Loaʻa i kāu pēpē nā kiko e like me kēia? E aʻo kākou e pili ana i ka Neonatal Lupus!

He mea maʻamau ke makaʻu iki inā ʻike koke ʻoe i kekahi mau kiko ʻulaʻula ma ke kino o kāu pēpē hānau hou, a inā ʻōlelo ke kauka i kekahi mea e pili ana i ka helu o ka puʻuwai o kāu pēpē. I kekahi manawa hiki ke hana ʻia kēia mau mea e kahi maʻi i kapa ʻia ʻo Neonatal Lupus. I kēia lā, e kamaʻilio mākou e pili ana i kēia me nā kikoʻī hou aku, e like me kāu e kamaʻilio ai me kahi hoaaloha.

He aha ka Lupus i ka wā hānau hou?

I ka ʻōlelo maʻalahi, ʻo Neonatal Lupus kahi maʻi e hiki ke hoʻopilikia i kahi pēpē i loko o ka ʻōpū (fetus) a i ʻole ma hope koke o ka hānau ʻana (pēpē). ʻO ka hapa nui o ka manawa, hoʻoulu ia i nā loli pōkole a ʻino ʻole, e like me ka ʻili ʻili. Nalo pinepine kēia mau mea ma mua o ka piha ʻana o ka pēpē i hoʻokahi makahiki. Eia nō naʻe, i kekahi manawa hiki i ka Neonatal Lupus ke hoʻopilikia i ka puʻuwai o ka pēpē.

ʻO ka pilikia weliweli loa e hiki ke hana ʻia ma kēia hihia ʻo ia ka "autoimmune congenital heart block." ʻO ia hoʻi, ʻo kēia "Neonatal Lupus" e pale i ka puʻuwai o ka pēpē mai ka paʻi pono ʻana. Hiki i kēia puʻuwai ke hoʻoweliweli i ke ola inā ʻoi loa ia, akā hiki ke mālama pinepine ʻia.

Inā ua ʻike ʻia kāu pēpē me Neonatal Lupus, he mea nui e hahai pono i nā ʻōlelo aʻoaʻo a ka hui mālama o kāu pēpē. E wehewehe lākou pehea e pili ai ka ʻike i kāu pēpē a inā pono ka lāʻau lapaʻau.

Pili anei kēia i ka lupus makua (Systemic Lupus Erythematosus - SLE)?

ʻAʻole, mai huikau i nā mea ʻelua. ʻAʻole like ka maʻi Neonatal Lupus me ka Systemic Lupus Erythematosus (SLE), a mākou e kapa pinepine ai he lupus. He ʻano ʻokoʻa ia me kona mau kumu ponoʻī a me ka mālama ʻana.

Hoʻokumu ʻia ka Neonatal Lupus e kekahi mau ʻano antibodies, e like me ka anti-SSA, anti-SSB, a me ka anti-U1-RNP, i hoʻoili ʻia mai ka makuahine a i ka pēpē ma o ka placenta. ʻO kēia mau antibodies he mau protein maoli e hoʻouka hewa ana i nā cell olakino i loko o ko mākou kino. I ka hapa nui o ka manawa, ʻaʻole pili kēia mau antibodies i ka pēpē e ulu ana i loko o ka ʻōpū. Akā i kekahi manawa, hiki iā lākou ke hoʻouka i nā cell olakino o ka pēpē, ʻoiai nā cell puʻuwai.

ʻIke wale ʻia ka Neonatal Lupus i nā pēpē nona nā makuahine i loaʻa kēia mau antibodies kikoʻī. ʻO ke ʻano maʻamau kēia he maʻi autoimmune ka makuahine. No ka laʻana, ʻo ka maʻi Sjögren, SLE, a i ʻole ka maʻi ʻiʻo pili i hui ʻia. Eia nō naʻe, i kekahi manawa hiki i kēia mau antibodies ke loaʻa i ke kino me ka ʻole o nā hōʻailona. I kekahi mau hihia, ʻo Neonatal Lupus paha ka hōʻailona mua e loaʻa ana i ka makuahine kekahi a ʻoi aku paha o kēia mau antibodies a hiki iā ia ke loaʻa i kahi maʻi autoimmune ikaika i ka wā e hiki mai ana.

