scholarly journals Assessment of cerebral blood flow in children with myopia in combination with undifferentiated connective tissue dysplasia

2021 ◽  
Vol 9 (2) ◽  
pp. 10-13
Author(s):  
T.V. Frolova
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Connective Tissue ◽  
Clinical Signs ◽  
Joint Hypermobility ◽  
Visual Analyzer ◽  
Elastic Parameters ◽  
Beighton Score ◽  
Connective Tissue Dysplasia ◽  
Progressive Myopia

Background. In practice, patients with numerous undifferentiated forms of connective tissue dysplasia are more common. With regard to the combination of myopia and undifferentiated connective tissue dysplasia, one of the links in the mechanism of origin of this refractive anomaly is decreased thickness of the sclera and a change in its elastic parameters. Myopia in children often occurs against the background of impaired blood supply to the visual analyzer associated with circulatory pathology in the vertebral vessels. The purpose was to evaluate cerebral blood flow in patients with myopia in combination with undifferentiated connective tissue dysplasia. Materials and methods. The study involved 86 children (170 eyes) diagnosed with progressive myopia. All patients were consulted by related specialists to detect undifferentiated connective tissue dysplasia and underwent a complete ophthalmological exa-mination. The diagnostic coefficient of expression of phenotypic and clinical signs of undifferentiated connective tissue dysplasia was also determined using the expert table of T. Kadurina. Results. Phenotypic and clinical signs of undifferentiated connective tissue dysplasia were noted in 41 (100 %) cases when evaluating according to the criteria of T. Mil-kovskaya-Dimitrova and T. Kadurina, and in 34 (83.0 %) children du-ring screening for joint hypermobility on Beighton score. As for increased axial length of the eye in the examined patients with myopia and undifferentiated connective tissue dysplasia, it was noted that an increase had a direct correlation with the severity of undifferentiated connective tissue dysplasia, with the greatest correlation obtained in children with pronounced phenotypic manifestations in the musculoskeletal system (correlation coefficient was 0.83). Chemical regulation of carotid artery tone (respiratory failure test) is altered in the vast majority of patients with myopia. Conclusions. All children with progressive myopia are re-commended to be examined by related specialists to identify undif-ferentiated connective tissue dysplasia and hemodynamic disorders. Only a comprehensive approach to this refractive anomaly can be effective in combating its progression.

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 CLINICAL AND GENETIC FEATURES OF CONNECTIVE TISSUE DYSPLASIA SYNDROME IN CYSTIC FIBROSIS IN CHILDREN

2019 ◽  
Vol 21 (4) ◽  
pp. 203-207 ◽  
Author(s):  
Anastasia V. Goryainova ◽  
P. V. Shumilov ◽  
S. Yu. Semykin ◽  
G. Yu. Zobkova ◽  
A. E. Donnikov
Keyword(s):  
Cystic Fibrosis ◽  
Connective Tissue ◽  
Matrix Metalloproteinase ◽  
Clinical Signs ◽  
Respiratory Disorders ◽  
Matrix Metalloproteinase 3 ◽  
Genetic Features ◽  
Connective Tissue Dysplasia ◽  
Modifying Effect ◽  
Severe Pathology

The aim of the study was to determine the clinical and genetic features of the syndrome of undifferentiated connective tissue dysplasia (CTD) in cystic fibrosis (CF) children and the possible modifying effect of polymorphisms of connective tissue genes on the development of severe pathology of the bronchopulmonary system in CF cases. 188 patients with the moderate to severe course of СF, aged from 3 to 17 years were examined. In СF patients significant associations have been established between polymorphisms of matrix metalloproteinase 3, the phenotypic signs of CTD and severe clinical signs of respiratory disorders.

scholarly journals Early detection of brainstem herniation using electroencephalography monitoring – case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Naresh Mullaguri ◽  
Jonathan M. Beary ◽  
Christopher R. Newey
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Obstructive Hydrocephalus ◽  
Multiorgan Failure ◽  
Clinical Signs ◽  
Quantitative Electroencephalography ◽  
First Case ◽  
Cerebral Herniation ◽  
Brainstem Herniation ◽  
Injured Patients

