Geospatial clustering of gastroschisis in Poland: Data from the Polish Registry of Congenital Malformations (PRCM).

OBJECTIVES: The aims of this study were: to evaluate the prevalence of abdominal wall defects in the Polish population, to analyze temporal trends in the prevalence, to identify areas (clusters) of high risk of abdominal wall defects, and to characterize, with respect to epidemiology, children with abdominal wall defects and their mothers in the area defined as a cluster. MATERIAL AND METHODS: We used isolated congenital malformations (gastroschisis Q79.3 and omphalocele Q79.2 according to the International Statistical Classification of Diseases and Related Health Problems, 10th revision (ICD-10, the extended version)) data reported to the Polish Registry of Congenital Malformations (PRCM) over the years 1998- 2008 based on the population of 2 362 502 live births. We analyzed 11 administrative regions of Poland with complete epidemiologic data. RESULTS: Of 11 regions, 2 had a significantly higher standardized prevalence of isolated gastroschisis: Dolnoslaskie (1.7/10 000 live births, p = 0.0052) and Slaskie (1.9/10 000 live births, p < 0.0001). Furthermore, within the region of Dolnoslaskie, we defined a clear prevalence of the isolated gastroschisis cluster (p = 0.023). We comprehensively examined demographic and socio-economic risk factors for abdominal wall defects in this area, and we found that these factors failed to account for the cluster. CONCLUSIONS: We identified a distinct prevalence cluster for isolated gastroschisis, although a precise reason for the disease clustering in this region remains unknown. Cluster identification enables more focused research aimed at identification of specific factors with teratogenic effects.

USING THE AUTOMATED FIO2- SPO2 CONTROL IN NEONATAL INTENSIVE CARE UNITS IN POLAND. A PRELIMINARY REPORT.

AIM: Analysis of the way in which a new method of implementing the automated control of oxygen therapy during respiratory support is applied in newborns with respiratory failure. MATERIAL, METHODS AND RESULTS: The AVEA-CLiO2 ventilator with automated FiO2- SpO2 control was used in our study of 121 newborns conducted between February 2014 and January 2015 in five neonatal intensive care units. A web-based database was used to gather information entered concurrently with using the FiO2- SpO2 control system. This included demographics, clinical status, clinical indications, as well as objective and subjective experience. Among the 121 newborns 94 were preterm and 27 were near-term (33-36 hbd). The primary indication for using the system was "routine management" of FiO2 during respiratory support and it was generally initiated within the first 2 days of life. Many of the newborns were managed with the system for more than a week. The control range was usually 90%-95% SpO2, though sometimes it was lower or wider. The control range was not related to the newborn's maturity or indication for use. The perception of more "frequent and persistent" SpO2 alarms was lower when the alarms were set loosely. There were no reports of the system not working effectively. CONCLUSIONS: We expect this first report of the routine use of automated FiO2- SpO2 control to be useful not only to other centers in Poland but also to all those adopting this important new technology. Our registry continues and we expect to have an update when we have experience with 1000 infants. Carefully controlled trials are also needed to refine the optimum use of automated FiO2- SpO2 control and to quantify its impact on neonatal outcomes.

Spatial and temporal clustering of isolated cleft lip with or without cleft palate in Poland.

BACKGROUND: Geographic variation in the prevalence of isolated cleft lip with or without cleft palate may be due to exogenous environmental factors or genetic variation. In this study, we aim to evaluate the prevalence of isolated cleft lip with or without cleft palate in Polish urban and rural environments in order to identify geographic areas with high prevalence (defect clusters). METHODS: We use all cases of congenital malformations reported to the Polish Registry of Congenital Malformations in the years 1998-2008 from the total population of 2,362,502 births. RESULTS: We detect a strong signal of increased prevalence of isolated cleft lip with or without cleft palate in a single region of Poland, the Dolnoslaskie voivodeship. Furthermore, we demonstrate a statistically significant prevalence differences between the urban and rural areas within this region. Through our comprehensive spatiotemporal analysis, we precisely define the cluster of the highest risk that comprises the eastern part of this voivodeship.

A multicenter randomized controlled trial comparing effectiveness of two nasal continuous positive airway pressure devices in very-low-birth-weight infants.

