He maha tonu ōu pāngia e te mate? Koinei pea te take: Me ako tātou mō te hypogammaglobulinemia.

He maha ngā wā ka pāngia koe e te makariri, te rewharewha, me te maremare? He maha rānei ngā wā ka pāngia tō tamaiti e ngā mate taringa, e ngā raruraru sinus rānei? I ētahi wā ka whakaaro tātou he mate noa ēnei, engari ki te hoki mai anō ēnei mate, tera pea he take kē atu. Ko tētahi mate ka taea te whakapae i ēnei wā ko te hypogammaglobulinemia. Ahakoa he ingoa roa, me mārama noa iho.

He aha te hypogammaglobulinemia?

Ki te whakarāpopototanga, ko te hypogammaglobulinemia he āhuatanga e iti ana te taumata o ngā paturopi i roto i tō tinana. Ko tētahi atu ingoa mō ēnei paturopi ko ngā immunoglobulin. Whakaarohia inaianei he rite tō tātou tinana ki tētahi pā. Ko ngā hoariri e tae mai ana ki tēnei pā ko ngā iroriki, pērā i ngā huaketo me ngā huakita. Nō reira, ko ngā hōia e tiaki ana i a tātou mai i ēnei hoariri ko tō tātou pūnaha ārai mate .

He momo pūtau motuhake tō tēnei pūnaha ārai mate e kiia nei ko ngā pūtau-B . He rite rātou ki ngā kōmana kua whakangungua motuhaketia. Ina uru mai he iroriki ki te tinana, ka whakaputahia e ēnei pūtau-B ngā paturopi e kiia nei ko ngā immunoglobulin. Ka haere ēnei paturopi ki te hopu i te iroriki ka whakangaro. Koinā te wā kāore tātou e pāngia e te mate, kāore rānei tātou e ora wawe i te mate.

Ka kitea noa tēnei āhuatanga ina whakamātauhia tō toto.

Me wehewehe ēnei kupu kia mārama ai:

• Haupō: ko tōna tikanga kei raro iho i te tikanga.
• Ngā globulin gamma: Koinei ngā momo immunoglobulin tino noa.
• Emia: ko tōna tikanga kei roto i te toto.

Nō reira, ina heke te taumata o tēnei immunoglobulin i roto i tō toto, ka ngoikore haere te pūnaha parepare o tō tinana. Kātahi ka taea e ngā iroriki te whakaeke ngāwari i tō tinana. Nō reira , ka nui ake te tūponotanga ka pāngia koe e ngā mate me te whakawhanake i ētahi atu mate.

He momo pēnei kei reira?

Ae, ka pāngia ngā tamariki me ngā pakeke e te mate e kiia nei ko te hypogammaglobulinemia. E rua ngā momo matua:

1. Hypogammaglobulinemia tuatahi (whanau): Ka puta tēnei i ētahi mate ira onge. Ka kiia ēnei ko ngā Mate Ārai Mate Tuatahi (PIDD) . I te kīanga māmā noa, ina whānau koe, ka whānau mai koe me tētahi hapa (whakarerekētanga) i roto i ō ira. Ko ēnei ira ngā mea e tohutohu ana i te pūnaha ārai mate me pēhea te mahi. Nō reira, ina he hapa i roto i taua tohutohu, ka whakaitihia te ārai mate. He maha ngā wā ka taea te tuku iho i ēnei hapa ira mai i tētahi whakatipuranga ki tētahi whakatipuranga.

2. Te hypogammaglobulinemia tuarua (kua whiwhi): KoineiKo te momo tino noa. Ka puta pea tēnei mate i ngā momo mate hauora, i te pānga taha rānei o ētahi rongoā. Ko te tikanga ka whānau hauora koe, ā, ka pāngia koe e tēnei mate i muri mai i te ao.

E hia te onge o tēnei āhuatanga?

Ko te hypogammaglobulinemia tētahi o ngā mate ārai mate tuatahi (PIDD) i whakahuatia i mua ake nei. Heoi, ehara ngā PIDD i te mate tino noa. Hei tauira, e kiia ana e tata ana ki te hawhe miriona tāngata i te Hononga o Amerika e noho ana me te PIDD.

