Syndrome Hammam-Rich: whakaahuatanga

syndrome Hamman Rich - he mate āhua onge (pā, ko te tekau anake wā ia iwi rau mano), e faaite ake i roto i te puka o te sclerosis o nga pūkahukahu me te hanganga o te kore romahā. Tohunga huanga tona takanga ki tukanga autoimmune.

whakamāramatanga

syndrome Hamman Rich - he mate e pā ana ki te tukanga pathological i roto i te kiko pūkahukahu, e arata'i ki tona sclerosis me te whanaketanga o te kore romahā. Hei ako anga reira anake i roto i te waenganui o te rau tau whakamutunga, ka mau papai o nga hautaka pūtaiao auau ki ngā whakaahuatanga o ngā tohu rite.

Ko atu noa tenei māuiuitanga i roto i nga tangata waenganui-koroheke, me te kaumātua. Kei te whakapono te reira e kia tika ki peu kino, he roa roa o te mahi, me nga āhuatanga o ngaio tikanga mahi kāore (welders, kaikeri, kaihanga, me te pera i runga i. D.) tenei.

taurite

He rata nei ko te tuatahi ki te ako i te māuiuitanga ko Scadding. fokotu'u ia e te wā pērā i "fibrosing alveolitis." Ko te tino hohoro timata reira ki te whakamahi i roto i te tikanga pai te whanui, e hūanga he rōpū o mate e whai rite tohu. Na reira, ka mau te reira, nui atu i te ingoa motuhake.

whakaingoatia māuiuitanga i mate Scadding, a ka ki te syndrome Hamman Rich, rite tahuri reira i roto i taua i whakaahuatia enei tākuta e rua ōrite māuiuitanga i roto i 1935. He atu taurite o tenei kōwae nosological, hei tauira, ahu whakamua kakakīrehe pūkahukahu interstitial, dysplasia kaiota, cirrhosis, pūkahukahu purulent kakā interstitial me ētahi atu. I roto i te rongoā hou, kua mana ki te whakamahi i ratou katoa.

etiology

tonu tonu Hamman Rich syndrome tetahi o nga mate o te etiology unknown, mai te āhuatanga o te kakakīrehe tupu i roto i tenei mate ko te pūkahukahu kiko tauhohenga nonspecific ki te tukanga pathological. Ko te tino rongonui i roto i te hapori pūtaiao i te ariā e meinga te mate e te tukanga autoimmune i runga i tauhohe mate pāwera o te toru o nga momo (immunocomplex).

He kaiwhakahau me polyetiology ariā. tautohe ratou e whai wāhi te kiko pūkahukahu te ki te maha māngai o kitakita, fungal āhua viral ranei, e tika ana ki te tauhohenga hyperergic o te pūnaha ārai mate.

Hoki te maha o pūtaiao whakaaro i mua-Rich syndrome Hamm rite tetahi ahua o collagenosis, ai he iti ki te marama anake te tukanga pathologic.

pathogenesis

Whakapeka pneumonitis interstitial (Hamman Rich syndrome) puta tika ki te hanganga o te wae kauae-capillary pera-ka karanga. Mai i pehea whakahuatia huringa pathological tei runga i te auau tūringiringi o ngā haurehu i te toto, me te, ka rite ki te putanga o te hypoxia. e kore te mea ahurei ki tenei mate ngā tenei tukanga, me te whakautu angamaheni whekau romahā ki whara.

