Regional Children's Hospital Research Articles

Cutting into the NICU: Improvements in Outcomes for Neonates with Surgical Conditions.

The Children's Hospitals Neonatal Consortium (CHNC), established in 2010, seeks to improve care for infants with medically and surgically complex conditions who are cared for in level IV regional children's hospital NICUs across North America. Through patient-level individual data collection, comparative benchmarking, and multicenter quality improvement work, CHNC has contributed to knowledge and improved outcomes, leveraging novel collaborations between and across institutions. Focusing on antenatal and inpatient care for infants with surgical conditions including congenital diaphragmatic hernia, gastroschisis, and necrotizing enterocolitis, we summarize the progress made in these infants' care. We highlight the ways in which CHNC has enabled multidisciplinary and multicenter collaborations through the facilitation of diagnosis-specific focus groups, which enable comparative observations of outcomes through quality improvement and research initiatives. Finally, we review the importance of postbirth hospitalization needs of these infants and the application of telemedicine in this population.

Clinical structure and features of the course of pain and non-specific somatoform disorders in adolescent children

Annotation. The research is devoted to the study of clinical signs of painful and nonspecific somatoform disorders in children of early and late adolescence, depending on sex, place of residence, and study. The study examined 56 adolescent children with pain and non-specific syndromes who were treated in the MNP “Khmelnytskyi Regional Children's Hospital” of the Khmelnytskyi Regional Council and in the MP “Khmelnytskyi City Children’s Hospital” of the Khmelnytsky City Council. The average age of the children was 14,0±4,53 years. Among them, children of early adolescence (10-14 years) accounted for 57.2%, and late adolescence (15 years 11 months 28 days) – 42.8%. The clinical signs of pain and nonspecific somatoform disorders in the children we examined were as follows: headache, thermoregulation disorder, fibromyalgia and arthralgia syndrome, nonspecific back pain, asthenic syndrome with decreased work capacity, increased meteorological sensitivity, and impaired concentration. Nonspecific persistent pain syndrome occurred in 56 children, in particular, cephalic pain – in 77.4 %, polyarthralgia – in 8.9%, fibromyalgia syndrome – in 8.5%, nonspecific back pain – in 13.4% of children; thermoregulatory disorders were identified in 42.7%, asthenic syndrome – in 35.3% of children. It shows that among pain somatoform disorders, headache, in particular, tension headache and migraine, occupies the first rank in children. Stress, tension, and sleep disturbances were predominant among the triggering factors. Therefore, among adolescent children with painful and non-specific somatoform disorders, cephalalgia, with a predominance of tension headaches, and long daytime low fevers are most often diagnosed, as a manifestation of autonomic somatoform dysfunctions with impaired thermoregulation. At the same time, migraine, episodic symptoms associated with migraine, as well as trigeminal variants of headache and long-term pain of other localization were found in isolated cases. The age dependence of primary headache has been revealed: tension headaches occur more often in children of early adolescence, and migraines in children of older adolescence, which is important to consider in the differential diagnosis of headaches, since the treatment and prevention of attacks of these forms of headache are completely different.

Results of treatment of patients with newly diagnosed immune thrombocytopenia: is it justified to follow clinical recommendations?

