He aha ka Klippel-Trenaunay Syndrome? Nā Hōʻailona, Nā Kumu, a me ka Lapaʻau

ʻO ka Klippel-Trenaunay syndrome he maʻi maʻamau ʻole ia e pili ana i ke ʻano o ka ulu ʻana o nā kīʻaha koko a me nā pūnaewele ma mua o ka hānau ʻana. Maʻamau, ʻekolu mau ʻano nui: kahi hōʻailona hānau port-wine, nā veins nui, a me ka ulu nui ʻana o nā iwi a me nā pūnaewele palupalu, maʻamau ma kahi lima hoʻokahi.

Hana ʻia kēia maʻi i ka wā o ka ulu mua ʻana i loko o ka ʻōpū i ka wā ʻaʻole i hana pono nā kīʻaha koko. ʻOiai paha e lohe ʻia he mea kaumaha, nui nā kānaka me ka Klippel-Trenaunay syndrome e ola ana i nā ola piha a hana nui me ka mālama pono a me ka hoʻokele.

He aha ka Klippel-Trenaunay Syndrome?

ʻO ka Klippel-Trenaunay syndrome, i kapa pinepine ʻia ʻo KTS, he maʻi ia kahi e ulu ai nā kīʻaha koko i kahi ʻano ʻokoʻa ma mua o ka maʻamau. Hoʻopili kēia i ke ʻano o ka holo ʻana o ke koko ma nā ʻāpana o kou kino, ʻoi aku ka maʻamau ma ka lima a i ʻole ka wāwae hoʻokahi.

Loaʻa i ka syndrome kona inoa mai nā kauka ʻekolu i wehewehe mua iā ia i ka makahiki 1900. ʻO ia ka mea a nā kauka i kapa ai ʻo ka “vascular malformation syndrome,” ʻo ia hoʻi, ua hana ʻia nā kīʻaha koko i kahi ʻano maʻamau ʻole i ka wā o ka ulu ʻana.

Hānau ʻia ka hapa nui o nā kānaka me KTS me ia, a ʻaʻole ia e hoʻoili ʻia mai nā mākua i nā keiki i ka hapa nui o nā hihia. Hoʻopili ka maʻi ma kahi o 1 i 100,000 mau kānaka, e hana ana iā ia he mea maʻamau ʻole akā ʻaʻole i lohe ʻole ʻia.

He aha nā Hōʻailona o ka Klippel-Trenaunay Syndrome?

ʻIke pinepine ʻia nā hōʻailona o KTS i ka hānau ʻana a i ʻike ʻia paha i ka wā kamaliʻi. E ʻike pinepine ʻoe i ka hui ʻana o nā loli o ka ʻili, nā pilikia vein, a me nā ʻokoʻa i ka nui o ka lima.

Eia nā hōʻailona nui āu e ʻike ai:

• Port-wine stain birthmark - kahi māka ʻulaʻula-ʻōniʻoniʻo pālahalaha ma ka ʻili
• Nā veins nui a huli paha āu e ʻike ai ma lalo o ka ʻili
• Hoʻokahi lima i nui aʻe ma mua o kekahi (maʻamau he wāwae a lima paha)
• ʻO ka pehu ʻana ma ka lima i hoʻopilikia ʻia, ʻoi aku hoʻi ma hope o ke kū ʻana a i ʻole ka hana
• ʻEha a ʻeha paha ma ka lima nui
• ʻIli e mahana ana i ka pā ma luna o ka wahi i hoʻopilikia ʻia

ʻIke kekahi poʻe i nā hōʻailona maʻamau ʻole e like me ke kahe koko mai nā veins nui a i ʻole nā maʻi ʻili. Hiki ke ʻokoʻa loa ka nui mai ke kanaka i ke kanaka - loaʻa i kekahi poʻe nā hōʻailona māmā ʻoiai pono kekahi poʻe i ka mālama nui.

He aha nā Kumu o ka Klippel-Trenaunay Syndrome?

Hana ʻia ʻo KTS ma muli o nā loli i ke ʻano o ka ulu ʻana o nā kīʻaha koko i nā wā mua loa o ka hāpai ʻana. Manaʻo nā ʻepekema ua hana ʻia ia ma muli o nā loli genetic i hana ʻia ma ka manawa kūpono i ka wā o ka ulu ʻana, ʻaʻole ma muli o kekahi mea i hana a i ʻole ʻaʻole i hana nā mākua.

Me he mea lā e pili ana ka maʻi i nā pilikia me nā gene e kaohi ana i ke ʻano o ka ulu ʻana a me ka hoʻohui ʻana o nā kīʻaha koko. Hoʻopili kēia mau loli i ka ulu maʻamau o nā veins, nā arteries, a me nā kīʻaha lymphatic i kekahi mau ʻāpana o ke kino.

