The structure and prevalence of dento-maxillary anomalies and deformations in adolescents with hereditarily caused pathology

2021 ◽  
pp. 22-31
Author(s):  
F. V. Samedov ◽  
R. D. Yusupov ◽  
Yu. S. Harutyunyan ◽  
T. A. Kondratyeva ◽  
D. A. Domenyuk

The high frequency of connective tissue dysplasia (CTD) in the pediatric population, its negative impact on the course of diseases of the dentoalveolar system predetermine the need to make additions to the standards of diagnosis and treatment of this category of patients. Depending on the severity of external phenotypic manifestations and laboratory, clinical and instrumental signs, among 114 adolescents 12–16 years old with general pathology, as well as the clinical symptom complex of CTD, groups with mild, moderate and severe undifferentiated dysplasia were formed. The control group consisted of 37 adolescents of the I and II health groups, matched by sex and age. The first stage of CTD diagnostics involves the identification and scoring of external dysplastic signs, the second stage of diagnostics includes an indepth clinical and instrumental examination to identify visceral CTD manifestations. It was found that the prevalence of dentoalveolar anomalies and deformities in combination with signs of undifferentiated connective tissue dysplasia in adolescence exceeds the frequency of detecting dentoalveolar anomalies and deformities in children of the control group by 1.4–10.9 times. It has been proven that the most informative phenotypic signs in patients with CTD and occlusive disorders are stigmas from the maxillofacial region (anomalies in the position of the teeth, high (Gothic) palate, deformation of the Spee occlusal curve, narrowing and deformation of the dentition, anomalies of attachment of the frenum of the tongue and lips ), bone-skeletal (hypermobility of joints, flat feet, clinodactyly, osteochondrosis, poor posture, anomalies of the skull, deformities of the limbs and chest), ectodermal (hyper-extensibility of the skin, thin, easily injured skin) and muscle (hypotonia of muscles). The presence of at least six informative dysplastic signs in dental patients is an objective criterion for an unclassified CTD phenotype.

Author(s):  
Elena E. Statovskaia

The purpose of this study was to determine the bodybuild, the basic anthropometric measurement parameters (body-weight index - BMI, Varga Index - VI, height, weight), the nature of variation thereof in relation to sex, age and possible correlation with dental health status, in adult patients with connective tissue dysplasia (CTD). A group of 360 dental patients of white race, aged 16-56, residents of St. Petersburg, including: control group of patients (n=84) not having the connective tissue dysplasia (CTD) and the basic group of patients (n=276) having the CTD, has been examined. Patients underwent complex clinical and instrumental examination of their dental, somatic status, CTD check. We have found that BMI, in comparison with VI, identifies the differences between the studied groups more accurately, and seems more preferable for diagnosis of the nutritional status and differential diagnosis of patients whether having or not having CTD. In the basic group, BMI is affected by the course of CTD and by age. In the control group, BMI is affected by age and gender. In the basic group, the course of CTD and gender affect VI. In the control group, gender and age affect VI. Correlations of the anthropometric parameters (n=320; p=0,0001) with CTD and dental parameters, have been identified. Varga Index has positive correlations with body-weight index, BMI (r=0.951), weight (r=0,837), periodontal biotype (r=0,289), and negative correlations with CTD (r = -0.290). BMI positively correlates with VI (r= 0,951), weight (r=0,856), and age (r=0.392), gingiva biotype (r=0,305), presence of dentition defects (r=0,301). BWI negatively correlates with the diagnostic bone criteria of CTD (r=-0,308; n=249) and the CTD phenotype (r=-0,323). In the examined patients, age correlates with the CFE index (index of caries-decayed, filled, extracted teeth) (r=508) positively, and correlates with the weight (r=-0.300) of the patient negatively (p=0,0001; n=320). Implementing the study of dental patients, it is necessary to take into account the values and correlations of the anthropometric measurement parameters (BMI, VI of weight, height), which correlate with age, gender and dental status characteristics (periodontal biotypes, CFE, dentition defects), as well as bone diagnostic criteria of CTD, feature of the CTD course and phenotype.


