LITERATURE REVIEWS
Surfactant is a secreted surface-active substance that provides the first breath of the newborn by reducing the surface tension of the alveoli. Surfactant preparations were the first and most popular drugs in neonatology, saving the lives of millions of premature babies. Successful treatment with surfactants is preceded by an individual personalized approach to each child and early detection of newborns with surfactant deficiency. Determining the indications for surfactant therapy, choosing an effective method of drug delivery, selecting concomitant respiratory support are current areas of neonatology to this day. The article reflects the history of use, new approaches to surfactant therapy and methods of administration of various surfactant preparations.
Renal scintigraphy remains the most informative method for imaging and assessing kidney function in children to this day. This method allows for the detection of scarring foci in the renal parenchyma and enables the prediction of the progression of chronic kidney disease. The ability of artificial intelligence to transform routine medical imaging into a fully automated diagnostic tool is currently being widely explored. In addition to qualitative analysis performed on renal medical imaging, texture analysis has been integrated with machine learning methods as a quantitative assessment of renal tissue heterogeneity, providing a promising additional tool in predicting renal function decline.
ORIGINAL ARTICLES
Magnetic resonance imaging is a sensitive method for visualizing changes in hypoxic-ischemic encephalopathy. Several classifications of the severity of such changes have been developed to date. Different classifications suggest different degrees of differentiation of the anatomical landmarks used, and in some cases, interpretation of MRI data is a problem for a general radiologist and a clinician. Objective. The aim of the work was to develop a classification accessible to a general radiologist and understandable to neonatologists, pediatricians, and neurologists.
Material and methods. The study group included 164 children with MRI signs of hypoxic-ischemic damage on MRI, regardless of the referring diagnosis, which made it possible to avoid the influence of anamnestic data on its formulation. The anamnestic and clinical data that were the most informative during the risk analysis were analyzed: complication of the mother’s anamnesis for somatic non-infectious and infectious pathology, complications of the obstetric-gynecological anamnesis, prolonged anhydrous interval, depression and seizures in the early postpartum period.
Results. Correlation of neuroimaging data, anamnesis and clinical picture allowed us to formulate a classification of MRI changes in newborns with hypoxic-ischemic brain damage: Grade I (mild) includes changes in the area of the external watershed and the posterior leg of the internal capsule; Grade II (moderate) — to those characteristic of the first degree are added those in the area of the internal watershed and the anterior leg of the internal capsule; At the III degree (severe) — to the changes in the area of the external and internal watersheds, the posterior and anterior legs of the internal capsule, a picture of selective neuronal necrosis is added, that is, damage to the basal ganglia and (or) thalamus; To the IV degree belong gross structural changes in the brain tissue with the formation of gliosis, cystic cavities, atrophy of the brain substance. We characterized them as gross. Phenomenological features of intraventricular hemorrhages do not allow them to be used as a market or a criterion for the severity of hypoxic-ischemic changes, and this sign can be used as an additional one, to clarify their nature.
Conclusion. The classification reflects the dynamics of the ischemic process and is available for use not only by doctors of radiation diagnostics, but also by clinicians — neonatologists, neurologists, pediatricians.
It is relevant to search for new opportunities for comprehensive rehabilitation of children with cerebral palsy in order to create highly effective rehabilitation programs based on the methods of Chinese traditional medicine.
The purpose of the study: to increase the effectiveness of comprehensive rehabilitation of children with spastic forms of cerebral palsy by including traditional Chinese methods in the rehabilitation program.
Methods: the study included 59 children with spastic forms of cerebral palsy. The average age was 2.9 ± 0.17 years. There were 30 girls (50.8%). In the main group, standard rehabilitation was used and additionally used methods of Chinese traditional medicine; in the comparison group there was only standard rehabilitation. To determine the effectiveness of complex medical rehabilitation, a modified Ashworth scale and a gross motor function scale (GMFCS) were used, and the adaptive potential of the circulatory system and the type of adaptive reactions were studied.
Results. When comparing adaptive potential after rehabilitation, no statistically significant difference was obtained (p=0.913). Stress reactions were not detected in any of the subgroups (p=0.927). In the main group, a statistically significant decrease in muscle tone was revealed (p=0.001), and improvement in motor functions according to the GMFCS scale was expressed (p=0.04).
