He aha te Mate Pūngao Granulomatous Mau? Tohutohu, Take, me te Maimoatanga

Ko te mate pūngao granulomatous mau (CGD) tētahi mate tuku iho onge kaore e taea e tō pūnaha mate te whawhai tika ki ētahi mate. Whakaarohia he tiaki haumaru kei a koe, kaore e taea e rātou te mahi katoa – ka taea e rātou te hopu i ētahi kaiwhakauru, engari ka ngaro atu ētahi atu, ka waiho koe kia ngoikore atu ki ētahi momo huakita me te harore.

Ka pā tēnei mate ki te mahi a ō pūtau toto ma, ā, ko ngā pūtau e kiia nei ko ngā phagocytes. Ko ēnei pūtau hei whakamate i ngā mate pūngao mā te whakaputa i ngā matū kaha, engari i roto i te CGD, kaore tēnei tukanga e mahi tika. Ahakoa he wehi tēnei, he tokomaha ngā tāngata e ora ana i te ora katoa, i te ora kaha me te tiaki hauora tika me te maimoatanga.

He aha ngā tohu o te Mate Pūngao Granulomatous Mau?

Ka puta ngā tohu CGD i te tamarikitanga wawe, ahakoa kaore etahi e whakatauhia kia tae ra ano ki o rātou tau tekau rānei, tae noa ki te pakeketanga. Ko te tohu matua ko te whiwhi i ngā mate maha, nui, ka uaua ake te whakakore i ngā mate tamariki noa.

Koinei ngā tohu tino noa ka kite koe:

• Ngā mate ngāwari o te manawa, mate huhu rānei e haere tonu ana
• Ngā mate kiri, ngā pupuhi, ngā whara rānei e whakaora ana i te roa
• Ngā ngutu ngutu e nui ana mo ngā wiki
• Te korere roa, ngā raruraru kopu rānei
• Ngā pupuhi ate, ngā mate rānei
• Ngā mate wheua e pā ana ki te mamae me te pupuhi
• Ngā kirika maha kaore he take mārama

Ka whānau hoki ētahi i ngā granulomas – ngā rōpū iti o ngā pūtau mate e hangaia ana ina ngana te tinana ki te whawhai ki te mate. Ka taea e ēnei te aukati i ngā whekau pēnei i te kopu, ngā whekau, te ara mimi rānei, ka puta mai ngā tohu atu pēnei i te uaua ki te kai, ki te mimi rānei.

I roto i ngā take onge, ka taea e te CGD te whakaputa i ngā raruraru nui ake pēnei i ngā pupuhi roro, ngā mate ngakau rānei. Ahakoa he wehi ēnei, he onge, he taea te whakaora ina hopukina wawe me te tiaki hauora tika.

He aha ngā take o te Mate Pūngao Granulomatous Mau?

Ka puta te CGD nā ngā huringa (mutations) i roto i ngā ira e whakahaere ana i te mahi a ō pūtau mate. Ka aukati ēnei huringa ira i ō pūtau toto ma ki te hanga i tētahi huinga enzyme e kiia nei ko te NADPH oxidase, he mea nui ki te whakamate i ētahi mate pūngao.

Ko te nuinga o ngā take o te CGD he tuku iho, arā, ka tukuna te mate i ngā mātua ki ngā tamariki. Ko te momo tino noa ka pā atu ki ngā tamaiti atu i ngā kōhine, no te mea kei te kaikawe X te ira he. He kotahi noa te kaikawe X kei ngā tamaiti, no reira, ki te kawe ia i te mutation, ka whiwhi rātou i te CGD.

He rua ngā kaikawe X kei ngā kōhine, no reira, ahakoa kotahi te kawe i te mutation, ka whakarato tonu tetahi atu hauora i te tiaki. Heoi, ka taea tonu e ngā kōhine te kawe, ā, ka taea pea te whiwhi i ngā tohu ngawari.

He momo onge atu o te CGD ka taea te pā ki ngā tamaiti me ngā kōhine. Ka puta ēnei ina puta mai ngā mutations i roto i ngā ira kei ētahi atu kaikawe. I roto i ngā take onge rawa, ka taea e te CGD te whānau mai kaore he hītori whānau nā ngā huringa ira hou.

He aha ngā momo o te Mate Pūngao Granulomatous Mau?

