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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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Créé pour représenter un lien approprié entre les Jeux Olympiques de Londres en 1948 et 2012 , la Seamaster Aqua Terra Co-Axial «London 2012" montre édition limitée commémore deux Jeux mémorables organisées par une grande ville et sert un classique intemporel. Ce modèle dispose d'un cadran bleu décoré avec le motif de Concept en teck reconnaissable et protégé par un verre saphir anti-rayures . Un enregistreur de 30 minutes, les enregistreurs de 12 heures , chronographe au centre et des secondes , et une fenêtre de date à la position 04:30 ornent également le cadran . La lunette est monté sur un boîtier en acier inoxydable de 44 mm et est présentée sur un bracelet en acier inoxydable . Le fond du boîtier est gravé de l'emblème des Jeux Olympiques de Londres 2012 . Ce chronographe est alimenté par le calibre Co -Axial 3313 .
lire la suite
proche
traits
dateLe jour du mois , s'affiche dans une fenГЄtre sur un cadran de montre gГ©nГ©ralement Г 3 heures ou 6 heures de position .
chronomètreÉtiquette donnée à une montre qui a subi des tests de précision et a reçu un certificat d'un organisme officiel ( COSC ) .
Couronne vissГ©eCouronne autobloquant , vissГ©e dans le tube de l'affaire , utilisГ© pour l'eau fortement les montres de plongГ©e rГ©sistants.
petites secondesUne main sur un sous-cadran qui suit secondes ; gГ©nГ©ralement il effectue une rotation complГЁte en une minute .
chronographeMontre compliquГ©e avec une fonction de mesure de courtes pГ©riodes de temps , en plus de sa fonction d'affichage permanent heures, minutes et secondes .
donnГ©es techniques
cristalGlace saphir bombГ©e anti-rayures avec traitement anti - reflet sur les deux cГґtГ©s
casacier
composer un numГ©robleu
RГ©sistance Г l'eau15 bar ( 150 mГЁtres / 500 pieds )
tailleDiamГЁtre : 44 mm

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    ntice', Donald Trump's reality program aimed to pick out a pick from a small grouping smart, resourceful as well as business minded people today.One from the tasks given to them on the series involved creating the advertisement to get a new performance.They were to manufacture a graffiti stimulated advertisement quietly of some building to be used being a billboard.<br />
    <br />
    Clearly, if Donald Trump offers given his approval<br />
    <br />
    These are games, another big hit with the 80's was video game titles - Pac Mankind, Mario Brothers while others were played by most teens at the same time or another.These teens turn out to be adults seeking back that has a hint for nostalgia at their earlier days, despite the truth that it was basically only 15-20 years ago.But engineering changes quickly and for that reason these icons from the 80's are getting featured in art galleries like Gallery Nineteen Eighty 6 in Movie.<br />
    <br />
    The gallery elements pieces that create a contemporary take a look at video games and is possibly helping to manufacture a new genre inside the field.<br />
    <br />
    A painting depicting Pac Person as over-the-hill and for the drip of pellets gone for $3000 in line with a Reuters press release.<br />
    <br />
    That artist, Greg Simkins, claimed he set off by portray graffiti in back walkways before using the services of canvas.And he's not on your own bringing this unique strange mix off video along with art towards the public.<br />
    <br />
    So what should we all expect yearly five yrs? Well, in the art world change is usually good, nevertheless often criticized in its formative years.So we may need to wait and discover if 'video-game art' can make a reliable stand in the art local community.What we do know is that the original online game players are now coming into their 40's as well as anyone according to that age have grown " up " with video games implementingwithin their childhood, just as 'I Love Lucy' was a piece of another generations'.Thanks to that, the images are going to have an emotional push and effect on new not to mention existing lovers today.
