.6% (0-5%); organ injury: 1.5 ± 2.3% (0-5%); cardiac arrhythmia: 1.5 ± 2.3% (0-5%), and dislocation: 8.5 ± 4.6% (0-30%). CONCLUSION: The catheter-related complication rates reported in this survey differed between UVCs and PICCs and were higher than those reported in the literature. To generate more reliable data on this clinically important issue, we plan to perform a large prospective multicenter randomized controlled trial investigating the non-inferiority of a prolonged UVC dwell time (up to 10 days) against the early change (up to 5 days) to a PICC.

Presumed prenatal closure of foramen ovale and persistent pulmonary hypertension of the newborn.

Prenatal closure of foramen ovale without CHD is a rarely reported entity. Therefore, clinical and echocardiographic findings are poorly defined in these patients. We report a patient with prenatal closure of foramen ovale that presented with severe pulmonary hypertension of the newborn and left ventricular failure. Judicious management strategies were utilised to successfully treat both life-threatening conditions.

Connatal Urinary Ascites in a Female Preterm.

BACKGROUND: Connatal urinary ascites is rare in females without associated malformations and occurs following bladder rupture. CASE PRESENTATION: A female very preterm was delivered by caesarean section because of abnormal Doppler findings. The mother suffered from viral pneumonia requiring intensive care in the third trimester of pregnancy. Serial fetal ultrasound examinations showed a megacystis and ascites. Postnatally, pronounced isolated ascites was drained and its urinary nature was confirmed. The bladder leak was demonstrated when blue dye, instilled via a Foley catheter, appeared in the ascitic drain. After removal of the catheter spontaneous micturition was unremarkable. A micturating cystourethrogram showed spontaneous closure of the bladder leak. CONCLUSION: The female infant experienced fetal bladder rupture and connatal urinary ascites due to maternal pneumonia and intensive care. The use of blue dye is an effective alternative method to any contrast media radiography and should be considered, especially in very preterm infants.

Amplitude-integrated electroencephalography can predict neurodevelopmental outcome at 12 months of corrected age in very preterm infants.

AIM: It is difficult to find diagnostic tools than can reliably predict neurodevelopmental outcomes in very preterm infants in clinical practice. This study evaluated whether amplitude-integrated electroencephalography predicted neurodevelopmental outcome in preterm infants when they reached 12 months of corrected age. METHODS: Between October 2007 and December 2013, we studied 232 preterm infants (51% male) at Innsbruck Medical University Hospital, Austria. Their mean birthweight was 1264 g, and their mean gestational age was 29.5 weeks. Amplitude-integrated electroencephalography was evaluated using the Burdjalov score, and outcomes were assessed using the Bayley Scales of Infant Development - Second Edition. RESULTS: The cohort was divided into three subgroups: 154 infants with normal outcomes, 53 infants with moderate delays and 25 infants with severe delays. The amplitude-integrated electroencephalography Burdjalov scores were lower in infants with moderate delays than in infants with normal outcomes and even lower in infants with severe delays. The highest area under the curve (0.776) for the Burdjalov score was at 18-24 hours of life. CONCLUSION: Our study confirmed the predictive value of amplitude-integrated electroencephalography and showed that this needed to be carried out early in life to provide reliable information on neurodevelopmental outcomes in very preterm infants.

Breast milk feeding in infants with inherited metabolic disorders other than phenylketonuria - a 10-year single-center experience.

BACKGROUND: Published data on breast milk feeding in infants suffering from inherited metabolic disorders (IMDs) other than phenylketonuria (PKU) are limited and described outcome is variable. OBJECTIVE: We aimed to evaluate retrospectively whether breastfeeding and/or breast milk feeding are feasible in infants with IMDs including organic acidemias, fatty acid oxidation disorders, urea cycle disorders, aminoacidopathies or disorders of galactose metabolism. METHODS: Data on breastfeeding and breast milk feeding as well as monitoring and neurological outcome were collected retrospectively from our database of patients with the mentioned IMD, who were followed in our metabolic center within the last 10 years. RESULTS: Twenty patients were included in the study, who were either breast fed on demand or received expressed breast milk. All the infants were evaluated clinically and biochemically at 2-4-week intervals, with weight gain as the leading parameter to determine metabolic control. Good metabolic control and adequate neurological development were achieved in all patients but one, who experienced the only metabolic crisis observed within the study period. CONCLUSION: Breast milk feeding with close clinical and biochemical monitoring is feasible in most IMD and should be considered as it offers nutritional and immunological benefits.

