Undifferentiated Connective Tissue Dysplasia Research Articles

Magnesium in Idiopathic Mitral Valve Prolapse

Abstract The aim of our research was to increase the effectiveness of the therapy administered to the patients with idiopathic mitral valve prolapse by pharmacological correction of magnesium deficiency. 79 patients (23 females and 56 males with average years of age 35.7±4.3 ) with undifferentiated connective tissue dysplasia and mitral valve prolapse of the 1st and 2nd degree were examined. The control group consisted of 20 healthy individuals, comparable by sex and age. A test by the UNESCO Institute for Microelements was used for the preliminary diagnostics of magnesium deficiency. Daily ECG monitoring with heart rate variability analysis, echodopplercardiography with the assessment of left ventricular diastolic function and determination of magnesium concentration in blood serum were performed. For the demonstration of autonomic dysfunction “the test for detection of the signs of vegetative changes” was used (10). For the assessment of situational and personal anxiety an “anxiety test” by Ch. D. Spielberg and Y. L. Hanin (25, 26) was used. The succeeding study was performed after 6 months. It was found that complex therapy with magnesium orotate in patients with idiopathic mitral valve prolapse helps to reduce the frequency of clinical manifestations of neurovegetative disturbances in the majority of examined patients contributing to harmonization of the autonomic nervous system function. It has a favorable effect on dysplastic changes and the state of bioelectrical activity of the heart, as well as correction of the psychoemotional state.

Спонтанный пневмоторакс: некоторые аспекты этиологии, патогенеза и лечения (обзор литературы)

The review article discusses the prevalence, etiology, pathogenesis, and treatment of spontaneous pneumothorax. The vascular, mechanical, obstructive, infectious, and enzymatic theories of the occurrence of emphysematous lung changes as the main cause of developing spontaneous pneumothorax are described; the role of smoking as the main etiological factor and its influence on the recurrence rate of spontaneous pneumothorax are studied. The issues of choosing primary cure tactic are considered. The effectiveness of conservative treatment, puncture and drainage of the pleural cavity as methods of treatment and pleurodesis for the prevention of recurrences of spontaneous pneumothorax are described. A comparativeassessment of various methods of pleural cavity obliteration is given: chemical pleurodesis (by sterile talc, minocycline, povidone-iodine, 50% glucose solution, autologous blood and its components, autologous adipose tissue, pseudomonas aeruginosa sensitive hemagglutinin, picibanil), apical pleurectomy, pleuroabrasion. The experience of using YAG-ND and CO2 as methods of physical pleurodesis, intraoperative Staple Line Coverage with polyglyconic acid and fibrin gel is described. The necessity of active surgical tactic in the treatment of patients with spontaneous pneumothorax developed on the base of undifferentiated connective tissue dysplasia has been substantiated. The advantages, disadvantages, indications and contrindications to different ways of surgical treatment (thoracotomy, video-assisted minithoracotomy, thoracoscopy) are discussed. The strategy for choosing treatment methods depending on the volume of pneumothorax, the severity of bullous changes, the presence of concomitant diseases, chronic obstructive pulmonary disease, and the value of the Charlson comorbidity index is considered. The expediency of using video-assisted minithoracotomy as the safest and most effective method of surgical treatment of spontaneous pneumothorax has been substantiated. The effectiveness of surgical interventions depending on the timing of their implementation is considered.

The Role of Vitamin D Metabolite Deficiency in the Development of Structural and Functional Disorders in Coxarthrosis

Summary. At present, there is a need to systematize the data of our own comprehensive research and literature in order to determine the role of active metabolites of vitamin D in the formation of structural and functional disorders (SFD) in osteoarthritis of the hip.
 Objective: on the basis of our own complex researches and data of literature, to determine the role of the insufficiency of vitamin D metabolites in the development of SFD in coxarthrosis.
 Materials and Methods. The basis for determining the role of active metabolites of vitamin D in the development of SFN in coxarthrosis was our own comprehensive studies of 506 patients with osteoarthritis of the hip and data from the literature.
 Results. On the basis of the theory of functional systems, a conceptual model for the development of SFD in coxarthrosis has been developed. The leading factor in the development of SFD in the rapid progression of idiopathic and dysplastic osteoarthritis of the hip is the lack of active metabolites of vitamin D. The presence of undifferentiated connective tissue dysplasia in turn causes a decrease in the absorption of provitamin D in the stomach and intestines. With a slow progression of idiopathic coxarthrosis, the leading factor in the development of these disorders is the excessive load on the hip joint. Factors of violation of biomechanical conditions and injury of the hip joint are factors of the progression of coxarthrosis of dysplastic and post-traumatic genesis. Pathogenic factors lead to functional and structural changes in systems of different levels and their elements with the development of inverted processes in the structures of the hip joint.
 Conclusions. Insufficiency of vitamin D metabolites on the background of undifferentiated connective tissue dysplasia leads to biochemical changes in articular cartilage and serum, affects both osteo- and chondrogenesis, leads to reduced immune status of patients and the development of clinical manifestations of rapid progression of idiopathic and dysplastic coxarthrosis. With a slow progression of idiopathic coxarthrosis, the main factor leading to the development of the above mentioned disorders is the excessive load on the hip joint. Factors of violation of biomechanical conditions and injury of the hip joint determine the development of SFD in coxarthrosis of dysplastic and post-traumatic genesis.

