Adequately organized nutritional support allows avoiding the development of protein-energy malnutrition in patients with bronchopulmonary diseases and cystic fibrosis (CF). The satisfying of increased protein and energy needs of CF patients can only be implemented under the administration.

The aim of the study is to analyze the tolerance and effectiveness of the use of domestic sterilized liquid specialized high-protein high-calorie mixtures «Nutrien Pulmo» and «Nutrien Energiya with  Dietary Fibers» in children over three years of age with acute (pneumonia) and chronic (cystic fibrosis) forms of bronchopulmonary pathology.

Patients and methods. Fifty-five children, including 41 patient suffered from CF, 14 cases with pneumonia, aged from 3 to 18years, were under observation. Of these, 23 (42%) children received Nutrien Pulmo, 32 (58%) children received Nutrien Ener­giya with Dietary Fibers. Nutritional status was assessed using the WHO-Anthro programs (for children from 3 to 5 years old) and WHO-Anthro Plus (for patients over five years) and clinical and biochemical blood test indices. The observation period was accounted for three weeks.

Results. All patients highly appreciated the taste of the products, there were no refusals to take specialized mixtures, neither adverse events during the observation period. While taking specialized mixtures, there was a positive trend in anthropometric indices and a significant improvement in biochemical markers of nutritional status: an increase in prealbumin, total protein, transferrin level.

Conclusion. The use of specialized domestic products «Nutrien Pulmo» and «Nutrien Energiya with Dietary Fibers» in the diet therapy of children with acute and chronic bronchopulmonary pathology has the safe and positive effect on the nutritional status of patients.

Contribution:
Bushueva T.V., Simonova O.I., Borovik T.E., Roslavtseva E.A. — the concept and design of the study;
Shen N.P., Tsiryatieva S.B., Lyabina N.V., Sokolov I.V., Burkina N.I., Chernyavskaya A.S. — collection of material;
Shen N.P., Tsirya­tieva S.B., Bushueva T.V. — statistical treatment;
Bushueva T.E., Borovik T.E., Roslavtseva E.A. — writing 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: March 22, 2021
Accepted: April 22, 2021
Published: May 14, 2021

The severity of the course of bronchopulmonary dysplasia (BPD) in the population changes due to the increase in the number of children born with very low and extremely low body weight, the introduction into the practice of new standards of respiratory support for premature infants, drug prevention and treatment of this pathology.

The aim of the work is to determine the influence of BPD form, gestational age, birth weight, concomitant diseases on the severity of its course in children.

Materials and methods. In 72 BPD children aged from 1 month to 3 years, the severity of the course of the disease was determined according to the computed tomography of the chest organs, using an assessment of the severity in points on a special scale as follows: 0–5 points for mild BPD, 6–10 points — moderate, 11–15 points — severe.

Results. In children born after 2012, a moderate course of BPD prevails (8.1 ± 0.32 points), being typical for both the classical (55.1%) and new (69.7%) forms of the disease. The classical form is more severe than the new one (9.0 ± 0.1 and 7.53 ± 0.38 points; p = 0.009). BPD was more severe in children with gestational age less than 28 weeks than in children with gestational age of 29–32 weeks (9.0 ± 0.4 and 7.4 ± 0.6 points; p = 0.000). BPD was more severe in children with birth weight less than 1000 g than in children with birth weight 1000–1500 g (8.79 ± 0.49 and 7.18 ± 0.61 points; p = 0.000). When BPD was combined with patent ductus arteriosus or pulmonary hypertension, the disease progressed more severely than in the absence of these forms of pathology (9.6 ± 0.8, 10.0 ± 0.7 and 7.22 ± 0.34 points; p = 0.031). The severity of changes detected by computed tomography of the chest organs decreases by the age of 3 years, but recovery was not complete in any of the examined patients: 8.6 ± 1.0 points at the age of 6 months and 6.2 ± 0.44 points by 3 years (p = 0.009).

