NEWS
PEDIATRICS
Acute leukemia accounts for up to 30% of all diseases of the hematopoietic and lymphoid systems in children. The initial period of leukemia is characterized by polymorphism of clinical symptoms, which makes it difficult to timely diagnose and treat this pathology. The reasons for the treatment of patients with leukemia for medical help, to doctors of various specialties, are: the presence of fever, hemorrhagic syndrome, increasing weakness, anemia, enlarged lymph nodes of the liver, spleen, pain in the joints and bones, impaired stool, etc. The article presents literature data and own observations of acute lymphoblastic leukemia in infants hospitalized in an infectious disease hospital with referral diagnoses: mumps; Intestinal infection – enteritis. The description of the clinical symptoms and the course of the disease is presented in the form of an analysis of the clinical situation for the purpose of medical education and differential diagnosis with mumps infection and enteritis of infectious etiology. An analysis of the clinical symptoms that a patient had with a referral diagnosis of mumps made it possible to establish significant deviations from the usual course of mumps infection, already at the initial request for medical help. Unilateral mumps submaxillitis is rare. More often both glands are affected in combination with parotid gland involvement. Widespread tissue edema is more characteristic of submandibular lymphadenitis rather than submaxillitis, which, in combination with hepatomegaly, testified against mumps infection. In a child with a referral diagnosis of intestinal infection, enteritis, the presence of lymphadenopathy, hemorrhagic syndrome, hepatosplenomegaly, with a mild intestinal syndrome, cast doubt on this diagnosis. Intestinal damage in leukemia is common and is caused by enteropathy syndrome, due to extramedullary lesions with hemorrhages in the intestinal mucosa, activation of opportunistic bacteria that make up the intestinal microflora. The reason for the errors in the diagnosis of leukemia, in the above observations, was the lack of an analysis of clinical symptoms, which would allow to establish discrepancies between the patient's symptoms and the proposed diagnosis. A one-sided approach to assessing the clinical symptoms that patients had, the lack of alertness of doctors in terms of blood diseases, were the reasons for the erroneous diagnosis during the initial treatment of patients for medical care.
One of the most common gastroenterological pathologies in childhood is constipation of functional origin. The frequency of constipation in children of the first year of life ranges from 10.7% to 17.6%. Constipation is considered as a rare, painful, difficult and/ or incomplete bowel movement with stools of varying degrees of density and diameter. When diagnosing constipation in young children, it is important to focus not only on the frequency of the stool, but also on its nature, using the Bristol stool consistency scale. Even with daily bowel movements, accompanied by painful sensations and dense feces, the condition must be regarded as constipation. Suggested triggers in the first year of life are most often related to dietary changes, previous intestinal infection, and the presence of anal fissures. After the first year, improper toilet training may be an additional factor. When toilet training is incorrect, children begin to retain feces, which leads to painful defecation, and further forms fear, leading to an even greater delay in defecation. For the prevention and correction of constipation in young children, the following points are important: the presence of support for the legs during defecation; Encouraging the child to consume more water, fruits and vegetables every day, as well as to get enough physical activity. In the diet therapy of functional constipation, the role of some probiotics (strains of Lactobacillus, Bifidobacterium lactis) and prebiotics (inulin, galacto- and fructooligosaccharides) has been proven.
Hypophosphatasia is a rare hereditary disease characterized by a very diverse clinical manifestations that can make it difficult to make a timely diagnosis. Due to the deficiency of alkaline phosphatase, multisystem disorders are observed in patients. First of all, these are bone changes (osteoporosis, rickety deformities, fractures), lung damage (hypoplasia with respiratory failure) and central nervous system (seizures), hypercalcemia with the development of nephrocalcinosis. In the absence of timely treatment, the prognosis of the disease in most cases is unfavorable for life. The article describes a family case of hypophosphatasia (GFF) – in a mother and her child. The first clinical symptoms in the child began to manifest at the age of 3 years, the diagnosis of the mother was established at the age of 34 years after the genetic confirmation of the diagnosis in the younger child as a result of examination of the whole family. According to the results of DNA diagnostics (Sanger sequencing), a pathogenic nucleotide variant of C. 595C>T (chr1:21890656C>T) was detected in both family members in a heterozygous state according to the ALPL gene. However, during the molecular genetic studies of Proband's father and sister, no mutations were found. Clinical manifestations were typical for GFF: pain in the lumbar region and in the child, in the knee joints of the mother. The late diagnosis led to the late appointment of substitution therapy. GFF can be suspected based on a combination of clinical symptoms of the disease, characteristic changes according to X-ray examination. For differential diagnosis, it is necessary to determine the activity of alkaline phosphatase. The described case showed that the manifestations of hypophosphatasia are different and differ depending on the age of the manifestation of the disease and the severity of the condition of a particular patient. After genetic verification of the diagnosis, all patients need the appointment of modern enzyme replacement therapy with asphotase alpha, which significantly improves the prognosis of the course of the disease, as well as dispensary observation by specialists of various profiles.
