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Муковисцидоз


Муковисцидоз_2.jpgМуковисцидоз представляет собой системное наследственное заболевание, при котором поражаются экзокринные железы. Причиной муковисцидоза является генная мутация, передающаяся по аутосомно – рециссивному типу.

Выделяют следующие основные формы муковисцидоза:

- смешанная (легочно-кишечная, 75-80%)

- респираторная (15-20%)

- кишечная (5%).

Причины

При развитии муковисцидоза основными являются три основных момента: поражение желез внешней секреции, изменения со стороны соединительной ткани, водно-электролитные нарушения. Причиной муковисцидоза служит генная мутация, в результате которой нарушается строение и функции белка МВТР (трансмембранного регулятора муковисцидоза), участвующего в водно-электролитном обмене эпителия, выстилающего бронхопульмональную систему, поджелудочную железу, печень, желудочно-кишечный тракт, органы репродуктивной системы.

При муковисцидозе изменяются физико-химические свойства секрета экзокринных желез (слизи, слезной жидкости, пота): он становится густым, с повышенным содержанием электролитов и белка, практически не эвакуируется из выводных протоков. Задержка вязкого секрета в протоках вызывает их расширение и формирование мелких кист, в наибольшей степени бронхолегочной и пищеварительной системах.

Муковисцидоз_3.jpg

Электролитные нарушения связаны с высокой концентрацией кальция, натрия и хлора в секретах. Застой слизи приводит к атрофии (усыханию) железистой ткани и прогрессирующему фиброзу (постепенному замещению ткани железы - соединительной тканью), раннему появлению склеротических изменений в органах. Осложняет положение развитие гнойного воспаления в случае вторичного инфицирования.

Поражение бронхолегочной системы при муковисцидозе происходит вследствие затруднения отхождения мокроты (вязкая слизь, нарушение функции мерцательного эпителия), развития мукостаза (застоя слизи) и хронического воспаления. Нарушение проходимости мелких бронхов и бронхиол лежит в основе патологических изменений органов дыхания при муковисцидозе. Бронхиальные железы со слизисто – гнойным содержимым, увеличиваясь в размерах, выпячиваются и перекрывают просвет бронхов. Формируются мешотчатые, цилиндрические и «каплевидные» бронхоэктазы, образуются эмфизематозные участки легкого, при полной обтурации бронхов мокротой – зоны ателектаза, склеротические изменения ткани легкого (диффузный пневмосклероз). При муковисцидозе патологические изменения в бронхах и легких осложняются присоединением бактериальной инфекции (золотистого стафилококка, синегнойной палочки), абсцедированием, развитием деструктивных изменений. Это связано с нарушениями в системе местного иммунитета (снижение уровня антител, интерферона, фагоцитарной активности, изменение функционального состояния эпителия бронхов).

Кроме бронхолегочной системы при муковисцидозе отмечается поражение желудка, кишечника, поджелудочной железы, печени.

Лечение

Для стимуляции роста эндогенной микрофлоры целесообразно назначать пробиотики - препараты, содержащие живые микробы (моно- или комбинированные), и пребиотики - препараты, не содержащие живых организмов, а только их метаболиты, стимулирующие рост нормальной микрофлоры.

Среди метаболитов следует особенно выделить лактулозу - синтетический дисахарид, не встречающийся в природе.

Лактулоза попадает в толстый кишечник в неизмененном виде, становится питательным веществом для сахаролитических бактерий и снижает кислотность содержимого толстой кишки. Также применение лактулозы понижает проходимость пищевых масс, что ведет к задержке жидкости в просвете кишки и усилению ее перистальтики. Лактулоза - источник углеводов и энергии, ее использование приводит к увеличению бактериальной массы, активной утилизации аммиака и азота аминокислот. Эти изменения в конечном итоге и обеспечивают профилактический и терапевтический эффекты. Особенно важна лактулоза при запорах, портосистемной энцефалопатии, энтеритах и сахарном диабете.

Лактулоза применяется для терапии больных муковисцидозом уже в течение 5 лет и зарекомендовала себя как эффективное и безопасное средство. 



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