Hemolytic kupererwa neropa-Chauffard Minkowski: kuongorora chirwere uye kurapwa

In kupererwa, ropa Chizenga pamwero anodonhedza nehasha. With chirwere ichi muupenyu hwavo vanotarisana yakawanda vanhu. Uyezve, hazvingaiti kunzvenga divi kunyange vana vaduku. nemarudzi Vazhinji chirwere zvinokonzerwa murombo zvokudya, kushaya mavhitaminzi, kana kutora zvimwe mishonga. Pashure kuchaitwa zvezviratidzo zvose afunge chinhu yakasvikawo. Pakati siyana pathological pezvakaitwa dzinounzwa imwe zvakaoma uye ngozi chaizvo. Izvi hemolytic kupererwa neropa-Chauffard Minkowski. Ndizvo nezvazvo ichakurukurwa yanhasi nyaya.

Chii kupererwa neropa?

Under kupererwa neropa unonyanyozivikanwa kunzwisiswa sezvo mamiriro muviri, dzinoratidzwa unopinza kuderera zviratidzi masero matsvuku eropa uye hemoglobin. Vamwe akasiyana hosha kunotungamirira kuchinja chimiro masero matsvuku eropa. Vanorasikirwa basa ravo zvikuru pamusoro nguva.

Kupererwa neropa kunowanzoguma siyana zvirwere, asi haana chikuru. Ndokusaka haufaniri kusiya pasina kuteerera nyonganyonga. Zvakafanira nekukurumidza kuwana chikonzero uye kuedza kubvisa.

Features hemolytic kupererwa neropa

Shoko rokuti "hemolytic kupererwa neropa" unosanganisira navanopinda boka zvirwere. Vose vari dzinoratidzwa zvakafanana pathogenesis. kuparadzwa masero matsvuku eropa yakawedzera kunokonzera kuwedzera zvavo manyowa zvinhu uye kuwedzera erythropoiesis. Loop inotungamirwa iri kwakakanganiswa masero matsvuku eropa. Pezvakaitwa kuparadzwa kwavo zvishoma nezvishoma kutanga zvipararire pamusoro zvigadziriswe pakati chidzoke uye kuibvisa.

haemolytic kupererwa neropa zvose conditionally mapoka mapoka maviri: nhaka uye akava. Munyaya ino, tine hwakadzama Kutarira pakutanga shanduro. Kuti kuva chaiwo, isu funga kuti ari nhaka kupererwa neropa-Chauffard Minkowski.

In zvokurapa mabhuku unogona kuwana mazita mashoma kuti kurondedzera pathological muitiro. Izvi microspherocytic kupererwa neropa uye nenhaka spherocytosis, uye zvirwere-Chauffard Minkowski. Zvinowanzoshandiswa rokupedzisira zita mazita masayendisiti, vawana.

The chirwere anoonekwa kuva zvakakurumbira zvikuru (1 muna 5 zviuru. Population ose). Zvinonzi chirwere kunyanya vanhu Northern Europe. Zviratidzo zvokutanga kunooneka vana vachiri vaduku. Kana iwe musatanga kurapa zvirwere nenguva netsika dzayo inokanganisa kushanda yose muviri.

Zvinokonzera mashandiro chirwere

Kupererwa Minkowski-Chauffard aine kuvhiringidza cellular membranes ose erythrocytes zvivako uye mabasa. Mugumisiro azvaiitwa, yochinja chimiro zvavo kuti dzeraundi makavharidzira, ave haungatani kuputsika. Zviratidzo zvokutanga hemolysis - kuparadzwa matsvuku masero pamwe mawa kusunungurwa hemoglobin.

Mune utano hwevanhu matsvuku masero ari chimiro chavo nowenyika munhu biconcave dhisiki, zvokuti kufamba takasununguka kuburikidza nhumbi. In kupererwa, ari Kamudzira zvinhu mapuroteni usage anonetseka. Izvi zvinotungamirira kuti kupinda pakati mvura kupinda masero. Nokuda kwechikonzero ichi, yochinja chimiro chavo. Achipfuura midziyo ropa, masero matsvuku eropa vane zvinorwadza akaremara, uye pashure penguva yakati vanotanga kuputsa. Tikafunga yaipfuurira urongwa, pachiyero masero matsvuku eropa anodonhedza zvinoshamisa, nekukudziridza hemolytic kupererwa neropa.

