Prevention of health losses in adolescents with phenotypic manifestations of connective tissue dysplasia

Aim. Develop a program to prevent health losses in adolescents with connective tissue dysplasia. Methods. Analyzed was the clinical data of 241 adolescents for the period 2006-2009, 113 adolescents formed the main group, 128 - the control group. The statistical rules and standards for determining the grouping signs and group boundaries on the basis of phenotypic and visceral traits of connective tissue dysplasia were taken into consideration. Results. The proposed program for prophylaxis of health loss provides the following stages: informational, analytical, and organizational-executive with an educational component along with curative and preventive measures. The proposed program of prophylaxis provides complete information on the health status of adolescents with connective tissue dysplasia, promotes the reduction of the frequency of progression of signs of dysplasia. Conclusion. The program provides an opportunity to optimize the existing approaches to identifying and assessing factors that influence health, and promote more efficient use of the available resources in the education and healthcare systems.

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MORPHOLOGICAL MARKERS OF THE POST EXERCISE STRUCTURAL AND FUNCTIONAL DISORDERS OF THE LOCOMOTOR APPARATUS

Human Sport Medicine ◽

10.14529/hsm190313 ◽

2019 ◽

Vol 19 (3) ◽

pp. 103-111

Author(s):

V Nikolenko ◽

M Oganesyan ◽

A Vovkogon ◽

M Sankova ◽

N Rizaeva

Keyword(s):

Connective Tissue ◽

Preventive Measures ◽

Statistical Processing ◽

Control Group ◽

Functional Disorders ◽

Diagnostic Significance ◽

Post Exercise ◽

Locomotor Apparatus ◽

Connective Tissue Dysplasia ◽

Severe Degree

Aim. The article deals with establishing the character of the correlations between the somatometrical features and morphological signs of connective tissue dysplasia in persons with frequent post exercise structural and functional disorders of the locomotor apparatus. This is required for creating treatment and disease preventive measures and recommendations to choose an adequate physical activity. Materials and methods. A comprehensive medical and anthropological examination of 48 people aged from 18 to 47 years (mean age 36.38 ± 6.02 years) with frequent post exercise structural and functional disorders of the locomotor apparatus was carried out. A questionnaire was developed to assess the status of their connective tissue. The control group consisted of 36 apparently healthy people aged 18.85 ± 0.56 years. Statistical processing of the data obtained was performed using Microsoft Excel 2010. Results. All patients with frequent post exercise structural and functional disorders of the locomotor apparatus had connective tissue dysplasia of moderate and severe degree, the total score of which was 49.44 ± 13.1. The analysis of the frequency of connective tissue dysplasia morphological signs occurrence depending on their diagnostic significance, made it possible to establish the pathognomonic predictors of high susceptibility to frequent disorders of the locomotor apparatus. Conclusion. Early diagnostics of frequent disorders of the locomotor apparatus will contribute to timely preventive measures in terms of physical load and a corresponding rehabilitation for eliminating the development of the main disorder.

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Assessment of heart rate variability and tone of the autonomic nervous system in puberty-aged children with undifferentiated connective tissue dysplasia

European Heart Journal ◽

10.1093/eurheartj/ehab724.3361 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

O V Sidorovich ◽

S Y U Elizarova ◽

A V Khizhnyak ◽

N A Kuznetsova ◽

E E Prosova

Keyword(s):

