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Vol 13, No 2 (2022)

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Role of ultrasound diagnostics in pediatric septic shock intensive care: a review and a clinical case

Ermolenko K.Y., Pshenisnov K.V., Alexandrovich Y.S., Konev A.I., Ironosov V.E., Nezabudkin S.N., Pogorelchuk V.V., Evgrafov V.A.


The article is devoted to the use of ultrasound diagnostics methods to assess the functional state of the cardiovascular system in critical conditions in children and the choice of optimal hemodynamic support. The need for careful detailed monitoring in patients in pediatric intensive care units has been demonstrated, the low sensitivity and specificity of the currently widely used clinical signs are reflected, which limits their use in the choice of treatment methods. As an alternative, it is proposed to use ultrasound diagnostics that assess cardiac output, allowing you to make an informed decision on medical measures taking into account the current clinical situation. The wide possibilities and numerous advantages of ultrasonic navigation in providing assistance to patients with a wide variety of life-threatening conditions are described. The main advantage is the possibility of obtaining information in real time, directly at the bedside. A clinical case of a target-oriented intensive therapy of left ventricular systolic dysfunction in a nine-year-old child against the background of a septic process using methods of ultrasonic assessment of hemodynamic status is presented. The use of ultrasound imaging methods made it possible to identify the cause of the deterioration of the condition and conduct a reasonable treatment correction, which ensured the fastest regression of hemodynamic disorders and contributed to a favorable outcome of the disease. The simplicity and accessibility of the Teicholz estimate of the ejection fraction was noted, which allows it to be used in routine practice to select the optimal hemodynamic support and assess the effectiveness of treatment over time.

Pediatrician (St. Petersburg). 2022;13(2):5-15
pages 5-15 views

Original studies

Clinical and laboratory characteristics of epiglottitis caused by Haemophilus influenzae type b in children

Sitkina E.L., Lioznov D.A., Ivanova R.A., Sabadash N.V., Meyrieva R.E., Gorchakova O.V., Antonova T.V.


BACKGROUND: Epiglottitis is an acute infectious disease accompanied by edema of the epiglottis, salivation, sore throat, and intoxication. In 95% of cases, the causative agent of epiglottitis in children is Haemophilus nfluenza type b (Hib). The relevance of the problem is that acute epiglottitis of Haemophilus etiology is not a localized, isolated disease of the ENT organs, but can result in development of an epiglottic abscess or phlegmon, as well as of other generalized forms of Hib infection, including sepsis.

AIM: The aim of the study was to assess the incidence of epiglottitis caused by Hib in children and provide its clinical and laboratory characteristics.

MATERIALS AND METHODS: We analyzed 23 cases of epiglottitis caused by Hib in children. The diagnosis was based on clinical and epidemiological data with mandatory verification by bacteriological, serological, and/or molecular biological methods.

RESULTS: Epiglottitis was detected in 23 of 93 children with Hib infection who were treated at the Children’s Municipal Clinical Hospital No. 5 named after N.F. Filatov over the period from 2002 to 2016. Hib etiology of epiglottitis was confirmed mainly by the bacteriological method. All patients were prescribed ceftriaxone, which is the drug of choice for suspected Hib infection. Monotherapy was prescribed in 57% cases; two or more antibiotics were used in other cases (a course of 7–14 days).

CONCLUSIONS: Epiglottitis is a severe, life-threatening disorder being a generalized Hib infection. Patients with epiglottitis should be prescribed antibiotics prior to confirmation of Hib etiology since Hib is the causative agent of epiglottitis in the vast majority of pediatric cases. Multiple methods should be used simultaneously to confirm the diagnosis. Bacteriological blood test made it possible to identify the pathogen in the overwhelming majority of patients. There were no risk factors for Hib infection and no important comorbidity.

Pediatrician (St. Petersburg). 2022;13(2):17-24
pages 17-24 views

Antioxidant system and lipid peroxidation in rat erythrocytes under low-dose exposure to mercury acetate

Shchepetkova K.M., Batotsyrenova E.G., Litvinenko L.A., Ramenskaya N.P., Kashuro V.A.