He aha ngā tohu o tēnei?

Ko te tohu matua o te hypogammaglobulinemia i roto i ngā tamariki me ngā pakeke ko ngā mate pāwera auau, roa rānei. Ka whakawhirinaki ngā tohu ki te momo mate e pā ana. Ko ngā mate pāwera me ngā tohu e pā ana ki te hypogammaglobulinemia ko:

• Ngā mate taringa: mamae taringa, whara te parāoa taringa (i ētahi wā he koha), kirika. (Whakaarohia kei te pāngia ētahi tamariki e ngā mate taringa roa, e hoki mai ana ahakoa te rongoā.)
• Ngā mate pāwera o te sinus: Te ihu pupuhi, te ihu pūngāwerewere rānei, te ngenge, te kirika. (Ka ara ake ētahi tāngata i te ata me te māuiui me te taumaha o te mahunga, tika? Ki te mau tonu, me āwangawanga koe.)
• Bronchitis: Te maremare tonu, te poto o te manawa, te kirika.
• Pneumonia: Te kikī o te uma, te mamae rānei, te uaua ki te manawa, te kirika.
• Rewharewha puku: korere, whakapairuaki, ruaki, kirika.
• Mate kiriuhi: Te kaki maro, te ānini, te kirika. (He ahua morearea tēnei, me tere te kimi.)
• Ngā mate kiri: ngā whara kiri, te pupuhi, te mākūkū, te kirika.

Waihoki, ka rerekē pea ngā tohu i runga i te āhua o te mate i puta ai te iti o ngā taumata immunoglobulin. Hei tauira, he nui ake te tūponotanga o te tamaiti whai mate pāwera ki ētahi momo PIDD. Ko ētahi atu momo ka whakaroa i te tipu me te whanaketanga.

He aha te take o tēnei?

E rua ngā take pea. Kāore e ranea te hanga o tō pūnaha ārai mate i ngā immunoglobulin, kāore rānei e ranea ngā immunoglobulin e hangaia ana e ia. He rerekē ngā take i runga i te mea he lupus tuatahi, he lupus tuarua rānei tōu.

Ngā take o te hypogammaglobulinemia tuatahi

Ko ētahi o ngā momo PIDD tino noa e hono ana ki tēnei ko:

• Te ngoikore o te ārai mate taurangi noa (CVID): He hapa ira e kore ai te tinana e hanga i ngā immunoglobulin e ranea ana. Koinei te tino take o te hypogammaglobulinemia tuatahi i roto i ngā pakeke.
• - (X-linked Agammaglobulinemia - XLA): X ‍ - (B-cells) . ‍ ( X ). XLA .
• Te Hypogammaglobulinemia Poto o te Pēpi (THI): Ka puta tēnei ina tīmata te heke o ngā taumata immunoglobulin o te pēpi i te 3 marama te pakeke. Ka pāngia pea ngā pēpi whai THI e ngā mate, engari he maha ngā wā kāore e kitea he tohu. Ka memeha haere te mate i a ia anō i te 3 tau te pakeke.
• Te ngoikore o te IgA whiriwhiri: Koinei te wā kāore e nui te hanga a te tinana i tētahi momo immunoglobulin motuhake e kiia nei ko te Immunoglobulin A.
• Ngā mate Hyper-IgM: I roto i tēnei, ko ētahi momo immunoglobulin ka puta noa, ka nui rawa rānei, engari ko ētahi kāore e puta i te nui.