I ka hapa nui o nā hihia, sporadic ʻo KTS, ʻo ia hoʻi, hana ia ma ka manawa ma mua o ka hoʻoili ʻia mai nā mākua. Eia naʻe, ua loaʻa nā hihia maʻamau loa kahi e holo ai i nā ʻohana, e hōʻike ana he mea hiki ke pāʻani i kahi kuleana i kekahi manawa nā kumu genetic.

I ka wā e ʻike ai i ke Kauka no ka Klippel-Trenaunay Syndrome?

Pono ʻoe e ʻike i ke kauka inā ʻike ʻoe i kekahi hui o nā hōʻailona maʻamau - kahi hōʻailona hānau port-wine, nā veins nui i ʻike ʻia, a me nā ʻokoʻa i ka nui o ka lima. Hiki i ka loiloi mua ke kōkua i ka pale ʻana i nā pilikia a hoʻomaikaʻi i ka maikaʻi o ke ola.

E ʻimi i ka mālama lapaʻau koke inā ʻike ʻoe i:

• ʻEha nui a koke i ka lima i hoʻopilikia ʻia
• Nā hōʻailona o ka maʻi e like me ka mahana, ka ʻulaʻula, a i ʻole ka mahana
• Ke kahe koko mai nā veins nui
• Paʻakikī ke hele a i ʻole ka hoʻohana ʻana i ka lima i hoʻopilikia ʻia
• Hoʻonui koke ʻana i ka pehu

ʻOiai inā he māmā nā hōʻailona, he mea kūpono ke loiloi ʻia e ke kauka e maʻa ana i nā kūlana vascular. Hiki iā lākou ke kōkua iā ʻoe e hoʻomaopopo i nā mea e nānā ai a hana i kahi hoʻolālā e hoʻokele i kou kūlana kikoʻī.

He aha nā Kumu Pōpilikia no ka Klippel-Trenaunay Syndrome?

ʻOiai pinepine ka hana ʻana o KTS ma ka manawa kūpono i ka wā o ka ulu ʻana, ʻaʻohe nui o nā kumu pōpilikia i ʻike ʻia āu e hiki ai ke kaohi. Me he mea lā hana ia ma ka manawa kūpono i ka hapa nui o nā hihia.

Eia naʻe, ua ʻike nā mea noiʻi i kekahi mau ʻano kūpono e ʻike ai:

• ʻO ka hapa nui o nā hihia sporadic me ka ʻole o ka mōʻaukala ʻohana
• Hoʻopili like ia i nā kāne a me nā wahine
• ʻAʻole like ka makahiki o nā mākua i ka hopena o ka pilikia
• ʻAʻole like nā kumu kūlohelohe i ka wā hāpai i kahi kuleana

I nā hihia maʻamau loa kahi e holo ai ʻo KTS i nā ʻohana, hiki i ka loaʻa ʻana o ka mākua me ka maʻi ke hoʻonui iki i ka pilikia. Akā he mea maʻamau ʻole kēia, a ʻaʻole loaʻa i ka hapa nui o nā kānaka me KTS nā lālā ʻohana i hoʻopilikia ʻia.

He aha nā Pilikia Hiki o ka Klippel-Trenaunay Syndrome?

ʻOiai he nui nā kānaka me KTS e ola ana i nā ola maʻamau, he mea nui ia e ʻike i nā pilikia hiki ke hiki iā ʻoe ke nānā i nā hōʻailona hoʻomaopopo a loaʻa i ke kōkua i ka wā e pono ai.

ʻO nā pilikia maʻamau loa e komo pū ana:

• Nā clots koko i nā veins nui (thrombosis)
• Nā maʻi ʻili mai ka holo koko maikaʻi ʻole
• Ke kahe koko mai nā veins nāwaliwali a nui
• ʻEha mau loa mai ka ulu nui ʻana o ka lima
• Paʻakikī ke loaʻa i nā kāmaʻa a i ʻole nā lole kūpono
• Nā pilikia manaʻo mai nā ʻokoʻa i ʻike ʻia

ʻOi aku ka maʻamau ʻole akā ʻoi aku ka pilikia o nā pilikia e komo pū ana i ka pulmonary embolism (blood clot i nā māmā) a i ʻole ke kahe koko nui. Hoʻomohala kekahi poʻe i ka maʻi ʻāʻī i nā hui i hoʻopilikia ʻia e ka ulu nui ʻana o ka lima.

ʻO ka lono maikaʻi, me ka mālama lapaʻau kūpono a me ka nānā ʻana, hiki ke pale ʻia a i ʻole hoʻokele pono ʻia ka nui o kēia mau pilikia. E hana pū kāu hui olakino me ʻoe e hoʻemi i nā pilikia.

