No 4 (2022)

Articles

Algorithm of local conservative treatment of children with burns

Budkevich L.I., Soshkina V.V.

Abstract

Background. This article presents the results of using atraumatic wound dressings Branolind N and sorbing bandage Zetuvit. In research there were 10 children with superficial scalds (I–II degree) and parcial deep (II–III degree) burns. Children were treated at the Burn Center of Speransky Pediatric City Hospital №9 in Moscow in June – November 2021. The analysis of the conducted research testifies to the effectiveness and safety of the medical products listed above. The absence of local complications during treatment was revealed. The possibility of their simultaneous use in patients with exudating wounds has been established. Branolind N and Zetuvit bandage can be recommended for use both in a on- and outpatient cases in children with local burns. The results of treatment of children with burn injury depend on adequate diagnosis of the severity of the burn trauma, reliable determination of the depth of scald and local treatment of burn wounds. Tactics of local treatment depends on the phase of wound process, the age of the child and the presence of concomitant diseases.

Aim. To assess the effectiveness and safety of using wound dressings – ointment dressing Branolind N with Peruvian balsam and Zetuvit sorbent dressing in treatment children with burns.

Materials and methods. Analysis of the using of Branolind N ointment dressing with Peruvian balsam and Zetuvit sorbent dressing in 10 children with burn injury was carried out. The study was completed in June – November 2021. Inclusion and exclusion criteria were determined by the physicians participating in the study. The studies were carried out in accordance with Helsinki Declaration. The age of the patients ranged from 3 years 2 months up to 17 years 1 month. The depth of burn wounds in the studied patients was assessed according to the classification of the damage degree of European Burn Association: patients with superficial scalds (I–II degree) and partial deep burns (II–III degree).

Results. The absence of local skin complications during the application of the Branolind N ointment dressing with Peruvian balsam and Zetuvit sorbent dressing was revealed. The possibility of using. These wound dressings in patients with exuding wounds was established.

Conclusion. The analysis of the conducted study testifies to the effectiveness and safety of the above medical products. Ointment bandage Branolind N with Peruvian balsam and sorbent bandage Zetuvit can be recommended for use both in out- and inpatients practice of treatment children with local skin burns.

Pediatrics. Consilium Medicum. 2022;(4):268-276
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Motherhood and infant feeding: Historical aspects and current practice: A review

Zakharova I.N., Sugian N.G., Kosareva A.A., German N.V., Orobinskaia I.V., Pupykina V.V.

Abstract

In recent years there has been a deterioration in demographic indicators: a decrease in the birth rate, an increase in adult mortality, etc., due to numerous causes. In this context, the article addresses demographic issues related to subcultures and ideologies, such as childfree. The main characteristics of people classified as rejecters, aficionados, refusers, and postponers are discussed. We present the first results of a study conducted at the Khimki regional hospital. The study included 25 pregnant women. The survey results showed that most were planning a pregnancy before 30. A survey of expectant mothers indicated a positive attitude towards breastfeeding. The article discusses the issues of breastfeeding as an essential stage in forming a child's immunity and the factors of breastfeeding success. Women's nutrition during pregnancy and breastfeeding should be diverse and rich in vitamins and micronutrients. Special maternal drinks, such as Amalthea, based on New Zealand goat milk, can benefit breastfeeding. In the absence of breast milk, the selection of formula for the baby is one of the most challenging issues. It is important that the infant formula meet the child's nutritional needs to the maximum extent possible, including the functional milk components. For instance, the Nanny formula is based on New Zealand goat milk, using a single heat treatment process without separating milk into fractions, which preserves the native functional components of goat milk (oligosaccharides, nucleotides, milk fat).

Pediatrics. Consilium Medicum. 2022;(4):278-285
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Results evaluation of the implementation of the “Program for Optimizing Feeding of Children in the First Year of Life in the Russian Federation” dated 2009

Skvortsova V.A., Borovik T.E., Fisenko A.P., Timofeeva A.G., Bushueva T.V., Lukoyanova O.L., Akoeva D.Y., Chernikov V.V., Sokolov I., Belousova T.V., Dzhumagaziev A.A., Lebedeva J.M., Mironenko I.I., Senkevich O.A., Furtsev V.I.

