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

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.

Author(s):  
Ye. H. Zaremba ◽  
N. O. Rak ◽  
O. V. Zaremba ◽  
O. V. Zaremba-Fedchyshyn ◽  
M. M. Virna ◽  
...  

The aim – to improve the diagnosis efficiency of patients with arterial hypertension (AH) combined with undifferentiated connective tissue dysplasia (UCTD) based on the study of clinical course, the severity of UCTD external and internal symptoms with determining the levels of IL-1, IL-6, TNF-a, free and general blood oxyproline. Material and Methods. The study implied examination of 90 patients (52 women and 38 men) with AH of stage 2 from first to third degrees with manifestations of UCTD, who were on inpatient treatment in the Cardiology Department of the Lviv City Communal Clinical Emergency Hospital. The mean age of patients was (61.14±2.58) years. Patients were divided into 3 groups depending on the stage and degree of hypertension. Group I (n=16) included patients with hypertension of the first degree, group II (n=35) – with AH of the second degree, group III (n=39) – with AH of the third degree. The control group consisted of 20 patients with hypertension without manifestations of CTD. Patients underwent clinical (checkup, palpation, percussion, auscultation), laboratory (determination of IL-1, IL-6, TNF-a, free and general oxyprolin blood levels) and instrumental studies (ECG, echocardiography, DBPM, ultrasound examination of internal organs and vessels of the lower extremities, ultrasound duplex examination of the carotid and vertebral arteries, radiological examination of the osteoarticular system), consultations of an ophthalmologist, neurologist, traumatologist and dentist. Results. As a result of the study, in the group I of patients (n=16) UCTD of slight degree of severity was revealed in 13 (81 %), of medium degree – in 3 (19 %) persons. In the group II of patients (n=35), UCTD of moderate severity was found in 30 (86 %), severe – in 5 patients (14 %). In the group III of patients (n=39), UCTD of moderate severity was found in 32 patients (82 %), severe – in 7 (18 %). In the study of the level of TNF-a, an increase compared to the control group was detected, particularly, in the group I of patients its level exceeded in 37.4 times, in the group II – in 39.6 times, in the group III – in 46.2 times (p<0.001). IL-1b increased by 2.6-fold (р<0.05) in the group I of patients compared to the control group, 3.1-fold (р<0.01) – in the group II, and by 3.7-fold, that was the foremost (р<0.001), in patients of the group III. In turn, IL-6 in the group I of patients exceeded the indicators of the control group by 4.3 times (p<0.001), in the group II – 4.8 times (p<0.001), in the group III – 5.7 times (р<0.001). The study of the level of free oxyproline revealed its increase in comparison with the control group, namely in the group I of patients exceeded in 6.12 times, in the group II – in 6.81 times, in the group III – by 7.56 times (р<0.01). The study of the general of bound oxyproline revealed its increase in comparison with the control group, namely in the group I of patients exceeded by 6.98 times, in the group II – by 7.79 times, in the group III – by 9.42 times (p<0.01), which indicates an increase in fibrillogenesis and more pronounced destructive and inflammatory processes in connective tissue. Conclusions. Patients with AH of the stage 2 from first to third degrees with manifestations of UCTD have increased levels of IL-1, IL-6 and TNF-a, which are not only sensitive markers of inflammation, but also play an important role in the pathogenesis and progression of vascular lesions, occurrence and destabilization of atherosclerotic plaques and thrombotic vessels occlusion. The determination of blood oxyprolin in patients with hypertension combined with UCTD confirms the presence of CTD, namely the decay of collagen is more pronounced in patients with severe dysplasia.


