scholarly journals CLINICAL AND ANAMNESTIC FEATURES OF THE COURSE OF ACUTE BRONCHITIS IN CHILDREN

2018 ◽  
Vol 5 (3) ◽  
pp. 143-147
Author(s):  
M. Strelkova ◽  
G. Senatorova
Keyword(s):  
Connective Tissue ◽  
Clinical Manifestations ◽  
Significant Risk ◽  
Acute Bronchitis ◽  
General Information ◽  
Duration Of Symptoms ◽  
History Of ◽  
Connective Tissue Dysplasia ◽  
Dynamic Examination ◽  
Severity Of The Disease

CLINICAL AND ANAMNESTIC FEATURES OF THE COURSE OF ACUTE BRONCHITIS IN CHILDRENStrelkova M., Senatorova G. The purpose of the study wasto detect clinical and anamnestic features of the course of acute obstructive bronchitis in children with a background of undifferentiated connective tissue dysplasia. Dynamic examination of 42 children with acute obstructive bronchitis was performed. The registration card for all children was developed and filled, it consisted of several sections: general information, family history, allergic history, life and past medical history, phenotypic assessment of undifferentiated connective tissue dysplasia. The results indicate that compromised allergic and genealogical history, as well as burdened obstetric history of the mother (threatened preterm delivery and gestosis) is significant risk factors. Assessment of clinical manifestations of acute obstructive bronchitis in children showed that the severity of the disease, which is determined by the degree of severity and duration of symptoms, was associated with the number of signs ofUCTD.Keywords: acute obstructive bronchitis, children, undifferentiated connective tissue dysplasia, wheezing, phenotypic assessment. РезюмеКЛИНИЧЕСКИЕ И АНАМНЕСТИЧЕСКИЕ ОСОБЕННОСТИ ТЕЧЕНИЯ ОСТРОГО БРОНХИТА У ДЕТЕЙСтрелкова М., Сенаторова Г.Метою дослідження було виявити клінічні та анамнестичні особливості перебігу гострого обструктивного бронхіту (ГОБ) у дітей на тлі недиференційованої дисплазії сполучної тканини. Проведено динамічне обстеження 42 дітей з гострим обструктивним бронхітом. Розроблено і заповнено реєстраційну картку для всіх дітей, вона складалася з кількох розділів: загальна інформація, сімейний анамнез, алергологічний анамнез, анамнез життя та хвороби дитини, фенотипічні оцінки недиференційованої дисплазії сполучної тканини. Результати свідчать про наявність факторів ризику - наявність обтяженого алергологічного та генеалогічного анамнезу, а також обтяжений акушерський анамнез матері (наявність загрози передчасних пологів та гестозів). При аналізі клінічних проявів ГОБ у дітей було встановлено, що ступінь тяжкості та тривалості симптомів захворювання була пов'язана з кількістю ознак недиференційованої дисплазії сполучної тканини.Ключові слова: гострий обструктивний бронхіт, діти, недиференційована дисплазія сполучної тканини, фенотипічні ознаки. Резюме.КЛИНИЧЕСКИЕ И АНАМНЕСТИЧЕСКИЕ ОСОБЕННОСТИ ТЕЧЕНИЯ ОСТРОГО БРОНХИТА У ДЕТЕЙСтрелкова М., Сенаторова Г.Целью исследования было выявить клинические и анамнестические особенности течения острого обструктивного бронхита у детей на фоне недифференцированной дисплазии соединительной ткани. Проведено динамическое обследование 42 детей с острым обструктивным бронхитом (ООБ). Разработаны и заполнены регистрационную карточку для всех детей, она состояла из нескольких разделов: общая информация, семейный анамнез, аллергологический анамнез, анамнез жизни и болезни ребенка, фенотипическая оценка признаков недифференцированнойдисплазиисоединительной ткани. Результаты свидетельствуют о наличии факторов риска - наличие отягощенного аллергологического и генеалогического анамнеза, а также отягощенный акушерский анамнез матери (наличие угрозы преждевременных родов и гестозов). При анализе клинических проявлений ГОБ у детей было установлено, что степень тяжести и продолжительности симптомов заболевания была связана с количеством признаков признаков недифференцированнойдисплазиисоединительной ткани.Ключевые слова: острый обструктивный бронхит, дети, недифференцированная дисплазия соединительной ткани, фенотипические признаки.