E hoʻomanaʻo, inā loaʻa iā ʻoe kahi maʻi autoimmune, ʻaʻole ia he manaʻo e loaʻa maoli ana i kāu pēpē ka Neonatal Lupus. ʻO kaʻoiaʻiʻo, ʻoi aku ka nui o ka hiki ʻole i kāu pēpē ke loaʻa ka Neonatal Lupus. Akā, he mea kēia e pono ai ʻoe e makaʻala a kamaʻilio me kāu kauka.

Pehea ka maʻamau o kēia kūlana?

ʻAʻole maʻamau ka Neonatal Lupus. ʻO kāna pilikia koʻikoʻi loa, ʻo ia ka poloka puʻuwai hānau autoimmune, e pili ana i ka liʻiliʻi ma mua o 2% o nā pēpē i hānau ʻia e nā makuahine i loaʻa ka antibodies i ka anti-SSA, anti-SSB, a i ʻole anti-U1-RNP. Eia nō naʻe, inā ua loaʻa iā ʻoe ka poloka puʻuwai hānau autoimmune i ka hāpai ʻana ma mua, ʻoi aku kou pilikia o ka loaʻa hou ʻana i ka hāpai ʻana ma hope.

He aha nā hōʻailona o ka `(Neonatal Lupus)`?

ʻO ka hōʻailona maopopo loa, he ʻōhū ʻili. Hiki ke ʻike ʻia kēia i ka wā hānau, a i ʻole hiki ke ʻike ʻia i loko o nā mahina mua o ke ola. He manawa pōkole kēia ʻōhū a ʻaʻole ia he mea ʻino. ʻIke ʻia ia he apo poepoe me kahi lihi kala (pinepine ʻulaʻula). Loaʻa i kēlā me kēia apo kahi kikowaena maopopo (ʻo ia hoʻi, ke kala ʻili maʻamau).

E ʻike paha ʻoe i kēia mau ʻeha ma ke poʻo a me ka maka o kāu pēpē. Eia nō naʻe, hiki iā lākou ke ʻike ʻia ma ka umauma, nā lima, a me nā wāwae. Ua like kēia mau ʻeha me nā mea i ʻike ʻia ma ke ʻano i kapa ʻia ʻo "subacute cutaneous lupus." ʻO ia ke kumu i kapa ʻia ai lākou he "neonatal lupus."

Aia kekahi mau hōʻailona manawa pōkole a ʻino ʻole hoʻi. ʻO lākou kēia:

• Ua emi ka nui o nā hunaola koko ʻulaʻula (anemia).
• Nā pae haʻahaʻa o nā platelets (nā pūnaewele e kōkua i ke kāpili ʻana o ke koko) (thrombocytopenia).
• Ka helu haʻahaʻa o nā keʻena koko keʻokeʻo (neutropenia).
• Nā pae enzyme o ke akepaʻa i hoʻokiʻekiʻe ʻia.

Nalo aku kēia mau hōʻailona a pau ke hoʻopau loa ʻia nā antibodies mai ka makuahine mai ke kino o ke pēpē. Hoʻomaikaʻi pinepine lākou ma waena o 6 a me 9 mahina o ka makahiki.

ʻO ka Lupus Neonatal a me ka Pale Puʻuwai

E like me kā mākou i ʻōlelo ai ma mua, hiki i ka Neonatal Lupus ke hoʻoulu i kahi pilikia koʻikoʻi i kapa ʻia ʻo ka autoimmune congenital heart block. He pilikia kēia me ka ʻōnaehana uila o ka puʻuwai o ka pēpē. Hoʻokele ʻia ka puʻuwai o ka pēpē e nā impulses uila e hele ana ma o ka puʻuwai. I ka Neonatal Lupus, hoʻouka nā antibodies mai ka makuahine i kahi hui o nā cell e kaohi ana i ka hana uila o ka puʻuwai, i kapa ʻia ʻo AV node.

I kekahi manawa, hoʻololi iki wale kēia i ka helu o ka puʻuwai o ka pēpē (pale puʻuwai kekelē mua a ʻelua paha). Hiki ke hoʻopau ʻia kēia mau mea ma hope o ka hānau ʻana. Eia nō naʻe, hiki i kekahi mau pēpē ke loaʻa i ka pale puʻuwai kekelē ʻekolu, a i ʻole ka pale puʻuwai piha, ma muli o Neonatal Lupus.

