Aim: to establish the patterns of changes in leukocyte blood parameters to determine their diagnostic significance in the different activity of Crohn’s disease (CD) and ulcerative colitis (UC) in children.

Materials and methods. 370 5–18-year children (198 boys, 172 girls) with inflammatory bowel disease (IBD) were examined. Leukocytic blood parameters, concentrations of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), faecal calprotectin (FC) levels were determined. Changes in clinical activity in Crohn’s disease (CD) and ulcerative colitis (UC) cases were determined using pediatric activity indices: PCDAI and PUCAI, respectively. Analysis of endoscopic activity in 160 CD and UC patients was determined using the scales: SES-CD and UCEIS, respectively.

Results. It was found that with an increase in the clinical activity of CD in children, the absolute numbers of leukocytes, lymphocytes and monocytes did not change significantly. In contrast, a significant increase in neutrophil reactivity was noted. In patients with high endoscopic CD activity an increase in the absolute number of monocytes and neutrophil reactivity was found. In patients with high clinical activity of UC, an increase in the number of leukocytes and the absolute number of neutrophils was found. At the same time, the granularity and reactivity of neutrophils increased from remission to moderate and high clinical activity of UC. In children with high endoscopic UC activity, the number of leukocytes, the absolute number of neutrophils and monocytes increased. At the same time, the granularity of neutrophils significantly increased as the endoscopic activity of UC increased from remission to moderate activity. Leukocyte parameters in UC patients correlated moderately with FC level, and the absolute number of neutrophils was closely related to its content. The granularity and reactivity of neutrophils positively correlated with ESR and CRP levels in children with CD and CRP concentrations in children with UC.

Conclusion. The established patterns of changes in the quantitative composition of leukocytes and their functional properties (reactivity and granularity) are directly determined by the different activity of IBD in children and have diagnostic value.

Contribution:
Semikina E.L., Tsvetkova V.S., Potapov A.S. — research concept and design;
Tsvetkova V.S., Surkov A.N., Lokhmatov M.M., Kopyltsova E.A., Akulova S.S. — collection and processing of material;
Tsvetkova V.S., Semikina E.L. — statistical processing;
Tsvetkova V.S., Semikina E.L. — text writing;
Fisenko A.P., Semikina E.L., Potapov A.S. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: May 21, 2021
Accepted: June 23, 2021
Published: July 16, 2021

Introduction. Children with cystic fibrosis often have gastrointestinal symptoms despite properly selected primary therapy and pancreatic substitution therapy. A possible reason is lactase deficiency.

Aim is to determine the frequency of lactase deficiency in children with cystic fibrosis and the need for appropriate dietary correction of their diets.

Materials and methods. In 213 children with cystic fibrosis, during esophagogastroduodenoscopy in biopsies of the small intestinal mucosa 359 rapid tests, «Lactose intolerance quick test” were performed. According to the results of an express test, some patients diagnosed with hypolactasia to determine their tolerance to lactose underwent a glycemic load test with lactose and measured the level of fecal calprotectin to assess the level of faecal calprotectin the degree of chronic intestinal inflammation.

Results. Normal lactase activity was observed in 129 (36%) biopsies studied, moderate hypolactasia in 91 (25%) biopsies, severe hypolactasia in 139 (39%) biopsies. In patients with moderate hypolactasia, according to the express test results, 20% confirmed intolerance to lactose as a result of the load test. In patients with severe hypolactasia, 72% were confirmed to be lactose intolerant, according to the results of an express test. There was no correlation between the level of calprotectin and the results of the glycemic load test with lactose.

Conclusion. Lactase deficiency is observed in a significant proportion of patients with cystic fibrosis, which may be a consequence of primary adult hypolactasia and chronic intestinal inflammation. In children with cystic fibrosis with dyspeptic symptoms persisted despite correctly prescribed basic therapy and pancreatic enzymes, it is necessary to exclude lactose intolerance and prescribe a low-lactose diet.

