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Ќаследственно обусловленные заболевани€ глаз
Ќаследственно обусловленные заболевани€ глаз

¬рожденные пороки развити€, в том числе аномалии глаз, составл€ют приблизительно от 1 до 2% всех новорожденных детей. Ќа сегодн€ учеными зафиксировано более 1200 генов, которые отвечают за передачу тех или иных болезней, но только мала€ их часть на сегодн€шний день изучена учеными.

Ќаследственно обусловленные болезни составл€ют большую группу, причем про€вл€ютс€ они в различном возрасте. Ѕолезнь может возникнуть еще во внутриутробный период, то есть быть врожденной.   таким, например, относ€тс€ анофтальм (полное или частичное недоразвитие глазного €блока), микрофтальм (уменьшение размеров глазного €блока), врожденна€ катаракта. Ќекоторые заболевани€ дают о себе знать сразу же после рождени€ ребенка или в первые годы его жизни.   этой группе относ€тс€ нистагм (беспор€дочные движени€ глазных €блок), злокачественна€ опухоль сетчатки — ретинобластома, близорукость. √ораздо позже про€вл€ютс€, например, глаукома, заболевани€ сетчатки.
ѕричины и патогенез врожденных аномалий глаз чрезвычайно разнообразны, сложны и наход€тс€ под вли€нием многочисленных внутренних факторов и вли€ни€ среды.
—реди внутренних факторов имеют значение нарушени€ по тем или иным причинам индуцирующего действи€ одних тканей на рост и дифференцировку других в процессе внутриутробного развити€ глаз, нарушени€ реактивности одних тканей в ответ на индуцирующие воздействи€ других, тератогенные терминационные периоды, нарушени€ гормональных коррел€ций, возраст родителей, резус-несовместимость матери и плода, врожденные аномалии обмена веществ, хромосомные аберрации эндогамные и родственные браки и другие факторы.
¬ основе патологической наследственности лежат, как известно, повреждени€ специальных €дерных наследственных структур половых клеток (хромосом и генов) Ч материальных носителей наследственности. Ёто так называемые идеокинетические повреждени€ (мутации генов), ведущие к по€влению аномалий, передающихс€ по наследству из поколени€ в поколение, могут возникать спонтанно, т.е. вследствие неизвестных нам причин. Ќаследственные аномалии могут, с другой стороны, возникать и в результате вредоносного воздействи€ на хромосомы и гены теперь уже известных многочисленных факторов среды. ¬се большую роль в происхождении наследственных аномалий, в том числе глазных, приписывают в последнее врем€ нарушени€м хромосомного набора половых клеток родителей и изменению взаимного расположени€ хромосом по сравнению с нормой (хромосомные заболевани€).
≈сли повреждение генов в €драх половых клеток родителей происходит еще до оплодотворени€ €йцеклетки и до начала зачати€, то в таких случа€х говор€т о гаметопати€х.
¬ происхождении врожденных аномалий большую роль играют тератогенные терминационные периоды, т.е. те критические периоды времени внутриутробного развити€ живого существа, когда происходит усиленна€ дифференцировка тканей глаза. ¬ эти периоды формирующиес€ ткани глаза наиболее чувствительны к воздействию эндо- и экзогенных повреждающих факторов. ƒл€ глаза человека тератогенный терминационный период падает в основном на врем€ от половины второй до конца седьмой недели беременности. ¬ это врем€ еще не имеетс€ плацентарного кровообращени€, развивающеес€ живое существо еще носит название эмбриона, а возникшие в это врем€ аномалии нос€т название эмбриопатий.
јномалии, возникшие в более позднем периоде, после установлени€ плацентарного кровообращени€, называют фетопати€ми (fetus Ч плод).
Ќаиболее распространенна€ форма наследуемого дефекта зрени€ Ч это пигментозный ретинит (ѕ–). Ќазвание охватывает гетерогенную группу патологий, суммарна€ встречаемость которой составл€ет около 1 на 3000. ѕ– может наследоватьс€ как аутосомна€ доминанта (adRP), аутосомный рецессив (arRP) или сцепленным с ’-хромосомой (xlRP). adRP Ч наиболее часта€ форма, наблюдаетс€ по крайней мере в половине семей, страдающих от ѕ–.
—обственно заболевание состоит в неизлечимой (в насто€щее врем€) дегенерации и гибели палочек и, в намного меньшей степени, колбочек. ƒегенерирующие наружные сегменты палочек удал€ютс€ и перевариваютс€ пигментным эпителием, что приводит к сильной пигментации, откуда и вз€лось название болезни. ”трата палочек приводит к каскаду событий, который в конце концов воздействуют и на колбочки, и на кровоснабжение сетчатки. –анние признаки заболевани€ Ч ночна€ слепота и аномалии электроретинограммы (Ё–√) . –ано или поздно, болезнь приводит к слепоте.

