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ʻO ka Idiopathic fibrosing alveolitis (IFA) kahi maʻi e mau nei kekahi o nā mea liʻiliʻi loa i aʻo ʻia, ma waena o nā pathologies o ka interstitium o nā māmā. Me kēia ʻano alveolitis, hiki mai ka mumū o ka interstitium pulmonary me kona fibrosis. ʻO ka ʻeha, me nā ala ea, parenchyma māmā. Hoʻopilikia maikaʻi kēia i ka mokuʻāina o nā ʻōpū hanu, alakaʻi i kā lākou hoʻololi paʻa, hoʻopau i ka hoʻololi kinoea a me ka hāʻule ʻole o ka hanu, kahi e make ai.
Ua kapa ʻia ʻo Idiopathic fibrosing alveolitis i ka idiopathic pulmonary fibrosis. Hoʻohana nui ʻia kēia huaʻōlelo e nā loea Pelekane (idiopathic pulmonary fibrosis), a me nā pulmonologists Kelemania (idiopa-thische Lungenfibrose). Ma UK, kapa ʻia ʻo ELISA "cryptogenic fibrosing alveolitis" (cryptogenic fibrosing alveolitis).
ʻO nā huaʻōlelo "cryptogenic" a me "idiopathic" he mau ʻokoʻa, akā ua hoʻohana ʻia i kēia manawa. ʻO ke ʻano o kēia mau ʻōlelo ʻelua, ʻaʻole maopopo ke kumu o ka maʻi.
Epidemiology a me nā mea pilikia
He kū'ē loa ka ʻikepili helu e hōʻike ana i ka laha ʻana o ka maʻi. Ua manaʻo ʻia ʻo ia mau ʻokoʻa ma muli o ka hoʻopili ʻana o nā maʻi ʻaʻole wale me ka idiopathic fibrosing alveolitis, akā pū kekahi me nā idiopathic interstitial pneumonias (IIP).
Ma loko o 100 mau kāne, 000 ka poʻe e ʻike i ka maʻi maʻi, a he 20 mau kānaka mai 100 mau wahine. I ka makahiki, 000 kanaka e ma'i no kela 13 kane, a he 100 kanaka no kela 000 wahine.
ʻOiai ʻaʻole ʻike ʻia nā kumu o ka idiopathic alveolitis i kēia manawa, ʻaʻole hoʻōki ka poʻe ʻepekema i ka hoʻāʻo ʻana e ʻike i ke ʻano maoli o ke kumu o ka maʻi. Aia kekahi manaʻo he kumu genetic ka pathology, ke loaʻa i kahi kanaka kahi predisposition hoʻoilina i ka hoʻokumu ʻana o nā ʻiʻo fibrous i loko o ka māmā. Hana ʻia kēia ma ka pane ʻana i kekahi pōʻino i nā keʻena o ka ʻōnaehana hanu. Hoʻopaʻa nā kānaka ʻepekema i kēia kuhiakau me ka moʻolelo ʻohana, ke ʻike ʻia kēia maʻi i nā pili koko. ʻO ke kumu hoʻi o ke kumu genetic o ka maʻi ʻo ia ka mea i hōʻike pinepine ʻia ka fibrosis pulmonary iā ia iho i nā mea maʻi me nā pathologies hoʻoilina, no ka laʻana, me ka maʻi o Gaucher.
Hoʻololi i ke kino o ka māmā
ʻO nā hiʻohiʻona nui o ke kiʻi morphological o idiopathic fibrosing alveolitis:
ʻO ka loaʻa ʻana o ka fibrosis dense o ka parenchyma pulmonary.
Hoʻokaʻawale ʻia nā loli morphological e like me kahi ʻano heterogeneous patchy. ʻO ia ʻano kikoʻī ma muli o ke ʻano o nā wahi o nā ʻiʻo olakino a pōʻino i loko o ka māmā. Hiki i nā hoʻololi ke fibrous, cystic, a ma ke ʻano o ka mumū interstitial.
Hoʻokomo mua ʻia ka ʻaoʻao o luna o ka acinus i ke kaʻina inflammatory.
