Hammam-Rich syndrome: Inkcazo

Hamman Rich syndrome - isifo inqabile kakhulu (izehlo na iimeko ezilishumi kuphela abantu ikhulu lamawaka nganye), esizibonakalisa ngokohlobo sclerosis emiphungeni kwaye ukuyilwa ukuphanza sokuphefumla. Iingcali bathi ivela neenkqubo sokuhlasela.

definition

Hamman Rich syndrome - isifo ezinxulumene inkqubo zophendlo lwezifo kwi inwebu lung, nto leyo ekhokelela sclerosis yayo kunye nophuhliso ukusilela sokuphefumla. Ukufunda oko yaqala kuphela phakathi kwenkulungwane yokugqibela, xa iincwadi achaza iimpawu ezifanayo ukuba neejenali rhoqo.

Le ngezifo ixhaphake kakhulu kubantu eliqina kunye nabantu abadala. Bakholelwa ukuba oku kubangelwa imikhwa emibi, ubude elide lweenkonzo kunye neempawu imisebenzi iimeko zokusebenza ezingafanelekanga (abatyhidi, mgodini, abakhi, njalo njalo. D.).

Izithethantonye

Ugqirha ngubani ngowokuqala ukufunda ngezifo yaba Scadding. Wacebisa ukuba kwithuba "fibrosing alveolitis." Kodwa kungekudala kakhulu oko waqalisa ukuba kusetyenziswa ingqiqo abanzi, elibhekisela iqela izifo ukuba babe iimpawu ezifanayo. Ngoko ke, kwadlula ngaphezu igama elithile.

Pathology labizwa isifo Scadding, nakuzo Hamman Rich syndrome, kwacaca ukuba ezi oogqirha ezimbini ezichazwe leengxaki efanayo ngo-1935. Kukho nezinye ntonye le yunithi nosological, umzekelo, progressive abanesigulo Embi pulmonary, dysplasia ezinezikhondo, ukuqina kwesibindi, umphunga ubovu inyumoniya Embi kunye nabanye. Xa yesilungu, ugunyaziwe ukuwasebenzisa bonke.

umyalezo weimeyile

Hamman Rich syndrome usekho enye izifo umyalezo ongaziwayo, kuba isenzeko of abanesigulo esivulekayo kwesi sifo kwemiphunga ijwabu reaction nonspecific nenkqubo yezifo. Ethandwa kakhulu kuluntu lwezenzululwazi yaba ingcamango yokuba esi sifo kubangelwa yinkqubo samathambo ngokusekelwe kukwalisa lohlobo lwesithathu (immunocomplex).

Kukho bathini kunye nethiyori polyetiology. Bathi inwebu imiphunga bayaboniswa bachazelwe kangangoko arhente eziliqela kwegciwane, amagciwane okanye uhlobo wentsholongwane egazini, nto leyo ekhokelela ukuba indlela hyperergic kwamajoni omzimba.

Kuba eziliqela izazinzulu ingqalelo ngaphambili-Rich syndrome Hamm njengenye sazo collagenosis, apho inkqubo pathologic ilinganiselwe ekukhanyeni kuphela.

Pathogenesis

pneumonitis Acute Embi (Hamman oluTyebileyo syndrome) ibonakala ngenxa ukuyilwa ekuthiwa-unit alveolar-capillary. Ukususela utshintsho indlela wakubonisa zophendlo lwezifo kuxhomekeke izinga nokuhanjiswa yeegesi egazini kunye, ngenxa hypoxia. Le nkqubo ayikho eyodwa kwesi sifo ethile, yaye amalungu zemiphunga enohlobo aphendula kukwenzakala.

Ukunciphisa Ukuncitshiswa yeegesi ngokusebenzisa alveoli kwenzeka ngenxa yezizathu eziliqela. Okokuqala, utshintsho Ebubanzini inwebu nezinto njengokuba lwefibrinogen kuwo plasma, okusaqhubekayo le ndawo, awenze zamahlathi ngakumbi kwaye ziqinile. Okwesibini, epithelium olunga- phakathi njengamaqhakuva ngokuma (alveoli) ngaphakathi uba cubic yaye ulahlekelwa iimpawu zayo. Okwesithathu, ukuba undigungqise inkqubo perfusion iigesi, oko kukuthi umoya okungena emiphungeni, kungekhona ukuqhagamshelana kwinkqubo yokujikeleza nayiphi na kule nkqubo lwenzeka kakhulu ngokukhawuleza. Ukongeza, kukho spasm reflex neempahla zonke emiphungeni ngenxa yokwanda uxinzelelo kwi kwegazi pulmonary, okwenza kukhule ngakumbi iindlela pathophysiological.

symptomatology

Hamman Rich syndrome ebantwaneni kunqabile. Esi sifo kakhulu eyenzeka eliqina kunye nabantu abadala, kodwa inokuba kwiintsana ngaphambi kwexesha kunye nomthamo zembuyekezo.