ALG8-CDG: novel patients and review of the literature.

BACKGROUND: Since 1980, about 100 types of congenital disorders of glycosylation (CDG) have been reported representing an expanding group of inherited disorders. ALG8-CDG (= CDG-Ih) is one of the less frequently reported types of CDG, maybe due to its severe multi-organ involvement with coagulation disturbances, edema, massive gastrointestinal protein loosing enteropathy, cataracts, and often early death. We report three additional patients, provide an update on two previously reported, and summarize features of ten patients reported in literature. RESULTS: Of 15 ALG8-CDG patients, three were homozygous and 12 compound heterozygous. There were multiple prenatal abnormalities in 6/12 patients. In 13/15, there were symptoms at birth, 9/15 died within 12 months. Birth weight was appropriate in 11/12, only one was small for gestational age. Prematurity was reported in 7/12. Hydrops fetalis was noticed in 3, edemas in 11/13; gastrointestinal symptoms in 9/14; structural brain pathology, psychomental retardation, seizures, ataxia in 12/13, muscle hypotonia in 13/14. Common dysmorphic signs were: low set ears, macroglossia, hypertelorism, pes equinovarus, campto- and brachydactyly (13/15). In 10/11, there was coagulopathy, in 8/11 elevated transaminases; thrombocytopenia was present in 9/9. Eye involvement was reported in 9/14. CDG typical skin involvement was reported in 8/13. CONCLUSION: In ALG8-CDG, isoelectric focusing of transferrin in serum or plasma shows an abnormal sialotransferrin pattern. The diagnosis is confirmed by mutation analysis in ALG8; all patients reported so far had point mutations or small deletions. The prognosis is generally poor. Thus, a timely and correct diagnosis is important for counselling.

Early sudden unexpected death in infancy (ESUDI)--three case reports and review of the literature.

AIM: Early sudden unexpected death in infancy (ESUDI) is a rare cause of death occurring in newborns during the first hours of life. Our aim was to find additional data for the identification of risk factors and establishment of prevention strategies. METHODS: We describe three cases of ESUDI and give an overview of the literature. RESULTS: ESUDI was observed in term babies with normal birth weight after good postnatal adaptation within the first four hours of life. Maternal age was between 29 and 36 years, mothers were primiparous and of normal weight. All three events occurred during unobserved early skin-to-skin contact. Autopsy and post-mortem metabolic screening revealed no cause of death. A search of the MEDLINE and Web of Science (Thomson Reuters) databases brought to light 132 cases of ESUDI reported in the literature from 1985 to 2012. Concurrent with our observations, first parity and unobserved early skin-to-skin contact seem to be prime predictors of ESUDI. Other pre-described risk factors such as mode of delivery, birth weight, mother's age and body mass index were not seen in our patients. CONCLUSION: Close observation during the first hours of life is essential and can be life-saving, especially during early skin-to-skin contact.

Early risk predictors for impaired numerical skills in 5-year-old children born before 32 weeks of gestation.

AIM: To unravel risk predictors for impaired numerical skills at 5 years of age in a population-based cohort of very preterm infants. METHODS: Between January 2003 and August 2006, we prospectively enrolled all infants born in Tyrol with <32 weeks of gestation. A total of 161 of 223 preterm infants (participation rate 72.2%) had a detailed examination at 5 years of age including cognitive assessment (Hannover-Wechsler Intelligence Test for preschool children, third edition (HAWIVA-III) or Snijders-Oomen Nonverbal Intelligence Test (SON-R)). In 135 children, numerical abilities were assessed with the dyscalculia test TEDI-MATH. The association between pre- and postnatal factors and impaired numerical skills was analyzed by means of logistic regression analysis. RESULTS: Dyscalculia test showed delayed numerical skills (TEDI-MATH Sum T-score <40) in 27 of 135 children tested (20.0%). In half of the children tested, delayed numerical abilities were related to lower IQ scores. Smoking in pregnancy, intracerebral haemorrhage and chronic lung disease were predictive of delayed numerical skills at 5 years of age in the multivariate analysis. CONCLUSION: This study identified risk predictors for impaired numerical skills in preterm infants. Our data support the role of both pre- and perinatal factors in the evolution of mathematical deficits.

