海外マルファン情報

米国マルファン症候群患者団体The Marfan Foundationからの情報を中心に、マルファン症候群や関連疾患についての海外情報を翻訳して発信します。

マルファン症候群の基礎知識 ~呼吸器~


Marfan 101 9/12/18 (Virtual Medical Symposium Series)

 

15:16-17:48

 

Pulmonary findings are also frequent.

呼吸器の所見も頻繁に見られます。

 

One of the most when the most frequently discussed one findings is a pneumothorax.This is a sudden lung collapse. If you have one, you'll know because it's pretty sudden and onset very severe chest pain and difficulty breathing. This occurs again suddenly and without necessarily any type of impact or any type of injury. It just occurs spontaneously.

最もよく見られる所見が気胸で、突然の肺虚脱(肺のしぼみ)が起こります。急な胸の激痛で呼吸ができなくなります。突然再発し、必ずしも気付くわけではなく、損傷が起きるとも限りません。何の前触れもなく発症します。

 

Other findings in the lungs because the lungs are also made up of connective tissue which is weak and doesn't hold that cells and tissues well together; they're the lung buds, which allow us to ventilate and oxygenate our blood and allow for air exchange when we breathe in and out, can rather than look like full bunches of grapes, they become kind of cystic and start to lose the walls between them. This leads to hyper expansion of the lungs and can also lead to breathing difficulties.

肺も結合組織でできていますが、マルファンの方の結合組織は、もろく、細胞や組織をしっかりと固定しておくことができません。所見が見られるのは、肺胞です。肺胞では、呼吸により、二酸化炭素との酸素の交換が行われます。(肺気腫になると、)肺胞の間の壁が壊され、たわわに実ったブドウの房というよりも、一つの嚢(ふくろ)のようになります。この結果、肺が大きく広がり、呼吸困難へとつながります。

 

And finally because of abnormalities not only in the connective tissue but also other abnormalities such as the high palate, dental crowding, chest wall abnormalities, individuals can have problems with sleeping.

最後になりますが、結合組織以外でも、高口蓋や叢生、胸壁異常により、睡眠に影響が出ることがあります。

 

Snoring is one example, in which when the head is laid flat, the airway falls back and actually causes obstruction during the breathing cycle and closes the upper airway because of collapse of the tissues to the backside of the airway. This can lead to snoring and this can lead to a frequent arousal during the evening or during the night hours so that someone really doesn't get very good and regular sleep.

その一例がいびきです。頭部を水平にして仰向けになると、呼吸サイクル中に気道が狭まり上気道が閉塞することで、いびきが発生します。組織が気道の後ろ側に落ち込むことが原因です。いびきのために睡眠中に何度も目が醒め、質の良い規則的な睡眠が得られなくなるのです。

 

Other problems can be related to, again, the skeletal and oral abnormalities that we see with Marfan syndrome.

その他に関連してくる問題としては、繰り返しになりますが、マルファン症候群に見られる骨格、口腔異常があります。

 

Sleep apnea or disorders of the sleep are very important not only for the effects that they have on our breathing but also for the effects that they have secondarily to how we perform our daily activities, what our ability to focus, our ability to perform during school, during work, and our mood changes and it can also have effects on the aorta and on the interthoracic pressure and there are currently studies being performed looking at other physiologic responses related to sleep disorder breathing.

睡眠時無呼吸あるいは睡眠障害は、呼吸に影響を与えるだけではなく、日常活動や集中力、学業、仕事、気分などに二次的な影響が及びます。また、大動脈や胸腔内圧に影響が出ることもあります。現在、睡眠時の呼吸障害に関する、その他の生理学的反応についての研究が行われています。

 

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。