Hammam-Rich ciwo: description

Hamman Rich ciwo - a gwada rare cuta (abin da ya faru shi ne kawai goma lokuta da dubu ɗari mutane), wanda bayyana kanta a cikin nau'i na sclerosis daga cikin huhu da kuma samuwar numfashi gazawar. Masana sun yi da'awar da ya faru ga autoimmune tafiyar matakai.

definition

Hamman Rich ciwo - wata cuta hade da wani pathological tsari a cikin huhu nama, wadda take kaiwa zuwa ta sclerosis da kuma ci gaban numfashi gazawar. Don karatu shi ya fara ne kawai a tsakiyar karni na karshe, a lokacin da mujallar kimiyya m wallafe da kwatancin kama cututtuka.

Wannan Pathology ne mafi kowa a tsakiyar-shekaru da tsaffi maza. An yi imani da cewa wannan na iya zama saboda miyagun halaye, a ƙara tsawon na sabis da kuma halaye na fasahohin m aiki yanayin (welders, hakar gwal, magina da sauransu. D.).

Kamancin

A likita wanda shi ne na farko a nazarin da Pathology ya Scadding. Ya nuna cewa wani lokaci kamar "fibrosing alveolitis." Amma sosai da ewa ba shi ya fara da za a yi amfani da wani m m ji, denoting wani rukuni na cututtuka da cewa suna da irin wannan bayyanar cututtuka. Saboda haka, shi ya ɗauki fiye da wani takamaiman sunan.

Pathology aka sake masa suna Scadding cuta, sa'an nan zuwa ga Hamman Rich ciwo, kamar yadda ya juya daga abin da waɗannan likitoci biyu sun bayyana kama Pathology a shekarar 1935. Akwai sauran ma'ana da wannan nosological naúrar, misali, m interstitial na huhu fibrosis, fibrous dysplasia, cirrhosis, huhu surkin jini interstitial ciwon huhu da sauransu. A zamani magani, yana da izini yin amfani da su duka.

etiology

Hamman Rich ciwo har yanzu ya kasance daya daga cikin cututtuka da ba a sani ba etiology, tun da sabon abu na fibrosis da ke faruwa a wannan cuta ne huhu nama nonspecific dauki ga pathological tsari. A mafi mashahuri a cikin kimiyya al'umma da wani ka'idar cewa cutar ne ya sa ta wani autoimmune tsari dangane da rashin lafiyan halayen na uku irin (immunocomplex).

Akwai proponents da polyetiology ka'idar. Suna jayayya da cewa huhu nama ne fallasa zuwa dama jamiái na kwayan cuta, fungal ko kwayar halitta, wadda take kaiwa zuwa hyperergic dauki na rigakafi da tsarin.

Domin a yawan masana kimiyya dauke baya-Rich ciwo Hamm matsayin daya embodiment collagenosis, cikinsa da pathologic tsari yana da iyaka zuwa kawai haske.

pathogenesis

M interstitial pneumonitis (Hamman Rich ciwo) bayyana saboda samuwar abin da ake kira alveolar-capillary naúrar. Daga yadda ya bayyana pathological canje-canje ya dogara da yadawa kudi na gas a cikin jini, kuma, kamar yadda wani sakamako na hypoxia. Wannan tsari ne ba musamman ga wannan musamman cuta, kuma hankula numfashi gabobin mayar da martani ga rauni.

Rage yadawa na gas ta hanyar da Alveoli faruwa ga dalilai da dama. Na farko, da canji na membrane kauri da kuma partitions kamar yadda fibrinogen ne a cikin jini, permeates wadannan yankunan, samar da su su m, kuma m. Abu na biyu, da epithelium rufi da airway sacs (Alveoli) a ciki zama cubic da kuma hasarar da kaddarorin. Na uku, shi gaji da damuwa da perfusion aiwatar gas, watau iska da cewa ta shiga huhu, ba a lamba tare da jijiyoyi da wani da wannan tsari ya auku ma da sauri. Bugu da kari, akwai wata reflex spasm na tasoshi da tukwane na huhu saboda da karuwa a matsa lamba a cikin huhu wurare dabam dabam, wanda kara exacerbates da pathophysiological sunadaran.

symptomatology

Hamman Rich ciwo a yara ne rare. Wannan cuta mafi yawa yakan faru a tsakiyar-shekaru da tsaffi, amma zai iya zama a cikin wanda bai kai ba jarirai da rage compensatory iya aiki.

Da jinkirin farko na cutar. A cikin mutane, akwai aukuwa busassun tari da kuma jin m, a cikin kirji. Sa'an nan kuma, shi ne ya koma da nakasa daga 'ya'yan numfashi da kuma exhale kadan Yunƙurin a zazzabi, wani rauni, gajiya. A binciken farko a fili a bayyane bluish sauki da kuma nasolabial alwatika. Watakila wani canji na m phalanges da ƙusa siffar.

Akwai kara bugun zuciya, kananan bubbling rales a cikin huhu. Tare da cutar ci gaban m aukuwa na tari, sputum bayyana, kuma a rare lokuta da hemoptysis (idan akwai capillary rata).

bincikowa da

Yaxuwa interstitial na huhu fibrosis (Hamman Rich ciwo) za a iya gano ta hanyar dakin gwaje-gwaje gwaje-gwaje ne kawai a cikin m, saukarwa, kamar yadda a wani jini gwajin zai zama maras takamaiman canje-canje:

• kara gina jiki.
• SSCHE hanzari (erythrocyte sedimentation rate).
• canje-canje a cikin rabo na gina jiki kasarun adadi.