Heart Rate ◽

Heart Rate Variability ◽

Nervous System ◽

Autonomic Nervous System ◽

Connective Tissue ◽

Control Group ◽

Healthy Children ◽

Autonomic Nervous ◽

Connective Tissue Dysplasia ◽

End Of Puberty

Abstract Within the framework of the research into pathological conditions and syndromes associated with undifferentiated connective tissue dysplasia (UCTD), an emphasis needs to be placed on the changes in heart rhythm. In puberty, the severity of neurovegetative disorders caused by the presence of UCTD might increase manifold. The features of heart rate variability (HRV) in pubertal children against the background of UCTD have been studied insufficiently and require more detailed analysis. This study aims to assess heart rate variability and tone of the autonomic nervous system (ANS) in puberty-aged children with UCTD. Two groups of children were examined, with methods including electrocardiography and cardiointervalography. The main group consisted of 36 puberty-aged children with UCTD, the control group consisted of 30 healthy children. ANS tone was assessed based on the HRV. In the control group of children aged 11, the value of variation range (VR) was 0.18±0.4, mode amplitude (MA) 35.2±2.4, stress index (IN) 72.8±4.3, which corresponds to the state of vegetative balance. As the puberty progresses, the state of eutonia is replaced by the activation of the sympathetic division of the ANS, which becomes most pronounced at the age of 13 years. In the control group of children aged 13, the value of VR was 0.08±0.02, MA 61.7±2.1, SI 210.6±8.6, which corresponds to moderate sympathicotonia. By the end of puberty, in healthy children, the severity of sympathetic activation begins to decrease, and at the age of 15 years, vegetative balance is again observed. However, this pattern is violated in children with UCTD. Upon entering puberty, moderate activation of the sympathetic division of the ANS is already observed in children with UCTD. At the age of 11 years in this group, the value of VR is 0.06±0.02, MA 72.4±3.1, SI 211.2±8.6. At the age of 13, pronounced sympathicotonia is observed, which is confirmed by the values of VR - 0.04±0.01, MA 80.2±4.3 and SI 34.3±14.7. By the end of puberty in children with UCTD, in contrast to the control group, the analyzed parameters indicate not an equilibrium of the ANS, but the predominance of parasympathetic activation. The research provides an insight into heart rate variability in puberty-aged children with UCTD. It was established that healthy children enter puberty in a state of equilibrium of the autonomic nervous system, which in the middle of puberty is replaced by moderate sympathicotonia, and then returns to its original values. Children with the presence of UCTD enter puberty with moderately pronounced sympathicotonia, which gradually increases to pronounced values and is replaced by moderate vagotonia. FUNDunding Acknowledgement Type of funding sources: None.

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Relevance of the Problem of Myopia in School-age Children with Signs of Undifferentiated Connective Tissue Dysplasia

Ophthalmology in Russia ◽

10.18008/1816-5095-2018-2s-58-64 ◽

2018 ◽

Vol 15 (2S) ◽

pp. 58-64

Author(s):

V. V. Li ◽

G. P. Smoliakova ◽

V. V. Egorov ◽

O. I. Kashura

Keyword(s):

Connective Tissue ◽

Choroidal Thickness ◽

School Age Children ◽

Comparative Characteristic ◽

Control Group ◽

Moderate Degree ◽

Ophthalmological Examination ◽

School Age ◽

School Myopia ◽

Connective Tissue Dysplasia

Purpose:to study the frequency and structure of undifferentiated connective tissue dysplasia (UCTD) in school-age children with myopia, their relationship to choroidal thickness and course of myopia.Patients and methods. The object of study was 120 children (240 eyes) aged 11–13 years with school axial myopia (axial length (AL) from 24.5 to 26.0 mm). Special ophthalmological examination included optical coherent tomography of macular zone (“RTVue 100” Optovue, USA) and “Cross Line” scan for measuring choroidal thickness in foveal area and 1000 μm from it in nasal and temporal regions.Results.According to studies, the phenotypic signs of UCTD were diagnosed in 56 children (46.7%) with school myopia, the largest group included phenotypic signs of locomotor syndrome; 14 children (25%) had a weak degree of UCTD, 34 children (60.7%) — moderate degree and 8 children (14.3%) — pronounced degree. Comparative characteristic of changes in choroidal thickness detected a high degree of interrelation between the severity of clinical manifestations of UCTD and the level of decrease in blood-flow in the choroid. In children of the main group with signs of UCTD, the annual growth of AL exceeded the same parameters in children of the control group by more than 2.5 times (p < 0.05); due of accumulation of phenotypic signs of UCTD, a more noticeable tendency to decrease of choroidal thickness and the increase of AL was observed (p < 0.05). The statistical analysis confirmed the presence of significant negative correlation between the decrease in average thickness index in segment of the macular map and the degree of increase of AL (p < 0.05).Conclusions. According to results of diagnostic screening, in 46.7% children with school myopia, we detected phenotypic signs of UCTD. The method of optical coherence tomography showed that with increasing severity of UCTD, indexes of choroidal thickness in the macular zone are significant decreased. In the catamnesis (duration 1 year), it was found that in children with myopia with moderate and pronounced degrees of UCTD, increase of AL is associated with degree of thinning choroidal thickness, which indicates violations of biomechanical status of sclera.