Ma keʻano laulā, ua like ka mōʻaukala o ka ʻiʻo māmā i ka idiopathic fibrosing alveolitis me ke kiʻi like me ka pneumonia interstitial.
Nā hōʻailona o ka idiopathic fibrosing alveolitis
ʻO ka pinepine, ʻike ʻia ka fibrosing idiopathic alveolitis i nā maʻi ma mua o 50 mau makahiki. ʻOi aku ka maʻi o nā kāne ma mua o nā wahine. ʻO ka lakio pili he 1,7:1.
Hōʻike nā maʻi i ka pōkole o ka hanu, e hoʻonui mau ana. ʻAʻole hiki i ka mea maʻi ke lawe i ka hanu hohonu (inspiratory dyspnea), ua hoʻopilikia ʻia ʻo ia e ka ʻū maloʻo ʻole me ka sputum. Loaʻa ka dyspnea i nā maʻi āpau me ka idiopathic fibrosing alveolitis.
ʻOi aku ka ikaika o ka hanu ʻana, ʻoi aku ka paʻakikī o ka maʻi. Ma hope o ka puka ʻana i hoʻokahi manawa, ʻaʻole ia e hala, akā e holomua wale ana. Eia kekahi, ʻaʻole pili kona hanana ʻana i ka manawa o ka lā, i ka mahana ambient a me nā mea ʻē aʻe. Hoʻopōkole ʻia nā manawa hoʻoulu i nā maʻi, a me nā manawa hoʻopau. No laila, wikiwiki ka hanu o ia mau maʻi. Loaʻa i kēlā me kēia o lākou ka hyperventilation syndrome.
Inā makemake ke kanaka e hanu hohonu, a laila e alakaʻi kēia i ka ʻu. Eia nō naʻe, ʻaʻole nā maʻi a pau e hoʻomohala i ka ʻāʻī, no laila ʻaʻole ia he hoihoi diagnostic. ʻOiai i loko o ka poʻe me ka maʻi pulmonary obstructive maʻi, i huikau pinepine ʻia me ELISA, e mau ana ka ʻū. Ke piʻi nei ka maʻi, ʻo ka pōkole o ka hanu e alakaʻi i ka ʻoiaʻiʻo e lilo ke kanaka i mea kīnā. Nalo ʻo ia i ka hiki ke haʻi i kahi huaʻōlelo lōʻihi, ʻaʻole hiki ke hele a mālama iā ia iho.
ʻAʻole ʻike ʻia ka manifesto o ka pathology. Hoʻomaopopo kekahi mau mea maʻi ua hoʻomaka ka fibrosing alveolitis i loko o lākou e like me ke ʻano o ka SARS. No laila, manaʻo kekahi poʻe ʻepekema he ʻano viral paha ka maʻi. Ma muli o ka ulu mālie ʻana o ka pathology, loaʻa i ke kanaka ka manawa e hoʻololi ai i kona pōkole hanu. Me ka ʻike ʻole iā lākou iho, hoʻemi ka poʻe i kā lākou hana a neʻe i kahi ola pasive.
ʻO kahi maʻi huahua, ʻo ia hoʻi, kahi maʻi i hui pū ʻia me ka hana sputum, e ulu aʻe ma mua o 20% o nā maʻi. Loaʻa paha i ka pus, ʻoi aku ka nui o nā poʻe maʻi i loaʻa i ka idiopathic fibrosing alveolitis koʻikoʻi. He mea weliweli kēia hōʻailona, ʻoiai e hōʻike ana i ka hoʻohui ʻana o kahi maʻi bacterial.
ʻO ka piʻi ʻana o ka mahana o ke kino a me ke ʻano o ke koko i loko o ka sputum ʻaʻole maʻamau no kēia maʻi. ʻOiai e hoʻolohe ana i ka māmā, hoʻolohe ke kauka i ka crepitus i ka hopena o ka hoʻoulu ʻana. Inā ʻike ʻia ke koko i loko o ka sputum, pono e hoʻouna ʻia ka mea maʻi no ka nānā ʻana no ka maʻi maʻi ʻaʻai. ʻO kēia maʻi i nā poʻe maʻi me ELISA ua ʻike ʻia he 4-12 mau manawa ma mua o nā kānaka olakino, ʻo ia hoʻi ka poʻe puhi.