Immune thrombocytopenia (ITP) is an acquired immune-mediated disease characterized by isolated transient or persistent thrombocytopenia < 100·109/L. The incidence of immune thrombocytopenia is 4–6,4 per 105 children/year.Purpose. To analyze the results of treatment newly diagnosed immune thrombocytopenia according to the clinical guidelines (ID699) published on the website of the Ministry of Health of Russia.Material and methods. The analysis included 13 patients (F–46%, M–54%, median age — 9.5 (4–17) years) with immune thrombocytopenia admitted to the Tver Regional children hospital in 2023. A history of infection preceding the immune thrombocytopenia was in 69% of cases and measles vaccination in 8%. The median period from the onset of infection was 11 (5–15) days. Degree of bleeding was — 1 gr. — 4 (31%), 2 gr. — 3 (23%), 3 gr. — 6 (46%). Hematuria was observed in 3 (23%), menorrhagia in 1 (8%) case. The mean platelet count at the time of admission was 9,0 (1.0–86) · 109/l.Results. The incidence was 5.7 per 105 children/year. Dexamethasone 20 mg/m2, days 1–3, was used in 54% of cases; IVIG 1000 mg/kg, day 1 in 15%, prednisolone 2 mg/kg, day 21 in 8% and in 23% of cases dynamic follow-up was performed. In 2 cases (17%), discontinuation of steroid therapy and switching to IVIG was required due to a hemorrhagic syndrome deterioration or/and complication of steroid therapy. Partial and complete responses were achieved in 8 (62%) and 4 (31%) cases respectively. In 1 (8%) case, the response could not be evaluated. The summary efficacy of first-line therapy was 92%.Conclusion. The clinical guidelines ID699 was highly effective in achieving a primary response and preventing recurrence of immune thrombocytopenia in children.

Building large-scale registries from unstructured clinical notes using a low-resource natural language processing pipeline

Building clinical registries is an important step in clinical research and improvement of patient care quality. Natural Language Processing (NLP) methods have shown promising results in extracting valuable information from unstructured clinical notes. However, the structure and nature of clinical notes are very different from regular text that state-of-the-art NLP models are trained and tested on, and they have their own set of challenges. In this study, we propose Sentence Extractor with Keywords (SE-K), an efficient and interpretable classification approach for extracting information from clinical notes and show that it outperforms more computationally expensive methods in text classification. Following the Institutional Review Board (IRB) approval, we used SE-K and two embedding based NLP approaches (Sentence Extractor with Embeddings (SE-E) and Bidirectional Encoder Representations from Transformers (BERT)) to develop comprehensive registry of anterior cruciate ligament surgeries from 20 years of unstructured clinical data at a multi-site tertiary-care regional children's hospital. The low-resource approach (SE-K) had better performance (average AUROC of 0.94 ± 0.04) than the embedding-based approaches (SE-E: 0.93 ± 0.04 and BERT: 0.87 ± 0.09) for out of sample validation, in addition to minimum performance drop between test and out-of-sample validation. Moreover, the SE-K approach was at least six times faster (on CPU) than SE-E (on CPU) and BERT (on GPU) and provides interpretability. Our proposed approach, SE-K, can be effectively used to extract relevant variables from clinic notes to build large-scale registries, with consistently better performance compared to the more resource-intensive approaches (e.g., BERT). Such approaches can facilitate information extraction from unstructured notes for registry building, quality improvement and adverse event monitoring.

Prevalence and Predictors of Concomitant Meniscal Surgery During Pediatric and Adolescent ACL Reconstruction: Analysis of 4729 Patients Over 20 Years at a Tertiary-Care Regional Children's Hospital.

The rate of concomitant meniscal procedures performed in conjunction with anterior cruciate ligament (ACL) reconstruction is increasing. Few studies have examined these procedures in high-risk pediatric cohorts. That (1) the rates of meniscal repair compared with meniscectomy would increase throughout the study period and (2) patient-related factors would be able to predict the type of meniscal operation, which would differ according to age. Cohort study (prevalence); Level of evidence, 2. Natural language processing was used to extract clinical variables from notes of patients who underwent ACL reconstruction between 2000 and 2020 at a single institution. Patients were stratified to pediatric (5-13 years) and adolescent (14-19 years) cohorts. Linear regression was used to evaluate changes in the prevalence of concomitant meniscal surgery during the study period. Logistic regression was used to determine predictors of the need for and type of meniscal procedure. Of 4729 patients (mean age, 16 ± 2 years; 54.7% female) identified, 2458 patients (52%) underwent concomitant meniscal procedures (55% repair rate). The prevalence of lateral meniscal (LM) procedures increased in both pediatric and adolescent cohorts, whereas the prevalence of medial meniscal (MM) repair increased in the adolescent cohort (P = .02). In the adolescent cohort, older age was predictive of concomitant medial meniscectomy (P = .031). In the pediatric cohort, female sex was predictive of concomitant MM surgery and of undergoing lateral meniscectomy versus repair (P≤ .029). Female sex was associated with decreased odds of concomitant LM surgery in both cohorts (P≤ .018). Revision ACLR was predictive of concomitant MM surgery and of meniscectomy (medial and lateral) in the adolescent cohort (P < .001). Higher body mass index was associated with increased odds of undergoing medial meniscectomy versus repair in the pediatric cohort (P = .03). More than half of the young patients who underwent ACLR had meniscal pathology warranting surgical intervention. The prevalence of MM repair compared with meniscectomy in adolescents increased throughout the study period. Patients who underwent revision ACLR were more likely to undergo concomitant meniscal surgeries, which were more often meniscectomy. Female sex had mixed effects in both the pediatric and adolescent cohorts.

