Minimizing infant mortality (IM) is the primary national objective of the Russian Federation. Aim: This study aimed to analyze IM indicators in the Russian Federation and identify factors that influence the dynamics of IM. The level of leading regional indicators of socioeconomic development and provision by doctors and hospital beds in the Russian Federation was studied and compared with the similar factors in foreign countries. Materials: Official and operative reports of Rosstat for 2011-2016 were assessed. Results. The coefficient of IM was 6.0 per 1000 live births in the Russian Federation, and varied from 4.8-9.2 in federal districts. The indicator increased in 2012 in connection with the transition to a new system of newborn registration. The indicator witnessed a permanent decline in 2016 and revealed a negative correlation between the regional bulk products and the coefficient of IM and a positive correlation with the overall IM. However, the relationship among the indicators of IM and the size of the region, the number of highways, the number of patients per one hospital bed or assigned to one doctor could not be established. Conclusion. In the Russian Federation, IM is higher than that indeveloped countries; has permanent tendency to decline; correlates with regional bulk products; does not depend on transport availability, provision with doctors, and hospital beds; and is determined by the organization and the quality of medical care.
The article describes the main historical periods of the study of hemorrhagic disease of newborns. Clinical observations, describing bleeding of newborns, which occurs without visible damage, known since the seventeenth century and are found in the works of Francois Mauriceau and Georg Wolfgang Wedel. The role of the liver injury in the development of bleeding of infants noticed English physicians John Huxham (1672-1768) and John Cheyne (1777-1836). In 1871 Ludwig Grandidier conducted differential diagnosis between umbilical bleeding of neonates and the hemophilia clinic. Later I.P. Pavlov showed, that the clotting time of dog’s blood significantly prolongs when you turn off the liver from the blood circulation. In that way, partly began to understand the etiology of hemorrhagic syndrome, but the pathogenesis of this suffering, in diseases of the liver remained unknown. Charles Wendell Townsend in 1894, describing 50 cases of coagulopathy of newborns, at the first time paid attention to the connection between breastfeeding and development of bleeding and offered to call this nosological form as "hemorrhagic disease of newborns". In the late 20s of the last century Henrik Dam have identified the unknown substances from green lucerne leaves and described them as the fat-soluble vitamin K (coagulation). In 1943 H. Dam and E. Doisy received the Nobel prize for the discovery and establishment of the chemical structure of vitamin K. In 1943, a group of Soviet biochemists have synthesized a water-soluble analogue of vitamin K (K3, vicasolum), quickly introduced into clinical practice and showed their high efficiency in patients with hypovitaminosis K. In 1945 Yu.F. Dombrovskaya suggested that haemorrhagic disease of newborns caused by deficit of vitamin K. Further researching of the development of haemorrhagic disease of newborns is associated with the study of the biochemistry of the pathogenesis of the disease, identify its various forms and development of treatment techniques and prevention, depending on the identified features.
Congenital disorders of glycosylation (CDG) is a genetically heterogeneous and clinically polymorphic group of diseases caused by defects in various enzymes, the synthesis and processing of N-linked glycans or oligosaccharides into glycoproteins. Approximately half of all proteins expressed in cells are glycosylated to achieve their full functionality. Basically there are 2 variants of glycosylation: N-glycosylation and O-glycosylation. N-glycans are bound to the amide group of aspartine, whereas O-glycans are bonded to the hydroxyl group of serine or threonine. Synthesis of N-glycans occurs in 3 stages: the formation of nucleotide-linked sugars, assembly (in the cytosol and endoplasmic reticulum) and treatment (in the Golgi apparatus). Synthesis of O-glycans occurs mainly in the Golgi apparatus. The most frequently identified types of CDG are associated with a defect in the N-glycosylation pathway. CDGs are typically multisystem disorders with varying clinical manifestations such as hepatomegaly, cholestasis, liver failure, developmental delay, hypotonia, convulsions, facial dysmorphism and gastrointestinal disorders. Also histological findings showed liver fibrosis, malformation of the ducts, cirrhosis, and steatosis. CDGs typically present in the first months of life, and about 20% of patients do not survive to 5 years. The first line of CDG screening is based on the analysis of N-glycosylation of transf ferin. Exome sequencing or targeted gene panel is used for diagnosis. Several CDG subtypes are amenable to teraphy with mannose and galactose.
Improving the cardiopulmonary resuscitation quality in the delivery room is one of the most pressing issues in modern neonatology. Despite the fact that in recent years the number of surviving infants with low and extremely low birth weight has been steadily increasing, the nursing outcome is not enough favourable with unsatisfying quality of life. The cardiopulmonary resuscitation protocols have been regularly updated and upgraded (every five years in the last twenty years). This helps to improve resuscitation outcome. In 2015 the American Heart Association and the European Resuscitation Council issued the new edition of the guidelines on basic and advanced cardiopulmonary resuscitation in children, including infants. The guidelines are believed to improve the quality of resuscitation care in the delivery room and to contribute to a better neurological outcome.
This article contents the results of computed tomography with intravenous bolus contrast media administration data analysis in children with transposition and congenitally corrected transposition of the great arteries with the consequental performing of the multiplanar heart-axis-oriented reformations. Among 148 examined children transposition of great arteries was detected in 13 patients (9 boys and 4 girls aged 1-144 day of life); congenitally corrected transposition was found in 4 cases of children aged from 6 months to 15 years and 6 months (2 boys and 2 girls). In this article comprehensive anatomical criteria of each heart chamber morphology are presented and reformations where these criteria can be seen are shown. Also in the article is given comparative characterization of heart and great arteries structures in transposition and congenitally corrected transposition in every certain heart-axis-oriented reformation. By the results of consequently performed heart-axis-oriented reformations data analysis the peculiar anatomical signs of transposition and congenitally corrected transposition are determined. The results of data analysis show that from the list of offered reformations the peculiar anatomical signs of both kinds of transposition are significantly determined in long-axis of right ventricle inflow tract reformation, left heart chambers reformation, supply ventricle division reformation, short-axis reformation at the level of great arteries. Computed tomographic angiocardiography heart-axis-oriented multiplanar reformations permit full and correct assessment of heart and main vessels, which is important for planning of surgical treatment in congenital heart diseases.
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