Syndrome Hammam-Olemera: kufotokoza

Hamman Olemera syndrome - matenda n'chosoŵa (zochitika Nthawi khumi okha pa anthu zikwi zana), amene kumaonekera mwa mawonekedwe a sclerosis ya m'mapapo ndi mapangidwe kulephera kupuma. Akatswiri amanena pakuwonekera kwake kwa njira autoimmune.

tanthauzo

Hamman Olemera syndrome - matenda kugwirizana ndi ndondomeko pathological mu minofu m'mapapo, imbaenda sclerosis ake ndi chitukuko cha kulephera kupuma. Kuliphunzira linayamba mu zaka za m'ma otsiriza, pamene mabuku magazini sayansi kawirikawiri za mmene zizindikiro zofanana.

kudwala Izi ambiri mwa amuna a usinkhu wapakati ndi okalamba. Akukhulupirira kuti mwina chifukwa zizolowezi zoipa, kutalika yaitali utumiki ndi makhalidwe a kudzinenera zinthu zosafunika ntchito (owotcherera, Anthuwa, omanga ndi zina zotero. D.).

Mau ofanana

Dokotala amene anali woyamba kuphunzira kudwala anali Scadding. Iye anandiuza kuti liwu monga "fibrosing alveolitis." Koma posachedwapa linayamba kugwiritsidwa ntchito m'njira m'malo waukulu, kutanthauza gulu la matenda amene ali ndi zizindikiro zofanana. Choncho, anatenga kuposa dzina yeniyeni.

Kudwala anadzatchedwa matenda Scadding, kenako syndrome Hamman Olemera, monga kunapezeka kuti madokotala awiriwa tafotokozeramu kudwala ofanana mu 1935. Pali ofanana matanthauzo ena a pachikhalidwe izi nosological Mwachitsanzo, mwapang'onopang'ono interstitial m'mapapo mwanga fibrosis, fibrous dysplasia, matenda enaake, m'mapapo purulent interstitial chibayo ndi ena. Mu mankhwala amakono, amaloledwa kugwiritsa ntchito iwo onse.

etiology

syndrome Hamman Olemera Zatsala wina wa matenda a etiology bwanji, chifukwa chodabwitsa fibrosis kunachitika matenda ndi m'mapapo minofu nonspecific anachita ndondomeko pathological. Wotchuka kwambiri mu asayansi anali chiphunzitso chimene matenda amayamba ndi ndondomeko autoimmune zochokera thupi lawo siligwirizana ya mtundu wachitatu (immunocomplex).

Pali Gululi ndi chiphunzitso polyetiology. Iwo amanena kuti m'mapapo minofu poyera kuti wothandizila angapo bakiteriya, mafangasi kapena chikhalidwe tizilombo, imbaenda hyperergic zimene chitetezo cha m'thupi.

Pakuti asayansi ambiri ankaona kale-Olemera syndrome Hamm ngati chimake chimodzi cha collagenosis, momwe ndondomeko pathologic yochepa kuwala kokha.

pathogenesis

Pachimake interstitial pneumonitis (Hamman Olemera syndrome) limapezeka chifukwa mapangidwe otchedwa alveolar-capillary unit. Kuchokera momwe anasonyeza kusintha pathological zimadalira mlingo mayamwidwe mpweya mu magazi ndi, monga chotsatira cha hypoxia. Zimenezi silili matenda imeneyi, ndi mmene ziwalo kupuma poyankha choipa.