Kana mumwe mubereki zvamboshandiswa nechirwere chirwere ichi, hazvo achapfuura napo nhaka mwana. Very kashoma vana vanorwara vanoberekwa moms gwindiri uye vanotiza. Muchiitiko chino, kupererwa neropa hwapedyo tikafunga kuchinja DNA. The chikuru Gene mutation kunoitika kunyange panguva fetus. Chinodiwa kuti kukura chirwere ndiyo nezvainoita raamai muviri zvinhu zvinotevera:

• nemwaranzi, X-ray;
• kudhaka pamwe munyu inorema simbi, narcotics, chikakananda;
• utachiwana kurwisa.

Pasi pokudzora izvi zvingakonzera kwete chete kupererwa neropa-Chauffard Minkowski, asiwo zvikuru zvokurwara. Naizvozvo, panguva pamuviri, mumwe mukadzi anofanira kuedza kudzivirira muviri wako.

Zviratidzo zvokutanga

The mufananidzo zvekuchipatara dzinongoita pakutsvaga kwokuomerwa ari pathological kwacho uye uwandu dzakachinjwa erythrocytes. Zviratidzo ake okutanga anogona kuonekwa vana kuenda kukireshi uye mangwanani chikoro zera. Panguva iyi mhando kupererwa neropa kazhinji undulating. Nezvipusha hemolytic dambudziko kana paine vazhinji kuparadzwa mawa masero matsvuku eropa, achiteverwa nguva kunyarara. Panyaya iyi, zviratidzo dzingasiyana zvishoma.

Somuenzaniso, mashoko interictal nguva hosha kunoratidzwa zviratidzo kupererwa neropa. Pakati pavo pane pallor ganda, mucous membranes kana maziso. Kana hemolytic dambudziko kunotoda mufananidzo unogadzurudzirwa uye achabatsirwa anotevera zviratidzo:

1. Kuwedzera tembiricha kuti 38 madhigirii, nomusoro, zvakawanda zvive.
2. Kukura jaundice.
3. Marwadzo mudumbu, inozivikanwa spastic hunhu.
4. Kusagadzikana mune chiropa nokuda nezvibereko zvayo.
5. Kuzvimba chirwatata.

Nhaka hemolytic kupererwa neropa-Chauffard Minkowski akawana nedzevakuru. Zvikuru kakawanda Chikonzero kutsvaka kurapwa mune iyi ndiyo yellowness ganda. Zvisinei, kazhinji, chirwere chacho asymptomatic. Varwere kudzidza chokuvapo wayo netsaona uye kazhinji panguva purogiramu vaongo-.

Medical ongororo

Chirwere kuti kupererwa neropa-Chauffard Minkowski iri nyore. Kana uchifungidzira chirwere, uye rinowanikwa kwokutanga zviratidzo vanofanira kutsvaka zano zvokurapa. Zvokurwara of hematopoietic ino vari mukati kukwanisa hematologist. Aongorora zvichemo murwere nemhuri yake nhoroondo nyanzvi vanofanira kuzoongorora ganda uye sclera, zvidzidzo abdominal palpation. Rokumanikidzirwa basa ultrasonography chiropa uye chirwatata, sezvo chiratidzo chirwere chiri kuwedzera kukura kwemiviri izvi.

Panguva imwe chete hematologist anopa nhungamiro dzakawanda mumarabhoritari nemiedzo. Hemolytic kupererwa neropa-Chauffard Minkowski akasimbisa kuvapo zvinotevera kuchinja:

1. Weti: hemoglobinuria, vakawedzera mapuroteni zviratidzo uye urobilin.
2. Nemakemikari omuviri ropa: achidzikisa remafuta akawandisa, lactate dehydrogenase, kukura, kuwedzera kunanga bilirubin.
3. Kudzidza masero matsvuku eropa: reticulocytosis raidudzwa, zvinoderedza muchitokisi kukura, tikarega zvavo osmotic pakusimba.
4. General Kuongororwa ropa: ESR kumhanyisa, zvishomanana kuderera platelet uye leukocyte rutapudzo ruvara chakarurama.