АБДОМИНО-СКРОТАЛЬНОЕ ГИДРОЦЕЛЕ: ОПЕРАЦИОННАЯ ТАКТИКА У ДЕТЕЙ

Abdominoscrotal hydrocele: tactics of the surgical treatment in children. Abdominoscrotal hydrocele (hydrocele en-bisac, dumbbell-shaped hydrocele) is a type of noncommunicating hydrocele consisting of scrotal and abdominal components connected by an isthmus in the inguinal canal. Until now, this disease has been a casuistry because since 1777 only 579 cases have been recorded in the world. In the surgical children's department of the Minsk Regional Children's Hospital during the period from October 2022 to October 2023 this diagnosis was made intraoperatively to 5 children. The paper presents the experience of using laparoscopic hydrocelectomy of the abdominal component through a single-trocar in combination with laparoscopic extraperitoneal ligature herniorrhaphy. The operation technique and the results of the treatment are described in the article. According to the authors, the given surgical technique has good prospects for wide practical application.

RESULTS OF BACTERIOLOGICAL MONITORING OF URINARY TRACT INFECTIONS IN CHILDREN OF ZAPORIZHZHIA REGION IN 2023

The aim of the study. To investigate the structure of pathogens of acute urinary tract infections in children of Zaporizhzhia region in 2023 and their sensitivity to antibacterial agents. Materials and methods. The analysis of the structure of pathogens in acute urinary tract infections and the determination of their sensitivity to antibiotics based on the results of bacteriological urine cultures of 322 children with acute urinary tract infections treated at the Zaporizhzhia Regional Children's Hospital in 2023 was performed. For each isolate, the multidrug resistance index was calculated, which allowed to judge the relative integral resistance of the isolated microorganisms to antibacterial drugs. Results. It was established that the most common cause of urinary tract infections in children were gram-negative microorganisms (61.0%), including Escherichia coli (37.4%) and Klebsiella pneumoniae (10.8%) which were most often verified. Gram-positive bacteria were identified in 38.5% of patients, among which the Enterococcus bacteria (29.2%) predominated. High resistance of Escherichia coli to amoxicillin/clavulanate (31.5%), ciprofloxacin (30.1%), cefepime (20.1%), and ceftazidine (20.5%) was detected, including 29 (39.7%) multidrug-resistant isolates of Escherichia coli. The highest resistance of Klebsiella pneumoniae was found to amoxicillin/clavulanate (76.2%), cefepime (33.3%), ceftazidine (33.3%), ciprofloxacin (42.8%), and phosphamycin (28.6%). Multidrug resistance was shown by 76.2% of Klebsiella pneumoniae isolates. The highest susceptibility of Enterococcus bacteria was found to ampicillin (98.2%), linezolid (98.2%), vancomycin (100%), nitrofurantoin (100%), and tigecycline (100%). In no case microorganisms showed complete sensitivity to imipenem, and 17.9% of Enterococcus isolates were resistant to this antibiotic. Conclusions: At the present stage, the dominance of gram-negative microflora among the pathogens of urinary system infections in children remains, among which Escherichia coli (37.4%) continues to be the leading pathogen. The growing role of Enterococcus faecalis (29.2%) as an etiologic factor of urinary tract infections in children was noted, which should be taken into account during the empirical use of antibiotics, especially if there is no effect within the first two days after the start of therapy.