Abstract

Background. Appropriate prescribing of complementary food allows to optimize the intake of macro- and micronutrients, provides adequate indicators of growth and development of the child, reduces the risk of non-infectious pathology. Taking into account the significance of this problem, World Health Organization (WHO) experts, together with The European Society for Paediatric Gastroenterology Hepatology and Nutrition, carried out a research where they studied issues related to the appointment of complementary food for children in the European Region and their compliance with existing recommendations.

Aim. To study the features of the introduction of complementary food in the Russian Federation on the basis of an assessment of the implementation of the recommendations of the "National program for optimizing the feeding of children in the first year of life in the Russian Federation", 2009 and to conduct a comparative analysis of the results obtained and the data presented in the study carried out by WHO experts.

Materials and methods. A multicenter retrospective, uncontrolled, non-randomized study was conducted in 7 cities of the Russian Federation.

Results. The median age at which complementary foods were introduced was 5 [4–6] months; vegetables (51.0%) and cereals (31.2%) were the first complementary food products; children began to receive fruit mainly (77.3%) in the second half of life; the age of introduction of meat puree was 7 [6–8] months, while every 4th child by 9 months did not receive meat; juices were introduced at 7 [6–9] months, but in 8.8% of children juices became the first product of complementary foods; median age for yolk administration was 8 [7–10] months, fish – 9 [8–10] months. Only 14.0% of mothers used products of exclusively industrial production in the nutrition of their children, 33.9% prepared it themselves. Correlation analysis did not revealed any relationship between the body weight of a child at the age of 12 months and the time of introduction of the first complementary food product, as well as with the age of introduction of each of the introduced products. A connection was established between the body weight of a child at the age of 12 months and the volume of individual products that children receive between the age of 6 and 12 months. The recommendations of the “National program for optimizing the feeding of children in the first year of life in the Russian Federation” were generally implemented. Our analyses revealed the most frequent violations during the introduction and use of complementary food products: late start of their introduction (after the age of 6 months) in 41.3% of children, delayed prescription of meat, frequent use of home-made cereals not enriched with micronutrients, dilution of dairy-free porridge with water, irregular inclusion in the diet of egg yolk.

Conclusion. The results obtained confirm the importance of implementation of the “Program for Optimizing Feeding of Children in the First Year of Life in the Russian Federation” updated in 2019 and approved by the Ministry of Health of Russia and indicate the need for further work on its improvement.

Pediatrics. Consilium Medicum. 2022;(4):286-294
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Does maternal postpartum depression affect child health and development? A review

Zakharova I.N., Berezhnaya I.V., Sugian N.G., Orobinskaia I.V., Karimova A.E.

Abstract

Postpartum depression (PD) is a worldwide issue. Researchers believe the high risk of depression after childbirth persists for the first 6 months. The rate of PD varies from 13 to 20%. This psychiatric disorder has been shown to have a negative impact on the quality of life not only for mothers (difficulty breastfeeding, changing relationships with their husbands, including intimacy) but also for children, initiating immediate effects (infant colic, lack of weight gain, sleep disturbances) and long-term effects on the cognitive and emotional development of the child. Due to a woman's busy schedule, she does not always have the opportunity to visit a doctor. In addition, she may not be aware of her mental health disorders. Often the pediatrician observes that the woman, due to inappropriate behavior, not only fails to cope with maternal responsibilities but may also be a danger to her child. A practical and simple screening technique that allows early detection of PD is the Edinburgh PD Scale. Its use will help women to receive professional care.

Pediatrics. Consilium Medicum. 2022;(4):295-300
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Iron deficiency in children: modern aspects of the problem, possibilities of primary prevention: A review

Samorodnova E.A.