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Morphological signs of connective tissue dysplasia as predictors of frequent post-exercise musculoskeletal disorders

BMC Musculoskeletal Disorders ◽

10.1186/s12891-020-03698-0 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

V. N. Nikolenko ◽

M. V. Oganesyan ◽

A. D. Vovkogon ◽

Yu Cao ◽

A. A. Churganova ◽

...

Keyword(s):

Connective Tissue ◽

Musculoskeletal Disorders ◽

Varicose Veins ◽

Morphological Characteristics ◽

Control Group ◽

Post Exercise ◽

Connective Tissue Dysplasia ◽

Physiological Evaluation ◽

Severe Degree ◽

Study Participants

Abstract Background Connective tissue dysplasia (CTD) is a risk factor for musculoskeletal disorders. Changes caused by disorganization of collagen and elastin fibers lead to the inability of withstanding heavy mechanical stress. In clinical practice, diagnosis of these disorders depends on physical and anthropomorphic evaluation. Methods Forty-eight patients with frequent post-exercise musculoskeletal disorders were evaluated for CTD. The control group included 36 healthy participants. Both groups were evaluated via therapeutic examination with assessment of anthropometric indicators and physical-physiological evaluation, surveying and gathering of anamnesis. Based on testing results, study participants were evaluated on CTD presence and risk factors. Results All experimental group patients had connective tissue dysplasia of moderate and severe degree, with a total score of 49.44 ± 13.1. Certain morphological characteristics showed prevalence, allowing to determine pathognomonic predictors of high predisposition to frequent post-exercise musculoskeletal disorders. Back pain (100%), asthenic syndrome and kyphotic spinal deformation (75%), high gothic palate, hypermobility of joints and the auricles, excessive elasticity (63%), varicose veins of the lower extremities (56%) and hemorrhoids (56%), changes in the shape of the legs and temporomandibular joint (50%) showed to be significant clinical factors indicating possible connective tissue dysplasia. Conclusions The presence of these diagnostically significant morphological signs of CTD in humans is a pathognomonic predictor of a high predisposition to frequent injuries. Their early detection helps promote proper appointment of adequate physical activity regimen and develop treatment for the underlying cause.

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The prevalence of minor heart anomalies and characteristics of intracardiac hemodynamics in patients with connective tissue dysplasia according to tissue Doppler imaging

Regional blood circulation and microcirculation ◽

10.24884/1682-6655-2015-14-4-42-50 ◽

2015 ◽

Vol 14 (4) ◽

pp. 42-50

Author(s):

E. V. Fomenko ◽

S. B. Tkachenko ◽

N. F. Beresten ◽

E. S. Pavochkina

Keyword(s):