Conclusion. The presence of positive dynamics of muscle tone in children with spastic forms of cerebral palsy allows us to conclude that the inclusion of methods of Chinese traditional medicine in comprehensive rehabilitation increases the effectiveness of rehabilitation measures.
Purpose. To evaluate the effect of intravitreal aflibercept on renal and hepatic function in preterm infants treated for retinopathy of prematurity.
Material and methods. A prospective cohort study was conducted, which included 16 children treated with aflibercept since 2021 for retinopathy of prematurity. Ultrasound with Doppler was used to assess the condition of the kidneys and liver. The study was carried out on modern equipment using linear and convex sensors.
Results. Most children (75–90%) did not show significant structural changes in the liver and kidneys. Average organ sizes and blood flow indices were within the age norm. The identified cases of pyelectasis and additional vessels correspond to the population frequency in premature infants and do not require correction of therapy. Pathological changes in the liver were absent in all examined children.
Conclusions. The results of the study indicate the safety of aflibercept in the treatment of retinopathy of prematurity in terms of kidney and liver health. The changes identified correspond to the general health of premature infants and cannot be unambiguously associated with the use of the drug. Further studies with a larger sample and a longer observation period are needed to clarify the long-term effects of VEGF inhibitor therapy.
It is known that biopsy and DNA sequencing methods are used to diagnose mitochondrial diseases, but such diagnostic options are not available for all tissues, especially for the central nervous system. This article analyzes the effectiveness of biomarkers — FGF-21 and GDF-15, lactate, pyruvate, creatinine, acylcarnitines — in the diagnosis of mitochondrial dysfunction.
The aim of the work: to improve the diagnostic panel of biomarkers for mitochondrial disorders in children based on a number of potential laboratory parameters, such as FGF-21 and GDF-15 lactate, pyruvate, creatinine, acylcarnitines.
Materials and methods. Eighty-two patients aged 1 to 17 years were analyzed. The levels of these biomarkers were measured in patients with suspected mitochondrial diseases. For each biomarker, sensitivity and specificity, area under the curve (AUC) were calculated.
Results. FGF-21 showed an AUC of 0.862 ± 0.091 and a sensitivity of 80% in patients with mitochondrial disorders, with specificity ranging from 88 to 100%. GDF-15 demonstrated the highest AUC among the studied biomarkers (0.898 ± 0.103), a sensitivity of 0.92, and a specificity of 91 to 100%, indicating its particularly high efficacy. The study of lactate revealed that with its increased level, there is a sensitivity of 75% in patients with mitochondrial disorders (MD), which was confirmed in 37 cases out of 52, and a specificity from 85 to 100%, the average AUC was 0.861 ± 0.049, and for creatinine with an increased level, the sensitivity was lower and was 35.7% (19 out of 52 children with MD. For pyruvate, AUC was 0.835 ± 0.106, confirming its usefulness as an additional biomarker together with lactate.
Conclusion. The studies have shown that the new indicators FGF-21 and GDF-15 are particularly distinguished by their diagnostic value and can be recommended for wide use in medical practice.
Epilepsy is one of the important problems of modern neurology. Various epileptic syndromes can be comorbid with other pathologies, in particular, with excess weight. Currently, various aspects of the etiopathogenesis of epileptic seizures against the background of obesity are actively studied.
Objective. The aim of the work is to identify the main metabolic and molecular genetic changes in epilepsy in children associated with obesity in order to develop a personalized approach to the diagnosis of this pathological condition.
Materials and methods. The study included 22 patients aged from 1 year to 16 years (mean age 11.7 years): 16 patients with various epileptic syndromes against the background of obesity and 6 patients of the comparison group. The study of the profile of 60 organic acids in urine was carried out by high performance liquid chromatography and tandem mass spectrometry. Whole exome sequencing was performed in 5 patients using a Helicon G50 genetic analyzer.
Results. In the group of patients with epilepsy against the background of obesity, the upper reference limit of a number of markers of the Krebs cycle (fumaric and 2-ketoglutaric acids), bacterial dysbiosis and lactic acid levels were exceeded. Also, in the sample of patients, in contrast to the comparison group, an increase in the levels of a number of markers of mitochondrial dysfunction was found. Whole exome sequencing of 5 patients with epilepsy against the background of obesity did not reveal known pathological mutations.