Abstract

Iron deficiency (ID) is common in the pediatric population, primarily due to inadequate iron intake from food and a high requirement due to rapid growth. The most significant for pediatric practice are latent iron deficiency and iron deficiency anemia. Hyposiderosis significantly impacts a child's physical and cognitive development and immunological reactivity. It can be an independent condition and complicate the course of several other diseases. The article presents data on the most significant factors contributing to the ID, risk groups, clinical presentation features (sideropenic and anemic syndromes), criteria of laboratory diagnostics of iron deficiency anemia according to the clinical guidelines "Iron deficiency anemia" approved by the Russian Ministry of Health in 2021, and the latent iron deficiency developed by the World Health Organization experts. Also, the algorithm of ID primary prevention, diet therapy approaches, and the use of functional products and dietary supplements to meet the iron requirement of a child's organism are discussed.

Pediatrics. Consilium Medicum. 2022;(4):302-308
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The role of vitamin D in the prevention and treatment of acute respiratory infections in children: A review

Lokshina E.E., Zaytseva O.V.

Abstract

The highest frequency of acute respiratory infections occurs in children aged 3–7 years (preschoolers), when the child begins to meet with an increased infectious load in children's organized groups. Vitamin D deficiency or insufficiency is often detected in children with recurrent respiratory infections. At the same time, vitamin D is a complex regulator of innate and adaptive immunity, has an immunomodulatory and anti-inflammatory effect, and protects from a severe infection. The results of international and Russian studies demonstrate the feasibility of using vitamin D for the prevention and treatment of a number of respiratory infections in children and adults.

Pediatrics. Consilium Medicum. 2022;(4):310-316
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Resolution of the Expert Council on the use of the anti-inflammatory drug ammonium glycyrrhizinate in the treatment of acute respiratory infections and allergic diseases of the respiratory tract

Geppe N.A., Malakhov A.B., Boitsova E.V., Gaimolenko I.N., Ermakova I.N., Zaitsev A.A., Ivakhnenko E.F., Il'enkova N.A., Kamaev A.V., Kliukhina I.B., Kondiurina E.G., Konova O.M., Kulagina V.V., Lev N.S., Liutina E.I., Megirian M.M., Mel'nikova I.M., Meshcheriakov V.V., Mizernitskii I.L., Mironova A.K., Mikhalev E.V., Mozzhukhina L.I., Odinaeva N.D., Pavlinova E.B., Pobedinskaia N.S., Skachkova M.A., Soroka N.D., Trishina S.V., Tsar'kova S.A., Shuliak I.P.

Abstract

In June 2022, within the XI International Educational Consensus on Respiratory Medicine in Pediatrics, an expert council on the anti-inflammatory agent ammonium glycyrrhizinate in treating acute respiratory infections and allergic diseases of the respiratory tract was held in Vladimir. The resolution of the Expert Council is presented. Based on these studies, experts recommend the use of ammonium glycyrrhizinate in patients with acute respiratory tract infection (including COVID-19); in the rehabilitation period of patients who recovered from the new coronavirus infection with lung involvement; patients with recurrent obstructive bronchitis, including those at high risk of bronchial asthma; patients with allergic rhinitis; patients with mild to moderate asthma.

Pediatrics. Consilium Medicum. 2022;(4):317-321
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One of the possibilities of optimizing the therapy of a new coronavirus infection in children with the inclusion of an extract from marshmallow root, chamomile flowers, horsetail grass, walnut leaves, yarrow grass, oak bark and dandelion grass: prospective open comparative cohort study

Vavilova V.P., Vavilov A.M., Tsarkova S.A., Nesterova O.L., Kulyabina A.A., Yakhno N.B., Anisimova A.V., Vavilov V.A., Elkina E.N., Dobryak T.A.

Abstract

Background. The issue of acute respiratory infections in children remains one of the most urgent. Therefore, in the pandemic due to the new coronavirus SARS-CoV-2, the search for effective treatment regimens for this disease is necessary.

Aim. To compare the change of clinical symptoms, terms of virus elimination and disease-related economic cost as well as utilization of outpatient healthcare services in children with new coronavirus infection using the herbal medicine Tonsilgon® N, oral solution, added to standard therapy.