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    [url=http://www.pjewelry.org/pandora-murano-kiss-me-im-irish-charm-with-sterling-core-sl86-p-238.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Glass-Beads/Pandora-Murano-Kiss-Me-I-m-Irish-Charm-with.jpg[/img]Pandora Murano Kiss Me, I'm Irish Charm with Sterling Core SL-86[/url]<a class="sidebox-products" href="http://www.pjewelry.org/pandora-murano-kiss-me-im-irish-charm-with-sterling-core-sl86-p-238.html">Pandora Murano Kiss Me, I'm Irish Charm with Sterling Core SL-86</a>$62.66 $39.99Save: 36% off<a href="http://www.pjewelry.org/pandora-sterling-and-14k-gold-i-do-with-diamond-dangle-charm-d00135-p-57.html"><img src="http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Love-Beads/Pandora-Sterling-and-14K-Gold-I-Do-with-Diamond.jpg" alt="Pandora Sterling and 14K Gold I Do with Diamond Dangle Charm D/00135" title=" Pandora Sterling and 14K Gold I Do with Diamond Dangle Charm D/00135 " width="130" height="130" /></a>[url=http://www.pjewelry.org/pandora-sterling-and-14k-gold-i-do-with-diamond-dangle-charm-d00135-p-57.html]Pandora Sterling and 14K Gold I Do with Diamond Dangle Charm D/00135[/url]$71.76 $34.99Save: 51% off[url=http://www.pjewelry.org/pandora-sterling-black-pave-heart-dangle-with-black-crystal-q105-p-154.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Love-Beads/Pandora-Sterling-Black-Pave-Heart-Dangle-with-1.jpg[/img]Pandora Sterling Black Pave Heart Dangle with Black Crystal Q/105[/url]
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    1[/b] [url=http://www.pjewelry.org/pandora-holiday-c-13.html?page=2&sort=20a]2[/url] [url=http://www.pjewelry.org/pandora-holiday-c-13.html?page=2&sort=20a][Next >>][/url]
    [url=http://www.pjewelry.org/pandora-present-charm-j91-p-431.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Present-Charm-J-91.jpg[/img]Pandora Present Charm J/91[/url]
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    [url=http://www.pjewelry.org/pandora-sterling-clear-pave-star-dangle-with-clear-cz-f94-p-23.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Clear-Pave-Star-Dangle-with.jpg[/img]Pandora Sterling Clear Pave Star Dangle with Clear CZ F/94[/url]
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    [url=http://www.pjewelry.org/pandora-sterling-dangle-candy-cane-with-red-enamel-s122-p-1029.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Candy-Cane-with-Red.jpg[/img]Pandora Sterling Dangle Candy Cane with Red Enamel S/122[/url]
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    [url=http://www.pjewelry.org/pandora-sterling-dangle-cheers-charm-b100-p-264.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Cheers-Charm-B-100.jpg[/img]Pandora Sterling Dangle Cheers Charm B/100[/url]
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    [url=http://www.pjewelry.org/pandora-sterling-dangle-reindeer-g119-p-1048.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Reindeer-G-119.jpg[/img]Pandora Sterling Dangle Reindeer G/119[/url]
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    [url=http://www.pjewelry.org/pandora-sterling-dangle-silver-bells-charm-c102-p-1010.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Silver-Bells-Charm-C-102.jpg[/img]Pandora Sterling Dangle Silver Bells Charm C/102[/url]<a href="http://www.pjewelry.org/pandora-sterling-dangle-silver-bells-charm-c102-p-1010.html">Pandora Sterling Dangle Silver Bells Charm C/102</a>Why Buy With Us: 1. 100% Authentic Quality. 2. Easy Returns Policy. 3. 30 Days...$97.24 $39.99Save: 59% off[url=http://www.pjewelry.org/pandora-holiday-c-13.html?products_id=1010&action=buy_now&sort=20a][img]http://www.pjewelry.org/includes/templates/polo/buttons/english/button_buy_now.gif[/img]Buy Now[/url]
    [url=http://www.pjewelry.org/pandora-sterling-dangle-snow-angel-with-silver-enamel-charm-l89-p-832.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Snow-Angel-with-Silver.jpg[/img]Pandora Sterling Dangle Snow Angel with Silver Enamel Charm L/89[/url]
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    <a href="http://www.pjewelry.org/pandora-sterling-dangle-winter-mittens-with-pink-enamel-n99-p-457.html"><div style="vertical-align: middle;height:200px"><img src="http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Dangle-Winter-Mittens-with-Pink.jpg" alt="Pandora Sterling Dangle Winter Mittens with Pink Enamel N/99" title=" Pandora Sterling Dangle Winter Mittens with Pink Enamel N/99 " width="200" height="200" class="listingProductImage" id="listimg" /></div></a>[url=http://www.pjewelry.org/pandora-sterling-dangle-winter-mittens-with-pink-enamel-n99-p-457.html]Pandora Sterling Dangle Winter Mittens with Pink Enamel N/99[/url]Why Buy With Us: 1. 100% Authentic Quality. 2. Easy Returns Policy. 3. 30 Days...$76.57 $39.99Save: 48% off[url=http://www.pjewelry.org/pandora-holiday-c-13.html?products_id=457&action=buy_now&sort=20a][img]http://www.pjewelry.org/includes/templates/polo/buttons/english/button_buy_now.gif[/img]Buy Now[/url]
