УДК 616.13-002-036-053.2-07:616.155.02

Хвороба Кавасакі (ХК)-це гострий системний васкуліт, який є найпоширенішою причиною набутих вад серця в дітей віком до 5 років із гіпертермією. Діагностика ХК є клінічною проблемою, враховуючи широкий спектр її проявів і схожість із багатьма вірусними та бактеріальними захворюваннями

УДК: 616.988.55:(616.31+616.321)]-022]-053.2-07

Лайм-бореліоз–це мультисистемне інфекційне захворювання, яке виникає внаслідок інфікування бактеріями комплексу Borrelia burgdorferi sensu lato. Перебіг захворювання характеризується трьома стадіями: ранньою локалізованою, ранньою дисемінованою та пізньою. Однією з рідкісних форм ранньої дисемінованої стадії хвороби Лайма у дітей є лімфоцитома. Нами описано клінічний випадок бореліозної лімфоцитоми, спричиненої поєднанням трьох видів борелій (Borrelia afzelii, Borrelia burgdorferi, Borrelia garinii) у дівчинки 6 років. Захворювання маніфестувало з множинної мігруючої еритеми в ділянці обличчя дитини, яку було прийнято за алергічний дерматит. Укусу кліща, в анамнезі, у дитини, зі слів батьків, не було. Діагноз підтверджено методом ІФА та імуноблотаналізом. Пацієнтці було призначене лікування доксицикліном упродовж 21 дня по 50 мг 2 р/д. Через три тижні після початку антибіотикотерапії спостерігалась повна регресія лімфоцитоми

The presence of coagulopathy as part of the systemic inflammatory response syndrome is a characteristic feature of severe coronavirus disease 2019 (COVID-19). Hematological changes (increased DD-dimer, prolonged activated partial thromboplastin clotting time [APTT] and prothrombin time [PT], high fibrinogen levels) have been observed in hospitalized patients with COVID-19, which characterize the risk of thrombotic events. Against the background of COVID-19 there is endothelial dysfunction, hypoxia and pulmonary congestion, mediated by thrombosis and microvascular occlusion. Up to 71.4% of patients who died from COVID-19 had disseminated intravascular coagulation syndrome, compared with only 0.6% of survivors. The main manifestation of COVID-19-associated coagulopathy is a significant increase in DD without a decrease in platelet count or prolongation of APTT and PT, indicating increased thrombin formation and the development of local fibrinolysis. An increase in DD levels of more than 3-4 times was associated with higher in-hospital mortality. Therefore, COVID-19 requires assessment of the severity of the disease for further tactics of thromboprophylaxis. The need for 1 continued thromboprophylaxis, or therapeutic anticoagulation, in patients after inpatient treatment for two weeks using imaging techniques to assess of thrombosis assessment.

The role of iodine as an anticarcinogenic agent is just beginning to be widely appreciated.
METHODS The aim of our study is to identify a link between iodine deficiency and the development of hematological malignancies in children. We screened iodine status in 36 children with oncohematological diseases and 32 healthy. Children were tested for iodine in the urine, ultrasound of thyroid glands were done.

UDC 616+616-053.2+616.151.5

Hemophilia A is an X-linked recessive disorder caused by a deficiency of plasma coagulation FVIII, which may be inherited or arise from a spontaneous mutation. FVIII deficiency leads to a decrease in normal hemostasis and is manifested by spontaneous or induced bleeding. As a result of hemorrhages in the central nervous system, neurological complications are possible. In such cases, doctors should be on the alert so as not to miss another accompanying pathology. Neurodegenerative disease with iron accumulation in the brain is a genetically and clinically heterogeneous group of hereditary progressive disorders of the central nervous system with pronounced iron accumulation in the basal ganglia, which have a specific picture on magnetic resonance imaging of the brain in combination with characteristic clinical signs. The aim is to describe a clinical case of a combination of two complex hereditary diseases in a 10-year-old boy, hemophilia A of moderate severity, complicated by an inhibitor, and a progressive neurodegenerative disease with accumulation of iron in the brain, with associated neurodegeneration associated with the protein of the mitochondrial membrane. The publication reports for the first time a clinical case of a combination of two complex hereditary diseases in a 10-year-old boy, confirmed by molecular genetic studies: hemophilia A of moderate severity, complicated by an inhibitor with the detection of a large deletion of exons 23-26 in the gene, and progressive neurodegeneration with brain iron accumulation, with the presence of a pathogenic mutation of the C19orf12 gene, variant c.204_214del (p.Gly69Argfs*10) in a homozygous state, autosomal recessive type of inheritance, Mitochondrial-membrane Protein-Associated Neurodegeneration. Coagulopathy is controlled by prophylactic administration of emicizumab subcutaneously. Neurodegeneration with brain iron accumulation in the child was manifested by: Friedreich's foot, equinus feet, positive Babinski symptom, high tendon reflexes, optic nerve atrophy; partial dysplasia of both eyes; with myopia of both eyes, impaired accommodation, progressively increasing paresthesias in both legs, impaired gait, ataxic gait, coordination difficulties, muscle atrophy of both legs, visual impairment, rapid fatigue with preserved intelligence and mental development. Magnetic resonance imaging of the brain showed a moderate bilateral symmetrical lesion of the globus pallidus. Our report confirms that the use of molecular genetic studies plays an important decisive role in the verification of the disease, often determining its type and possible complications. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.