Haunga tēnei, kei reira anō ētahi atu momo PIDD e hua ai te hypogammaglobulinemia tuatahi:

• Ataxia-telangiectasia
• Agammaglobulinemia heke-ā-roto (ARA)
• Te mate pai (GS)
• Ngā ngoikoretanga motuhake o te immunoglobulin kore-IgG
• Te ngoikore nui o te ārai mate (SCID)
• Te ngoikore o te paturopi motuhake (SAD)
• Te mate Wiskott-Aldrich (WAS)

Ngā take o te hypogammaglobulinemia tuarua

Ko ngā take e whai ake nei ka puta ai tēnei āhuatanga i muri mai:

• Ngā Rongoā: Ka taea e ētahi rongoā te whakaiti i ngā taumata immunoglobulin. Hei tauira, ko ngā rongoā ārai mate, ngā rongoā ārai-hōhā, ngā corticosteroids, me ngā rongoā chemotherapy.
• Te mate nephrotic: I tēnei āhua, ka nui rawa te pūmua e tukuna ana e ō whatukuhu i roto i tō mimi. Ka taea te tuku i ngā immunoglobulins, he pūmua anō hoki, mā tēnei ara.
• Te enteropathy ngaro-pūmua: Koinei te wā ka ngaro rawa te pūmua mai i tō tinana mā roto i tō pūnaha gastrointestinal.
• Matepukupuku:Ka taea e ngā mate pukupuku e pā ana ki ō pūtau-B te whakaiti i ngā taumata immunoglobulin. Hei tauira, ko ngā mate pukupuku pēnei i te mate pukupuku lymphocytic leukemia mau tonu, te lymphoma, me te myeloma maha.
• HIV: He mate urutā te HIV (STI). Ka taea hoki e ia te whakaputa i te hypogammaglobulinemia me ētahi atu raruraru pūnaha ārai mate.

He aha ngā raruraru ka pā mai i tēnei?

Ki te kore e rongoatia, ka taea e te hypogammaglobulinemia te arahi ki ngā mate kino, tae atu ki ngā raruraru morearea ki te ora . Ko tētahi raruraru noa ko te bronchiectasis . He āhuatanga tēnei ka pakaru tonu ngā pukahukahu. He nui ake te kitea o tēnei āhuatanga i roto i te hunga e pāngia ana e ngā mate urutā o runga.

Whakaarohia tētahi tangata e pāngia ana e ngā raruraru pūkahukahu, arā, te maremare tonu me te huware. Ina pakaru tonu ōna pūkahukahu, ka noho hei mate pumau.

Hei tāpiri, ka whakanui ake te hypogammaglobulinemia i te mōrearea o:

• Ngā mate pūnaha ārai mate: Koinei ngā āhuatanga e whakaekea ai e tō ake pūnaha ārai mate ngā pūtau o tō tinana.
• Te kino ki ngā whekau o roto.
• Matepukupuku.
• Sepsis: He mate kino tēnei e horapa puta noa i te tinana.

Me pēhea koe e mōhio ai ki tēnei?

Ka whakahaerehia e ngā tākuta he whakamātautau toto hei ine i ō taumata immunoglobulin. Mena he iti iho ō taumata i te mea noa, tera pea ka kitea he hypogammaglobulinemia koe. Kātahi ka whakarōpūtia e tō tākuta tō āhua ki te ngawari, ki te āhua waenga, ki te kino rānei .

Hei kimi i te take o tēnei taumata iti, ka whakaarohia e te tākuta ngā mea e whai ake nei:

• Ō tohumate: Ina koa mēnā he maha ō mate pāwera.
• Tō hītori hauora whānau: Kei roto i tō whānau tetahi o te PIDD?
• Ngā rongoā e tangohia ana e koe: Ina koa ko ngā rongoā ka pāngia ai te hypogammaglobulinemia hei pānga taha.

Me whai whakamātautau anō pea koe, tae atu ki:

• Ētahi atu whakamātautau toto: Hei tauira, ngā tatau toto katoa, ngā whakamātautau paturopi, me ngā whakamātautau e tirotiro ana i te mahi a ō whekau.
• Ngā tikanga whakaahua: Hei tirotiro mēnā he kino te kiko nā te mate, nā te pukupuku rānei (hei tauira, he hihi-X, he matawai CT)
• Ngā whakamātautau ira: Tirohia mēnā he hapa ira e pā ana ki te PIDD.
• Whakamātautau koiora:Te tango tauira kiko me te whakamātautau kia kitea ai mēnā he pūtau matepukupuku.