Pehea ka Diagnosis ʻana o ka Klippel-Trenaunay Syndrome?

Maʻamau, hoʻomaopopo nā kauka iā KTS ma ka nānā ʻana i kāu mau hōʻailona a me ka mōʻaukala lapaʻau. ʻO ka hui ʻana o ka port-wine stain, nā veins nui, a me ka ulu nui ʻana o ka lima maʻamau e hana i ka diagnosis maopopo.

Hiki i kāu kauka ke hoʻohana i kekahi mau ho'āʻo e loaʻa i kahi kiʻi piha:

• Nānā kino e loiloi i nā birthmarks, nā veins, a me nā ana lima
• Ultrasound e nānā i ke kahe koko a me ke ʻano vein
• MRI a i ʻole CT scans e ʻike i nā kiʻi kikoʻī o nā kīʻaha koko a me nā pūnaewele
• Nā X-rays e nānā i nā ʻokoʻa ulu iwi
• Nā ho'āʻo koko e kāpae i nā maʻi clotting

I kekahi manawa, manaʻo ʻia ka ho'āʻo genetic, ʻoi aku hoʻi inā loaʻa ka mōʻaukala ʻohana o nā kūlana like. Eia naʻe, ʻaʻole loaʻa nā loli genetic kikoʻī i nā hihia a pau o KTS.

He aha ka Lapaʻau no ka Klippel-Trenaunay Syndrome?

Hoʻokumu ka lapaʻau no KTS i ka hoʻokele ʻana i nā hōʻailona a me ka pale ʻana i nā pilikia ma mua o ka ho'ōla ʻana i ka maʻi. E hana kāu hui mālama i kahi hoʻolālā pilikino ma muli o nā hōʻailona e hoʻopilikia nui iā ʻoe.

ʻO nā ala lapaʻau maʻamau e komo pū ana:

• Nā lole komo e hoʻemi i ka pehu a hoʻomaikaʻi i ke kahe koko
• Lapaʻau kino e mālama i ka ikaika a me ka neʻe
• Laser therapy no nā port-wine stains
• Sclerotherapy e pani i nā veins pilikia
• ʻO ka ʻoki kino no nā ʻokoʻa nui o ka lōʻihi o ka lima
• Nā mea hoʻoemi koko inā pilikia ʻoe i nā clots

Hiki i kāu hui lapaʻau ke komo pū me nā loea vascular, nā dermatologists, nā kauka orthopedic, a me nā therapists kino. E hana pū lākou e hoʻoponopono i kāu mau pono kikoʻī a kōkua iā ʻoe e mālama i ka maikaʻi o ke ola.

Pehea ka Mālama Home i ka wā o ka Klippel-Trenaunay Syndrome?

ʻO ka hoʻokele ʻana iā KTS ma ka home e komo pū ana i nā hana i kēlā me kēia lā e kākoʻo ana i ke kahe koko maikaʻi a pale i nā pilikia. Hiki i nā ʻanuʻu liʻiliʻi a mau ke hana i kahi ʻokoʻa nui i kou manaʻo.

Eia nā mea āu e hiki ai ke hana ma ka home:

• E komo i nā lole komo a i ʻole nā sleeves e like me ka mea i ʻōlelo ʻia
• E hoʻokiʻekiʻe i ka lima i hoʻopilikia ʻia i ka wā e hoʻomaha ai
• E noho ikaika me nā hana haʻahaʻa e like me ka ʻauʻau
• E mālama i kou ʻili maʻemaʻe a moisturized
• E pale i ke kū ʻana a i ʻole ka noho ʻana i hoʻokahi kūlana no nā manawa lōʻihi
• E pale i kou ʻili mai nā ʻoki a me nā hōʻeha

E makaʻala i nā loli i kāu mau hōʻailona a mālama i kahi papa maʻalahi inā kōkua. Hiki i kēia ʻike ke waiwai i ka wā o nā kipa kauka a kōkua iā ʻoe e hopu i nā pilikia hiki ke hiki koke.

Pehea ʻoe e hoʻomākaukau ai no kāu Appointmen Kauka?

ʻO ka hoʻomākaukau ʻana no kāu appointment e kōkua iā ʻoe e loaʻa ka nui loa mai kou manawa me ke kauka. Hiki i ka hoʻomākaukau iki ke alakaʻi i ka mālama maikaʻi a me nā pane maopopo i kāu mau nīnau.

