Ke nānā mākou i ka ʻōpū o kahi pēpē hānau hou, ʻike pinepine mākou he laumania a piha hoʻi, ʻeā? Akā, i kekahi manawa, me ka manaʻo ʻole ʻia, ʻike mākou ua ʻāwili loa ka ʻili ma ka ʻōpū o ke pēpē, e like me ka prune. Ke ʻike nei i kēia, ma ke ʻano he makuahine a makua paha, e makaʻu paha ʻoe a pūʻiwa hoʻi. "Auē, he aha ka mea i hana i kaʻu pēpē?" Manaʻo paha ʻoe. He maʻi laha ʻole kēia. I kēia lā, ke kamaʻilio nei mākou e pili ana i ke ʻano laha ʻole e hiki ai ke hana i kēia, ʻo ia hoʻi ʻo Prune Belly Syndrome . Mai hopohopo, e kamaʻilio mākou e pili ana i nā mea āpau me ka maʻalahi a me ka maopopo.
I ka pōkole, he aha ka Prune Belly Syndrome?
He maʻi hānau laha ʻole ka Prune Belly Syndrome. Loaʻa kona inoa no ka mea ua like ka ʻili ma ka ʻōpū o kahi pēpē me ka prune. ʻEkolu mau hōʻailona nui. ʻO ia ke kumu i kapa ʻia ai i kekahi manawa ʻo 'Triad Syndrome'. Ua kapa ʻia hoʻi ʻo 'Eagle-Barrett Syndrome' no ka hoʻohanohano ʻana i nā kauka nāna i ʻike.
ʻO kēia mau hiʻohiʻona nui ʻekolu:
1. Nā ʻiʻo ʻōpū nāwaliwali a ʻaʻohe paha: Loaʻa i ko kākou ʻōpū kahi papa o nā ʻiʻo e kōkua ana i ka mālama ʻana i kona ʻano a pale i nā ʻōpū i loko, e like me nā ʻōpū. I nā pēpē me kēia maʻi, ʻaʻole i hoʻomohala pono ʻia kēia mau ʻiʻo. I kekahi manawa ʻaʻole lākou i hoʻokumu iki ʻia. ʻO ia ke kumu e ʻike ʻia ai ka ʻili ma ka ʻōpū he ʻāwili a palupalu.
2. Nā testicles i iho ʻole: I loko o kahi pēpē kāne, aia nā testicles ma waho o ke kino i loko o ka scrotum. Eia nō naʻe, i nā pēpē me kēia maʻi, aia nā testicles i loko o ka ʻōpū. ʻAʻole lākou i iho i lalo.
3. Nā pilikia o ka ʻōnaehana mimi: ʻO kēia ka ʻāpana koʻikoʻi loa o ke kūlana. ʻAʻole i hoʻomohala piha ʻia nā puʻupaʻa o ka pēpē, ka ʻōpū mimi, a me nā ʻōmole e hoʻopili ai iā lākou (ureters). Hiki i kēia ke hana i nā pilikia koʻikoʻi me ka mimi ʻana.
Ma waho aʻe o kēia mau hōʻailona nui ʻekolu, hiki i kekahi mau pēpē ke loaʻa nā pilikia like ʻole me ka puʻuwai, nā māmā, nā ʻōpū, a me ka ʻōnaehana iwi.
Pehea ka maʻamau o kēia ʻano?
He maʻi laha ʻole kēia. Hoʻopilikia ia ma kahi o hoʻokahi i loko o 30,000 a 40,000 mau pēpē hānau hou. ʻO ka mea kūikawā, ʻo 95% o kēia mau hihia i hōʻike ʻia i nā keikikāne . ʻO ia no ka mea, ʻo ka pilikia o ka testicle i iho ʻole i lalo a mākou i kamaʻilio ai ma mua e pili wale ana i nā keikikāne.
He aha nā hōʻailona e hiki ke kōkua iā ʻoe e ʻike inā loaʻa kēia maʻi i kāu pēpē?
Ua ʻōlelo ʻia he ʻoi aku ma mua o 50 mau hōʻailona o ka Prune Belly Syndrome. Eia nō naʻe, hiki ke ʻokoʻa kēia mau hōʻailona mai kēlā pēpē a i kēia pēpē. Hiki i kekahi mau pēpē ke hoʻopilikia nui ʻia, a ʻo kekahi ʻaʻole paha i koʻikoʻi loa. E nānā kāu kauka i kāu pēpē a hana i nā hoʻokolohua e pono ai e hōʻoia i kēia.