scholarly journals THE ROLE OF CONNECTIVE TISSUE DYSPLASIA IN CHILDREN’S CYSTIC FIBROSIS. CLINICAL AND GENETIC ASPECTS

2018 ◽  
Vol 63 (5) ◽  
pp. 20-28
Author(s):  
A. V. Goryainova ◽  
P. V. Shumilov ◽  
N. Yu. Kashirskaya ◽  
S. Yu. Semykin
Keyword(s):  
Cystic Fibrosis ◽  
Liver Fibrosis ◽  
Connective Tissue ◽  
Autosomal Recessive ◽  
Autosomal Recessive Disease ◽  
Clinical Manifestations ◽  
History Of ◽  
Connective Tissue Dysplasia ◽  
Severe Fibrosis

The article considers the issue of cystic fibrosis – a monogenic autosomal recessive disease. It describes the history of the CFTR gene discovery, the further search for modifier genes to explain the variability of the clinical manifestations of cystic fibrosis. The review discusses problems of connective tissue dysplasia and somatic pathology, which is formed due to the connective tissue dysmorphogenesis in patients with cystic fibrosis; and also the article contains justification for the connection between the formation of severe fibrosis of the lungs and liver and the presence of clinical and genetic markers of connective tissue dysplasia. The author assumes that the clinical and genetic polymorphisms of connective tissue influence the course of cystic fibrosis, formation of bronchiectasis, interstitial pneumofibrosis, cystic fibrosis dysplasia, liver fibrosis and cirrhosis.

Etiology and clinical features of epididymo-orchitis: A single-center study in Tehran, Iran

2020 ◽  
Vol 20 ◽  
Author(s):  
Sara Abolghasemi ◽  
Mohammad Alizadeh ◽  
Ali Hashemi ◽  
Shabnam Tehrani
Keyword(s):  
Chlamydia Trachomatis ◽  
Clinical Symptoms ◽  
Urine Culture ◽  
Clinical Manifestations ◽  
Urinary Frequency ◽  
Serological Tests ◽  
Duration Of Symptoms ◽  
Two Samples ◽  
History Of ◽  
Tract Infections

Introduction: Epididymo-orchitis is a common urological disease among men. Little is known about the clinical and epidemiological aspects of the disease in Iran. Thus, the present study was aimed to investigate the etiology, clinical sequelae and risk factors of patients with epididymo-orchitis in Tehran, Iran. Materials and Methods: Patients presenting with epididymo-orchitis were prospectively analyzed in order to study the etiology and pattern of the disease. Bacteriological, molecular and serological tests were undertaken to look for Chlamydia trachomatis, Neisseria gonorrhoeae, Brucella spp., Mycoplasma spp, and other bacteria. Results: Fifty patients with epididymo-orchitis were evaluated according to their clinical symptoms, duration of symptoms, physical examination, and laboratory studies. The mean age of the patients was 53 years. Fever, dysuria, pain in the flanks, urinary frequency and discharges occurred in 58.0%, 50.0%, 50.0%, 28.0% and 6.0%, respectively. Bacterial pathogen was identified in 26% (13/50) of patients by urine culture. Escherichia coli was the etiological agent in 11/13 patients (84.6%). Two out of 50 patients (4.0%) were also positive for Chlamydia trachomatis. Two samples were serologically positive for Brucella spp. High Mean age, fever, urinary frequency, history of the underlying disease and history of urinary tract infections were found to have a significant association with the positive bacteriologic urine culture (P<0.05). Conclusions: The most common clinical manifestations were fever, dysuria, and abdominal pain. E. coli and C. trachomatis were the major causative agents. Use of a set of diagnostic approaches including clinical symptoms, urine culture and more precise techniques such as PCR should be taken into consideration for the definitive diagnosis.

