Vol 12, No 2 (2021)

Background. Inflammatory cytokines and growth factors are involved in various mechanisms of coronary artery disease. Clinical studies have shown the correlation between the increase in the level of proinflammatory cytokines and the severity of coronary artery disease, while the data on the role of proinflammatory interleukin IL-8 and anti-inflammatory interleukin IL-4 are contradictory.

The aim of the study is to assess the levels of proinflammatory cytokines (IL-8, TNF-α) and anti-inflammatory interleukin (IL-4) in patients with various forms of coronary artery disease who underwent coronary artery stenting.

Materials and methods. By the method of enzyme-linked immunosorbent assay, the levels of cytokines were determined in 30 patients with acute coronary syndrome who underwent primary stenting of the coronary arteries and in 24 patients with chronic coronary syndrome who had previously had myocardial infarction with stenting of an infarction-associated artery, who were admitted to the clinic for staged stenting of the coronary arteries.

Results. In patients with chronic coronary syndrome the levels of IL-4 a do not exceed the reference values, in patients with acute coronary syndrome the levels of IL-4 there was an increase 3,70 ± 0,24 and 240,85 ± 49,25 pg/ml, р ≤ 0,001. In patients with chronic coronary syndrome the levels of IL-8 a do not exceed the reference values, in patients with acute coronary syndrome the levels of IL-8 there was an increase 7,34 ± 1,29 and 110,33 ± 27,67 pg/ml, р ≤ 0,001.

Conclusion. Most likely the increase in the level of IL-4 has a compensatory character and, along with a slight increase in TNF-α, can be considered as a positive factor stabilizing the course of the disease. There may be some relationship between of the increase in the level of interleukins in patients with acute coronary syndrome on the degree of stenosis of the coronary arteries (90–95%) and impaired myocardial contractility was established.

The aim of the study was a comparative characteristic of the size of internal organs according to ultrasonography data in subjects with various deviations in stature, determined using international standardized norms.

Materials and methods. The stature was measured in 93 adolescents, aged 13 to 17 years. Based on the measurements, the Z-score of body length was calculated according to the WHO Growth Reference, 2007 and three groups were formed for comparing the sizes of internal organs: “average”, “above average”, “below average”. Ultrasonography data of the internal organs dimensions and thyroid gland was performed using a Toshiba Aplio 500 ultrasound scanner.

Results. There were statistically significantly lower values of the liver span and the longitudinal size of the gallbladder in the examined subjects from the “below average” group compared to the rest of the subjects. The length of the spleen and the total volume of the thyroid gland were statistically significantly different in subjects from all three groups, with the highest values in volunteers from the “above average” group. A weak direct correlation was shown between the Z-score of body length and liver span, the length of the cauda of the pancreas, and the width of the spleen. An average direct statistical relationship was found between the Z-score of stature and the length of the spleen, as well as the total volume of the thyroid gland.

Conclusion. To a greater extent, body length is associated with the size of the parenchymal organs with a pronounced connective tissue frame - the liver and spleen, as well as the thyroid gland due to the relationship of its volume with hormones that regulate growth and development. Clinical substantiation of the relationship between the structure of the body and internal organs opens up the possibility of creating anatomical standards that allow ultrasound morphometric assessment of internal organs, taking into account the individual characteristics of the patient’s body size.

Background. Currently, the development of medicine in the field of perinatology is aimed at improving the quality of medical care for newborns, especially those born prematurely. Premature newborns are most likely to develop hemorrhagic disorders, which often aggravates their condition and determines high morbidity and mortality. On modern hematological analyzers, it has become possible to evaluate a larger number of blood parameters, including platelet parameters, however, there are a small number of studies devoted to the study of platelet parameters in premature newborns.

The aim was to study the morphofunctional features of platelets in premature newborns with very low and extremely low body weight.

Materials and methods. The study included 78 newborns born at 25–34 weeks of gestation, with a body weight of less than 1500 grams. On the 3^rd–5^th day of life, a clinical blood test was performed on the Advia 2120i hematological analyzer (Siemens), with the determination of platelet parameters: PLT, ×10³ cells/µl, PCT, %, PDW, %, Large Plt, ×10³ cells/µl, MPC, g/dl, MRM, pg.

Results. In newborns with ELBW, a decrease in the number and granularity of platelets in the blood, an increase in the average dry mass of platelets was found. Newborns with intrauterine growth retardation have a reduced platelet count and reduced thrombocrit. In premature newborns with a gestational age of 25–27 weeks, blood circulation of platelets with a reduced volume was established, and in newborns who were born at a gestational age of 32–34 weeks with increased granularity. In the absence of antenatal prevention of respiratory distress syndrome in newborns, there is a decrease in thrombocrit, the number of platelets, including large forms in the blood. In premature newborns born in a state of severe asphyxia, a decrease in platelet granularity was noted. The use of high concentrations of O₂ in the oxygen-air mixture during respiratory therapy leads to a decrease in the number of platelets in the blood.