Особенности хориоидального кровотока и их влияние на формирование периферических дегенераций сетчатки у детей школьного возраста с недифференцированной дисплазией соединительной ткани

Purpose. To study choroidal thickness (CT) and assess its effect on frequency of occurrence dangerous types of peripheral retinal degenerations (PRD) in school-age children with myopia associated with undifferentiated connective tissue dysplasia (UCTD). Material and methods. The object of study was 62 children (124 eyes) aged 10–15 years (mean age 12.3±0.8 years) with progressive myopia associated with moderate (48 people, 77.4%) and pronounced (14 people, 22.6%) degree of UCTD. Special ophthalmologic examination included macular imaging by optical coherence tomography (RTVue 100, «Optovue», USA) to measure CT. Diagnostic criteria by T.M. Milkovskaya-Dimitrova et al (1987) were used to identify phenotypic characters of UCTD. Results. Comparative characteristic of CT in macula showed high (p <0.05) relationship between the blood-filled of choroidal vessels and degree of UCTD. Analysis frequency of occurrence of PRD depending on CT demonstrated that against the background accumulation of phenotypic characters of UCTD increase in frequency of occurrence of PRD was observed. Statistical analysis confirmed presence of significant negative correlation between decrease CT and increase in incidence of prognostically dangerous forms of PRD. Conclusion. It was found that in school-age children with myopia with increase degree of UCTD, CT decreases. With choroidal thinning in children with myopia associated with UCTD, the frequency of occurrence dangerous forms of PRD increases. The research results are of clinical importance for the development of rational approaches to clinical examination and treatment children with school myopia with clinical signs of UCTD. Key words: progressive myopia, undifferentiated connective tissue dysplasia, choroidal thickness, peripheral retinal degeneration, rhegmatogenous retinal detachment.

The state of bone mineral density in the prolapse of the pelvic organs

Background. Plausible predictions of future mortality and disability are useful aids in making decisions about priorities for medical research. Recent advances in medicine have led to the fact that some countries, such as Japan, have the highest life expectancy in the world. Osteoporotic fractures make a significant contribution to the development of morbidity and mortality in the aging population. Despite an active study of the mechanisms of the development of pelvic organ prolapse and disorders of bone mineral density separately, the general background of these diseases has previously been little studied.
 Aim. To study bone density according to densitometry data in peri- and postmenopausal women.
 Materials and methods. To carry out this study, 2 groups of women were formed. The first (experimental) group included 76 women admitted for routine surgical treatment of genital prolapse, the second group (control) 68 women without clinical manifestations of pelvic floor descent, who underwent a medical examination by a gynecologist on an outpatient basis.
 Results. Analysis of extragenital diseases in women included in the study showed a high prevalence in the experimental group of hypertension (p=0.028), osteochondrosis (p=0.001), scoliosis (p=0.028), hemorrhoids (p=0.013). Also, women in this group more often noted stretch marks on the skin (p=0.002) and were more often operated on for other diseases (p=0.043), which indicates the association of genital prolapse with undifferentiated connective tissue dysplasia and hypertension. The diagnosed severe osteoporosis (according to 1 or more densitometry indicators, where the T-score is less than -2.5) had a direct correlation with the duration of menopause. For a detailed analysis of the factors influencing severe osteoporosis in peri- and postmenopausal women, logistic regression analysis and ROC analysis were performed. The independent variable was the presence of severe osteoporosis, and the dependent variables were: stage according to POP-Q, the presence of hypertension, the presence of diabetes mellitus in history.
 Conclusion. This study confirms the presence of a comorbidity decrease in bone density according to densitometry and the presence of hypertension (p=0.028) and genital prolapse. According to the ROC analysis, an increase in the stage of genital prolapse according to the POP-Q classification increases the risk of developing severe osteoporosis.