Conclusion. In children aged 1 month to 3 years, the severity of BPD is influenced by the form of the disease, gestational age, birth weight, concomitant patent ductus arteriosus or pulmonary hypertension, and the age at which the examination is performed.

Contribution:
Kazakova K.A., Akoeva D.Yu. — research concept and design;
Kazakova K.A., Кustova О.V., Akoeva D.Yu., Davydova I.V. — material collecting and processing;
Kazakova K.A. — statistical processing, writing text;
Fisenko A.P., Kazakova K.A. — 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: March 22, 2021
Accepted: April 22, 2021
Published: May 14, 2021

Introduction. Autism spectrum disorders are a widespread pathology, but the etiological factors of their occurrence have not yet been established. The aim of the study was to compare the levels of I-FABP in children with autism spectrum disorders, depending on the adherence to a gluten-free diet.

Materials and methods. The study included 36 patients with autism committed to a gluten-free diet for more than six months and 49 patients with autism spectrum disorders who did not have dietary restrictions. There are no statistically significant age differences between the groups. The patients were evaluated and compared with the level of intestinal protein binding fatty acids.

Results. Children using diet therapy were found to have significantly lower intestinal permeability. In particular, the average level of I-FABP in children with autism spectrum disorders followed a gluten-free diet is 156.20 ± 102.16 pg/ml, with non-compliance with diet therapy. The average value of the index is diagnosed at the level of 528.26 ± 255.95 pg/ml because the protein binding fatty acids are a marker of small intestine permeability. Children with autism spectrum disorders without adherence to a gluten-free diet show intestinal permeability higher than that of their peers with autism, who long exclude gluten from the diet.

Conclusions. Further research is needed to assess the significance of the intestinal fatty acid-binding protein in the etiology and clinical presentation of various diseases, particularly in autism spectrum disorders. Before using diet therapy, all patients with autism need to consult a gastroenterologist to objectively assess the need for its compliance and dynamic monitoring of its effectiveness in case of use.

Acknowledgment. The author of the article is a scholarship of the President of the Russian Federation for 2019–2021 for young scientists and postgraduates engaged in promising research and development in priority areas of modernization of the Russian economy.

Conflict of interest. The author declares no conflict of interest.

Informed consent. Informed consent was received from the patients’ parents.

Received: March 19, 2021
Accepted: April 22, 2021
Published: May 14, 2021

The aim of the study was to determine changes in the content of matrix metalloproteinases (MMP) and their tissue inhibitor in children with uncomplicated compression fractures of the spine (UCFS).

Materials and methods. Eighty-five children, including 69 patients with UCFS (average age 12.3 ± 2.6 years), were comprehensively examined. The reference group consisted of 16 children (average age 11.8 ± 2.7 years) without spinal pathology. During the diagnostic period for 1–3 days, changes in the MMP content and their tissue inhibitor (TIMP-1) in blood serum were determined by the enzyme immunoassay method in all children after trauma.

Results. It was found that in the acute period after spinal injury, the blood levels of gelatinases (MMP-2 and ММР-9), stromelysin (MMP-3), and collagenases (MMP-8) significantly increased compared to their levels in children of the reference group. At the same time, the levels of TIMP-1 and the ratio of MMP/TIMP-1 concentrations in the blood of patients with UCFS significantly decreased compared to the control, which indicates the predominance of the proteolytic effect of MMP. Analysis of changes in the content of MMP in the blood in UCFS boys and girls did not reveal significant differences in the levels of the studied MMP and TIMP-1, except for a significant increase in the concentrations of stromelysin (MMP-3) in the blood serum of boys compared with its level in girls and the control. With different severity of the course of UCFS in children, a significant increase in MMP concentrations associated with an increase in the severity of the injury was revealed, and a substantial decrease in the content of TIMP-1 in the blood of patients compared to its levels in children with 1–2 degrees of severity and control.

Conclusion. The established patterns indicate that the determination of the content of MMR and TIMP-1 in the blood in UCFS children allows monitoring the course of the reparative process after injury to the vertebral bodies in children.

