Hammam-Rich syndrome: nkọwa

Hamman Rich syndrome - a dịtụ obere ọrịa (omume bụ naanị iri ikpe kwa narị puku ndị mmadụ), nke na-egosipụta onwe n'ụdị sclerosis nke ngụgụ na guzobe akụkụ okuku ume na ọdịda. Ọkachamara na-ekwu ya na omume na autoimmune Filiks.

definition

Hamman Rich syndrome - a ọrịa metụtara na a enweghị nchịkwa usoro ke akpa ume anụ ahụ, nke na-eduga ná ya sclerosis na mmepe nke akụkụ okuku ume na ọdịda. Ịmụ ya malitere naanị na n'etiti narị afọ gara aga, mgbe n'akwụkwọ sayensị Ugboro akwụkwọ nke kọwara yiri ihe mgbaàmà.

Nke a daa ọrịa bụ ihe nkịtị na n'etiti-agadi na okenye. Ọ na-kweere na nke a nwere ike ịbụ n'ihi àgwà ọjọọ, a ogologo ogologo nke ije ozi na ndị e ji mara Ọrụ ekwesighi arụ ọrụ na ọnọdụ (welders, miners, na-ewu ụlọ na na. D.).

Synonyms

A dibịa bụ onye mbụ na-amụ daa ọrịa bụ Scadding. Ọ tụrụ aro na a okwu ndị dị ka "fibrosing alveolitis." Ma nnọọ anya na ọ malitere ga-eji na a kama sara mbara uche, nke na-anọchi a otu ọrịa na-enwe ụdị ihe mgbaàmà. Ya mere, ọ were ihe karịrị a kpọmkwem aha.

Pathology e renamed Scadding ọrịa, na mgbe ahụ ka Hamman Rich syndrome, dị ka ọ tụgharịa si na abụọ ndị a dọkịta kọwara yiri daa ọrịa na 1935. E nwere ndị ọzọ synonyms a nosological unit, n'ihi na ihe atụ, na-aga n'ihu interstitial akpa ume fibrosis, fibrous dysplasia, imeju, akpa ume purulent interstitial oyi baa na ndị ọzọ. Na nkà mmụta ọgwụ ọgbara, na-ikike iji ha niile.

etiology

Hamman Rich syndrome ka na-anọgide otu n'ime ọrịa ndị na-amaghị etiology, ebe onu nke fibrosis aa na ọrịa a bụ akpa ume anụ ahụ nonspecific mmeghachi omume-enweghị nchịkwa na usoro. The kasị ewu ewu na ndị ọkà mmụta sayensị nwere a nchepụta nke na ọrịa na-kpatara ihe autoimmune usoro dabeere na nfụkasị Jeremaya nke atọ ụdị (immunocomplex).

E nwere na-akwado echiche na polyetiology Ozizi. Ha na-ekwu na akpa ume anụ ahụ na-gụrụ ọtụtụ mmadụ nke nje, fungal ma ọ bụ malitere ịrịa uwa, nke na-eduga hyperergic mmeghachi omume nke dịghịzi usoro.

N'ihi na a ọnụ ọgụgụ nke ndị ọkà mmụta sayensị na-atụle na mbụ-Rich syndrome Hamm dị ka otu n'onwe collagenosis, agaghi asu pathologic usoro dị nanị naanị ìhè.

pathogenesis

Nnukwu interstitial pneumonitis (Hamman Rich syndrome) pụtara ihe ruru ka e guzobere na-akpọ alveolar-capillary unit. Si otú kwuru na-enweghị nchịkwa na mgbanwe na-adabere na mgbasa ọnụego, haruru na ọbara na, dị ka a N'ihi ya nke hypoxia. Usoro a bụ bụghị nanị na nke a karịsịa ọrịa, na-ahụkarị akụkụ okuku ume na akụkụ omume ọjọọ.

