Ua lohe paha ʻoe ua hānau ʻia kekahi mau pēpē me hoʻokahi wale nō puʻupaʻa, a i ʻole me ka ʻole o nā puʻupaʻa ʻelua? He mea weliweli paha kēia, akā he mea nui e makaʻala i kēia maʻi. I kēia lā e kamaʻilio mākou e pili ana i kēia maʻi i kapa ʻia ʻo (Renal Agenesis). Mai hopohopo, e wehewehe maʻalahi kākou i nā mea āpau.
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He aha ka (Renal Agenesis)? E hoʻomaopopo maʻalahi kākou, ʻeā?
I ka ʻōlelo maʻalahi, ʻo (Renal Agenesis) ka wā e hānau ʻia ai kahi pēpē me hoʻokahi wale nō puʻupaʻa a i ʻole me ka ʻole o nā puʻupaʻa ʻelua. ʻO ka maʻamau, loaʻa iā mākou āpau ʻelua mau puʻupaʻa i loko o ko mākou kino. Pono ka liʻiliʻi o hoʻokahi puʻupaʻa e hana pono e ola ai. He kūlana (Congenital) kēia, ʻo ia hoʻi he mea ia e kū nei mai ka hānau ʻana.
I kēia manawa, inā ʻoe e nānā i ke ʻano o kēia mau huaʻōlelo ʻelua ma ke kaʻawale, e ʻoi aku ka maopopo iā ʻoe:
- Renal: ʻO kēia ka inoa lapaʻau no nā " kidneys ."
- ʻAgenesis: Pili kēia i ka hiki ʻole o kahi ʻōkana e ulu piha i ka wā e noho ana ke pēpē i loko o ka ʻōpū o ka makuahine, ʻo ia hoʻi, ka uterus.
No laila, ke ʻōlelo mākou he (Renal Agenesis), ʻo ia hoʻi, ʻaʻole i hoʻokumu pono ʻia nā puʻupaʻa o ke pēpē.
He aha nā ʻano nui o ka (Renal Agenesis)?
Hiki ke ʻike ʻia kēia kūlana ma nā ʻano nui ʻelua:
1. ʻO ka Agenesis Renal Unilateral
ʻO ka mea e hana ʻia ma kēia, e like me ka inoa e hōʻike nei, ʻo ia ka nalowale ʻana o kahi puʻupaʻa o ka pēpē ma kekahi ʻaoʻao. ʻO ia hoʻi, he maʻamau ka puʻupaʻa ʻē aʻe. ʻO ka mea pōmaikaʻi, hiki ke ola i kahi ola maʻamau a piha me hoʻokahi puʻupaʻa wale nō. I nā hihia he nui, hiki i ka puʻupaʻa hoʻokahi i koe ke hoʻomaka e lawe i ka hana o ka puʻupaʻa i nalowale, no laila hiki ke nui iki aʻe. Ua like ia me ke keiki e hana ana i ʻelua mau haʻawina home ma ka papa.
2. Ka nalowale ʻana o nā puʻupaʻa ʻelua (Bilateral Renal Agenesis)
He ʻano koʻikoʻi iki kēia. Maanei, ʻaʻohe puʻupaʻa ʻelua o ka pēpē. Eia kekahi, ʻaʻole i hana ʻia nā ʻōmole liʻiliʻi "Ureters" e lawe ana i ka mimi mai nā puʻupaʻa a i ka "Bladder". Me kēia, ʻaʻole i ulu pono nā "Lungs" o ka pēpē, ʻo ia hoʻi he "Pulmonary Hypoplasia" liʻiliʻi loa. ʻO ka hui pū ʻana o kēia hemahema o ka puʻupaʻa a me ka hāʻule ʻana o ka māmā ua kapa ʻia hoʻi ʻo "Potter Syndrome". ʻO ka mea kaumaha, he mea make pinepine kēia ʻano.
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He ʻāpana koʻikoʻi loa nā puʻupaʻa ʻelua i loko o ko kākou kino o kā kākou ʻōnaehana mimi. E noʻonoʻo iā lākou e like me ka ʻōnaehana hoʻolei ʻōpala i loko o ko kākou hale. ʻO ka hana nui o nā puʻupaʻa, ʻo ia ke kānana a wehe i nā ʻōpala i makemake ʻole ʻia mai ko kākou koko. A laila hoʻokuʻu ʻia kēia mau ʻōpala mai ke kino aku ma ke ʻano he mimi.
Pehea ka maʻamau o kēia ʻano (Renal Agenesis)?
