Hammam-Rich syndrome: incazelo

Hamman Rich syndrome - isifo ayeyivelakancane (legciwane kuyinto amacala ayishumi kuphela ngamunye abantu abayizinkulungwane ezingamakhulu), esizibonakalisa ifomu yokuqina kwamathishu kwamaphaphu kanye kumiswa nokuvaleka kwemigudu yokuphefumula. Ochwepheshe bathi ivela khona izinqubo sokuhlasela kwamasosha omzimba.

definition

Hamman Rich syndrome - isifo elihlobene ne inqubo sokugembula kule izicubu wamaphaphu, okuholela sclerosis yayo kanye nokuthuthukiswa nokuvaleka kwemigudu yokuphefumula. Ukuze ukufunda yaqala kuphela phakathi nekhulu leminyaka eledlule, lapho izincwadi emaphephabhukwini esayensi njalo nge izincazelo nezimpawu ezifanayo.

Lokhu zokugula landile amadoda asebeqinile nasebekhulile. Kukholakala ukuthi lokhu kungenzeka kubangelwa imikhuba emibi, ubude eside wenkonzo izici ukuzibonakalisa izimo zokusebenza lungafanelekile (welders, abavukuzi basezimayini, abakhi nokunye. D.).

Synonyms

Udokotela owayengowokuqala ukutadisha zokugula kwaba Scadding. Wasikisela ukuthi eside ezifana "fibrosing alveolitis." Kodwa ngokushesha kakhulu yokuthi yaqala ukukhulunywa lisetshenziswa ngomqondo abanzi, elisho leqembu lezifo ukuthi unezimpawu ezifanayo. Ngakho-ke, wathatha ngaphezu igama othize.

Pathology iqanjwe kabusha Scadding isifo, bese ukuze lesi sifo Hamman Rich, njengoba Kwavela ukuthi lab 'odokotela ababili baye bachaza zokugula efanayo ngo-1935. Kunezinye omqondofana yalolu phiko nosological, isibonelo, lesichubekako interstitial cystic yamaphaphu, dysplasia lunemicu ngokoma kwesibindi, wamaphaphu ubovu inyumoniya interstitial nabanye. Ngo inqubo yokwelapha yesimanje, ogunyazwe ukusebenzisa bonke.

omsakazo

Hamman Rich syndrome namanje uhlala omunye yezifo omsakazo akukaziwa ngoba lo mkhuba cystic ezenzeka kule sifo iphaphu izicubu ukuphendula nonspecific ukuze inqubo sokugembula. Ethandwa kakhulu emphakathini yesayensi kwadingeka umbono ukuthi sifo sibangelwa inqubo sokuhlasela kwamasosha omzimba esekelwe komzimba yohlobo wesithathu (immunocomplex).

Kukhona abasekela futhi polyetiology theory. Bathi izicubu wamaphaphu echayeke ejenti eziningana webhaktheriya, fungal noma imvelo enobuthi, okuholela ukuphendula hyperergic kwesimiso somzimba sokuzivikela ezifweni.

Kwaphela ososayensi kubhekwe ngaphambili-Rich syndrome Hamm njengenye samuntu collagenosis, lapho inqubo pathologic kukhawulwe ukukhanya kuphela.

pathogenesis

Acute pneumonitis interstitial (Hamman Rich syndrome) ubonakala ngenxa kumiswa okuthiwa iyunithi alveolar-capillary. Kusukela izinguquko sokugembula kanjani uzwakalise incike izinga okusheshayo yamagesi egazini futhi, ngenxa hypoxia. Le nqubo asigcini kule isifo esithile, futhi ejwayelekile izitho zokuphefumula ekuphenduleni ukulimala.