Improving Pharmacist-Led Pediatric Patient Education on Oral Chemotherapy at Home.

Oral chemotherapy (OC) has been increasingly used in pediatric patients diagnosed with cancer, which is primarily managed in the outpatient setting. Different from adults, pediatric patients face unique challenges in administering these hazardous medications at home. Because of the complexity of pediatric pharmaceutical care and the hazardous nature of chemotherapy agents, comprehensive patient education is imperative to mitigate the potential safety risks associated with OC administration at home. Pharmacists play a vital role in patient education and medication consultations. However, the lack of practice guidelines and limited resources supporting OC counseling are noted. Additional barriers include insufficient knowledge and training on OC, which can be improved by continuing education. In a regional children's hospital, a comprehensive OC education checklist was developed for pediatric patients and their caregivers to standardize consultations led by pharmacists. An infographic OC handout was also formulated to improve patient knowledge and awareness. Moreover, innovative approaches such as using telepharmacy, smartphone applications, and artificial intelligence have been increasingly integrated into patient care, which can help optimize OC consultations for children and adolescents. Further studies are warranted to enhance oral chemotherapy education specifically tailored for pediatric patients in outpatient settings.

Age and anatomical-functional nuances of traumatic brain injury in children

The purpose. Traumatic brain injury in children is a serious medical problem that requires special attention and research. As the child's body develops and grows, the anatomical and functional characteristics of children can affect the pathological consequences of traumatic brain injury. Given the growing number of children who are injured in sports events, accidents and other factors, understanding the characteristics of this category of injury can play an important role in improving their treatment and rehabilitation. Children respond to injury differently than adults and may exhibit different symptoms and require specialized medical care and approaches to treatment and rehabilitation. Understanding these age and anatomical and functional differences can help improve the diagnosis and treatment of brain injury in children and increase their chances of full recovery and normal development. Materials and methods. 154 charts of inpatients aged from 1 month to 17 years who were receiving inpatient treatment at the regional children's hospital during 2022 were analyzed. The basic methods of examination were radiographs, MRI of the brain.Research results. The most craniocerebral traumas were observed in children aged 7-17 years, which constituted 47.4%. Children aged 3-6 were often injured, accounting for almost 20%, older children (15-17 years old) were injured less often, accounting for 15%. Babies under one year old (9.7%) and children under 2 years old (8.4%) suffered the least amount of damage. The largest number of injured children (60.4%) was observed in the warm season when they are active outside without proper parental supervision.Conclusions. The largest number of brain injuries is characteristic of adolescence, against the background of changes in all body systems, the formation of new behavioral reactions for them, which were not characteristic of them before, and contribute to the increase in traumatism. Babies suffered the least damage, which is due to the anatomical features of the structure of the child's head.

Неспецифічний аортоартеріїт (хвороба Такаясу): клінічний випадок у педіатричній практиці

The article presents modern data on a relatively rare and life-threatening disease - non-specific aortoarteritis (Takayasu's disease) and its features in childhood. This disease belongs to the group of systemic vasculitis, which is based on non-specific non-infectious inflammation of the aorta and its branches, which leads to deformation of the vascular lumen and secondary tissue ischemia. Damage to vital organs in this disease leads to the development of severe complications and the risk of sudden death. Purpose - to cite a case of Takayasu's disease from our own practice, taking into account the rarity of the disease, lack of awareness and clinical vigilance of pediatricians and family doctors regarding this pathology. A clinical case of non-specific aortoarteritis in a 17-year-old patient who was examined and treated at the Ivano-Frankivsk Regional Children's Hospital was considered. Features of clinical symptoms, laboratory biomarkers, methods of visualization the level of lesions of main vessels are given. Information on methods of treatment of this patient according to the updated recommendations of the European Antirheumatic League (EULAR, 2018) is provided. Conclusions. Nonspecific aortoarteritis is a rare, difficult to diagnose and insufficiently studied autoimmune disease. Late diagnosis significantly increases the risk of a child's sudden death. Modern non-invasive imaging methods are crucial for making a diagnosis, assessing the extent of the disease and choosing treatment tactics. The research was carried out in accordance with the principles of the Declaration of Helsinki. The informed consent of the child's parents and the patient was obtained for the description of the clinical case. No conflict of interests was declared by the authors.