ʻO nā hōʻailona'ē aʻe o ELISA e komo pū me:
Hoʻohui naʻau.
ʻEha ka ʻili.
Nā deformities o nā phalanges nail e hoʻomaka ana e like me nā pahu pahu. Loaʻa kēia hōʻailona ma 70% o nā maʻi.
E ʻoi aku ka ikaika o nā crepitations ma ka hope o ka inhalation, a i ka hoʻomaka ʻana e ʻoi aku ka mālie. Hoʻohālikelike ka poʻe akamai i ka crepitus hope i ka pahū ʻana o cellophane a i ʻole ke kani i hana ʻia ke wehe ʻia kahi zipper.
Inā i ka wā mua o ka hoʻomohala ʻana o ka maʻi, lohe ʻia ka crepitations ma nā ʻāpana basal hope, a laila i ka wā e holomua ana, e lohe ʻia nā creaks ma luna o ka ʻili āpau o ka māmā. ʻAʻole i ka hopena o ka hanu, akā i kona lōʻihi holoʻokoʻa. I ka hoʻomaka ʻana o ka ulu ʻana o ka maʻi, ʻaʻole paha ka crepitus i ka wā e hili ʻia ai ka ʻōpū i mua.
Lohe ʻia nā rale maloʻo ma mua o 10% o nā maʻi. ʻO ke kumu maʻamau ka bronchitis. ʻO ka ulu hou ʻana o ka maʻi e alakaʻi i nā hōʻailona o ka hāʻule ʻana o ka hanu, ka ulu ʻana o ka cor pulmonale. Loaʻa ka waihoʻoluʻu o ka ʻili i kahi waihoʻoluʻu lehu-cyanotic, ʻoi aku ka leo 2 ma luna o ke aʻa pulmonary, wikiwiki ka puʻuwai, pehu nā ʻaʻa cervical, pehu nā lālā. ʻO ka pae hope loa o ka maʻi e alakaʻi i kahi pohō kaumaha o ke kanaka, a hiki i ka ulu ʻana o ka cachexia.
ʻO ka hōʻailona o ka idiopathic fibrosing alveolitis
Ua hoʻoponopono hou ʻia nā ala no ka ʻike ʻana i ka idiopathic fibrosing alveolitis i kēia manawa. ʻOiai ʻo ia ʻano noiʻi noiʻi e like me ka open lung biopsy e hāʻawi i ka hopena hilinaʻi loa a manaʻo ʻia ʻo ia ka "standard gula" o nā diagnostics, ʻaʻole ia e hana mau.
ʻO kēia ma muli o nā pōʻino koʻikoʻi o kahi biopsy māmā ākea, ʻo ia hoʻi: he invasive ke kaʻina hana, he kumukūʻai, ma hope o kona hoʻokō ʻana, pono e hoʻopanee ʻia ka mālama ʻana a hiki i ka hoʻi ʻana o ka mea maʻi. Eia kekahi, ʻaʻole hiki ke hana i kahi biopsy i nā manawa he nui. ʻAʻole hiki loa i kekahi ʻāpana o nā mea maʻi ke hana, no ka mea ʻaʻole ʻae ke kūlana o ke olakino kanaka.
ʻO nā pae hoʻohālikelike kumu i kūkulu ʻia e ʻike i ka idiopathic fibrosing alveolitis:
Hoʻokaʻawale ʻia nā pathologies ʻē aʻe o ka interstitium o nā māmā. Pili kēia i nā maʻi i hiki ke hoʻoulu ʻia e ka lawe ʻana i nā lāʻau lapaʻau, ka hoʻohu ʻana i nā mea ʻino, ka pōʻino ʻōnaehana i ka ʻiʻo pili.
Hoʻemi ʻia ka hana o ka hanu waho, hoʻopilikia ʻia ka hoʻololi kinoea i loko o ka māmā.
I ka wā o ka CT scan, ʻike ʻia nā loli mesh bilateral i loko o ka māmā, ma kā lākou mau ʻāpana basal.
ʻAʻole hōʻoia ʻia nā maʻi ʻē aʻe ma hope o ka biopsy transbronchial a i ʻole bronchoalveolar lavage.