Ukwehlisa ukwanda okusheshayo yamagesi ngokusebenzisa ama-alveoli kwenzeka ngenxa yezizathu eziningana. Okokuqala, ushintsho we ukushuba ulwelwesi kanye ngodonga njengoba fibrinogen kuyinto yi-plasma, ogcwele lezi zindawo, abenze ayazika futhi kube lukhuni. Okwesibili, epithelium ezazigudla sacs umgudu (alveoli) ngaphakathi iba cubic kanye ilahlekelwa impahla yayo. Okwesithathu, eyayiphazamisa perfusion inqubo amagesi, okusho umoya ongena emaphashini, hhayi nabo isimiso sokujikeleza kwegazi kwanoma iyiphi le nqubo kwenzeka ngokushesha kakhulu. Ngaphezu kwalokho, kukhona spasm reflex imithambo kwamaphaphu ngenxa ukwanda ukugandeleleka yokujikeleza kwegazi eliya emaphashini, okuyinto kwandzisa izindlela pathophysiological.

symptomatology

Hamman Rich syndrome ezinganeni ezingavamile. Lesi sifo ikakhulukazi kwenzeka asebeqinile nasebekhulile, kodwa kungenzeka ezizalwa ngaphambi kwesikhathi kanye linganciphisa umthamo wamandla compensatory.

Ukuqala kancane lesi sifo. Kubantu, kukhona iziqephu isifuba esomile kanye nomuzwa liqinile esifubeni. Khona-ke, uhlangana iphika exhale ukwenyuka okuncane nje kwamazinga okushisa, ubuthakathaka, ukukhathala. Ngesikhathi luhlolo kokuqala kuyabonakala kalula bluish futhi unxantathu Nasolabial. Mhlawumbe ushintsho phalanges esibulalayo futhi nail ukuma.

Kube yashesha ukushaya kwenhliziyo, rales encane ogobhozayo emaphashini. Nge ukuqhubekela phambili kwesifo iziqephu kaningi ukukhwehlela, isikhwehlela livela, futhi ezimweni ezingavamile futhi hemoptysis (uma sikhona capillary igebe).

ukuhlola

Ehlasela umzimba interstitial cystic yamaphaphu (Hamman Rich syndrome) kungatholakala esebenzisa ukuhlolwa laboratory kuphela izigaba esibulalayo, njengasezulwini ukuhlolwa igazi kuyoba izinguquko ze-non-ethize:

• okwandisa amaprotheni;
• SSCHE ukushesha (erythrocyte sedimentation izinga);
• izinguquko isilinganiso amaprotheni izingxenyana zaso.

Lezi zinombolo zibonisa khona ukuvuvukala, kodwa akazange abike noma isiphi isimo sawo, futhi indawo. A kancane kungasiza uhlelo esimweni kwamaphaphu. Ezigabeni zokuqala kukhona iphethini ezikhulayo wamaphaphu impande ke inetha graininess Lung izicubu. Ngokuhamba kwesikhathi, izithombe avele izindawo obala anda, okuyinto ethatha ngesimo honeycombs. Inhliziyo uzophinde ukushintshwa ngenxa ukwanda kwe-senhliziyo esifanele.

ukuxilongwa umehluko

isifo Hammam-Rich - isifo kunzima ukuxilonga ngokunembile ngenxa yezinguquko nonspecific emaphashini. Ukuze ngokunembile uqinisekile kwesifo, ukubekela eceleni konke okunye.

Udokotela udinga iziqhathanise:

• esinobuthi iguliswa alveolitis fibroziruschim;
• inyumoniya lokuvunyelenwe ngato;
• sarcoidosis;
• eqonda isifo sofuba yamaphaphu;
• letindzala izifo ezicutshini (lupus, i-rheumatoid arthritis, scleroderma, njll);
• umdlavuza wamaphaphu;
• hemosiderosis yamaphaphu;
• iqembu pneumoconiosis.

Kuphela uma kwenzeka ukuthi bonke okokukhetha kulahliwe kutholakala ukuthi uphethwe yini syndrome Hammer-Rich. idingekile ukuze postmortem luhlolo ngokuvamile. Ukuze wenze lokhu, lo dokotela sinquma evulekile noma avaliwe inaliti biopsy, kuncike izigaba okuhloswe lesi sifo.

anatomy okukhwantabalisayo

Izimpawu kanye Sifo Hammam-Rich syndrome kuxhomeke endleleni izinguquko emhlabeni wonke izicubu wamaphaphu. Ukuze sihlole kubo, susa ucezu wamaphaphu ukuyihlola ngesibonakhulu. Imiphumela yalolu cwaningo lungase lungahambisani nalolo ukubonakaliswa zokwelapha, kodwa esinembile ngoba zibonisa ngqo emzimbeni isimo.

