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Russian Pediatric Journal

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Vol 28, No 4 (2025)
https://doi.org/10.46563/1560-9561-2025-28-4

ORIGINAL ARTICLES

248-252 7
Abstract

Introduction. Crohn’s disease in girls is associated with a high risk of impaired sexual development and reproductive dysfunction. Surgical interventions required for complicated disease may further affect pelvic organs. Aim: to assess the reproductive system in girls with Crohn’s disease (CD) after surgical interventions.

Materials and methods. A prospective observational study included 56 CD girls aged of 3–17 years who underwent laparoscopic surgery. Anthropometric parameters, Tanner sexual development, menstrual function, gynecological status, pelvic ultrasound, frequency of adhesions and ovarian cysts were assessed. The course was analyzed before and after surgery.

Results. Before surgery, 53 patients had delayed sexual development, 34 had primary amenorrhea, 24 had pelvic adhesions, and 13 had ovarian cysts. Twelve months postoperatively, there was improvement in nutritional status, restoration of menstrual function in 49 of pubertal girls, reduced adhesions, and no recurrence of cysts.

Conclusions. Surgical treatment of CD in girls contributes to normalization of reproductive function, but requires long-term multidisciplinary follow-up.

Contribution:
Dyakonova E.Yu. — study concept and design;
Gusev A.A., Bekin A.S., Okulov E.A., Shchukina A.A., Tarzyan A.O. — material collection, surgical treatment;
Pereladova D.A. — data analysis, statistical processing.
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: July 02, 2025
Accepted: July 25, 2025
Published: August 28, 2025

253-258 7
Abstract

Introduction. One of the key manifestations of achalasia cardia (AC), a rare chronic esophageal disease, is severe dysphagia leading to protein-energy malnutrition, which worsens the results of surgical treatment.

Objective. Comprehensive assessment of the nutritional status in children with achalasia cardia.

Materials and methods. A retrospective analysis of twenty clinical AC cases was performed in children treated in the thoracic department of the National Medical Research Center for Children’s Health from 2019 to 2022. To assess the nutritional status of patients, BAZ (BMI/age) and HAZ (height/age) Z-criteria were used, calculated using the WHO Anthro Plus software (2009). The severity of clinical symptoms was assessed using the Eckardt scale, Statistical data processing was performed in StatTech v. 4.8.5 and Microsoft Excel.

Results. Pronounced disorders of the nutritional status were revealed in AC children: malnutrition was established in 12 children, while more severe disorders were noted in younger children. Patients with BAZ < –2 were hospitalized 2.3 times more often, stayed in hospital longer and more often required repeated surgical interventions. The severity of symptoms according to the Eckardt scale before surgery was higher in children with severe.

Conclusion. The study confirmed the high prevalence of nutritional deficiency in children with achalasia cardia, associated with a longer hospital stay, which justifies the need to assess the nutritional status throughout the entire period of hospitalization and its timely correction.

Contribution:
Burkina N.I., Bushueva T.V. — research concept and design of the study;
Burkina N.I., Bushueva T.V., Ratnikov S.A., Komina E.I. — collection and processing of material;
Burkina N.I. — statistical processing;
Burkina N.I., Bushueva T.V., Alkhasov A.B. — writing the text;
Bushueva T.V., Alkhasov A.B., Borovik T.E., Fisenko A.Р. — editing the text.
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 24, 2025
Accepted: July 25.2025
Published: August 28, 2025