Discharge Best Practices of High-Risk Infants From Regional Children's Hospital NICUs.

Lack of discharge preparedness after NICU hospitalization is associated with risk of readmission and parental stress. Complex infants cared for at regional children's hospital NICUs would benefit from a systematic approach to transition home. Our objective was to identify potential best practices for NICU discharge and examine priorities for incorporating these best practices in regional children's hospital NICUs. We used techniques from quality improvement, including fish bone and key driver diagrams, yielding 52 potential best practice statements for discharge preparation. Using the modified Delphi method, we surveyed stakeholders on their level of agreement for the statement to be included in the final guideline regarding discharge processes and parental education. Consensus was defined as 85% agreement among respondents. To identify implementation feasibility and understand unit-level priorities, a prioritization and feasibility assessment survey was used to rank the top best practices and performed gap analyses for the first prioritized intervention. Fifty of the 52 statements met the predefined criteria for consensus. The prioritization survey of potential best practice statements named assessment of families' social determinants of health with a standardized tool as the top priority among respondents. Conducting gap analyses enabled an understanding of current practice, barriers, and affordances, allowing for implementation planning. This multicenter and interdisciplinary expert panel reached a consensus on multiple potential best practices for complex discharge preparation from regional children's hospital NICUs. Better support for families navigating the complex NICU discharge process has the potential to improve infant health outcomes.

Triglyceride glucose index, pediatric NAFLD fibrosis index, and triglyceride-to-high-density lipoprotein cholesterol ratio are the most predictive markers of the metabolically unhealthy phenotype in overweight/obese adolescent boys.

The prevalence of obesity constantly increases worldwide and definitely increases the risk of premature death in early adulthood. While there is no treatment yet with proven efficacy for the metabolic clamp such as arterial hypertension, dyslipidemia, insulin resistance, diabetes type 2, and fatty liver disease, it is imperative to find a way to decrease cardiometabolic complications. Early prevention strategies beginning in childhood are the most logical step to reduce future cardiovascular morbidity and mortality. Therefore, the aim of the current study is to determine the most sensitive and specific predictive markers of the metabolically unhealthy phenotype with high cardiometabolic risk in overweight/obese adolescent boys. This study was carried out at the Ternopil Regional Children's hospital (Western Ukraine) and involved 254 randomly chosen adolescent overweight or obese boys [median age was 16.0 (15.0,16.1) years]. A control group of 30 healthy children with proportional body weight comparable in gender and age to the main group was presented. A list of anthropometrical markers with biochemical values of carbohydrate and lipid metabolism with hepatic enzymes was determined. All overweight/obese boys were divided into three groups: 51.2% of the boys with metabolic syndrome (MetS) based on the IDF criteria; 19.7% of the boys were metabolically healthy obese (MHO) without hypertension, dyslipidemia, and hyperglycemia; and the rest of the boys (29.1%) were classified as metabolically unhealthy obese (MUO) with only one criterion (hypertension, dyslipidemia, or hyperglycemia). Based on multiple logistic regression analysis that included all anthropometric and biochemical values and calculated indexes in boys from the MHO group and MetS, it was revealed that the maximum likelihood in the prediction of MetS makes the combination of triglyceride glucose index, pediatric nonalcoholic fatty liver disease fibrosis index (PNFI), and triglyceride-to-high-density lipoprotein cholesterol ratio (R2 =0.713, p<0.000). By tracing the receiver operating characteristic curve, the model is confirmed as a good predictor of MetS (AUC=0.898, odds ratio=27.111 percentage correct=86.03%) in overweight and obese boys. Triglyceride glucose index, pediatric NAFLD fibrosis index, and triglyceride-to-high-density lipoprotein cholesterol ratio are a valuable combination of predictive markers of the metabolically unhealthy phenotype in Ukrainian overweight/obese boys.