Contribution:
Smirnov I.Е., Каraseva О.V., Кucherenko А.G. — concept and design of the study;
Каraseva О.V., Pоrоkhina Е.А., Sarukhanyan О.О. — collection of material;
Кucherenko А.G., Smirnov I.Е. — statistical processing;
Smirnov I.Е. — writing of the text;
Fisenko A.P., Mitish V.A. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Informed consent: written voluntary informed consent to participate in the study was obtained from the parents of the patients.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare that there is no conflict of interest.

Received: April 21, 2021
Accepted: April 22, 2021
Published: May 14, 2021

The aim of this work is to evaluate the experience of Ladd’s laparoscopic operations in malabsorption syndrome (SM) without midgut volvulus in children aged 1–6 months.

Materials and methods. The manifestations of pathology in all patients were clinical signs of recurrent duodenal obstruction. The diagnosis was confirmed by ultrasound Doppler, contrast study of the upper gastrointestinal tract, irrigography, and fibrogastroduodenoscopy. The age of children at the time of surgical treatment was from 36 ± 142 days; body weight was 4100 ± 6800 g.

Results. Laparoscopic Ladd’s operation was performed on 6 patients. Concomitant pathology in the form of gastroesophageal reflux was found in 1 case. The operation time was 76 ± 125 minutes. There were no conversions per open transaction. The average length of hospital stay is ten days.

Discussion. Our experience in performing Ladd’s laparoscopic operations in children of the first six months of life without midgut volvulus shows their high efficiency and safety. To determine the significance of these operations in pediatric surgeons’ practice, it is necessary to accumulate experience and further research.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The author declares no conflict of interest.

Received: March 26, 2021
Accepted: April 22, 2021
Published: May 14, 2021

The aim is to identify parents’ (legal representatives’) satisfaction with the quality of medical care provided to their disabled children to improve this type of service further.

Materials and methods. A sociological survey was conducted of 506 legal representatives of minors (aged from birth to 17 years) with the status of a disabled child. The study design is single-center, non-randomized, uncontrolled.

Results. An analysis of the living conditions of a disabled child in the family, the parents’ assessment of his health status, the problems arising in the registration of disability, in the provision of medical and rehabilitation assistance, and issues of medical and social support made it possible to determine the position of this part of the child population in current legal and medical and social conditions. The main problems were the collection of a large number of documents when registering a disability, a long wait for the day of examination, the remoteness of the medical and social examination bureau, the lack of specialist doctors, problems with obtaining subsidized drugs, the lack of consideration of the individual needs of the child when conducting unique rehabilitation programs, the need to apply at the same time to various organizations and departments, violation of rights in the provision of medical services to a disabled child.

Conclusion. The obtained information is very significant for further improving the provision of medical and social assistance to disabled children and children with disabilities. The main task today is to develop mechanisms for implementing the declared rights and freedoms of persons with disabilities, the obligations assumed by the State concerning them.

Contribution:
Fisenko A.P., Terletskaya R.N. — concept and design of the study;
Alexeeva E.I., Basargina E.N., Kazakova K.A., Murashkin N.N., Surkov A.N., Tsygin A.N., Chomahidze A.M., Opryatin L.A. — collection and processing of material;
Chernikov V.V. — statistical processing;
Terletskaya R.N., Vinyarskaya I.V. — writing of the text;
Antonova E.V. — 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: March 26, 2021
Accepted: April 22, 2021
Published: May 14, 2021

