He aha ka maʻi maʻi VATER?

Anter

• Hōʻike Overview
• He aha ke kumu?
• He aha nā hōʻailona?
• Nā hemahema vertebral
• Nā hemahema ʻino
• Nā kīnā kīnā ʻole
• Tristoesophageal fistula
• Nā kīnā kīnā ʻole
• Nā hemahema o ka pūpū
• Nā ʻōuli ʻē aʻe
• Pehea ia ʻike?
• He aha nā koho lapaʻau?
• Outlook

Hōʻike Overview

ʻO ka maʻi VATER, i kapa pinepine ʻia ʻo ka hui ʻo VATER, kahi hui o nā hānau kīnā e hana pinepine ʻia. ʻO ka VATER kahi inoa inoa.Kū kēlā me kēia leka i kahi ʻāpana o ke kino i hoʻopili ʻia.

• vertebrae (iwi kuamoʻo)
• anus
• tracheoesophageal (trachea a me ka esophagus)
• hakuʻala (hakuʻala)

Kapa ʻia ka hui ʻo VACTERL inā pili ka puʻuwai (cardiac) a me nā lālā. ʻOiai he mea maʻamau kēia, ʻo VACTERL ka manawa ʻoi aku ka pololei.

E ʻike ʻia me ka hui VATER a i ʻole VACTERL, pono e loaʻa i kahi pēpē nā kīnā hānau ma ʻekolu o kēia mau wahi.

Kākaʻikahi ka hui ʻo VATER / VACTERL. Kuhi ʻia he 1 mai kēlā me kēia 10,000 a 40,000 mau pēpē i hānau ʻia me kēia hui o nā kūlana.

He aha ke kumu?

ʻAʻole maopopo maopopo i nā kauka ke kumu o ka hui ʻana o VATER. Manaʻoʻiʻo lākou i nā hemahema i ka wanaʻao.

Hoʻohui pū ʻia kahi genes a me nā mea pili i ke kaiapuni. ʻAʻole i ʻike ʻia kahi ʻāpana hoʻokahi, akā ua loaʻa i nā kānaka noiʻi kekahi mau kino chromosomal abnormalities a me nā hoʻololi ʻana o nā gen (mutations) e pili ana i kēia ʻano. I kekahi manawa e hoʻopili ʻia ma mua o hoʻokahi kanaka o ka ʻohana like.

He aha nā hōʻailona?

Pili nā ʻōuli i ka hemahema o ka pēpē.

Nā hemahema vertebral

Hiki i ka 80 pākēneka o ka poʻe me ka hui VATER i nā kīnā i nā iwi o ko lākou iwi kuamoʻo (vertebrae). Hiki i kēia mau pilikia ke:

• nalowale iwi i ka iwi kuamoo
• iwi keu i ka iwi kuamoo
• iwi pili kino
• iwi i hoʻopili ʻia
• iwi kuapo (scoliosis)
• iwi ʻaoʻao hou

Nā hemahema ʻino

Ma waena o 60 a me 90 pākēneka o ka poʻe me ka hui VATER i kahi pilikia me ko lākou anus, e like me:

• kahi uhi lahilahi ma luna o ka anus e ālai ana i ka wehe
• ʻaʻohe passageway ma waena o ka lalo o ka ʻōpū nui (pololei) a me anus, no laila ʻaʻole hiki i ka stool ke hele aʻe mai ka ʻōpū mai ke kino.

Hiki i nā pilikia me ka anus ke kumu i nā ʻōuli e like me:

• he ʻōpū pehu
• ka luaʻi
• ʻaʻohe neʻe o ka ʻōpū, a i ʻole he kakaikahi loa o nā ʻōpū

Nā kīnā kīnā ʻole

ʻO ka "C" ma VACTERL no ka "puʻuwai." Hoʻopilikia nā pilikia puʻuwai i 40 a 80 pakeneka o ka poʻe me kēia ʻano. Hiki i kēia mau mea ke:

• Hewa septal Ventricular (VSD). He puka kēia i ka paia e hoʻokaʻawale i nā keʻena ʻākau a hema o ka puʻuwai (ventricles).
• Hewa septal Atrial. ʻO kēia ke hoʻokaʻawale kahi puka i ka paia i nā keʻena kiʻekiʻe ʻelua o ka puʻuwai (atrium).
• ʻO Tetralogy o Fallot. ʻO kēia kahi hui o nā kīnā o ka naʻau ʻehā: VSD, kahi kiwikā aortic hoʻonui ʻia (overriding aorta), hoʻoliʻiliʻi o ka pulmonary valve (pulmonary stenosis), a me ka mānoanoa o ka ventricle ʻākau (hypertrophy ventricular pono).
• ʻO ka maʻi puʻuwai hema hema. ʻO kēia ka manawa ʻaʻole kū pono ka ʻaoʻao hema o ka puʻuwai, e pale ana i ke kahe ʻana o ke koko ma waena o ka puʻuwai.
• Patent ductus arteriosus (PDA). Hana ʻia ʻo PDA ke loaʻa kahi wehe kīnā ʻole i loko o kekahi o nā kīʻaha koko o ka puʻuwai e pale i ke koko mai ka hele ʻana i nā māmā e ʻohi i ka oxygen.
• Transposition o nā aʻa nui. ʻO nā aʻa nui ʻelua i waho o ka puʻuwai i hope (transposed).