Ka whakarōpū ngā tākuta i te CGD i runga i te ira motuhake e pā ana, me te āhua o te tuku iho o te mate. Ko te mārama ki tō momo ka āwhina i tō rōpū hauora ki te whiriwhiri i te huarahi maimoatanga pai.

Ko te momo tino noa ko te CGD X-linked, e 65% te nui o ngā take katoa. Ko tēnei momo ka pā nui ki ngā tamaiti, ā, ka nui ake ngā tohu. Ko ngā tamaiti e whai ana i te CGD X-linked ka maha tonu o rātou mate nui tuatahi i mua i te tau 2.

Ko te CGD autosomal recessive te toenga o ngā take, ā, ka pā ki ngā tamaiti me ngā kōhine. Ko tēnei momo ka iti ake ngā tohu, ā, kaore pea e whakatauhia kia tae ra ano ki te muri o te tamarikitanga, tae noa ki te pakeketanga. Ka iti pea ngā mate o ngā tāngata e whai ana i tēnei momo, iti rānei ngā raruraru.

I roto i ēnei kāwai matua, he maha ngā momo iti i runga i te wāhanga motuhake o te pūnaha NADPH oxidase kaore e mahi tika. Ka taea e tō tākuta te whakatau i tō momo motuhake mā te whakamātautau ira, ka āwhina ki te kī he aha ngā mate ka whiwhi koe, ā, ka arataki i ngā rautaki aukati.

Nōhea te haere ki te tākuta mō te Mate Pūngao Granulomatous Mau?

Me whakapiri atu koe ki tō tākuta ki te kite koe i te tauira o ngā mate maha, nui, ranei onge. Ahakoa ka mate ngā tamariki katoa i etahi wā, ka nui ake te kino o ngā mate CGD, ka uaua ake te whakaora i ngā mate tamariki noa.

Rapua te awhina hauora wawe ki te pāngia koe e te mate huhu ngāwari, ngā pupuhi kiri kaore e whakaora mā te maimoatanga noa, ngā kirika roa rānei kaore he take mārama. Ka taea e ēnei te tohu kei te hiahia tō pūnaha mate ki te tautoko atu.

Ki te whai koe i te hītori whānau o te CGD, ngā mate onge rānei, kōrerohia tēnei ki tō kaiwhakarato hauora. Ko te whakatau me te maimoatanga wawe ka nui te rerekētanga ki te aukati i ngā raruraru nui, ki te āwhina i a koe ki te noho hauora.

Mō ngā tāngata kua whakatauhia te CGD, waea atu ki tō tākuta tonu ki te whānau mai ngā tohu hou pēnei i te mamae o te kopu nui, te uaua ki te manawa, te ruaki roa, ngā tohu mate rānei. Ka taea e te maimoatanga tere te aukati i ngā take iti mai i te riro hei raruraru nui.

He aha ngā take morearea mō te Mate Pūngao Granulomatous Mau?

Ko te take morearea nui mō te CGD ko te whai i roto i tō whānau. No te mea he mate tuku iho tēnei, ka whakawhirinaki tō morearea ki te hītori ira o tō whānau.

Koinei ngā mea e whakapiki ana i ō tūmanako ki te whai CGD:

• Te whai mātua e kawe ana i te mutation ira CGD
• Ko te tane (mō te momo X-linked tino noa)
• Te whai hītori whānau o ngā mate maha, nui
• Te whai whānau i mate tamariki nā ngā mate
• Ngā mātua e hono ana tetahi ki tetahi (ka whakapiki i te morearea o ngā momo autosomal recessive)

Kāore i rite ki ngā āhuatanga hauora maha, kāore te CGD e pā ana ki ngā take oranga pēnei i te kai, te whakakori tinana, ngā whakaaturanga taiao rānei. Kāore e taea e koe te aukati, te whakaputa rānei i te CGD mā ō mahi – he ira noa iho.

Ki te whakarite koe i tētahi whānau, kei te CGD i roto i tō hītori whānau, ka taea e te tohutohu ira te āwhina i a koe ki te mārama ki ngā morearea me ngā kōwhiringa. Ka āwhina ēnei korero i a koe ki te whakatau whakamārama mō te whakarite whānau.

He aha ngā raruraru pea o te Mate Pūngao Granulomatous Mau?