Surgically treated pediatric tongue masses: A 20Year review.

Pediatric tongue lesions represent a rare, diverse group of pathologies related to congenital malformations, inflammatory changes, and neoplastic masses. We present the largest-to-date series of pediatric tongue lesions managed surgically. Charts of all pediatric patients with a tongue lesion surgically treated at a regional children's hospital from February 1st, 1997 to August 30th, 2019 were reviewed. After obtaining institutional review board approval, a retrospective review was completed of all pediatric patients with a tongue lesion surgically treated at a regional children's hospital from February 1st, 1997 through August 30th, 2019. Charts were evaluated for patient demographics as well as symptoms at presentation, surgical details, pathologic evaluation of the lesion, and rates of recurrence. Categorical variables were compared between various groups using Chi-square and/or Fisher's exact tests. Univariate and multivariable logistic regression analysis was performed unadjusted and adjusted to further explore these relationships. 159 patients were included in the study with ages distributed relatively even across infants (27.7%), children 1-5 years (27.7%), 6-10 years (23.3%), and greater than 10 years of age (21.4%). Patient/family reported race/ethnic background was most commonly Caucasian (34.8%) or Hispanic/Latino/Spanish Origin (34.0%) followed by Black or African American (21.3%). The most commonly reported symptoms were pain (23.3%), difficulty with oral intake (12.6%), bleeding (11.3%), and respiratory distress (11%). Patients with lesions of the base of tongue were significantly more likely to present with respiratory distress or difficulty with oral intake (p<0.001). The likelihood of recurrence was observed to be higher with lesions managed in infancy (OR 3.85, CI 1.47-10.0, p=0.005), those greater than 2cm (OR 3.45, CI 1.34-8.71, p=0.009), or with laser (OR 4.52, CI 1.25-15.00), p=0.015). Pediatric patients with tongue lesions may present with a wide array of symptoms. Base of tongue lesions should be managed carefully as they are most likely to create barriers to the airway and oral intake. Larger lesions and those managed in infancy are at increased risk for recurrence.

Penza Regional Children's Hospital named after N.F. Filatov

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Functional neurological disorder in children and young people: Incidence, clinical features, and prognosis.

To report incidence, demographic and clinical characteristics, and symptom outcome of functional neurological disorder (FND) in children. Children diagnosed with FND at a regional children's hospital were prospectively recruited by weekly active surveillance for 36 months. Demographic, clinical, and follow-up data were retrospectively extracted by review of electronic records. Descriptive statistical analyses were used. Ninety-seven children (age range 5-15 years) met the case definition of FND (annual incidence 18.3 per 100 000 children). Children with FND were likely to be female (n =68 [70%]) and older (median 13 years) with no difference in the Scottish Index of Multiple Deprivation (marker of socioeconomic status) compared with the general childhood population. Functional motor (41%) and sensory (41%) symptoms were most common; other somatic symptoms such as headache (31%) and pain (27%) were frequent. Self-reported psychiatric symptoms and infection/inflammation were the most common predisposing and precipitating factors respectively. At a median of 15 months follow-up, 49% of 75 children reported improvement or resolution of FND symptoms with no prognostic factors found. At this regional centre, FND in children had a higher incidence than previously reported and a less optimistic outcome than in some other studies.

