Vol 29, No 1 (2022)

Polypharmacy increases the risk of drug-drug interactions, leading to poor effectiveness and side effects. Drug-drug interactions can be divided into two main groups: pharmacokinetic and pharmacodynamic. Drugs, food, drinks can change the effect of drugs, affect pharmacodynamic mechanisms by synergistic, additive or antagonistic effects or pharmacokinetic processes such as absorption, metabolism and excretion, which leads to a decrease in the effectiveness of drugs or an increase in its toxicity. Drugs can also alter the ability to absorb essential to health nutrients. One of the main causes of drug-drug interactions is the induction or inhibition of cytochrome P450 (CYP450) enzymes. The carriers of drugs, especially P-glycoprotein (P-gp), can cause interactions between drugs and therefore it plays an important role in the pharmacokinetic drug-drug interactions. Further study is needed on drug-food interactions. However, it is known that widely-used drugs (such as sugar-lowering, antihypertensive drugs, including diuretics, renin-angiotensin-aldosterone system inhibitors, ß-blockers, analgesics, etc.) usually prescribed without taking into account meal times. Moreover, it’s known about drug-herbs interaction and interactions with dietary supplements. Drug-drug interactions is one of the most important risk factors for drug-induced diseases because the diagnosis of drug-drug interactions sometimes is difficult due to different pharmacological mechanisms. Clinicians should always evaluate both patient-related factors (genetic polymorphism, age, sex, physiologic status, etc.) and drug-related factors. Knowing the significant drug-drug interactions can help the clinician to prevent side effects and drug-induced diseases and optimize pharmacotherapy.

Acute respiratory viral infections (ARVI) are the most common form of acute pathology. Herbal preparations, representing complementary medicine, can help improve the course of the disease. The article discusses various plants from the point of view of direct antiviral and immunotropic actions. From the standpoint of evidence-based medicine, data on the clinical efficacy of certain drugs in ARVI are presented.

Hereditary angioedema is a rare condition that usually manifests with recurrent episodes of potentially life-threatening angioedema during childhood or adolescence. Early diagnosis and optimal treatment of this disease is very important in order to avoid ineffective methods of treatment and to prescribe adequate therapy, thereby improving the quality of life of patients. This article discusses some of the features of the course of this disease in childhood and adolescence.

Background. Pompe disease, also known as type II glycogenosis, is a metabolic disorder that causes glycogen to be deposited within lysosomes in muscle tissue. CRIM status may influence the management and prognosis of patients with infantile Pompe disease. The article presents the experience of managing a patient with a CRIM-negative status in Pompe disease and demonstrates the importance of early diagnosis of the disease. Objective. Substantiation of the relevance of determining the CRIM status in patients with Pompe disease and the importance of early diagnosis of the disease. Methods. In the period from 2011 to 2020, 12 patients (6 boys and 6 girls) with infantile Pompe disease, confirmed both by enzyme diagnostics and by the results of genetic testing, were followed-up by doctors of the Cardiology Department of the National Research Center for Children’s Health.all patient received ERT by intravenous recombinant human acid α-glucosidase. Currently, 2 patients continue to be followed-up in the clinic. Clinical (family history taking, examination of the patient), laboratory (determination of the creatinine phosphokinase, creatine phosphokinase-MB, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase, natriuretic peptide levels), instrumental (echocardiography, electrocardiography, chest x-ray, ultrasound examination of the kidneys and abdominal organs), molecular genetic (GAA gene sequencing by direct automatic Sanger sequencing) and biochemical (enzyme diagnostics by tandem mass spectrometry) were used. Results. ERT is most promising when it is started before the onset of clinical manifestations, which makes early diagnosis of the disease extremely important. The induction of immune tolerance in CRIM-negative patients may reduce the formation of antibodies to alglucosidase α, allowing to enhance the effectiveness of therapy in children of this category. In patients with Pompe disease who demonstrate an insufficient clinical response to standard ERT regimens, an increase in the dose and/or frequency of administration of the drug can be considered.

Data on the prevalence of helminthiases in the world are pressented; the clinical picture, approaches to the diagnosis, treatment and prevention of helminthiases in children and adults are discussed. It is shown that a feature of helminthiases is considered to be a wide prevalence among the child population. The variety of symptoms of helminthiases indicates the complexity of diagnosing this group of diseases and the need to use a complex of clinical and laboratory-instrumental methods. After establishing a diagnosis, patients undergo anthelmintic therapy, including dietary treatment, the administration of etiotropic, pathogenetic and symptomatic agents. Specific treatment is the basis for the therapy of most human helminthiases. Among anthelmintic drugs, albendazole is one of the most effective in both tissue and luminal forms of helminthiases.

The article discusses the main, as well as little-studied effects of ambroxol, a mucoactive drug widely used as a mucokinetic and mucolytic. Modern data on pharmacokinetics, pathogenetic mechanisms of action, efficacy and safety of ambroxol use in children with acute respiratory diseases are presented. Long-term experience of using ambroxol indicates that it has unique properties that actively influence the basic mechanisms of coughing, physiological production and transport of bronchial secretions, and the activity of the surfactant system.

Background. Primary adrenal insufficiency (PAI) is a disease characterized by decreased production of adrenal hormones. As a rule, this term refers to various variants of hypocorticism vary in etiology and pathogenesis. In childhood, in terms of genesis, hereditary syndromes prevail, in which adrenal insufficiency (AI) with a gradual onset and slow progression is diagnosed. Gastrointestinal disorders - loss of appetite, nausea, vomiting - are sometimes observed already at the onset of the disease and necessarily occur with an augmentation of AI. Description of the clinical case. The article deals with a clinical case of PAI in a child of 11 years old, which manifested itself against the background of a novel coronovirus infection COVID-19. Conclusion. Most symptoms of cortisol and aldosterone deficiency are nonspecific and may be a sign of diseases of other organs and systems (gastrointestinal tract, central nervous system, etc.). Diagnostic search for a specific nosological form of AI allows predicting the course of the disease, the likelihood of the appearance of pathology in other organs and systems, and determining the tactics of management of the patient.

Background. DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is a syndrome caused by a potentially life-threatening side effect to drugs with an estimated mortality of 10%. The cause of DRESS in most cases in both adults and children is anticonvulsant drugs. In the domestic literature, we did not find publications on the features of the course of DRESS in children. Description of the clinical case. The article presents a clinical case of the development of severe DRESS syndrome with fever and lung damage at the onset, severe skin syndrome and high-grade hepatitis against the background of anticonvulsant therapy in a child with newly diagnosed epilepsy. During the differential diagnostic search, allergic, infectious, incl. COVID-19 infection, and autoimmune diseases were excluded. The main treatment included the cessation of anticonvulsant therapy with replacement with less toxic drugs, the appointment of systemic corticosteroids - parenterally with the transition to oral administration and gradual withdrawal over 2 months, as well as the introduction of intravenous immunoglobulin. Against the background of the therapy, skin syndrome and cytolysis syndrome regressed. The child’s condition did not worsen, the skin syndrome did not recur, and cytolysis syndrome was not recorded during 6 month follow-up. Conclusion. The combination of episodes of febrile fever with morbilliform exanthema, the presence of lung damage according to X-ray computed tomography, acute hepatitis against the background of antiepileptic therapy during the period of a new coronavirus infection, which has a tendency to polysystemicity, was a feature of this clinical case. The complexity of the initial diagnosis was attributable to the polymorphisms of the first clinical manifestations, the rarity and insufficient awareness of specialists about the DRESS syndrome.