Materials and methods. A prospective open comparative cohort study of the effectiveness of coronavirus infection treatment in children using the standard of care alone and the standard of care with Tonsilgon® N was conducted. Group 1 children (n=720) received the standard therapy for COVID-19 and Tonsilgon® N oral solution for 10 days, and group 2 patients (n=670) only received the standard therapy for COVID-19. Treatment efficacy was evaluated according to the change of clinical symptoms in the observed patients, the results of SARS-CoV-2 coronavirus RNA detection in nasopharyngeal smears by polymerase chain reaction, the utilization of healthcare services, temporary disability of parents, the economic cost of the disease.

Results. Studied patients of both groups had a typical clinical presentation of mild to moderate severity new coronavirus infection. Fatigue (96.5–99.7%) and sore throat (93.8–93.9%) were the most frequent symptoms. Posterior pharynx hyperemia was noted slightly less frequently. Low-grade fever was noted in 85.7–86.6% of the patients, equally often in patients of both groups. The significant therapeutic effect of the herbal medicine containing extract of marshmallow root, chamomile flowers, horsetail herb, walnut leaf, yarrow herb, oak bark, and dandelion herb includes reducing the time of sore throat relief in 93.2% of main group patients. After 10 days of treatment, sore throat persisted only in 6.8% of group 1 and 68.1% of group 2 patients (p=0.0001). These results indicate that Tonsilgon combined with standard of care is a promising way to improve the quality of life of children with the new coronavirus infection. The addition of Tonsilgon in therapy for new coronavirus infection in children shortens the time of acute inflammation resolution and reduces the duration of inflammatory intoxication. The therapy of new coronavirus infection under the provisional guidelines leads to a reduction in the time for clinical recovery and virus elimination in children and adolescents (p=0.0001) and a decrease in the utilization of outpatient healthcare services are observed: 3/4 of Group 1 patients and 1/2 of Group 2 patients (p=0.0001) applied for medical help twice during the observation period, 14.6% of Group 1 patients and 29.1% of Group 2 parents applied for medical help more than 3 times (p=0.0001). Thus, the direct and indirect economic costs of the disease decreased. The lower economic cost for parents of children in Group 1 was due to less need for medication and a lower reduction in family income due to temporary disability compared to those for parents in Group 2 (p=0.0001). No treatment-related adverse events was reported.

Conclusion. The above data indicate that Tonsilgon therapy is an affordable way to optimize the treatment of new coronavirus infection in children.

Pediatrics. Consilium Medicum. 2022;(4):322-330
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Agreed Expert Opinion of the XI Educational International Consensus on Respiratory Medicine in Pediatrics on the organization of pertussis vaccination

Geppe N.A., Malakhov A.B.

Abstract

Pertussis remains a relevant public health issue in the Russian Federation. The recorded high incidence rate and trends indicate wide circulation of the pertussis pathogen. There has been increased pertussis incidence in children under 14 years of age due to the lifting of restrictive measures. From January through May 2022, there is a 100% increase in the pertussis incidence compared to the same period in 2021. The optimal strategy for reducing morbidity and mortality is vaccination during the first year of life and the first booster vaccination during the second year, ensuring maximum coverage of preventive vaccinations for young children, as well as the introduction of the second revaccination of children of 6–7 years old and subsequent revaccinations of children 14 years old, adolescents and adults from 18 years old – every ten years from the last vaccination, using a combined vaccine for revaccination against pertussis (cell-free), diphtheria (with a reduced antigen content) and tetanus. During the implementation of the regional programs at stage I, it is possible to introduce additional revaccinations for children, adolescents, and adults at risk who need revaccination against pertussis, primarily with TDaP for children from 3 months to 3 years 11 months 29 days; acellular TDaP (Infanrix) for children from 2 months to 7 years of age; acellular TDaP-IPV-HBV/Hib (Infanrix Hexa) in children from 2 months to 2 years of age; acellular TDaP-IPV/Hib (Pentaxim) for children from 2 months of age; acellular TDaP (Adacel) for children 4 years of age and older following their labels.