Loaʻa nā pae hoʻohālikelike hou aku:
ʻO ka mea maʻi ma mua o 50 mau makahiki.
Hiki ke pōkole o ka hanu me ka ʻike ʻole ʻia no ka mea maʻi, hoʻonui me ka hoʻoikaika kino.
He lōʻihi ko ka maʻi (mai 3 mahina a ʻoi aʻe paha).
Lohe ʻia ʻo Crepitus ma nā ʻāpana basal o ka māmā.
I mea e hiki ai i ke kauka ke hana i kahi hōʻailona, pono e ʻike i ka hōʻoia o 4 mau kumu nui a me 3 mau mea hou aʻe. ʻO ka loiloi o nā pae hoʻokolohua e hiki ai ke hoʻoholo i ka ELISA me kahi kiʻekiʻe o ka probability, a hiki i ka 97% (nā ʻikepili i hāʻawi ʻia e Raghu et al.), akā ʻo ka sensitivity o nā koina ponoʻī e like me 62%. No laila, ma kahi o ka hapakolu o nā maʻi e pono e hana i kahi biopsy māmā.
Hoʻomaikaʻi ka tomography computed kiʻekiʻe i ka maikaʻi o ka nānā ʻana i ka māmā a hoʻomaʻamaʻa i ka ʻike o ELISA, a me nā maʻi like ʻole. Ua like kona waiwai noiʻi me 90%. He nui nā poʻe akamai e koi nei e haʻalele loa i ka biopsy, inā ua hōʻike ka tomography kiʻekiʻe i nā loli ʻano o ka idiopathic alveolitis. I kēia hihia, ke kamaʻilio nei mākou e pili ana i kahi māmā "honeycomb" (inā he 25%) ka wahi i hoʻopilikia ʻia, a me ka hōʻoia ʻana o ka histological i ke alo o ka fibrosis.
ʻAʻohe koʻikoʻi honua o ka diagnostics Laboratory e pili ana i ka ʻike pathology.
ʻO nā hiʻohiʻona nui o nā loiloi i loaʻa:
Hoʻonui haʻahaʻa i ka ESR (ʻike ʻia ma 90% o nā maʻi). Inā piʻi nui ka ESR, a laila hōʻike paha kēia i kahi maʻi maʻi kanesa, a i ʻole kahi maʻi maʻi.
Hoʻonui i nā cryoglobulins a me nā immunoglobulins (ma 30-40% o nā maʻi).
ʻO ka hoʻonui ʻana i nā mea antinuclear a me nā rheumatoid, akā me ka ʻole o ka hōʻike ʻana i ka pathology systemic (ma 20-30% o nā mea maʻi).
ʻO ka piʻi ʻana o ka pae serum o ka lactate dehydrogenase a pau, ʻo ia ka mea ma muli o ka hoʻonui ʻana o ka hana o nā macrophages alveolar a me nā ʻano 2 alveocytes.
Hoʻonui ʻia ka hematocrit a me ke koko ʻulaʻula.
Hoʻonui i ka pae o nā leukocytes. He hōʻailona paha kēia hōʻailona o kahi maʻi, a he hōʻailona paha o ka lawe ʻana i nā glucocorticosteroids.
No ka mea e alakaʻi ana ka fibrosing alveolitis i nā haunaele i ka hana ʻana o ka māmā, he mea nui e loiloi i kā lākou leo, ʻo ia hoʻi, ko lākou mana koʻikoʻi, ka nui o ka nui, ka nui o ke koena a me ka hiki ke koena hana. I ka hana ʻana i ka hoʻāʻo, ʻo ka Coefficient Tiffno i loko o ka laulā maʻamau, a i ʻole e hoʻonui. E hōʻike ana ka ʻike ʻana o ka pihi kaomi-nui i kona neʻe ʻana i ka ʻākau a i lalo. Hōʻike kēia i ka emi ʻana o ka extensibility o nā māmā a me ka emi ʻana o ko lākou leo.
