Meyer dysplasia in children’s connective-tissue disorders phenotype (the results of observation in the Municipal Children’s Polyclinic №2, Stavropol city)

Aim. The research of the prevalence, nosological structure, clinical presentations, and pathogenetic features of different forms of coxal epiphyseal pathology including Meyer dysplasia in small children based on the results of clinical and instrumental observation on outpatient orthopedic admission. Material and methods. In 12,000 children from 0 to 5 years, an orthopedic examination was conducted with an assessment of external dysmorphogenetic signs of connective tissue dysplasia, an ultrasonic examination of hip joints, an in-depth X-ray examination. The step-by-step examination algorithm was structured and detailed according to the presence and severity of the coxal region pathology, children’s age. Conclusion. The combination of dysplastic phenotype with joints formation inhibition in children aged 1 year is a predictor of Mayer’s dysplasia and must be the reason for a detailed examination of the hip joints with use of visual diagnostic methods.

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Risk factors for venous thrombosis in patients with endoprosthetics of hip joints

Journal of Education, Health and Sport ◽

10.12775/jehs.2021.11.09.102 ◽

2021 ◽

Vol 11 (9) ◽

pp. 875-885

Author(s):

Ihor Venher ◽

Sviatoslav Kostiv ◽

Dymytrii Khvalyboha

Keyword(s):

Connective Tissue ◽

Femoral Neck ◽

Inferior Vena Cava ◽

Venous Thrombosis ◽

Hip Joint ◽

Hip Replacement ◽

Inferior Vena ◽

Vena Cava ◽

Hip Joints ◽

Connective Tissue Dysplasia

Background. Important part of orthopaedic surgery is endoprosthetics of hip joints, which eliminates pain syndrome, restores the amplitude of movements and the support ability of the lower limb. But there is a number of complications; venous thromboembolism among them occupies a leading place. Material and methods. 219 patients with a mean age of 64.7 ± 3.8 years were operated. In 137 (62.1%) observations, total cement hip replacement was performed for osteoarthritis. 82 (37.4%) patients received total and unipolar cement hip replacement for cervical femoral neck fractures. Results. Clinical manifestations of non-specific connective tissue dysplasia were detected in 83 (37.9%) patients, which were confirmed by the laboratory determination of the level of general, bound and free oxyproline. In the postoperative period, the thrombotic process in the venous system of the inferior vena cava was diagnosed in 23 (10.5%) observations. Operative intervention on the hip joint in patients with nonspecific dysplasia of connective tissue in 11 (13.3%) cases was complicated by the development of venous thrombosis. In patients without non-specific connective tissue dysplasia, postoperative thrombosis in the system of the inferior vena cava was diagnosed in 12 (8.8%) observations. Conclusions. Patients with osteoarthrosis of the hip joint and the femoral neck fracture accompanied by the non-specific dysplasia of the connective tissue are characterized by expressed levels of endothelial dysfunction and increased activity of the blood-coagulation system.

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Role of connective tissue dysplasia in obstetrics and gynecology

Kazan medical journal ◽

10.17816/kmj2001 ◽

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.

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NONSPECIFIC DYSPLASIA OF THE CONNECTIVE TISSUE – A FACTOR OF VENOUS THROMBOEMBOLIC COMPLICATIONS IN ENDOPROSTHETICS OF HIP JOINTS

International Journal of Medicine and Medical Research ◽

10.11603/ijmmr.2413-6077.2020.1.11047 ◽

2020 ◽

Vol 6 (1) ◽

pp. 35-42

Author(s):

I. K. Venher ◽

N. I. Herasymiuk ◽

S. Ya. Kostiv ◽

I. I. Loyko ◽

D. V. Khvalyboha

Keyword(s):

