Hammam-Rich syndrome: description

Hamman Rich syndrome - cudur dhif (dhacdooyinka waa Xaaladaha toban keliya per boqol oo kun oo qof), taas oo u muuqato in qaab sclerosis ee sambabada iyo formation of ay neefta. Khubarada u yeelaan in ay dhacdo in ay geedi socodka, shalalka.

qeexid

Hamman Rich syndrome - cudur la xidhiidha geedi socodka a bahal ah ee unugyada sambabada, taas oo horseedaysa in ay sclerosis iyo horumarinta neefta. Si aad u barato waxa bilaabay oo kaliya ee bartamihii qarnigii la soo dhaafay, marka publications ee joornaalada sayniska soo noqnoqda la tilmaanta calaamado la mid ah.

cudurada Tani waxay ku badan yahay ragga da'da dhexe iyo waayeelka. Waxaa la rumeysan yahay in ay tani sabab u noqon kara caadooyinka xun, dhererka dheer adeega iyo sifooyinka xirfadaha xaalado shaqo oo ku habboonayn (alxanle, macdan qodayaasha, oo kuwii ku dhisayna iyo D. sidaas on.).

micne ah

dhakhtarka A oo ahaa ugu horeeyay ee ku baran cudurada ahaa Scadding. Waxa uu soo jeediyay in muddo ah sida "alveolitis fibrosing." Laakiin si dhakhso ah u bilaabay inuu la isticmaalay dareen halkii ballaadhan, in midani koox ka mid ah cudurada in ay leeyihiin calaamado la mid ah. Sidaa darteed, waxaa ka badan magac gaar ah qaaday.

Pathology waxaa magacooda loo baxshay cudur Scadding, ka dibna u syndrome Hamman Rich, maadaama ay ka leexatay in labo takhtar ay ku tilmaamay cudurada la midka ah ee 1935. Waxaa jira micne kale ee cutubkan nosological, tusaale ahaan, horusocod fibrosis pulmonary interstitial, Kobac buunshada ku, cirrhosis, purulent sambabada pneumonia interstitial iyo kuwa kale. In daawo casri ah, waxa la oggol yahay in ay iyaga oo dhan ay isticmaalaan.

etiology

Hamman Rich syndrome weli mid ka mid ah cudurada of etiology aan la garanayn, tan iyo arrin ku fibrosis dhaca cudurkan waa unug sambabada falcelinta nonspecific hanaanka bahal ah. The ugu caansan ee bulshada sayniska lahaa aragtida ah, in cudurka waxaa keena geedi shalalka ku salaysan dareen diidmo oo xasaasiyadeed ee nooca saddexaad (immunocomplex).

Waxaa jira Taageerayaasha iyo aragti polyetiology. Waxay ku doodayaan in unugyada sambabka la kulmin dhowr wakiilada bakteeriyada, fangas ama dabeecadda viral, taas oo horseedaysa in ay dareen-celin hyperergic ee habka difaaca jirka.

Waayo, tiro ka mid ah aqoonyahaniinta tixgeliyo hore-Rich syndrome Hamm sida mid muuqashadii of collagenosis, diidanyihiin habka pathologic waa xadidan oo ay iftiinka kaliya.

pathogenesis

pneumonitis interstitial Ba'an (Hamman Rich syndrome) muuqataa ay sabab u formation of sidaas-yiraahdo qaybta alveolar-gaddoonka ah. Laga soo bilaabo isbedelka bahal ah sida ka muujiyay ku xiran tahay heerka sida- of gaasas ku jira dhiigga iyo, sida natiijo ah oo hypoxia. Hawlgalkani waxa aan gaar u tahay cudurka this gaar ah, iyo xubnaha caadiga ah neefsashada jawaabayo dhaawac.