¬нутриутробные гормональные дискоррел€ции, привод€щие к разнообразным аномали€м развити€ глаза (складки сетчатки, анофтальм и др.), могут наблюдатьс€ при расстройствах функций различных желез внутренней секреции, в особенности паращитовидных и поджелудочной железы (врожденные катаракты, афази€ и др.).
“акже иногда резус-несовместимость матери и плода может быть причиной разнообразных аномалий глаза (колобомы, микрофтальм, катаракта и др.). ¬рожденные и наследственные аномалии обмена веществ у матери без корректирующего лечени€ до и воврем€ беременности, способны вызвать серьезные заболевани€ у детей, сопровождаемые разнообразными аномали€ми и заболевани€ми глаз (альбинизм, болезнь √оше, галактоземи€, диабет, гаргоилизм, и др).
≈сли же говорить о наиболее распространенных, и в то же врем€ наиболее известных широким массам заболевани€х глаз, передающихс€ по наследству, то в первую очередь, необходимо упом€нуть дальтонизм. «аболевание названо по имени ƒжона ƒальтона, описавшего это €вление на основе собственных ощущений. ƒальтонизм заключаетс€ в человеческой неспособности различать один или несколько цветов, или путать их. “ри основных цвета, которые не различают дальтоники: синий, красный и зеленый. ѕо этому признаку их дел€т на три группы. —уществуют также дальтоники, которые не различают все три цвета. јльбинизм также достаточно распространенное наследственное заболевание. ќно возникает как результат отсутстви€ в организме фермента тирозиназы, а именно этот фермент синтезирует меланин, вещество, отвечающее за окраску тканей. јльбинизм распростран€етс€ не только на органы зрени€, но и на кожу. јльбиносы часто плохо вид€т, страдают косоглазием, отсутствием пигментации радужки, предрасположены к частичной или полной потере зрени€.

¬ св€зи со значительными достижени€ми последних дес€тилетий в изучении проблемы наследственности все большее внимание удел€етс€ роли факторов среды в происхождении врожденных наследственных и ненаследственных (перистатических) аномалий глаз. јномалии, развившиес€ на почве вредоносного воздействи€ средовых факторов, в насто€щее врем€ сильно превалируют над аномали€ми спонтанно-генетического происхождени€, которым длительный период времени до этого отводилась главенствующа€ роль.

‘акторы среды, окружающей зародыш и плод, способны вызывать развитие таких аномалий (фенокопий), которые внешне невозможно отличить от истинно наследственных аномалий глаза.
—реди таких факторов особо важна€ роль принадлежит различным видам ионизирующей радиации (лучи рентгена, ради€, 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.
“акже нередко наблюдаетс€ поражение органа зрени€, в котором участвует наследственный компонент в сочетании с другими глазными или общими заболевани€ми. “ак, при мукополисахаридозе (наследственном поражении соединительной ткани) могут возникнуть и нарушени€ зрени€. Ќекоторые врожденные нарушени€ обмена веществ сопровождаютс€ дистрофией сетчатки, глаукомой, атрофией зрительного нерва, катарактой. ќдна и та же форма заболевани€ может быть и наследственной, и ненаследственной. ¬есьма неблагопри€тное воздействие на орган зрени€ развивающегос€ плода оказывают инфекционные и вирусные заболевани€ матери (краснуха, ветр€на€ оспа, грипп), различного рода интоксикации во врем€ беременности (алкоголь, некоторые снотворные препараты). ќсобенно чувствителен глаз плода к повреждающему агенту на 3 Ч 7-й неделе беременности.

Ћечение врожденных и наследственных аномалий возможно во многих случа€х при помощи соответствующих хирургических вмешательств, устран€ющих имеющийс€ анатомический и функциональный недостаток.
¬ р€де других случаев возможно предупредить развитие глазного заболевани€, а иногда спасти и самую жизнь ребенка при раннем применении определенных лечебных меропри€тий. —юда относ€тс€, 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. –аннее назначение соответствующей диеты при галактоземии, диеты и инсулина при сахарном диабете в таких случа€х €вл€ютс€ критически важными и могут избавить от серьезных последствий дл€ здоровь€ и зрени€ плода.
Ѕудущим родител€м, в семье у которых заболевание органа зрени€ передаетс€ из поколени€ в поколение или семье, у которых первый ребенок родилс€ с дефектом зрени€ необходимо об€зательно пройти консультацию у врача-генетика.  роме подробного изучени€ возможных аномалий генетического развити€ и наследственных факторов с целью их предупреждени€, в некоторых случа€х дл€ уточнени€ диагноза и характера наследственного дефекта прибегают к специальным методам исследовани€ Ч например, цитогенетическому или биохимическому. “акой ответственный подход позволит предупредить заболевани€, передающиес€ генетически.