Wahi a nā helu helu, ma kahi o hoʻokahi i loko o 2,000 mau pēpē i hānau ʻia e loaʻa paha i kahi maʻi i kapa ʻia ʻo "Unilateral Renal Agenesis", kahi i nalowale ai kekahi puʻupaʻa. ʻOi aku ka kakaikahi o ke ʻano o "Bilateral Renal Agenesis", kahi i nalowale ai nā puʻupaʻa ʻelua. Loaʻa ia ma kahi o hoʻokahi i loko o 8,500 mau pēpē hānau hou. ʻOiai ʻaʻole ʻike ʻia ke kumu pololei, ʻoi aku ka maʻamau o kēia ʻano o ka nalowale ʻana o nā puʻupaʻa ʻelua i nā pēpē kāne.
He aha nā kumu o ka (Renal Agenesis)?
ʻO ka hapa nui o ka manawa, ʻo ke kumu nui o ka (Renal Agenesis) he loli a i ʻole ka mutation i loko o nā genes (Gene mutation) . Ua ʻike nā kānaka noiʻi a hiki i kēia manawa ma kahi o ʻehiku mau mutations gene e pili ana me ia. Ma muli o kēia mutation gene, ua hoʻopilikia ʻia ke kūkulu ʻia ʻana o nā puʻupaʻa i loko o ka ʻōpū o ka pēpē i nā mahina ʻekolu mua o ka hāpai ʻana (First trimester).
ʻO nā kumu liʻiliʻi ʻē aʻe:
- Loaʻa i ka makuahine ka maʻi diabetes i ka wā hāpai (gestational diabetes).
- Nā hāpai keiki he nui (e like me nā māhoe, nā triplets).
- Ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau ʻino i ka wā hāpai.
He aha nā hōʻailona o ka (Renal Agenesis)?
ʻO kahi pēpē me ka Potter Syndrome, ka mea i nele i nā puʻupaʻa ʻelua a ʻaʻole i ulu pono kona mau māmā, loaʻa iā ia nā pilikia hanu e hoʻoweliweli ana i ke ola ma hope koke iho o ka hānau ʻana.
ʻAʻole paha e hōʻike ʻia kekahi mau hōʻailona e kahi pēpē i hānau ʻia me hoʻokahi puʻupaʻa. Akā inā ʻike ʻia nā hōʻailona, e komo pū paha lākou me:
- Ke koko kiʻekiʻe i nā keiki.
- Hoʻonui ʻia ka nui o ka protein i loko o ka mimi (Proteinuria).
- ʻO ka reflux vesicoureteral.
I kekahi manawa, hiki i nā pēpē i hānau ʻia me hoʻokahi puʻupaʻa ke loaʻa i nā kūlana ʻē aʻe e like me:
- Ka wāwae kalapu.
- Nā maʻi puʻuwai hānau ʻia, e like me nā lua ma waena o ka atria a i ʻole nā ventricles o ka puʻuwai (ASD a i ʻole VSD).
- Nā ʻano ʻē o ka urological hānau ʻole.
- ʻO ka imperforate anus a i ʻole ka anal atresia.
Pehea e ʻike ai i ka (Renal Agenesis) i ka wā hāpai?
ʻO ka hapa pinepine, hana ʻia ka ultrasound prenatal i ka wā hāpai o ka makuahine.Hiki ke ʻike ʻia kēia maʻi. Hōʻike kēia scan ua nalowale nā puʻupaʻa. Eia kekahi, inā haʻahaʻa ka wai amniotic a puni ka ʻōpū o ka pēpē (Oligohydramnios), hiki nō hoʻi ke lilo i hōʻailona o kahi maʻi i kapa ʻia ʻo Renal Agenesis. ʻO kēia no ka mea ʻaʻole hana nā puʻupaʻa o ka pēpē i ka mimi, ka mea e emi ai kēia wai. Ke lawa ʻole kēia wai, ʻaʻole e ulu pono nā māmā o ka pēpē.
Pehea e ʻike ai i ka (Renal Agenesis) ma hope o ka hānau ʻana o ke pēpē?
Hiki ke hana ʻia nā scan kiʻi ma hope koke o ka hānau ʻana o kahi pēpē e hōʻoia i ka hōʻailona. Eia nō naʻe, i kekahi manawa ʻaʻole paha e ʻike ʻia ke kūlana a hiki i ka wā e ʻelemakule iki ai ke keiki. I kekahi manawa hiki ke ʻike ʻia ke kūlana i ka wā e loaʻa ai i ke keiki nā hōʻailona ʻē aʻe a ke scan ʻia nei, a i ʻole ke hana ʻia nei kahi scan ultrasound no kekahi kumu ʻē aʻe, e like me ka maʻi urinary tract (UTI).