BACKGROUND: Low-dose exposure of mercury compounds to the human body for a long time leads to the accumulation of a toxicant in tissues, causing damage to health. Mercury can be delivered to a developing fetus through the placenta or to an infant through breast milk. Erythrocytes are the preferred cell for mercury accumulation, reaching a concentration 20 times higher than the concentration in blood plasma. Erythrocytes have powerful antioxidant protection. The antioxidant protection system of the cell plays an important role in maintaining of homeostasis in the cell. Despite of the apparent vastness of researches of the antioxidant system and lipid peroxidation, changes after subacute poisoning with heavy metals have not been sufficiently studied.

AIM: Study changes in biochemical parameters in Wistar rats erythrocytes with subacute poisoning with mercury acetate.

MATERIALS AND METHODS: 30 days and 44 days after the administration of mercury acetate at a dose of 4 mg/kg in the hemolysate of red blood cells of rats, the indicators of the antioxidant system and lipid peroxidation were determined.

RESULTS: The administration of mercury acetate for 30 days significantly increased the activity of SOD, GP and reduced the activity of GT. An increase of DC concentration was noted. 14 days after the end of the injection of the toxicant, the imbalance of the AOS enzyme link persists. An increase of DC and MDA concentrations was revealed.

CONCLUSIONS: The data obtained demonstrate a violation of the antioxidant balance in erythrocytes after a 30-day administration of mercury acetate. 14 days after the end of the injection of the toxicant, changes in the enzyme link of AOS persist. Intensification of the processes of lipoperoxidation of erythrocyte membranes has been established. In the delayed period after poisoning, there is a tendency to disturbance the balance of AOS of erythrocytes, the intensity of LPO processes increases.

Pediatrician (St. Petersburg). 2022;13(2):25-34
pages 25-34 views

State of liver functional activity in children with atopic dermatitis

Buryak V.N., Antonova T.I., Dudko M.V., Shepelenko K.K., Malysheva I.V.


BACKGROUND: The problem of atopic dermatitis today remains one of the unsolved problems of pediatrics and pediatric allergology. Among the etiopathogenetic mechanisms of atopic dermatitis, the role of endo-allergens formed as a result of impaired digestion and absorption processes in the gastrointestinal tract of a child is separately noted. In such conditions, the load on the detoxification function of the liver increases. In connection with this circumstance, the study of the functional activity of the liver in children with atopic dermatitis is of particular interest. One of its criteria is the nature of intrahepatic hemodynamics.

AIM: To find out the features of intrahepatic hemodynamics in the development of atopic dermatitis in children.

MATERIALS AND METHODS: 83 children aged 6 months to 5 years with a diagnosis of atopic dermatitis, who made up the main research group, and 33 practically healthy children of the same age, who formed a control group, were examined. The nature of heredity for allergic pathology and morbidity of the gastrointestinal tract was found out in all patients. To obtain information about the functional activity of the liver in all children, hepatic blood flow in the hepatic artery and hepatic vein was studied.

RESULTS: Violation of intrahepatic hemodynamics was revealed in children with atopic dermatitis. The blood flow in the hepatic artery in them was characterized by a decrease, and the blood flow in the hepatic vein was characterized by an increase in speed indicators. The detected violations of intrahepatic hemodynamics in children with atopic dermatitis are regarded by the authors of this study as a manifestation of a decrease in the functional activity of hepatocytes in conditions of increased allergenic load.

CONCLUSIONS: The study clearly demonstrated a violation of the functional state of hepatocytes in children suffering from atopic dermatitis, expressed in the suppression of hepatic hemodynamics. The question of the primary or secondary nature of this violation is the subject of discussion and further scientific research.

Pediatrician (St. Petersburg). 2022;13(2):35-41
pages 35-41 views

The state of blood flow of the main arteries of the head in children with neurocirculatory dysfunction and connective tissue dysplasia

Arsentev V.G., Kalyadin S.B., Terenteva J.N., Pakhomova M.A.