Annotation: The prevalence of microbial inflammatory diseases of the urinary system, according to epidemiological studies, is 29.0 per 1000 children. Over the past decades, an unfavourable tendency towards an increase in the pediatric population of chronic pathology has been observed, treatment complicated by a significant pathomorphosis of the disease. Aim of the research: analysis of the frequency of birth defects in the development of organs of the urinary system, as a visceral marker of violation of the fibrillogenase, in children with different variants of pyelonephritis. Materials and methods: 148 children with pyelonephritis from 3 to 18 years were examined. According to the results of catamnestic observation, they were divided into 2 groups: I - 92 people, children with pyelonephritis, in which catamnesis was diagnosed 3 or more episodes of recurrence of pyelonephritis in 2 years, II - 56 children with pyelonephritis, in which for 2 years no relapse of the disease was noted. The control group consisted of 65 somatically healthy children of the same age. All children had a routine comprehensive clinical and laboratory examination and clinical and laboratory markers of fibrillogenic disorder (phenotypic signs of undifferentiated connective tissue dysplasia (UCTD), excretion of oxyproline with urine) were established. Results: In children with recurrent pyelonephritis, phenotypic signs of undifferentiated connective tissue dysplasia. For this purpose, the activity of endothelin-1 fraction, alkaline phosphatase and serum creatine phosphokinase, excretion of creatinine and glycosaminoglycans with daily urine in children with pyelonephritis was studied. On average, the level of endothelin-1 in blood plasma in practically all children in group I was significantly higher (1.815±0.03 fmol/l, q=0.92, p≤0.01) more than in the 10th time, than in children with APN (0.179±0.02 fmol/l, q=0.78 p≤0.01) and healthy children (0.077±0.01 fmol/l, q=0.03). Conclusions: In children with recurrent pyelonephritis, compared with the data of children with acute non-recurrent pyelonephritis, phenotypic signs of undifferentiated dysplasia of connective tissue were significantly more marked. Thus, the negative role of UCTD in the course of pyelonephritis in children has been confirmed. It has been shown that UCTD in children with pyelonephritis is manifested not only by phenotypic features, but also by visceral, such as BD US, which leads to the chronization of the process with frequent relapses.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
O V Sidorovich ◽  
S Y U Elizarova ◽  
A V Khizhnyak ◽  
N A Kuznetsova ◽  
E E Prosova

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.


2018 ◽  
Vol 15 (2S) ◽  
pp. 58-64
Author(s):  
V. V. Li ◽  
G. P. Smoliakova ◽  
V. V. Egorov ◽  
O. I. Kashura

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. 


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
V. N. Nikolenko ◽  
M. V. Oganesyan ◽  
A. D. Vovkogon ◽  
Yu Cao ◽  
A. A. Churganova ◽  
...  

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.


2015 ◽  
Vol 14 (4) ◽  
pp. 42-50
Author(s):  
E. V. Fomenko ◽  
S. B. Tkachenko ◽  
N. F. Beresten ◽  
E. S. Pavochkina

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.


2012 ◽  
Vol 93 (4) ◽  
pp. 570-575
Author(s):  
Sh M Magomedova ◽  
Yu M Belozerov ◽  
K A Masuev ◽  
I M Osmanov

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.


2012 ◽  
Vol 93 (1) ◽  
pp. 93-97 ◽  
Author(s):  
E A Ivanova ◽  
O V Plotnikova ◽  
A V Glotov ◽  
V G Demchenko

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.


2020 ◽  
Vol 25 (4) ◽  
pp. 308-316
Author(s):  
E. Е. Statovskaya

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.


2021 ◽  
Vol 29 (1) ◽  
pp. 99-108
Author(s):  
Olga P. Saryeva ◽  
Aleksej P. Vahromeev ◽  
Violetta V. Parejshvili

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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