Yaraynta sida- ee gaasaska dhex Alfiyoli ku dhacdaa sababo dhowr ah. First, isbeddel ah ee dhumucda xuub iyo qoruhu sida fibrinogen waa in plasma ah, galay goobahaas, iyaga more cufan oo adag. Marka labaad, epithelium lasoo kiishashka haweedka (Alfiyoli) gudaha noqdo cubic iyo lumiyo hantida. Saddexaad, waxaa dhibtay gaasas habka perfusion, ie hawada gala sambabada, aan xiriir la nidaamka wareegga dhiigga ee wax habka this si degdeg ah dhacdaa. Intaa waxaa dheer, waxaa jira waa dabac ah reflex of weelal sambabada ay sabab u tahay kororka cadaadis wareegga pulmonary, taas oo sii kordhineysa ah hababka pathophysiological.

symptomatology

Hamman Rich syndrome ee carruurta waa dhif. Cudurku wuxuu inta badan ku dhacaa da'da dhexe iyo waayeelka, laakiin waxaa laga yaabaa in dhallaanka dhicis ah oo leh awood magdhawga la dhimay.

bilawga gaabis ah cudurka. In aadanaha, waxaa jira dhacdooyin of qufac qalalan oo giigsan xabadka. Markaas, waxaa ku biiray by neefta oo ku qabata iyo neefta siidaysid kicitaan yar oo heerkulka, daciifnimo, daal. At baaritaanka hore si cad u arki karo faraha buluug iyo xagal nasolabial. Waxaa laga yaabaa in wax isbedel ah oo phalanges terminal iyo qaabka cidiyaha.

Waxaa la dedejiyey garaaca wadnaha, crackling qeexnayn yar ee sambabada. Iyadoo faafidda cudurka dhacdooyin soo noqnoqda ee qufac, xaako muuqataa, iyo marmar dhif iyo hemoptysis (marka farqiga u gaddoonka ah la heli karo).

Baadhista xanuunada

Waajihi fibrosis pulmonary interstitial (syndrome Hamman Rich) waxaa lagu ogaan karaa iyadoo qofka la sheybaar oo kaliya ku jira marxaladaha terminal, sida in tijaabo dhiig laga beddelo non-gaar ah:

• sii kordhaya protein,
• SSCHE dardargelinta (heerka Kimikada erythrocyte);
• isbeddel ah ee saamiga jajabyada protein.

Tirooyinkan ayaa tilmaamaya jiritaanka barar, laakiin ma sheegaan wax kasta oo ay dabiicadda, ama goobta. A yar oo kaa caawin kara raajada qorshaha sambabada. In marxaladaha hore waxaa jira qaab kordhaya xididka sanbabka, ka dibna a unugyada Sambabka graininess net. Waqti ka, waxay u muuqdaan sawirrada gobollada ee daahfurnaanta kordhay, taas oo qaadataa foomka of abaaraha. Heart sidoo kale loo beddeli doonaa sabab u tahay kororka qalbiga saxda ah.

cudurka kala duwan

cudurka Hammam-Rich - cudur adag tahay in la ogaado si hufan sababta oo ah isbedel nonspecific ee sambabada. Si aad u si sax ah u hubi ee cudurka, si looga saaro wax walba oo kale.

Dhakhtarku waxa uu u baahan yahay inuu is bar bar dhigo:

• sun iyo xasaasiyad alveolitis fibroziruschim;
• pneumonia laba geesoodka ah,
• sarcoidosis;
• faafiyay qaaxada sambabada,
• cudurada systemic connective unugyada (lupus, rheumatoid arthritis, scleroderma, iwm);
• kansarka sanbabada,
• hemosiderosis pulmonary;
• group of pneumoconiosis.

Kaliya ay dhacdo in dhammaan fursadaha kor ku tuuraa cudurka syndrome Hammer-Rich. lagama maarmaan ah in ay fuliyaan Baaritaanka neef inta badan. Si arrintan loo sameeyo, dhakhtarka kuu qoray ah biopsy irbad furan ama xiran yihiin, waxayna ku xiran tahay marxaladaha loogu talagalay cudurka.

anatomy silica

Calaamadaha iyo Cilad Hammam-Rich syndrome ku xiran tahay sida isbedellada caalamka in unugyada sambabada. Si loo qiimeeyo, ka saar goosin ka mid ah sambabka oo uu fiiriyo microscope ka yar. Natiijada daraasaddan laga yaabaa in aan ku beeganto daliilka kiliinikada, laakiin si ka badan sax yihiin maxaa yeelay, waxay si toos ah u fikiri jirka gobolka.