Pediatrics. Consilium Medicum. 2022;(4):331-334
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Recurrent growth of pharyngeal lymphoid tissues in children: a literature review

Kulmakova M.A., Radtsig E.I., Polunin M.M.

Abstract

Hypertrophy of the nasopharyngeal and/or palatine tonsils is a common condition in children that reduces patients' quality of life. Surgical treatment is widely used in the Russian Federation and worldwide, but some patients experience a relapse of the disease. From 0.55% to 25.79% of children with a history of surgery undergo repeat adenotomy; in 0.12–11.9% of patients, repeat tonsillotomy/tonsillectomy is performed. The reasons for the re-growth of lymphoid tissue in the pharynx in some patients remain unclear. It is difficult to estimate the recurrence rate due to the lack of definite clinical and diagnostic criteria for the concept of "recurrence" itself. Factors associated with a higher frequency of re-growth of lymphoid structures in the pharynx include some conditions (allergy, bronchial asthma, gastroesophageal reflux), the history aspects (tonsillitis, multiple courses of antibiotic therapy), and early age at the time of primary operation and adenotomy without visual monitoring. The issues discussed are relevant to E.N.T. specialists and pediatricians in predicting and reducing the risk of recurrence in children before primary surgery.

Pediatrics. Consilium Medicum. 2022;(4):336-339
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Hemangioma of temporal bone. Case report

Saidulaev V.A., Diab K.M., Daikhes N.A., Garashchenko T.I., Iunusov A.S., Pashchinina O.A., Umarov P.U.

Abstract

Hemangioma of temporal bone or intratemporal hemangioma is a rare benign tumor that has no specific symptoms and signs according to computed tomography, magnetic resonance imaging), which makes it difficult to diagnose it early. The percentage of diagnostic errors is quite high. The symptoms of hemangioma of temporal bone depends on its localization. A common localization of hemangioma in the temporal bone is geniculate ganglion and internal auditory canal. According to CT and MRI data, there is no difference between many tumors usually localized in the temporal bone. Angiography complements the preoperative examination, increases the likelihood of a correct diagnosis, and is used to assess the possibility of preoperative embolization. Surgical treatment used in many cases, the purpose of which is the total removal of the tumor. A clinical case of temporal bone hemangioma in a 12-year-old child is presented.

Pediatrics. Consilium Medicum. 2022;(4):340-343
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Features of microcirculation changes in children with a burdened allergic history. Case Report

Berezhanskiy P.B., Gutyrchik T.A., Malakhov A.B., Kovalenko I.V., Gutyrchik N.A., Dustbabaeva N.D.

Abstract

Microcirculatory changes play an important role in the pathogenesis of allergic diseases. The definition of microcirculation includes the movement of fluid between the circulatory and lymphatic networks through intracellular systems, the transmembrane exchange of substrates, gases, including metabolic products and signal molecules. Due to the complexity of the pathogenesis of microcirculatory changes, it is necessary to use sensitive methods for diagnosing the degree of capillary blood flow disorder and associated changes in the microvessels of the arteriolar and venular parts of the microvasculature. Today, there is an available non-invasive method for assessing microcirculation using capillaroscopy of the nail-fold, which allows assessing the structure of microvessels in real time in vivo. Evaluation of capillaries by nail-fold capillaroscopy is the safest method for children and does not cause them concern during the study. With the help of capillaroscopy of the nail-fold, it is possible to assess the quantitative parameters of microcirculation, the width and length of the capillaries, the diameter of the arterial and venous sections, the outer and inner diameters, the width of the apex and the distance between the capillaries. The speed of blood flow in the arterial and venous sections is also taken into account. It is important to note that capillaroscopy also assesses the presence of avascular zones, the appearance of which indicates tissue hypoxia, and therefore can be considered as an important prognostic factor. Capillaroscopy is of great diagnostic value, as it allows developing tactics for treating patients, and, most importantly, assessing the effectiveness of the therapy and predicting the outcome of the disease. The presented clinical observation convincingly demonstrates the need to assess microcirculation during nail-fold capillaroscopy in children with aggravated allergic anamnesis.

