Hammain-Rich Syndrome: Tsanangudzo

Hammain-Rich syndrome chirwere chisingawanzovi (chiitiko chematambudziko gumi chete nemazana evanhu), inoratidzirwa nenzira ye lung lung sclerosis uye kuumbwa kwekufema kusakundikana. Nyanzvi dzinobatanidza chitarisiko chake nemagadzirirwo emagetsi.

Tsanangudzo

Hammain-Rich syndrome chirwere chinokonzerwa nehutano hwemapfupa mumapfupa emapapu anotungamirira ku-sclerosis uye kukurudzirwa kwekufema kusakundikana. Chidzidzo chacho chakatanga pakati pezana remakore rapfuura, apo mumagazini ewesayenzi, mabhuku ane tsanangudzo yezviratidzo zvakafanana akawedzera.

Izvi zvirwere zvinowanzozivikanwa pakati pevarume vane makore mapakati uye vakwegura. Zvinofungidzirwa kuti izvi zvingave zvichibva pamitemo yakaipa, ruzivo rwakareba rwebasa uye ruzivo rwemabasa ane zvisina kukodzera mashizha ekushanda (welders, miners, builders, etc.).

Synonyms

Chiremba uyo akatanga kutanga kudzidza chirwere ichi chakanga chiri Scudding. Akaronga izwi rokuti "fibrosing alveolitis". Asi munguva pfupi yakatanga kushandiswa nenzira yakazara, achitaura nezveboka rezvirwere zvine zviratidzo zvakafanana. Nokudaro, zita rinonyanya kudikanwa raidiwa.

Chirwere chacho chakanzizve chirwere cheScudding, uye zvakare kupinda muHammain-Rich syndrome, sezvo zvakaitika kuti vanachiremba vaviri vakarondedzera chirwere chakafanana zvakare muna 1935. Kune mamwe mafananisiro aya eology, pamwe chete, kubudirira kwepistitial lung fibrosis, fibrotic dysplasia, cirrhosis yemapapu, kwete- pulmonary interstitial pneumonia nevamwe. Mukurapa kwemazuva ano, zviri pamutemo kuti uzvishandise zvose.

Etiology

Hammain-Rich syndrome ichiri chimwe chezvirwere zvine etiology isingazikamwi, sezvo chiitiko che fibrosis chiri kuitika muchirwere ichi ndechekusaziva kwemafupa emapapu kumararamiro epfungwa. Kunyanya kuzivikanwa munharaunda yezvesayenzi yaiva nheyo yokuti chirwere ichi chinokonzerwa nehutachiona hwemotokari zvichienderana nehutano hwehutano hwechipiri chetatu (immunocomplex).

Iko kune vatsigiri veshoko re polyethiologic. Vanopikisa kuti mapapu emapapu anowanikwa kune dzimwe nhengo dzakasiyana-siyana dzebhakitiriya, fungal kana utachiona hunhu, izvo zvinotungamirira kumagadzirirwo emagadzirirwo ezvirwere zvemuviri.

Masayendisiti akawanda akambofunga nezvehutano hweHammen-Rich seimwe yemhando dzakasiyana dze collagenosis, umo chirwere chechirwere chinokwana chete kumapapu.

Pathogenesis

Idiopathic fibrosing alveolitis (Hammain-Rich syndrome) inooneka nemhaka yekuumbwa kweiyo inonzi "alveolar-capillary block". Muyero wekuparadzirwa kwegasi muropa uye, semugumisiro, hypoxia inofanirwa nezvakataurwa sei kuti pathological changes ndeye. Izvi hazvirevi kune chirwere ichi, asi chimiro chaicho chemhepo yekufema inoparadza.

Kuderedza kuparadzirwa kwemagasi kuburikidza nealveoli kunoitika nekuda kwezvikonzero zvakawanda. Chokutanga, uku ndiko kuchinja kwehupamhi hwemakumbo uye zvikamu, sezvo fibrinogen inowanikwa mu plasma inogadzirisa nzvimbo idzi, zvichiita kuti dzive dzakaoma uye dzakasimba. Chechipiri, epithelium iri kuvhara mabhesi (alveoli) kubva mukati inova cubic uye inorasikirwa nayo. Chechitatu, nzira yekuperesa magasi yakavhuniwa, kureva, mhepo inopinda mumapapu, haisviki nehutachiona, kana izvi zvinotanga kuitwa nokukurumidza. Mukuwedzera, nekuda kwekuwedzera kukamanikidzwa mumutsara muduku weropa, kuputika kwemapapu emapapu kunoonekwa, izvo zvinowedzera kuwedzera nzira dzepathophysiological.