259-267 4
Abstract
Introduction. With the continuous aggressive course of rheumatic disease and the presence of side effects of treatment with glucocorticosteroids (GCS), patients children are at high risk of developing disorders of nutritional status.
The aim of the work: to determine the changes in the nutritional status of children with rheumatic diseases against the background of therapy with GCS.
Materials and methods. 113 children aged 3 to 18 years with a confirmed diagnosis of a rheumatic disease (systemic lupus erythematosus, juvenile arthritis with systemic onset, juvenile dermatomyositis, systemic vasculitis) who received oral corticosteroid therapy at a daily dose of at least 0.15 mg/kg in terms of prednisolone for at least 3 months were examined. The analysis of clinical and anamnestic data and the course of the main disease was carried out, and anthropometric indices were calculated: Z-scores of body weight/age, height/age, and BMI/age using the Anthro and AnthroPlus programs. The data obtained were interpreted according to the WHO classification of nutritional disorders.
Results. Adequate indicators in the structure of the nutritional status of children with rheumatic diseases were established in less than half (n = 48; 42.5%) of patients. Excess body weight/obesity and malnutrition were detected in almost the same percentage of cases (28.3 and 29.2%, respectively). Children with systemic juvenile arthritis and juvenile dermatomyositis were predominantly overweight and obese (40% and 40.9%, respectively). Regardless of the nosological form of rheumatic pathology, malnutrition was observed in 25.5–32.2% of cases. At the same time, a significant inverse moderate correlation (rxy = –0.510; p < 0.001) was found between the duration of GCS use and the Z-score for height/age. The use of GCS therapy in the treatment of rheumatic diseases in children led to the development of severe complications such as drug-induced Cushing’s syndrome, steroid-induced osteoporosis and osteopenia, compression fractures of vertebral bodies, and aseptic bone necrosis. Steroid cataracts and musculoskeletal disorders were detected with equal frequency. Erosive-ulcerative lesions of the gastrointestinal tract were diagnosed somewhat less frequently.
Conclusion. The use of GCS improves the prognosis of rheumatic diseases in children. Timely diagnosis of metabolic disorders, pathology of the musculoskeletal and digestive systems, assessment of nutritional status in children with rheumatic diseases against the background of GCS therapy, and adequate correction of the identified disorders through the optimization of therapeutic diets will have a positive effect on the course and prognosis of rheumatic disease in children.
268-272 4
Abstract
Introduction. Assessment of nutritional status in children with severe psoriasis is very important, considering the high incidence of overweight and obesity in patients with psoriasis, disorders of lipid metabolism and glucose tolerance, and the pathogenetic relationship between these conditions and psoriasis. The aim: to assess the growth of children with severe psoriasis.
Materials and methods. A retrospective cohort study included 500 children (275 boys, 55%; 225 girls, 45%) aged of from 4 to 17 years 11 months with severe vulgar psoriasis (PASI > 10). Patients’ medical histories were used to obtain data. The severity of clinical manifestations of psoriasis was assessed using the PASI index. In all patients, clinical assessment, anthropometric data (height, weight) were performed on admission. Anthropometric data analysis was accomplished through Z-scores calculation with Anthro/AnthroPlus software.
Results. Adequate nutritional status was established in 66% of patients. Malnutrition was diagnosed in 34% of children. Overweight (19,5%) and obesity (13%) were detected in 32,6% of patients. Moderate acute undernutrition was diagnosed in 1.1%. A direct moderate statistically significant correlation (r = 0.58; p = 0.05) was revealed between the PASI and the WAZ, and a direct strong statistically significant correlation (r = 0.68; p = 0.02) between the PASI and body weight in children aged of from 4 to 7 years.
Conclusion. A trend toward more severe progression of skin lesions has been observed in patients with psoriasis with higher body weight indicers, suggesting an increased risk of developing metabolic syndrome in this category of patients and highlighting optimization of their nutrition.

