Ke maʻi pinepine nei ʻoe? ʻO kēia paha ke kumu: E aʻo kākou e pili ana i ka hypogammaglobulinemia.

Loaʻa pinepine iā ʻoe ke anu, ka maʻi palū, a me ke kuni? A i ʻole loaʻa pinepine i kāu keiki liʻiliʻi nā maʻi pepeiao a i ʻole nā ​​pilikia sinus? I kekahi manawa manaʻo mākou he mau maʻi maʻamau kēia, akā inā hoʻi hou mai kēia mau maʻi, aia paha kekahi kumu ʻē aʻe. ʻO kahi kūlana e hiki ke kānalua ʻia i ia mau manawa ʻo ia ka hypogammaglobulinemia. ʻOiai he inoa lōʻihi iki ia, e hoʻomaopopo maʻalahi kākou.

He aha ka hypogammaglobulinemia?

I ka ʻōlelo maʻalahi, ʻo ka hypogammaglobulinemia kahi kūlana kahi i loaʻa ai i kou kino kahi pae haʻahaʻa o nā antibodies. ʻO kekahi inoa no kēia mau antibodies he immunoglobulins. I kēia manawa e noʻonoʻo ʻoe ua like ko mākou kino me kahi pā kaua. ʻO nā ʻenemi e hele mai ana i kēia pā kaua he mau germs, e like me nā virus a me nā bacteria. No laila, ʻo nā koa e pale mai iā mākou mai kēia mau ʻenemi ʻo kā mākou ʻōnaehana pale .

Loaʻa i kēia ʻōnaehana pale maʻi kahi ʻano cell kūikawā i kapa ʻia ʻo B-cells . Ua like lākou me nā commandos i hoʻomaʻamaʻa kūikawā ʻia. Ke komo kahi germ i loko o ke kino, hana kēia mau B-cell i nā antibodies i kapa ʻia ʻo immunoglobulins. Hele kēia mau antibodies a hopu i ka germ a luku iā ia. ʻO ia ka manawa ʻaʻole mākou e maʻi, a i ʻole e ola koke mākou mai ka maʻi.

E ʻike wale ʻoe i kēia maʻi ke loaʻa iā ʻoe kahi hoʻāʻo koko.

E wehewehe kākou i kēia mau huaʻōlelo i hiki ke hoʻomaopopo ʻia:

• Hypo: ʻo ia hoʻi ma lalo o ka maʻamau.
• ʻO nā gamma globulins: ʻO kēia ke ʻano maʻamau o ka immunoglobulin.
• ʻEmia: ʻo ia hoʻi i loko o ke koko.

No laila, ke emi ka pae o kēia immunoglobulin i loko o kou koko, e nāwaliwali ka ʻōnaehana pale o kou kino. A laila hiki i nā germs ke hoʻouka maʻalahi i kou kino. No laila , ʻoi aku ka nui o kou loaʻa ʻana i nā maʻi a me nā maʻi ʻē aʻe.

Aia kekahi mau ʻano o kēia?

ʻAe, hiki i kahi maʻi i kapa ʻia ʻo hypogammaglobulinemia ke hoʻopilikia i nā keiki a me nā pākeke. ʻElua ʻano nui:

1. Hypogammaglobulinemia mua (congenital): Hoʻokumu ʻia kēia e kekahi mau maʻi genetic kakaikahi. Kāhea mākou i kēia mau maʻi Immunodeficiency Primary (PIDD) . I ka ʻōlelo maʻalahi, ke hānau ʻia ʻoe, hānau ʻia ʻoe me kahi hewa (mutation) i loko o kāu mau genes. ʻO kēia mau genes nā mea e kuhikuhi i ka ʻōnaehana pale pehea e hana ai. No laila, i ka wā e hewa ai kēlā kuhikuhi, hoʻemi ʻia ka palekana. Hiki ke hoʻoili pinepine ʻia kēia mau hewa genetic mai kēlā hanauna a i kēia hanauna.