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 &#177; 0.4 points in the main group against 4.4 &#177; 0.2 points in the comparison group (p &#60;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 &#60;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 &#60;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 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.

scholarly journals 14. A severe case of connective tissue disease related interstitial lung disease in a young woman

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Kate Grant ◽  
Patrick McShane ◽  
Kathryn Kerr ◽  
Martin Kelly ◽  
Philip Gardiner ◽  
...  
Keyword(s):  
Weight Loss ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Biopsy ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Connective Tissue Disorder ◽  
Young Age ◽  
History Of

Abstract Introduction Interstitial lung disease (ILD) can occur in any connective tissue disease, with varying degrees of respiratory clinical manifestations. In the majority of cases, patients have an established connective tissue diagnosis that precedes the development of ILD by many years. This discussion will focus on the unusual presentation of an 18 year old female admitted with a short history of weight loss and breathlessness. Investigations showed extensive established ILD with strongly positive autoantibodies, but in the absence of clinical signs of an underlying connective tissue disorder apart from Raynaud’s phenomenon. Case description 18-year-old female presented with a three-month history of unintentional 25kg weight loss, six weeks of fatigue/malaise, and a two-week history of worsening breathlessness. She was a student, non-smoker, with no past medical history except for class I obesity, and not on regular medications. On examination she had fine bibasal end-inspiratory crackles, SaO2 96% RA and Raynaud’s phenomenon was observed. Her CXR demonstrated bibasal consolidation. CT imaging identified bilateral symmetrical peripheral patchy ground glass opacities and patchy consolidation with basal predominance. Bloods revealed rheumatoid factor 491.2, anti-RNP A ab 7.91, anti-Sm ab > 8 and anti-chromatin ab 7.3, speckled ANA positive titre of 40, Complement C4 0.08, ESR 29 and HIV negative. Pulmonary function tests demonstrated a restrictive pattern FEV1 2.08L (72%), FVC 2.43L (73%), Ratio 85% and reduced transfer factor - DLCO 41%, KCO 61%. Ambulatory oxygen assessment showed desaturation to 77% RA. Bronchoscopy revealed inflamed airways and a bronchoalveolar lavage (BAL) cell count of 0.6 x 106 - 42% macrophages, 32% neutrophils, 24% eosinophils, 2% lymphocytes. At the local ILD MDT a differential diagnosis of LIP or NSIP was considered. Following discussion with rheumatology she was referred to the thoracic surgical team for lung biopsy. She proceeded to surgical biopsy of her right lung without complication. Unfortunately, she continued to experience worsening breathlessness and myalgia and she was commenced on prednisolone (40mg), with some radiological improvement but no symptomatic benefit. The pathology from her lung biopsy demonstrated significant fibrosis with scattered lymphoid aggregates, microscopic honeycombing with multiple fibroblastic foci and diffuse changes, in keeping with a fibrotic NSIP pattern. Her case was discussed at Freeman Hospital Newcastle ILD MDT who advised that her presentation was in keeping with a mixed connective tissue/lupus-related NSIP, and suggested commencing methylprednisolone, cyclophosphamide and rituximab. Discussion On initial assessment, the patient’s age and symptoms of rapid weight loss and profound exertional dyspnoea were concerning. Her resting oxygen saturations were satisfactory, but she became markedly hypoxic on ambulating short distances, indicating serious respiratory pathology. The initial CXR showed ‘faint patchy consolidation’, but CT scan showed extensive interstitial changes, accounting for her dyspnoea and desaturation on exertion. Further investigations including rheumatoid factor, anti-RNP and anti-Sm antibody were found to be strongly positive, suggesting an underlying mixed connective tissue disorder. However, the patient did not complain of any symptoms related to arthritis, SLE, systemic sclerosis or polymyositis and no positive clinical findings were noted on examination in support of these diagnoses. The BAL analysis was consistent with CT-ILD but not specific enough for diagnosis. A lung biopsy was performed on advice of the ILD lung MDT as the abnormalities on CT imaging could be in keeping with several pathologies with very different associated prognosis and management. The biopsy appearance correlated poorly with the cell count in BAL fluid. Discussion at local and regional ILD MDTs was particularly helpful given the severity of ILD and her young age. The ILD MDT provided a consensus of expert advice on optimal management and confirmed our concern about the extent of established fibrosis and the need for aggressive management. This obviously has significant implications for the patient in many ways, but particularly regarding fertility given her young age and she was therefore referred to the regional fertility clinic for counselling. Key learning points This was a particularly unusual case because the patient presented acutely at a very young age with established fibrotic damage on lung biopsy. It is also noteworthy that she presented so acutely with advanced ILD even though there were no positive clinical signs on examination, and no symptoms or signs of an underlying connective tissue disease. Lung biopsy is not routinely indicated in patients with progressive (respiratory) clinical manifestations of CT ILD, particularly in patients with an established diagnosis of rheumatoid arthritis or systemic sclerosis, as corticosteroids and/or immunosuppression are the mainstay of treatment regardless of the underlying CT pathology. However, lung biopsy is indicated where there is diagnostic uncertainty due to atypical presentations. In this case the biopsy findings were unexpected and resulted in a change to the initial management plan. Considerations about fertility and long term toxicity further complicated our choice of optimal therapy. This was a challenging case and highlighted the importance of multidisciplinary management of complex ILD cases. Discussions between local rheumatology, radiology and respiratory clinicians led to the decision that a biopsy was necessary. Subsequently the ILD MDT in the Freeman hospital provided clear expert guidance on in favour of a more aggressive treatment regimen than may have been otherwise initially considered. Conflict of interest The authors declare no conflicts of interest.