Ahakoa ngā raruraru CGD e rangona ana he nui, mahara ko te nuinga ka taea te aukati, te whakaora rānei me te tiaki hauora tika. Ko te matua ko te noho i runga i tō mahere maimoatanga, me te mahi tata atu ki tō rōpū hauora.

Ko ngā raruraru tino noa ko ēnei:

• Ngā mate manawa nui ka taea te whakaputa i te toka
• Ngā pupuhi ate pea e hiahia ana ki te whakaheke
• Ngā granulomas e aukati ana i te kopu, ngā whekau, te ara mimi rānei
• Te korere roa me te iti o te taumaha
• Ngā mate wheua me ngā hononga
• Ngā mate kiri me ngā whara e whakaora ana i te roa

Ko ngā raruraru iti ake, nui ake te kino, ka taea te whakauru i ngā pupuhi roro, ngā mate ngakau, te mumura o te whekau nui rānei. Ahakoa he wehi ēnei, he onge ina whakahaere tika te CGD me ngā rongoā aukati me te arotautanga hauora noa.

Ka whānau hoki ētahi o ngā tāngata e whai ana i te CGD i ngā raruraru mate pūnaha, kei reira te pūnaha mate e whakaeke ana i ngā kiko tinana hauora. Ka taea e tēnei te whakaputa i ngā āhuatanga pēnei i te mate whekau mumura, te mate pūkahukahu rānei. Ka titiro tō rōpū hauora ki ēnei take, ka whakaora i a rātou ki te whānau mai.

Me pēhea te whakatau i te Mate Pūngao Granulomatous Mau?

Ko te whakatau i te CGD ka tīmata mā tō tākuta e kite i te tauira o ngā mate onge, maha rānei. Ka ui rātou i ngā pātai taipitopito mō tō hītori hauora, me tetahi hītori whānau o ngā raruraru mate pūnaha, ngā mate tamariki rānei i mate i ngā mate.

Ko te whakamātautau matua mō te CGD ko te whakamātautau dihydrorhodamine (DHR). Ka ine tenei whakamātautau toto i te pai o te whakamate a ō pūtau toto ma i ngā mate pūngao. I roto i ngā tāngata e whai ana i te CGD, ka whakaatu tēnei whakamātautau kaore ngā pūtau e mahi tika.

Ka taea hoki e tō tākuta te tono i te whakamātautau ira hei whakau i te whakatau, hei whakatau i te momo CGD e whai ana koe. Ka whakauruhia tēnei ki te tātari i tō DNA hei kimi i ngā mutations ira motuhake e whakaputa ana i te mate.

Ka taea e ngā whakamātautau atu te whakauru i ngā tikanga o ngā wāhi kua pangia hei whakatau he aha ngā mate pūngao e whakaputa ana i ngā raruraru. Ka āwhina tēnei i tō rōpū hauora ki te whiriwhiri i ngā rongoā antibiotic me ngā rongoā antifungal tino whai hua mō tō āhuatanga.

He aha te maimoatanga mō te Mate Pūngao Granulomatous Mau?

Ko te maimoatanga CGD e aro nui ana ki te aukati i ngā mate, ki te whakaora wawe i a rātou ina puta mai. Ahakoa kaore he rongoā mō te mate ira taketake, he maimoatanga pai te āwhina i te nuinga o ngā tāngata e whai ana i te CGD ki te ora i te ora noa, hauora.

Ko te pūtake o te maimoatanga CGD ko te tango i ngā rongoā aukati i ngā rā katoa. Ko te nuinga o ngā tāngata e tango ana i te trimethoprim-sulfamethoxazole (e kiia ana hoki ko Bactrim, Septra rānei) hei aukati i ngā mate huakita. Ka tango pea koe i te rongoā antifungal pēnei i te itraconazole hei aukati i ngā mate harore.

He tokomaha ngā tāngata e whiwhi ana i ngā werohanga interferon gamma, e hoatuia ana i te toru o ngā wiki. Ka āwhina tēnei rongoā ki te whakapiki i te kaha o tō pūnaha mate ki te whawhai ki ngā mate. Ahakoa te hoatu i a koe ngā werohanga e rangona ana he wehi, ka tere te noho mārama o te nuinga o ngā tāngata ki te mahi auau.

Ina puta mai ngā mate, ka whakaorahia rātou me ngā rongoā antibiotic, antifungal rānei kaha. Me noho pea koe ki te hōhipera mō ngā rongoā intravenous, ina koa mō ngā mate nui pēnei i te mate huhu, ngā pupuhi ate rānei.