Haddii cudurka uu ka yar yahay alveolitis fibrosing, pathologist arko unugyada dareere barar la content sare ah fibrin ee Lumen alveolar, qoruhu dahaadhida unugyada jawaab bararka. Haddii habka bahal socdo, waa isbedel histologically cad in qaab-dhismeedka gacanta iyo endothelial alveolar hoostiisa gaddoonka dhagid xuub. Sidoo kale burburinta suurto gal ah qayb ka mid ah Alfiyoli ah meelaha of xiriir la weelasha.

Waxaa jira shan heer oo isbeddel ah ee sambabada,

1. Barar iyo dhexgalka gacanta derbiyada alveolar.
2. Dheecaan buuxinta ka Alfiyoli iyo unugyada desquamated ama nuugista qoruhu exudate iyo shaabadda.
3. burburinta Alfiyoli ah, formation of fiix in hawomareenno yaryar.
4. dhexdhexaad ah Multiple iyo buurbuur yar.
5. light "Gacanta" sabab u ah fiix waaweyn.

Koorsada iyo saadaal

Kelloidoz (Hamman Rich syndrome) ayaa saadaal ah si cadaalad ah dadka saboolka ah, maadaama ay tahay wax aan macquul aheyn in la joojiyo horumarka cudurka. Xitaa daawo habka bahal noqon kartaa gaabis ah, laakiin si degdeg ah ama waqti dambe cudurka weli ku guuleysan doono. Bukaanka ayaa laga yaabaa in ay ka dhawr bilood ilaa dhowr sano ee nolosha. Sida sii daridda doonista iyo muddooyinka dembidhaafka deganayn inta lagu jiro waqtigan.

Waqti ka, ee horumar kaafi wadnaha iyo keenaysaa dadka dhinaca naafada, ka dibna waa in la dilaa by-qabatin. In kiiska ugu fiican, bukaanka uu noqon doono shan ilaa toddoba sano oo kale laga bilaabo wakhtiga cudurka. Shardi tahay in uu u hoggaansamo doonaa dhammaan talooyinka dhakhtarka. Marar dhif ah, dhamaadka yimaado laba bilood ka dib, iyadoo aan loo eegin therapy ah. Haddii daaweynta ayaa la qorsheeyay marxaladaha hore ee cudurka, marmar dhif ah waxaa suurtagal ah in "loo ilaalin lahaa" waxaa xilligan la joogo iyo nin ee nolosha, iyo caafimaadka qaraabo badbaadiyo.

daaweynta

Maxaa la sameeyaa xanuunsan, ka dib markii uu helay syndrome ee Hammam-Rich? Daaweynta isla markiiba bilaabmaa. Ma loo baahan yahay in dib u bilowga daweynta, sida cudurku uga sii darayo sano ka badan oo badan. daaweynta gaarka ah weli ma horumariyo, si dhakhaatiir isku dayaan in ay joojiyaan calaamadaha iyo hoos xawaaraha fibrosis unugyada.

In marxaladaha hore ee cudurka ku steroids la xaqiijiyay ugu waxtarka badan. Waxay yareeyo daliilka bararka: maadoyinka ah ee dareeraha, dhexgalka gacanta, hoos u dhigi formation of unugyo iyo wax soo saarka ee antibodies. therapy bolusdose sameeyaa, i.e. bilowdo qaadasho sare (ilaa 60-80 mg maalintii) iyo si tartiib tartiib ah loo yareeyo xaddiga daroogada ilaa 20 mg. Waxaa muhiim ah si ay ula socdaan dhaqdhaqaaqa ee kiliyaha adrenal, si aanay u keeni Kushingoid syndrome.

Marxaladaha dambe, markay ku filan loogu dhawaaqaa fibrosis, doorashada waxaa la siiyaa daawooyin uu hakiyo nidaamka difaaca jirka. qaybinta Gaarka helay therapy isku dhafan ee steroids iyo daroogada cytotoxic ah. Waxa ay ahayd ka waxtar badan isticmaalka daroogada, kuwaas oo si gaar ah.

The magacaabay therapy sida astaamaha caburka, neefsado Oksijiinka oo cadaadis, vitamins B ah, iyo sidoo kale antibiotics loo isticmaalaa sida loo baahan yahay (marka lifaaqaaya caabuq a), glycosides wadnaha iyo diuretics (la kordhiyo wadnaha).