Connective Tissue ◽

Right Ventricle ◽

Diastolic Function ◽

Tissue Doppler ◽

Doppler Imaging ◽

Control Group ◽

Tei Index ◽

Connective Tissue Dysplasia ◽

Systolic And Diastolic Function ◽

The Right

Introduction and aim. Minor heart anomalies (MHA) are the visceral form of connective tissue dysplasia (CTD) that lead to a deterioration in both systolic and diastolic function of the left ventricle (LV). Information about functional state of the myocardium of the right ventricle (RV) of patients with MHA is not available widely. The aim of the study is to evaluate the frequency of occurrence of different types of MHA and features of intracardiac hemodynamic using Tissue Doppler (TD) examination of patients with CTD. Materials and methods. Total were surveyed 1240 people, of which were selected 67 patients with SHD and control group of 27 healthy persons (average age 30.1±4.0 years). Evaluation of systolic and diastolic function of LV and RV was carried out based on results of pulse-wave tissue Doppler of lateral and medial parts of the mitral fibrous annulus (MFAlat, MFFm) and tricuspid fibrous annulus (TEA), including the calculation of the index Tei. Results and discussion. The incidence of SHD was 23 %. Patients of first and second groups both have significantly higher Tei index MFAlat, MFFm and TFA, and at the same time the maximum values of Tei index were observed of patients with multiple MHA (0.50±0.09; 0.56±0.08; 0.49±0.10 standard units respectively). Conclusions. Evaluation of central hemodynamics using TD is very useful to identify disorders of myocardial performance of both the left and right ventricle. Increased Tei index is an early marker of diastolic dysfunction of both ventricles.

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Vascular aging phenotypes based on VaSerа-screening results in young people with hypertension: Place of connective tissue dysplasia

Arterial’naya Gipertenziya (Arterial Hypertension) ◽

10.18705/1607-419x-2021-27-2-188-205 ◽

2021 ◽

Vol 27 (2) ◽

pp. 188-205

Author(s):

M. E. Evsevyeva ◽

M. V. Eremin ◽

M. V. Rostovtseva ◽

E. N. Fursova ◽

A. V. Rusydi ◽

...

Keyword(s):

Young People ◽

Connective Tissue ◽

Preventive Measures ◽

Risk Groups ◽

Vascular Aging ◽

Group Data ◽

Connective Tissue Dysplasia ◽

The University ◽

Design And Methods ◽

Expert Recommendations

Objective. To assess the occurrence of various vascular aging phenotypes among young people, depending on the level of blood pressure (BP) and in relation to risk factors (RF) and the presence of connective tissue dysplasia (CTD).Design and methods. In total, 250 young people (88 boys and 162 girls) aged from 18 to 25 years old were examined at the University Health Center. All the subjects were divided into tercile groups based on cardio-ankle vascular index (CAVI), determined by the VaSera-1500 (Fucuda Denshia, Japan). According to the latest expert recommendations the third tercile group corresponds to early vascular aging or EVA syndrome. The first tercile group corresponds to favorable vascular aging. We have analyzed the representation of various vascular phenotypes in individuals with elevated and normal BP, as well as the main RF and external signs of CTD in each of CAVI-tercile group. Data processing was performed using the software package “Statistica 10.0” (StatSoft Inc., USA).Results. Among young people with office hypertension and high normal BP, the EVA phenotype and phenotypes of normal and favorable vascular aging are equally distributed. In persons with office normal and elevated BP, vascular stiffness correlates with weight, body mass index and waist circumference (with the increase in arterial rigidity these indicators decrease). CTD is registered among subjects with severe vascular remodeling both in boys and girls.Conclusions. Our data indicate the feasibility of angiology screening for identification of vascular aging phenotypes as a part of preventive measures among young people, regardless of the level of initial office BP. This strategy strategy will contribute to a more differentiated identification of risk groups and more individualized preventive interventions based on vascular aging phenotypes.

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Auscultatory manifestations of mitral valve prolapse in children with connective tissue dysplasia

Kazan medical journal ◽

10.17816/kmj1545 ◽

2012 ◽

Vol 93 (4) ◽

pp. 570-575

Author(s):

Sh M Magomedova ◽

Yu M Belozerov ◽

K A Masuev ◽

I M Osmanov

Keyword(s):

