Background. The LoeysDietz syndrome is a rare autosomal dominant connective tissue disorder characterized by the pathology of the cardiovascular system in combination with various anomalies of the musculoskeletal system. In modern literature, there is neither any information about the frequency of pathology nor any algorithm of examination and treatment for patients with this syndrome. Clinical case. The article presents a clinical observation of a 7-year-old patient with LoeysDietz syndrome with a genetically confirmed diagnosis. Discussion. This article provided a literature review, examined diagnosis issues and differential diagnosis, and presented the clinical picture of the syndrome. The main symptoms of LoeysDietz syndrome are artery aneurysms (most often in the aortic root), arterial tortuosity (mainly the vessels of the neck), hypertelorism, and bifid (split) or broad uvula. However, the combination of these symptoms is not found in all patients with this disease. Conclusions. The article emphasized the importance of a genetic verification of the disease, as well as a multidisciplinary approach to treatment with mandatory dynamic monitoring by specialists such as a cardiologist, neurologist, orthopedist, and pediatrician, which help prevent the development of complications and increase the life expectancy of this group of patients.
BACKGROUND:Osteochondritis dissecans of the femoral condyles is characterized by subchondral bone lesions, with subsequent formation ofanosteonecrosis area. In nearly half of the cases, gonarthrosis developed in the long-term period despite timely treatment of such patients, including children. The development of new techniques and the improvement of existing ones will help enhance the treatment results of patients with this pathology. AIM:To evaluate the efficacy of treatment inasmall clinical series of pediatric patients with osteochondritis dissecans by triple injections of platelet-rich plasma (PRP) according to the developed scheme in combination with revascularizing tunnelization of the lesion area. MATERIALS AND METHODS:Seven patients with stage I or II osteochondritis dissecans were treated by revascularizing stimulation of the osteonecrosis center by triple injections of PRP (the first procedure was conducted intraoperatively intraosseously and the two other injections subsequently intraarticularly). The follow-up period was10 (611)months, withamaximum duration of 12 months. RESULTS:The observation results demonstrateahigh efficacy of PRP therapy to enhance the effect of mechanical methods of osteochondrogenesis stimulation in children with osteochondritis dissecans. CONCLUSIONS:The use of orthobiological technologies isanactively developing and promising approach in the complex treatment of children with osteochondritis dissecans of the femur condyles. However, further observation is required to evaluate the long-term results.
Дистальный лучелоктевой сустав (ДЛЛС) входит в состав кистевого сустава, стабильность ДЛЛС придают такие структуры, как треугольный фиброзно-хрящевой комплекс (ТФХК), локтевой разгибатель кисти, межкостная мембрана, квадратный пронатор предплечья, а также ладонные и тыльные лучелоктевые связки. По мнению авторов, травматический тип нестабильности ДЛЛС у детей наиболее часто возникает при «свежих» переломах и переломовывихах дистальных структур локтевой и лучевой костей, в результате которых происходит повреждение стабилизаторов ДЛЛС. В зависимости от характера травмы костей предплечья головка локтевой кости может вывихиваться в ладонную или тыльную сторону-волярный или дорзальный типы смещения. Посттравматическая нестабильность ДЛЛС формируется вследствие неправильно срастающихся переломов и остановки роста одной из костей предплечья. В отдельную группу авторы выделяют изолированное повреждение стабилизаторов ДЛЛС без предшествующего перелома костей предплечья. В статье представлен анализ литературных данных о методах диагностики и принципах лечения нестабильности дистального лучелоктевого сустава у детей. Отсутствие четко обоснованной тактики обследования и диагностического алгоритма, нацеленного на обнаружение поврежденных структур ДЛЛС у детей, а также данных об эффективных методиках лечения нестабильности в ДЛЛС, направленных на восстановление функции кистевого сустава, обусловливает актуальность исследования данной патологии у пациентов детского возраста. Ключевые слова: кистевой сустав, треугольный фиброзно-хрящевой комплекс, травма, нестабильность, дети
BACKGROUND: The distal radial physis is involved in the injury process in approximately 15% of distal radius fractures. Distal radius physeal arrest and the normal functioning of the distal ulna growth lead to lengthening and dislocation of the head of the ulna in the distal radioulnar joint (DRUJ). These changes, in turn, lead to pain syndrome and forearm dysfunction, which is a manifestation of DRUJ instability. AIM: This study aims to evaluate the results of a study of pediatric patients with traumatic DRUJ instability. MATERIALS AND METHODS: An analysis of the results of the examination of 11 children aged from 13 to 17 years with traumatic type DRUJ instability due to the distal radius growth arrest. RESULTS: According to the X-ray examination data, all children showed closure of the distal growth zone of the radius and ulnar positive variant (ulna +). The shortening of the radius was calculated. Also, the time interval between the injury and the discovery of the wrist joint pathology was estimated. All types of radius fractures with growth plate involvement can cause physeal arrest, leading to a DRUJ instability. The interval from the acute wrist injury with damage to the distal radius growth zone to develop clinical manifestations of a DRUJ instability is 2.4 years on average. CONCLUSION: The development of this type of traumatic DRUJ instability is typical only in children since changes occur in the presence of an active growth zone and are associated with previous fractures of the distal radius. Therefore, a long-term dispensary observation by a traumatologist-orthopedist is necessary for patients with this pathology.
