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Наследственно обусловленные заболевания глаз
Наследственно обусловленные заболевания глаз

Врожденные пороки развития, в том числе аномалии глаз, составляют приблизительно от 1 до 2% всех новорожденных детей. На сегодня учеными зафиксировано более 1200 генов, которые отвечают за передачу тех или иных болезней, но только малая их часть на сегодняшний день изучена учеными.

Наследственно обусловленные болезни составляют большую группу, причем проявляются они в различном возрасте. Болезнь может возникнуть еще во внутриутробный период, то есть быть врожденной. К таким, например, относятся анофтальм (полное или частичное недоразвитие глазного яблока), микрофтальм (уменьшение размеров глазного яблока), врожденная катаракта. Некоторые заболевания дают о себе знать сразу же после рождения ребенка или в первые годы его жизни. К этой группе относятся нистагм (беспорядочные движения глазных яблок), злокачественная опухоль сетчатки — ретинобластома, близорукость. Гораздо позже проявляются, например, глаукома, заболевания сетчатки.
Причины и патогенез врожденных аномалий глаз чрезвычайно разнообразны, сложны и находятся под влиянием многочисленных внутренних факторов и влияния среды.
Среди внутренних факторов имеют значение нарушения по тем или иным причинам индуцирующего действия одних тканей на рост и дифференцировку других в процессе внутриутробного развития глаз, нарушения реактивности одних тканей в ответ на индуцирующие воздействия других, тератогенные терминационные периоды, нарушения гормональных корреляций, возраст родителей, резус-несовместимость матери и плода, врожденные аномалии обмена веществ, хромосомные аберрации эндогамные и родственные браки и другие факторы.
В основе патологической наследственности лежат, как известно, повреждения специальных ядерных наследственных структур половых клеток (хромосом и генов) — материальных носителей наследственности. Это так называемые идеокинетические повреждения (мутации генов), ведущие к появлению аномалий, передающихся по наследству из поколения в поколение, могут возникать спонтанно, т.е. вследствие неизвестных нам причин. Наследственные аномалии могут, с другой стороны, возникать и в результате вредоносного воздействия на хромосомы и гены теперь уже известных многочисленных факторов среды. Все большую роль в происхождении наследственных аномалий, в том числе глазных, приписывают в последнее время нарушениям хромосомного набора половых клеток родителей и изменению взаимного расположения хромосом по сравнению с нормой (хромосомные заболевания).
Если повреждение генов в ядрах половых клеток родителей происходит еще до оплодотворения яйцеклетки и до начала зачатия, то в таких случаях говорят о гаметопатиях.
В происхождении врожденных аномалий большую роль играют тератогенные терминационные периоды, т.е. те критические периоды времени внутриутробного развития живого существа, когда происходит усиленная дифференцировка тканей глаза. В эти периоды формирующиеся ткани глаза наиболее чувствительны к воздействию эндо- и экзогенных повреждающих факторов. Для глаза человека тератогенный терминационный период падает в основном на время от половины второй до конца седьмой недели беременности. В это время еще не имеется плацентарного кровообращения, развивающееся живое существо еще носит название эмбриона, а возникшие в это время аномалии носят название эмбриопатий.
Аномалии, возникшие в более позднем периоде, после установления плацентарного кровообращения, называют фетопатиями (fetus — плод).
Наиболее распространенная форма наследуемого дефекта зрения — это пигментозный ретинит (ПР). Название охватывает гетерогенную группу патологий, суммарная встречаемость которой составляет около 1 на 3000. ПР может наследоваться как аутосомная доминанта (adRP), аутосомный рецессив (arRP) или сцепленным с Х-хромосомой (xlRP). adRP — наиболее частая форма, наблюдается по крайней мере в половине семей, страдающих от ПР.
Собственно заболевание состоит в неизлечимой (в настоящее время) дегенерации и гибели палочек и, в намного меньшей степени, колбочек. Дегенерирующие наружные сегменты палочек удаляются и перевариваются пигментным эпителием, что приводит к сильной пигментации, откуда и взялось название болезни. Утрата палочек приводит к каскаду событий, который в конце концов воздействуют и на колбочки, и на кровоснабжение сетчатки. Ранние признаки заболевания — ночная слепота и аномалии электроретинограммы (ЭРГ) . Рано или поздно, болезнь приводит к слепоте.