Mbenata mgbasa nke gas site alveoli emee n'ihi ọtụtụ ihe. Akpa, mgbanwe nke akpụkpọ ahụ ọkpụrụkpụ na partitions ka fibrinogen bụ na plasma, na-emetụta ndị a ebe, na-eme ha ihe na ok na-agbanwe agbanwe. Nke abuo, na epithelium lining na airway sacs (alveoli) n'ime aghọ cubic na tụfuru ya Njirimara. Nke atọ, ọ maa jijiji perfusion usoro, haruru, ie ikuku na-abanye n'akpa ume, ọ bụghị na kọntaktị na nrugharị ọbara usoro nke ọ bụla na usoro a emee ngwa ngwa. Ke adianade do, e nwere a mpiaji spasm arịa nke ngụgụ ruru ka na-abawanye na mgbali na akpa ume mgbasa, nke ozokwa njọ na pathophysiological usoro.

symptomatology

Hamman Rich syndrome na ụmụ bụ obere. Nke a na ọrịa na-akasị adị na n'etiti-agadi na ndị agadi, ma nwere ike ịbụ na akaghi aka ụmụ ọhụrụ na-ebelata compensatory ikike.

The ngwa ngwa mmalite nke ọrịa. Na ụmụ mmadụ, e nwere ndị ji nke akọrọ ụkwara ma na-eche uko na obi. Mgbe ahụ, ọ na-esonyere iku ume ọkụ ọkụ na-elo a nta ịrị elu okpomọkụ, adịghị ike, ike ọgwụgwụ. Mgbe mbụ ule doro anya na anya bluish aka na nasolabial triangle. Ma eleghị anya, a mgbanwe nke ọnụ phalanges na ntu udi.

E accelerated obi otiti, obere bubbling rales na ngụgụ. Na ọrịa progression Ugboro ji nke, ụkwara, sputum na-egosi, na na obere okwu na hemoptysis (mgbe dị capillary ọdịiche).

nchọpụta nsogbu

Asị ibe interstitial akpa ume fibrosis (Hamman Rich syndrome) nwere ike achọpụtara site laabu ule naanị na ọnụ nkebi, dị ka a ọbara ule ga-ndị na-abụghị kpọmkwem mgbanwe:

• amụba protein;
• SSCHE osooso (erythrocyte sedimentation ọnụego);
• mgbanwe ndị na-ruru nke protein fractions.

A na ọnụ ọgụgụ na-egosi na ọnụnọ nke mbufụt, ma ghara ịkọrọ ọ bụla nke ya agwa, ma ọ bụ na ọnọdụ. A ntakịrị nwere ike inyere plan radiography nke ngụgụ. Na mbụ nkebi e a na-eto eto ụkpụrụ na akpa ume mgbọrọgwụ, mgbe ahụ, a net graininess Lung anụ ahụ. N'ime oge ahụ, foto na-egosi na mpaghara nke mụbara nghọta, nke na-ewe n'ụdị honeycombs. Heart ga-ga-gbanwere n'ihi na-abawanye nke na obi ziri ezi.

esi nchoputa

Hammam-Rich ọrịa - a ọrịa na-na-esi ike ịchọpụta kpọmkwem n'ihi nonspecific mgbanwe ngụgụ. Iji n'ụzọ ziri ezi ijide n'aka nke nchoputa, ewepu ihe ọ bụla ọzọ.

The dọkịta kwesịrị na-atụnyere:

• na-egbu egbu na-adịghị anabata alveolitis fibroziruschim;
• bilateral oyi baa;
• sarcoidosis;
• akwadebe akpa ume ụkwara nta;
• systemic connective anụ ahụ ọrịa na (lupus, ọrịa ogbu na nkwonkwo, scleroderma, wdg);
• ọrịa cancer akpa ume;
• akpa ume hemosiderosis;
• ìgwè nke pneumoconiosis.

Naanị na ihe omume ahụ na niile nke nhọrọ n'elu tụfuo chọpụtara syndrome Hammer-Rich. dị mkpa na-ebu postmortem ule mgbe. Iji mee nke a, dọkịta na-edepụta ihe na-emeghe ma ọ bụ mechiri emechi agịga biopsy, dabere n'obi nkebi nke ọrịa.

n'oké Mmebe

Mgbaàmà na Diagnosis Hammam-Rich syndrome-adabere na otú ụwa na mgbanwe na akpa ume anụ ahụ. Iji inwale ha, wepụ a mpempe akpa ume na-enyocha ya n'okpuru a mikroskopu. The results nke ọmụmụ a nwere ike ọ gaghị adaba adakarị ngosipụta, ma ha ihe ezi n'ihi na ha na-egosipụta na ala ahu.