PUBLIC HEALTH, MANAGEMENT AND SOCIOLOGY OF HEALTH CARE, MEDICAL AND SOCIAL EXAMINATION

273-281 4
Abstract
Introduction. Knowledge of the epidemiological characteristics of orphan diseases is necessary for developing solutions regarding financing and planning of medical care, improving routing, prevention, diagnosis, and treatment of patients. Aim: to analyze the trend in the incidence of eleven orphan diseases in children over a 10-year period.
Materials and methods. We analyzed official statistics for 2015–2024 for the following diseases: pituitary hyperfunction (ICD-10 code E22), hypopituitarism (E23.0), adrenogenital disorders (E25), phenylketonuria (E70.0), galactosemia (E74.2), Gaucher disease (E75.2), mucopolysaccharidosis (E76), cystic fibrosis (E84), multiple sclerosis (G35), Duchenne muscular dystrophy (G71.0), neurofibromatosis (Q85.0).
Results. The averaged index of total and primary morbidity for the period of 2020-2023 exceeded the average index for the period of 2015–2019 for three diseases: pituitary hyperfunction by 1.7 times and 1.9 times, respectively; multiple sclerosis — 1.4 times and 1.2 times; neurofibromatosis — 1.3 times and 1.5 times. The average annual growth rate of the number of patients with 11 orphan diseases was 4.3%. With preventive examination, 6.4% of new cases of diseases are detected. The period of taking children with orphan diseases for dispensary observation is reduced, which is subject to 85.5% (2020) to 96.7% (2023) of children.
Conclusion. There is an increase in the proportion of children with 11 orphan diseases among all patients (adults and children) from 15.9% in 2015 to 16.7% in 2024 (p < 0.001). Over the past 10 years, the total number of patients with orphan diseases has increased by 54 976 people, including children — by 10 077 people, and adults — by 44 899 people. This is due to the improved detection of pituitary hyperfunction, neurofibromatosis, as well as an increase in the primary incidence of multiple sclerosis. There are problems in identifying diseases subject to neonatal screening. An increase in the number of patients with orphan diseases is predicted.

REVIEWS

282-287 1
Abstract
Introduction. Artificial intelligence (AI) and neural networks are powerful tools that can analyze large amounts of data, identify patterns, and make forecastings. Ultrasound, radiography, scintigraphy, and the quantitative indices (degrees, sizes, indices) used in the diagnosis of urological diseases in children are an ideal object for training computer vision algorithms for the purpose of automatic analysis and calculation of the indices of interest to the doctor. Various AI models have been proposed for predicting treatment outcomes, risks of complications, and developing personalized therapy. AI can reduce the workload on medical personnel by automating routine tasks. A decision support system, remote patient monitoring capabilities, virtual simulators, chatbots, and assistants are technologies that are in high demand among health workers. However, there is a number of limitations to the use of AI. It is important to remember that algorithms are tested on ideally prepared data sets, while in real practice, doctors are faced with incomplete information, technical artifacts, and atypical cases. Therefore, for the successful integration of AI models into pediatric urology, it is necessary to ensure high quality of data for machine learning, security of collection and storage of personal patient data, and compliance with ethical standards. Aim. To analyze the literature data on the use of AI in pediatric urology in the world and Russia in the diagnosis of vesicoureteral reflux, hydronephrosis, hypospadias, posterior urethral valve.
A systematic search of scientific publications was conducted in the PubMed, Scopus, Google Scholar, eLibrary.ru databases for the period 2018–2024. The sample included clinical studies (n ≥ 50), meta-analyses, and PRISMA methodology was used for systematic reviews. The integration of AI technologies into clinical practice has great potential for solving clinical problems in pediatric urology. The limitations for successful implementation remain the insufficient reliability of existing models and the lack of algorithms adapted for the clinical use.