Внутриутробные гормональные дискорреляции, приводящие к разнообразным аномалиям развития глаза (складки сетчатки, анофтальм и др.), могут наблюдаться при расстройствах функций различных желез внутренней секреции, в особенности паращитовидных и поджелудочной железы (врожденные катаракты, афазия и др.).
Также иногда резус-несовместимость матери и плода может быть причиной разнообразных аномалий глаза (колобомы, микрофтальм, катаракта и др.). Врожденные и наследственные аномалии обмена веществ у матери без корректирующего лечения до и вовремя беременности, способны вызвать серьезные заболевания у детей, сопровождаемые разнообразными аномалиями и заболеваниями глаз (альбинизм, болезнь Гоше, галактоземия, диабет, гаргоилизм, и др).
Если же говорить о наиболее распространенных, и в то же время наиболее известных широким массам заболеваниях глаз, передающихся по наследству, то в первую очередь, необходимо упомянуть дальтонизм. Заболевание названо по имени Джона Дальтона, описавшего это явление на основе собственных ощущений. Дальтонизм заключается в человеческой неспособности различать один или несколько цветов, или путать их. Три основных цвета, которые не различают дальтоники: синий, красный и зеленый. По этому признаку их делят на три группы. Существуют также дальтоники, которые не различают все три цвета. Альбинизм также достаточно распространенное наследственное заболевание. Оно возникает как результат отсутствия в организме фермента тирозиназы, а именно этот фермент синтезирует меланин, вещество, отвечающее за окраску тканей. Альбинизм распространяется не только на органы зрения, но и на кожу. Альбиносы часто плохо видят, страдают косоглазием, отсутствием пигментации радужки, предрасположены к частичной или полной потере зрения.

В связи со значительными достижениями последних десятилетий в изучении проблемы наследственности все большее внимание уделяется роли факторов среды в происхождении врожденных наследственных и ненаследственных (перистатических) аномалий глаз. Аномалии, развившиеся на почве вредоносного воздействия средовых факторов, в настоящее время сильно превалируют над аномалиями спонтанно-генетического происхождения, которым длительный период времени до этого отводилась главенствующая роль.

Факторы среды, окружающей зародыш и плод, способны вызывать развитие таких аномалий (фенокопий), которые внешне невозможно отличить от истинно наследственных аномалий глаза.
Среди таких факторов особо важная роль принадлежит различным видам ионизирующей радиации (лучи рентгена, радия, нейтроны), способной вызвать как повреждение хромосом, генов и нерасхождение хромосом половых клеток, так и повреждение соматических клеток, с последующим возможным развитием у потомства наследственных глазных аномалий.
Также нередко наблюдается поражение органа зрения, в котором участвует наследственный компонент в сочетании с другими глазными или общими заболеваниями. Так, при мукополисахаридозе (наследственном поражении соединительной ткани) могут возникнуть и нарушения зрения. Некоторые врожденные нарушения обмена веществ сопровождаются дистрофией сетчатки, глаукомой, атрофией зрительного нерва, катарактой. Одна и та же форма заболевания может быть и наследственной, и ненаследственной. Весьма неблагоприятное воздействие на орган зрения развивающегося плода оказывают инфекционные и вирусные заболевания матери (краснуха, ветряная оспа, грипп), различного рода интоксикации во время беременности (алкоголь, некоторые снотворные препараты). Особенно чувствителен глаз плода к повреждающему агенту на 3 — 7-й неделе беременности.