Pediatrics. Consilium Medicum. 2022;(4):344-348
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Norwegian scabies in children. Case report

Belikov A.N., Golubeva P.A., Petrenko E.V., Sheremeteva N.V., Iuditskaia M.V., Korsunskaya I.M.

Abstract

Norwegian scabies is a highly contagious form of scabies that is quite common in immunosuppressed patients. Given the atypical clinical presentation, this form of the disease may remain undiagnosed for a long time. Norwegian scabies is often reported in elderly patients but can also occur in children. This article presents a clinical case of a 6-year-old patient to draw the attention of specialists to this disease in pediatric practice.

Pediatrics. Consilium Medicum. 2022;(4):349-351
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Current concepts of the intestinal microbiota and prospects for microbiocenosis adjustment: A review

Khavkin A.I., Vashura A.I., Kovtun T.A., Tabulovich E.V.

Abstract

The review presents current data on the interaction between the human body and the community of microorganisms inhabiting its gastrointestinal tract. The terminology and methods for analyzing quantitative and qualitative characteristics of gut microbiota are presented. The review also focuses on the concepts of eubiosis, dysbiosis, and the possibilities of correcting dysbiosis with probiotics and probiotic foods.

Pediatrics. Consilium Medicum. 2022;(4):352-357
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Familial chylomicronemia syndrome in children and adolescents: diagnosis and treatment

Pshenichnikova I.I., Zakharova I.N., Osmanov I.M., Trunina I.I., Pupykina V.V., Arsel'gova I.K., Koba Y.V., Bocharova T.I., Abazova A.R.

Abstract

Familial chylomicronemia syndrome is a rare inherited disease. Recessive mutations in genes encoding lipoprotein lipase or modulator proteins result in loss of enzyme function. As a result, the removal of triglyceride-rich lipoproteins from plasma is impaired, severe hypertriglyceridemia develops, and the risk of acute pancreatitis sharply increases. The mainstay of treatment for patients with familial chylomicronemia syndrome is a specialized, very low-fat diet.

Pediatrics. Consilium Medicum. 2022;(4):358-360
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Celiac disease: history and modern era

Dmitrieva Y.A., Zakharova I.N., Osmanov I.M., Maikova I.D., Gostyukhina A.D., Radchenko E.R., Dubovets N.F., Abdurakhmanova L.S.

Abstract

Celiac disease is one of the oldest human diseases, dating back to the Neolithic Era with the advent of the farming culture. In over 10,000 years, celiac disease has evolved from an unknown and frightening "sickness of the coeliac" to one of the most common genetically determined conditions with a thoroughly understood pathogenesis and effective treatment. Current epidemiological data suggest that the global prevalence of celiac disease averages 1%, with most cases unrecognized. Given the diversity of clinical manifestations, a selective screening program for celiac disease among patients with some suspicious symptoms proved its value only partially. Most experts point to the need for universal screening to effectively detect asymptomatic forms of celiac disease and early initiation of nutritional therapy to avoid serious complications.

Pediatrics. Consilium Medicum. 2022;(4):361-365
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Three-stage surgical treatment for colon lesions in children with Crohn's disease. Retrospective-prospective study

Bekin A.S., Dyakonova E.Y., Gusev A.A., Potapov A.S., Lokhmatov M.M., Kulikov K.A., Yatsyk S.P.

Abstract

Background. Crohn's disease (CD) in children is characterized by a more severe and progressive course, with the development of a form that is not susceptible to drug therapy, and the development of surgical complications. Up to 50% of cases, inflammation is localized in the ileocecal region. Isolated inflammation of the colon is diagnosed in the smallest number of patients with Crohn's disease, in adults from 18.1 to 28.3%, in pediatric patients from 5.1 to 24.4%.

Aim. To substantiate the effectiveness of three-stage surgical treatment in children with colon lesions in CD.