Mitral Valve ◽

Connective Tissue ◽

Children And Adolescents ◽

Mitral Valve Prolapse ◽

Control Group ◽

Ehlers Danlos Syndrome ◽

Psychoemotional Stress ◽

Connective Tissue Dysplasia ◽

Cardiac Pain ◽

Leaflet Prolapse

Aim. To study the features of auscultatory symptoms in children with mitral valve prolapse secondary to connective tissue dysplasia. Methods. Auscultation and phonocardiography was performed in 545 children and adolescents with mitral valve prolapse, including a prolapse, which is a variant of the asthenic constitution - 140, with a prolapse with undifferentiated connective tissue dysplasia - 340, with mitral valve prolapse with differentiated connective tissue dysplasia - 65 children (patients with Marfan syndrome and Ehlers-Danlos syndrome). The control group consisted of 200 children and adolescents of similar age. Results. In children with an asthenic constitution and mitral valve prolapse in most cases (90.7%) established were isolated clicks, and only iin a small percentage of cases the clicks combined with the late systolic murmur. Complaints of these children did not differ significantly in the frequency from the control values. Compared with the control group up to 6 times more frequently found was the symptom of chronic psychoemotional stress. Chronic psychoemotional stress was also observed more frequently in adolescents with mitral valve prolapse in the background of undifferentiated and differentiated connective tissue dysplasia. Children with asthenic constitution the frequency of cardiac pain did not differ from the control values. A significant increase in the frequency of cardialgia was reported in children and adolescents with undifferentiated and differentiated connective tissue dysplasia. Cardiac pain was described as stabbing, pressing, aching, and was felt in the left side of the chest without irradiation. In most children the pain continued for 5-20 min, were usually enduced by physical exertion and emotional stress, often accompanied by autonomic disturbances, which resolved spontaneously or after administration of the tincture of valerian or valokordin. The absence of ischemic changes in the myocardium according to the comprehensive investigation can be regarded as a manifestation of cardialgia as a sympathalgia related to psychoemotional features of children with mitral valve prolapse («cardiac pain is inseparable from the personality»). The auscultatory pattern suring mitral valve prolapse in the background of undifferentiated and differentiated connective tissue dysplasia was very similar. Conclusion. According to the phonocardiography and auscultatory data we can conclude that there is a certain parallelism of the severity of valve leaflet prolapse and of the sound phenomena: in isolated clicks the leaflet prolapse degree is usually minor, however in cases of isolated late systolic and holosystolic murmurs - the prolapse degree is significant.

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Comparative analysis of chronic constipation clinical course in children with connective tissue dysplasia

Российский педиатрический журнал ◽

10.18821/1560-9561-2016-19-1-20-23 ◽

2019 ◽

Vol 19 (1) ◽

pp. 20-23

Author(s):

Valentina N. Peregoedova ◽

I. K. Bogomolova ◽

E. V. Luzina

Keyword(s):

Comparative Analysis ◽

Connective Tissue ◽

Chronic Constipation ◽

Physical Disability ◽

Preventive Measures ◽

Clinical Course ◽

Diagnostic Signs ◽

Complicated Course ◽

Connective Tissue Dysplasia ◽

The One

Chronic constipation is the one of the most common pathologies of gastrointestinal tract in childhood. The aim of the study was to carry out a comparative analysis of clinical characteristics of chronic constipation in children with both connective tissue dysplasia symptoms and without them. 96 children aged of 4-17 years with chronic constipation were examined. The diagnosis of connective tissue dysplasia signs was executed with the aid of table by V.G. Arsentiev consisted of modified diagnostic signs. Children with connective tissue dysplasia were revealed to manifest chronic constipation with more severe complicated course 6.7 times more often, that serves a cause to detect a physical disability due to disease. 42% of patients with chronic constipation have deficit of body mass. Despite a long period of time from the onset of symptoms of constipation in children, over 36% of parents had not sought medical help. This indicates to their insufficient medical activity and determines the needfor early preventive measures aimed at prevention of the chronicity of this pathology

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The state of microcirculation and identification of microcirculatory predictors of chronic pain in younger patients with functional disorders of the masticatory system affected by connective tissue dysplasia