ʻO nā ʻano nui o nā scan i hoʻohana ʻia no ka ʻike ʻana i ka (Renal Agenesis):
Pehea e mālama ʻia ai ka Bilateral Renal Agenesis?
A hiki i kēia manawa koke nei, ʻaʻohe manaʻolana o kahi pēpē me nā puʻupaʻa ʻelua a me nā māmā i ulu ʻole e ola ma mua o kekahi mau hola ma waho o ka ʻōpū. Eia nō naʻe, ke hāʻawi nei nā lāʻau lapaʻau hoʻokolohua hou i kekahi manaʻolana. Pili kēia i ka hoʻokomo ʻana i kahi hopena saline i loko o ka ʻōpū i ka wā hāpai e hoʻihoʻi i ka pae wai amniotic. ʻAe kēia wai i nā māmā o ka pēpē e ulu. A laila, ma hope o ka hānau ʻana o ka pēpē, hoʻokomo ʻia ka pēpē i ka dialysis a hiki i ka hiki ke hana ʻia kahi hoʻoili puʻupaʻa. Akā e hoʻomanaʻo, aia nō kēia lāʻau lapaʻau i ke kahua hoʻokolohua a ʻaʻole loaʻa ma nā wahi āpau.
Pehea e mālama ʻia ai ka Unilateral Renal Agenesis?
ʻO ka poʻe me hoʻokahi puʻupaʻa e pono paha i kekahi manawa ke lawe i nā lāʻau lapaʻau e hoʻohaʻahaʻa i ko lākou koko . Inā loaʻa iā ʻoe a i ʻole kāu keiki kēia maʻi, he mea nui e ʻike pinepine i kahi loea puʻupaʻa (Nephrologist) a loaʻa nā hoʻokolohua kino, urinalysis, a me nā scan ma ka liʻiliʻi hoʻokahi i ka makahiki, a ʻoi aku paha. Hiki i kāu keiki ke loaʻa i kēia mau hoʻokolohua:
- Nā hoʻāʻo hana puʻupaʻa.
- Hoʻāʻo protein mimi.
He aha kāu e hana ai e pale aku i kou puʻupaʻa hoʻokahi wale nō?
Ua ʻōlelo ʻia he nui ka poʻe me hoʻokahi puʻupaʻa e hahai i ka papaʻai kidney-friendly (DASH) . ʻO kēia papaʻai e pili ana i ka hōʻemi ʻana i ka paʻakai a me ke kō a me ka ʻai ʻana i nā meaʻai kiʻekiʻe i ka fiber a me ka potassium.
Hiki i nā keiki a me nā mākua me Renal Agenesis ke hana i kēia mau mea e pale ai i ko lākou olakino puʻupaʻa:
- E pale i ka lawe ʻana i nā lāʻau anti-inflammatory non-steroidal (NSAIDs) a me kekahi mau lāʻau ʻeha. Hiki i kēia ke hōʻino i kou mau puʻupaʻa. E hoʻohana i kahi mea e like me ka acetaminophen.
- E mālama i ke kaumaha olakino a e noho ikaika. Eia nō naʻe, ʻoi aku ka maikaʻi o ka noho mamao aku mai nā haʻuki koʻikoʻi e like me ka pôpeku, ka rugby, a me nā hana koa, no ka mea hiki iā lākou ke hoʻopilikia i kou puʻupaʻa i koe.
- E nānā pinepine i kou koko ma ka home.
ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)
He mea maʻamau ke kaumaha a me ka hopohopo ke lohe ʻoe ua loaʻa iā ʻoe, kāu pēpē i hānau ʻole ʻia, a i ʻole kāu keiki liʻiliʻi ka "Renal Agenesis." Akā e hoʻomanaʻo, ʻoiai ʻo nā keiki i hānau ʻia me ka "Unilateral Renal Agenesis" e pilikia ana i ka loaʻa ʻana o nā pilikia puʻupaʻa i ka wā makua, nui ka poʻe e ola hauʻoli a olakino. He mea nui e kamaʻilio me ke kauka o kāu keiki e pili ana i nā ala maikaʻi loa e pale ai i ke olakino o nā puʻupaʻa o kāu keiki.
He maʻi koʻikoʻi ka Bilateral Renal Agenesis, a pinepine e make. Eia nō naʻe, me nā holomua lapaʻau hou, aia kekahi manaʻolana i kēia manawa.
ʻO ka mea nui loa, ʻaʻole ia e hopohopo no kēia, akā, e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau kūpono a hana e like me ia. ʻAʻole ʻoe hoʻokahi, aia nā kauka a me nā limahana mālama ola e kōkua iā ʻoe ma kēia huakaʻi.
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