Living a Life with LDS (September 12, 2020) E3 Summit
1:12:50-1:17:01
Q:
A couple questions around puberty. One is about, "Are there is, there anything to suspect that a late onset of puberty would be caused or be an effect of LDS?" and then more questions about "How does puberty affect teens with LDS?" "Does it significantly lower muscle tone, decreased joint stability, aortic changes, other impacts?"
思春期に関し、「LDSが原因で思春期の開始が遅れることがあるか？」「LDSの若者には思春期による影響があるか？」「思春期による筋肉の張りの低下、関節の不安定化、大動脈の変化などの影響はあるか？」といった質問をいただいています。
A:
So "Does LDS influence the timing of puberty?" I think in general the answer is no that we each are programmed to start puberty at a certain time. Often if you talk to parents of a child their experience as to when they went through puberty will predict when their child will go through puberty. Young women tend to go through puberty at an earlier age than young men as a, as a broad rule, you know.
LDSによる思春期の開始時期への影響ですが、一般にはないといえます。思春期が始まる時期は決まっており、親が思春期を迎えた年齢を確認すれば、子どものケースも予測できます。大まかなルールとして、女子の方が男子よりも早く思春期を迎えます。
I think the exception about, might LDS in some way influence puberty would include children that have severe gastrointestinal issues and have a really severe problem putting on weight. It's known that you need a certain body mass and particularly fat mass to have the right mix of hormones that, you know, will initiate puberty so it is possible to have delayed puberty on the basis of a really, really low body mass and again that's typically only seen in the subset of children that have severe gastrointestinal issues.
LDSの子どもにおいて思春期による影響があるとすれば、重篤な胃腸疾患があり、体重増加に大きな問題を抱えている場合です。複数のホルモンが適量分泌されることで、思春期が始まります。そのためには一定の体重、具体的には脂肪が一定量必要となるのですが、あまりに体重が少ない場合には脂肪が足りず、思春期の開始が遅くなる可能性があります。繰り返しになりますが、こうしたケースがみられるのは、一般に重篤な胃腸疾患がある場合に限られます。
"How can puberty influence features severity manifestations of Loeys-Dietz syndrome?" The answer to that is yes. We know, for example, that many of the skeletal features of connective tissue disorders like Loeys-Dietz syndrome and Marfan syndrome are driven by growth, so as you go through the rapid growth spurt that's associated with puberty that can make chest wall deformity worse that can cause an acceleration of curvature of the spine, for example, you know.
LDSの症状に対する思春期の影響はあります。LDSやMFSなどの結合組織疾患における骨格的特徴の多くは成長によって引き起こされます。思春期には成長が急激に進むためです。それにより、胸骨の変形や側弯症などが一層進行することになります。
Also around the time of puberty there's the release of hormones that we've already talked about, can in, at least in some circumstances influence blood vessels and how they perform. Also around the time of puberty blood pressure often goes up so it's possible that a higher blood pressure might lead to some acceleration of cardiovascular issues.
先ほどお話ししましたが、思春期には複数のホルモンが分泌されますので、それにより血管とその機能に影響が出る場合があります。思春期には血圧が上昇することが多く、それにより心血管系の問題が悪化する可能性もあります。
And it's also known around the puberty that around the time of puberty that some behaviors change, you know, young boys, for example, might start getting really interested in heavy weight lifting, which is something, that might also influence how blood vessels perform
特に男子ですが、思春期には行動の変化もみられます。例えば、ウェイトリフティングに興味を持つかもしれません。それにより血管に影響が出る可能性があります。
So it is a time when we, you know, pay close attention not that we're not paying close attention before but, you know, it, I, I, I certainly wouldn't become more relaxed around the time of puberty. I would certainly be maintaining all the frequent surveillance. That's, that's done throughout childhood.
以上のように思春期には注意が必要となります。もちろんそれ以前はしっかりと診ないというわけではありませんが、思春期に気を抜くことはできません。定期的に経過観察を続けていきますし、小児の間は継続します。
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。
