DYNAMICS OF PHENOTYPIC MANIFESTATIONS OF CONNECTIVE TISSUE DYSPLASIA IN CHILDREN WITH UROANDROLOGICAL PATHOLOGY IN THE AGE ASPECT



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Abstract

Introduction. The article includes a comparative study of phenotypic manifestations of undifferentiated connective tissue dysplasia in children of different age groups with urological and andrological pathology. Material and methods. The trial performed included 214 children aged from 1 month to 17 years with clinical manifestations of undifferentiated connective tissue dysplasia who were treated in the children’s uroandrological department for congenital malformations of the urogenital system. The generally accepted table of external phenotypic markers in scores was used in establishing undifferentiated connective tissue dysplasia . Results. In infants, the most frequent disorders were in ears and skin. In the preschool age, in the majority of cases, craniocephalic abnormalities, pathology of osteo-articular system and ears were met. The trail has revealed that the rate of ocular abnormalities increases with age. Moreover, two or more ocular problems were most common in older children. Anomalies of the oral cavity were diagnosed only in a quarter of infants and were manifested in them by a single sign. In older age groups, oral cavity anomalies were in more than half of the patients, and often they were not isolated. During the survey of senior schoolchildren, it was noted that all children of this age group had this or that disorder in their osteo-articular system, and almost always they were multiple. Conclusion. Somatic manifestations of undifferentiated connective tissue dysplasia are often multiorganic and often have non-specific clinical symptoms. Identification of patterns of formation of uroandrological pathology in patients of different age groups with connective tissue insufficiency will ensure timely prevention and correction of visceral manifestations of dysplasia in children.

About the authors

I. G. Vasilyeva

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: noemail@neicon.ru
Россия

S. M. Sharkov

Morozovsky Municipal Children’s Hospital, Healthcare Department of Moscow; I.M. Sechenov First Moscow State Medical University

Email: noemail@neicon.ru
Россия

B. G. Safronov

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Email: noemail@neicon.ru
Россия

I. E. Volkov

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Email: noemail@neicon.ru
Россия

I. S. Burov

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Email: noemail@neicon.ru
Россия

A. P. Dementyev

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Email: noemail@neicon.ru
Россия

A. V. Mozhayev

Federal State Budgetary Educational Institution of Higher Education “Ivanovo State Medical Academy” of the Ministry of Healthcare of the Russian Federation

Email: mozhaev@isma.ivanovo.ru
Россия

References

  1. Кадурина Т.И., Гнусаев С.Ф., Аббакумова Л.Н. и др. Наследственные и многофакторные нарушения соединительной ткани у детей. Проект российских рекомендаций. Педиатрия. 2014; 93(5): 40-5.
  2. Кулешов А.В. Состояние вегетативной нервной системы у детей с недифференцированной дисплазией соединительной ткани. Педиатрия. 2017; 96(3): 101-6.
  3. Горяинова А.В., Шумилов П.В., Семыкин С.Ю., Зобкова Г.Ю., Донников А.Е. Клинико-генетические особенности синдрома дисплазии соединительной ткани при муковисцидозе у детей. Российский педиатрический журнал. 2018; 21(4): 203-7.
  4. Тимофеева Е.П., Рябиченко Т.И., Скосырева Г.А., Карцева Т.В. Здоровье детей и подростков с недифференцированной дисплазией соединительной ткани в онтогенезе. Journal of Siberian Medical Sciences. 2015; 3: 20-3.
  5. Чемоданов В.В., Горнаков И.С., Буланкина Е.В. Дисплазии соединительной ткани у детей Иваново. Ивановский издательский дом. 2004
  6. Pyeritz R.E. The Marfan syndrome. Annu Rev Med. 2000; 51. 1043-5.
  7. Шарков С.М., Васильева И.Г., Стрельников А.И., Полозов В.В. Сравнительная характеристика морфологических изменений и фенотипических маркеров дисплазии соединительной ткани у детей с различной уроандрологической патологией. Детская хирургия. 2018; 22(3): 120-3.
  8. Капитан Т.В. Пропедевтика детских болезней с уходом за детьми. 3-е изд, доп. М.: ММЕДпресс-информ, 2007. 98 стр.

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