E nānā kākou i ka papa ma lalo nei e ʻike ai i nā hōʻailona maʻamau.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| ʻIli ʻāwili ma ka ʻōpū | Ma muli o ka nāwaliwali a ʻaʻohe paha o nā ʻiʻo o ka ʻōpū, hiki i ka ʻili ke luʻuluʻu a ʻike ʻia he ʻūmiʻi e like me ka hua waina maloʻo. |
| Loaʻa ka ʻōpū nui | No ka mea, ʻaʻohe ʻiʻo i loko o ka ʻōpū, me he mea lā ua hoʻokuke ʻia i waho nā mea e like me nā ʻōpū i loko. I kekahi manawa, ʻike ʻia nā ʻōpū wili ma o ka ʻili lahilahi. |
| Nā Testicles i iho ʻole | I nā pēpē kāne, noho kekahi a i ʻole nā testicles ʻelua i loko o ka ʻōpū me ka ʻole o ka iho ʻana i loko o ka scrotum. |
| Nā pilikia o ka ʻōnaehana mimi | Nā pilikia koʻikoʻi e like me ka hoʻonui ʻia ʻana o ka ʻōpū mimi a me nā puʻupaʻa, ka paʻa ʻana o ka urethra, a me ka reflux vesicoureteral. |
| Nā pilikia o ka ʻōnaehana hoʻoheheʻe | Nā pilikia ʻōpū a me nā kūlana e like me ka constipation. |
| Nā loli i loko o ka ʻōnaehana iwi | Nā kūlana e like me ka scoliosis a i ʻole ka wāwae kalapu. |
| Nā pilikia o ka puʻuwai | Ka maʻi puʻuwai hānau a i ʻole kekahi mau loli i ke ʻano o ka puʻuwai. |
No ke aha e hana ai kēia i nā pēpē?
ʻO kaʻoiaʻiʻo, ʻaʻole i loaʻa i nā kauka ke kumu pololei o kēia, akā aia kekahi mau manaʻo.
Manaʻo kekahi mau kauka ua hoʻokumu ʻia kēia ʻano ma muli o kahi ʻano ʻē i loko o ka ʻōpū mimi e ulu ana i ka wā e ulu ana ka pēpē i loko o ka ʻōpū. ʻO ia hoʻi, i ka wā e ālai ʻia ai ke ala mimi, hōʻiliʻili nā mimi a pau i loko o ka ʻōpū mimi a pehu loa. I ka ulu ʻana o ka ʻōpū mimi ma kēia ʻano, hoʻopilikia ia i ka ulu ʻana o nā ʻiʻo o ka ʻōpū.
Manaʻo kekahi poʻe e kū mai kēia pilikia ma muli o ka ulu pono ʻole ʻana o nā ʻiʻo ʻōpū i ka wā mua. Wahi a kekahi poʻe, ma muli paha ia o ke kumu hoʻoilina. Ua hōʻike ʻia hoʻi kēia ʻano ma waena o nā hoahānau. Eia kekahi, ʻike pū kekahi mau keiki me nā ʻano chromosomal e like me ka 'Trisomy 18 (Edwards Syndrome)' a me ka 'Trisomy 21 (Down Syndrome)' i kēia Prune Belly Syndrome.
He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?
ʻO ke koʻikoʻi o ka Prune Belly Syndrome e pili ana i nā pilikia ʻē aʻe e pili ana, ʻoiai ka nui o ka hōʻino ʻia o ka ʻōnaehana mimi.
ʻO nā pilikia maʻamau:
- Paʻa ʻōpū: ʻO nā ʻiʻo ʻōpū nāwaliwali e paʻakikī ai ke kaomi ikaika e hele i ka ʻōpala.
- Nā kīnā o ka iwi: nā mea e like me ke kuʻe ʻana o ka ʻūhā, nā kīnā o nā lima, nā manamana lima, a me nā manamana wāwae paha.
- Nā maʻi pinepine o ke ala mimi: Hiki ke loaʻa maʻalahi nā maʻi no ka mea ʻaʻole i hoʻokahe pono ʻia ka mimi. Hiki i kēia mau maʻi ke hōʻino i nā puʻupaʻa.
- ʻAʻohe keiki: ʻO ka hiki ʻole o nā testicles ke iho i lalo hiki ke hoʻopilikia i ka hiki ke loaʻa nā keiki i ka wā e hiki mai ana. Aia kekahi pilikia o ka maʻi ʻaʻai testicular.
- ʻO ka Hypoplasia Pulmonary: ʻAʻole paha e ulu pono nā māmā ma muli o ka nele o kahi i loko o ka ʻōpū.