Cosmetic Syndrome Correction with Calcium Hydroxylapatite-Based Filler in Patients with Connective Tissue Dysplasia.

Undifferentiated connective tissue dysplasia is one of the most common diseases of nowadays, which does not fit into the group of hereditary syndromes. This condition is diagnosed in 20–50% of the population at any age. The study aimed to correct the facial soft tissues of patients with undifferentiated connective tissue dysplasia through the cosmetic procedure of calcium hydroxylapatite injection. In 2018, a 36-year-old patient addressed the beauty salon with signs of undifferentiated connective tissue dysplasia, such as severe asymmetry of the face, infraorbital and nasolabial sulci, and thin and easily folding skin. Signs were observed from the age of 22, i.e., for 14 years. The therapy was performed using special features of the correction of facial soft tissue changes in patients with connective tissue dysplasia (CTD) using calcium hydroxylapatite-based products (Radiesse®, Merz North America, Inc., USA). Particular attention is given to the need for early correction to prevent premature skin aging related to this condition. After 14 days, a significant improvement of the patient's skin condition was noted after the passing of two procedures. Her condition was estimated as consistent with T1-2P0G0A1Zh1 P1M1K1 and corresponded to grade I age-related changes in the superficial soft tissues. The performed treatment showed high efficacy in case of mild connective tissue dysplasia diagnosis. The results showed that when collecting information from anamnesis, the diagnostic criteria for dysplasia should be considered. If the criteria are met, the cosmetological correction with collagen stimulators becomes possible.

Predictors of genitourinary syndrome in women of elderly reproductive age after hysterectomy

The article presents an analytical assessment of risk factors for distant postoperative urogenital disorders in women of older reproductive age after radical surgical treatment for uterine fibroids. The aim of the study was to optimize the diagnostic algorithm of genitourinary syndrome in women of older reproductive age after hysterectomy for uterine fibroids. Materials and methods – 80 women aged 40 to 50 years were examined: 40 patients after vaginal and laparoscopically assisted vaginal hysterectomy, 40 patients with abdominal hysterectomy, control – 30 relatively healthy women with asymptomatic fibroids. The examination was performed at the stage of preoperative observation and for 3 years after surgery. Research methods: data on the assessment of pelvic floor and urogenital dysfunction using a standardized POP-Q system, physical examination, the ICS classification was used to assess the severity of urinary incontinence; quality of life assessment using the MOS SF-36 questionnaire; standard methods of variation statistics. Research results and their discussion. The most important risk factors for genitourinary syndrome have been identified. These included obstetric and gynecological history (birth of large fetuses, episiotomies, birth trauma, high birth parity, ovarian surgery, the use of gonadotropin-releasing hormone agonists), chronic diseases accompanied by increased intramuscularly; the presence of stigmas of undifferentiated connective tissue dysplasia; clinical manifestations of pelvic floor failure and functional disorders of the urethral sphincter. Conclusions. In the case of surgical treatment of uterine fibroids, it is advisable to take into account not only the presence of vaginal prolapse and initial dysuric manifestations, but also their predictors in the preoperative stage of the examination, as this may affect the choice of method and access in the case of surgical treatment of uterine fibroids. rehabilitation program and will obtain the expected effect of surgical recovery.

FEATURES OF APONEUROSIS PATHOMORPHOLOGICAL CHANGES IN PATIENTS WITH EVENTRATION

Objective – to improve the treatment results of patients with eventration by determiningthe role of undifferentiated connective tissue dysplasia in the development of thiscomplication.Material and methods. The object of the study comprises 33 patients with eventrationwho were treated in the department of thoracoabdominal surgery of Shalimov NationalInstitute of Surgery and Transplantology during 2017-2020.Results. At complex pathomorphological research of aponeurosis fragments similarmorphological changes have been revealed in groups of patients with phenotypic signsof undifferentiated connective tissue dysplasia and eventration. Immunohistochemicalexamination of tissues with monoclonal antibodies to α-SMA revealed uneven, focalexpression in smooth muscle differentiation cells and fibroblasts in both groups. In thestudies with monoclonal antibodies to Collagen IV, moderate positive expression in thebasement membrane of the blood vessels, in smooth muscle cells of the muscular layerof the vascular wall, in areas of the connective tissue was observed, that is a sign ofpathological remodeling of the connective tissue.Conclusions. Similar pathomorphological changes of the aponeurosis in groups withphenotypic signs of undifferentiated connective tissue dysplasia and post-operativeeventration confirm the role of the connective tissue pathology in the development of thiscomplication.