ʻO kēia ka manawa e hiki ʻole ai i nā hōʻailona uila mai nā keʻena o luna o ka puʻuwai ke hele i nā keʻena o lalo. Ke ālai loa ʻia kēia mau hōʻailona, ​​​​kuʻi ka puʻuwai i ke ʻano kūpono ʻole, e paʻakikī ai i ka puʻuwai o ka pēpē ke pamu i ke koko i ke kino. ʻO ke ʻano poloka puʻuwai o ke kekelē ʻekolu ke ʻano koʻikoʻi loa. I kekahi manawa hiki ke make ma mua a ma hope paha o ka hānau ʻana.

ʻO ka maʻamau, ulu ka poloka puʻuwai ma waena o 18 a me 24 mau pule o ka hāpai ʻana. Hiki i nā kauka ke ʻike iā ia i ka wā hāpai ma ka hoʻolohe ʻana i ka puʻuwai o ka pēpē. Inā ʻoi aku ka lohi o ka helu o ka puʻuwai ma mua o ka mea i manaʻo ʻia (i kapa ʻia ʻo bradycardia), he hōʻailona paha ia o ka poloka puʻuwai.

He aha nā kumu o ka Neonatal Lupus?

E like me kā mākou i ʻōlelo ai ma mua, hoʻokumu ʻia ka Neonatal Lupus e nā antibodies e hele mai ana mai ka makuahine a i ka pēpē ma o ka placenta. Ua hoʻopili nā kānaka noiʻi i kēia mau ʻano antibodies kikoʻī i ka Neonatal Lupus:

• `(ʻO ka antigen A e pili ana i ka maʻi Sjögren (anti-SSA/Ro))`
• `(ʻO ka antigen B e pili ana i ka maʻi Sjögren (anti-SSB/La))`
• `(Anti-U1 ribonucleoprotein (anti-U1-RNP))`

Loaʻa kēia mau `(antibodies)` i nā poʻe me nā maʻi autoimmune like ʻole, e like me `(Sjögren's syndrome)`, `(SLE)`, `(rheumatoid arthritis)` a me `(mixed connective tissue disease)`. Eia nō naʻe, hiki ke loaʻa kēia mau `(antibodies)` i nā poʻe i loaʻa ʻole nā ​​​​hōʻailona a i ʻole ʻaʻole i ʻike ʻia me ka maʻi.

Ke komo kēia mau antibodies i ke kino o ka pēpē, hiki iā lākou ke hana i nā loli no ka manawa pōkole a mau loa paha. ʻAʻole maopopo loa i nā kānaka noiʻi pehea e hana ai kēia mau antibodies i kēia mau loli. Akā ua ʻike lākou ʻo nā loli koʻikoʻi a mau loa loa ia i ka puʻuwai o ka pēpē .

Ke komo i loko o ka puʻuwai, hoʻomaka kēia mau antibodies i kahi kaʻina hana mumū, kahi e alakaʻi ai i ka hoʻokumu ʻia ʻana o nā ʻiʻo ʻeha. Ke ālai nei kēia ʻiʻo ʻeha i nā hōʻailona uila, e pale ana i ka puʻuwai o ke pēpē mai ka hana pono ʻana. I nā hihia ʻino loa, hiki i kēia ke lilo i mea pilikia olakino i ka wā hāpai a i ʻole ke kumu o nā pilikia e hoʻoweliweli i ke ola ma hope o ka hānau ʻana.

Pehea e ʻike ʻia ai kēia maʻi "Neonatal Lupus"?

Hoʻoholo nā kauka i ka Neonatal Lupus i ka wā hāpai a i ʻole ma hope koke o ka hānau ʻana. Inā ʻike lākou ua lohi ka puʻuwai o ka pēpē ma mua o ka mea i manaʻo ʻia, e hana lākou i kahi echocardiogram fetal. He hoʻāʻo palekana kēia, ʻeha ʻole, ʻaʻole invasive. Hiki iā ia ke nānā i ke ʻano a me ka hana o ka puʻuwai o ka pēpē.

