scholarly journals Role of connective tissue dysplasia in obstetrics and gynecology

2014 ◽  
Vol 95 (6) ◽  
pp. 897-904
Author(s):  
A S Gasparov ◽  
E D Dubinskaya ◽  
I A Babicheva ◽  
N V Lapteva ◽  
M F Dorfman
Keyword(s):  
Connective Tissue ◽  
Musculoskeletal Diseases ◽  
Molecular Genetic ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Diagnostic Methods ◽  
Obstetrics And Gynecology ◽  
Special Importance ◽  
Life Threatening ◽  
Connective Tissue Dysplasia

A review of literature on the problem of connective tissue dysplasia in obstetric and gynecological practice is presented. The questions of terminology, classification, clinical manifestations and diagnosis are surveyed. Currently, many experts note the change of the classical clinical course of a significant number of internal diseases, increased prevalence of allergic and autoimmune diseases. Since the 90s of the last century, connective tissue dysplasia is considered one of the main reasons of the above mentioned conditions. Connective tissue dysplasia is the malformation, which is the basis of a significant number of internal diseases, with diverse symptoms and no clear diagnostic criteria. Researches on connective tissue dysplasia are mainly focused on cardiologic and pulmonary diseases, musculoskeletal diseases, and autoimmune processes. Researches addressing connective tissue diseases in a number of gynecological diseases and conditions in obstetrics appeared only recently. The presence of connective tissue dysplasia in women is a major problem in obstetrics and gynecology. Most reliable biochemical and molecular genetic studies are currently unavailable to a practicing doctor due to technical difficulties and considerable cost. So clinical-genealogical method of examination of patients and their families, as well as the widespread use of instrumental diagnostic methods are of special importance. Obstetricians and gynecologists while managing such patients should pay close attention to the phenotypic features of connective tissue dysplasia and carefully evaluate the cardiovascular and pulmonary systems, blood coagulation to avoid possible serious, often life-threatening, complications.

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

2021 ◽  
Vol 74 (10) ◽  
pp. 2566-2568
Author(s):  
Tunzala V. Ibadova ◽  
Vitalii V. Maliar ◽  
Volodymyr V. Maliar ◽  
Vasyl V. Maliar
Keyword(s):  
Connective Tissue ◽  
Premature Infants ◽  
Distress Syndrome ◽  
Comparison Group ◽  
Clinical Manifestations ◽  
Main Group ◽  
Phenotypic Markers ◽  
Group I ◽  
Neonatal Respiratory Distress ◽  
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.

scholarly journals Diagnostic difficulties and features of endovideosurgical treatment of a patient with mucocele of the appendix

2020 ◽  
Vol 12 (2) ◽  
pp. 85-90
Author(s):  
Badri V. Sigua ◽  
Vyacheslav P. Zemlyanoy ◽  
Elguja L. Lataria ◽  
Alexey A. Kurkov ◽  
Vyacheslav A. Melnikov ◽  
...  
Keyword(s):  
Benign Tumor ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Vermiform Appendix ◽  
Diagnostic Methods ◽  
Number Of Patients ◽  
Mucocele Of The Appendix ◽  
Diagnostic Difficulties ◽  
Life Threatening ◽  
The Right

The mucocele of the appendix is the expansion of the appendix with the accumulation of a large amount of mucus. The mechanism and causes of mucocele are not fully understood. According to some authors, such changes in the appendix can occur due to cicatricial narrowing of the lumen of the appendix, compression or blockage of its base. Other authors believe that the mucocele of the appendix is a benign tumor that develops from the remnants of primitive mesenchyme and is sometimes prone to malignancy. Clinical manifestations of mucocele of the appendix are nonspecific. In a number of patients, this disease causes pain in the right abdomen, more often pulling, intermittent. However, the disease is often asymptomatic. In this regard, diagnosis is established only during performing an operation, most often, regarding acute appendicitis. Nevertheless, instrumental diagnostic methods such as ultrasound and computed tomography of the abdominal and pelvic organs make it possible to suspect mucocele. Despite the frequent asymptomatic, non-aggressive course, a number of life-threatening complications can become the outcome of the mucocele of the vermiform appendix. The most formidable complication is the rupture of the appendix with mucus entering free abdominal cavity, followed by the development of peritoneal pseudomyxoma due to implantation of mucus-forming cells. The only option for radical treatment of the mucocele of the appendix is a surgical intervention. A presented clinical case demonstrates the difficulties of diagnosis, as well as the features of surgical treatment of a patient with a mucocele of the appendix.