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

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.

Characteristics of physical qualities and vegetative regulation in adolescents with postural disorders

Objective. To characterize the physical qualities and autonomic regulation in adolescents with postural disorders.
 Material and methods. 124 teenagers aged 16-17 including 58 boys and 66 girls were examined. There were 2 groups of patients: with postural disorders in the sagittal (n = 57) and frontal plane (n = 67). The examination program included an assessment of physical development, somatotype, general endurance and static endurance of the large muscles of the back and trunk, autonomic regulation by analyzing heart rate variability, levels of personal and situational anxiety, as well as self-esteem.
 Results. Adolescents with impaired posture in the sagittal plane are characterized by the predominance of the asthenoid somatotype, a high frequency of body weight deficiency, a decrease in overall endurance, as well as endurance of the back and abdominal muscles, and a predominance of the activity of the parasympathetic autonomic nervous system. In this category of patients, the thoracic and muscular types of physique predominate in the frontal plane of postural disorders, and there is a significant asymmetry in the static endurance of the lateral muscles of the trunk, and an increase in the functional activity of the sympathetic autonomic nervous system. In both of these variants of postural disorders in adolescents, there is a high frequency of undifferentiated connective tissue dysplasia, sympathetic hyperreactivity, personal and situational anxiety, and a decrease in self-esteem.
 Conclusions. Features of physical qualities, vegetative regulation and psychological sphere should be taken into account when developing differentiated programs for correcting posture in adolescents, depending on the plane of its violation.

Influence of Connective Tissue Dysplasia Syndrome on Male Reproductive Function

The work demonstrates the influence of undifferentiated connective tissue dysplasia on male reproductive function, shows which dysplastic-dependent pathology of the male genital area affects the spermogram indices in men. Shown are local problems of deep pathophysiological and histological prerequisites in their development of the male factor of infertility in a married couple. The paper demonstrates the importance of the principles of joint work of related specialists at various stages of diagnosis, treatment and subsequent dispensary observation of patients with DSTS, taking into account the polyetiology and multifactorial nature of the pathologies of the genitourinary system caused by DSTS.
 The main diagnostic criteria for collagenopathies are minor anatomical anomalies of development, namely, the presence of three or more minor anatomical anomalies of development indicates a high probability of morphogenesis disorders in the form of congenital malformations of the male genital area. The role of the unfavorable course of the early prenatal period, during which the influence of an unfavorable factor can lead to one or another anomaly, for many organs, including the reproductive organs in men, has been revealed, since they are associated with DSTS pathology and are caused by a wide representation of connective tissue. It also demonstrated a great role in the development of non-hereditary forms of exposure to environmental factors, which makes it possible to make a correction with the help of appropriate recommendations. The data presented in the robot can be considered as a certain stage in the study of DSTS problems and its impact on the male reproductive system.

Connective tissue dysplasia in the genesis of cervical incompetence

Aim: to study a role of undifferentiated connective tissue dysplasia (uCTD) in etiology of cervical incompetence and its effect on pregnancy and childbirth course.Material and Methods. There were enrolled 60 patients with cervical incompetence. Patients were divided into 3 groups according to the modified CTD scale: group 1 - patients without uCTD (n = 21); group 2 - patients with mild uCTD (n = 25); group 3 - patients with moderate-to-severe uCTD (n = 14). Intensity of CTD clinical signs was assessed based on health status, gynecological and obstetric history, the course of pregnancy, labor and the postpartum period.Results. It was found that patients with more prominent CTD developed earlier (r-Spearman = -0.26) and more marked (r-Spearman = -0.29) cervical shortening and opening of the internal orifice of the uterus (r-Spearman = 0.28). It was also noted that likelihood of occurring preterm labor was significantly higher in patients with CTD (p = 0.02).Conclusion. The data obtained evidence that uCTD affects intensity of manifested cervical insufficiency and rate of preterm delivery.

EFFICACY OF PERSONALIZED THERAPY FOR PELVIC ORGAN PROLAPSE DEPENDING ON THE PRESENCE OF UNDIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA

We analyzed treatment outcomes in patients with pelvic organ prolapse (POP), some of whom had signs of undifferentiated connective tissue dysplasia (uCTD). Women from subgroup 1 used a pessary, while women from subgroup 2 underwent electromyostimulation in an outpatient department and performed Kegel exercises. After two treatment courses and pharmacotherapy, all patients demonstrated positive dynamics. Electrostimulation in combination with Kegel exercises was more effective than pessary; the effect was more pronounced in women without uCTD than in patients with uCTD. The results of our study were used for the development of a scale to assess POP severity, which enables a tailored approach to the diagnosis and management of such patients depending on the presence of uCTD.

