Eye Issues and Treatment in Marfan Syndrome, Stickler Syndrome, and Other Related Conditions
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Let's go over every one of those. Dislocated ones or "Ectopia Lentis." This is a schematic of I, I walk you through. I think, yeah I do have a, can you see that my cursor, my mouse? Excellent. I just want to make sure.
では、これらの疾患を一つずつ見ていきましょう。まず、水晶体亜脱臼です。この図を使って説明していきます。
So this is a schematic eye. Just imagine, now walk you through. So this is high, this is the front part and the half the back part I chopped it off basically. So the yellow line is a cornea, which is clear so that light goes through from the top going through this dark hole, this aperture, and the dark holes formed, there's a broken line here and you see the orange part. This is iris. Iris gives you the color. Some people have blue eyes. I have brown eyes. That's the color is from.
これは眼の模型です。上から見た図で、こちらが前方で、後ろ半分はカットしてあります。黄色の部分が角膜で、透き通っています。光は上から入り込み、この黒い穴、つまり隙間を通ります。破線があって、オレンジの部分がありますが、オレンジの部分が虹彩です。青い目の方もいますし、私の目は茶色ですが、虹彩が眼の色を決めています。
And go through this lens. Why the lines on the left, the right they're different? I'll go over just a little bit but nonetheless this lens supposed to be clear. Okay?
そして、光はレンズ（水晶体）を通過します。左右分かれていますが、これについては後で説明します。レンズは透明だと考えてください。
So the light go through the cornea, go through the aperture, which is a pupil, the dark hole, go through the clear lense eventually reach the back part of the eye. That's how you see. Very simple.
まとめると、光は角膜から入り、この隙間（瞳孔）を通過し、透明なレンズを抜けて、眼の後方に到達します。
But I want you to take a look at this lens. You can see that this lens is in a pretty precarious position. It's literally suspended in the middle of nowhere just in the eye, in the eye cavity, suspended by and held by those, those stringy structures. Those structures, they are called zonules and those are almost like elastic bands.
レンズに注目してください。非常に不安定な場所にあることがおわかりになると思います。眼の中の何もない空間につながれており、つないでいるひものような構造をチン小帯と呼びます。ゴムバンドと考えて構いません。
They can relax, they can get tensed up. When they are relaxed, the lenses gets flatter. That's on the left side. This will allow you to focus the distance objects, so you can see far away if you relax your zonules and that the lens becomes rounder and this will get accommodated. You hear this accommodation of the eye, that would allow you to read up close.
チン小帯はゆるんだり、縮んだりします。チン小帯がゆるむと、レンズは平坦になります。これが左側の図です。チン小帯がゆるむと遠くのものにピントが合います。チン小帯が縮むとレンズは丸い形状となり、近くのものにピントが合うようになります。
So do me a favor. You can do this now. Look at the computer screen, look at the far in your room. You're not even aware of this. You change the focal point in the milliseconds, so that's thanks to those are the elastic bands, those zonules.
パソコンの画面を見て、部屋の隅を見てください。お気付きにならないと思いますが、数ミリ秒という間に焦点が切り替わります。これはチン小帯の働きによるものです。
They contract, they relax very easily without you even being aware of that, and they provide the structure stability to suspend the lens in the location.
チン小帯は、気付かないうちに伸びたり、縮んだりしながら、レンズを安定した位置に保ちます。
And guess what? Those elastic bands are mainly made of fibrillin-1, which is encoded by the gene FBN1. So now you got it. So you have the mutation in the FBN1 gene and the zonules can be broken. They're not doing their job they're for. The lens can be dislocated. That's how you get it dislocated lens.
チン小帯は主にフィブリリン1でできており、フィブリリン1は、FBN1遺伝子によりコードされています。お分かりかと思いますが、FBN1遺伝子に変異があると、チン小帯が切れてしまうことがあります。そうなると、本来の役割を果たせなくなり、レンズが外れてしまいます。これが水晶体亜脱臼が起こるしくみです。
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
