Ua ʻāwili ʻia ka ʻōpū o kāu pēpē? ʻO Prune Belly Syndrome anei ia? E makaʻala kākou i kēia.

Ke nānā ʻoe i kāu pēpē hānau hou, ua ʻike anei ʻoe ua ʻāwili ʻia ka ʻili ma kona ʻōpū e like me ka prune? A i ʻole ke ʻano nui loa o kona ʻōpū? He mea maʻamau no kekahi makuahine a makuakāne paha ke makaʻu i ka ʻike ʻana i kēia mau mea. Akā he hōʻailona paha kēia o kahi maʻi laha ʻole i kapa ʻia ʻo Prune Belly Syndrome. Mai hopohopo, e kamaʻilio mākou e pili ana i nā mea āpau me ka maopopo a me ka maʻalahi.

He aha ka Prune Belly Syndrome?

I ka ʻōlelo maʻalahi, he maʻi laha ʻole ka Prune Belly Syndrome i loaʻa i ka wā hānau (congenital). Loaʻa ka inoa mai ka ʻoiaʻiʻo he ʻano wrinkled ka ʻili ma ka ʻōpū o kēia mau keiki a like me ka prune. Hoʻokumu ʻia kēia e ʻekolu mau kumu nui.

1. Nā ʻiʻo ʻōpū nāwaliwali a ʻaʻohe paha: ʻAʻole i ulu piha nā ʻiʻo ʻōpū o ke pēpē, ʻo ia ke kumu e ʻike ʻia ai ka ʻili ma ka ʻōpū he palupalu a he ʻāwili.

2. Nā testicles i iho ʻole: I nā keikikāne, ʻaʻole i iho nā testicles mai ke kino a i loko o ka scrotum kahi e pono ai lākou.

3. Nā pilikia o ka ʻōnaehana mimi: Aia nā pilikia me ke ʻano o ka hana ʻana o ka ʻōnaehana mimi o ka pēpē, ʻo ia hoʻi, nā puʻupaʻa a me ka ʻōpū mimi.

Ma waho aʻe o kēia mau pilikia nui ʻekolu, hiki i kēia maʻi ke hoʻopilikia i nā ʻāpana ʻē aʻe o ke kino o ka pēpē. No ka laʻana, hiki ke hoʻopilikia ʻia ka puʻuwai, nā māmā, nā ʻōpū, a me ka ʻōnaehana iwi. Ua kapa ʻia kēia ʻo Triad Syndrome a i ʻole Eagle-Barrett Syndrome.

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 kupanaha loa, ʻo 95% o nā hihia o kēia maʻi i hōʻike ʻia i nā keikikāne .

He aha nā hōʻailona nui o kēia maʻi?

Aia ma mua o 50 mau hōʻailona o ka Prune Belly Syndrome. Eia nō naʻe, hiki ke ʻokoʻa kēia mau hōʻailona mai kekahi pēpē a i kekahi. E nānā kāu kauka i kāu pēpē a hana i nā hoʻokolohua e pono ai e hōʻoia i ke kūlana.

E nānā kākou i kekahi o nā hiʻohiʻona nui i ʻike pinepine ʻia.

No ke aha lā kēia e hana nei? He aha ke kumu?

ʻAʻole i loaʻa i nā kānaka noiʻi ke kumu o kēia, akā aia kekahi mau manaʻo e hōʻike ana he kumu paha ia.

• Nā pilikia o ka ʻōpū mimi: Manaʻo kekahi poʻe ua hana 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. ʻAʻole hiki ke kahe pono ka mimi a hōʻiliʻili ʻia, e hoʻoulu ai i ka ʻōpū mimi, nā ureters, a me nā puʻupaʻa e pehu. I ka ulu ʻana o ka ʻōpū mimi, kau ia i ke kaomi ma luna o nā ʻiʻo o ka ʻōpū, e hoʻoulu ai iā lākou i ka atrophy.
• Pilikia ka ulu ʻana o nā ʻiʻo: Manaʻo kekahi poʻe ʻo ke kumu nui ʻaʻole e ulu pono ana nā ʻiʻo o ka ʻōpū o ka pēpē.
• Nā Kumu Hoʻoilina: Ua manaʻoʻiʻo ʻia he mana hoʻoilina ko kēia maʻi, e like me ka mea i ʻike ʻia i nā kaikaina. ʻIke ʻia ka Prune Belly Syndrome i nā keiki me nā maʻi hoʻoilina e like me Trisomy 18 (Edwards syndrome) a me Down syndrome (Trisomy 21).

He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?

Nui nā hopena ʻaoʻao a me nā pilikia e hele mai me ka Prune Belly Syndrome. He ʻokoʻa ia mai kēlā pēpē a i kēia pēpē. ʻO kēia nā pilikia nui e ʻike ʻia.

• Paʻa ʻōpū: Paʻakikī i ka hele ʻana i ka ʻōpū ma muli o ka nāwaliwali o nā ʻiʻo o ka ʻōpū.
• Nā kīnā o ka iwi: nā mea e like me ke kuʻe ʻana o ka ʻūhā, nā lima i nalowale, nā manamana lima, a i ʻole nā ​​​​manamana wāwae.
• Nā maʻi ʻōpū mimi: Loaʻa pinepine nā maʻi ʻōpū mimi (UTI).
• ʻO ka hānau ʻole a i ʻole ke kanesa: ʻO ka hiki ʻole ke iho i lalo i nā testicles e alakaʻi i ka hānau ʻole a i ʻole ka pilikia o ka maʻi kanesa i ka wā e hiki mai ana.
• Hoʻemi ʻia ka ulu ʻana o ka māmā: ʻAʻole ulu pono nā māmā (pulmonary hypoplasia).
• ʻO ka hāʻule ʻana o nā puʻupaʻa mau loa: ʻO kēia ka pilikia koʻikoʻi loa.