Endothelial Dysfunction ◽

Connective Tissue ◽

Femoral Neck ◽

Hip Joint ◽

Hip Replacement ◽

Inferior Vena ◽

Vena Cava ◽

Hip Joints ◽

Neck Fracture ◽

Connective Tissue Dysplasia

Background. Important part of orthopedic surgery is endoprosthetics of hip joints, which eliminates pain syndrome, restores the amplitude of movement and the support ability of lower limbs. But some complications usually take place; venous thromboembolism is leading among them. Objective. The aim of the study was to investigate endothelial dysfunction and activity of the hemocoagulation system at different levels of VTEC risks and thus to work out the strategy of thromboprophylaxis in patients with osteoarthrosis of the hip joint and femoral neck fracture combined with non-specific dysplasia of the connective tissue. Methods. 219 patients of a mean age of 64.7±3.8 years old underwent surgery. In 137 (62.1%) cases, a total cement hip replacement was performed for osteoarthritis. 82 (37.4%) patients underwent total and unipolar cement hip replacement for cervical femoral neck fractures. Results. Clinical manifestations of non-specific connective tissue dysplasia were detected in 83 (37.9%) patients that was confirmed by the laboratory determination of the level of general, bound and free oxyproline. In the postoperative period, the thrombotic process in the venous system of the inferior vena cava was diagnosed in 23 (10.5%) cases. The level of indicators of endothelium status dysfunction was much more significant in the patients in cases of nonspecific dysplasia of connective tissue. Operative intervention on the hip joint in the patients with nonspecific dysplasia of connective tissue in 11 (13.3%) cases was complicated by development of venous thrombosis. In the patients without non-specific connective tissue dysplasia, postoperative thrombosis in the system of the inferior vena cava was diagnosed in 12 (8.8%) cases. Conclusions. Patients with osteoarthrosis of the hip joint and the femoral neck fracture accompanied by the non-specific dysplasia of the connective tissue are characterized by high levels of endothelial dysfunction and increased activity of the blood-coagulation system.

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Minor anomalies of heart as a manifestation of connective tissue dysplasia: modern diagnostic methods

Practical medicine ◽

10.32000/2072-1757-2019-2-28-31 ◽

2019 ◽

Vol 17 (2) ◽

pp. 28-31

Author(s):

M. Yu. Smetanin ◽

◽

S. Yu. Nurgalievsa ◽

N. Yu. Kononova ◽

L. T. Pimenov ◽

...

Keyword(s):

Connective Tissue ◽

Diagnostic Methods ◽

Connective Tissue Dysplasia ◽

Minor Anomalies

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Imaging Diagnosis of Lateral Meningocele Syndrome

Neurographics ◽

10.3174/ng.2000027 ◽

2021 ◽

Vol 11 (2) ◽

pp. 66-71

Author(s):

S. Raichandani ◽

D. Van Tassel ◽

K. Wong

Keyword(s):

Connective Tissue ◽

Clinical Management ◽

Genetic Disorder ◽

Facial Features ◽

Imaging Features ◽

Imaging Diagnosis ◽

Connective Tissue Disorders ◽

Multisystem Involvement ◽

Clinical Presentations ◽

Urogenital Anomalies

Lateral meningocele syndrome, also known as Lehman syndrome, is an exceptionally uncommon genetic disorder, which is characterized by specific facial features and multisystem involvement, including skeletal, cardiac, and urogenital anomalies, akin to other connective tissue disorders, but it is set apart by the unique occurrence of multiple lateral meningoceles. Knowledge of the distinctive imaging features can strongly suggest the diagnosis in patients with complex clinical presentations to assist in the guidance of appropriate and timely clinical management.

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Improving diagnostics of periodontal diseases in children with connective tissue dysplasia based on X-ray morphometric and densitometric data

Periodontology ◽

10.33925/1683-3759-2020-25-4-266-275 ◽

2020 ◽

Vol 25 (4) ◽

pp. 266-275

Author(s):

B. N. Davydov ◽

D. A. Domenyuk ◽

S. V. Dmitrienko ◽

T. A. Kondratyeva ◽

Yu. S. Harutyunyan

Keyword(s):