Whakaiti i te tūringiringi o ngā haurehu i roto i nga alveoli puta mo te maha ngā take. Tuatahi, ko te huringa o te matotoru membrane me pātaki rite pūtaitoto i te wē, tomo ēnei wāhanga, hanga ratou mātotoru ake me mārō. Tuarua, ko te epithelium ngohi te nga sacs airway (alveoli) roto riro pūtoru me mona ona āhuatanga. A toru, i whakaohooho reira perfusion tukanga haurehu, arā, te rangi e tomo te pūkahukahu, e kore i roto i te whakapā atu ki te pūnaha circulatory o tetahi hohoro rawa tenei tukanga puta. I tua atu, i reira he he spasm pūpū o oko o te pūkahukahu tika ki te hua i roto i te pēhanga i roto i te tohanga pūkahukahu, e atu kē atu te tikanga pathophysiological.

tohutanga

He onge Hamman Rich syndrome i roto i ngā tamariki. Tenei mate te nuinga puta i roto i te waenganui-koroheke, me te toulekeleka, engari kia waiho i roto i nga kohungahunga taimi halá, ki heke kaha e vai.

Ko te tīmatanga puhoi o te mate. I roto i te tangata, i reira he wāhanga o te mare maroke, me te ite raru i roto i te pouaka. Na, kua piri te reira i te hāhā o te manawa, me te exhale he ara paku i roto i te pāmahana, ngoikore, te rohirohi. I te whakamātautau tuatahi mārama kitea matimati kikorangi, me te tapatoru nasolabial. Pea te huringa o phalanges kāpeka me titi āhua.

He whakatere mokowhiti, rales koropupū iti i roto i te pūkahukahu. Ki te mate haereraa wāhanga auau o te maremare, puta sputum, a i roto i wā onge me hemoptysis (ka āputa capillary wātea).

tātaringa

E ruia ana te kakakīrehe pūkahukahu interstitial taea (Hamman Rich syndrome) kia kitea e te tikanga o whakamātautau taiwhanga anake i roto i te wāhanga kāpeka, pera i roto i te whakamātautau toto ka hei huringa kore-motuhake:

• te whakanui ake pūmua;
• whakaterenga SSCHE (ngeniwhero auau parataiao);
• huringa i roto i te ōwehenga o hautau pūmua.

Enei whika tohu te aroaro o te mumura, engari kihai i pūrongo tetahi o ona natura, e kore i runga i te wāhi. Ka taea e te iti bit te āwhina mahere Radiography o te pūkahukahu. I roto i te wāhanga tuatahi i reira ko te tauira tipu i roto i te pukapuka pakiaka, ka he graininess Lung kiko kupenga. Neke atu i te wa, puta nga whakaahua rohe o nui haere te māramatanga, e tango te ahua o honeycombs. Ka hoki kia puta ke Heart tika ki te hua o te ngakau tika.

tātaritanga pārōnaki

mate Hammam-Rich - he mate e he uaua ki te tātari mea ïa no te mea o huringa nonspecific i roto i te pūkahukahu. I roto i te tikanga ki te tika kia tino o te tātaritanga, ki tutakina katoa atu.

Me te tākuta ki te hanga faaauraa:

• paitini, me te pāwera alveolitis fibroziruschim;
• kakā më;
• sarcoidosis;
• tohatohaina kohi pūkahukahu;
• nahanaha mate kiko tūhonohono (lupus, kaikōiwi, Scleroderma, me ētahi atu);
• pūkahukahu mate pukupuku;
• hemosiderosis pūkahukahu;
• rōpū o te pneumoconiosis.

Anake i roto i te hui e katoa o te kōwhiringa i runga ake mahue pāngia syndrome Hammer-Rich. e tika ana ki te kawe i roto i whakamātautau tirotiro tūpāpaku maha. Ki te mahi i tenei, whakatakoto te tākuta tuwhera kati ranei he unuhanga ngira, i runga i nga wāhanga tikanga o te mate rānei.

anatomy mehameha

Tohumate me tātaritanga Hammam-Rich syndrome whakawhirinaki i runga i te āhua o te huringa ao i roto i te kiko tana pukapuka. I roto i te tikanga ki te arotake i a ratou, te tango i tetahi wahi o te pūkahukahu, me te titiro i te reira i raro i te karu. Ko nga hua o tenei ako e kore e hāngai ki te mau faaiteraa haumanu, engari he ratou tika atu no te whakaata tika ratou i te tinana kāwanatanga.