Abstract Background Continuous electroencephalography (cEEG) is an important neuromonitoring tool in brain injured patients. It is commonly used for detection of seizure but can also be used to monitor changes in cerebral blood flow. One such event that can cause a change in cerebral blood flow is imminent, cerebral herniation. cEEG monitoring and quantitative electroencephalography (QEEG) can be used as neurotelemetry to detect cerebral herniation prior to onset of clinical signs. Case presentation We discuss two cases highlighting the use of cEEG in cerebral herniation accompanied by clinical examination changes. The first case is a patient with multiorgan failure and intracerebral hemorrhage (ICH). Given his coagulopathy status, his ICH expanded. The second case is a patient with intraventricular hemorrhage and worsening obstructive hydrocephalus. In both cases, the cEEG showed increasing regional/lateralized slowing. The Quantitative electroencephalography (QEEG) showed a decrease in frequencies, worsening asymmetry, decreasing amplitude and increasing burst suppression ratio corresponding with the ongoing herniation. Clinically, these changes on cEEG preceded the bedside neurological changes by up to 1 h. Conclusions The use of cEEG to monitor patients at high risk for herniation syndromes may identify changes earlier than bedside clinical exam. This earlier identification may allow for an earlier opportunity to intervene.

Cerebral blood flow in basal ganglia is correlated with clinical signs of hepatic encephalopathy in patients with liver cirrhosis

2002 ◽  
Vol 97 (3) ◽  
pp. 763-764 ◽  
Author(s):  
Motoh Iwasa ◽  
Masahiko Kaito ◽  
Yukihiko Adachi ◽  
Yuri Watanabe ◽  
Kaname Matsumura ◽  
...  
Keyword(s):  
Blood Flow ◽  
Liver Cirrhosis ◽  
Cerebral Blood Flow ◽  
Hepatic Encephalopathy ◽  
Basal Ganglia ◽  
Clinical Signs

The state of microcirculation and identification of microcirculatory predictors of chronic pain in younger patients with functional disorders of the masticatory system affected by connective tissue dysplasia

2020 ◽  
Vol 25 (4) ◽  
pp. 308-316
Author(s):  
E. Е. Statovskaya
Keyword(s):  
Chronic Pain ◽  
Blood Flow ◽  
Connective Tissue ◽  
Pain Syndrome ◽  
High Amplitude ◽  
Masticatory Muscles ◽  
Main Group ◽  
Control Group ◽  
Doppler Flowmetry ◽  
Connective Tissue Dysplasia

Relevance. Occlusal splints, made for the patients with temporomandibular disorders (TMD), influence proprioceptive sensitivity, sensory characteristics of pain, parameters of microcirculation of the pulp and periodontium of the healthy teeth. Condition of the pulp and periodontium of the healthy teeth should be assessed in patients with TMD associated with connective tissue dysplasia (CTD).Materials and methods. The study examined 36 TMD patients aged 26.3 ± 1.3 лет (М ± m) with CTD (n = 20, main group) and without CTD (n = 16, control group); with natural teeth, without complaints of TMD, masticatory muscles and periodontal condition. Laser doppler flowmetry (LDF) helped to analyze blood flow parameters in the periodontium and pulp of the healthy teeth. Received results were statistically analyzed.Results. Wavelet analysis revealed different microcirculatory flowmotion: high-amplitude irregular type was in patients of the main group, low-amplitude regular type was in controls. In the main group, perfusion is supported by high values of flux and vasomotion, ergotropic sympathetic effects (controlled by catecholamines) prevail, myogenic tone is reduced, elevated microvascular tone reflects possible changes in rheologic blood properties, microcirculatory signs of pain syndrome and relatively elevated ischemic index in pulp microcirculation of healthy teeth.Conclusion. Sensory and regulatory mechanisms, reflected by the condition of periodontium and pulp blood flow, can be regarded as early signs of inflammation and chronic pain in the diagnosis of TMD in patients without complaints.

scholarly journals Electrical function of the abdominal muscles after combined plastic of extensive median hernias in the context of connective tissue dysplasia

2019 ◽  
Vol 12 (3) ◽  
pp. 152-157
Author(s):  
Radu Railianu
Keyword(s):  
Connective Tissue ◽  
Abdominal Wall ◽  
Anterior Abdominal Wall ◽  
Clinical Signs ◽  
Clinical Severity ◽  
Abdominal Muscles ◽  
Abdominal Hernias ◽  
Connective Tissue Dysplasia ◽  
Optimal Rehabilitation ◽  
Relaxing Effect