scholarly journals Clinical manifestations of connective tissue systemic injury in women of early reproductive age having small pelvis varicosis

2006 ◽  
Vol 5 (1) ◽  
pp. 87-90
Author(s):  
V. G. Mozes ◽  
K. B. Mozes
Keyword(s):  
Connective Tissue ◽  
Clinical Examination ◽  
Clinical Manifestations ◽  
Sialic Acids ◽  
Reproductive Age ◽  
Severe Dysplasia ◽  
Study Results ◽  
Connective Tissue Dysplasia ◽  
Tissue Metabolites

The aim of the study was to define manifestations of non-differentiate forms of connective tissue dysplasia in women of early reproductive age having small pelvis varicosis. The study results showed that patients having small pelvis varicosis revealed more often phenotype manifestations of non-differentiate forms which were seen in clinical examination. Increased level of serum sialic acids and increasing excretion of connective tissue metabolites (free oxyproline and glycosaminoglycanes) with urine were consequence of connective tissue systemic injury in these patients which is in accordance with data of non-severe dysplasia process. The study performed allowed to conclude that the small pelvis varicosis in women of early reproductive age is a manifestation of systemic injury of connective tissue.

scholarly journals CARDIAL MANIFESTATIONS OF NON-DIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA AND THEIR INFLUENCE ON PREGNANCY

2021 ◽  
pp. 14-22
Author(s):  
V. Yu. DOBRIANSKA ◽  
S. M. HERYAK ◽  
L. M. MALANCHUK ◽  
M. I. SHVED ◽  
I. V. KORDA
Keyword(s):  
Mitral Valve ◽  
Connective Tissue ◽  
Ventricular Arrhythmia ◽  
Pregnant Women ◽  
Mitral Valve Prolapse ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Adequate Treatment ◽  
Pregnancy And Childbirth ◽  
Connective Tissue Dysplasia

Among the visceral manifestations of NDST in pregnant women most often diagnosed mitral valve prolapse (20-25%) that accompanied by more cardiovascular and obstetric complications during pregnancy. It demonstrates the high clinical significance of the problem of connective tissue dysplasia with mitral valve prolaps for pregnancy and requires adequate treatment programs for prevention of complications and management of pregnant women with connective tissue dysplasia. Aim. Determination of the frequency of pregnancy pathology in women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia. Materials and methods. 138 pregnant women with MVP and concomitant signs of NDST and 54 healthy pregnant women were selected for analysis. Clinical manifestations of NDST, different variants of arrhythmias and the total number of complications of pregnancy and childbirth were evaluated. Results. In pregnant women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia, cases of frequent sinus extrasystole were significantly more often compared to frequent ventricular arrhythmia (47.8% vs. 18.1%, p<0.001) and cases of combination of frequent sinus extrasystole and ventricular arrhythmia (13.3 % vs. 1.5%, p <0.05). They significantly more often identified both symptoms of arrhythmological nature and symptoms that indicated a violation of autonomic status. The presence of NDST syndrome is more often accompanied by the development of complications of pregnancy and childbirth. These pregnant women have genetic and phenotypic risk factors for the development of pathological pregnancy and childbirth, birth trauma, disability of mother and newborn, which justifies such patients in a separate risk group for individualized programs of the prevention and treatment of visceral (cardiac) manifestations of NDST and possible complications of pregnancy and childbirth. Conclusions. 3.1% of pregnant women are diagnosed with phenotypic signs (stigma) of undifferentiated connective tissue dysplasia, and the most common visceral cardiac manifestation is mitral valve prolapse. The presence of mitral valve prolapse and extrasystolic arrhythmia in pregnant women with NDST is accompanied by significantly more frequent development of pregnancy and childbirth complications in these patients.