Contribution:
Roslavtseva E.А., Simonova O.I. — concept and design of the study;
Sokolov I., Lokhmatov M.M. — collection of material;
Sokolov I., Bushueva T.V. — statistical processing;
Roslavtseva E.А., Sokolov I., Simonova O.I., Borovik T.E., Chernevich V.P. — writing of the text;
Simonova O.I., Smirnov I.E., Borovik T.E., Bushueva T.V. — editing.
All authors — approval of the final version of the manuscript, responsibility for integrity.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: May 24, 2021
Accepted: June 23, 2021
Published: July 16, 2021

Introduction. Vaccination is the primary method of preventing hepatitis B (HBV). Immunization performed according to the standard schedule often provides protective level of antibodies against HBV. However, the frequency deviation of the immunization schedule in children due to unjustified medical contraindication is the current problem in Russia. At the same time, there is currently no clear strategy for patients with significant deviations of the vaccination schedule, especially regarding extending the interval between the first and third administration of the HBV vaccine. The aim is to evaluate the immunological effects of vaccination against hepatitis B in the vaccination schedule deviation in healthy children and children with chronic diseases.

Materials and methods. Eighty-one 0.7–11.7 year child with a disrupted schedule of vaccination against HBV was observed. The children were divided into two groups: children vaccinated within 12–35 months (group 1, n = 48) and children immunized more than 36 months after the first vaccination (group 2, n = 33). Children with chronic forms of pathology were included in both study groups. Blood tests for HBV antibodies after vaccination were performed 1–3 months after the third vaccination.

Results. The average concentration of antibodies in the range of 10–1000 mMEd/ml in children of group 1 was significantly higher than in children of group 2 (p = 0.037). In addition, children with chronic diseases were significantly more likely to have an anti-HBs titer higher than 1000 mMEd/ml after the third vaccination than healthy children (p = 0.012).

Conclusion. An increase in the interval between the first and third administration of the hepatitis B vaccine leads to a rise in the number of children who are not immune to hepatitis B. Chronic diseases fail to affect the immune response due to the introduction of the hepatitis B vaccine, even if the immunization schedule is disrupted.

Contribution:
Tkachenko N.E., Yasakov D.S. — the concept and design of the study;
Yasakov D.S., Vershinina M.G., Alyabieva N.M. — collecting and processing material;
Yasakov D.S., Makarova S.G. — writing text;
Fisenko A.P., Makarova S.G. — text editing.
All authors — approval of the final version of the article.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 11, 2021
Accepted: June 23, 2021
Published: July 16, 2021

An increase in the incidence of whooping cough has been noted in recent years in both unvaccinated and vaccinated children and adults. In Russia, recently, new possibilities of protection against pertussis infection have appeared for children over four years old and adults. Purpose: to analyze the specific immunoprophylaxis and safety of the vaccine against diphtheria, tetanus and pertussis for children over four years old in Krasnodar.

Materials and methods. We examined 393 children over four years and 125 adults who were immunized with a vaccine for the prevention of diphtheria (with a reduced antigen content), tetanus, pertussis (acellular), combined, adsorbed (Adasel) at the SKIB city hospital in Krasnodar in the period from July 2018 until October 2019. The respondents had a vaccination history and did not suffer from whooping cough, according to medical records.

Results. The number of people vaccinated with acellular vaccine doubled among the child population (2018 — 129; 2019 — 264) and adults (2018 — 39; 2019 — 86). General post-vaccination reactions among children amounted to 12.2% (48), weak responses prevailed — 36 (9.2%), strong responses were found in 12 people (3%). In adults, general post-vaccination responses were not observed. Local post-vaccination reactions among children amounted to 38.2% (150), weak reactions prevailed — 103 (26.2%), strong ones were found in 47 children (12%).

Conclusions. During the study period, immunoprophylaxis with this vaccine doubled, showing a satisfactory safety profile. However, to reduce the incidence of whooping cough, it is advisable to examine the immunological protection and conduct revaccination with a booster dose to achieve stable post-vaccination immunity.

Keywords: children; pertussis infection; vaccination; adsorbed vaccines; safety

For citation: Sutovskaya D.V., Burlutskaya A.V., Dubova L.V., Krylova D.R. Use of a combined acellular vaccine against pertussis infection in children over four years of age: a cross-sectional study. Rossiyskiy Pediatricheskiy Zhurnal (Russian Pediatric Journal). 2021; 24(3): 168–172. (In Russian). https://doi.org/10.46563/1560-9561-2021-24-3-168-172

For correspondence: Diana V. Sutovskaya, 

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Informed consent: informed consent was received from the patients parents for the participation of a study.