Me pēhea te rongoā mō tēnei?

Ko te maimoatanga e whakawhirinaki ana ki te pūtake o tō hypogammaglobulinemia me tōna kaha. Tērā pea ka tukuna koe ki tētahi tohunga ārai mate.

Mena he mate tuarua tēnei nō tētahi mate hauora , ka rongoāhia e ngā tākuta te mate taketake. Hei tauira, ki te iti ō taumata immunoglobulin nā te pānga taha o tētahi rongoā e tangohia ana e koe, tera pea ka whakarerekētia te rongoā.

Mena kei a koe te hypogammaglobulinemia tuatahi , me noho koe i raro i te tirotiro a te taote mō te toenga o tō oranga hei tiaki i a koe mai i ngā mate kino. Ko ngā kōwhiringa maimoatanga pea ko:

• Te tatari mataara: Ki te kore e nui te mōrearea hauora o ō taumata immunoglobulin iti, ka aroturukihia pea tō āhua e tō tākuta. Ko ngā āhuatanga ka memeha noa iho, pērā i te hypogammaglobulinemia o te pēpi (THI), i whakahuatia i mua ake nei, kāore pea e hiahiatia he maimoatanga.
• Ngā rongoā paturopi: Tērā pea ka hoatu he rongoā paturopi ki a koe hei rongoā i te mate kitakita, hei ārai rānei i te mate.
• Te rongoā whakakapinga Immunoglobulin (IgG): Mena he tino iti ō taumata immunoglobulin, ka tukuna pea he immunoglobulin ki a koe mai i tētahi kaituku. Ka tukuna tēnei hei werohanga ki roto i tētahi uaua (IV) i raro rānei i te kiri (i raro i te kiri). Ko te hunga whai PIDD e hiahia ana ki tēnei maimoatanga me tango i tēnei maimoatanga mō te toenga o ō rātou oranga.
• Te whakawhitinga pūtau take: Ki te kore e taea e tō tinana te hanga pūtau ārai mate hauora, ka taea e koe te whiwhi whakawhitinga pūtau take mai i tētahi kaituku. Ka whanake ēnei pūtau take hei pūtau toto hauora i muri mai, tae atu ki ngā pūtau-B.

He āhuatanga kino tēnei?

Ka taea e te hypogammaglobulinemia te kino rawa atu. Kei te pūtake me te iti o ngā taumata o tō immunoglobulin. He rite ngā immunoglobulin ki ngā hōia i roto i ō tātou tinana e whawhai ana ki ngā mate kino pēnei i ngā huaketo, ngā huakita, ngā pirinoa, me ngā harore. Nō reira, ina kore ēnei hōia e tiaki i a tātou, ka nui ake te tūponotanga ka pāngia tātou e ngā mate kino, maha. Ki te kore e rongoatia, ka taea e ngā mate kino te whakararu i ngā kiko mō te wā roa, tae atu ki te mate.

Nō reira, kaua tēnei āhuatanga e whakahaweatia. Mēnā ka puta ngā tohu, he mea nui kia rapu tohutohu hauora tonu.

Ka aha mēnā ka kore e rongoatia?

He mea nui kia tirohia e te tākuta te pūtake o te hypogammaglobulinemia. Ki te tīmata wawe koe i te maimoatanga, he nui ake te tūponotanga ka noho hauora koe, ka ārai hoki i ngā raruraru ka puta i ngā tau i muri mai. Mena he mate tōu e whakararu ana i a koe ki te hypogammaglobulinemia, ka aroturukihia e tō tākuta ō taumata immunoglobulin i ia wā. Ka tūtohu rātou i tētahi mahere maimoatanga hei āwhina i te ārai i ngā mate kino.

Ka taea te ārai i tēnei?

Kāore e taea e mātou te ārai i ngā āhuatanga (pēnei i ngā hapa ira) e hua ai te hypogammaglobulinemia. Heoi, ka taea e tō tākuta te tūtohu i ngā maimoatanga hei āwhina i te ārai i ngā raruraru , pērā i te mate urutā auau me te kino o ngā pukahukahu.