Hiki i kēia `(fetal echocardiogram)` ke hōʻike i nā loli e hōʻike ana i ka `(Neonatal Lupus)`. Inā pēlā, e haʻi aku kāu kauka iā ʻoe e pili ana i ke koʻikoʻi o ke kūlana a me nā mea e manaʻo ai i ka wā e hiki mai ana. E nānā pū lākou i kou koko no ke alo o kēlā mau `(antibodies)` kūikawā.

I kekahi manawa, ʻike ʻia nā hōʻailona o Neonatal Lupus, e like me nā ʻeha ʻili, ma hope o ka hānau ʻana o ka pēpē. Inā hana ʻia kēia, e hana ke kime lapaʻau i kahi hoʻokolokolo kino e kāpae i nā kumu ʻē aʻe o nā ʻeha. Hiki iā lākou ke hoʻāʻo iā ʻoe, kāu pēpē, a i ʻole ʻolua ʻelua no nā antibodies e kōkua i ka hana ʻana i kahi hōʻailona.

E hōʻoia kāu kauka iā ʻoe me Neonatal Lupus inā loaʻa kēia mau kūlana ʻelua:

• Inā ʻoe a/a i ʻole kāu pēpē he `(maikaʻi)` no `(anti-SSA)` a/a i ʻole `(anti-SSB)` a/a i ʻole `(anti-U1-RNP)`.

a me

• Inā loaʻa i kāu pēpē hoʻokahi ma ka liʻiliʻi o nā hōʻailona i hōʻoia ʻia o "Neonatal Lupus".

Pehea e mālama ʻia ai ka Neonatal Lupus?

Aia ka lāʻau lapaʻau i ke koʻikoʻi o ke kūlana. ʻAʻole pono ka lāʻau lapaʻau no nā ʻeha ʻili, nā helu koko ʻē, a i ʻole nā ​​​​enzymes akepaʻa kiʻekiʻe. Hoʻoponopono pinepine kēia mau pilikia iā lākou iho ma lalo o hoʻokahi makahiki.

Eia nō naʻe, pono paha i nā pēpē i hānau ʻia me ka poloka puʻuwai ke hoʻokomo ʻia kahi pacemaker mau loa i ka wā hānau a i ʻole i ka wā kamaliʻi. He hana ʻoki kino kēia e hoʻokomo i kahi mea e kōkua i ko lākou puʻuwai e paʻi pono. E haʻi aku ka hui lapaʻau o kāu pēpē iā ʻoe e pili ana i kēia.

Ua hōʻike ʻia kekahi mau noiʻi e hiki i ka lawe ʻana i kekahi mau lāʻau lapaʻau i ka wā hāpai ke hōʻemi i ka pilikia o ka loaʻa ʻana o ka poloka puʻuwai i kāu pēpē. Inā loaʻa iā ʻoe kahi maʻi autoimmune i ʻike ʻia, a inā hoʻāʻo maikaʻi ʻoe no kekahi mau antibodies, hiki i kāu kauka ke ʻōlelo aku iā ʻoe e lawe i kahi lāʻau i kapa ʻia ʻo hydroxychloroquine i ka wā hāpai. ʻO ka hana ʻana pēlā e kōkua paha i ka pale ʻana i kāu pēpē mai nā hopena koʻikoʻi loa o Neonatal Lupus.

He aha ka prognosis no nā pēpē me Neonatal Lupus?

ʻO ke ʻano o kāu pēpē e pili ana i ka hopena o Neonatal Lupus i kona kino. No nā pēpē me nā ʻano ʻili, akepaʻa, a/a i ʻole ke koko wale nō, maikaʻi loa ke ʻano. Pono nā mea āpau e hoʻi i ka maʻamau i ka 6 a 9 mau mahina.

Eia nō naʻe, inā loaʻa ka poloka puʻuwai, hiki ke ʻokoʻa loa ke ʻano. Hiki i ka poloka puʻuwai ke make i kekahi manawa ma mua a ma hope paha o ka hānau ʻana. Pono nā pēpē he nui me ka poloka puʻuwai i kahi pacemaker mau loa i ka wā kamaliʻi.

Hiki i kahi helu liʻiliʻi o nā pēpē me Neonatal Lupus ke hoʻomohala i kahi maʻi autoimmune e like me kēia:

• ʻO ka ʻāʻī idiopathic ʻōpio
• ʻO ka psoriasis
• ʻO ka maʻi thyroid autoimmune
• ʻAno 1 diabetes

"He ʻokoʻa kēlā me kēia pēpē. ʻAʻole paha he hopena nui a ʻaʻohe paha o ka Neonatal Lupus i ke ola o kāu pēpē. E hāʻawi kā lākou hui lapaʻau iā ʻoe i nā kikoʻī hou aku e pili ana i nā mea e manaʻo ai a pehea e hoʻomākaukau ai."