Materials and methods. From 2018 to 2022, 75 children with complicated Crohn's disease underwent surgical interventions in the National Medical Research Center for Children's Health. A single-stage retrospective-prospective study of 7 (9.3%) children with isolated colon lesion was conducted. Four (57%) children – stenosis in the left colon; 3 (43%) children – continuously recurrent inflammation of the colon, not amenable to drug therapy. The effectiveness of staged surgical treatment was evaluated after 6, 12, 18, 24 months based on statistical analysis of clinical, endoscopic and laboratory activity of CD, changes in anthropometric indicators.

Results. In 4 children, the onset of remission was noted, and in 3 children, clinical activity was mild (p<0.05). The onset of endoscopic (p<0.05) and laboratory remission (p<0.05) was noted in all children. The assessment of the growth-to-age ratio also showed statistically significant positive dynamics, however, when assessing the ratio of body mass index to age, no statistically significant changes were detected (p=0.066).

Conclusion. Isolated colon lesion is the rarest phenotype in children with CD. The formation of colon stricture requires surgical treatment, but predicting the volume of surgical treatment at the preoperative stage is a difficult task. The chosen tactics of conducting surgical treatment in three stages, with the correction of drug therapy after disconnecting the colon from the digestive tract, allowed not only to avoid postoperative complications associated with the formation of an anastomosis on the affected intestinal wall, but also to achieve remission of the disease.

Pediatrics. Consilium Medicum. 2022;(4):366-372
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Comparison of the use of intramedullary telescopic system and titanium elastic rods in children with osteogenesis imperfecta I and III types

Solodovnikova K.N., Zherdev K.V., Сhelpachenko O.B., Soloshenko M.A., Yatsyk S.P., Ovechkina A.A., Butenko A.S.

Abstract

Osteogenesis imperfecta (OI) is a rare disease characterized by frequent fractures and deformities of the bone skeleton due to collagen abnormalities. Clinically, OI is heterogeneous in its features and varies in severity. Frequent fractures as a result of brittle bones lead to malunion and deformity, which increases the risk of refraction. Surgical treatment of children with imperfect osteogenesis is aimed at reducing the number of fractures, the formation of deformities and improving the quality of life of the child. According to the literature, two main methods of intramedullary osteosynthesis in the surgical treatment of children with OI can be distinguished: static rods and a “growing” metal structure.

Aim. To compare the frequency of revisions and complications when using titanium elastic rods (TEN) and the Fassier-Duval telescopic system (FD) in the treatment of fractures and deformities of long bones in children with OI type I (mild disease) and type III (severe disease).

Materials and methods. A retrospective and prospective analysis of the results of surgical treatment in 38 children with OI using two different methods of intramedullary osteosynthesis was carried out. Among them, 26 children (68% of all studied patients) were of type III and 12 (32%) children of type I. The mean age of the patients was 7.8 years [5.2, 10.8]. The 1st study group (retrospective) included 17 patients (45% of all patients) who underwent surgical treatment with the installation of titanium elastic rods (TEN). A total of 46 surgical interventions were performed. Surgical treatment with the installation of titanium elastic rods (TEN) for patients was carried out in other medical institutions before hospitalization at the National Medical Research Center for Children's Health. The 2nd study group (prospective) included 21 patients (55% of the total number of patients in the study), who were implanted with FD telescopic rods, 53 surgical interventions were performed. Patients of the prospective group received surgical treatment on the basis of the neuroorthopedic department of the National Research Center for Children's Health. The study groups were structured as follows: Study Group 1 consisted of 2 subgroups. Subgroup A included 6 patients with OI type I, subgroup B included 11 patients with OI type III; The 2nd study group was represented by two subgroups. Subgroup C included 6 patients with OI type I, and subgroup D included 15 patients with OI type III. A total of 228 segments of the upper and lower extremities (humerus, femur and tibia) were examined. Surgical treatment using intramedullary osteosynthesis was performed on 99 segments. The average period of postoperative follow-up was 20 months (from 16.5 to 24.5 months), the data collection of the retrospective group was carried out in the period from 2015–2022, the prospective group from 2017–2022. Analysis of the results of surgical treatment according to the following criteria: the frequency of migration of metal structures, the formation of bone deformities, the number of bone fractures with an installed metal fixator in two different methods of osteosynthesis, as well as the number of revisions in OI types I and III. The results of motor activity in the retrospective and prospective groups were assessed using two scales (the Hoffer–Bullock scale, the Gillette Functional Assessment Questionnaire score scale), before the start of surgical treatment and after 20 months. The statistical analysis was performed using the Matplotlib, SciPy, Pandas и NumPy modules in Python 3.8. In all cases, the distribution was different from normal. The comparison of the independent groups was carried out using the Mann–Whitney test (in the case of comparing two samples), while the comparison of the dependent groups was carried out using the Wilcoxon test. To compare the distribution of the categorical features, the Pearson chi-square test and Fisher's exact test (with the number of observations in one of the cells in the table 2×25) for the independent groups, and McNemar’s test for the dependent groups were used. In all cases, when the multiple comparisons were made, the level of the significance of p was recalculated using the Bonferroni correction. Hypothesis testing was two-sided; the values of p<0.05 were considered statistically significant.