Wadannan alkaluma sun nuna gaban kumburi, amma bai bayar da rahoton wani daga cikin halitta, kuma a kan location. A kadan iya taimaka shirin radiography daga cikin huhu. A matakin farko akwai mai girma juna a cikin huhu tushen, sa'an nan a net graininess Huhu nama. A tsawon lokaci, da hotunan bayyana yankuna na ƙara nuna gaskiya, wanda daukan nau'i na sakar zuma. Zuciya ma za a canza saboda karuwa da dama zuciya.

bambanci ganewar asali

Hammam-Rich cuta - wata cuta da cewa da wuya a gane asali daidai saboda nonspecific canje-canje a cikin huhu. Domin a daidai tabbata daga cikin ganewar asali, to ware komai.

Likita yana bukatar yin kwatancen:

• mai guba da kuma rashin lafiyan alveolitis fibroziruschim.
• hadin ciwon huhu.
• sarcoidosis.
• watsa na huhu da tarin fuka.
• tsari connective nama cututtuka (lupus, rheumatoid amosanin gabbai, scleroderma, da dai sauransu).
• huhu ciwon daji.
• na huhu hemosiderosis.
• kungiyar na pneumoconiosis.

Kawai a cikin taron cewa duk da zabin sama jefar da suka kamu da ciwo Hammer-Rich. Dole a gudanar da wani postmortem jarrabawa sau da yawa. Don yin wannan, likita ya furta wani bude ko rufe allura biopsy, dangane da nufin matakai na cutar.

mugun jin haltta

Alamun da ganewar asali Hammam-Rich ciwo ya dogara ne a kan yadda za a duniya canje-canje a cikin huhu nama. Domin kimanta su, cire wani yanki na huhu da kuma nazarin da shi a karkashin wani hange. A sakamakon wannan binciken zai iya ba ta zo daidai da na asibiti manifestations, amma sun kasance mafi m saboda su gani kai tsaye a jihar jiki.

Idan cutar ne a karkashin fibrosing alveolitis, da sani akan cututtuka gani nama busa ruwa tare da wani babban abun ciki na fibrin a alveolar lumen, impregnation partitions kumburi amsa Kwayoyin. Idan pathological aiwatar progresses, shi ne histologically bayyananne canje-canje a cikin cell tsarin da endothelial alveolar capillary ginshiki membrane thickening. Har ila yau, zai yiwu m halakar da Alveoli a cikin wurare na lamba tare da tasoshin.

Akwai biyar digiri na canje-canje a cikin huhu:

1. Edema da kuma salon salula infiltration na alveolar katangu.
2. Bewafa ciko da Alveoli da desquamated Kwayoyin ko sha na exudate partitions da hatimi.
3. Halakar da Alveoli, da samuwar cysts a cikin kananan bronchi.
4. Mahara matsakaici da kuma kananan cysts.
5. "Salon salula" haske saboda manyan cysts.

Course da kuma hangen nesa

Kelloidoz (Hamman Rich ciwo) yana da wani fairly matalauta hangen nesa, tun ba shi yiwuwa a dakatar da ci gaban da cutar. Ko da tare da magani pathological tsari na iya zama m, amma jima ko daga baya da cutar zai har yanzu lashe. A haƙuri iya zama daga 'yan watanni a' yan shekaru na rayuwa. Kamar yadda za exacerbation da kuma gafarar lokaci m lokacin wannan lokaci.

A tsawon lokaci, da m cardiopulmonary insufficiency kaiwa mutane zuwa ga tawaya, sa'an nan zuwa ga mutuwa ta asphyxia. A cikin mafi kyau idan, haƙuri zai zama wani shekaru biyar zuwa bakwai daga lokacin da ganewar asali. Bayar da cewa zai bi da dukan shawarwari na likita. A rare lokuta, da karshen zo bayan kamar wata biyu, ko da kuwa da far. Idan magani da aka shirya domin farkon matakai na cuta, a rare lokuta yana yiwuwa a "tsare" shi a wannan mataki da kuma ajiye mutum rayuwar, da kuma zumunta kiwon lafiya.

magani

Abin da ya yi rashin lafiya, bayan ya kamu da ciwo Hammam-Rich? Jiyya ta fara nan da nan. Ba lallai ba ne a jinkirta fara jiyya, kamar yadda cutar progresses a tsawon shekaru fiye da kuma mafi. Specific magani ne ba tukuna raya, don haka likitoci kokarin dakatar da cututtuka da kuma rage gudu gudun nama fibrosis.

A matakin farko na cutar da mafi tasiri tabbatar steroids. Su rage kumburi bayyananen: exudation na ruwa, salon salula infiltration, rage gudu da samuwar connective nama da kuma samar da antibodies. Bolus far yi, Ina nufin fara da high allurai (har zuwa 60-80 MG da rana) da kuma hankali rage adadin miyagun ƙwayoyi har zuwa 20 MG. Yana da muhimmanci a saka idanu na ayyuka na adrenal bawo, saboda haka kamar yadda ba su sa Kushingoid ciwo.

A baya, saukarwa, a lokacin da isasshe furta fibrosis, baicin aka bai wa kwayoyi da damuarn tsarin na rigakafi. Special rarraba samu a hade far na steroids da cytotoxic kwayoyi. Ya fi tasiri fiye da yin amfani da wadannan kwayoyi akayi daban-daban.

A nada a matsayin symptomatic far decongestants, inhalation na oxygen a karkashin matsin lamba, da B bitamin, kazalika da maganin rigakafi amfani da zama dole (idan attaching da sakandare kamuwa da cuta), cardiac glycosides da diuretics (tare da karuwa a zuciya rashin cin nasara).