Kuchepetsa mayamwidwe mpweya kudzera alveoli kumachitika pa zifukwa zingapo. Choyamba, kusintha kwa Kakhungu makulidwe ndi partitions monga fibrinogen ali plasma, ndipo amalimbikitsa malo awa, kuwapanga kwambiri wandiweyani ndi okhwima. Kachiwiri epithelium mwake airway sacs (alveoli) mkati amakhala kiyubiki ndi wotaya katundu wake. Chachitatu, izo kusokonezedwa perfusion ndondomeko mipweya, mwachitsanzo mpweya wolowera mapapo, osati pokhudzana ndi mitsempha ya magazi a aliyense ndondomeko izi zimachitika mwa patalipatali. Komanso, pali kuphipha reflex ziwiya za m'mapapo chifukwa kuwonjezeka kuthamanga mu makope m'mapapo mwanga, zomwe zina pofuna kukwaniritsa zolinga ndi njira pathophysiological.

symptomatology

Hamman Olemera syndrome ana ali osowa. matenda makamaka amapezeka mu usinkhu wapakati ndi okalamba, koma akhale makanda msanga ndi mphamvu yafupika compensatory.

Isanayambike wozengereza matenda. Anthu, pali nkhani ya chifuwa youma ndi kumva zolimba mu chifuwa. Ndiye, wagwirizana ndi kufupika kwa mpweya ndi kutulutsa nyamuka pang'ono kutentha, kufooka, kutopa. Pa kuona koyamba kuoneka bwino mosavuta bluish ndi nasolabial makona. Mwina kusintha phalanges osachiritsika ndipo mawonekedwe misomali.

Pali inapita patsogolo kugunda, rales yaing'ono kuonekeratu mu mapapo. Ndi kupitirira matenda nkhani pafupipafupi wa kukhosomola, sputum zikuoneka, ndipo nthawi zina ndi hemoptysis (pamene zilipo capillary kusiyana).

diagnostics

Akakufunsani interstitial m'mapapo mwanga fibrosis (Hamman Olemera syndrome) angathe kuonedwa mwa mayesero zasayansi yekha mu magawo osatha, monga mwa kuyesa kwa magazi adzakhala kusintha sanali chindunji:

• kuwonjezeka mapuloteni;
• SSCHE mathamangitsidwe (erythrocyte sedimentation mlingo);
• kusintha mu chiŵerengero cha tuzigawo twa mapuloteni.

Chiwerengerochi akusonyeza pamaso kutupa, koma sanauze kulikonse kwa uthunthu wake, kapena malo. A pang'ono angathandize dongosolo radiography a m'mapapo. Mu masiteji koyamba pali chitsanzo kukula muzu m'mapapo, ndiye ukonde graininess M'mapapo minofu. M'kupita kwa nthawi, zithunzi kuoneka zigawo za kuchuluka chilungamo, zimafunika mawonekedwe a uchi. Mtima komanso kusinthidwa chifukwa cha kuchuluka kwa mtima chabwino.

matenda masiyanidwe

Hammam-Olemera matenda - matenda ovuta matenda ndendende chifukwa cha kusintha nonspecific mu mapapo. Pofuna molondola onetsetsani matenda, kuti achotse china chirichonse.

dokotala ayenera kudziyerekezera:

• poizoni ndi matupi awo sagwirizana alveolitis fibroziruschim;
• amgwirizano chibayo;
• sarcoidosis;
• mwiniwakeyo TB m'mapapo mwanga;
• zokhudza zonse matenda connective minofu (lupus, nyamakazi, matendawa, etc.);
• m'mapapo khansara;
• hemosiderosis m'mapapo mwanga;
• gulu la pneumoconiosis.

Only kukachitika kuti tonse mwa njira pamwamba anataya matenda syndrome Hammer-Olemera. zofunika kuchita postmortem Kupenda zambiri. Kuchita izi, dotolo n'kukulemberani lotseguka kapena chatsekedwa singano biopsy, malingana ndi ndondomeko anafuna matendawa.

kuvulazidwa thunthu

Zizindikiro ndi matenda Matendawa Hammam-Olemera zimadalira mmene kusintha padziko lonse minofu ya m'mapapo. Kuti asa- iwo, kuchotsa chidutswa cha m'mapapo ndi tichiphunzire pansi simungathe kutiona. Zotsatira za phunziroli zingasiyane ndi mawonetseredwe matenda, koma ndi zolondola kwambiri chifukwa amasonyeza mwachindunji boma thupi.