2. Hypogammaglobulinemia lua (loaʻa): ʻO kēiaʻO ke ʻano maʻamau. Hiki ke hana ʻia kēia maʻi e nā ʻano maʻi like ʻole a i ʻole he hopena ʻaoʻao o kekahi mau lāʻau lapaʻau. ʻO ia hoʻi, ua hānau olakino ʻoe a laila loaʻa i kēia maʻi ma hope o ke ola.

Pehea ka laha ʻole o kēia maʻi?

ʻO Hypogammaglobulinemia kekahi o nā maʻi immunodeficiency mua (PIDD) i ʻōlelo ʻia ma mua. Eia nō naʻe, ʻaʻole maʻamau loa nā PIDD ma ke ʻano laulā. No ka laʻana, ua manaʻo ʻia he hapalua miliona mau kānaka ma ʻAmelika Hui Pū ʻIa e noho nei me PIDD.

He aha nā hōʻailona o kēia?

ʻO ka hōʻailona nui o ka hypogammaglobulinemia i nā keiki a me nā pākeke he mau maʻi pinepine a lōʻihi paha. Pili nā hōʻailona i ke ʻano o ka maʻi e kū mai ana. ʻO nā maʻi maʻamau a me nā hōʻailona e pili ana me ka hypogammaglobulinemia:

• Nā maʻi pepeiao: ʻeha pepeiao, ʻeha ka pahu pepeiao (i kekahi manawa he ʻeha), kuni. (E noʻonoʻo i kekahi mau keiki he mau maʻi pepeiao mau, e hoʻi hou mai ana me ka ʻole o ka lāʻau lapaʻau.)
• Nā maʻi sinus: Ihu wai a ihu paʻa paha, luhi, kuni. (Ala kekahi poʻe i ke kakahiaka me ka manaʻo paʻa a kaumaha ke poʻo, ʻeā? Inā hoʻomau ia, pono ʻoe e hopohopo.)
• Bronchitis: ʻŪhā mau, pōkole o ka hanu, kuni.
• Pneumonia: Paʻa ka umauma a ʻeha paha, paʻakikī ka hanu ʻana, kuni.
• ʻO ka maʻi ʻōpū: ka maʻi pākī, ka nausea, ka luaʻi ʻana, ke kuni.
• Meningitis: ʻĀʻī ʻoʻoleʻa, ʻeha poʻo, kuni. (He mea weliweli iki kēia, pono ʻoe e ʻike koke.)
• Nā maʻi ʻili: nā ʻeha ʻili, ka pehu ʻana, ka maneʻo, ke kuni.

Eia kekahi, hiki ke ʻokoʻa nā hōʻailona ma muli o ke kūlana kumu i hoʻokumu ai i nā pae immunoglobulin haʻahaʻa. No ka laʻana, ʻoi aku ka nui o ka loaʻa ʻana o nā maʻi allergies i kahi keiki me kekahi mau ʻano PIDD. Hiki i nā ʻano ʻē aʻe ke hoʻopaneʻe i ka ulu ʻana a me ka hoʻomohala ʻana.

He aha ke kumu o kēia?

ʻElua mau kumu hiki. ʻAʻole hana kāu ʻōnaehana pale i nā immunoglobulins lawa, a i ʻole ua luku ʻia nā mea āna i hana ai no kekahi kumu. ʻOkoʻa nā kumu ma muli o ka loaʻa ʻana o ka lupus mua a lua paha.

Nā kumu o ka hypogammaglobulinemia mua

ʻO kekahi o nā ʻano maʻamau o ka `PIDD` e hiki ke pili pū me kēia:

• ʻO ka maʻi pale ʻole loli maʻamau (CVID): He kīnā genetic e pale ai i ke kino mai ka hana ʻana i nā immunoglobulins lawa. ʻO kēia ke kumu maʻamau o ka hypogammaglobulinemia mua i nā pākeke.
• ʻO ka Agammaglobulinemia i hoʻopili ʻia me X (XLA): ʻO kahi kīnā genetic ma ka chromosome X e pale aku i ke kino mai ka hana ʻana i nā cell-B a i ʻole nā ​​​​immunoglobulins lawa. Hoʻopilikia nui kēia i nā pēpē kāne (no ka mea hoʻokahi wale nō chromosome X). ʻO XLA kahi kumu maʻamau o ka hypogammaglobulinemia i nā keiki.
• ʻO ka Transient Hypogammaglobulinemia o ka pēpē (THI): Hana ʻia kēia i ka wā e hoʻomaka ai ka emi ʻana o nā pae immunoglobulin o kahi pēpē ma kahi o 3 mau mahina. Hiki i nā pēpē me THI ke loaʻa i nā maʻi, akā ʻaʻole lākou e hōʻike pinepine i nā hōʻailona. Hoʻopau pinepine ke ʻano ma kāna iho i ka piha ʻana o 3 mau makahiki.
• Ka hemahema IgA koho: ʻO kēia ka manawa ʻaʻole hana ke kino i ka lawa o kahi ʻano immunoglobulin kikoʻī i kapa ʻia ʻo Immunoglobulin A.
• Nā maʻi Hyper-IgM: Ma kēia, hana ʻia kekahi ʻano immunoglobulin ma ke ʻano maʻamau a keu paha, akā ʻaʻole i hana ʻia kekahi i nā nui lawa.

Ma waho aʻe o kēia, aia kekahi mau ʻano PIDD e hoʻoulu ai i ka hypogammaglobulinemia mua:

• ʻAtaxia-telangiectasia
• ʻO ka agammaglobulinemia recessive autosomal (ARA)
• ʻO ka maʻi maikaʻi (GS)
• Nā hemahema immunoglobulin non-IgG i hoʻokaʻawale ʻia
• ʻO ka hemahema immunocompromised koʻikoʻi (SCID)
• Ka hemahema o ka antibody kikoʻī (SAD)
• ʻO ka maʻi Wiskott-Aldrich (WAS)

Nā kumu o ka hypogammaglobulinemia lua

Eia nā kumu e hiki ai ke kū mai kēia kūlana ma hope:

• Nā lāʻau lapaʻau: Hiki i kekahi mau lāʻau lapaʻau ke hoʻohaʻahaʻa i nā pae immunoglobulin. No ka laʻana, nā immunosuppressants, nā lāʻau anti-seizure, corticosteroids, a me nā lāʻau chemotherapy.
• ʻO ka maʻi Nephrotic: Ma kēia ʻano, hoʻokuʻu nui kou mau puʻupaʻa i ka protein i loko o kou mimi. Hiki ke hoʻokuʻu ʻia nā immunoglobulins, ʻo ia hoʻi nā protein, ma kēia ʻano.
• ʻO ka enteropathy nalowale protein: ʻO kēia ka wā e nalowale ai ka nui o ka protein mai kou kino ma o kāu ʻōnaehana gastrointestinal.
• Ka maʻi ʻaʻai:ʻO nā maʻi kanesa e hoʻopilikia i kāu mau cell-B hiki ke hana i nā pae immunoglobulin haʻahaʻa. No ka laʻana, nā maʻi kanesa e like me ka leukemia lymphocytic maʻi, lymphoma, a me ka myeloma maha.
• HIV: He maʻi laha pili i ka moekolohe (STI) ka HIV. Hiki iā ia ke hana i ka hypogammaglobulinemia a me nā pilikia ʻē aʻe o ka ʻōnaehana pale.

He aha nā pilikia e hiki ai i kēia ke hana?

Inā ʻaʻole mālama ʻia, hiki i ka hypogammaglobulinemia ke alakaʻi i nā maʻi koʻikoʻi a me nā pilikia e hoʻoweliweli ana i ke ola . ʻO kahi pilikia maʻamau ka bronchiectasis . He kūlana kēia e hōʻino mau ʻia ai nā māmā. ʻOi aku ka maʻamau o kēia kūlana i ka poʻe i loaʻa pinepine i nā maʻi ʻōpū hanu luna.