Pehea e ʻike ai i kēia maʻi?

I ka hapanui o nā hihia, hiki ke ʻike mua ʻia kēia kūlana i ka wā o kahi scan (ultrasound fetal) āu e hana ai i kou wā hāpai. Hiki i ke scan ke ʻike i ke kauka i ka pēpē:

• Nā ʻano ʻino o ka iwi a i ʻole nā ​​​​ʻiʻo
• Nā pilikia o ka puʻuwai
• Ka ulu ʻana o ka māmā haʻahaʻa
• Nā pilikia o ka ʻōnaehana hoʻoheheʻe
• Ua pehu ka ʻōpū mimi.
• Ua hoʻonui ʻia nā puʻupaʻa.

Ma hope o ka hānau ʻana o ka pēpē, hiki i ke kauka ke kānalua ua loaʻa i ka pēpē kēia maʻi ma ka nānā wale ʻana iā ia. No ka hōʻoia ʻana i kēlā kānalua, hiki ke hana ʻia kekahi mau hoʻokolohua, e like me:

• Nā hoʻāʻo koko
• ʻO ke kiʻi ʻana o ka ultrasound
• X-ray
• Nānā CT
• Nānā MRI
• ʻO ka Voiding cystourethrogram (VCUG) - Pili kēia i ka hoʻokomo ʻana i kahi wai kūikawā i loko o ka ʻōpū mimi a me ka lawe ʻana i nā kukuna X i ka wā e mimi ana ke pēpē e nānā i ka hana o ka ʻōnaehana mimi.

Pehea e mālama ʻia ai?

ʻOkoʻa ke ʻano o ka mālama ʻana i kēia mai kēlā keiki a kēia keiki. E hana pū kahi hui o nā loea e hoʻomohala i ka hoʻolālā mālama kūpono loa no ke keiki. E noʻonoʻo kēia i nā kumu he nui, me ka makahiki o ke keiki, ke kūlana olakino, ke koʻikoʻi o ke kūlana, a me nā makemake o nā mākua.

• Nā hihia ʻoluʻolu: ʻO kekahi mau keiki i ʻike ʻole ʻia ko lākou mau hōʻailona i ka ʻino e pōmaikaʻi paha mai nā lāʻau antibiotics wale nō e pale a mālama paha i nā maʻi ʻōpū mimi.
• ʻO ke ʻoki kino: Eia nō naʻe, e pono ana nā keiki he nui i nā ʻoki kino he nui e hoʻoponopono i ka paia ʻōpū, nā genitals, ka ʻōpū mimi, a me nā ʻāpana ʻē aʻe o ka ʻōnaehana urinary.

Eia kekahi o nā ʻano hana nui i hana ʻia:

• Vesicostomy: Hana ʻia kahi puka liʻiliʻi ma o ka ʻōpū i loko o ka ʻōpū mimi, e ʻae ana i ka mimi e kahe ma loko.
• Orchiopexy: Hoʻohaʻahaʻa ʻia nā testicles mai ke kino mai a waiho ʻia i loko o ka scrotum.
• ʻO ke kāpili ʻana o ka ʻiʻo:He ʻoki kino paʻakikī e kūkulu hou ana i ka ʻōpū mimi.

I kekahi mau hihia koʻikoʻi, pono paha i ke keiki kahi hoʻoili puʻupaʻa .

Pehea lā ka wā e hiki mai ana o kēia mau keiki?

ʻO ke ola o kēia mau keiki e pili ana i ke koʻikoʻi o ka maʻi. ʻO ka mea kaumaha, ma waena o 10% a me 25% o nā keiki i hānau ʻia me kēia maʻi e make ana i loko o ka ʻōpū a make paha mai nā pilikia i loko o nā pule mua o ke ola.

Eia nō naʻe, ma kahi o 40% o nā keiki me Prune Belly Syndrome he hana maʻamau ko lākou a hiki ke ola i kahi ola maʻamau.

Pono kekahi 30% i kahi hoʻoili puʻupaʻa i kekahi manawa o ko lākou ola.

He aha nā manawa kūpono loa e ʻike ai i ke kauka?

Loaʻa i nā keiki me ka Prune Belly Syndrome ka pilikia nui no nā maʻi ʻaʻai mimi (UTIs), no laila inā ʻike ʻoe i kekahi mau hōʻailona o ka maʻi ʻaʻai mimi, e kāhea koke aku i kāu kauka.

Eia kekahi, e ʻimi i ke kōkua lapaʻau inā loaʻa i kāu keiki kekahi pilikia ʻē aʻe i ka mimi ʻana a i ʻole e hōʻike ana i kekahi mau hōʻailona ʻē aʻe. He mea nui loa hoʻi e lawe i kāu keiki no kahi nānā lapaʻau ma ka liʻiliʻi hoʻokahi manawa i ka makahiki .

Hiki ke paʻakikī ka mālama ʻana i kahi pēpē i hānau ʻia me kēia maʻi. Akā e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. ʻO ka hana ʻana me ke kauka a me kahi hui o nā loea i ʻike i kēia maʻi hiki ke hāʻawi iā ʻoe a me kāu pēpē i ka ikaika a me ke alakaʻi a lākou e pono ai.