Dystonia is a motor disorder characterized by sustained muscle contractions producing twisting, repetitive, and patterned movements or abnormal postures. Dystonia is among the most commonly observed motor disorders in clinical practice in children. Unlike dystonia in adults that typically remains focal or spreads only to nearby muscle groups, childhood dystonia often generalizes. Classification of dystonia has direct implications for narrowing down the differential diagnosis, choosing the diagnostic work-up, predicting the prognosis, and choosing treatment options. The etiology of pediatric dystonia is quite heterogeneous. The etiological classification distinguishes primary dystonia with no identifiable exogenous cause or evidence of neurodegeneration and secondary syndromes. Dystonia can be secondary to any pathological process that affects the basal ganglia. The treatment options of childhood dystonia include several oral pharmaceutical agents, botulinum toxin injections, and deep brain stimulation therapy. Botulinum toxin treatment is the first choice treatment for most types of focal dystonia. In children it is less used because dystonic forms are mainly generalized, but it might also be helpful in controlling the most disabling symptoms of segmental or generalized dystonia. Long-term electrical stimulation of the globus pallidum internum is now established as an effective treatment for various types of movement disorders including dystonia. However, this method has not yet found its application in Russia due to the difficulty of implementation and the lack of patient routing. To increase the effectiveness of complex therapy of dystonia in children, new pathogenetic methods of treatment of common forms of primary dystonia and dystonic syndromes in the structure of degenerative diseases of the central nervous system are needed, as well as the development of optimal algorithms for the diagnosis and treatment of these patients.

Contribution:
Pak L.A., Lyalina A.A. — study concept and design, text writing;
Lyalina A.A., Kondakova O.B. — collection and treatment of materials, statistical processing;
Fisenko A.P., Smirnov I.E. — text editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Informed consent. Informed consent was received from the patients’ parents.

Acknowledgment. The study had no sponsorship.

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

Received: April 20, 2021
Accepted: April 22, 2021
Published: May 14, 2021

Introduction. The term «chronic kidney disease» (CKD) is used to define the outcomes of various forms of chronic progressive kidney disease, characterized by kidney damage, or a decrease in their function for three months or more, regardless of the nosological diagnosis.

The aim of the work was to determine the frequency, structure, and clinical and paraclinical features of CKD in children to substantiate the principles of its management in primary health care.

Results. In CKD patients, tubulointerstitial renal lesions were established to prevail (80%). Associated with congenital malformations of the urinary system, reflux uropathy, and urolithiasis in 98% of cases tubulointerstitial renal lesions are complicated by renal infection. In 8.9% of children, CKD was formed as an outcome of glomerulopathies; in 6.7% of children — with congenital and hereditary nephropathies; in 4.4% of children an outcome of a hemolytic uremic syndrome. The observed children dominated by I–II (74.3%), less often III and IV (25.7%) stages of CKD. The revealed clinical and paraclinical features of CKD in children are presented.

The modern principles of early diagnosis of CKD in children and the identification of risk factors for the progression of nephropathy have been substantiated. For the diagnosis of CKD in childhood there has been created a. n algorithm, including at the outpatient stage. Authors suggested a set of measures for the prevention of nephropathy.

Conclusion. For early diagnosis of CKD in childhood there was developed an algorithm based on the analysis of a complex of clinical and paraclinical, structural and functional parameters of the kidneys in combination with the parameters of endothelial function and cytokine status.

Contribution:
Vyalkova A.A. — research concept and design of the study, editing;
Chesnokova S.A., Gaikova L.A. — collection and processing of material;
Chesnokova S.A., Ustinova O.O. — text writing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Informed consent. Written voluntary informed consent was obtained from the patients’ parents to participate in the study.

Acknowledgement. The study was carried out within the framework of the grant support of the Orenburg State Medical University. University Research Grant «Predictors and personalized prevention of chronic kidney disease in children» (Agreement No. 2019 RCD/2019/04-02-2-Gr/M/1 of 14.01.2019).

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

Received: March 29, 2021
Accepted: April 22, 2021
Published: May 14, 2021

Introduction. Idiopathic hypercalciuria (IH) is one of the most common metabolic disorders in children and is one of the leading causes of calcium urolithiasis and osteoporosis. The strategic goal of treatment for IH is to reduce urinary calcium excretion.