Relevance. Improving the electrical function of abdominal muscles in patients with extensive median hernias of the anterior abdominal wall can be facilitated by optimizing the choice of methods for combined hernioplasty, taking into account the clinical severity of connective tissue dysplasia among the hernia carriers. Objective. Study the electrical function of abdominal muscles after the methods of combined hernioplasty of extensive median hernias, taking into account the severity of connective tissue dysplasia among the hernia carriers. Results.  The average numbers of electromyogram frequencies of the abdominal muscles of patients with extensive median hernias from the group without clinical signs of connective tissue dysplasia were almost the same after all the applied methods of combined hernioplasty. The predominance of the rectus muscles electric function over the lateral abdominal muscles among the patients of this group was 33,5 %. Electrofunctional rehabilitation of the abdominal muscles of patients in the second group of examinations depended on the method of combined hernioplasty and changed in the event of an increase or decrease in the severity of connective tissue dysplasia.  The recovery of electroactivity of abdominal muscles in the postoperative period in patients from the second group was more significant with an increase in the number of abdominal muscles applied to aponeuroses and vagina of relaxing sections, which contributed to an increase in mobility and contractility of the musculoaponeurotic layers of the anterior abdominal wall. Conclusions. Thus, with an increase in the severity of connective tissue dysplasia in patients with extensive median abdominal hernias, the method of combined hernioplasty with the most pronounced relaxing effect in relation to the anterior abdominal wall promoted optimal rehabilitation of abdominal muscles.

scholarly journals The role of polymorphic variants of matrix metalloproteinases genes in the formation of disruption of connective tissue homeostasis and pathology of the musculoskeletal system.

2018 ◽  
Vol 96 (8) ◽  
pp. 754-761
Author(s):  
A. V. Tyurin ◽  
D. A. Shapovalova ◽  
L. Z. Lukmanova ◽  
V. B. Golubyatnikov ◽  
B. A. Bakirov ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Polymorphic Locus ◽  
Age Groups ◽  
Study Groups ◽  
Ethnic Origin ◽  
Joint Hypermobility ◽  
Clinical Genetic ◽  
Symptom Complex ◽  
Connective Tissue Dysplasia ◽  
Polymorphic Variants

A clinical assessment of the presence of osteoarthritis (OA) with various localizations, undifferentiated connective tissue dysplasia (uCTD) and joint hypermobility (JHM) in 484 individuals of both sexes of different age groups was carried out. We searched for associations of 4 polymorphic variants of matrix metalloproteinase genes (rs35068180 (MMP3), rs2252070 (MMP13) ), rs226794 and rs2830585 (ADAMTS5)) with the development of osteoarthrosis as a whole, taking into account the localization of the pathological process, the age of the patients, the ethnic origin of the study groups and the presence of undifferentiated connective tissue dysplasia as a whole and its individual phenotypic markers, as well as in the comorbid state with osteoarthritis was carried out. 158 patients had osteoarthritis, 252 had a symptom complex of uCTD, 92 of them were in the comorbid state with OA. The significance of the polymorphic loci of MMP3, MMP13, ADAMTS5 genes in the formation of the symptom complex of uCTD in general and its individual phenotypes was detected. The polymorphic locus of MMP3 gene was associated with OA in the comorbid state with uCTD. Statistically significant models based on clinical-genetic data using the method of multiple logistic regression, that allow predicting the development of osteoarthrosis of knee, hip joints and polyosteoarthrosis were calculated.

scholarly journals CLINICAL-LABORATORY MARKERS OF FIBRILOGENESIS DISORDERS IN THE SEVERITY OF PYELONEPHRITIS IN CHILDREN

2018 ◽  
Vol 2 ◽  
pp. 30-38
Author(s):  
Natalia Lukyanenko ◽  
Mariana Iskiv
Keyword(s):  
Connective Tissue ◽  
Acute Pyelonephritis ◽  
Clinical Laboratory ◽  
Joint Hypermobility ◽  
Chronic Pyelonephritis ◽  
Control Group ◽  
Healthy Children ◽  
Laboratory Markers ◽  
Frequent Relapses ◽  
Connective Tissue Dysplasia