Connective Tissue ◽

Bone Tissue ◽

Bone Structure ◽

Periodontal Diseases ◽

Healthy Children ◽

Strong Positive Correlation ◽

X Ray ◽

Lower Jaw ◽

Connective Tissue Dysplasia ◽

Peripheral Skeleton

Relevance. Detection of maxillofacial pathomorphological changes in children with connective tissue dysplasia (CTD) allows identifying the nature and severity of disorders, as well as it serves the basis for selecting the best treatment options in view of the pathogenetic features. The purpose is to improve the diagnosis of periodontal diseases in children with CTD based on x-ray morphometric indices of the lower jaw and peripheral skeleton ultrasound osteodensitometry.Materials and methods. 92 children with varying CTD severity, and 43 healthy children, underwent cone-beam computed tomogram examination with a further analysis of their X-ray morphometric (quantitative, qualitative) values and the lower jaw optical density indices. Besides, the status of the peripheral skeleton bone tissue was also determined through quantitative ultrasonic densitometry.Results. Quantitative X-ray morphometric indices in healthy children and children with CTD revealed strong positive correlation with the Z-criterion of osteodensitometry, offering an objective reflection of the bone tissue status in the peripheral skeleton.Conclusion. CTD progression in children correlates with the bone structure destruction intensity in the maxillofacial area, an increase in chronic productive inflammation, a decrease in the bone density, bone tissue fibrous transformation, a decrease in the thickness of cortical, and fiber-dissociation in closing, plates, of the lower jaw, prevalence of mid- and fine-meshed bone pattern, disturbed spatial orientation and thinning of bone trabeculae, as well as the development of pathologies in the periodontium.

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PECULIARITIES OF MORPHOLOGOCAL AND FUNCTIONAL INDICES YOUNG MEN'S CARDIOVASCULAR SYSTEM WITH CONNECTIVE TISSUE DYSPLASIA SYMPTOMS

Vestnik of Russian military medical Academy ◽

10.17816/brmma12161 ◽

2017 ◽

Vol 19 (1) ◽

pp. 25-31

Author(s):

Semen V Efimov ◽

Dmitriy V Cherkashin ◽

Viktor P Andrianov ◽

Andrey E Alanichev ◽

Gennadiy G Kutelev ◽

...

Keyword(s):

Connective Tissue ◽

Cardiovascular System ◽

Healthy Person ◽

Connective Tissue Disorders ◽

Functional Parameters ◽

Connective Tissue Dysplasia ◽

Functional Indices ◽

All Diseases

Nowadays there is no uniform classification and methods of diagnosis of the dysplasia presence in the examined human connective tissue, however it is known that all diseases are depending especially on the severity of the connective tissue disorders. By itself, the connective tissue dysplasia, affecting all systems and organs in the process of embryogenesis, is manifested mainly in the bodies of one of the systems. So far, the cardiovascular system has been studied the most fully. It is considered as an obligatory element of adaptation to the environment, testing the most expressed load at any activity and pathology. The aim of the study was to determine peculiarities of morpho-functional parameters of a healthy person activity with a minimum of the severity of the symptoms of cardiovascular system connective tissue dysplasia, and also to determine quantitative criteria for the evaluation of DST (6 tables, bibliography: 17 refs).

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Treatment of an asymptomatic penetrating aortic ulcer in a young patient

VASA ◽

10.1024/0301-1526/a000024 ◽

2010 ◽

Vol 39 (2) ◽

pp. 175-179

Author(s):

Hakimi ◽

Geisbüsch ◽

Gross ◽

Hyhlik-Dürr ◽

Hausser ◽

...

Keyword(s):

Electron Microscopy ◽

Connective Tissue ◽

Aortic Arch ◽

Open Surgery ◽

Open Repair ◽

Thoracic Endovascular Aortic Repair ◽

Concomitant Disease ◽

Connective Tissue Disorders ◽

Penetrating Aortic Ulcer ◽

Pathological Analysis

We want to report and discuss the indication for open surgery for an asymptomatic penetrating aortic ulcer (PAU) in the era of thoracic endovascular aortic repair (TEVAR). A 31-year-old female presented with the diagnosis of an aneurysm in the distal aortic arch. With respect to the patients young age, the controversial status of connective tissue disorders and in the absence of concomitant disease, open repair was indicated. There was no proof of a mycotic plaque or connective tissue disease in the microbiological-, pathological analysis and at electron-microscopy. The patient was discharged on the thirteenth postoperative day. In spite of good preliminary results of TEVAR in PAU, in selective cases there is still an indication for open surgery.

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