Postpartal psychosis: Serbian experience

2011 ◽  
Vol 26 (S2) ◽  
pp. 1098-1098
Author(s):  
M. Jasovic-Gasic ◽  
A. Damjanovic ◽  
M. Ivkovic ◽  
B. Dunjic-Kostic
Keyword(s):  
Family History ◽  
Psychiatric Disorders ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Psychiatric Disturbances ◽  
Psychological Disturbances ◽  
Clinical Center ◽  
History Of

IntroductionChildbirth and the postpartal period present a form of specific maturational crisis and an extremely vulnerable period for every woman, especially for those who have potential for some psychological disturbances.AimWe explored sociodemographic and clinical manifestations of women in the postpartal period who were hospitalized at the Institute of Psychiatry, Clinical Center of Serbia.MethodThis retrospective study included 60 patients with psychiatric disorders developed within six months after childbirth. Inclusion criteria were: negative psychiatric hystory, negative history of puerperal episode, and postpartal disorder as a first manifestation of psychiatric disturbances. Patients were diagnosed according to RDC criteria (research diagnostic criteria).ResultsPatients with psychotic features were predominant, average age 23.6; married; mothers of male offspring and with positive family history of psychiatric disorders in 30%. Subacute development of clinical manifestations was noticed, 3.5 weeks after childbirth on average. No psychopathology was observed before third postpartal day. Obstetric manifestations did not influence psychopathology.ConclusionChildbirth is a significant risk factor for the expression of mental dysfunction in the puerperal period. The most vulnerable group is women with clinical expression of dysfunction, specific sociodemographic characteristics, and positive family history of psychiatric disorders.

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.

scholarly journals CONNECTIVE TISSUE DYSPLASIA IN CHILDREN. THE EXPERIENCE OF AN ORTHOPEDIST IN AN OUT-PATIENT UNIT

2019 ◽  
Vol 23 (5) ◽  
pp. 281-284
Author(s):  
A. I. Metalnikov ◽  
A. A. Osipov ◽  
Yury V. Ten ◽  
D. A. Yelkova
Keyword(s):  
Connective Tissue ◽  
Clinical Symptoms ◽  
Adult Population ◽  
Clinical Manifestations ◽  
Decisive Role ◽  
Spherical Shape ◽  
Locomotor Apparatus ◽  
Metatarsal Bones ◽  
Prophylactic Measures ◽  
Connective Tissue Dysplasia