In an open observational prospective comparative study based on the National Center for Maternal and Child Health (Department of Pathology of Newborns and Premature Infants) Kyrgyzstan included 103 newborn children with a confirmed diagnosis of intrauterine infections of various etiologies, randomized into two groups: the main group – 54 children receiving standard therapy (antibacterial, antifungal, symptomatic) in combination with interferon alpha-2b with antioxidants in the dosage form of rectal suppositories (150,000 IU); the comparison group consisted of 49 newborns who received only standard therapy. There was a significant improvement in the general condition of children, which was assessed based on the positive dynamics of the main clinical symptoms of the disease against the background of interferon therapy compared with the comparison group, as well as an increase in the physical development of newborns. Statistically significant positive dynamics of laboratory parameters in the main group compared with the comparison group was determined: a decrease in leukocytosis, neutrophil intoxication index, procalcitonin and lactate levels, leukocyturia and bacteriuria (p < 0.05). There was an increase in the level of serum IFN, the ability of cells to produce both IFN-α and IFN-γ by immunocompetent cells, an increase in the phagocytic function of neutrophils (an increase in phagocytosis from 42% to 51%, phagocytic index from 2.1 ± 0.1 to 3.4 ± 0.2, absolute phagocytic index from 5462 ± 1136 to 10845 ± 1127, a decrease in percent of immature forms of neutrophils from 17.5 ± 2.3 to 7.8 ± 1.2). The use of drugs of recombinant interferon alfa-2b in the early neonatal period led to normalization of the number of lymphocytes, the number of activated cytotoxic T-lymphocytes (5.7 ± 0.9), normalization of immunoglobulin indices of the main classes. No deaths have been recorded. The complete absence of adverse events during the research period and unmotivated refusals of mothers from therapy proved the high tolerability and safety of this drug. The results obtained make it possible to recommend the inclusion of IFN-a2b preparations with antioxidants (rectal suppositories 150,000 IU) in standard treatment regimens for intrauterine infections in full-term and premature infants.
The totality of microorganisms closely interacting with each other and with human cells is currently considered as another organ in the human body and is called the «microbiota». The functions of the intestinal microbiota are very diverse: anti-infective protection, modulation of the inflammatory and immune response, synthesis of compounds necessary for the body (for example, vitamins, shortchain fatty acids), participation in the digestion of a number of nutrients, as well as the regulation of intestinal motility, proliferation and differentiation of intestinal epithelial cells. It is the intestine that is the most densely populated biotope of the human body, and the population of microorganisms of the gastrointestinal tract is the most diverse and numerous. The settlement of the gastrointestinal tract begins in utero and enters the active phase after birth. Further progressive colonization of the child's digestive tract in the first months of life is provided by the mother's vaginal microflora during natural childbirth, breastfeeding, contact with the environment, as well as the consistent introduction of a variety of foods into the diet. In the study of L. Wang et al. (2021) analyzed the growth of several species of bacteria that dominate the intestines of infants by culturing on calcium palmitate media. This study was carried out by the Department of Medical Microbiology of the University of Groningen (Netherlands), the Medical Clinic of the University of Groningen and a goat milk formula company. Of all the tested bacteria, the growth of bifidobacteria and F. prausnitzii decreased when calcium palmitate was introduced into the medium, and the growth of Bifidobacterium infantis was completely stopped. Calcium palmitate reduced the thickness of the F.prausnitzii cell membrane, disrupted the fatty acid composition of the cell membrane and the function of membrane proteins involved in ion transport. The findings indicate that modification of the fat of formula-feeding formulas by the administrationof β-palmitate may promote the development of the gut microbiota in formula-fed infants by supporting the colonization of important beneficial bacteria at an early age.