Nā ʻōuli o nā pilikia puʻuwai e:

• pilikia hanu
• pokole o ka hanu
• kala polū i ka ʻili
• luhi
• manaʻo puʻuwai puʻuwai
• wikiwiki o ka puʻuwai
• ʻōhumu ka naʻau (kani kani)
• ʻai ʻilihune
• ʻaʻole loaʻa kaupaona

Tristoesophageal fistula

ʻO ka fistula kahi pilina maʻamau ma waena o ka trachea (windpipe) a me ka esophagus (ka paipu e lawe i ka meaʻai mai ka waha a hiki i ka ʻōpū). ʻAʻole pili iki kēia mau hale ʻelua i nā mea āpau. Hoʻopilikia ia i ka ʻai e hele ana mai ka ʻāʻī a i ka ʻōpū, e hoʻohuli i kekahi mau meaʻai i loko o nā akemāmā.

ʻO nā hōʻailona me:

• ka hanu ʻana i ka meaʻai i loko o nā akemāmā
• ʻeha a ʻehaʻeha paha ʻoiai e hānai ana
• ka luaʻi
• kala polū i ka ʻili
• pilikia hanu
• ʻōpū pehu
• loaʻa kaumaha kaumaha

Nā kīnā kīnā ʻole

Ma kahi o 50 pākēneka o ka poʻe me VATER / VACTERL i loaʻa i nā kīnā kīnā ʻole. Hiki i kēia mau mea ke:

• hakuʻala maikaʻi ʻole i hana ʻia (s)
• nā puʻupaʻa i kahi kūpono ʻole
• kahi paʻa o ka mimi mai nā puʻupaʻa
• kope o ka mimi mai ka pūpū i loko o nā puʻupaʻa

Hiki i nā kīnā kīnā ʻole ke kumu i nā maʻi urinary pinepine. Hiki i nā keikikāne ke loaʻa kahi kīnā i ka wehe ʻana o kā lākou peni ma lalo, ma kahi o ka piko (hypospadias).

Nā hemahema o ka pūpū

Hiki i ka 70 pākēneka o nā pēpē me VACTERL i nā hemahema o nā lālā. Hiki i kēia mau mea ke:

• n. manamana lima nalo a maikaʻi ʻole
• manamana hou a manamana wāwae (polydactyly)
• nā manamana lima a me nā manamana wāwae (syndactyly)
• poʻomanaʻo hoʻomohala maikaʻi ʻole ʻia

Nā ʻōuli ʻē aʻe

ʻO nā mea ʻē aʻe, ʻoi aku ka nui o nā ʻōuli o ka hui VATER me:

• ulu lohi
• ka loaʻa ʻole o ke kaupaona
• helehelena kūlike ʻole (asymmetry)
• kīnā o ka pepeiao
• kīnā kīhā
• nā pilikia me ka ʻōpū a i ʻole ka peni

He mea nui e hoʻomaopopo i ka pili ʻole o ka hui VATER / VACTERL i ke aʻo ʻana a i ʻole ka hoʻomohala ʻike.

Pehea ia ʻike?

No ka mea ʻo ka hui VATER kahi hui o nā kūlana, ʻaʻole hiki i kahi hōʻike hoʻokahi ke kuhikuhi iā ia. Hana nā kauka i ka hōʻailona ma muli o nā hōʻailona a me nā ʻōuli maʻi. ʻO nā pēpē me kēia ʻano aia ma ka liʻiliʻi ʻekolu mau kīnā o VATER a i ʻole VACTERL. He mea nui e kāpae i nā syndrome a me nā kūlana ʻē aʻe i hiki ke kaʻanalike i nā hiʻohiʻona me ka hui VATER / VACTERL.

He aha nā koho lapaʻau?

Hoʻokumu ʻia ka mālama ʻana i nā ʻano o nā hānau hānau i pili. Hiki i ke ʻoki kino ke hoʻoponopono i nā hemahema he nui, e like me nā pilikia me ka wehe ʻana o ka anal, nā iwi o ka iwi kuamoʻo, ka puʻuwai, a me nā puʻupaʻa. Hana pinepine ʻia kēia mau kaʻina hana ma hope koke o ka hānau ʻana o ke keiki.

Ma muli o ka pili ʻana o ka hui VATER i nā ʻōnaehana o ke kino, mālama kekahi mau kauka ʻokoʻa iā ia, me kahi:

• cardiologist (nā pilikia puʻuwai)
• gastroenterologist (GI tract)
• loea orthopedic (iwi)
• urologist (nā puʻupaʻa, nā pūpū, a me nā ʻāpana ʻē aʻe o ka ʻōnaehana urinary)

Pono nā keiki me ka hui ʻo VATER i ka manawa a pau i ka mālama ʻana a me ka mālama ʻana e pale ai i nā pilikia e hiki mai ana. Pono paha lākou i kōkua mai nā loea e like me ka mea hoʻoponopono kino a me ka mea hoʻoponopono ʻoihana.

Outlook

Aia ka nānā i nā ʻano kīnā o ke kanaka, a pehea e mālama ʻia ai kēia mau pilikia. ʻO ka manawa pinepine ka poʻe me ka hui ʻo VACTERL e loaʻa nā hōʻailona ma ko lākou ola. Akā me ka lapaʻau kūpono, hiki iā lākou ke alakaʻi i ke ola olakino.