Ngati matenda ndi pansi fibrosing alveolitis, ndi dokotala wodziwa za vutoli amaona minofu kutupa madzi ndi okhutira mkulu wa Ulimbo mu alveolar lumen, impregnation partitions maselo yotupa ayankhe. Ngati ndondomeko pathological m'kati, ndi kusintha histologically chimaonetsa dongosolo selo ndi za indofeliyamu alveolar capillary chapansi Kakhungu thickening. N'zothekanso tsankho chiwonongeko cha alveoli mu malo kukhudzana ndi ziwiya.

Pali madigiri asanu kusintha mu mapapo:

1. Edema ndi kusalola ma makoma alveolar.
2. Madzimadzi nadzaza alveoli ndi maselo desquamated kapena mayamwidwe partitions exudate ndi chisindikizo.
3. Chiwonongeko cha alveoli, ndi mapangidwe cysts mu bronchi yaing'ono.
4. Angapo sing'anga ndi cysts yaing'ono.
5. "Ma" kuwala chifukwa cysts lalikulu.

Inde ndi madokotala ananena zawo

Kelloidoz (Hamman Olemera syndrome) ali ndi madokotala ananena zawo mwachilungamo osauka, chifukwa n'zosatheka kusiya kupitirira kwa matenda. Ngakhale mankhwala ndondomeko pathological akhoza kukhala odekha, koma posakhalitsa kapena mtsogolo matenda adakali apambane. The moleza mtima kwa miyezi ingapo kuti zaka zingapo moyo. Monga chifuniro exacerbation ndi nthawi chikhululukiro wosakhazikika pa nthawi imeneyi.

M'kupita kwa nthawi, vutoli mouzirira insufficiency amatsogolera anthu kwa kulumala, kenako anaphedwa ndi asphyxia. Mu nkhani zabwino, wodwalayo kukhala zaka zisanu ndi zisanu ndi ziwiri kuyambira nthawi ya matenda. Zoperekedwa kuti angavomereze malangizo onse a dokotala. Nthawi zina, mapeto patapita miyezi ingapo, kaya chithandizocho. Ngati mankhwala inyamuka kwa magawo oyambirira a matenda, mu nthawi zina n'zotheka "ndi" pa siteji iyi ndi kupulumutsa moyo wa munthu ndi thanzi wachibale.

chithandizo

Kodi kuchita kudwala, iye anapezeka ndi matenda Hammam-Wolemera? Chithandizo akuyamba yomweyo. Sikuti akuchedwa chiyambi cha mankhwala, monga matenda m'kati zaka zambiri. chithandizo Specific sanayambebe anayamba zimenezi madokotala anatiyangata zizindikiro ndi m'mbuyo liwiro minofu fibrosis.

Mu masiteji koyamba kwa matenda ndi zothandiza kwambiri mankhwala yasonyeza. Iwo kuchepetsa maonekedwe yotupa: exudation wa madzimadzi, kusalola ma, m'mbuyo mapangidwe minofu connective ndi kupanga chitetezo. Bolus mankhwala anachita, i.e. kuyamba ndi mlingo waukulu (mpaka 60-80 mg wa pa tsiku) ndipo pang'onopang'ono kuchepetsa mankhwala mpaka 20 mg. Nkofunika kuti aziona ntchito za kotekisi adrenal, kuti chifukwa syndrome Kushingoid.

Mu masiteji kenako, pamene mokwanira kutchulidwa fibrosis, zokonda zapatsidwa kwa mankhwala kumabwezera mmbuyo chitetezo cha m'thupi. yogawa Special analandira osakaniza mankhwala a mankhwala ozunguza bongo cytotoxic. Zinali kwambiri kuposa ntchito mankhwala payekha.

Yoikidwiratu monga symptomatic mankhwala decongestants, inhalation ya mpweya opanikizika, mavitamini B, komanso mankhwala ntchito ngati kuli kofunika (pamene kuika matenda sekondale), glycosides mtima ndi okodzetsa (ndi kuwonjezeka mu mtima kukanika).