OBJECTIVE: Many studies suggest nasal continuous positive airway pressure is an effective and relatively complication-free means of respiratory support in premature infants. However, only limited data exist regarding the practical aspects of nasal continuous positive airway pressure delivery, including the best way to provide the positive airway pressure. DESIGN: Our aim was to compare the results of treatment using two different nasal continuous positive airway pressure devices: variable flow Infant Flow and constant flow nasal continuous positive airway pressure in two different groups of very-low-birth-weight infants in a multicenter randomized controlled trial. The indication groups were elective to avoid intubation and weaning from mechanical ventilation. SETTING: Twelve leading tertiary care neonatal centers in Poland. PATIENTS: Among 276 infants (weighing between 750-1500g, with a gestational age ≤32 wks) enrolled, 51% were randomized to receive Infant Flow and 49% to receive constant flow nasal continuous positive airway pressure. MEASUREMENTS AND MAIN RESULTS: Treatment success (i.e., no need for intubation/reintubation) occurred in 75% of our patients with a nonstatistically significant advantage seen with Infant Flow. The incidence of severe nasal complications and necrotizing enterocolitis were statistically significantly lower in the infants treated with Infant Flow. In our study, factors associated with elective nasal continuous positive airway pressure failure were birth weight ≤1000 g, gestational age ≤28 wks, clinical risk index for babies score >1, and PaO(2)/FIO(2) ratio of <150. Only birth weight ≤1000 g was associated with weaning failure. CONCLUSIONS: We found fewer severe nasal complications but no statistically significant advantage in treatment success in infants assigned to Infant Flow nasal continuous positive airway pressure compared with those assigned to constant flow nasal continuous positive airway pressure treatment. Significant risk factors of treatment failure include small size, maturity, and severity of respiratory distress syndrome.

[Difficult therapeutic decision making in treatment of children with oesophageal atresia and trisomy of chromosome 18 - comments by geneticist, surgeon, neonatologist, paediatrician and anaesthesiologist]. / Trudne decyzje dotyczace leczenia noworodków z zarosnieciem przelyku oraz z trisomia chromosomu 18 - rozwazania genetyka, chirurga, neonatologa, pediatry i anestezjologa.

Oesophageal atresia is a congenital defect of alimentary tract concerning the interruption of oesophagus with or without connection with the trachea. Its incidence is 1:3000-3500 of live-born. Associated anomalies including genetic disorders occur in 50% of patients. Edwards syndrome which is trisomy of chromosome 18 with poor prognosis. The incidence of Edwards syndrome is 1:5000 of live-born. About 5% of these children live more than 1 year. The aim of this article is a retrospective analysis of the course of treatment of newborn with oesophageal atresia and Edwards syndrome and making of therapeutic decision. The authors from different medical specializations: clinical genetics, paediatric surgery, paediatrics and neonatology, paediatric intensive care and palliative medicine, have undertaken a discussion regarding surgical treatment of children with oesophageal atresia and chromosomal, lethal syndrome.

[Evaluation of diagnostic value of procalcitonin (PCT) as a marker of congenital infection in newborns]. / Ocena wartosci oznaczania prokalcytoniny jako wykladnika zakazenia wrodzonego u noworodków.

UNLABELLED: Systemic bacterial infections still remain one of the major causes of neonatal morbidity and mortality. Early detection of neonatal sepsis can be difficult, because the first signs of the disease may be unspecific and similar to symptoms of other non-infectious processes. Procalcitonin became a new, sensitive marker of bacterial infections in newborns. The aim of our study was to assess the value of PCT as a diagnostic and prognostic tool of neonatal maternofetal infections. We also tried to estimate normal ranges of PCT in uninfected newborns. MATERIAL AND METHODS: 74 newborns, born in the Department of Obstetrics and Gynaecology, University of Medicine of Wroclaw, then hospitalized in the Department of Neonatology entered the study. They were divided into 2 groups: group 1-29 neonates with recognized materno-fetal infection, group 2-45 newborns without infection. In both groups blood samples to measure PCT concentrations were obtained by venipuncture on the 1st, 2nd, 3rd, 5th and between the 10th and 14th day of life (in the group of infected neonates) Sera were stored at -40 degrees C before analysis. PCT was determined using an immunoluminometric assay (BRAHMS Diagnostica). RESULTS: Serum procalcitonin values were significantly higher in the infected group than in the uninfected neonates (p < 0.001). The most significant differences were noted on the 2nd and 3rd day of life (p < 0.0001). After the treatment had been finished, the PCT levels in both groups were not statistically different. CONCLUSIONS: PCT is a useful tool in early diagnosing and monitoring the course of early-onset infections in neonates, particularly when blood cultures obtained from neonates remain negative. The decreasing concentrations of PCT level in children treated due to infection, indicate successful treatment and may help one to take a decision on termination of antibiotic therapy.

[Role of IVIG in modern neonatology]. / Rola IVIG we wspólczesnej neonatologii.

A newborn presents capacity to respond to specific antigens, but his immune system is still immature. Deficiency of immune function in the neonate is concerned with both innate and acquired immunity and provides to higher morbidity and mortality at this age of life. Therefore many multicentral studies on this field are performed. In the first part of the paper the authors present the development of humoral response and the role of immunoglobulins in infections of fetuses and newborns. The second part contains current views on immunoglobulin therapy in these children.