- Ka Hāʻule Mau ʻana o nā Puʻupaʻa: ʻO kēia ka pilikia koʻikoʻi loa. Hiki i nā pilikia mau me ka ʻōnaehana mimi ke alakaʻi i ka hāʻule mālie ʻana o nā puʻupaʻa.
Pehea e ʻike ʻia ai kēia maʻi?
I ka hapanui o nā hihia, hiki ke ʻike mua ʻia kēia ʻano i ka wā hāpai me kahi scan ultrasound fetal.E kānalua nā kauka i kēia inā hōʻike ka scan he nui loa ka ʻōpū mimi o ka pēpē a i ʻole ua pehu nā puʻupaʻa. Hiki i ka scan ke hōʻike pū ʻaʻohe ʻiʻo ʻōpū o ka pēpē.
Ma hope o ka hānau ʻana o ka pēpē, hiki i ke kauka ke kānalua i kēia ma muli o ke ʻano o waho o ka pēpē, ʻo ia hoʻi ke ʻano o ka ʻōpū me ka ʻāʻī. A laila, hana ʻia nā hoʻokolohua like ʻole e hōʻoia i ka maʻi a hoʻoholo i ka nui o ka hōʻino ʻia o nā kino.
He aha nā hoʻokolohua e hana ʻia?
- Nā hoʻāʻo koko: E aʻo e pili ana i ka hana o nā puʻupaʻa.
- ʻO ke scan Ultrasound: E nānā i ke kūlana o nā ʻōpū i loko o ka ʻōpū, e like me nā puʻupaʻa a me ka ʻōpū mimi.
- X-ray: No ka nānā ʻana i nā pilikia me ka ʻōnaehana iwi.
- CT Scan a i ʻole MRI Scan: E kiʻi i kahi kiʻi maopopo a kikoʻī hoʻi o nā kino.
- VCUG (Voiding Cystourethrogram): He hoʻāʻo X-ray kūikawā kēia. Pili kēia i ka hoʻokomo ʻana i kahi wai kūikawā i loko o ka ʻōpū mimi a me ka nānā ʻana i ke kahe ʻana o ke wai ma o ia mea i ka wā e mimi ai ke pēpē. Kōkua kēia i ka hoʻoholo ʻana inā e kahe ana ka mimi i hope (reflux).
He aha nā lāʻau lapaʻau no kēia?
ʻOkoʻa ka lāʻau lapaʻau no ka Prune Belly Syndrome mai kēlā pēpē a i kēia pēpē. No ka mea, ʻaʻole like ka hopena o kēlā me kēia pēpē. ʻO ka maʻamau, hana pū kahi hui o nā loea, e like me nā pediatricians, nā pediatric urologists, a me nā nephrologists, e hoʻomohala i ka hoʻolālā lapaʻau maikaʻi loa no kāu pēpē.
I ke koho ʻana i ka lāʻau lapaʻau, ua noʻonoʻo ʻia kēia mau mea:
- Ke olakino holoʻokoʻa o ka pēpē.
- Ka makahiki o ka pēpē.
- Ke koʻikoʻi o ka maʻi a me nā ʻōkana i hōʻino ʻia.
- Ka hiki o ke pēpē ke hoʻomanawanui i ke ʻoki kino a me ka lāʻau lapaʻau.
- Kou mau manaʻo a me kou makemake ma ke ʻano he mau mākua.
Pono paha kekahi mau pēpē e hoʻomau i ka lawe ʻana i nā lāʻau antibiotic e pale aku i nā maʻi ʻōpū mimi inā ʻaʻole koʻikoʻi ko lākou mau hōʻailona.
Eia nō naʻe, pono e hana ʻia nā ʻoki he nui i nā pēpē he nui. Ua manaʻo ʻia kēia mau ʻoki e hoʻoikaika i ka paia o ka ʻōpū, hoʻohaʻahaʻa i nā testicles, a hoʻoponopono i nā pilikia me ka ʻōnaehana mimi.
Aia kekahi mau ʻano hana nui i hana ʻia:
- Vesicostomy: ʻO ke ʻoki ʻana i kahi puka liʻiliʻi ma ka ʻōpū lalo i loko o ka ʻōpū mimi. ʻAe kēia i ka mimi e kahe pololei i waho. Hiki i kēia ke hōʻemi i ka hōʻino ʻia o nā puʻupaʻa.
- Orchiopexy: ʻO kēia ke ʻoki ʻana i nā testicles, aia i loko o ka ʻōpū, i lalo i loko o ka scrotum a hoʻopaʻa iā lākou.