Mō ngā take nui, ka taea e ngā tākuta te tūtohutia he whakawhiti wheua wheua (e kiia ana hoki ko te whakawhiti pūtau pūtau). Ka taea e tēnei tukanga te whakaora i te CGD mā te whakakapi i ō pūtau mate he ki ngā pūtau hauora mai i tētahi kaiwhakawhiwhi. Heoi, he morearea nui ngā whakawhiti, kāore hoki e tika mō te katoa.

Me pēhea te whakahaere i te Mate Pūngao Granulomatous Mau i te kāinga?

Ko te ora pai me te CGD ko te tango i tētahi wāhi kaha ki te aukati i ngā mate, me te pupuri i te ora noa i te mea ka taea. Ko te nuinga o ngā tāngata e whai ana i te CGD ka taea te haere ki te kura, te mahi, te whakakori tinana, te koa ki ngā mahi noa me ētahi whakamaramatanga ngāwari.

Tangohia ō rongoā tika i te mea i whakaritea, ahakoa kei te hauora koe. Ko te ngaro i ngā rongoā aukati, antifungal rānei ka whakapiki nui i tō morearea mate. Whakaritea ngā pūnaha pēnei i ngā kaiwhakarite papa, ngā whakamaumahara waea rānei hei āwhina i a koe ki te noho mau.

Whakamahia te horoi pai mā te horoi i ō ringa maha, me te tino horoi. Kaua e mahi i ngā mahi ka taea te whakaputa i a koe ki ngā huakita, harore rānei nui, pēnei i te mahi mā te kāinga kaore he karapu, te horoi i ngā paru kararehe, te kauhoe i ngā roto, ngā puku wai wera rānei.

Pupuri i tō taiao kia ma, kaua e manukanuka nui ki a ia. Ko te horoi whare noa ka nui – kāore koe e hiahia ki te noho i roto i te pokapū mā. Arotake ki ngā wāhi e huihui ana ngā mate pūngao, pēnei i ngā ruma kaukau me ngā kīhini.

Noho whakahoutia ki ngā werohanga katoa i tūtohutia, engari kaua e werohanga ora ki te kore e whakaae motuhake tō tākuta. Kāore pea tō pūnaha mate e taea te whakahaere i ngā werohanga ora, no reira ka arataki tō rōpū hauora i a koe ki ngā whakangungu e tika ana.

Me pēhea te whakarite mō tō hui tākuta?

Ko te whakarite mō ō hui CGD ka āwhina i te whakarite ka whiwhi koe i te nuinga mai i tō wā me tō rōpū hauora. Haere mai ki te kōrero i ngā tohu hou, ngā awangawanga rongoā, ngā pātai rānei mō te whakahaere i tō mate.

Pupuri i tētahi raarangi tohu e tohu ana i ngā kirika, ngā mate, ngā tohu onge rānei mai i tō haerenga whakamutunga. Whakauruhia ngā kōrero pēnei i te wā i tīmata ai ngā tohu, te roa o te roa, me ngā maimoatanga i āwhina. Ka āwhina ēnei korero i tō tākuta ki te kite i ngā tauira, ki te whakarereke i tō maimoatanga ki te hiahiatia.

Kawe mai i te rārangi katoa o ngā rongoā katoa e tangohia ana e koe, tae atu ki ngā rāpoi tika me te auau o te tango. Kōrerohia hoki ngā taapiri, ngā rongoā-kore-whakahaunga, ngā rongoā otaota rānei e whakamahia ana e koe, no te mea ka taea e ēnei te pā ki ō rongoā CGD.

Tuhia ngā pātai i mua i tō hui kia kaua e wareware i tetahi mea nui. Ko ngā pātai noa pea ko ngā awangawanga mō ngā tohu hou, ngā pānga o ngā rongoā, ngā herenga mahi, te mahi rānei ki te mate koe.

Ki te kite koe i tētahi tākuta hou, tohunga rānei, kawe mai ngā kape o ngā hua whakamātautau hou, ngā rēkōta hōhipera, me te whakarapopototanga o tō hītori CGD. Ka āwhina ēnei korero papamuri i ngā kaiwhakarato hou ki te mārama ki tō āhuatanga motuhake, ki te whakatau i ngā whakatau maimoatanga pai ake.