Ki te he i raro i fibrosing alveolitis te mate, kite te kairangahau mate kikokiko huamo wai ki te ihirangi tiketike o pūtōtepe i roto i nga lumen kauae, pātaki impregnation pūtau whakautu inflammatory. Ki te kauneke te tukanga pathological, he reira huringa histologically kitea i roto i te hanganga pūtau me te endothelial kauae capillary whawhe membrane thickening. Ano taea whakangaromanga wāhanga o te alveoli i nga wahi o te whakapā atu ki te oko.

E rima nga nekehanga o huringa i roto i te pūkahukahu:

1. Edema me te kookuhutanga pūkoro o taiepa kauae.
2. Wai whakakī i te alveoli me ngā pūtau desquamated absorption o pātaki exudate me hiri ranei.
3. Ko te whakangaromanga o nga alveoli, te hanganga o cysts i roto i te bronchi iti.
4. reo maha me cysts iti.
5. "Pūkoro" te marama e tika ana ki cysts nui.

Akoranga me waitohunga

Kelloidoz (Hamman Rich syndrome) he waitohunga tika rawakore, mai te mea e taea ki te mutu i te ahunga whakamua o te mate. Ahakoa ki te tukanga pathological rongoā e taea e puhoi, engari maoro, e riro tonu te mate. pea te manawanui i te marama torutoru ki te tau torutoru o te ora. Ka rite ki te hinaaro exacerbation me wā murunga iu i roto i tenei wa.

Neke atu i te wa, te ahu whakamua iti hoki manawa arata'i iwi ki hauātanga, a ka ki te mate i te asphyxia. I roto i te take pai, ka waiho i te manawanui tetahi atu e rima ki te whitu tau i te wa o te tātaritanga. Engari e ka ū ai ki ngā taunakitanga katoa o te rata. I roto i te wā onge, mai te mutunga i muri i o nga marama i te rua,, ahakoa o te therapy. Ki te whakaritea te maimoatanga e mo te tīmatanga o te mate, i roto i wā onge e taea ki te "tiaki" i te reira i tenei wā, me te whakaora i te ora o te tangata, me te hauora whanaunga.

maimoatanga

Aha ki te mahi kino, i muri i pāngia ia ki te syndrome Hammam-Rich? tīmata tonu Treatment. Ehara i te mea e tika ana ki te whakaroa i te tīmatanga o te maimoatanga, rite te mate kauneke i runga i nga tau atu me te ake. e kore te ano whakawhanakehia maimoatanga motuhake, kia ngana kaiwhakaako ki te aukati i nga tohu, me te pōturi iho te tere o te kiko kakakīrehe.

I roto i te wāhanga tuatahi o te mate nga steroids tamatahia whai hua te nuinga. whakaiti ratou nga faaiteraa inflammatory: exudation o inu, kookuhutanga pūtau, pōturi iho te hanganga o te kiko tūhonohono, me te hanga o paturopi. Bolus therapy whakamana, i.e. timata ki pota tiketike (ki runga, ki te 60-80 mg ia ra), me te āta whakaiti i te nui o tarukino ki runga ki te 20 mg. He mea nui ki te aroturuki i te mahi o te uho adrenal, kia rite kore ki te meinga Kushingoid syndrome.

I roto i te wāhanga i muri mai, ka makona korerotia kakakīrehe, kua hoatu hiahia ki taero e inhibits te pūnaha ārai mate. riro tohatoha Special te therapy huinga o steroids me taero cytotoxic. Ko reira nui atu whai hua atu i te whakamahi o enei taero fakafo'ituitui.

whakaritea te therapy rite symptomatic decongestants, inhalation o te hāora i raro i te pēhanga, te huaora B, me te paturopi rite tika whakamahia (ka āpiti i te mate tuarua), glycosides cardiac me Diuretics (ki te hua i roto i te kore te ngakau).