E noʻonoʻo i kahi kanaka i loaʻa nā pilikia māmā me ka ʻū mau a me ka phlegm. Ke hōʻino mau ʻia ko lākou mau māmā, hiki iā ia ke lilo i maʻi mau loa.

Eia kekahi, hoʻonui ka hypogammaglobulinemia i ka pilikia o:

• Nā maʻi Autoimmune: ʻO kēia nā kūlana kahi e hoʻouka ai kāu ʻōnaehana pale ponoʻī i nā hunaola o kou kino ponoʻī.
• Ka hōʻino ʻana i nā ʻōpū o loko.
• Ka maʻi ʻaʻai.
• Sepsis: He maʻi ʻino kēia e laha ana ma ke kino holoʻokoʻa.

Pehea ʻoe e ʻike ai i kēia?

E hana nā kauka i nā hoʻokolohua koko e ana i kāu mau pae immunoglobulin. Inā haʻahaʻa kou mau pae ma mua o ka maʻamau, hiki ke ʻike ʻia ʻoe me ka hypogammaglobulinemia. A laila e hoʻokaʻawale kāu kauka i kou kūlana ma ke ʻano he ʻoluʻolu, waena, a koʻikoʻi paha .

No ka ʻike pono ʻana i ke kumu o kēia pae haʻahaʻa, e noʻonoʻo ke kauka i kēia mau mea:

• ʻO kāu mau hōʻailona: ʻOi loa aku inā loaʻa pinepine ʻoe i nā maʻi.
• ʻO kou mōʻaukala lapaʻau ʻohana: Aia kekahi o kou ʻohana i loaʻa i ka PIDD?
• Nā lāʻau lapaʻau āu e lawe nei: ʻOi aku ka nui o nā lāʻau lapaʻau e hiki ai ke hoʻoulu i ka hypogammaglobulinemia ma ke ʻano he hopena ʻaoʻao.

Pono paha ʻoe e loaʻa nā hoʻokolohua hou aʻe, e komo pū ana me:

• Nā hoʻāʻo koko ʻē aʻe: No ka laʻana, nā helu koko piha, nā hoʻāʻo antibody, a me nā hoʻāʻo e nānā i ke ʻano o ka hana ʻana o kāu mau kino.
• Nā kaʻina hana kiʻi: No ka nānā ʻana i ka hōʻino ʻia o nā ʻiʻo ma muli o ka maʻi a i ʻole nā ​​​​pūpū (e like me X-ray, CT scan)
• Nā hoʻokolohua genetic: E nānā no nā kīnā genetic e pili ana me PIDD.
• Hoʻāʻo Biopsy:Ke lawe nei i kahi hāpana ʻiʻo a hoʻāʻo iā ia e ʻike inā he mau hunaola kanesa.

Pehea e mālama ʻia ai kēia?

Aia ka lāʻau lapaʻau i ke kumu o kou hypogammaglobulinemia a me kona koʻikoʻi. Hiki ke kuhikuhi ʻia ʻoe i kahi immunologist.

Inā pili ke kūlana i kahi kūlana olakino , e mālama pinepine nā kauka i ke kūlana kumu. No ka laʻana, inā haʻahaʻa kāu mau pae immunoglobulin ma muli o ka hopena ʻaoʻao o kahi lāʻau āu e lawe nei, hiki ke hoʻololi ʻia ka lāʻau.

Inā loaʻa iā ʻoe ka hypogammaglobulinemia mua , pono ʻoe e noho ma lalo o ka nānā ʻana o ke kauka no ke koena o kou ola e pale iā ʻoe iho mai nā maʻi koʻikoʻi. Hiki ke komo pū nā koho lapaʻau:

• Ke kali makaʻala: Inā ʻaʻole kou pae immunoglobulin haʻahaʻa e hoʻopilikia nui i ke olakino, hiki i kāu kauka ke nānā i kou kūlana. ʻO nā kūlana e hoʻopau iā lākou iho, e like me ka transient hypogammaglobulinemia of infancy (THI), i ʻōlelo ʻia ma mua, ʻaʻole pono paha ka lāʻau lapaʻau.
• Nā lāʻau lapaʻau: Hiki ke hāʻawi ʻia iā ʻoe nā lāʻau lapaʻau e mālama i kahi maʻi bacteria a i ʻole e pale aku i ka maʻi.
• ʻO ka lāʻau hoʻololi Immunoglobulin (IgG): Inā haʻahaʻa loa kāu mau pae immunoglobulin, hiki ke hāʻawi ʻia iā ʻoe ka immunoglobulin mai kahi mea hāʻawi. Hāʻawi ʻia kēia ma ke ʻano he injection i loko o kahi vein (IV) a i ʻole ma lalo o ka ʻili (subcutaneously). ʻO ka poʻe me PIDD e pono ai kēia lāʻau lapaʻau e pono pinepine e lawe i kēia lāʻau lapaʻau no ke koena o ko lākou ola.
• Hoʻoili ʻana i nā pūnaewele kumu: Inā ʻaʻole hiki i kou kino ke hana i nā pūnaewele palekana olakino, hiki iā ʻoe ke loaʻa i kahi hoʻoili ʻana i nā pūnaewele kumu mai kahi mea hāʻawi. E ulu aʻe kēia mau pūnaewele kumu i nā pūnaewele koko olakino, me nā pūnaewele B.

He kūlana koʻikoʻi anei kēia?

Hiki i ka Hypogammaglobulinemia ke koʻikoʻi. Aia ia i ke kumu a me ka haʻahaʻa o kāu mau pae immunoglobulin. Ua like nā Immunoglobulin me nā koa i loko o ko mākou kino e hakakā ana i nā pathogens ʻino e like me nā virus, bacteria, parasites, a me nā fungi. No laila, i ka wā ʻaʻole e pale aku kēia mau koa iā mākou, ʻoi aku ka nui o ka loaʻa ʻana o nā maʻi pinepine a koʻikoʻi. Inā ʻaʻole mālama ʻia, hiki i nā maʻi koʻikoʻi ke hōʻino i nā ʻiʻo no ka manawa lōʻihi a me ka make.

No laila, ʻaʻole pono e lawe māmā ʻia kēia ʻano. Inā loaʻa iā ʻoe nā hōʻailona, ​​​​he mea nui e ʻimi koke i ke kōkua lapaʻau.

He aha ka mea e hana ʻia inā ʻaʻole mālama ʻia?

He mea nui e loaʻa i ke kauka ke kumu o ka hypogammaglobulinemia. Inā hoʻomaka mua ʻoe i ka lāʻau lapaʻau, ʻoi aku ka maikaʻi o kou noho olakino a pale i nā pilikia e hiki mai ana i nā makahiki ma hope. Inā loaʻa iā ʻoe kahi maʻi e hoʻoweliweli iā ʻoe no ka hypogammaglobulinemia, e nānā mau kāu kauka i kāu mau pae immunoglobulin. E paipai lākou i kahi hoʻolālā lapaʻau e kōkua i ka pale ʻana i nā maʻi koʻikoʻi.

Hiki ke pale ʻia kēia?

ʻAʻole hiki iā mākou ke pale aku i nā kūlana (e like me nā kīnā genetic) e hoʻoulu ai i ka hypogammaglobulinemia. Eia nō naʻe, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e kōkua i ka pale ʻana i nā pilikia , e like me nā maʻi pinepine a me ka hōʻino ʻana o ka māmā.

Pehea e hiki ai iaʻu ke mālama iaʻu iho? He aha kaʻu e hana ai e pale aku i nā maʻi?