Pediatric orthopedists in out-patient pediatric units - unlike traumatologists- orthopedists for adult population - face a great number of various congenital malformations in the muscular-skeletal system of children, and, in the end, malformations of the connective tissue. Unfortunately, diagnosis “dysplasia of the connective tissue”, one of the most widespread diagnosis in the pediatric practice, is frequently a result of “hyper diagnostics” at the primary stage of pediatric control. A decisive role in putting this diagnosis, undoubtedly, belongs to pediatric orthopedists. Dysplasia of the connective tissue occupies a leading place in the pathology of locomotor apparatus in children who are brought to trauma units with injuries for consultations and medical aid. So, the basic purpose of this work was to develop an algorithm for monitoring patients with connective tissue dysplasia and to define ways for increasing the efficiency of prophylactic measures, treatment and rehabilitation of children with the discussed pathology. 30 children with congenital dysplastic impairments in the connective tissue, who were consulted and treated in 2011 - 2018, were included into the trial. All of them (100%) had fractures of upper and lower extremities in anamnesis ( tibia, metatarsal bones, lower third of the radius, transcondylar fractures of the humerus). There were no fractures in the skull, pelvis, fingers. No displacement of bone fragments. The cranial vault was disproportionately large, had a spherical shape. In anamnesis, delayed fontanel closure. During the dynamic observation, main clinical symptoms of the disease were: distorted limbs due to fractures ( 3 and more in each patient), muscle hypotension, vicious posture (scoliotic, kyphotic, kyphoscoliotic, flat back), blue sclera, carious teeth. Findings of laboratory, radiological and densitometric examinations were the final chain in the clinical diagnosis. Modern highly professional medical and rehabilitation measures can lead to good results in children with the connective tissue dysplasia and significantly improve their quality of life. To know the basic clinical manifestations of the disease and ways of its treatment is a need not only for orthopedists in pediatric clinics, but, first of all, for pediatricians who carry out the primary patronage of newborns. Early detection of connective tissue pathologies allows to avoid the most serious complications of the disease, such as idiopathic scoliosis, early juvenile osteochondrosis, and Shoerman-Mau disease.

Assessment of the Influence of Undifferentiated Connective Tissue Dysplasia on the Course of Myopia in School Age Children

2020 ◽  
pp. 19-25
Author(s):  
V.V. Li ◽  
◽  
O.V. Kolenko ◽  
V.V. Egorov ◽  
G.P. Smolyakova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Retinal Degeneration ◽  
Clinical Manifestations ◽  
Main Group ◽  
School Age Children ◽  
Control Group ◽  
Moderate Degree ◽  
School Myopia ◽  
Significant Difference ◽  
Connective Tissue Dysplasia

Purpose. To study the effect of undifferentiated connective tissue dysplasia (UCTD) on the clinical features of the course of school myopia. Material and methods. The object of study – 120 children (240 eyes) aged 12–15 years with moderate axial myopia. In addition to standard examination methods, computer accommodography and measure of convergence were carried out. Two observation groups were formed: the main – 80 people (160 eyes) with clinical manifestations of UCTD, the control – 40 people (80 eyes) without clinical manifestations of UCTD. According to the severity of clinical manifestations of UCTD in the main group, 3 subgroups were distinguished: 1–A – 25 children (50 eyes) with a weak degree of UCTD, 1–B – 38 children (76 eyes) with a moderate degree of UCTD, 1–C – 17 children (34 eyes) with a pronounced degree of UCTD. Results. Locomotor syndrome prevails in the structure of UCTD in children of the main group. A comparative analysis of the frequency of peripheral retinal degeneration revealed that most often they are detected in children with myopia associated with UCTD. In children of the main observation group, weakness of accommodation prevails in the structure of accommodation disorders (p<0,05). In the general population of examined in 39 children (32,5%), the presence of esophoria of varying degrees of compensation was diagnosed. A statistically significant difference was revealed between the control group and the subgroup of children with myopia associated with a pronounced degree of UCTD. Conclusion. Phenotypic signs of locomotor syndrome prevails in the structure of UCTD. School myopia in children with UCTD is characterized by: an increase in the frequency of peripheral retinal degeneration in 2,9 times; an increase in the proportion of children with a weakness of accommodation – in 1,7 times and violations between accommodation and convergence – in 2,2 times, compared with children with myopia without UCTD.

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