Background. At present, the literature describes in sufficient detail the use of various methods of X-ray examination of the bones of the forearm in the diagnosis of distal radioulnar joint instability (DRJI), but there are no data on radiometric parameters for DRJI of traumatic origin in children. Quantitative diagnostics becomes mandatory for determining the tactics of treating DRJI of traumatic origin in children. The purpose of study - to analyze the radiometric parameters of the forearm in case of DRJI of traumatic origin in children. Materials and methods. The paper presents an analysis of the results of X-ray examination of 23 children with instability of the distal radioulnar joint of traumatic origin aged 9 to 17 years (mean age - 14.21 2.5 years) - the main group. For comparison, radiographs of the contralateral forearms of the same patients were analyzed - the comparison group (23 children), and radiographs of the forearm of 69 pediatric patients without signs of DRJI (control group). On radiographs in the anteroposterior and lateral projections, the following radiometric parameters were evaluated: radioulnar and volar angles, radioulnar index, radioulnar distance, and the difference between the radioulnar distances of both forearms. Results. In 19 patients of the main group, a "positive variant" of the radioulnar index with dislocation of the head of the ulna was revealed, while the indicators of the radioulnar and volar angle were characterized by variability in values. The average values of radiometric parameters of DRJI in children without bone-traumatic changes of the forearm are comparable to normal values in adults. Conclusion. In children with DRJI of traumatic origin, various changes were revealed radiometric indicators of the distal parts of the bones of the forearm, which depend on the type of forearm fracture. In a particular pediatric patient with DRJI of traumatic origin, these indicators reflect the biomechanical features of the wrist joint, which must be taken into account when planning surgical intervention and predicting the recovery of the anatomy and function of the forearm.
Summary. Seismic isolation systems of kinematic type, selected as promising ones, first proposed in the former Soviet Union by Professor Yu.D. Cherepinsky, are considered. This technology was further developed in the works of Professor A.M. Kurzanov, who introduced his developments at the facilities of the Krasnodar Territory of the Russian Federation. However, the analyzed innovative domestic technology is not without its drawbacks. Subject of research: the design and technology of seismic isolation of overground floors of civil buildings using kinematic systems made of pipe-concrete columns. Materials and methods: analysis of the state of the art with a description of the positive and negative aspects of the existing technology, substantiation of the ways of possible progressive development of structures and technology of seismic isolation systems of the kinematic type, increasing the reliability of their operation under seismic load. Creation of graphic models of the nodes of support of concrete columns, implementing the intended directions in specific design solutions and technology for their implementation. Results. Patented designs of joints for pivotal support of pipe-concrete columns of kinematic systems for seismic isolation of frames of civil objects and a technological scheme for their alignment and high-precision installation. Conclusions: New design solutions have been proposed for the joints of the hinged support of pipe-concrete columns of kinematic systems of seismic isolation, as well as an improved technology for their installation, implying an increase in the reliability of their long-term operation in areas of high seismic activity.
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