ʻO ka hoʻāʻo i wehewehe ʻia he koʻikoʻi koʻikoʻi, no laila hiki ke hoʻohana ʻia no ka ʻike mua ʻana o ka pathology, ke ʻike ʻole nā haʻawina ʻē aʻe i nā loli. No ka laʻana, ʻaʻole hōʻike ʻia kahi hoʻāʻo kinoea koko i ka wā hoʻomaha. ʻIke ʻia ka emi ʻana o ka haʻahaʻa o ka oxygen i ke koko arterial i ka wā o ka hoʻoikaika kino.
I ka wā e hiki mai ana, aia ka hypoxemia i ka wā hoʻomaha a hele pū me ka hypocapnia. Hoʻokumu wale ka Hypercapnia i ka pae hope o ka maʻi.
I ka hana ʻana i ka lekiō, ʻike pinepine ʻia nā loli o ke ʻano reticular a i ʻole reticulonodular. E loaʻa iā lākou ma nā māmā ʻelua, ma ko lākou ʻaoʻao haʻahaʻa.
ʻO ke kino reticular me ka fibrosing alveolitis e lilo i mea paʻakikī, ua hoʻokumu ʻia nā kaula i loko, nā kukui cystic me ke anawaena o 0,5-2 cm. Hoʻokumu lākou i ke kiʻi o kahi "honeycomb lung". Ke hiki ka maʻi i ka pae hope, hiki ke ʻike i ka ʻae ʻana o ka trachea i ka ʻākau a me ka tracheomegaly. I ka manawa like, pono e noʻonoʻo ka poʻe loea i ka 16% o nā maʻi, hiki ke noho ke kiʻi x-ray i loko o ka pae maʻamau.
Inā pili ka pleura i ke kaʻina hana pathological i ka mea maʻi, ulu ka intrathoracic adenopathy a ʻike ʻia ka mānoanoa parenchymal, a laila hōʻike paha kēia i ka hoʻopiʻi ʻana o ELISA e kahi maʻi maʻi maʻi, a i ʻole maʻi ʻē aʻe. Inā hoʻomohala ka mea maʻi i ka alveolitis a me ka emphysema i ka manawa like, a laila hiki ke noho ka nui o ka māmā i loko o ka pae maʻamau, a i ʻole e hoʻonui ʻia. ʻO kekahi hōʻailona diagnostic o ka hui ʻana o kēia mau maʻi ʻelua, ʻo ia ka nāwaliwali o ke ʻano vascular ma ka ʻaoʻao o luna o ka māmā.
I ka wā o ka tomography computed kiʻekiʻe, ʻike nā kauka i kēia mau hōʻailona:
Nā aka laina pololei ʻole.
Cystic lucidity.
ʻO ke kiko kiko o ka liʻiliʻi liʻiliʻi o nā māla māmā o ke ʻano "aniani hau". ʻO ka wahi o ka pōʻino i ka māmā he 30%, akā ʻaʻole hou.
ʻO ka mānoanoa o nā paia o ka bronchi a me kā lākou hana ʻole.
Disorganization o ka māmā parenchyma, traction bronchiectasis. ʻO nā ʻāpana basal a me nā ʻāpana subpleural o ka māmā.
Inā loiloi ʻia ka ʻikepili CT e kahi loea, a laila ʻo 90% pololei ka ʻike.
Hiki i kēia haʻawina ke hoʻokaʻawale i waena o ka idiopathic fibrosing alveolitis a me nā maʻi ʻē aʻe i loaʻa ke kiʻi like, me:
Pneumonitis hypersensitivity mau. Me kēia maʻi, ʻaʻole i loaʻa i ka mea maʻi nā hoʻololi "cellular" i loko o ka māmā, ʻike ʻia nā nodules centrilobular, a ʻo ka mumū ponoʻī i ka ʻaoʻao o luna a me waena o ka māmā.
Asbestosis. I kēia hihia, hoʻomohala ka mea maʻi i nā pleural plaques a me nā parenchymal bands o fibrosis.
Desquamative interstitial pneumonia. E hoʻolōʻihi ʻia nā ʻeleʻele o ke ʻano ʻano "frosted glass".