Periodontology ◽

10.33925/1683-3759-2020-25-4-308-316 ◽

2020 ◽

Vol 25 (4) ◽

pp. 308-316

Author(s):

E. Е. Statovskaya

Keyword(s):

Chronic Pain ◽

Blood Flow ◽

Connective Tissue ◽

Pain Syndrome ◽

High Amplitude ◽

Masticatory Muscles ◽

Main Group ◽

Control Group ◽

Doppler Flowmetry ◽

Connective Tissue Dysplasia

Relevance. Occlusal splints, made for the patients with temporomandibular disorders (TMD), influence proprioceptive sensitivity, sensory characteristics of pain, parameters of microcirculation of the pulp and periodontium of the healthy teeth. Condition of the pulp and periodontium of the healthy teeth should be assessed in patients with TMD associated with connective tissue dysplasia (CTD).Materials and methods. The study examined 36 TMD patients aged 26.3 ± 1.3 лет (М ± m) with CTD (n = 20, main group) and without CTD (n = 16, control group); with natural teeth, without complaints of TMD, masticatory muscles and periodontal condition. Laser doppler flowmetry (LDF) helped to analyze blood flow parameters in the periodontium and pulp of the healthy teeth. Received results were statistically analyzed.Results. Wavelet analysis revealed different microcirculatory flowmotion: high-amplitude irregular type was in patients of the main group, low-amplitude regular type was in controls. In the main group, perfusion is supported by high values of flux and vasomotion, ergotropic sympathetic effects (controlled by catecholamines) prevail, myogenic tone is reduced, elevated microvascular tone reflects possible changes in rheologic blood properties, microcirculatory signs of pain syndrome and relatively elevated ischemic index in pulp microcirculation of healthy teeth.Conclusion. Sensory and regulatory mechanisms, reflected by the condition of periodontium and pulp blood flow, can be regarded as early signs of inflammation and chronic pain in the diagnosis of TMD in patients without complaints.

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Pregnancy and delivery in women with undifferentiated connective tissue dysplasia: clinical and morphological aspects

I P Pavlov Russian Medical Biological Herald ◽

10.23888/pavlovj202129199-108 ◽

2021 ◽

Vol 29 (1) ◽

pp. 99-108

Author(s):

Olga P. Saryeva ◽

Aleksej P. Vahromeev ◽

Violetta V. Parejshvili

Keyword(s):

Connective Tissue ◽

Placental Insufficiency ◽

Structural Basis ◽

Control Group ◽

Morphological Examination ◽

Morphological Aspects ◽

Prophylactic Measures ◽

Pregnancy And Delivery ◽

Connective Tissue Dysplasia

Aim. This study aimed to identify peculiarities of the course of pregnancy, delivery, and morphology of the placenta in women with undifferentiated connective tissue dysplasia (UCTD). Materials and Methods. The main group included 60 pregnant women with UCTD, and the control group was composed of 30 somatically healthy women with physiological course of pregnancy. All patients were examined as regards the use of general clinical and laboratory methods. Moreover, 65 placentas were subjected to complex morphological examinations, including organometry, macro- and microscopic examinations, and immunohistochemistry. Results. Pregnancy and delivery in women with UCTD encounter various complications, such as threat of premature birth and miscarriage, pre-eclampsia, prenatal amenorrhea, and placental insufficiency manifested as fetal growth restriction syndrome and confirmed in the course of morphological examination. The structural basis of chronic placental insufficiency is associated various pathologies of the umbilical cord and disorders in maturation of the villous chorion with the underlying weak adaptive and compensatory processes. Connective tissue dysplasia in fetal membranes is manifested as disorders in histo- and cytoarchitectonics and in increased expression of matrix metalloproteinase-9 by amniocytes and cytotrophoblast cells. Conclusion. UCTD produces unfavorable effect on the course of pregnancy and delivery. The results of the clinical and morphological examinations demonstrate the multiorgan characteristics of injuries in the mother-placenta-fetus system, which necessitates further studies for the determination of complex prophylactic measures.

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