Ọ bụrụ na ọrịa bụ n'okpuru fibrosing alveolitis, na pathologist ahụ anụ ahụ ọzịza na mmiri mmiri na a elu ọdịnaya nke fibrin na alveolar lumen, impregnation partitions mkpali omume mkpụrụ ndụ. Ọ bụrụ na-enweghị nchịkwa na usoro na-aga n'ihu, ọ bụ histologically doro mgbanwe cell Ọdịdị na endothelial alveolar capillary n'okpuru ala akpụkpọ ahụ ndim. Na-ekwe omume ele mmadụ anya n'ihu mmebi nke alveoli na ebe nke na kọntaktị na arịa.

E nwere ise degrees nke mgbanwe ndị na-akpa ume:

1. Edema na cellular infiltration nke alveolar mgbidi.
2. Fluid ejuputa alveoli na desquamated mkpụrụ ndụ ma ọ bụ absorption nke exudate partitions na akara.
3. The mbibi nke ndị na alveoli, guzobe cysts na obere bronchi.
4. Multiple ọkara na obere cysts.
5. "Cellular" ìhè n'ihi nnukwu cysts.

N'ezie na prognosis

Kelloidoz (Hamman Rich syndrome) nwere a pụtara ogbenye prognosis, ebe ọ gaghị ekwe omume ka ọ kwụsị progression nke ọrịa. N'agbanyeghị na ọgwụ enweghị nchịkwa usoro nwere ike ịbụ onye na-adịghị, ma n'oge ọ na ọrịa ka ga-emeri. Onye ọrịa nwere ike si na a ọnwa ole na ole ka a afọ ole na ole nke ndụ. Dị ka uche exacerbation na ngbaghara oge ejighị n'aka n'oge a.

N'ime oge ahụ, na-aga n'ihu cardiopulmonary insufficiency edu ndị kwupụta nkwarụ, na mgbe ahụ ka ọnwụ site asphyxia. Na nke kacha mma ikpe, onye ọrịa ga-ọzọ ise asaa afọ site n'oge nchoputa. Nyere na ọ ga-esoriri na niile na-atụ aro nke onye dibịa. Na obere okwu, ndị ọgwụgwụ abịa mgbe a di na nwunye nke ọnwa, n'agbanyeghị nke ọgwụ. Ọ bụrụ na ọgwụgwọ na ndokwa maka mmalite nkebi nke ọrịa, na obere okwu ọ bụ ike "chebe" ọ na a ogbo na chekwaa ndụ mmadụ, na ikwu ike.

ọgwụgwọ

Gịnị na-eme na-arịa ọrịa, mgbe a chọpụtara na ọrịa ahụ syndrome Hammam-Ọgaranya? Ọgwụgwọ-amalite ozugbo. Ọ dịghị mkpa ka oge na mmalite nke ọgwụgwọ, dị ka ọrịa na-aga n'ihu ihe karịrị afọ ọzọ na ndị ọzọ. Specific ọgwụgwọ na-erubeghị mepụtara, ka dọkịta na-agbalị ka ha kwụsị ihe mgbaàmà na-akwụsịlata ọsọ nke anụ ahụ fibrosis.

Na mbụ nkebi nke ọrịa ndị kasị dị irè nwapụtaworo Steroid. Ha belata mkpali ngosipụta: exudation nke ọmụmụ, cellular infiltration, jiri nwayọọ guzobe connective anụ ahụ na mmepụta nke-alụso ọrịa ọgụ. Bolus ọgwụ rụrụ, i.e. amalite na elu doses (ruo 60-80 mg kwa ụbọchị) na-eji nwayọọ nwayọọ belata ego nke ọgwụ ruo 20 mg. Ọ dị mkpa inyocha na ịchọpụtakwa ọrụ nke adrenal cortex, ka ọ ghara ime ka Kushingoid syndrome.

Na mgbe e mesịrị nkebi, mgbe zuru ezu akpọ fibrosis, mmasị e nyere ọgwụ ndị na-egbochi ndị dịghịzi usoro. Special nkesa natara a Nchikota ọgwụ nke Steroid na cytotoxic ọgwụ ọjọọ. Ọ bụ irè karịa nke ihe ndị a ọgwụ ọjọọ eme ihe n'otu n'otu.

The họpụtara ka symptomatic ọgwụ decongestants, inhalation nke oxygen nrụgide, ndị B vitamin, nakwa dị ka ọgwụ nje mee ihe dị ka ọ dị mkpa (mgbe attaching a abụọ ọrịa), gbasara obi glycosides na diuretics (na-abawanye na obi ọdịda).