Xa eqala olucothayo kwesi sifo. Xa abantu, kukho iziqephu ukhohlokhohlo owomileyo kwaye uzive ngokuqinileyo esifubeni. Emva koko, ke uncediswa ukuphelelwa umoya kunye nokukhupha ukunyuka okungephi lobushushu, ubuthathaka, ukudinwa. Kumaziko okubhalela iimviwo sokuqala ibonakala ngokucacileyo zeminwe esiluhlaza kunye unxantathu nasolabial. Mhlawumbi utshintsho phalanges terminal kunye nail imilo.

Kukho zekhapitali kwentliziyo, rales ophuphumayo encinane emiphungeni. Nge phambili kwesifo iziqephu rhoqo ekhohlela, isikhohlela livela, yaye kwiimeko ezinqabileyo kunye hemoptysis (xa umsantsa capillary ekhoyo).

ekuxilongweni

Ehlasela abanesigulo Embi miphungeni (Hamman oluTyebileyo syndrome) kungabonwa ngokusebenzisa iimvavanyo kuphela ilebhu kwi zigaba terminal, njengoko kuvavanyo igazi liya kuba utshintsho non-ngqo:

• ukwandisa protein;
• SSCHE lunikezelo (neentlenge rate erythrocyte);
• utshintsho umlinganiselo amaqhekeza protein.

La manani zibonisa ubukho ukudumba, kodwa akazange ingxelo ngazo naziphi na uhlobo layo, okanye kwi indawo. Incinane nje nga kunceda isicwangciso ray emiphungeni. Kumanqanaba okuqala kukho ipateni ikhula kwingcambu lung, ngoko umnatha graininess Lung kwithishyu. Ekuhambeni kwexesha, imifanekiso kuvela kwimimandla eyandileyo ekhusini, nto leyo ethatha ukuba uhlobo honeycombs. Heart nazo ziya kutshintshwa ngenxa yokwanda yentliziyo ekunene.

diagnosis umehluko

isifo Hammam-Rich - sisifo ekunzima ukuxilonga kanye ngenxa yeenguqu nonspecific emiphungeni. Ukuze ngokuchanileyo baqiniseke uxilongo, ukuba ecaleni yonke enye into.

Ugqirha kufuneka ukwenza uthelekiso:

• alveolitis ziyityhefu kwaye ziyabagulisa fibroziruschim;
• inyumoniya mazwe;
• sarcoidosis;
• esasaziweyo tuberculosis pulmonary;
• systemic izifo zomzimba nokuqina (lupus, isifo samathambo, scleroderma, njl);
• umhlaza wemiphunga;
• hemosiderosis pulmonary;
• iqela of pneumoconiosis.

Kuphela xa kuthe zonke iinketho ngentla ezilahliweyo ngoxilongo syndrome Hammer-Rich. ukuze sifeze iimviwo kuhlolwe rhoqo. Ukuze benze oku, ugqirha ubeka i evulekileyo okanye evaliweyo yenaliti biopsy, kuxhomekeke amanqanaba bekujongwe sifo.

komzimba bathuthumele

Iimpawu kunye Uxilongo Hammam-Rich syndrome zixhomekeke kwindlela utshintsho ehlabathini ijwabu semiphunga. Ukwenzela ukuba kujongwe kubo, susa isiqwenga wemiphunga sihlolisise phantsi ngemicroscope. Iziphumo zesi sifundo ukuze hayi wabonakala lwezonyango ungqamane, kodwa zichanile ngakumbi kuba kubonisa ngqo umzimba karhulumente.