Aim of the research: to establish the role of undifferentiated connective tissue dysplasia, as a manifestation of violation of fibrillogenesis, in the severity of the course of pyelonephritis in children. 154 children with pyelonephritis from 3 to 18 years were examined. As a result of catamnestic surveillance, they were divided into 2 groups: I – 92 persons, children with chronic pyelonephritis in which were diagnosed 3 or more episodes of relapse of pyelonephritis during the year, and II – 56 children with acute pyelonephritis, in which during the year no relapses were noted. The control group were 65 somatically healthy children of the same age (III - health-control). All children had a routine comprehensive clinical and laboratory examination and clinical and laboratory markers of a fibrillogenic disorder were established. In children with chronic pyelonephritis, the frequency of all analyzed complaints was significantly higher than in children with acute pyelonephritis without relapses: frequent headaches – 56.52 % versus 25.0 %, appetite loss – 28.26 % vs. 19.64 %, frequent abdominal pain – 52.17 % vr. 32.14 %, increased fatigue – 41.30 % vr. 28.57 %. In children with chronic pyelonephritis, phenotypic signs of undifferentiated connective tissue dysplasia (UCTD) were significantly more marked, such as joint hypermobility (in 52.0 % of children versus 5.4 %), asthenic body structure (59.0 % vs. 26.78 %), visual disturbance (84.8 % vs. 32.14 %), chest deformity (42.4 % vs. 8.9 %), scoliosis (52.17 % vs 10.7 %), arachnodactyly and predisposition to bleeding were observed only in children of the 1st group (22.5 % and 4.34 % respectively). In practically all children with chronic pyelonephritis, the values of free and bound oxyproline fractions in blood plasma were significantly increased (47.14±0.03 μmol/l and 40.08±0.03 μmol/l, respectively), according to arithmetic meanings, reliably differing from the data of children with acute pyelonephritis (17.65±0.01 μmol/l and 17.22±0.02 μmol/l), in which these oxyproline fractions were elevated only in 12.0 % and 16.0 % of the subjects. In 97.0 % of children with chronic pyelonephritis, the level of oxyproline in urine was elevated and significantly exceeded the level of excretion of oxyproline in urine in children with acute pyelonephritis. The presence of UCTD in a child plays an important role in the process of chronic pyelonephritis, and children with its manifestations have a heavier course of disease with frequent relapses, therefore, the presence of signs UCTD is prognostically unsuccessful, which dictates the need for the appointment of metabolic therapy in the first episodes of the disease in children, if they have clinical and laboratory manifestations of UCTD.

scholarly journals The level of microelements and heterogeneity of joint hypermobility as an endophenotype of undifferentiated connective tissue dysplasia

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Luiza Z. Lukmanova ◽  
Rashit A. Davletshin ◽  
Rita I. Khusainova ◽  
Anton V. Tyurin
Keyword(s):  
Connective Tissue ◽  
Statistical Significance ◽  
Serum Magnesium ◽  
Colorimetric Method ◽  
Serum Zinc ◽  
Joint Hypermobility ◽  
Control Group ◽  
Serum Concentrations ◽  
Copper Zinc ◽  
Connective Tissue Dysplasia

Objective — The aim of the work was to study serum concentrations of magnesium, copper, zinc, phosphorus and calcium in individuals with undifferentiated connective tissue dysplasia (uCTD) and joint hypermobility (JH) in an isolated and combined state. Material and methods — the concentrations of magnesium, copper, zinc, phosphorus and calcium were measured by the direct colorimetric method in 55 people with joint hypermobility and in 34 – without hypermobility. Results — There were no significant differences between serum concentrations of microelementsin groups with and without JH. In patients with mild and severe uCTD significant decrease in serum magnesium concentrations was noted (U=2.12, p=0.034 and U=3.7, p=0.012). In patients with isolated JH significant serum zinc concentrationdecrease was revealed compared with the control group (U=3.12, p=0.022). Serum magnesium concentrations were reduced in all patients with uCTD and JH; in the groups with isolated dysplasia and combined pathology, the differences reached the level of statistical significance (U=2.78, p=0.024 and U=3.2, p=0.018). Conclusion — The study revealed significant associations of a decrease in serum magnesium concentrations with the development of uCTD in an isolated and combined with JH state and decrease in serum zinc concentrations with the development of isolated JH.

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