Conclusions. Factors determining the morphofunctional state of platelets in premature newborns were established: the presence of a full course of antenatal prevention of respiratory distress syndrome of newborns, gestational age, the severity of asphyxia at birth, as well as the concentration of O₂ in the oxygen-air mixture used in respiratory therapy. Newborns with ELBW have a reduced platelet count, low-granulated platelets, and an increased average dry platelet mass. Newborns with intrauterine growth retardation have a reduced number of platelets and thrombocrit in the blood. The revealed morphofunctional features of platelets allow us to clarify the nature of changes in the platelet link of hemostasis in premature newborns for the timely prevention of complications during the underlying disease.

Endocrine diseases such as obesity, diabetes mellitus, Cushing’s syndrome, hypothyroidism and hyperthyroidism, acromegaly and hyperandrogenism in children and adolescents are often manifested by changes in the skin. Moreover, skin symptoms can be a marker of systemic, in this case, endocrine and metabolic diseases. Obesity and diabetes are chronic diseases that affect people all over the world, and their incidence is increasing in both children and adults. Clinically, they affect a number of organs, including the skin. The cutaneous manifestations caused or aggravated by obesity and diabetes are varied and usually bear some relation to the time that has elapsed since the onset of the disease. They include acrochordons, acanthosis nigricans, striae, xerosis, keratosis pilaris, plantar hyperkeratosis, fungal and bacterial skin infections, granuloma annulare, necrobiosis lipoidica. In obese patients, psoriasis and atopic dermatitis are more common than in the control group. With the pathology of the thyroid gland, diseases such as alopecia, pretibial myxedema, urticaria, and some others develop. Hyperandrogenism (polycystic ovary syndrome) is accompanied by skin lesions such as acne vulgaris, hirsutism, androgenic alopecia, acanthosis nigricans. This literature review focuses on the main skin syndromes accompanying endocrine pathology in children and adolescents. Information about such clinical associations can make it easier for pediatricians and endocrinologists to diagnose and treat endocrine diseases in a timely manner and, thereby, prevent long-term adverse consequences.

The classification and epidemiology of hereditary metabolic disorders are presented. That is a large group consisting from more them 800 monogenic diseases, each of which caused by inherited deficiency of certain metabolic fate. Many of these disorders are extremely rare, but their total incidence in the population is close to 1:1000–5000. Lysosomal storage diseases (LSD) resulting from inherited deficiency in lysosomal functions occupy a special place among hereditary metabolic disorders. The defects of catabolism cause the accumulation of undigested or partially digested macromolecules in lysosomes (that is, ‘storage’), which can result in cellular damage. About 60 diseases take part in this group with total incidence of about 1:7000–8000. LSDs typically present in infancy and childhood, although adult-onset forms also occur. Most of them have a progressive neurodegenerative clinical course, although symptoms in other organ systems are frequent. The etiology and pathogenetic aspects of their main clinical entities: mucopolysaccharidosis, glycolipidosis, mucolipidosis, glycoproteinosis, etc, are presented. Mucopolysaccharidoses caused by malfunctioning of lysosomal enzymes needed to break down glycosaminoglycans are more frequent among LSD. Sphingolipidoses caused by defects of lipid catabolism are second for frequency group of LSD. The state-of-art in field of newborn screening. clinical, biochemical and molecular diagnostics of these grave diseases are discussed. The main directions of modern lysosomal storage diseases therapy are characterized: transplantation of hematopoietic stem cells; enzyme replacement therapy; therapy with limitation of substrate synthesis (substrate-reducing therapy); pharmacological chaperone therapy. Perspective directions for LSD therapy are gene therapy and genome editing which are at advanced preclinical stages.

Dysplasia of the great veins (DMV) is known by the names of the authors who described this pathology as Klippel–Trenone syndrome. The clinical picture of the Klippel–Trenone syndrome in the classic description of the authors is characterized by a triad of symptoms: vascular spots, atypical varicose veins, hypertrophy of soft tissues and bones with an increase in the volume and length of the affected limb. It should be emphasized that the severity of these symptoms depends, first of all, on the type of lesion (embryonic or fetal) and the severity of the lesion. Klippel–Trenone syndrome is almost always sporadic, meaning that it develops in people with no family history of the disorder. Research shows that this condition is due to gene mutations that are not inherited. These genetic changes, called somatic mutations, occur randomly in a single cell during the early stages of development before birth. Klippel–Trenone syndrome can be caused by mutations in the PIK3CA gene. This article presents a clinical observation – the course of the disease of a 1-year-old child, with an extremely severe form of dysplasia of the great veins. In the presented clinical observation, attention is drawn to the difficulties of treating this patient against the background of the underlying chronic disease. The treatment of these patients should be carried out on the basis of a multidisciplinary hospital, which includes specialists in vascular surgery, an orthopedist and an intensive care physician. On the example of the described case, diagnostic tactics and surgical treatment are demonstrated. It is obvious that timely surgical and conservative treatment of pathology in children with dysplasia of the great veins improves the quality of life and social adaptation of children.