Ukuba sifo phantsi fibrosing alveolitis, lo sezifo ubona ijwabu ulwelo ukudumba ezinomthamo ophakamileyo fibrin kwi alveolar sayo, ukumitha izahlulelo iiseli impendulo arhabaxa. Ukuba inkqubo lwezidumbu iqhubeka, kuba utshintsho kubonakala histologically kubume iseli kwaye endothelial alveolar capillary ongaphantsi ndizayo ukuqina. Kwakhona kuyenzeka ubuso ukutshatyalaliswa alveoli, Ekuhlanganeni kweendlela zonxibelelwano kunye neempahla.

Kukho degrees ezintlanu yeenguqu emiphungeni:

1. Nokudumba kunye nokungena zeselula yeendonga alveolar.
2. Ulwelo agcwalise alveoli kunye iiseli desquamated okanye nokufunxwa izahlulelo exudate kunye ngetywina.
3. Ukutshatyalaliswa alveoli, ukuyilwa cysts kwi bronchi encinane.
4. Multiple aphakathi kunye cysts ezincinane.
5. "Zeeselula" ukukhanya ngenxa cysts ezinkulu.

Ikhosi nabanye abantu abane

Kelloidoz (Hamman oluTyebileyo syndrome) has a ALS ngokwanelisayo ihlwempu, kuba akunakwenzeka ukuba uyeke ukuqhubela phambili kwesifo. Nokuba ngamayeza inkqubo zophendlo lwezifo kunokuba kade, kodwa kungekudala okanye kamva isifo iya kuphumelela. Isigulane inokuba ukusuka kwiinyanga ezimbalwa ukuya kwiminyaka embalwa yobomi. Njengoko ukuthanda exacerbation kunye namaxesha noxolelo ezingazinzanga ngeli xesha.

Ekuhambeni kwexesha, imo ende yexesha insufficiency cardiopulmonary usabakhokela abantu ngokubhekiselele abakhubazekileyo, uze emva koko abulawe zizalwa. Kwimeko ilungileyo, isigulana siya kuba eminye iminyaka emihlanu ukuya esixhenxe ukususela ngexesha loxilongo. Kuxhomekeke ekubeni kuya zihambelane zonke iingcebiso gqirha. Kwiimeko ezinqabe kakhulu, isiphelo kuza emva kokuba isibini kweenyanga, kungakhathaliseki ukuba unyango. Ukuba unyango ucwangciselwe nisaqala sifo, kwiimeko ezinqabileyo kunokwenzeka ukuba "balondoloze" wobubuyisela ngeli nqanaba ze usindise ubomi bomntu, kunye nempilo isalamane.

unyango

Yintoni ukwenza uyagula, emva kokuba kwafunyaniswa ukuba syndrome Hammam-Rich? Unyango iqala ngoko nangoko. Akukho mfuneko ukuba ukulibazisa ukuqala unyango, njengokuba isifo siqhubela phambili kule minyaka ngakumbi nangakumbi. ayikenziwa kuphuhliswa unyango oluthile, ngoko oogqirha bazama ukuphelisa iimpawu wehlise isantya yethishyu abanesigulo.

Kumanqanaba okuqala lesi sifo steroids kakhulu esiqinisekisiweyo. Banciphisa wabonakala ukudumba: exudation kolwelo, nokungena zeselula, isantya ukuyilwa kwizicwili kunye nokuveliswa omzimba. unyango Bolus lwenziwa, ngamanye iqale ngeedosi eziphezulu (ukuya ku 60-80 mg ngosuku) kwaye ngcembe ukwehlisa isixa kweziyobisi ukuya ku-20 mg. Kubalulekile ukuba esweni umsebenzi lo neuron adrenal, ukuze ukuba Kushingoid syndrome.

Kumanqanaba kamva, xa libizwa ngokwaneleyo abanesigulo, kukhethwa iziyobisi abangaphangeliyo amajoni omzimba. ukusasazwa Special wafumana unyango indibaniselwano steroid kunye neziyobisi cytotoxic. Yaphumelela ngaphezu ukusetyenziswa kwala machiza ngamnye.

Uthomalaliso njengokuba kwangumqondiso wamisa ukuxinana, inhalation oksijini phantsi koxinzelelo, le iivithamini B, kwakunye antibiotics zisetyenziswa kangangoko kuyimfuneko (xa lokunithumelela ingxelo usuleleko eziziisekondari), glycosides ezesifuba womchamo (kunye nokwanda ukusilela entliziyweni).