Epidemiologic characteristics of amniotic band sequence with limb malformations without body wall defect: data from the Polish Registry of Congenital Malformations

Abstract Amniotic Band Sequence (ABS) is a rare disruptive condition, with a variable spectrum of congenital defects caused by fibrous bands emerging as a result of amniotic rupture in the first trimester of gestation. Several factors, such as young parental age, primigravidity, febrile maternal illness, and drug use in the first trimester, were postulated to have substantial influence on ABS prevalence rate. We aimed our study to determine the prevalence of ABS with limb defects, but no body wall affectation, in a Polish population. We also examined the influence of different parental, gestational and environmental factors on the ABS prevalence value, and assessed the rate of gestational complications associated with this disorder. Among 1 706 639 births surveilled between 1998 and 2005, 36 liveborn infants with ABS-L were reported to the Polish Registry of Congenital Malformations, giving a global prevalence for a Polish population of 1 per 47 619 livebirths. We found that young maternal age, young paternal age, and primigravidity significantly increase the risk of ABS-L, when their effect was analyzed independently. However, because of a close relationship of these variables, we analyzed their mutually adjusted effect using conditional logistic regression models, and found that young maternal age proved the strongest risk factor for ABS-L (p = 0.0508). The condition was also more prevalent in infants with low birthweight (OR = 5.71; p < 0.0001). Since gestational complications are often relevant to maternal age and birth order, we introduced an adjustment for these variables, and found that respiratory tract infections and vaginal bleeding/spotting convey approximately fourfold increased risk of ABS-L (OR = 3.72/p = 0.0058 and OR = 3.70/p = 0.0014 respectively)

Balanced cross section as a tool in the tectonic interpretation on the basis of the Polish Flysch Carpathians

Przekroje zbilansowane i odrestaurowane pozwalają na wielorakie badania budowy Ziemi. Obecnie do wszelkich badań wykorzystuje się liczne programy komputerowe. Światowym liderem w geologii strukturalnej jest firma Midland Valley, która stworzyła oprogramowanie Move, oferujące szeroki pakiet programów do łączenia i interpretacji danych geologicznych. Przekroje zbilansowane wykorzystali Nemčok et al. (2000) i Gągała et al. (2012) do obliczenia skrócenia orogenicznego Karpat fliszowych, Gągała także zaproponował przebieg ewolucji płn-wsch części Karpat Zewnętrznych. Nemčok & Henk w 2006, badali zależność między budową Karpat a migracją węglowodorów. W 2014 Dudek et al. wykorzystując metodę bilansowania przekroi zaproponowali nowy wygląd jednostki skolskiej. Probulski et al. (2009) wykorzystali tę metodę do weryfikacji autentyczności budowy jednostki śląskiej.The balanced and restored cross sections allow for multiple studies of the Earth construction. Currently, many computer programs are used for research. The world leader in structural geology is Midland Valley, which has developed the Move software, which offers a wide range of programs for combining and interpreting geological data. The balanced cross sections were used by Nemčok et al. (2000) and Gagala et al. (2012) to calculate orogenic shortening of Flysch Carpathians, Gagala also proposed the course of evolution of the eastern part of the Outer Carpathians. In 2006 Nemčok & Henk explored the relationship between the Carpathian structure and the hydrocarbon migration. In 2014 Dudek et al. using the method of balancing cross section, they suggested a new appearance of the Skole unit. Probulski et al. (2009) used this method to verify the authenticity of construction of the Silesian unit

Clear distinction between CAC and CMC revealed by high-resolution NMR diffusometry for a series of bis-imidazolium gemini surfactants in aqueous solutions

The aggregation behavior in the transition region was studied for a series of dicationic surfactants 3,3′-[α,ω-(dioxaalkane)]bis(1-dodecylimidazolium)dichlorides with varied spacer length from two to twelve carbon atoms. We employed Nuclear Magnetic Resonance diffusometry and Bayesian DOSY analysis to obtain the aggregate size distribution in the transition region. The critical concentrations CC were independently obtained from surface tension, electric conductivity, UV-Vis and NMR methods. The micelle aggregation numbers were estimated from the self-diffusion coefficients and were independently confirmed using steady-state fluorescence quenching. The morphology of the aggregates was characterized by small-angle scattering of synchrotron radiation and molecular dynamics simulations. The obtained CC values are identified as critical aggregation concentrations CAC. A broad transition region was observed, and stable micelles were obtained at much higher concentrations than CAC. The accurate CMC values could not be identified for the systems in the study. We indicated that the distribution of aggregate size becomes small and the system becomes homogeneous at much larger concentrations than CAC (typically 15–20 mM). The existence of a slow exchange between two environments, an aggregate and aqueous environment, was confirmed by 1H NMR and 2D HSQC NMR spectroscopy.KS EUSMI – European infrastructure for spectroscopy, scattering and imaging of soft matter, contract number GA731019, funded under H2020-EU.1.4.1.2.RIA, MK National Science Centre (Poland

Genetic and Environmental Risk Factors for Isolated Hemangiomas in Infants

The goal of this analysis is to identify risk factors for infantile hemangiomas (IH) to better delineate hemangioma predisposition. We analyzed live birth children with isolated cutaneous hemangioma that were reported to the Polish Registry of Congenital Malformations from across Poland between the years 1998 and 2016. Lower birthweight and gestational age were the most significant risk factors associated with IH. We also observed a trend for a higher risk of IH with a lower level of maternal and paternal education. Moreover, mothers with IH have a higher probability of having a child with IH compared to fathers. However, this association is only present when the child is female. Similarly, a higher risk of hemangioma in a female child is found among mothers having relatives of the first degree with IH, compared to fathers with a similar pedigree. Our results suggest the role of exogenous factors in the etiology of IH. The analysis of familial cases suggests a multifactorial model of inheritance. The study indicates that female gender is an important risk factor for the expression of familial IH. Potential interaction of genetic risk factors with exposure to female sex hormones may play a role in the development of IH

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: Dolnośląskie (1.7/10 000 live births, p = 0.0052) and Śląskie (1.9/10 000 live births, p < 0.0001). Furthermore, within the region of Dolnośląskie, 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