He aha te mea nui mō te Mate Pūngao Granulomatous Mau?

Ko te CGD he mate nui engari ka taea te whakahaere e hiahia ana ki te tiaki hauora haere tonu, ki te aro ki te aukati. Ahakoa ka rangona te whakatau he nui, mahara kua pai ake ngā maimoatanga i ngā tau kua pahure, ā, ka taea e te nuinga o ngā tāngata e whai ana i te CGD te ora i te ora katoa, kaha.

Ko te mea nui rawa atu ka taea e koe ko te tango i ō rongoā aukati tonu, me te mahi tata atu ki tō rōpū hauora. Ka āwhina tēnei huarahi hoa ki te hopu i ngā raruraru wawe, ki te pupuri i a koe i te hauora i te mea ka taea.

Kaua e tuku i te CGD ki te whakatau, ki te whakaiti rānei i a koe. Me ngā whakamaramatanga tika me te tiaki hauora, ka taea e koe te whai i te mātauranga, ngā whāinga mahi, ngā hononga, ngā hākinakina pēnei i tetahi atu. Ko te matua ko te kimi i te taurite tika i waenganui i te noho tupato tika me te ora katoa.

Noho hono atu ki ngā rōpū tautoko, ngā hapori ipurangi rānei mō ngā tāngata e whai ana i te CGD me ō rātou whānau. Ko te whakawhiti wheako ki ētahi atu e mārama ana ki ō wero ka taea te whakarato i ngā tohutohu mahi me te tautoko ngākau e whakaputa ana i te rerekētanga pono ki tō oranga o ia rā.

Ngā pātai maha e uihia ana mō te Mate Pūngao Granulomatous Mau

Ka taea e ngā tāngata e whai ana i te CGD te ora i ngā tau ora noa?

Ae, ka taea e te nuinga o ngā tāngata e whai ana i te CGD te ora i ngā tau ora noa, tata noa rānei ki te tiaki hauora tika. Ko te whakatau wawe me te maimoatanga mau me ngā rongoā aukati kua pai ake ngā hua. Ahakoa e hiahia ana te CGD ki te whakahaere hauora haere tonu, ka taea e te nuinga o ngā tāngata te whai i te mātauranga, ngā mahi, ngā hononga, me te ora whānau.

He mate pāhoko te CGD?

Kāo, kāore te CGD i te mate pāhoko. He mate ira i whānau mai koe, ehara i te mea ka taea te hopu mai i ētahi atu tāngata, te hoatu rānei ki ētahi atu tāngata. Heoi, he nui ake te ngoikore o ngā tāngata e whai ana i te CGD ki ētahi mate, no reira me tupato rātou ki ētahi atu e mate ana hei tiaki i a rātou.

Ka taea e ngā wāhine e whai ana i te CGD te whānau tamariki?

Ae, ka taea e ngā wāhine e whai ana i te CGD te whānau tamariki, ahakoa e hiahia ana te haputanga ki te arotautanga hauora atawhai. Ko ngā mea nui ko te whakahaere i ngā rongoā i te wā hapu, me te mārama ki ngā morearea ira mō te pēpi. Ko te tohutohu ira i mua i te hapu ka āwhina i ngā whānau ki te mārama ki ngā tauira tuku iho, ki te whakatau whakamārama.

He aha ngā mate ka whiwhi pea ngā tāngata e whai ana i te CGD?

He tino ngoikore ngā tāngata e whai ana i te CGD ki ngā mate mai i ngā huakita pēnei i te Staphylococcus, Serratia, me te Burkholderia, me ngā harore pēnei i te Aspergillus me te Candida. He āhuatanga motuhake kei ēnei mea e uaua ai te whawhai a ngā pūnaha mate CGD. Heoi, ka whakaiti nui ngā rongoā aukati i te morearea o ēnei mate.

Ka taea te whakaora i te CGD?

I tēnei wā, ko te whakawhiti wheua wheua anake te rongoā pea mō te CGD, engari kāore e tika mō te katoa nā ngā morearea e pā ana. Ko te nuinga o ngā tāngata e whakahaere ana i te CGD me ngā rongoā aukati me te arotautanga atawhai. Ko te rangahau rongoā ira e whakaatu ana i te tumanako mō te heke mai, engari he whakamātautau tonu ēnei maimoatanga, kāore hoki i te whānuitia.