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GMT - Master II
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[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-18-ct-gult-gull-m116718ln-0001-p-700.html][img]http://www.rolexwatchesforsale.top/no/images/_small//rolex_fake/GMT-Master-II/Rolex-GMT-Master-II-Watch-18-ct-yellow-gold-5.jpg[/img]Rolex GMT - Master II Watch : 18 ct gult gull - M116718LN - 0001[/url]<a href="http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-18-ct-gult-gull-m116718ln-0001-p-700.html">Rolex GMT - Master II Watch : 18 ct gult gull - M116718LN - 0001</a>modell saken modell saken Oyster , 40 mm , gult gull Oyster arkitektur Monoblokk...NOK 84,923 NOK 1,959Du f√•r 98% avslag<a href="http://www.rolexwatchesforsale.top/no/gmt-master-ii-c-31.html?products_id=700&action=buy_now&sort=20a"><img src="http://www.rolexwatchesforsale.top/no/includes/templates/polo/buttons/norwegian/button_buy_now.gif" alt="Kj√Єp n√•" title=" Kj√Єp n√• " width="108" height="30" class="listingBuyNowButton" /></a>
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-18-ct-gult-gull-m116718ln-0002-p-793.html][img]http://www.rolexwatchesforsale.top/no/images/_small//rolex_fake/GMT-Master-II/Rolex-GMT-Master-II-Watch-18-ct-yellow-gold-7.jpg[/img]Rolex GMT - Master II Watch : 18 ct gult gull - M116718LN - 0002[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-18-ct-gult-gull-m116718ln-0002-p-793.html]Rolex GMT - Master II Watch : 18 ct gult gull - M116718LN - 0002[/url]modell saken modell saken Oyster , 40 mm , gult gull Oyster arkitektur Monoblokk...NOK 190,766 NOK 1,933Du f√•r 99% avslag[url=http://www.rolexwatchesforsale.top/no/gmt-master-ii-c-31.html?products_id=793&action=buy_now&sort=20a][img]http://www.rolexwatchesforsale.top/no/includes/templates/polo/buttons/norwegian/button_buy_now.gif[/img]Kj√Єp n√•[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-904l-st%C3%A5l-m116710blnr-0002-p-619.html][img]http://www.rolexwatchesforsale.top/no/images/_small//rolex_fake/Rolex-Watches/Rolex-GMT-Master-II-Watch-904L-steel-M116710BLNR-3.jpg[/img]Rolex GMT - Master II Watch : 904L stål - M116710BLNR - 0002[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-904l-st%C3%A5l-m116710blnr-0002-p-619.html]Rolex GMT - Master II Watch : 904L st√•l - M116710BLNR - 0002[/url]modell saken modell saken Oyster , 40 mm , st√•l Oyster arkitektur Monoblokk midt...NOK 162,441 NOK 1,786Du f√•r 99% avslag[url=http://www.rolexwatchesforsale.top/no/gmt-master-ii-c-31.html?products_id=619&action=buy_now&sort=20a][img]http://www.rolexwatchesforsale.top/no/includes/templates/polo/buttons/norwegian/button_buy_now.gif[/img]Kj√Єp n√•[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-904l-st%C3%A5l-m116710ln-0001-p-701.html][img]http://www.rolexwatchesforsale.top/no/images/_small//rolex_fake/GMT-Master-II/Rolex-GMT-Master-II-Watch-904L-steel-M116710LN-3.jpg[/img]Rolex GMT - Master II Watch : 904L stål - M116710LN - 0001[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-904l-st%C3%A5l-m116710ln-0001-p-701.html]Rolex GMT - Master II Watch : 904L st√•l - M116710LN - 0001[/url]modell saken modell saken Oyster , 40 mm , st√•l Oyster arkitektur Monoblokk midt...NOK 87,446 NOK 1,847Du f√•r 98% avslag[url=http://www.rolexwatchesforsale.top/no/gmt-master-ii-c-31.html?products_id=701&action=buy_now&sort=20a][img]http://www.rolexwatchesforsale.top/no/includes/templates/polo/buttons/norwegian/button_buy_now.gif[/img]Kj√Єp n√•[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-gul-rolesor-kombinasjon-av-904l-st%C3%A5l-og-18-ct-gult-gull-m116713ln-0001-p-699.html][img]http://www.rolexwatchesforsale.top/no/images/_small//rolex_fake/GMT-Master-II/Rolex-GMT-Master-II-Watch-Yellow-Rolesor-3.jpg[/img]Rolex GMT - Master II Watch : Gul Rolesor - kombinasjon av 904L stål og 18 ct gult gull - M116713LN - 0001[/url]
[url=http://www.rolexwatchesforsale.top/no/rolex-gmt-master-ii-watch-gul-rolesor-kombinasjon-av-904l-st%C3%A5l-og-18-ct-gult-gull-m116713ln-0001-p-699.html]Rolex GMT - Master II Watch : Gul Rolesor - kombinasjon av 904L st√•l og 18 ct gult gull - M116713LN - 0001[/url]modell saken modell saken Oyster , 40 mm , st√•l og gult gull Oyster arkitektur ...NOK 170,140 NOK 1,708Du f√•r 99% avslag[url=http://www.rolexwatchesforsale.top/no/gmt-master-ii-c-31.html?products_id=699&action=buy_now&sort=20a][img]http://www.rolexwatchesforsale.top/no/includes/templates/polo/buttons/norwegian/button_buy_now.gif[/img]Kj√Єp n√•[/url]