Conclusion. Thus, we have identified a number of metabolic markers associated with epilepsy in children against the background of obesity. Molecular genetic testing of a number of patients did not reveal significant pathological mutations. The results of the study confirm the importance of a personalized approach to the diagnosis and treatment of this pathological condition and further study of metabolomic and molecular genetic markers of this pathological condition.
CLINICAL CASES
The most common cause of infravesical obstruction in children is the posterior urethral valve. At least a third of patients with this diagnosis by puberty face the progression of chronic kidney disease and dilation of the upper urinary tract. The most likely cause of the listed complications is intrauterine urethral obstruction, which affects the development of the entire urinary tract. Compliance with diagnostic and treatment algorithms can lead to a decrease in complications in this group of children and an increase in their quality of life.
The report aims to illustrate the successful comprehensive and staged treatment of a patient with a posterior urethral valve using chemodenevation of the bladder detrusor.
Materials and methods. A clinical case of the treatment of a 2-year-old patient with a posterior urethral valve and a subclinical infectious process is presented. The patient received surgical care using intraluminal endosurgical methods and surgical interventions with open traditional correction methods, without taking into account the age, anatomical, and pathophysiological features of the urinary tract. These errors subsequently led to the development of chronic kidney disease stage 3. Moving forward, the child required rehabilitation and preoperative correction of complications to prepare for the repeated radical elimination of urinary diversion disorders and the restoration of urinary tract urodynamics.
Results. The child underwent successful surgical treatment using a combined endosurgical approach after staged chemodenervation of the bladder detrusor with Botulinum toxin type A. This included extravesical mobilization of the ureters on both sides and the classic correct formation of ureterocystoanastomosis according to the Cohen method. In the 1.5-year follow-up, a significant improvement in the urine passage through the urinary tract, relief of the infectious process, stabilization of nitrogen metabolism parameters, and glomerular filtration rate are noted.
Conclusion. Logistics and surgical treatment of such a complex developmental defect as the posterior urethral valve should always be built taking into account the age-specific anatomy and the characteristics of pathological changes in order to achieve the desired goal.
Introduction. Hydronephrosis in children is a well-studied disease in terms of etiopathogenesis, examination methodology and surgical methods. In the practice of a pediatric urologist-andrologist, it is challenging to exclude multifactorial pathological processes that are caused by multi etiologies. In such cases, the diagnostic algorithm can be expanded, and the tactics of surgical treatment changes depending on the new data obtained. The purpose of the report is to demonstrate the algorithm of diagnostic approaches with diagnosis verification and subsequent successful one-stage combined surgical treatment of patient with hydronephrosis on the right, caused by an aberrant vessel in combination with nephroptosis.
Materials and methods. On the basis of clinical observation, the case history of a 14-year-old girl with a combined pathology — nephroptosis and hydronephrosis on the left, caused by an additional lower polar vessel, is presented. For two years, clinical manifestations of the disease were recurrent headaches and pain in the lumbar region on the left, for pain relief the patient regularly took non-steroidal anti-inflammatory drugs (NSAIDs). Despite, the typical results of the instrumental examination for hydronephrosis, the algorithm was expanded, due to the peculiarities of the clinical picture, which included headaches, lower back pain, and episodes of increased blood pressure. As a result of additional examination, a combined pathology was detected — nephroptosis on the left. Based on the results, the child underwent one-stage laparoscopic antevasal pyeloplasty with nephropexy. The result of the surgical treatment was the relief of pain syndrome — the complaints of headaches and pain in the lumbar region disappeared, normalization of blood pressure figures, restoration of urodynamics, by the results of instrumental investigations it was confirmed a reduction in the size of the renal collecting system and a decrease in its mobility during orthostatic testing. Conclusions. One-stage laparoscopic nephropexy and isolating pyeloplasty can be considered the operation of choice for nephroptosis combined with an additional lower polar renal vessel. If the algorithm for performing this surgical treatment and postoperative management is followed, a satisfactory result is most likely achieved.