ʻO ka mea nui loa, ʻo ia ka hahai ʻana i nā ʻōlelo a kāu kauka. Hiki i kēia mau mea ke kōkua i ka pale ʻana i ka maʻi:

• E holoi pono i kou mau lima: E holoi i kou mau lima me ke kopa a me ka wai no 20 kekona ma ka liʻiliʻi. E holoi pono i kou mau lima ma hope o ka hele ʻana i waho, ma mua o ka ʻai ʻana, a ma hope o ka hoʻohana ʻana i ka lua.
• Ke kau nei i ka pale maka i ka wā e pono ai e pale i ka laha ʻana o nā germs ea: He mea nui loa kēia i ka wā e hele ai i nā wahi piha kanaka a i nā manawa o ka hana nui o nā maʻi.
• E noho mamao aku mai ka poʻe maʻi a me nā wahi piha kanaka i ka hiki: hiki ke laha maʻalahi nā germs ma ia mau wahi.
• ʻO ka loaʻa ʻana o nā lāʻau pale āpau i ka manawa kūpono: Eia nō naʻe, e nīnau i kāu kauka inā kūpono kekahi mau lāʻau pale ola iā ʻoe, no ka mea, ʻaʻole kūpono kekahi mau lāʻau pale ola no ka poʻe me nā ʻōnaehana pale nāwaliwali.

He aha kaʻu e nīnau aku ai i ke kauka?

Ke ʻike ʻoe e pili ana i kēia maʻi, nui paha kāu mau nīnau. Mai makaʻu. E kamaʻilio me kāu kauka a wehewehe i kēia mau mea:

• He aha ke kumu e emi ai koʻu pae immunoglobulin?
• He aha nā hoʻokolohua e pono ai iaʻu e loaʻa no ka ʻike/nānā ʻana i koʻu kūlana?
• He aha nā lāʻau lapaʻau āu e paipai nei?
• Aia kekahi mau hopena ʻaoʻao i ka lāʻau lapaʻau? He aha lākou?
• He aha nā hoʻololi o ke ʻano o ka nohona e pono iaʻu e hana ai e pale aku i nā maʻi?
• He aha nā hōʻailona e pono ai iaʻu e ʻike koke i ke kauka? A i ʻole e hele wau i kahi lumi pilikia (ETU) ?

Ke ʻike ʻoe ua haʻahaʻa kāu mau pae immunoglobulin, manaʻo paha ʻoe he palupalu a nāwaliwali. Manaʻo paha ʻoe, "Auē, pehea inā ʻaʻole lawa kaʻu mau koa e pale iaʻu?"

Akā, mai hopohopo. ʻO ke kamaʻilio ʻana me kāu kauka ke kaʻina mua e aʻo hou ai e pili ana i kou kūlana, nā kumu, a pehea e hiki ai i nā lāʻau lapaʻau ke kōkua. E hana pū me kāu kauka e hoʻokele i kou kūlana a loaʻa ka mālama āu e pono ai.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻO ka Hypogammaglobulinemia kahi kūlana i emi ai ka ʻōnaehana pale o ke kino i nā antibodies (immunoglobulins), e hakakā ana i nā germs. Hiki i kēia ke alakaʻi i nā maʻi pinepine.

ʻO ka mea nui loa, ʻo ia ke ʻimi i ke kōkua lapaʻau inā loaʻa mau iā ʻoe nā maʻi, ʻoiai ke ʻano maʻi like, ma kahi o ka hoʻokuʻu ʻana iā ia e like me ka mea maʻamau.

• Hiki ke loaʻa kēia ʻano i ka wā hānau, a i ʻole hiki ke ulu aʻe ma hope ma muli o kekahi maʻi a lāʻau lapaʻau paha.
• Inā ʻike ʻoe i ke kumu a loaʻa ka lāʻau kūpono, hiki iā ʻoe ke pale i nā pilikia a ola i kahi ola olakino.
• He mea nui loa e hahai i nā ʻōlelo aʻoaʻo a ke kauka, e lawe i kahi nohona olakino, a e hana i nā hana e pale ai iā ʻoe iho mai nā maʻi.

ʻAʻole ʻoe hoʻokahi, aia nā kauka a me nā limahana olakino e hiki ke kōkua iā ʻoe e hoʻoponopono i kēia kūlana. Mai makaʻu e kamaʻilio me lākou.

ʻO Hypogammaglobulinemia, Nā Antibodies, Immunoglobulin, ʻŌnaehana Palekana, Nā Maʻi, PIDD