2020 ◽  
Vol 24 (1) ◽  
pp. 45-50
Author(s):  
Yu. F. Lobanov ◽  
D. Y. Latyshev ◽  
Ya. F. Zverev ◽  
N. A. Tekuteva ◽  
N. M. Mikheeva

THE AIM: To study the characteristics of phosphorus-calcium metabolism in patients with neurogenic disorders of urination, taking into account the severity of the manifestations of connective tissue dysplasia. PATIENTS AND METHODS. The study included 90 children, including 60 children with neuro-muscular dysfunction of the bladder (NMDB) and 30 children with enuresis from the age of 5 to 15 years. The diagnosis was established based on a comprehensive examination and according to industry standards. Connective tissue dysplasia was diagnosed in children with the detection of 6 or more small external or visceral manifestations involving 3 or more organs from different systems. Assessment of the severity (severity) of connective tissue dysplasia was carried out according to the point system proposed by T.I. Kadurina et al. Each group was divided into subgroups depending on the severity of the manifestations of connective tissue dysplasia. The determination of the level of calcium and phosphorus in the blood and urine, as well as the calculation of the calcium-creatinine coefficient followed by a comparison of the results in these groups and subgroups. To assess the significance of differences, the Mann-Whitney test was calculated, p <0.05 was considered significant. RESULTS. he level of calcium and phosphorus in the urine was slightly higher in children with enuresis, especially in the morning portion of urine, where the concentration of calcium was 26% higher than in patients with NMDB. At the same time, the value of calcium /creatinine coefficient was significantly higher in the group of patients with enuresis and was 2 times higher than the normative indicators, which indicates the importance of hypercalciuria in the development of enuresis. СONCLUSION. According to the obtained data, the severity of calciuria, determined by the value of the calcium-creatinine coefficient, is significantly higher in patients with enuresis than with NMDB.


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. 


2018 ◽  
pp. 67-72
Author(s):  
T.G. Romanenko ◽  
◽  
O.M. Sulimenko ◽  

The objective: was to reduce the incidence and severity of the development of «Great Obstetrical Syndromes»: miscarriage of pregnancy, placental insufficiency, fetal growth retardation and / or fetal distress, preeclampsia, premature detachment of a normally located placenta, premature delivery, by developing an algorithm for prophylaxis during pregnancy in women at high risk for their development. Materials and methods. Clinical and statistical analysis of pregnancy in 88 pregnant women with high risk of decompensation of placental insufficiency was conducted. Depending on the method of preventing complications, 2 groups were formed. The I group included 58 pregnant women who were offered the following algorithm: micronized progesterone 200 mg PV from 6 to 20 weeks of gestation; from 21 to 26 weeks of gestation and from 31 to 36 weeks; phleboprotector with ultra-micronized fractions of bioflavonoids (Flego) 15 ml per os; with subsequent appointment from 21 to 26 weeks of gestation; and from 31 to 36 weeks. Group II included 30 pregnant women who had abandoned any proposed prophylactic measures. The control group consisted of 30 healthy pregnant women. Clinical and statistical analysis of pregnancy in the study groups was conducted. During statistical processing, personal computer and software Microsoft Excel XP and Statistica 6.0 Windows, methods of descriptive statistics, correlation analysis were used. The reliability of the difference between the indicators is estimated by the Student-Fisher criterion. Results. The method of prophylaxis of «Great Obstetrical Syndromes» by sequential administration from the early stages of pregnancy of micronized progesterone with the following appointment – from the second trimester of pregnancy – showed a high efficacy of the phleboprotector, which is manifested in a significantly lower number of cases of preeclampsia in the main group of 3.5% (2) versus 50% (15) in the control group, placental insufficiency was 13.8% (8) versus 100% (30); fetal growth retardation was 5.2% (3) versus 56.7% (17); fetal distress was 3.5% (2) versus 43 , 3% (13), preterm labor 1.7% (1) versus 13.3% (4) and in the absence of premature detachment normal but located on the placenta, severe forms of preeclampsia and placental insufficiency. Conclusions. Pregnant of «Great Obstetrical Syndromes» risk groups need timely prophylactic measures to reduce the likelihood of a pathological pregnancy and improve perinatal outcomes. The proposed prophylaxis scheme significantly lowered the incidence of large obstetric syndromes and improved neonatal outcomes. Key words: great obstetrical syndromes, pathological pregnancy, placental dysfunction, phleboprotector.


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.


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