Symptomatics

Iyo syndrome yeHammam-Rich muvana haina kuwanikwa. Chirwere ichi chinonyanya kuitika pakati pevanhu vane makore nemakore, asi zvinogonawo kuva mumucheche vacheche vane mikana yekudzikinura yakaderedzwa.

Kutanga kwechirwere chacho kunonoka. Munhu wacho ane zvikamu zvekukanganiswa zvakaoma uye kunzwa kwekudzidzira mubhokisi. Izvi zvinoteverwa nekupfupika kwemhepo pane kuputika uye kuwedzera zvishoma kwekushisa, kusimba, kuneta kunonoka. Pamusoro pekuongororwa kwepakutanga, zvigunwe zvebhuruu uye katatu nasolabial zviri pachena. Panogona kuve nekushandura mu-terminal phalanges yeminwe uye chimiro chezvipikiri.

Iko kunomhanya kunomhanya, zvinyorwa zviduku mumapapu. Nekuwedzera kwechirwere ichi, zvikamu zvekukandira zvinowedzera, zvinowanikwa, uye munguva dzisingashamisi hemoptysis (kana pane capillary rupture).

Diagnostics

Kuparara kwepstitial lung fibrosis (Hammain-Rich syndrome) kunogona kuonekwa kuburikidza neongororo yeongorori chete mumagariro ekupedzisira, sezvo kuchinja kwekuongorora ropa kunenge kusina chairo:

• Kuwedzera kwemapuroteni;
• Kukurumidza kweSSE (sedimentation rate ye erythrocytes);
• Shanduko muhuwandu hweprotein fractions.

Izvi zviratidzo zvinoratidza kuvapo kwekuvhiringidza kwekuita, asi hapana chinotaurwa pamusoro pechiratidzo kana nzvimbo. Chinobatsiro chiduku chinogona kuonekwa kubva kumafirimu emapapu. Pazvikamu zvekutanga, kune kuwedzera kwemuenzaniso pamidzi yemapapu, ipapo ganda remapapu remapapu rinooneka. Nokufamba kwenguva, zvifananidzo zvinowanikwa munzvimbo dzekuwedzera pachena, izvo zvinotora mawero emasero. Mwoyo unogonawo kuchinjwa nekuwedzera kugovana kwakarurama.

Kusiyanisa kutsvaga

Chirwere cheHammer-Rich chirwere chinonetsa kuti chiongororwe nekuda kwekuchinja kusina kuitika kunowanikwa mumapapu. Kuti uratidze zvakajeka nezvekuongororwa, unofanirwa kuisa kunze kwezvose.

Chiremba anoda kuenzanisa:

• Ne allergic uye toxic fibrosing alveolitis;
• Kubata pineumonia;
• Sarcoidosis;
• Kuparadzirwa kwepammonary tuberculosis;
• Zvirwere zvinokonzera zvirwere (lupus, rheumatoid arthritis, scleroderma, nezvimwe);
• Kanza yemaperembudzi;
• Hemosiderosis yemapapu;
• Boka repneumoconiosis.

Chero kana zvisarudzo zvose zviri pamusoro apa zvichitorerwa, Hammer-Rich syndrome inowanikwa. Kazhinji zvakakosha kuita zvinyorwa zvepathoanatomical. Nokuda kweizvi, chiremba anotsanangura kuzarura kana kuvharwa kushandiswa kwechipiri, zvichienderana nechinangwa chechirwere chacho.

Pathomorphology

Zviratidzo uye kuongororwa kweHinnson-Rich syndrome zvinotarisa kuti kuchinja kwenyika kwakaitika sei mumapapu emapapu. Kuti uzviongorore, bvisa chikamu chemapapu uye uchitarise pasi pekroscope. Migumisiro yenyaya ino inogona kunge isingaenzani nemakiriniki ezviratidzo, asi yakarurama kwazvo, nokuti inoratidza zvakananga mamiriro echirongwa.