Results. According to the comparison of two subgroups of the 1st study group, it was noted that fractures in two subgroups after the installation of static intramedullary nails occurred with an equal frequency (p-value>0.999). Among patients with OI type I, migration was observed in 13% of cases (2 segments), with OI type III in 48%. Deformity in type I OI was formed in 13% of cases, in type III in 39%. Revisions in children with type I OI were required in 13% of cases, in children with type III in 32%. According to the comparison of two subgroups of the 2nd study group, it was noted that fractures and migrations in two subgroups after the installation of a telescopic metal structure occurred with equal frequency (p-value>0.999). Deformity in type I OI was not formed, in type III it was 18%. Revisions in children with type I OI were required in 7.7% of cases, in children with type III in 15%. A comparative analysis of the results of surgical treatment of children with type I OI who underwent TEN osteosynthesis and children with type III OI who used a telescopic metal fixator demonstrates the absence of statistically significant differences in deformities, migrations, and revisions (p-value>0.999). Also, in the group of children with OI type III, osteosynthesis of which was performed by FD, there is a decrease in the risk of re-fracture by 10%, in comparison with the group of children with OI type I, osteosynthesis of which was performed by TEN. Also, according to the data of statistical processing, surgical treatment of children with OI type III using an intramedullary telescopic system makes it possible to achieve a level of motor activity comparable to the group of patients with OI type I (p-value=0.344), where osteosynthesis was performed using TEN.

Conclusion. The TEN method is a reliable method of treatment in children with OI type I, it is comparable to the method of telescopic metal construction in children with OI type I in terms of the frequency of migrations (p-value>0.999). However, the risk of repeated surgical interventions is increased by 5.3% compared with FD, the frequency of fractures is higher by 12.3%, the formation of deformities by 13%. The use of “growing” hardware in children with type III reduces the risk of possible refracture by 9%, migration by 36%, deformity by 21% and the number of revisions by 17% compared with static rods. Patients who underwent FD osteosynthesis showed higher results of motor activity than patients who underwent TEN osteosynthesis. The results of the frequency of complications and revisions in the group of patients with OI type III, in which osteosynthesis was performed by the telescopic FD system, are comparable with the group of patients with OI type I, in which osteosynthesis was performed with TEN static rods. The quality of life and motor activity of children in the group with a severe course, whose osteosynthesis was carried out with a “growing” metal structure, reaches the level of patients with a mild course of the disease, whose osteosynthesis was performed using TEN (p-value=0.344). The method of choice in the surgical treatment of children with OI, both in type I and type III, is a “growing” intramedullary metal structure. The use of static rods is acceptable in OI type I, however, it should be taken into account that this metal structure is effective for the first 12 months, later, due to the inability to reinforce the bone throughout its entire length, the risk of possible complications increases.

Pediatrics. Consilium Medicum. 2022;(4):373-379
pages 373-379 views


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