CLINICAL CASE

288-292 4
Abstract
Aortic coarctation is a congenital heart defect characterized by narrowing of the aortic isthmus, often remaining undiagnosed in childhood and adolescence. Aim. To describe a clinical case of diagnosis and treatment of aorta coarctation in the adolescent.
Materials and methods. This article presents a clinical report of a 15-year female patient with arterial hypertension resistant to standard therapy. The diagnosis of aortic coarctation was confirmed through comprehensive examination, including echocardiography, 24-hour blood pressure monitoring, and contrast-enhanced computed tomography.
Results. The patient successfully underwent surgical correction in the form of resection of the coarctation with aortic prosthetic grafting.
Conclusion. This case highlights the importance of excluding secondary causes of hypertension in adolescents, especially when treatment resistance is observed, and underscores the need for a multidisciplinary approach and modern diagnostic methods for the timely detection and management of congenital heart defects.
293-298 4
Abstract
Introduction. Eosinophilic and herpetic esophagitis are difficult for diagnosis. Moreover, their combination in immunocompetent children is rare. Aim. To present a clinical report on a combination of rare forms of esophagitis in the child and the role of endocytoscopy in the diagnosis of these conditions.
Materials and methods. A clinical case of herpetic and eosinophilic esophagitis in a 16-year boy with complaints of dysphagia and severe odynophagia is presented.
Results. At the esophagogastroduodenoscopy (EGD) and endocytoscopy with optical magnification up to 520× virus esophagitis was suspected. Histological examination of the mucosal biopsy and blood polymerase chain reaction (PCR) confirmed herpes virus types 1 and 2. In 3 months after the treatment, follow-up EGD with endocytoscopy revealed endomicroscopic signs of eosinophilic esophagitis (EoE). The patient was prescribed a proper therapy.
Conclusion. Modern endoscopic technologies, such as endocytoscopy, improve diagnosis, determine the volume and effectiveness of therapy of these esophageal lesions in children.
299-304 2
Abstract
Introduction. Autosomal dominant tubulointerstitial kidney disease (ADTID) is a rare genetically determined disease associated with a slowly progressive decline in renal function in the absence of changes in urine tests. Mutations in the genes for uropodulin (UMOD), renin (REN), mucin-1 (MUC1), and hepatocyte nuclear factor 1β (HNF1β) are known. Pathology in the UMOD gene is clinically manifested by minimal proteinuria (or its absence), a slowly progressive course of chronic kidney disease with onset in adolescence, and hyperuricemia. The aim: to present a clinical observation of autosomal dominant tubulointerstitial kidney disease associated with a mutation in the UMOD gene.
Materials and methods. A clinical description of a case of molecular genetic diagnosis of ADTID in the child with a complicated hereditary history on the mother’s side. All the patient’s close relatives had gout and chronic tubulointerstitial nephritis with a progression to chronic renal failure.
Results. Based on the patient’s medical history, the prognosis for autosomal dominant tubulointerstitial kidney disease remains unfavorable. Most patients eventually develop end-stage chronic kidney disease. In adolescents and young adults, the disease tends to progress more rapidly than in later-onset cases.
Conclusion. The low awareness of pediatricians and insufficient vigilance regarding rare forms of kidney pathology, coupled without specificity of their early clinical manifestations, make it difficult to detect the disease in a timely manner, which in turn worsens the prognosis for patients. ATDBP-UMOD presents a challenging diagnostic task that requires a comprehensive approach, including the analysis of family history, clinical presentation, and genetic testing results. This is crucial for early detection, timely initiation of treatment, and successful kidney transplantation.

HISTORY OF MEDICINE

305-310 2
Abstract
Kondratiy Ivanovich Grum-Grzhimailo (1794–1874), Doctor of Medicine, public figure, the publisher of the “Friend of Health” newspaper, author of a number of works on medicine, was one of the leading Russian doctors who contributed to the development in Pediatrics in Russia. The most significant work by K.I. Grum-Grzhimailo, “Guide to the Upbringing, Education, and Preservation of Children’s Health”, published in 1843–1844, contains the basic provisions on childhood hygiene typical of the mid-19th century. His works provide recommendations on the proper management of nutrition, conditions for keeping a child, maintaining and preserving children’s health, both physical and mental. The article analyzes Dr. Grum-Grzhimailo contribution to the formation of hygienic ideas about child care, and studies in detail his views on childhood hygiene, outlined in the “Guide to the Upbringing, Education, and Preservation of Children’s Health”. In K.I. Grum-Grzhimailo works a trend being still relevant today can be traced, which is aimed at popularizing advanced knowledge about childhood, publishing literature aimed not only at parents, but also at other adults interacting with children such as educators, nannies, teachers. Based on the works of K.I. Grum-Grzhimailo, materials from the newspaper “Friend of Health”, and archival data on the specialist’s activities, the authors of the article come to the conclusion about the importance of the paradigm he formulated, based on up-to-date medical data for its time and implying comprehensive attention to the physical and moral development of the child, for the development of childhood hygiene in the Russian Empire.


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ISSN 1560-9561 (Print)
ISSN 2413-2918 (Online)