Wahi a ka computed tomography, hiki ke hana i ka prognosis no ka mea maʻi. ʻOi aku ka maikaʻi no nā poʻe maʻi me ka ground glass syndrome, a ʻoi aku ka maikaʻi no nā maʻi me nā loli reticular. Hōʻike ʻia kahi prognosis waena no nā maʻi me nā hōʻailona like ʻole.
ʻO kēia ma muli o ka pane maikaʻi ʻana o nā mea maʻi me ka ground glass syndrome i ka glucocorticosteroid therapy, i hōʻike ʻia e nā hōʻailona ʻano i ka wā HRCT. I kēia manawa, ʻoi aku ka alakaʻi ʻana o nā kauka e ka ʻikepili tomography computed i ka wā e hana ai i kahi prognosis ma mua o nā ʻano hana ʻē aʻe (bronchial a me alveolar lavage, nā hoʻāʻo ʻana i ka māmā, biopsy lung). Hoʻopili ʻia ka tomography e hiki ai ke loiloi i ke ʻano o ke komo ʻana o ka parenchyma māmā i ke kaʻina pathological. ʻOiai ʻo ka biopsy hiki ke nānā i kahi ʻāpana o ke kino.
ʻAʻole pono e haʻalele ʻia ka bronchoalveolar lavage mai ka hana diagnostic, no ka mea e hiki ai ke hoʻoholo i ka prognosis o ka pathology, kona ala a me ke alo o ka mumū. I ka holoi ʻana me ELISA, ʻike ʻia ka nui o nā eosinophils a me nā neutrophils. I ka manawa like, ʻo kēia hōʻailona ke ʻano o nā maʻi ʻē aʻe o ka ʻiʻo māmā, no laila ʻaʻole pono e hoʻonui ʻia kona koʻikoʻi.
ʻO ke kiʻekiʻe o nā eosinophils i loko o ka lavage e hōʻeha i ka wānana o ka idiopathic fibrosing alveolitis. ʻO ka ʻoiaʻiʻo ʻo ia mau mea maʻi e pane pinepine ʻole i ka mālama ʻana me nā lāʻau corticosteroid. Hiki i kā lākou hoʻohana ke hoʻemi i ke kiʻekiʻe o nā neutrophils, akā mau ka helu o nā eosinophils.
Inā loaʻa nā kiʻekiʻe o nā lymphocytes i loko o ka wai holoi, hōʻike paha kēia i kahi prognosis maikaʻi. Ma muli o ko lākou hoʻonui pinepine ʻana me ka pane kūpono o ke kino i ka mālama ʻana me nā corticosteroids.
ʻO ka biopsy Transbronchial hiki iā ʻoe ke loaʻa i kahi wahi liʻiliʻi o ka ʻili (ʻaʻole ʻoi aku ma mua o 5 mm). No laila, hoʻemi ʻia ka waiwai ʻike o ka haʻawina. Ma muli o ka palekana o kēia ʻano no ka mea maʻi, hoʻomaʻamaʻa ʻia i ka wā mua o ka maʻi. Hiki i kahi biopsy ke hoʻokaʻawale i nā pathologies e like me sarcoidosis, hypersensitivity pneumonitis, cancerous tumors, maʻi maʻi, eosinophilic pneumonia, histocytosis, a me alveolar proteinosis.
E like me ka mea i ʻōlelo ʻia, ua manaʻo ʻia kahi biopsy open-type he ʻano maʻamau no ka hoʻomaʻamaʻa ʻana iā ELISA, hiki iā ʻoe ke ʻike pololei, akā hiki ʻole ke wānana i ka ulu ʻana o ka pathology a me kāna pane ʻana i ka mālama ʻana i ka wā e hiki mai ana me kēia ʻano. Hiki ke pani ʻia kahi biopsy hāmama e kahi biopsy thoracoscopic.
ʻO kēia haʻawina e pili ana i ka lawe ʻana i ka nui o ka ʻiʻo, akā ʻaʻole lōʻihi ka lōʻihi o ka hoʻokahe ʻana o ka lua pleural. Hoʻemi kēia i ka manawa o ka mea maʻi i ka haukapila. ʻAʻole maʻamau nā pilikia mai kahi kaʻina thoracoscopic. E like me ka hōʻike ʻana o nā haʻawina, ʻaʻole ʻōlelo ʻia kahi biopsy hāmama e kuhikuhi i nā maʻi āpau me ka ʻole. Pono maoli ia e 11-12% o nā maʻi, akā ʻaʻole hou.