Received: May 21, 2021
Accepted: June 23, 2021
Published: July 16, 2021

Purpose: to compare the course of the disease in the dilated phenotype of cardiomyopathy with a non-compact myocardium and increased trabecularity, verify the molecular genetic diagnosis using the new generation sequencing method, and study the segregation of nucleotide variants in families.

Materials and methods. The study included 50 patients, divided into two groups: 27 patients with a dilated phenotype of cardiomyopathy and non-compact myocardium and 23 patients with a dilated phenotype and increased trabecularity. Changes in the laboratory and instrumental parameters, events and outcomes were analyzed. The massively parallel sequencing of a panel of genes developed at the National Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation (81 genes) was applied. For data processing, the IBM SPSS Statistics 24.0 application package was used for bioinformatic analysis and assessment of the pathogenicity of the identified nucleotide variants, the Russian guidelines for interpreting human DNA nucleotide data, Alamut software and the HGMD Professional database were used.

Results. Following a year of therapy for chronic heart failure in DF CMP patients, the content of terminal natriuretic peptide in the blood of patients with increased trabecularity was found to decline significantly. In patients in both groups, myocardial contractility improved and left ventricular end-diastolic size decreased. Significant nucleotide variants when using the cardiopanel were verified in 85% of cases in patients with non-compact myocardium and 91% in patients with increased trabecularity. At the same time, predictors of poor prognosis and severe course of cardiomyopathy were identified — pathogenic variants c.2647G>A in the MYH7 gene, c.688G>A in the TPM1 gene, c.2350C> T in the CACNA1C gene. In one clinical case, when laminopathy was detected, a cardioverter-defibrillator was installed as prophylaxis for sudden death. In addition, 18 families were examined, 3 cases of de novo mutation were identified, confirming the high frequency of asymptomatic and low-symptom carriers of nucleotide variants.

Conclusion. The determination of the molecular and genetic cause of the dilated cardiomyopathy phenotype allows optimizing the management tactics of sick children. Furthermore, the identification of family segregation of mutations with the identification of carriers ensures timely monitoring by specialists.

Contribution:
Sdvigova N.A., Basargina E.N., Savostyanov K.V. — concept and design of the study;
Sdvigova N.A., Basargina E.N., Savostyanov K.V., Pushkov A.A. — collection and processing of material;
Sdvigova N.A. — statistical processing, text writing;
Basargina E.N., Savostyanov K.V., Pushkov A.A., Zharova O.P. — text editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgement.  The study had no financial support

Conflict of interest. Authors declared no conflict of interest.

Informed consent: informed consent was received from the patients’ parents for the participation of a study.

Received: May 20, 2021
Accepted: June 23, 2021
Published: July 16, 2021

The number of premature babies, including those with extremely low birth weight (ELBW), is steadily growing every year. In surviving ELBW infants, retinopathy of prematurity (ROP) more often develops, which has a more severe course, depending not only on the degree of immaturity and somatic burden of premature babies but also on the quality of nursing.

The aim of this work is to determine the risk factors for ROP progression in profoundly premature ELBW infants to optimize the tactics of their treatment.

Materials and methods. We studied the case histories of 155 surviving premature ELBW infants with ROP of various stages treated in perinatal centres of the Belgorod region in 2014–2019.

Results. In premature ELBW infants, ROP of varying severity was found to be diagnosed in 90% of cases. The anamnesis of the mothers of the examined patients with moderate and severe ROP was dominated by unfavorable preceding pregnancies (miscarriages, silent miscarriage and ectopic pregnancies, medical abortions).The need for preterm infants in mechanical ventilation was established to depend on the severity of ROP and the duration of mechanical ventilation (p < 0.05). Frequent, not always justified red blood transfusions leading to the replacement of fetal haemoglobin with adult haemoglobin are also essential factors in ROP progression.

Contribution:
Dyumin I.I., Balakireva E.A. — the concept and design of the study;
Dyumin I.I., Nikolaenko I.V. — collection and processing of material;
Dyumin I.I., Sevostyanov A.I. — statistical processing;
Dyumin I.I., Sevostyanov A.I., Yaroshevich E.A. — writing text;
Balakireva E.A., Yaroshevich E.A. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: May 31, 2021
Accepted: June 23, 2021
Published: July 16, 2021

The purpose of the work is to determine the clinical and pathogenetic markers of secondary kidney damage in endocrine diseases in children to optimize the diagnosis of secondary nephropathies.