- Cystoplasty: He ʻoki kino paʻakikī iki kēia. Pili ia i ke kūkulu hou ʻana i ka ʻōpū mimi.
I kekahi mau hihia koʻikoʻi, inā hāʻule loa nā puʻupaʻa,Pono paha kahi hoʻoili puʻupaʻa.
Pehea lā ka wā e hiki mai ana o kēia mau keiki?
ʻIke wau he mea paʻakikī iki kēia ke lohe. Akā, he mea nui e ʻike i ka ʻoiaʻiʻo. Aia ke ola o kēia mau keiki i ke koʻikoʻi o ka maʻi. ʻO ka mea pōʻino, ma waena o 10% a me 25% o nā pēpē e make ana i loko o ka ʻōpū a make paha mai nā pilikia i loko o nā pule mua o ka hānau ʻana.
Akā, aia kahi ʻaoʻao mālamalama. Ma kahi o 40% o nā keiki me ka Prune Belly Syndrome, ʻoiai he mau pilikia liʻiliʻi me kā lākou ʻōnaehana mimi, mālama lākou i ka hana maʻamau o nā puʻupaʻa a alakaʻi i nā ola maʻamau.
ʻO kekahi 30% paha e pono ai i kahi hoʻoili puʻupaʻa i kekahi manawa o ko lākou ola. ʻO ka mea nui loa, pono i kekahi me kēia maʻi ke mālama ʻia e ke kauka no ke koena o ko lākou ola. He mea nui ka nānā mau ʻana i ka ʻōnaehana mimi e hōʻemi i kekahi pōʻino i nā puʻupaʻa.
Pehea e mālama ai i kāu keiki a i ka manawa hea e ʻike ai i ke kauka?
Inā ua ʻike ʻia kāu keiki me ka Prune Belly Syndrome, ʻo ka mea nui loa, ʻo ia ka loaʻa ʻana o kahi kauka akamai i ʻike i ke ʻano. E wehewehe ʻo ia i nā mea āpau iā ʻoe a alakaʻi iā ʻoe i ke ʻano e mālama ai i kāu keiki.
He mea weliweli loa nā maʻi ʻaʻai mimi (UTI) no kēia mau keiki. No laila, inā ʻike ʻoe i kahi hōʻailona liʻiliʻi loa o kahi maʻi ʻaʻai mimi, pono ʻoe e kāhea koke i kāu kauka.
| Nā hōʻailona e makaʻala ai inā loaʻa kahi maʻi urinary tract | |
|---|---|
| Kuni a i ʻole ke anu | Pono pinepine e mimi |
| Aia ka pono wikiwiki e mimi, akā he liʻiliʻi wale nō ka nui e puka mai ana. | ʻEha a wela paha i ka wā e mimi ai |
| ʻEha o ka ʻōpū haʻahaʻa a i ʻole ke kua | Mimi ʻeleʻele a noe paha |
| He ʻala ikaika a ʻoluʻolu ʻole mai ka mimi | Koko i loko o ka mimi |
Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, mai hoʻokaʻulua. E ʻimi koke i ke kōkua lapaʻau. Eia kekahi, he mea nui e hele i nā keʻena lapaʻau i nā manawa i hoʻonohonoho ʻia a e nānā ʻia kāu keiki, e like me ka ʻōlelo a ke kauka.
Leka Lawe-Home
- He maʻi hānau laha ʻole ka Prune Belly Syndrome e hoʻoulu ai i nā ʻiʻo ʻōpū nāwaliwali, nā testicles i iho ʻole i lalo, a me nā pilikia mimi.
- Ua loaʻa kēia inoa mai ka ʻoiaʻiʻo he ʻano mimino ka ʻili ma ka ʻōpū o ke pēpē e like me ka hua waina maloʻo.
- Hiki ke ʻike pinepine ʻia kēia ʻano i ka wā mua ma o nā scan i ka wā hāpai.
- ʻOkoʻa nā ʻano lapaʻau ma muli o ke kūlana o ka pēpē, a koi pinepine ʻia nā ʻoki kino he nui.
- He mea weliweli loa nā maʻi ʻaʻai mimi no kēia mau keiki. Inā ʻike ʻoe i kahi hōʻailona liʻiliʻi loa o kahi maʻi, e ʻike koke i ke kauka.
- Pono kēia mau keiki i ka nānā a me ka mālama ʻana no ke ola holoʻokoʻa mai nā kauka loea. Me ka mālama pono ʻana, hiki i nā keiki he nui ke ola i kahi ola maikaʻi.
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