Systematic analysis of molecular biological mechanisms for supporting connective tissue metabolism with chondroitin sulfate

Objective: to analyze the roles of undifferentiated connective tissue dysplasia (UCTD) in the development of cartilage and ligamentous apparatus diseases.Material and methods. This paper presents the results of analyzing the literature on fundamental and clinical studies of relationships between chondroitin sulfate (CS) and connective tissue (CT) disease. A total of 922 publications on the relationship between CT dysplasia and CS and 2249 publications on CS receptor molecules were analyzed. These arrays of publications were analyzed using topological and metric approaches to data analysis.Results and discussion. The genetic predisposition to UCTD is substantially aggravated by inadequate nutrition that leads to deficiency of certain micronutrients that support CT reconstructive processes. The paper presents the results of a systematic analysis of prospects for the use of drugs based on standardized CS substances in patients with UCTD. CS is a material for CT reconstruction. CS increases the activity of growth factors and reduces CT inflammatory destruction (inhibition of the secretion of histamine, pro-inflammatory chemokines, Toll-like receptors, and the NF-κB cascade through exposure to the CD44 receptor).Conclusion. The pharmacological effects of CS indicate the importance of using standardized CS forms in the treatment of patients with UCTD.

Epidemiology and clinical features of congenital malformations of uri-nary system organs in conditions of environmental distress

Objective. To establish the features of epidemiology and clinical manifestations of congenital malformations of the urinary system in the zone of environmental distress.
 Material and methods. We analyzed the overall prevalence of nephropathies and the proportion of congenital malformations of the urinary system in the dynamics from 2001 to 2019; 697 patients aged 118 years were examined with assessment of clinical and anamnestic, laboratory, instrumental and radiological data in accordance with diagnostic standards and protocols. We studied the features of epidemiology and clinical manifestations of congenital kidney and urinary tract diseases in an area with an unfavorable environmental situation.
 Results. Over the past 2 decades, the general prevalence of nephropathies in the children's population has increased with an elevation in the proportion of congenital malformations of the urinary system. These indicators are significantly higher in an area with a high level of anthropogenic environmental pollution than in an area with a relatively favorable environmental situation. Features of congenital pathology of the kidneys and urinary tract in patients living in an environmentally unfavorable zone were revealed: the presence of mass deficiency, reduced resistance, combination with undifferentiated connective tissue dysplasia syndrome, more frequent attachment of microbial-inflammatory process, decreased concentration and evacuation function of the kidneys.
 Conclusions. These features of epidemiology and clinical manifestations of congenital malformations of the urinary system in patients living in a region with a high level of environmental pollution should be taken into account when carrying out their medical support.

Features of the health status of women with genital prolapse

Introduction. In recent years, more and more attention has been paid to the issues of genital prolapse. This is largely due to the increase in women life expectancy and the need to provide them with a decent quality of life, as prolapse is quite often accompanied by dysfunction of the pelvic organs. In recent years, there has been an increasing amount of literature on undifferentiated connective tissue dysplasia (UCTD). However, most of these studies consider therapeutic or vertebro-neurological problems, while the high prevalence rates of this pathology call for additional research on the pathogenetic mechanisms of the effect of UCTD on pelvic organ prolapse, which will allow us to decide on the choice of subsequent treatment.The purpose and objectives. Asess the effect of undifferentiated connective tissue dysplasia (UCTD) on the formation of disorders of the somatic, gynecological and reproductive health status in women with genital prolapse.Materials and methods. To achieve this goal, we examined 204 women with genital prolapse in the POP-Q classification of stage 2–3, of which 97 were diagnosed with UCTD (the main group), and the remaining 107 patients made up the comparison group.Results. To evaluate the influence of UCTD on the formation of somatic, gynecological and reproductive health disorders in women with genital prolapse. We analyzed anamnestic indications for somatic, gynecological and reproductive pathology of women, as well as assessed the current state of health of patients with genital prolapse. It was found that it is UCTD that determines the development of a particular form of gynecological pathology and affects the formation of genital prolapse.Conclusion. UCTD manifests as various forms of somatic and gynecological health disorders throughout the life of women with genital prolapse.