I ka hoʻohālikelike honua o nā maʻi o ka 10th revision, ua wehewehe ʻia ʻo ELISA ʻo "J 84.9 - Interstitial pulmonary disease, unspecified."
Hiki ke hoʻonohonoho ʻia ka diagnostic e like me kēia:
ʻO ELISA, ka wā mua, ka hanu ʻana o ka degere 1.
ʻO ELISA ma ke kahua o ka "cellular lung", ka hanu ʻana o ka degere 3, ka maʻi cor pulmonale.
Ka mālama ʻana i ka idiopathic fibrosing alveolitis
ʻAʻole i kūkulu ʻia nā ala kūpono no ka mālama ʻana iā ELISA. Eia kekahi, paʻakikī ke hāʻawi i ka hopena e pili ana i ka hopena o nā hopena o ka lāʻau lapaʻau, no ka mea he liʻiliʻi ka ʻikepili i ke ʻano kūlohelohe o ka maʻi.
Hoʻokumu ʻia ka lāʻau lapaʻau ma ka hoʻohana ʻana i nā lāʻau lapaʻau e hōʻemi i ka pane ʻana o ka inflammatory. Hoʻohana ʻia nā corticosteroids a me nā cytostatics, e hoʻopilikia i ka ʻōnaehana pale kanaka a kōkua i ka hōʻemi ʻana i ka mumū. Ua wehewehe ʻia kēlā ʻano lapaʻau e ka manaʻo e ulu ana ka idiopathic fibrosing alveolitis e kūʻē i ke ʻano o ka mumū mau loa, kahi e komo ai i ka fibrosis. Inā kāohi ʻia kēia hopena, a laila hiki ke pale ʻia ke kūkulu ʻana i nā loli fibrotic.
ʻEkolu mau ala o ka therapy:
ʻO ka mālama ʻana me nā glucocorticosteroids wale nō.
ʻO ka mālama ʻana me nā glucocorticosteroids me ka azathioprine.
ʻO ka mālama ʻana me nā glucocorticosteroids me ka cyclophosphamide.
ʻO ka ʻae ʻana o ka honua, i mālama ʻia i ka makahiki 2000, e aʻo i ka hoʻohana ʻana i nā regimens 2 hope loa i ka mālama ʻana, ʻoiai ʻaʻohe manaʻo hoʻopaʻapaʻa e pili ana i kā lākou hopena i hoʻohālikelike ʻia me ka monotherapy glucocorticosteroid.
Nui nā kauka i kēia lā e kuhikuhi i nā glucocorticosteroids no ka hoʻokele waha. ʻOiai hiki ke hoʻokō i nā hopena maikaʻi wale nō ma 15-20% o nā maʻi. ʻO ka poʻe ʻōpio ma mua o 50 mau makahiki, ʻo ka hapa nui o nā wahine, ʻoi aku ka maikaʻi o ka pane ʻana i kēlā ʻano lapaʻau inā ua hoʻonui lākou i nā waiwai o nā lymphocytes i ka holoi ʻana mai ka bronchi a me ka alveoli, a ʻike pū ʻia nā loli aniani honua.
Pono e hoʻomau ka mālama ʻana i ʻeono mahina. No ka loiloi i kona pono, e hoʻolohe i nā hōʻailona o ka maʻi, nā hopena o nā x-ray a me nā ʻano hana ʻē aʻe. I ka wā o ka mālama ʻana, pono ia e nānā i ka maikaʻi o ka mea maʻi, no ka mea, pili ka lāʻau lapaʻau me ka nui o nā pilikia.
Aia kekahi mau loea e kū'ē i ka hoʻohana ʻana i nā cytostatics i ka mālama ʻana iā ELISA. Hōʻoiaʻiʻo lākou i kēia ma ka ʻōlelo ʻana he kiʻekiʻe loa ka nui o nā hoʻopiʻi me ia ʻano lapaʻau. Heʻoiaʻiʻo loa kēia i ka hihia o ka hoʻohana ʻana o Cyclophosphamide. ʻO ka hopena maʻamau ka pancytopenia. Inā hāʻule nā platelets ma lalo o 100/ml, a i ʻole ka pae o nā lymphocytes ma lalo o 000/ml, a laila e hoʻemi ʻia ke ʻano o nā lāʻau.