Uma lesi sifo ngaphansi fibrosing alveolitis, le sezifo ubona ukuvuvukala izicubu ketshezi nge wokuqukethwe lelisetulu fibrin ku alveolar inesikhala, impregnation ngodonga ukuvuvukala impendulo amaseli. Uma inqubo sokugembula iqhubeka, kuba nezinguquko sobala histologically kuseli isakhiwo kanye endothelial alveolar capillary engaphansi ulwelwesi ukuqina. Futhi kungenzeka ezicekeleké we-alveoli ezindaweni ukuxhumana izitsha.

Kukhona degrees ezinhlanu izinguquko emaphashini:

1. Edema kanye kokuziphatha yeselula izindonga alveolar.
2. Fluid ukugcwalisa alveoli namaseli desquamated noma ukumuncwa exudate ngodonga kanye uphawu.
3. Ukubhujiswa kwabantu alveoli, ukwakheka izinhlumba ku bronchi encane.
4. naphakathi Multiple kanye izinhlumba encane.
5. "Yeselula" ukukhanya ngenxa izinhlumba ezinkulu.

Course futhi Izibikezelo

Kelloidoz (Hamman Rich syndrome) has a Izibikezelo sasivumela abampofu, ngenxa yokuthi kunzima ukuyeka ukuqhubeka kwesifo. Ngisho ngemithi inqubo sokugembula kungahamba kancane, kodwa ngokuhamba kwesikhathi lesi sifo isazoqhubeka uwine. Isiguli kungenzeka kunezinyanga ezimbalwa kuya eminyakeni embalwa yokuphila. Njengoba intando exacerbation kanye ukuthethelelwa nezikhathi engazinzile phakathi nalesi sikhathi.

Ngokuhamba kwesikhathi, lesichubekako esingamahlalakhona cardiopulmonary ubudlelwane obuholela abantu ekwenzeni ukukhubazeka, bese ukufa asphyxia. Endabeni engcono, isiguli kuyoba eminye iminyaka emihlanu kuya eziyisikhombisa kusukela ngesikhathi uphethwe. Kuncike ekutheni ke izohambisana nazo zonke izincomo udokotela. Ezimweni ezingavamile, kungakhathaliseki ukuthi ukuphela kufika emva kwezinyanga ezimbalwa, kungakhathaliseki ukuthi ukwelashwa. Uma impatho kuhlelelwe kusaqalwa sifo, ezimweni ezingajwayelekile kungenzeka ukuba "balondoloze" ke kulesi sigaba bese ugcine ukuphila wale ndoda, futhi impilo isihlobo.

ukwelashwa

Okufanele ukwenze iyagula, emveni kokusuka kwakhe banalesi sifo Hammam-Rich? Ukwelashwa iqala ngokushesha. Akubalulekile uyephuza kokuqala ukwelashwa, njengoba isifo siqhubeka eminyakeni ezingasho. oluthile lokwelapha ayikahlanganiswa athuthukile, ngakho odokotela bazama ukuzivimba lezi izimpawu bese wehlisa ijubane ijubane izicubu cystic.

Ezigabeni zokuqala zesifo ephumelela kunazo steroid efakazelwe. Zinciphisa ukubonakaliswa ukuvuvukala: exudation uketshezi, kokuziphatha yeselula, ukubambezela kumiswa sezicubu zezinzwa futhi ukukhiqizwa kwamasosha omzimba. yokwelapha Bolus kwenziwe, okungukuthi ukuqala nge ebulilini (kufika ku 60-80 mg ngelilanga) kanye kancane kancane inciphise izidakamizwa kufika ku-20 mg. Kubalulekile ukuqapha umsebenzi cortex adrenal, ngakho abangele Kushingoid syndrome.

Ngo ezigabeni kamuva, lapho ngokwanele liphinyiselwe cystic, kubonelelwa kakhulu ama-izidakamizwa ukuthi kuvimbela izivikeli mzimba. ukusatshalaliswa Special Uthole ukwelashwa kwezidakamizwa nezidakamizwa cytotoxic. Kwaba nemiphumela engcono kunokuthi ukusetshenziswa kwalezi izidakamizwa ngamunye.

I aqokwa njengelungu lingaze yokwelapha yokuvula amakhala, ephefumula umoya-mpilo sicindezelekile, amavithamini B, kanye imithi elwa namagciwane esetshenziswa njengoba kudingekile (lapho unamathisela igciwane zesibili), glycosides zenhliziyo isisu (begodu kukhula senhliziyo).