The effect of sex on outcome of preterm infants - a population-based survey.

AIM: To provide comprehensive data on potential sex differences in maternal and neonatal characteristics, short-term morbidity and neurodevelopmental outcome within an entire geographically determined collective of infants born at a gestational age <32 weeks. METHODS: Between 2003 and 2008, we prospectively enrolled all infants born in Tyrol at <32 weeks of gestation; the association between sex, and a wide set of pre- and postnatal factors, post-discharge morbidity and neurodevelopmental outcome was analysed. RESULTS: Girls less frequently suffered from early-onset sepsis than boys (p = 0.030). After adjustment for multiple corrections (Bonferroni's p = 0.003), no sex differences were seen within any maternal or neonatal parameter. Analysis of morbidity revealed a higher readmission rate in boys (p < 0.0001), which was primarily caused by a greater incidence of respiratory problems (p = 0.003). Boys did not show a greater adverse neurodevelopmental outcome at the age of 12 or 24 months. CONCLUSION: Parents of boys should be prepared for a potentially higher frequency of readmission after initial discharge, but our data currently give no reason for parents of boys to be disproportionately anxious about their neurodevelopmental outcome. Whether boys also enjoy a rosy prognosis for developmental outcome at school age remains to be elucidated.

Rehospitalization in the first 2 years of life in children born preterm.

AIM: Aim of the study is to investigate the frequency of and predictors for rehospitalization within the first 2 years of life among preterm infants. METHODS: All children born before 32 weeks of gestation in Northern Tyrol between January 2003 and July 2008 were prospectively enrolled. Data on rehospitalizations were obtained from hospital admission records. The association between candidate risk factors and readmission was analysed by means of logistic regression analysis. RESULTS: In the first and second years of life, 151 and 93 of 377 children (40.1% and 24.7%), respectively, were readmitted to one of the hospitals in Northern Tyrol. The most common causes of rehospitalization were respiratory disorders, accounting for 42.1% and 47.4% of total readmissions in the first and second years of life. Chronic lung disease (CLD), male sex and smoking in pregnancy were risk conditions relevant to readmission in the first year of life, but only CLD in the second year. CONCLUSION: Infants born before 32 weeks of gestation have a high risk of rehospitalization with respiratory illness significantly contributing to postdischarge morbidity. Neonatal intensive care should aim to further improve respiratory health in preterm infants, and adequate follow-up services must be offered.

Need for quality control for aEEG monitoring of the preterm infant: a 2-year experience.

AIM: To establish and apply a questionnaire for the evaluation of amplitude-integrated electroencephalogram (aEEG) with respect to practicability and feasibility in the NICU, to reveal strategies for improvements in daily use and to investigate the level of staff know-how with regard to performance and evaluation of bedside aEEG for the purpose of quality control. METHODS: Amplitude-integrated electroencephalogram was routinely applied in preterm infants below 32 weeks of gestational age for a period of 2 years. Practicability, feasibility and the level of know-how with regard to aEEG were assessed using a multiple-choice questionnaire. RESULTS: Major problems in the use of aEEG in preterm infants were identified as time needed for placement and frequent readjustment of electrodes. Quality control showed the following problems: seizure activity was correctly diagnosed in 60.0%, discontinuous (DC) background in 45.5% and burst suppression (BS) in 41.8% of patients. Overall, BS and DC were the patterns most frequently interpreted incorrectly. CONCLUSION: Amplitude-integrated electroencephalogram is frequently used in neonatology. In preterm infants with several challenges, repeated theoretical and practical trainings as well as quality control are needed in order for aEEG to become a routinely used monitoring instrument in daily preterm care.