BACKGROUND: The article studies the relationship between connective tissue dysplasia and neurocirculatory dysfunction. They are united by a systematic and multi-organ nature of complaints and violations. The frequency of occurrence of neurocirculatory dysfunction in connective tissue dysplasia depends on the severity of phenotypic signs and is 23.5–31.1%.

AIM: The aim of this study is to evaluate the significance of the method of duplex scanning of the main cerebral vessels, as well as to evaluate the relationship of the obtained parameters with the data of neurosonography, radiography of the cervical spine in children with signs of neurocirculatory dysfunction and connective tissue dysplasia.

MATERIALS AND METHODS: The duplex scanning method was used to study the main vessels of the neck that provide blood supply to the brain: common and internal carotid, vertebral arteries in 240 children hospitalized for somatic various diseases.

RESULTS: The presence of diagnostically significant changes in the cervical spine was found in 111 of them (46.3%): hypoplasia of the CI or СII vertebrae with cranial subluxations, instability of the cervical spine, and juvenile osteochondrosis. In children diagnosed with neurocirculatory dysfunction without signs of connective tissue dysplasia, disturbances in the form of asymmetry of blood flow along the entire vertebral artery prevail in comparison with the comparison group. In patients with more pronounced signs of connective tissue dysplasia, structural features of the cervical spine (hypoplasia of the CI or СII vertebrae with cranial subluxations, instability, juvenile osteochondrosis) and impaired blood flow of the main arteries are significantly more common.

CONCLUSIONS: According to the data of duplex scanning of the main arteries of the neck, disorders in the form of hemodynamically significant asymmetry of blood flow in the vertebral arteries at different levels are significantly more common. In patients with pronounced signs of connective tissue dysplasia, structural features of the cervical spine and impaired blood flow of the main arteries of the neck are significantly more common. The presence of connective tissue dysplasia may be one of the causes of the above disorders.

Pediatrician (St. Petersburg). 2022;13(2):43-48
pages 43-48 views


Immune response to primary glioblastoma

Sklyar S.S., Trashkov A.P., Matsko M.V., Safarov B.I., Vasiliev A.G.


Immunotherapy is a promising and rapidly developing method of therapy patients with different oncological pathology. Considering the demonstrated efficacy in the treatment of patients with some solid tumors, as well as low survival rates and the absence of a significant effect in the standard treatment of most patients with glioblastomas, the question of the use of immunotherapy for malignant gliomas of the brain was raised. However, to decision this problem, it is necessary to consider the interaction of the immune system with tumors of this group. The modern view of the interaction of immunity and glioblastoma is presented. Special attention is paid to the mechanisms of tumor escape and suppression of the functional activity of immune system. The current immunotherapeutic approaches in the treatment of patients with glioblastoma are presented. Interaction of glioblastoma with immune system at all the stages of tumor growth is a complex process. While planning the immunotherapy of this pathology it’s necessary to take into consideration all mechanisms used by the tumor cells to avoid the immune response and suppress it. However, the clinical studies of this type of therapy proved to be less successful than expected. Further detailed studies of immune-resistance and escape of gliobloastoma must contribute to working out more effective immunotherapy tactics.

Pediatrician (St. Petersburg). 2022;13(2):49-60
pages 49-60 views

Наследственные болезни обмена

Lysosomal storage diseases. Sphingolipidoses — Fabry, Gaucher and Farber diseases

Gorbunova V.N., Buchinskaia N.V., Janus G.A., Kostik M.M.