SHARING EXPERIENCES
A team of authors reported their outcomes of treatment of 9 children with ROHHAD syndrome and severe respiratory insufficiency at the stage of formation of ventilator dependency. The diagnostic criteria were evaluated based on different clinical variables and the incidence of the disease. This article highlights the problems of early diagnosis of respiratory insufficiency, prevention of severe hypoventilation complications and the prosthesis of respiratory function. The authors discussed an efficacy of diaphragm pacing as an alternative method for traditional mechanical ventilation.
Personalized medicine is methods for preventing of pathological conditions, diagnosing and treating based on the individual characteristics of the patient. Prevention of hereditary and congenital pathologies is the main goal of institutions and departments of medical genetic services. The need for genetic testing of children with congenital and hereditary diseases is 25,3%. The diversity of hereditary diseases was established based on the results of the study and analysis. The capabilities of the genetic laboratory are shown and the use of methods of molecular genetic diagnosis in children is scientifically justified. It has been shown that confirmation of more than 50% of genetic diseases by molecular genetic methods makes possibility to implement genotype-phenotype correlations and plan further medical observation of the child. The clinical effectiveness of whole exome sequencing to establish molecular diagnosis of hereditary and congenital pathologies was 73,3%. As part of the implementation of the strategy for the development of molecular genetic diagnostics, the spectrum of identified nucleotide sequence variants in genes responsible for hereditary and congenital pathologies was analyzed. Based on the obtained data and identified options, recommendations were given to doctors on the effectiveness of treatment, and individual rehabilitation programs were developed, in some cases excluding surgical correction of the defect in patients with movement disorders.
The article presents the experience of organizational and therapeutic work as part of a mobile specialized pediatric surgical team during the period of liquidation of the medical consequences of devastating earthquakes. An analysis of the treatment of children with open bone fractures and soft tissue injuries at all stages of medical evacuation was carried out, the main causes of complications and adverse outcomes were diagnosed and identified. Special attention is paid to therapeutic, tactical and surgical errors, incorrectly performed operations and primary surgical treatment of wounds, which were carried out at the stage of inpatient treatment in children before the arrival of mobile specialized pediatric surgical team. In all cases, the errors presented were the direct causes of severe purulent-septic complications. The guideline of surgical treatment of complications and the features of management of this patient population are substantiated. The experience of treating children with complicated course of soft tissue injuries and open fractures indicates the need for complex, multi-stage operations under general anesthesia in most of the victims. A rational scheme of medical, organizational and advisory work in the general system of medical and sanitary provision for children in emergency situations, developed and consistently applied by mobile specialized pediatric surgical team specialists in practice, is presented, namely, the interaction of the hospital (the main hospital for the concentration and treatment of the children’s contingent) based with other medical institutions and hospitals where injured people were admitted and treated after the earthquake. children.
GUIDELINES FOR THE PRACTITIONER
Hemorrhagic disorder of the newborn (or Vitamin K Deficiency Bleeding, VKDB) is a pathological condition caused by a deficiency of vitamin K, which is necessary for the synthesis of blood clotting factors, in children aged 8 days to 6 months. Vitamin K Deficiency Bleeding manifests as bleeding from various localizations. The late form of disease is typically diagnosed after the first week of life and can present as severe intracranial hemorrhages, which in turn can lead not only to residual neurological symptoms but also to fatal outcomes. This condition is observed in newborns who have not received prophylactic vitamin K administration at the delivery and are exclusively breastfed. This underscores the necessity for prevention, timely diagnosis, and treatment. Diagnosis is based on the medical history, clinical picture, and laboratory blood clotting analyses. The updated clinical recommendations for Vitamin K Deficiency Bleeding prevention have been implemented globally and in Russia, but the concerns of management children with late form of Vitamin K Deficiency Bleeding are still relevant. This article presents three clinical cases of full-term infants with late forms of Vitamin K Deficiency Bleeding. They did not receive prophylactic vitamin K administration and were exclusively breastfed. Special attention is given to the diagnosis and treatment of neurological disorders associated with intracranial hemorrhage, as well as the importance of timely prevention to avoid complications.
HISTORY OF PEDIATRICS
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