Hiki ke pale ʻia ka Neonatal Lupus?

ʻAʻohe ala e pale aku ai i nā antibodies mai ke komo ʻana i ka placenta i kou wā hāpai. Eia nō naʻe, hiki iā ʻoe ke pale i ka hōʻeha nui ʻana i ka puʻuwai o kāu pēpē, a i ʻole e hoʻolālā mua no kekahi lāʻau lapaʻau e pono ai kāu pēpē ma hope o ka hānau ʻana. Inā loaʻa iā ʻoe ka anti-SSA, anti-SSB, a i ʻole anti-U1-RNP, hiki i kāu kauka ke ʻōlelo aku i kēia mau mea:

• ʻO ka lawe ʻana i kekahi mau lāʻau lapaʻau i ka wā hāpai mua a i ka wā hāpai hiki ke kōkua i ka pale ʻana i ka pēpē mai ka loaʻa ʻana o ka poloka puʻuwai.
• Ke nānā ʻana no ka poloka puʻuwai (fetal echocardiograms) mai kahi o 16 mau pule o ka hāpai ʻana.
• Ke nānā ʻana i ka hana o ka puʻuwai o ka pēpē i ka mahina mua o ke ola. ʻO kēia ke nānā ʻana i nā hōʻailona o ka poloka puʻuwai (inā ʻaʻole i ʻike ʻia), no ka mea, ʻaʻole hōʻike kekahi mau pēpē i kekahi mau hōʻailona a hiki i kekahi mau pule ma hope o ka hānau ʻana.

Āhea wau e ʻimi ai i ke aʻo lapaʻau?

He mea nui e hahai pono i ka papa hana hoʻāʻo a me ka manawa i hāʻawi ʻia e ka hui lapaʻau o kāu keiki. E haʻi lākou iā ʻoe i ka pinepine e lawe mai ai i kāu keiki no nā nānā ʻana a me nā hahai ʻana.

He mea nui hoʻi e mālama i kou olakino. Inā loaʻa iā ʻoe kekahi mau antibodies akā ʻaʻohe ou hōʻailona a ʻōuli paha o ka maʻi autoimmune, hiki i kāu kauka ke kuhikuhi iā ʻoe i kahi rheumatologist, he kauka loea i ka ʻeha hui a me nā maʻi pili. ʻO kēia no ka mea aia kahi manawa e hiki ai iā ʻoe ke loaʻa i kahi maʻi autoimmune ikaika i nā makahiki e hiki mai ana. E wehewehe kāu kauka i nā mea āu e manaʻo ai a nānā pono iā ʻoe i hiki iā ʻoe ke loaʻa koke ka lāʻau.

He aha nā nīnau e pono iaʻu e nīnau aku i ke kauka o kaʻu keiki?

Hiki i kēia mau nīnau ke kōkua iā ʻoe e aʻo hou e pili ana iā Neonatal Lupus ma hope o kahi hōʻoia:

• Pehea ka hopena o ka Neonatal Lupus i koʻu hāpai ʻana/kaʻu pēpē?
• He aha nā koho lapaʻau e loaʻa ana?
• He aha ka wānana?
• He aha ka manawa kūpono e hoʻopilikia ai ka Neonatal Lupus i ka hāpai ʻana i ka wā e hiki mai ana?

Hiki i nā pākeke ke loaʻa i ka "Neonatal Lupus"?

ʻAʻole, ʻaʻole ʻo Neonatal Lupus kahi hōʻailona no nā pākeke. Loaʻa wale ia i nā ʻōpū a me nā pēpē. ʻAʻole like ʻo Neonatal Lupus me ka lupus pākeke.

ʻO ka Systemic Lupus Erythematosus (SLE), ka mea a mākou e kapa pinepine ai ʻo lupus, he maʻi autoimmune ia e hoʻopilikia ai i nā keiki a me nā pākeke. He systemic ka SLE, ʻo ia hoʻi ke hoʻopilikia nei i ke kino holoʻokoʻa a pono e mālama ʻia a me ka nānā ʻana i ke ola holoʻokoʻa.