Me pēhea taku tiaki i ahau anō? He aha ngā mea ka taea e au te mahi hei ārai i ngā mate urutā?

Ko te mea nui ko te whai i ngā tohutohu a tō tākuta. Mā ēnei mea ka āwhina i te ārai i te mate:

• Horoia ō ringaringa kia tino horoia: Horoia ō ringaringa ki te hopi me te wai mō te 20 hēkona pea. Kia tino horoia ō ringaringa i muri i te haerenga ki waho, i mua i te kai, me muri i te whakamahinga o te wharepaku.
• Te mau i te kopare kanohi ina hiahiatia hei ārai i te horapa o ngā iroriki e rere ana i te rangi: He mea nui rawa tēnei ina haere ki ngā wāhi kī tonu i te tangata, me ngā wā e nui haere ana te mate.
• Kia matara atu i te hunga tūroro me ngā wāhi kī tonu i te tangata: ka horapa ngāwari ngā iroriki ki aua wāhi.
• Te tiki i ngā kano kano katoa i te wā tika: Heoi, pātai atu ki tō tākuta mēnā he tika ētahi kano kano ora māu, nā te mea kāore pea ētahi kano kano ora e tika mō te hunga ngoikore te pūnaha ārai mate.

He aha taku e pātai atu ai ki te tākuta?

Ina rongo koe mō tēnei mate, tera pea he maha āu pātai. Kaua e mataku. Kōrero ki tō tākuta, ka whakamārama i ngā mea e whai ake nei:

• He aha te take e heke ai ngā taumata o taku immunoglobulin?
• He aha ngā whakamātautau e hiahiatia ana e au hei tātaritanga/aroturuki i tōku āhua?
• He aha ngā maimoatanga e taunaki ana koe?
• He pānga taha kei te rongoā? He aha ēnei?
• He aha ngā huringa āhua noho me mahi e au hei ārai i ngā mate urutā?
• He aha ngā tohu e tika ana kia kite tonu ahau i te tākuta? Me haere rānei ahau ki te ruma ohorere (ETU) ?

Ina kitea e koe he iti te taumata o tō immunoglobulin, tera pea ka ngoikore koe, ka ngoikore hoki. Tērā pea ka whakaaro koe, "Āe, me pēhea mēnā kāore i te nui aku hōia hei tiaki i ahau?"

Kaua e manukanuka. Ko te kōrero ki tō tākuta te taahiraa tuatahi ki te ako atu mō tō āhua, ngā take, me ngā huarahi e taea ai e ngā maimoatanga te āwhina. Mahi tahi me tō tākuta ki te whakahaere i tō āhua me te tiki i te tiaki e hiahiatia ana e koe.

Hei whakamutunga, ngā mea hei maumahara

Ko te hypogammaglobulinemia he āhuatanga e iti ai ngā paturopi (immunoglobulin) o te pūnaha ārai mate o te tinana, e whawhai ana ki ngā iroriki. Ka taea e tēnei te arahi ki ngā mate maha.

Ko te mea nui ko te rapu tohutohu hauora mēnā kei te pāngia tonu koe e ngā mate urutā, inā koa ko taua momo mate urutā anō, kaua e waiho noa iho he mea noa.

• Ka kitea pea tēnei āhuatanga i te whānautanga mai, ka pā mai rānei i muri mai nā tētahi atu mate, nā tētahi atu rongoā rānei.
• Ki te kitea e koe te take, ā, ka whiwhi koe i te maimoatanga tika, ka taea e koe te ārai i ngā raruraru me te ora i te oranga hauora.
• He mea tino nui kia whai i ngā tohutohu a te tākuta, kia ora ai te noho, kia mahi hoki i ngā mahi hei tiaki i a koe anō mai i ngā mate urutā.

Kāore koe i te mokemoke, kei reira ngā tākuta me ngā kaimahi hauora hei āwhina i a koe ki te whakatau i tēnei āhuatanga. Kaua e wehi ki te kōrero ki a rātou.