Materials and methods. We examined 120 children with endocrinopathies aged 3 to 17 years: with secondary kidney damage in type 1 diabetes mellitus (25), exogenous constitutional obesity (20), autoimmune thyroiditis (15) and 60 children with endocrine diseases without kidney damage. All children underwent a clinical and paraclinical examination with an assessment of the endocrine and nephrological status: determination of the parameters of the lipid spectrum, glycemic profile, indicators of daily monitoring of blood pressure and the functional state of the kidneys.

Results. Structural and functional parameters of the kidneys in patients with secondary nephropathies in endocrine diseases are characterized by: impaired echographic parameters of the kidneys and a decrease in intrarenal hemodynamics; increased blood pressure and hyperfiltration increased albuminuria/proteinuria in combination with dyslipidemia (increased low-density lipoprotein cholesterol, triacylglycerides, decreased high-density lipoprotein cholesterol), impaired carbohydrate metabolism (increased glycated haemoglobin levels, impaired glucose tolerance).

Conclusion. The optimization of the diagnosis of secondary nephropathies in endocrine diseases in children is discussed based on the determination of a complex of clinical and pathogenetic factors that affect the formation of kidney pathology in children with endocrine diseases.

Contribution:
Kutsenko L.V., Vyalkova A.A., Zorin I.V. — concept and design of research, collection and processing of material;
Kutsenko L.V. — writing text;
Vyalkova A.A. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all its parts.

Acknowledgement. The study had no financial support.

Conflict of interest. Authors declared no conflict of interest.

Informed consent: informed consent was received from the patients’ parents for the participation in a study.

Received: May 31, 2021
Accepted: June 23, 2021
Published: July 16, 2021

A significant increase in the incidence of enterovirus infections (EVI) with the development of multiple organ damage does not exclude the involvement of the urinary system organs in the pathological process.

Materials and methods. 151 EVI children (103 cases with enterovirus meningitis (EVM), 48 children with other forms of EVI without damage of central nervous system) aged from 1 to 18 years. The frequency and nature of changes in urinary sediment and kidney function in patients with various EVI forms were studied. A highly sensitive marker of acute kidney injury — KIM-1 was used as a marker along with standard methods (determination of blood creatinine level with calculation of glomerular filtration rate) in 25 EVM patients.

Results. Pathological changes in urinary sediment in EVI are registered in 37.75% of cases (27.1–42.72%, depending on the clinical form), more often in preschool children, are represented by unexpressed proteinuria and abacterial leukocyturia, and are of a short-term transient nature. 40% of EVM children patients showed an increase in the KIM-1 level above the standard values.

Conclusion. The detection of KIM-1 in the urine of EVM children patients indicates the possible development of acute transient tubulointerstitial renal damage. At risk there are children with cerebrospinal fluid pleocytosis greater than 200×10⁶/L, abacterial leukocyturia, and decreased glomerular filtration rate.

Contribution:
Kazartseva N.V., Kokoreva S.P. — research concept and design;
Kazartseva N.V. — collection and processing of material, statistical processing, text writing;
Kokoreva S.P. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 18, 2021
Accepted: June 23, 2021
Published: July 16, 2021

Introduction. The impact of a mother’s MS on the intrauterine and postnatal development of the infant remains controversial. Aim: to study the influence of mother’s MS, and pathogenetic therapy, on the formation of psychomotor skills in first-year infants under comparison with a group of infants from healthy mothers (“case-control”).

Materials and methods. Рrospective observational study (“case-control”) at months 3, 6, 9 and 12. The WHO-recommended scale of motor development (WHO six milestones), 46 infants from MS mothers and 46 infants from the control group.

Results. The duration of the mother’s MS disease, the severity of the neurological deficit, and the disease course’s activity was found not significantly to affect the motor and neuropsychiatric development of the child in the first year of life. Significant differences were established between the groups of children and patients with MS from the control group in terms of mastering the skills of standing and walking; the presence of better motor parameters in children from patients receiving pathogenetic therapy was established too. The negative influence of interferons in the first trimester on speech skills in infants was revealed.

Conclusion. The main influencing factor was mother multiple sclerosis and the pathogenetic therapy received by the mother before and during pregnancy. This fact can play a significant role in managing this infant cohort. Special exercises such as stimulation of motor and speech skills need to be included.