Telehealth Coverage for a Pediatric Gastroenterology Consult Service at a Regional Hospital: An Accepted, Useful, and Sustainable Model of Care

To evaluate a novel telehealth inpatient pediatric gastroenterology (GI) consult service at a regional children's hospital in regard to acceptance, utility, quality, sustainability, and provider resiliency. Patients requiring GI care at a regional children's hospital between July 2020 and June 2021 were treated by an in-person or telehealth physician with physician assistant support, randomly assigned based on a weekly preset staffing schedule. A retrospective, multidomain program evaluation was performed based on the RE-AIM (reach, effectiveness, adoption, implementation, and maintenance) and STEM (SPROUT Telehealth Evaluation and Management) frameworks, using statistical analysis to compare the patient cohorts and anonymous surveys to assess provider perceptions. In total, 1051 patient-days of GI care were provided for 348 patients, 17% by telehealth and 83% in-person. There were no significant differences in diagnosis, transfer, or readmission rates between the cohorts. No transfers occurred for reasons other than need to access specialized services not available at the regional hospital. Daily consult workload was slightly greater for telehealth physicians. Primary and consult team providers accepted the practice. The model continued beyond the first year. In total, 75% of local GI physicians reported greater Brief Resilience Scores in the context of shifting 20% of their inpatient call weeks to another campus's physicians. Episodic pediatric GI consult service coverage via telehealth at a regional hospital was well accepted, useful, and sustainable, with improved physician resilience and no adverse outcomes seen. Telehealth holds promise for leveraging pediatric subspecialty physicians across hospitals, allowing complex patients to be admitted closer to home while reducing inpatient coverage requirements for smaller physician groups.

Anemic Syndrome in Children in the Children’s Regional Hospital

The study of a general blood test determining the nature of anemic syndrome in young children (0-12 years old) and children (3-6 years old) with infectious processes in the infectious department of the Penza Regional Children's Hospital was carried out. In the study, 4 groups were identified according to blood parameters in the age group 0-12 years. The study of the indicators of the general blood test showed that the first group cannot be attributed to the manifestations of severe anemia syndrome. In the second – normochromic, normocytic anemia syndrome. The third group had a manifestation of anemic normochromic, normocytic syndrome. In children in the fourth group, microcytosis of erythrocytes, anisocytosis, poikilocytosis and hypochromia were detected in blood smears. The data of the general blood test indicated the presence of concomitant anemia microcytic, hypochromic syndrome in children. And in children in the age group of 3-6 years, only in 25.8% of cases, the manifestation of anemic syndrome was found in the test’s of 1010 children. In children, the revealed anemic syndrome corresponded to the normochromic, normocytic process. Based on the data obtained, it can be concluded that young children (0-12 years old) and aged 3-6 years who are being treated for the underlying disease have concomitant anemia syndromes in most cases of normochromic, normocytic and in a minority of cases of hypochromic, microcytic nature.

THE KNEE JOINT IS THE ACHILLES' HEEL OF A CHILD'S BODY

The study objective was to draw practicing physicians’ attention to the risk of knee joint disorders in children during intensive growth to ensure timely detection and treatment. Materials and Methods. We analyzed the results of studies on the diseases of the knee joint carried out by modern scientists over the past ten years. We also took into account the clinical observations of children receiving inpatient treatment at the regional children's hospital. The examination methods were radiography, computer tomography, magnetic resonance imaging, and ultrasound. Results and Discussion. During growth, a child's knee joint is subjected to enormous loads and is sensitive to external and internal factors. The knee joint plays a dominant role in the limb’s longitudinal growth; the growth zones grow so intensively that they can be compared to the work of a "bone nuclear reactor." The bones of the skeleton grow unevenly and intermittently. The peak height velocity is observed at 10–14 years in girls and at 13–18 years in boys; then, height velocity slows down, and after 19 years, height growth almost stops. During the period of intensive growth, bones are very sensitive to internal and external factors of shape formation (growth hormones, physiological loads, radiation, vibration, injuries, hypothermia, metabolic disorders, etc. Therefore, this part is most sensitive to various endogenous and exogenous factors, which is manifested by various dysplastic changes and diseases. Conclusions. During the intensive growth of the child, the knee joint is most vulnerable to various endogenous and exogenous negative factors, which is manifested by various diseases. Osteogenic sarcoma, fibrous cortical defect, and juvenile osteocartilaginous exostoses most often affect the weakest place of the knee joint – the metaphyses of the femur and tibia (Achilles’ heel).