    <a href="http://www.pjewelry.org/pandora-sterling-gingerbread-man-dangle-with-golden-enamel-charm-v101-p-127.html"><div style="vertical-align: middle;height:200px"><img src="http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Gingerbread-Man-Dangle-with.jpg" alt="Pandora Sterling Gingerbread Man Dangle with Golden Enamel Charm V/101" title=" Pandora Sterling Gingerbread Man Dangle with Golden Enamel Charm V/101 " width="200" height="200" class="listingProductImage" id="listimg" /></div></a>[url=http://www.pjewelry.org/pandora-sterling-gingerbread-man-dangle-with-golden-enamel-charm-v101-p-127.html]Pandora Sterling Gingerbread Man Dangle with Golden Enamel Charm V/101[/url]Why Buy With Us: 1. 100% Authentic Quality. 2. Easy Returns Policy. 3. 30 Days...$62.31 $39.99Save: 36% off[url=http://www.pjewelry.org/pandora-holiday-c-13.html?products_id=127&action=buy_now&sort=20a][img]http://www.pjewelry.org/includes/templates/polo/buttons/english/button_buy_now.gif[/img]Buy Now[/url]
    [url=http://www.pjewelry.org/pandora-sterling-perfect-gift-charm-67v80-p88-p-756.html][img]http://www.pjewelry.org/images/_small//pandora0115_/Pandora-Holiday/Pandora-Sterling-Perfect-Gift-Charm-67V-80-P-88.jpg[/img]Pandora Sterling Perfect Gift Charm 67V-80 P/88[/url]
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    Pandora compatible charms will make the perfect gift for someone you care about - it is unique and special, just like her!





    Pandora Jewellery UK Outlet

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    ey don't have a concept involving lying, or fanciful jest.Misguided beliefs, childhood stories, make-believe, wonderful fantasies of the sorts and in some cases bogeymen the fact that lurk throughout their closets at night are most real for many years.Kids are convinced it due to the fact we declare it.Similar to the sleeping canine, their total absence of guile it seems to last such a little while! This innocence generally vanish in the first three years or so of your lives, not to ever be gotten back.
    What takes the location of idiotic innocence? We can only hope to be wisdom.As children learn the methods of the globe, this knowledge can occasionally be disappointing.There's certainly no such detail as Santa claus Claus as well as Superman.The modest puppy grew suitable big k9 that articles if the country's ears are generally yanked.Nanna died :she is not actually "sleeping." And one can find monsters, they also don't have a home in the closet after sunset; they are generally teachers as well as babysitters along with the nice man to your neighbors who comes with some unexpected pictures associated with naked little ones.
    Life stands out as the wind that erodes all the soft small gravel of chasteness.Over instance, kids normally start accepting the earth as it is, not as it must be.A daughter's or son's reasoning will get things can be what they're just.Unfortunate happenings are reduced to the simplest versions:a tornado blew the house down; some from another place don't like us in order that they blew " up " our property; people pick up sick and look at live through God.Much of right here is the way many of us as mother and father handle daily life ourselves.Do barstools2u .com offers simple stories in order to explain occurrences and problems? Or accomplish we tell the youngster what individuals understand for being the simple fact and please let them work it out from there?
    Unexpected, isn't the item? Even whenever they know truthfully, little little ones still provide the wisdom to express to it love it is and cope with it, whatever "it" may very well be.They will not have ulcers, drink excessively alcohol, or generation about tips on how to take vengeance on somebody who wounded them.They trust that they will have food to enjoy and clothing to dress in.They don't stress about paying your mortgage; these just position their figures away well before bed want Mommy talked about.As parents, it sounds incredible that any of us too had been once mainly because innocent like our most youthful children at this moment.Then lifetime happened - so does divorce, cravings, unemployment, world war and health problem.
    Many times, it is actually all too easy to sigh and additionally say that figuring out how life really needs to be for our kids too.But when Pandora released evil upon the earth, hope remained during the box.Maybe our little ones will inherit an even better world; you can easliy certainly accomplish our better to hope for that.Not most people surrenders all the innocence involving youth but some folks are that are fortunate enough to maintain a portion than me as individuals age.If this toddlers is capable of this without having becoming unhealthy or jaded because of the worries of this world, certainly they have gotten true information.

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