Лечение врожденных и наследственных аномалий возможно во многих случаях при помощи соответствующих хирургических вмешательств, устраняющих имеющийся анатомический и функциональный недостаток.
В ряде других случаев возможно предупредить развитие глазного заболевания, а иногда спасти и самую жизнь ребенка при раннем применении определенных лечебных мероприятий. Сюда относятся, например, раннее заменное переливание крови при наследственных эритробластозах, возникших на почве резус-несовместимости; раннее назначение правильной диеты при галактоземии и сахарном диабете, назначение инсулина при диабете; назначение специфических эффективных препаратов белка при лечении гемофилии; назначение других препаратов и гормонов при лечении других аномалий. Раннее назначение соответствующей диеты при галактоземии, диеты и инсулина при сахарном диабете в таких случаях являются критически важными и могут избавить от серьезных последствий для здоровья и зрения плода.
Будущим родителям, в семье у которых заболевание органа зрения передается из поколения в поколение или семье, у которых первый ребенок родился с дефектом зрения необходимо обязательно пройти консультацию у врача-генетика. Кроме подробного изучения возможных аномалий генетического развития и наследственных факторов с целью их предупреждения, в некоторых случаях для уточнения диагноза и характера наследственного дефекта прибегают к специальным методам исследования — например, цитогенетическому или биохимическому. Такой ответственный подход позволит предупредить заболевания, передающиеся генетически.


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Displaying 1 to 18 (of 21 products)
1[/b] <a href="http://www.barbourjacket.cn/ladies-barbour-jackets-barbour-polarquilt-c-3_6.html?page=2&sort=20a" title=" Page 2 ">2</a> [url=http://www.barbourjacket.cn/ladies-barbour-jackets-barbour-polarquilt-c-3_6.html?page=2&sort=20a][Next >>][/url]
[url=http://www.barbourjacket.cn/2014-barbour-ladies-utility-polarquilt-olive-online-p-8.html][img]http://www.barbourjacket.cn/images/_small//barbour02_/Ladies-Barbour/Barbour-Polarquilt/2014-Barbour-Ladies-Utility-Polarquilt-Olive.jpg[/img]2014 Barbour Ladies Utility Polarquilt Olive Online[/url]
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<a href="http://www.barbourjacket.cn/2014-uk-womens-barbour-new-polarquilt-jacket-sandstone-p-236.html"><div style="vertical-align: middle;height:240px"><img src="http://www.barbourjacket.cn/images/_small//barbour02_/Ladies-Barbour/Barbour-Polarquilt/2014-UK-Womens-Barbour-New-Polarquilt-Jacket.jpg" alt="2014 UK Womens Barbour New Polarquilt Jacket Sandstone" title=" 2014 UK Womens Barbour New Polarquilt Jacket Sandstone " width="200" height="230" class="listingProductImage" id="listimg" /></div></a>[url=http://www.barbourjacket.cn/2014-uk-womens-barbour-new-polarquilt-jacket-sandstone-p-236.html]2014 UK Womens Barbour New Polarquilt Jacket Sandstone[/url]Womens Barbour New Polarquilt Jacket Sandstone Online Sale Classic...$505.00 $247.00Save: 51% off
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[url=http://www.barbourjacket.cn/new-barbour-ladies-utility-polarquilt-purple-outlet-p-725.html][img]http://www.barbourjacket.cn/images/_small//barbour02_/Ladies-Barbour/Barbour-Polarquilt/New-Barbour-Ladies-Utility-Polarquilt-Purple.jpg[/img]New Barbour Ladies Utility Polarquilt Purple Outlet[/url]
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Displaying 1 to 18 (of 21 products)
1[/b] [url=http://www.barbourjacket.cn/ladies-barbour-jackets-barbour-polarquilt-c-3_6.html?page=2&sort=20a]2[/url] [url=http://www.barbourjacket.cn/ladies-barbour-jackets-barbour-polarquilt-c-3_6.html?page=2&sort=20a][Next >>][/url]




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Pandora Single Black Leather Bracelet with Silver Pandora Clasp TY-94
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