Abstract 4535: Therapeutic Angiogenesis by Mononuclear Cell Implantation for Critical Digit Ischemia of Patients with Connective Tissue Diseases

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Masafumi Takahashi ◽  
Yoshiaki Ishigatsubo ◽  
Kazuteru Fujimoto ◽  
Masaaki Miyamoto ◽  
Seiji Minota ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Connective Tissue ◽  
Peripheral Blood ◽  
Mononuclear Cells ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Therapeutic Angiogenesis ◽  
Diffuse Fibrosis ◽  
Dramatic Improvement ◽  
Skin Ulcers

Systemic sclerosis (SSc or scleroderma) is an autoimmune connective tissue disease characterized by diffuse fibrosis, degenerative changes, and microvascular abnormalities. The vasculopathy mainly affects small arteries and capillaries and causes insufficient blood flow, which leads to clinical manifestations, such as Raynaud’s syndrome, fingertip ulcers, and gangrene. Recently, implantation of bone marrow-derived mononuclear cells (MNCs) has been successfully used for therapeutic neovascularization in Buerger’s disease that is thought to be an “autoimmune” vasculitis. The purpose of this study is to determine the efficacy of autologous MNC implantation into the ischemic digits of patients with connective tissue diseases. This study was performed as a prospective, non-randamized, and multicenter clinical trial. Thirty-four patients (19 SSc, 5 SLE, 2 CREST, 2 MCTD, 4 APS, 2 PN) who had painful ischemic digits with skin ulcers were enrolled in this study. Autologous MNCs obtained from bone marrow or peripheral blood were implanted into the ischemic digits. Ischemic pain and ulcers improved remarkably after MNC implantation. In particular, SSc patients showed dramatic improvement of these parameters (18 of 19 patients, 94.7%). In patients with other types of connective tissue diseases, pain and ulcers improved in 12 of 15 patients (80.0%). No serious adverse event was observed. These results demonstrate that implantation of autologous MNCs from bone marrow or peripheral blood into ischemic digits is feasible, safe and effective for improvement of pain and skin ulcers in patients with connective tissue diseases including SSc. Thus, larger, randomized and controlled trials for this cell therapy in patients with connective tissue diseases will be warranted.

THE IMPORTANCE OF ANTIPHOSPHOLIPID SYNDROME IN DEVELOPED CEREBROVASCULAR DISEASES ON THE BACKGROUND OF SYSTEMATIC CONNECTIVE TISSUE DISEASES

2021 ◽  
Vol 2 (1) ◽  
pp. 9-13
Author(s):  
O. Ya. Bustanov ◽  
◽  
Yu. N. Madjidova ◽  
N. A. Nasirdinova ◽  
O. B. Kuchkarova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Early Diagnosis ◽  
Antiphospholipid Syndrome ◽  
Rheumatic Diseases ◽  
Cerebrovascular Disorders ◽  
Connective Tissue Diseases ◽  
Modern Medicine ◽  
Cerebrovascular Diseases ◽  
Diagnostic Methods

The article developed criteria for early diagnosis of cerebrovascular disorders, one of the complications of antiphospholipid syndrome in patients with systemic connective tissue, and proposed diagnostic methods to prevent this process’s exacerbationand developsevere irreversible complications. This will help stop the development of antiphospholipid syndrome from the urgent problems of modern medicine and manage the effectiveness of treatmentand improve patients’quality of life.Keywords:cerebrovascular pathology, rheumatic diseases, antiphospholipid syndrome, anticardiolipid antibodies

scholarly journals Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey

RMD Open ◽  
2020 ◽  
Vol 6 (2) ◽  
pp. e001344
Author(s):  
Rosaria Talarico ◽  
Diana Marinello ◽  
Stefano Bombardieri ◽  
Gerd Burmester ◽  
Joao Fonseca ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Connective Tissue ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Healthcare Professionals ◽  
Musculoskeletal Diseases ◽  
Family Members ◽  
Disease Patient ◽  
Connective Tissue Diseases ◽  
Reference Network