GENETIC ASPECTS OF CERVICAL INSUFFICIENCY PROBLEM

Cervical insufficiency is the main cause of miscarriage in the second trimester of pregnancy (up to 40 %), and in the third trimester occurs in a one third of women with preterm birth. Diagnosis of this pathology is based on the identification of structural changes in the cervix, which determines the impossibility of predicting this pathology before pregnancy. Current morphological studies have shown that connective tissue predominates in the cervix, represented mainly by collagen, which provides up to 85 % of the weight of the cervical tissue, elastin and proteoglycans. A key factor in the development of CI is currently considered a defect in collagen synthesis and the development of cervical incompetence is considered as a manifestation of undifferentiated connective tissue dysplasia. The results of recent studies show that cervical insufficiency is a genetically determined condition and polymorphisms in genes related to the metabolism of connective tissue can play a role in the development of CI.

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

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.

PECULIARITY OF ADAPTATION OF BABIES ARE BORN PREMATURELY FROM MOTHERS WITH UNDIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA

The aim: To evaluate the peculiarity of clinical manifestations of neonatal respiratory distress syndrome (NRDS) in deeply premature infants from mothers with phenotypic markers of undifferentiated connective tissue dysplasia (UCTD). Materials and methods: The study represent the results of a retrospective clinical and statistical analysis of 268 premature birth report card and newborn report sheet. .The main (1 group) included 50 pregnants with obvious phenotypic markers of UCTD, the comparison group (group 2) consisted of 50 pregnant women without phenotypic markers of UDCTD. Results: According to the study, in 12 (24%) pregnant women of the main group at the time of admission to the clinic had contractions,which required specific therapy. Cervical cerclage was performed in 38 (76%) patients of the main group due to the presence of cervical insufficiency (CI). In these cases, the severity of the CI on the Steinber scale was 7.2 ± 0.4 points in the main group against 4.4 ± 0.2 points in the comparison group (p <0.05). Group I patients were more likely to have complications of labor such as:premature rupture of membranes, uterine contraction abnormalities and fetal distress, which required in most cases cesarean delivery (7% and 2%), respectively (p <0.05). The incidence of neonatal complications requiring respiratory support was 67% in group I and 48% in group II. According to our observations, the clinical manifestations of bronchopulmonary dysplasia were twice as high in infants of the main group (66%) against (44%) of the comparison group (p <0.05). Conclusions:1.Neonatal respiratory distress syndrome in premature infants is more often associated from mothers with UDCTD. 2. The high importance of steroid prophylaxis of NRDS and antioxidant therapy in reducing the frequency of mechanical ventilation and the development of bronchopulmonary pathology, especially in infants from mothers with UDCTD syndrome, has been proven. 3. The possibility of diagnosing disorders of functional maturation of the lungs in the fetal period using a non-invasive method of ultrasonography has been confirmed.

Dynamics of Phenotypic Manifestations of Connective Tissue Dysplasia in Children with Uroandrological Pathology in the Age Aspect

<i>Introduction</i>. A comparative study of the phenotypic manifestations of undifferentiated connective tissue dysplasia in children of various age groups with urological and andrological pathology is carried out in the article. <i>Materials and methods</i>. The study included 214 children aged 1 month to 17 years with clinical manifestations of undifferentiated connective tissue dysplasia, who were treated in the pediatric uroandrological department for congenital malformations and diseases of the genitourinary system. When establishing undifferentiated connective tissue dysplasia, a generally accepted table of external phenotypic markers was used with their scores. <i>Results</i>. In infants, abnormalities of the auricles and skin are most often detected. In pre-school age, craniocephalic abnormalities, pathology of the osteoarticular system, and abnormalities of the auricles were detected in the largest percentage of cases. The study found that an increase in the percentage of detection of ocular abnormalities increases with age. Moreover, 2 or more eye abnormalities were most common in older children. Anomalies of the oral cavity were diagnosed in only a quarter of infants and were manifested in them by a single sign. In older age categories, anomalies of the oral cavity were observed in more than half of patients, and often they were not single. During the examination of senior schoolchildren, it was noted that all children of this age category had some kind of abnormality from the osteoarticular system, and almost always they were multiple. <i>Conclusion</i>. Somatic manifestations of undifferentiated connective tissue dysplasia are often multi-organ in nature and often have nonspecific clinical symptoms. Identification of the patterns of formation of uroandrological pathology in patients of various age groups against the background of connective tissue failure will provide timely prevention and correction of visceral manifestations of dysplasia in children.