Manifestations of mitochondrial dysfunction in children with connective tissue dysplasia and chronic gastroduodenitis

According to the literature, signs of mitochondrial dysfunction are found in 15–20% of children in the population. It often accompanies physical illness. The digestive tract is an energy-intensive system of organs, so violations of energy metabolism affect its functioning. The aim of this study was to determine the basal level of carnitine and its fractions in children with connective tissue dysplasia and chronic gastroduodenitis and to evaluate their dynamics after L-carnitine therapy. 77 children with this somatic pathology were examined. Depending on the presence of connective tissue dysplasia, they were divided into 2 groups – the main and the comparison group. Blood levels of total and bound carnitine were determined in all patients using liquid tandem chromatomass-spectrometry. Free carnitine was calculated as the difference between total and bound. It was found that in children with connective tissue dysplasia basal levels of total carnitine and its fractions are significantly lower than in patients of the comparison group. Carnitine and fractions increased in all children after a 30-day course of therapy with L-carnitine, reaching normal values with a reduced basal level. The degree of increase in indicators was greater in patients with initially lower levels, which was observed primarily in a group with connective tissue dysplasia. This study showed that connective tissue dysplasia in most cases is accompanied by mitochondrial dysfunction. Most patients with connective tissue dysplasia and chronic gastroduodenitis require correction of energy metabolism disorders using energy-stimulating therapy.

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

Wiadomości Lekarskie ◽

10.36740/wlek202110206 ◽

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.

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Method of diagnostics of undifferentiated connective tissue dysplasia

Bukovinian Medical Herald ◽

10.24061/2413-0737.xxv.2.98.2021.18 ◽

2021 ◽

Vol 25 (2(98)) ◽

pp. 114-118

Author(s):

O. Usenko ◽

Ya. Voitiv ◽

S. Shcherbyna

Keyword(s):

Statistical Analysis ◽

Connective Tissue ◽

Comparison Group ◽

Morphological Changes ◽

Surgical Pathology ◽

Main Attention ◽

White Line ◽

Connective Tissue Dysplasia ◽

Immunohistochemical Studies ◽

Connective Tissue Pathology

Objective – to develop a method for diagnosing undifferentiated connective tissue dysplasia (UCTD) in patients with surgical pathology.Material and methods. The object of the study comprises 96 patients with surgical pathology who were treated in the department of thoracoabdominal surgery of the Shalimov National Institute of Surgery and Transplantology during 2017-2020 and 20 practically healthy people (comparison group). Laboratory, instrumental, histological, immunohistochemical studies and statistical analysis were performed.Results. The developed method contains an assessment of the most informative phenotypic and visceral signs of connective tissue pathology. The main attention is paid to the features that characterize the visceral manifestations of UDCT, especially functional and morphological changes of the digestive organs, which is extremely important in abdominal surgery. Ultrasonography is used to analyze the width of the white line of the abdomen and the condition of the abdominal and retroperitoneal organs, determining the presence of diastase of the rectus abdominis and splanchnoptosis. Studies of the effectiveness of the proposed method have shown that it can be used to diagnose UDCT in patients with surgical pathology, which is necessary to select effective treatment tactics and prevent complications in such patients.Conclusions. A method for diagnosing undifferentiated connective tissue dysplasia has been developed, which evaluates the most informative phenotypic signs of connective tissue pathology and considers the peculiarities of pathology in patients of a surgical hospital.

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he specifics of posture formation among schoolchildren living in the city of Khanty-Mansiysk against the background of undifferentiated connective tissue dysplasia

10.34014/mpphe.2021-236-239 ◽

2021 ◽

Author(s):

I.A. Shevnin ◽

O.N. Ragozin ◽

O.V. Ragozina ◽

N.A. Ilyushchenko ◽

B.Z. Jafarova

Keyword(s):

Connective Tissue ◽

Comparison Group ◽

Physical Development ◽

Study Group ◽

The North ◽

Negative Effect ◽

Connective Tissue Dysplasia ◽

The City ◽

Khanty Mansi Autonomous

Undifferentiated forms of DST are widespread among children living in Khanty-Mansiysk, in connection with which it is relevant to assess the influence of this pathology on the formation of posture in schoolchildren of the North. Determination of the type of posture and the identification of stigmas of dysembryogenesis was carried out in 208 schoolchildren of the city of Khanty-Mansiysk. Posture types were divided into: normal, round, flat and stooped. Participants with the number of stigmas from 0 to 5 were allocated to the comparison group (n = 66), the rest (6 or more) made up the study group (n = 143). A card was entered, including 43 hair dryers. The study found that the incidence of UCTD among children in the Khanty-Mansi Autonomous Okrug-Yugra is noticeably higher than among those living in temperate latitudes and has a negative effect on the formation of posture. Key words: North, posture, physical development, undifferentiated connective tissue dysplasia.

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Pathogenic pattern of somatic disorders in children with congenital cleft lip and palate in associated connective tissue dysplasia

Russian Journal of Woman and Child Health ◽

10.32364/2618-8430-2021-4-4-362-369 ◽

2021 ◽

Vol 4 (4) ◽

pp. 362-369

Author(s):

E.V. Neudakhin ◽

◽

A.G. Prityko ◽

A.Yu. Kugushev ◽

T.I. Meshcheryakova ◽

...

Keyword(s):

Matrix Metalloproteinases ◽

Connective Tissue ◽

Mitochondrial Dysfunction ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Organ System ◽

Keywords Children ◽

Somatic Disorders ◽

Connective Tissue Dysplasia ◽

Congenital Cleft

In children with cleft lip and palate (CLP), various organ and organ system abnormalities occur under the effect of endogenous (hereditary) and/ or exogenous (resulting in impaired embryogenesis) factors. The community of CLP and multiorgan disorders is accounted for by connective tissue dysplasia (CTD). According to a theory of mesodermal migration, the development of connective tissue is associated with impaired formation and development of mesodermal elements during embryogenesis. Mitochondrial dysfunction accompanied by significant energy metabolism abnormalities with signs of oxidative stress, hypoxia, and acidosis has a crucial role in CTD. Matrix metalloproteinases are actively involved in developing CTD and CLP. Genotyping of gene polymorphism involved in lip and palate morphogenesis is required to predict the risk of congenital CLP in compromised families. This paper addresses data helpful in developing preventive and treatment approaches, predicting and managing children with CLP from an individual and familial viewpoint. KEYWORDS: children, cleft lip and palate, connective tissue dysplasia, mitochondrial dysfunction, matrix metalloproteinases, energetic therapy, L-carnitine. FOR CITATION: Neudakhin E.V., Prityko A.G., Kugushev A.Yu. et al. Pathogenic pattern of somatic disorders in children with congenital cleft lip and palate in associated connective

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