Sphingolipidoses are genetically heterogeneous group of rare monogenic metabolic diseasesб caused by inherreted deficiency of enzymes involved in the degradation of sphingolipids. Sphingolipids are catabolized in lysosomes, where glycohydrolases degrade them by separation of terminal sugars to core ceramide. All sphingolipidoses are characterized by abnormal deposition of a large amount of sphingolipids and other unsplit products of lipid metabolism, mainly in parenchymal organs, bone marrow and brain. Among sphingolipidoses, such groups of diseases as glycosphingolipidoses, gangliosidoses and leukodystrophies are distinguished. This review presents the epidemiology, clinical, biochemical and molecular characteristics of the three main types of glycosphingolipidoses — Fabry disease, Gaucher disease and Farber disease, caused by the mutations in the genes of α-galactosidase A (GLA), glucocerebrosidase (GBA) and acid ceramidase (ASAH1), respectively. Currently, there is an increased interest in glycosphingolipidoses due to the identification of the spectrum and frequencies of mutations in the GLA, GBA and ASAH1 genes in various populations, including Russia, and the practical availability of individual molecular diagnostic methods. A description of the existing experimental models, their role in the study of the biochemical basis of the pathogenesis of these severe hereditary diseases and the development of various therapeutic approaches are given. We discuss, firstly, the possibility of early diagnosis of Fabry disease, Gaucher and Farber based on neonatal screening and examination of high risk groups of patients in order to improve the effectiveness of their prevention and treatment, as well as (secondly) the advantages and disadvantages of the main approaches to the treatment of these serious diseases, such as bone marrow and hematopoietic stem cell transplantation, enzyme replacement therapy, substrate reduction therapy, gene therapy and genome editing.

Pediatrician (St. Petersburg). 2022;13(2):61-88
pages 61-88 views

Clinical observation

Clinical case of neuronal ceroid lipofuscinosis type II in a child

Belykh N.A., Starodubtseva Y.B., Sologub M.A., Pisnyur I.V.


Neuronal ceroid lipofuscinosis is a group of hereditary neurodegenerative diseases inherited by an autosomal recessive trait. This disease is the most common neurodegenerative pathology in children with a prevalence of 1:1,000,000 to 1:14,000 in the world. Currently, 14 genetically different forms of this pathology have been identified, which are characterized by the accumulation of abnormal lipofuscin-like material in the lysosomes of nerve cells, progressive and selective destruction of neurons. In neuronal ceroid lipofuscinosis, there is a defect in the gene that translates various lysosomal enzymes. Due to the insufficiency of the enzyme tripeptidyl peptidase 1, an accumulation of pathological autofluorescent lipopigment is observed in the central nervous system (CNS) of the patient, leading to violations of the normal function of neurons. Clinically, the disease manifests at the age of 3–4 years, in the form of progressive myoclonic epilepsy, mental and motor disorders, delays and stops in development. The article describes data on the prevalence, clinical features and therapy of this pathology, and also presents a clinical case with the onset of the disease in a child aged 2 years 11 months. The clinical case demonstrates the difficulties of diagnosing this pathology due to the rarity of this pathology, a wide range of differential diagnostics, the duration and high cost of molecular genetic studies. An early diagnosing would make it possible to explain the nature of epilepsy, to choose a rational therapy for this disease in a timely manner.

Pediatrician (St. Petersburg). 2022;13(2):89-97
pages 89-97 views

Staphylococcal-scalded skin syndrome, a clinical case

Milyavskaya I.R., Revnova M.O., Leina L.M., Felker E.Y., Mineeva O.K., Bolshakova E.S.


Staphylococcal scalded skin syndrome (SSSS) is the most severe form of staphyloderma in newborns and young children. The disease is associated with the production of exfoliatin toxin by Staphylococcus aureus, which breaks down desmoglein 1 in the cells of the granular layer of the epidermis, which leads to the formation of superficial blisters. The differential diagnosis is with toxic epidermal necrolysis (Lyell’s syndrome, TEN). The development of TEN is more often associated with the use of drugs such as sulfonamides, anticonvulsants, antibiotics, etc. As an illustration of the difficulty of differential diagnosis, we present our clinical observation of a 1-year-old girl. The girl was admitted in serious condition to the intensive care unit with a diagnosis of Lyell’s syndrome. At the admission, extensive skin lesions were noted in the form of multiple flaccid blisters and erosions. At the same time, the mucous membranes were not affected. The department was diagnosed with staphylococcal scalded skin syndrome. Thus, the differential diagnosis of the syndrome of SSSS and TEN presents certain difficulties. When making a diagnosis, it is necessary to take into account the anamnesis, clinical manifestations, paying special attention to the defeat of the mucous membranes.

Pediatrician (St. Petersburg). 2022;13(2):99-107
pages 99-107 views

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