MARBLE DISEASE (CASE REPORT).

We present clinical case of marble disease in 5 yo girl. The management of this child was made in Vinnitsia Regional Children's Hospital (Vinnitsia, Ukraine). CBC, X-ray of bones, bone marrow biopsy, genetical testing, MRI of the brain and CT of the skull were done during this period. Marble disease is a very rare disease with very serious consequences, the prevention of which requires timely diagnosis and treatment, namely the prevention of infectious complications and early allogenic transplantation of stem cells. As it is a genetically determined disease, it is not possible to prevent the development of osteopetrosis. Genetic screening and proper treatment will allow the patient to lead an almost normal life.

КАДРОВЫЕ РЕСУРСЫ МЕДИКО-ГЕНЕТИЧЕСКОЙ СЛУЖБЫ ОТДЕЛЬНЫХ СУБЪЕКТОВ ПРИВОЛЖСКОГО ФЕДЕРАЛЬНОГО ОКРУГА

In the structure of major causes of infant mortality in most Russian regions, hereditary and congenital malformations rank second, substantiating an extraordinary medical and social significance of these diseases for practical healthcare. In this regard, neonatal screening was introduced as an important public health program to ensure early diagnosis of hereditary diseases. The work organization of medical and genetic consultations largely depends on the availability of medical personnel. The main problem of medical and genetic consultations is the imbalance in the number of medical personnel: across Federal districts, constituent entities of the Russian Federation; medical and nursing staff. The purpose of the study is to evaluate the number of medical personnel for medical and genetic consultations in individual regions of the Volga Federal District. Material and methods. With the help of the MedCalcstatisticalsoftware - freetrialavailable software package (Medcalc 2.0.), statistical processing of data on regional programs of the regions of the Volga Federal District, reporting forms of the Federal Statistics Service No. 30, official data from the websites of medical universities, and a sociological survey of the head non-staff specialists was carried out. Results. In most regions of the Russian Federation, medical and genetic consultations are part of the structural divisions of republican (regional) children's (perinatal) hospitals. Based on the analysis of staffing standards, a calculation and comparative analysis of the staffing level of medical and genetic consultations was carried out on the example of 7 regions of the Volga Federal District. This comparison made it possible to identify imbalance in the number of geneticists and specialists of other related specialties in the constituent entities of the Russian Federation, with due regard to size of residing population. Analysis of the geneticist staffing of the regions of the Volga Federal District under study showed that there is an imbalance associated with the number of residents of the district and the number of organized medical and genetic consultations. Analysis of the number of geneticists and other specialists of the medical and genetic service in the regions of the Volga Federal District allows us to identify a disparity between the practical number of medical personnel and the real need of the population for medical and genetic assistance. In the Russian Federation, training of health professionals is carried out in accordance with the nomenclature of specialties. Among all medical universities in Russia, residency training in the specialty "genetics" (as of August 2023) is conducted in 26 universities (15.5% of all universities in the Russian Federation). A significant concentration of universities in the European part of Russia and two training centers beyond the Urals have been shown. Lack of medical and organizational solutions can result in the near future in a limited availability of specialized medical care of the "genetics" specialty, which will lead to the increased rate of congenital malformations and other hereditary conditions, including higher childhood disability and mortality. Conclusions. A). Analysis of the human potential of the medical and genetic service in individual regions of the Volga Federal District showed a significant deficit, which is due to a number of reasons, including insufficient number of educational institutions in the regions, lack of the possibility to get specialty in medical genetics and laboratory genetics through professional retraining. B). The assessment of staffing with geneticists and other specialists of the medical and genetic service in the regions of the Volga Federal District identified a disproportion between the number of medical personnel and the real need of the population for medical and genetic care. C). The staff composition recommended by the Order of the Ministry of Health of the Russian Federation dated April 21, 2022 No. 274n "On approving the Procedure for providing medical care to patients with congenital malformations (or) hereditary diseases" can be implemented only after two calendar years, due to the duration of training in residency.