Ma mua o kāu kipa, e ʻohi i kēia ʻike:

• Papa inoa o nā hōʻailona o kēia manawa a me ka wā i hoʻomaka ai lākou
• Nā kiʻi e hōʻike ana i nā loli i kou kūlana i ka manawa
• Nā lāʻau a me nā mea hoʻohui o kēia manawa
• Mōʻaukala ʻohana o nā kūlana like
• Nā nīnau e pili ana i nā hana i kēlā me kēia lā a me ke ola

Mai kānalua e nīnau e pili ana i kekahi mea e hopohopo ai ʻoe, mai nā koho lapaʻau a hiki i ka nānā lōʻihi. Makemake kāu hui olakino e kōkua iā ʻoe e hoʻomaopopo i kou maʻi a manaʻo hilinaʻi e pili ana i ka hoʻokele ʻana.

He aha ka Key Takeaway e pili ana i ka Klippel-Trenaunay Syndrome?

ʻO ka Klippel-Trenaunay syndrome he maʻi hoʻokele ia e hoʻopilikia like ʻole i kēlā me kēia kanaka. ʻOiai pono ia i ka nānā mau a me ka mālama, nui nā kānaka me KTS e alakaʻi i nā ola piha a hana nui me ke kākoʻo a me ka lapaʻau kūpono.

ʻO ka mea nui loa ka hana pū ʻana me nā mea hoʻolako mālama olakino akamai e hoʻomaopopo ana i nā kūlana vascular. Hiki iā lākou ke kōkua iā ʻoe e hoʻokele i nā koho lapaʻau, pale i nā pilikia, a hoʻoponopono i nā hopohopo i ka wā e kū mai ai.

E hoʻomanaʻo ʻaʻole i hoʻomaopopo ʻia kou mau palena e ka loaʻa ʻana o KTS. Me ka mālama kūpono, nui nā kānaka e ʻimi i nā ala e hoʻololi a ulu ai i ka wā e hoʻokele pono ai i kā lākou mau hōʻailona.

Nā Nīnau Pinepine e pili ana i ka Klippel-Trenaunay Syndrome

Q1: Inherited anei ka Klippel-Trenaunay syndrome?

I ka hapa nui o nā hihia, ʻaʻole hoʻoili ʻia ʻo KTS a hana ia ma ka manawa kūpono i ka wā o ka ulu ʻana. Eia naʻe, aia nā hihia maʻamau loa kahi e holo ai i nā ʻohana. Inā loaʻa iā ʻoe KTS a ke hoʻolālā nei ʻoe i kahi ʻohana, hiki i ka ʻōlelo aʻo genetic ke hāʻawi i ka ʻike pilikino e pili ana i nā pilikia.

Q2: E ʻoi aku anei ka ʻino o kaʻu mau hōʻailona i ka manawa?

Hiki i nā hōʻailona KTS ke loli i kou ulu ʻana a me kou mau makahiki, akā ʻokoʻa loa kēia mai ke kanaka i ke kanaka. ʻIke kekahi poʻe i nā loli mālie ʻoiai ke noho mau kekahi no nā makahiki. Ke kōkua nei ka nānā mau ʻana me kāu hui olakino e hahai i nā loli a hoʻoponopono i ka lapaʻau e like me ka pono.

Q3: Hiki iaʻu ke hoʻoikaika kino a pāʻani i nā haʻuki me KTS?

Hiki i nā kānaka he nui me KTS ke hoʻoikaika kino a komo i nā haʻuki me nā palekana kūpono. Manaʻo pinepine ʻia nā hana haʻahaʻa e like me ka ʻauʻau, ʻoiai pono paha nā haʻuki hoʻopili i ka noʻonoʻo hou aku. Hiki i kāu kauka ke kōkua iā ʻoe e ʻimi i nā ala palekana e noho ikaika ai ma muli o kāu mau hōʻailona kikoʻī.

Q4: Loaʻa anei kahi lāʻau lapaʻau no ka Klippel-Trenaunay syndrome?

ʻAʻohe lāʻau lapaʻau no KTS i kēia manawa, akā hiki i nā lapaʻau ke hoʻokele pono i nā hōʻailona a pale i nā pilikia. Ke hoʻomau nei ka noiʻi i ka hoʻomaopopo maikaʻi ʻana i ka maʻi a me ka hoʻomohala ʻana i nā ala lapaʻau hou. ʻO ka mea nui ka kōkua ʻana iā ʻoe e ola me ka ʻoluʻolu me ka maʻi.

Q5: Pehea ka hopena o KTS i koʻu ola i kēlā me kēia lā?

ʻO ka hopena o KTS i ke ola i kēlā me kēia lā he ʻokoʻa loa ia ma muli o kāu mau hōʻailona kikoʻī a me ko lākou nui. Hana nui nā kānaka i nā hoʻoponopono maʻalahi e like me ke komo ʻana i nā lole komo a i ʻole ke koho ʻana i nā kāmaʻa kākoʻo a hoʻomau me nā hana maʻamau. Hiki i kāu hui olakino ke kōkua iā ʻoe e ʻike i nā hoʻolālā e hana no kou ola.