Ma kahi o ka leukopenia, pili ka mālama ʻana me ka cyclophosphamide me ka hoʻomohala ʻana i nā hopena ʻaoʻao e like me:
Ka maʻi maʻi ʻaʻai.
Hemorrhagic cystitis.
Stomatitis.
ʻAha noho.
Kiʻekiʻe maʻalahi o ke kino i nā maʻi lele.
Inā ua kuhikuhi ʻia ka mea maʻi i nā cytostatics, a laila i kēlā me kēia pule e hāʻawi ʻo ia i ke koko no ka loiloi maʻamau (i nā lā 30 mua mai ka hoʻomaka ʻana o ka mālama ʻana). A laila hāʻawi ʻia ke koko 1-2 mau manawa i 14-28 mau lā. Inā mālama ʻia ka lāʻau me ka Cyclophosphamide, a laila i kēlā me kēia pule e lawe mai ka mea maʻi i ka mimi no ka nānā ʻana. He mea nui ka nānā ʻana i kona kūlana a mālama i ke ʻano o ke koko i loko o ka mimi. Hiki ke paʻakikī ke hoʻokō ʻana i kēlā mana i ka mālama home, no laila, ʻaʻole i hoʻohana mau ʻia kēlā ʻano hoʻoponopono.
Manaʻo nā kānaka ʻepekema ʻo ka hoʻohana ʻana i nā interferon e kōkua i ka hoʻokō ʻana i ka idiopathic fibrosing alveolitis. Kāohi lākou i ka ulu ʻana o nā fibroblasts a me ka protein matrix i loko o nā keʻena o ka ʻiʻo māmā.
ʻO kahi ala radical e mālama ai i ka pathology ʻo ka hoʻololi ʻana i ka māmā. ʻO ke ola o nā maʻi i loko o 3 mau makahiki ma hope o ke kaʻina hana he 60%. Eia naʻe, nui nā poʻe maʻi me ELISA i ʻelemakule, no laila ʻaʻole hiki iā lākou ke ʻae i kēlā ʻano hana.
Ka mālama ʻana i nā pilikia
Inā hoʻomohala ka mea maʻi i kahi maʻi hanu, a laila ua kuhikuhi ʻia ʻo ia i nā antibiotics a me nā antimycotics. Ke koi nei nā kauka e hoʻopaʻa ʻia kēlā mau maʻi i ka maʻi influenza a me ka maʻi pneumococcal. Hoʻokō ʻia ka lāʻau lapaʻau o ka pulmonary hypertension a me ka decompensated chronic cor pulmonale e like me nā protocol pili.
Inā hōʻike ka mea maʻi i ka hypoxemia, a laila hōʻike ʻia ʻo ia i ka oxygen therapy. ʻO kēia ka mea hiki ke hōʻemi i ka pōkole o ka hanu a hoʻonui i ka hoʻomanawanui hoʻoikaika kino o ka mea maʻi.
Forecast
ʻAʻole maikaʻi ka prognosis i nā maʻi me ka idiopathic fibrosing alveolitis. ʻAʻole ʻoi aku ka nui o ke ola o kēlā mau maʻi ma mua o 2,9 mau makahiki.
ʻOi aku ka maikaʻi o ka prognosis i nā wahine maʻi, i nā maʻi ʻōpio, akā aia wale nō ma ke ʻano o ka maʻi ʻaʻole i ʻoi aku ma mua o hoʻokahi makahiki. Hoʻonui ia i ka wānana o ka pane maikaʻi o ke kino i ka mālama ʻana me nā glucocorticosteroids.
ʻO ka pinepine, make nā maʻi mai ka hanu a me ka puʻuwai puʻuwai. Ke ulu nei kēia mau pilikia ma muli o ka holomua o ELISA. Hiki ke make ma muli o ka maʻi maʻi maʻi.