Neonatal sepsis is a life-threatening condition associated with significant morbidity and mortality. Sepsis-induced coagulopathy is a well-recognized entity, signifying the strong cross-talk between inflammation and coagulation. The aim of the present study was to compare the coagulation profile between the acute phase of sepsis and recovery in term and preterm neonates. Additional comparisons to healthy neonates were undertaken. Levels of clotting, anti-clotting factors and ADAMTS-13 (A disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF), were measured in 16 term and preterm neonates in the acute phase of infection and following recovery, as well as in 18 healthy neonates. Clotting times were prolonged, while levels of particular clotting factors were lower in the acute phase of infection compared to controls and recovery. On the other hand, levels of fibrinogen, factor VIII (FVIII) and VWF were significantly higher in the acute phase in comparison to controls and recovery, while they remained persistently higher in the infection group compared to controls. In regard to the anticlotting mechanism, a clear suppression was observed in septic neonates. ADAMTS-13 levels were significantly lower in the acute phase of infection in comparison to controls and recovery (p = 0.015 and 0.004, respectively), while a trend toward superimposed normalization was demonstrated post infection, as higher ADAMTS-13 levels were measured in recovered neonates compared to controls (p = 0.002). The coagulation profile is considerably deranged in neonatal sepsis. ADAMTS-13 deficiency in septic neonates is a novel finding with promising future implications, as ADAMTS-13 substitution may serve as a useful therapeutic option in neonatal sepsis, prompting further investigation in future studies.

Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.

The concept of ‘developmental hemostasis’ from birth to infancy and onwards to childhood and adulthood was introduced in the 1980s and is used to indicate the fundamental discrepancies of hemostatic mechanism between children and adults. The underlying differentiations are more pronounced in term and even more in preterm neonates. Hemostatic alterations tend to improve throughout childhood and adolescence but still imply a great example of the basic concept that children do not simply represent small adults. Many neonatal coagulation disorders lead to severe morbidities, such as intraventricular hemorrhage and intracerebral infarct, with critical consequences on long-term neurodevelopmental outcome. As the limits of viability have decreased and many preterm and severely affected neonates survive and grow up, a broad understanding of hemorrhagic and thrombotic complications in neonates is very important, in order to provide prompt identification and treatment. Coagulation abnormalities are usually induced by specific pathophysiologic disorders, and neonatal sepsis is a significant trigger of hemostatic derangement. Despite the initial protective role of coagulation activation during the early stages of sepsis, ultimately hemostatic abnormalities exert a substantial impact on clinical outcome and prognosis. This review explores developmental aspects of coagulation, particularly in relation to neonatal sepsis.

Extracellular vesicles (EVs) represent nanoparticles released by numerous cell types and exerting distinct biological roles. Extracellular vesicles are enclosed in a lipid layer and contain proteins, lipids, nucleic acids, carbohydrates or even mitochondria. The fundamental utility of EVs is intercellular communication, while their implication in various biological processes, such as inflammation and hemostasis, is well-documented. Growing evidence indicates the significant usefulness of EVs as biomarkers in certain prematurity-related diseases, like bronchopulmonary dysplasia (BPD) and necrotizing enterocolitis (NEC). Moreover, many in vitro and in vivo studies have been undertaken in order to explore the potential therapeutic impact of EVs in BPD, NEC and retinopathy of prematurity (ROP), some of them yielding promising results. The present narrative review aims towards the summarization of the existing data on the application of EVs in diverse neonatal disorders, such as BPD, NEC, ROP, sepsis, multi-organ failure, coagulopathy and brain injury. The widespread investigation of EVs in neonatology and their potential implementation in clinical practice may lead towards a revolutionary approach and management of neonatal diseases, as well as an innovative perception of prematurity.