Materials and methods. The study included 93 IH children aged from 3 to 14 years with identified IH. At the first stage, IH children have been prescribed low sodium and high potassium diet with increased fluid intake for three months without additional drug therapy. For children with persistent IH at the second stage, the diet was supplemented daily with 1000–1500 mg of fish oil for children for 3 months. At the third stage of treatment, persistent IH patients were prescribed hydrochlorothiazide at a dose of 1 mg/kg for 3 months. After each stage of treatment, we analyzed IH’s manifestations in dynamics and monitored urinary calcium excretion by calcium/creatinine ratio (CCR).

Results. Evaluation of clinical and laboratory manifestations of IH after the first stage of treatment showed the effectiveness of the diet in 59.1% of patients. Among 38 patients included in the second stage of therapy, the normalization of urinary calcium excretion was noted in 52.6% of cases. The third stage of therapy was performed in 18 patients (19% of the initial group of IH children patients). The normalization of CCR was achieved in 16 (88.9%) children.

Conclusion. For the correction of IH in children, a step-by-step approach should be recommended, starting with recommendations on the drinking regimen and nutrition, then prescribing fish oil preparations and resorting to thiazide diuretics in the lack of an effect from the first stages of treatment.

Contribution.
Mikheeva N.M., Zverev Ya.F., Strozenko L.A. — research concept and design of the study;
Mikheeva N.M., Zverev Ya.F. — collection and processing of material;
Strozenko L.A. — statistical processing; 
Mikheeva N.M., Zverev Ya.F. — text writing;
Zverev Ya.F., Strozenko L.A., Lobanov Yu.F. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

The study protocol was approved by the local ethics committee. The patients’ parents gave written voluntary informed consent to participate in the study.

Acknowledgment. The study had no sponsorship.

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

Received: March 29, 2021
Accepted: April 22, 2021
Published: May 14, 2021

The purpose of the work is comprehensive examination of a newborn with cholestasis syndrome to determine congenital hypopituitarism.

Materials and methods. The child was hospitalized on the 30^th day of his life. At admission, complaints were noted about the ictericity of the skin, low body weight gain, convulsive syndrome (history). Differential diagnosis was carried out between diseases such as: impaired liver function, against the background of the course of the infectious-inflammatory process; diseases of the liver and bile ducts of hereditary nature; congenital malformations of the bile tract; metabolic and hormonal disorders.

Results. During the survey, the following deviations from the norm were obtained: in the biochemical analysis there was an increase in the level of transaminases, alkaline phosphatase, total and direct bilirubin, hypoglycemia. When evaluating the hormonal profile, an increase in the level of prolactin, thyroid hormone, a decrease in the level of T4 free, insulin, and a complete absence of cortisol were revealed. Magnetic resonance imaging (MRI) of the brain — a picture of subependymal nodes of gray matter heterotopia, ectopia of the neurophysis.

Conclusions. Lowering glucose levels, especially when combined with cholestasis syndrome, may be an early but nonspecific sign of congenital hypopituitarism. Hypoglycemia in newborns occurs as a result of intrauterine insufficiency of somatotropic hormone and cortisol related to contrinsular hormones. Cortisol deficiency also contributes to the development of cholestasis syndrome by reducing the expression of tubule transport proteins that regulate bile secretion into bile tubules. In addition to studying the hormonal profile, a brain MRI is performed to verify the diagnosis. Patients with congenital hypopituitarism are characterized by the detection of characteristic signs: the picture of the “empty” or “partially empty” Turkish saddle, as well as the classic triad of symptoms: hypoplasia/pituitary leg aplasia, neurophysis ectopia, adenohypophysis hypoplasia.

This clinical case demonstrates that cholestasis syndrome may lie in the debut of a more severe pathology. At the same time, early diagnosis and adequately selected hormone replacement therapy leads to a rapid cessation of life-threatening conditions and an improvement in the quality of life of the child.

Contribution:
Kharitonova N.A. — concept and design of the study, statistical processing;
Kharitonova N.A., Evloeva Kh.S. — collection and processing of material, writing the text;
Kharitonova N.A., Basargina M.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: April 06, 2021
Accepted: April 22, 2021
Published: May 14, 2021