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Bombas del dedo del pie puntiagudo Jimmy Choo Allure Plata Espejo de cuero [ee4f]
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[url=http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html][img]http://www.jimmychoooutlets.cn/es/images/images/j225.jpg[/img]Bombas del dedo del pie puntiagudo Jimmy Choo Allure Plata Espejo de cuero [ee4f][/url]
Bombas del dedo del pie puntiagudo Jimmy Choo Allure Plata Espejo de cuero [ee4f]
€963.48 €145.08Ahorre: 85% descuento
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Size
US10=UK8=EUR42
US5=UK2.5=EUR35
US6.5=UK4=EUR37
US6=UK3.5=EUR36
US7.5=UK5=EUR38
US8.5=UK6=EUR39
US9.5=UK7=EUR41
US9=UK6.5=EUR40

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Description[/b]
La forma del dedo del pie bastante y tacón bajo las bombas hacen estos perfectos para todos los días. Talón mide 50mm / 2 [url=http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html][img]http://www.jimmychoooutlets.cn/es/images/images/j225.jpg[/img]Jimmy Choo Cósmico lagarto en relieve Tan [9e9f][/url] [url=http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html][img]http://www.jimmychoooutlets.cn/es/images/images/j226.jpg[/img]Jimmy Choo Cósmico lagarto en relieve Tan [9e9f][/url] [url=http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html][img]http://www.jimmychoooutlets.cn/es/images/images/j227.jpg[/img]Jimmy Choo Cósmico lagarto en relieve Tan [9e9f][/url] <a href="http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html" ><img src="http://www.jimmychoooutlets.cn/es/images/images/j228.jpg"></a> [url=http://www.jimmychoooutlets.cn/es/bombas-del-dedo-del-pie-puntiagudo-jimmy-choo-allure-plata-espejo-de-cuero-ee4f-p-503.html][img]http://www.jimmychoooutlets.cn/es/images/images/j229.jpg[/img]Jimmy Choo Cósmico lagarto en relieve Tan [9e9f][/url]
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mreromicab Holland
07.02.2020 22:16
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A Champs
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Legal requirements and Order
The pup:
Convict - paper prisoner suit or fruit jumpsuit, imitation handcuffs.
The girl's:
Policeman - orange shirt, dark colored pants, police arrest cap, police arrest badge, Billy pub, handcuff major.
Traditional Vacation
The pup:
Holiday - high in volume Hawaiian tee shirt, shorts, shoe or tennis shoes, sunglasses, dslr camera, flower lei.
The girl's:
Hula Dancer -- hula skirts, bikini very best, flower lei, shoe, long ebony wig, plant for curly hair.
50s Legends
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Little Elvis -- black imitation leather jacket, black t-shirt or paper striped t-shirt, dark colored jeans, dark colored loafers, white colored socks, eyewear, hair really should be slick and additionally full, including a mini cassette recorder having "Jailhouse Rock" (or "Hound Dog") pre-recorded to be able to play an individual's latest hit single for the people.
Different Elvis appearance:50s times rocker meet, tie, white colored bucks, sunglasses
The girl's:
Marilyn Monroe -- 50s hottie dress (or along with white Marilyn dress), knocks out, platinum blond hairpiece with Marilyn coiffure, glam makeup to search like Marilyn, cubic zirconia diamonds (optional).
1960s A-Go-Go
The pup:
Hippie -- bell lower part pants, tie-dyed tshirt, head piece, fringe jacket, peace warning sign necklace or possibly happy confront button, extensive hair wig.
The girl's:
Visit Go Dancer -- colorful 1960s style micro skirt or maybe dress, black or white knee substantial boots, make use of hairstyle as well as bob slice wig, white colored gloves (optional).

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