Contribution:
Vinogradova T.V., Kasatkin D.S. — study concept and design;
Vinogradova T.V. — collection and handling of material;
Vinogradova T.V., Kasatkin D.S. — statistical analysis;
Vinogradova T.V. — writing up the manuscript;
Sitnikova E.P. — text editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: May 22, 2021
Accepted: June 23, 2021
Published: July 16, 2021

The aim of the study was to assess the features of the course of new coronavirus infection in newborns and children of the first year of life.

Materials and methods. Thirty-six children aged eight days to 11 months were hospitalized in the covid centre of the National Medical Research Center for Children’s Health (of which nine children were transferred from one of the surgical hospitals in Moscow due to the contact with SARS-CoV-2).

Results. Despite severe concomitant surgical pathology in 25% of patients, 98% of children had mild to moderate disease. Febrile temperature (up to a maximum of 39 °C) was observed in 4 infants, sub-febrile temperature in 5 people. In other children, for the entire period of treatment, there was no increase in temperature. Mothers’ complaints about lethargy dominated the clinical picture of the disease, followed by refusal to eat, runny nose, sneezing, swallowing voice, coughing, shortness of breath in their children. During the examination, the following signs were revealed: yawn hyperemia, mucous separated from the nose, hard breathing and dry wheezing (in 1 infant). The general blood test showed anemia (mostly related to prematurity anemia, or iron deficiency anemia), leftward leukocytosis (due to concomitant surgical pathology), leukopenia, various changes in the leukocytic formula of blood. In some instances, thrombocytopenia increased in D-dimer C-RP, procalcitonin, ferritin, bilirubin, and alanine aminotransferase. In only six infants with a moderate disease course, changes characteristic of a new coronavirus infection were identified on the chest CT scan. Six infantas had rhythm disorders detected in electrocardiographic examination (no deviations were found in echocardiographic examination).

Conclusion. According to both foreign literature and data obtained during our study, it can be concluded that, compared with adults, infants have a milder course of the disease and less pronounced changes obtained during laboratory and instrumental studies.

Contribution:
Kharitonova N.A. — concept and design of the study, writing the text;
Kharitonova N.A., Illarionova M.S., Nesterovich M.I. — collection and processing of material;
Kharitonova N.A., Basargina M.A. — statistical processing, editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 18, 2021
Accepted: June 23, 2021
Published: July 16, 2021

Aim. To demonstrate a clinical case of orphan hereditary disease from the group of lysosomal storage diseases — Pompe disease (glycogenosis type II) in a five-year child.

Materials and methods. Anamnestic data, clinical and laboratory parameters and treatment of a five-year child with Pompe disease. The patient was observed in the gastroenterological department of the Children’s Regional Clinical Hospital.

Results. Child admitted with complaints of muscle weakness, difficulty climbing stairs, rare headaches. According to his history, his mother turned to the pediatrician about the prolonged course of a respiratory infection. An outpatient examination revealed a significant increase in the levels of alanine aminotransferase and aspartate aminotransferase. Viral hepatitis was excluded. The child was hospitalized in a hospital for further examination with hepatitis, unspecified etiology (non-infectious). Examination of the child revealed a lag in physical development, muscle hypotension, hepatomegaly, liver dysfunction, and increased total creatine phosphocanase and IgE. A decrease in the activity of acid α-1,4-glucosidase in the spot of dried blood was determined according to tandem mass spectrometry and gene mutations GAA DNA diagnostic method. Diagnosed with Pompe disease (glycogenosis type II). Myopathic syndrome. The treatment with the genetic engineering enzyme-substituting drug Mayozayme was started at a dose of 20 mg/kg, intravenously, once every two weeks.

Conclusion. Pompe disease is a rare pathology characterized by a low frequency of prevalence and polymorphism of clinical manifestations, complicating the diagnosis. Timely diagnosis of the disease and the earliest possible appointment of pathogenetic therapy are required to improve patients’ quality of life, slow down the progression of the disease, and prevent the development of life-threatening complications.

Contribution:
Shashel V.A., Firsova V.N. — concept and design, collection of material;
Trubilina M.M. — writing the text, processing;
Shashel V.A. — editing.
All co-authors — approval of the final version of the clinical case, responsibility of all parts of the article.

Compliance with ethical standards: The study was conducted in full compliance with ethical standards defined in the Declaration of Helsinki. Official representatives of the patient gave free written informed consent to publish the clinical case description and photomaterials.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 3, 2021
Accepted: June 23, 2021
Published: July 16, 2021