Kana chirwere ichi chiri muchitanda che fibrosing alveolitis, chiremba anoona hutu hwekudzivirira, ruzivo rwakasvibiswa nehuwandu hwe fibrin mu lumen ye alveoli, kuiswa kwe septa nemasero ezvokuvhiringidza. Kana chirwere ichi chichifambira mberi, saka chinoratidzwa nekushandura mumagadzirirwo emagetsi e-endothelium e-alveoli uye capillaries, kuwedzerwa kwemuviri wepasi. Uyewo zvinogona kuitika kuparadzwa kwemafuroti munzvimbo dzakasangana nemidziyo.

Kune zvidimbu zvishanu zvekuchinja mumapapu:

1. Edema uye masero ekupinda mumasvingo e-alveoli.
2. Kuzadza alveoli nemasero emvura uye exfoliated kana kushandiswa kwekutsvaira ne septa uye compaction yavo.
3. Kuparadzwa kwe-alveoli, kuumbwa kwe-cysts muduku duku rwebronchi.
4. Multiple medium and small cysts.
5. "Cellularity" yemapapu nemhaka yemashizha makuru.

Current and forecast

Kelloidosis (Hammain-Rich Syndrome) ine chirevo chakanyanya kufadza, nokuti hazvibviri kumisa kufambira mberi kwechirwere chacho. Kunyangwe nemishonga, chirwere chetachiona chinogona kuderedzwa, asi nokukurumidza kana kuti gare gare chirwere chicharamba chichikunda. Murwere angave achibva kumwedzi yakawanda kusvika kumakore akawanda ehupenyu. Munguva ino pachava nekuwedzera uye nguva dzekuregererwa kusingaperi.

Nokufamba kwenguva, kufambira mberi kwema cardiopulmonary kusakwana kunotungamirira vanhu kuremara, uye kurufu kubva kuAphyphyxiation. Pakupedzisira, murwere achava nemamwe makore mashanu kusvika kune manomwe kubvira panguva yekuongorora. Zvichienderana nekuti achabvumirana nemazano ose echiremba anoenda. Muzviitiko zvisingawanzoitiki, kuguma kunouya mumwedzi mishomanana, pasinei nemishonga. Kana kurapwa kunorayirirwa mukutanga kwechirwere, saka mune zvisingashamisi zviitiko zvinokwanisika "kuzvichengetedza" panguva iyi uye kuchengetedza hupenyu hwomunhu uye hutano hwakanaka.

Kurapa

Chii chinofanirwa nemurwere mushure mekunge aongororwa ane Hammain-Rich syndrome? Kurapa kunotanga pakarepo. Iwe haufaniri kunonoka nekutanga kwerapi, sezvo chirwere chinopfuurira nezera rinowedzera. Kurapa kunonyanya kusati kwave kwakagadzirwa, saka vanachiremba vanoedza kumira zviratidzo uye kunonoka kuderedza chiyero che fibrosis chemuviri.

Pazvikamu zvekutanga zvechirwere, mishonga ye-steroid yainyanya kushanda. Vanoderedza kuratidzira kuratidza: kubuda kwemvura, kushandiswa kwemasero, kunonoka kwekugadzirwa kwehutano hunoshandiswa uye kugadzirwa kwezvirwere. Therapy inoshandiswa bolus, kureva kuti, kutanga nemitambo yakakura (kusvika pa 60-80 mg pazuva) uye zvishoma nezvishoma kuderedza kuwanda kwemishonga kusvika 20 mg. Zvakakosha panguva imwe chete kutarisa basa reAdrenal cortex kuitira kuti isakonzera Cushing's syndrome.

Mune zvikamu zvekupedzisira, apo fibrosis inokwana zvakatumidzwa, zvinoshandiswa zvinopiwa kune zvinodhaka zvinoderedza hutachiona. Kusanganiswa kweairwere kubva kune steroid uye cytostatics yakanyanya kufanirwa. Yakaratidza kuva yakakosha kupfuura kushandisa mishonga iyi zvakasiyana.

Sechirwere chechirwere chechirwere, maitiro ekuderedza, oxygen inhalations pasi pekumanikidzwa, B mavitamini anorayirwa, maantibiotics (kana chirwere chechipiri chinowanikwa), mwoyo glycosides uye diuretics (pamwe nekuwedzera kwekushaya mwoyo) zvinoshandiswa sezvinodiwa.