IntroductionThe European Reference Network (ERN) ReCONNET is the ERN aimed at improving the management of rare and complex connective tissue and musculoskeletal diseases (rCTDs) across the European Union (EU). In the mission of ERN ReCONNET, clinical practice guidelines (CPGs) play a crucial role, representing a valid tool towards the harmonisation of the management of rCTDs while improving effectiveness and quality of care delivered to patients.MethodsERN ReCONNET developed two surveys to map the adherence to rCTDs CPGs among healthcare providers and to assess the knowledge and awareness of CPGs for their diseases among patients, family members and caregivers.ResultsThe results of the surveys highlighted that healthcare professionals find it useful to apply CPGs in clinical practice (93%), while 62% of them experience difficulties and barriers in the application in their centres. Healthcare professionals also highlighted the need to develop CPGs for all rCTDs and to implement the use of the existing CPGs in clinical practice. On the other hand, patients, families and caregivers are relatively aware of the purpose of CPGs (51%) and 62% of them were aware of the existence of CPGs for their disease. Patient-friendly versions of CPGs and patients’ lifestyle guidelines should be systematically developed contributing to the empowerment of patients in the disease management.ConclusionERN ReCONNET is addressing the main issues identified in the results of the survey, promoting practical actions for the local adaptation of CPGs across Europe, improving their routine clinical use and increasing the awareness on CPGs among rCTDs patients, family members and caregivers.

Vasculitis

2018 ◽  
Author(s):  
Raashid Luqmani
Keyword(s):  
Connective Tissue ◽  
Blood Vessel ◽  
Blood Vessels ◽  
Vessel Wall ◽  
Connective Tissue Diseases ◽  
Blood Vessel Wall ◽  
Target Organs ◽  
Laboratory Features ◽  
Life Threatening ◽  
Pulmonary Capillaritis

The vasculitides are a heterogeneous group of disorders that can range from mild inflammation of blood vessels in the skin, to organ- and life-threatening diseases. The term ‘vasculitis’ is a pathological description of blood vessel wall inflammation which leads to ischaemia and infarction of the target organs. Definitions and classifications of the primary vasculitides are mainly based on the predominant calibre of the blood vessels involved but incorporate clinical, pathological, and laboratory features. The secondary vasculitides usually occur in the context of other connective tissue diseases and are not discussed further in this section. Goodpasture’s disease is not usually included in the primary vasculitides, but has compatible clinical features of pulmonary capillaritis and glomerulonephritis.

scholarly journals Pulmonary Hypertension Associated With Scleroderma and Connective Tissue Disease: Potential Molecular and Cellular Targets

2017 ◽  
Vol 16 (2) ◽  
pp. 61-67
Author(s):  
Maria Trojanowska
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Molecular Mechanisms ◽  
Critical Role ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Response To Treatment ◽  
Organ Systems

Systemic sclerosis (SSc) is characterized by autoimmunity, small-vessel vasculopathy, and fibrosis causing damage in multiple organ systems. Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of SSc, occurring in patients with the limited (lcSSc) and diffuse (dcSSc) forms of the disease and affecting 8% to 15% of patients.12 While pulmonary hypertension associated with connective tissue disease (CTD-PAH) has similar clinical features as idiopathic PAH, 1-year survival and freedom from hospitalization are lower in CTD-PAH.3 SSc-PAH has the worst 1-year survival rate at 82% compared with other connective tissue diseases, including systemic lupus erythematosus, mixed connective tissue disease, and rheumatoid arthritis.34 Despite the recent progress in the development of disease-targeted therapies, patients with SSc-PAH have a poorer response to treatment and a worse prognosis than other subgroups of PAH.1 Autoimmunity and prolonged vasculopathy preceding the development of clinical manifestations of SSc-PAH may play a critical role in the poorer outcome of SSc-PAH patients.1 This article will provide an overview of the recent findings related to cellular and molecular mechanisms associated with the development of PAH, with an emphasis on SSc-PAH.

scholarly journals Difficulties in the Differential Diagnosis of Tracheobronchomegaly in Children

2020 ◽  
Vol 101 (3) ◽  
pp. 175-182
Author(s):  
N. A. Il’ina ◽  
L. E. Vorob’eva ◽  
M. D. Bakradze ◽  
A. S. Polyakova
Keyword(s):  
Cystic Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Tracheobronchial Tree ◽  
Developmental Defect ◽  
Upper Respiratory Tract ◽  
Upper Respiratory

Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.

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