URONEPHROLOGY
Chronic kidney diseases (CKD) in children of our country develop against the background of hypovitaminosis, characteristic of the child population. Both in our and foreign studies, vitamin D deficiency is noted in children with CKD. However, the decrease in vitamin D in the blood depends on the nosological unit that underlies the development of CKD. In tubulointerstitial kidney diseases, which are mainly characterized by slow progression of CKD, vitamin D deficiency is less significant. In children with glomerulonephritis, especially when using glucocorticoids, vitmin D deficiency and phosphorus-calcium disorders are more pronounced. The concomitant probable deficiency of vitamins C, B2, B6, E, K, folic acid further aggravates the metabolism of vitamin D and its reception system, requires research to clarify the degree of hypovitaminosis among children with CKD and those on renal replacement therapy. Hypervitaminosis A is observed already in the early stages of CKD due to a slowdown in the excretion of retinoids by the kidneys, and its levels are highest in children receiving supplemental nutrition containing vitamin A, compared with diet alone. Increased retinoids in the blood serum significantly predetermine the development of hypercalcemia. When using multivitamin preparations in children with CKD, a differentiated approach is necessary, preferably justified by the preliminary determination of vitamins in the blood.
Pseudomembranous colitis in recent years has become a fairly aggressive and widespread form of infection associated with the use of antibacterial therapy, refers to the most severe inflammatory bowel diseases, the cause of which is the grampositive sporeforming bacterium Clostridium difficile. The main symptoms of pseudomembranous colitis include: frequent watery loose stools, fever (up to 3-40 °C), abdominal pain. This article presents a clinical observation of a patient with pseudomembranous colitis, the peculiarity of which is that the patient had no stool disorder and no abdominal pain against the background of prolonged subfebrility (1.5 months). The debut of the disease began with a collapse, a convulsive attack, the phenomena of acute kidney damage, which did not allow for a timely correct diagnosis. Women received inpatient care, examined by many specialists, including a urologist, an infectious disease doctor, received therapy with linezolid (400 mg × 2 times a day – 10 days), bakperazone (1 g × 2 times a day), meropenem 1000 mg × 3 times a day with a working diagnosis of «Acute pyelonephritis. Acute kidney injury in the resolution stage». Untimely diagnosis of the disease can lead to life-threatening complications: intestinal translocation syndrome, severe sepsis, multiple organ dysfunction syndrome. The above, dictates the need to inform doctors of the practical level of healthcare about the problem of pseudomembranous colitis, since it is known that with timely diagnosis of pseudomembranous colitis K and adequate anticlostridiosis therapy, it is possible to save the patient's life. It is necessary to inform doctors more widely about the possibility of an atypical course of the disease and to increase the alertness of doctors regarding the disease caused by pseudomembranous colitis, Clostridium difficile, antibacterial pneumonia, acute kidney injury.
TOPICAL THEME
Malignant neoplasms are one of the main causes of death in the world, which in 2020 ended the lives of about 10 million people. High mortality rates from malignant neoplasms are considered one of the negative trends in the dynamics of the health status of the Russian population. More than half of the deaths of the working-age population in Russia are due to diseases from the group of preventable deaths, and a third – to preventable causes, which depend on primary and secondary prevention, as well as the quality of medical care provided. The article presents data on the state of the oncological service in the Republic of Mordovia for 2019-2021. The purpose of the study is to analyze the organization of oncological care to the population of the Republic of Mordovia, the dynamics of morbidity and mortality of the population with malignant neoplasms. In this work, statistical data from Mordovia Statistics (the territorial body of the Federal State Statistics Service for the Republic of Mordovia) are used. The incidence of malignant neoplasms over a 3-year period decreased by 15.2% and amounted to 465.4 per 100 thousand population in 2021. The detection rate of patients in the early stages of malignant neoplasms (stage I-II) in the Republic of Mordovia in 2021 was 60.1%. There is a decrease in mortality to 170.0 per 100 thousand population, which is 0.1% lower than in 2019. The mortality rate within 1 year after the diagnosis of a malignant neoplasm in 2021 was 19.6%, which is 2% lower than in 2019. The proportion of patients registered at the dispensary for 5 years or more from the moment of diagnosis increased by 2% and amounted to 56.3% in 2021. The main task facing the oncological service is to reduce the mortality of the population from cancer pathology. The ways to solve it lie in the active development of the preventivedirection of cancer control, close cooperation with the primary health care unit, in which primary medical care should be reoriented to the predominantly preventive direction.