Hoʻopilikia wale ka Neonatal Lupus i nā mahina mua o ke ola o kahi pēpē. ʻAʻole ia e ulu ma hope o ka wā kamaliʻi a i ka wā makua.

Eia nō naʻe, hiki i ka Neonatal Lupus ke loaʻa nā hopena lōʻihi ma ke kino o ke kanaka, ʻoiai inā ʻaʻole i ʻike ʻia a hiki i ka wā hānau. ʻO ka pilikia koʻikoʻi loa ka poloka puʻuwai, kahi e pono ai kahi pacemaker no ke ola holoʻokoʻa. Hōʻike kekahi mau noiʻi e piʻi aʻe ka pilikia o nā pēpē i loaʻa iā Neonatal Lupus i ka loaʻa ʻana o nā maʻi autoimmune ma hope o ke ola.

Inā loaʻa iā ʻoe ʻo Neonatal Lupus i kou wā kamaliʻi, e kamaʻilio me kāu kauka e ʻike i ke ʻano o ka nānā ʻana a i ʻole ka hoʻāʻo ʻana āu e pono ai i kou wā ʻelemakule.

Ke hāpai ʻoe a loaʻa iā ʻoe kahi hōʻailona hānau hou e like me kēia, hiki ke kaumaha a hopohopo. Hiki ke paʻakikī loa ka ʻike ʻole i ka mea e hana ʻia i ka wā e holomua ai kou hāpai ʻana. ʻAʻohe hewa o ka haʻi ʻana i kou mau makaʻu a me kou hopohopo me kāu hui lapaʻau. E hana lākou i nā mea āpau e hiki ai iā lākou ke kōkua iā ʻoe e hoʻomaopopo i ka mea e hana nei a me ka mea hiki iā ʻoe ke hana e kōkua iā ʻoe e loaʻa kahi hāpai olakino.

Inā ʻike ʻia kāu pēpē ma hope o ka hānau ʻana, pono paha e nānā ʻia lākou i ka mahina mua o ke ola e nānā i ka hana o ko lākou puʻuwai. Loaʻa paha iā lākou nā ʻeha ʻili e mau ana no kekahi mau mahina. E wehewehe ka hui lapaʻau o kāu pēpē i ka mea e manaʻo ai a hāʻawi i ke kākoʻo no ʻoe a me ka ʻohana holoʻokoʻa.

Nā mea e pono ai kākou e hoʻomanaʻo mai kēia moʻolelo

ʻAe, e hōʻuluʻulu kākou i kekahi o nā mea nui e pili ana i ka Neonatal Lupus a mākou i kamaʻilio ai:

• ʻO Neonatal Lupus kahi maʻi e kū mai ana i nā pēpē hānau hou ma muli o nā antibodies kūikawā i hoʻoili ʻia mai ka makuahine i ke pēpē.
• ʻAʻole like kēia me ka lupus (SLE) i loaʻa i nā pākeke.
• ʻO ka hapa nui o ka manawa, he manawa pōkole a ʻaʻohe mea ʻino nā hōʻailona, ​​e like me nā ʻeha ʻili. E ho'ōla ʻia kēia mau mea i loko o 6-9 mau mahina.
• Eia nō naʻe, hiki i kekahi mau pēpē ke loaʻa i kahi maʻi koʻikoʻi i kapa ʻia ʻo congenital heart block, kahi e pono ai ka pacemaker.
• Inā he maʻi autoimmune ko ka makuahine, a inā he mau antibodies kikoʻī kona (anti-SSA, anti-SSB), he mea nui loa ia e kūkākūkā i kēia me ke kauka i ka wā hāpai.
• Hoʻōla piha ka hapa nui o nā pēpē mai kēia maʻi. Inā loaʻa nā pilikia puʻuwai, hiki ke mālama ʻia.
• Inā he mau nīnau a kānalua paha kāu, mai makaʻu e nīnau i ke kauka. Aia lākou ma laila e kōkua iā ʻoe.

`ʻO Neonatal Lupus, nā keiki hānau hou, nā maʻi pēpē, ka poloka puʻuwai, ka autoimmune, nā antibodies makuahine, ka ʻulaʻula ʻili