Despite the large number of studies in the world, the peculiarities of the course of psoriasis remain relevant. In the conditions of the Aral Sea region they are especially in demand due to the great chronicity of the process and increased percentage of disability among this category of patients. The analysis of clinical forms, seasonality of exacerbations, concomitant pathology in patients with psoriasis living in the Aral Sea region is carried out in this article based on the observation and data analysis of 135 patients who received hospital treatment in the Republican CVD, Nukus, Republic of Karakalpakstan. The age of the patients ranged from 15 to 70 years old. It was found that progressive stage of psoriasis was registered in 90% of patients, in 10% of patients – stationary stage. Affection of nail plateswas observed in 16% of patients with psoriasis. According to clinical form the vulgar form was revealed in 89% of patients, arthropathic – in 34% of patients, palm plantar form – in 3%, exudative – in 1.5%, seborrheic – in 1.5%, erythroderma – in 6%. Concomitant somatic pathology was detected in 66% of patients. In the structure of somatic pathology of psoriasis patients in the general group the diseases of digestive organs and bone system prevail, pathology of cardiovascular system is revealed to a lesser degree. Cardiovascular system pathology increased up to 36% in the older group. In men there were more often concomitant diseases of bone system in 25% of cases, of digestive organs in 24%, of cardiovascular system in 9%. In women, concomitant diseases of the digestive organs were more frequent in 19% of cases, of the endocrine system in 3%, and of the genitourinary system in 7%. Frequent exacerbation of psoriasis is characteristic for mixed type 44%. Psoriasis is a systemic disease, the course of which is influenced by disturbances in all body systems. The data we obtained revealed a large percentage of concomitant pathology in patients with psoriasis living in the Aral Sea region, which dictates the need to review the therapy options for these patients, taking into account the concomitant pathology and personalization of treatment.
Allergic reactions are quite common in pregnant women. The most common manifestations of an allergic reaction during pregnancy are: from the respiratory tract – allergic rhinitis and bronchial asthma, from the skin and subcutaneous tissue (allergodermatosis) – urticaria, angioedema, atopic dermatitis. As a rule, allergies do not affect the course of pregnancy, and pregnancy does not affect the allergic process, however, allergic reactions in pregnant women can cause difficulties associated with the selection of pharmaceuticals. The article discusses practical issues of pregnant women with allergic diseasesmanagement. Attention is paid to the place of non-drug methods: hypoallergenic diet and enterosorbents for food allergies, the use of sprays based on saline solutions for allergic rhinitis. Medication management of pregnant women suffering from allergic diseases is a compromise between the potential adverse effect of medications on the fetus and the course of pregnancy and the consequences of uncontrolled allergy. If it is necessary to prescribe antihistamines in the secondthird trimesters, it should be assumed that category B according to the FDA classification («no evidence of risk») includes chlorpheniramine, ciproheptadine (Peritol), diphenhydramine (Dimedrol), chlorotripelenamine (Suprastin), cetirizine (Zirtek, Cetrin), loratadine (Claritin), levocetirizine (Xizal). It is contraindicated to take astemizole, terfenadine, Tavegil (klemastin) because of the teratogenic or fetotoxic effect, desloratadine and ketotifen due to their ability to penetrate the placenta. Glucocorticosteroids are the drugs of choice in the treatment of severe forms of allergic diseases in pregnant women. Nasal corticosteroids are widely used in the treatment of moderate and severe allergic rhinitis. Inhaled glucocorticosteroids occupy a central place as a basic means for bronchial asthma. The drug of choice is budesonide, which has the best safety profile and has the greatest evidence base. Topical glucocorticosteroids (Advantan, Locoid, Elokom) in the form of creams and ointments are widely used in atopic dermatitis in pregnant women. If it is necessary to prescribe oral glucocorticosteroids (generalized urticaria, angioedema, severe exacerbation of atopic dermatitis), prednisone is preferred. Deposited glucocorticosteroids are categorically contraindicated. The use of cromoglycic acid, monoclonal antibodies, decongestants, bronchodilators is also discussed.
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ALMA MATER
ISSN 2687-1181 (Online)


