Pakupedzisira asimbise rokutanga mhedziso kunobatsira mufambiro electrophoresis kwemapurotini of erythrocyte Kamudzira pachiimbwa quantitation yavo.

differential kuongorora chirwere

Hemolytic kupererwa neropa Minkowski-Chauffard vana dzimwe nguva anoita matambudziko panguva kuongorora chirwere. chirwere ichi zvakafanana nemamwe autoimmune pathologies zviratidzo. Naizvozvo, vanachiremba vanofanira kungwarira vamwe nevamwe uye tsika iyi mhando kupererwa neropa zviratidzo.

Chokutanga zvose tiri kutaura pamusoro majini kubatwa. Chete aakatipa kacho vabereki vose vari zvachose utano. Ukuwo, kuva mwana anorwara anogona kurondwa vaakasarudza kuchinja mapfupa dehenya. In zeza Muzviitiko anowedzera basa Coombs mharidzo. Kana nenyaya akaipa, murwere zvinotsigirwa kupererwa neropa-Chauffard Minkowski. Chirwere ari Ndizvo rakapera.

kuchinja mabatiro

Kupererwa neropa jekiseni vakasarudzwa nokuda kwayo zvakaoma. Munguva takanga tisisanakidzwi, sezvinoita mutemo, kupindira kunodiwa. Muvhiki kurwisa murwere muchipatara pakarepo.

Kuchinja Mabatirwo kupererwa neropa-Chauffard Minkowski inosanganisira zvinotevera matanho:

1. Replacement mushonga erythrocyte gobvu, kana ropa Chizenga pamwero anodonhedza kusvikira pachiyero 70 g / l.
2. Sealbumin zvinoratidzwa kunorapwa pamatunhu dziine bilirubin.
3. Nokuti detoxification akashandisa aizomupa mishonga.
4. Pasina zvakajeka hemolytic dambudziko inoratidza kugamuchirwa choleretic zvinodhaka.

Ya yokurapa kwakadaro, zvinodhaka chaizvo uye avo chipimo chemushonga - nyaya dzose idzi akatemwa nachiremba nomunhu.

kushanda kupindira

Kana microspherocytic hemolytic kupererwa neropa-Chauffard Minkowski nehasha, kuchinja kurapwa havagoni kukurira ravaida zvinangwa, murwere achaudzwa kuvhiyiwa kubvisa chirwatata. Akadaro nzira havagoni zvachose kurapa chirwere. Ukuwo, pashure kupindira uwandu aparadza masero matsvuku eropa yekudyidzana ipfupi uye miforo yavo upenyu yatinofema.

Hemolytic dambudziko avhiyiwa haina kudzokororwa, asi ine nhamba contraindications. Somuenzaniso, nokubviswa chirwatata haina varumbidzwa vana pasi 5 makore nokuda hunofa yakakwirira prices iri postoperative munguva. The Zvakashatira nzira anoonekwa kutapudzwa kurwisa muviri panyaya nemavhairasi uye fungal zvirwere.

An nzira musimboti ichaongororwa kubvisa chirwatata endovascular occlusion. Ndiyo imwe nzira yokurapa kubatsira rinowanzoshandiswa kutsvaka mune chirwere che "microspherocytic kupererwa neropa-Chauffard Minkowski." Munguva nzira, chiremba inoisa muMutumbi mumwe mushonga kuti chinokurwadzai kurwadza kwacho, uye chirwatata kunotungamirira chomwoyo. Vamwe Zvino anoponesa zvizere ropa rive uye asingauyi havachakwanisi kurwisa zvirwere.

zvinogoneka yematambudziko

kazhinji Hemolytic kupererwa neropa-Chauffard Minkowski kuenda kukireshi vana kunotungamirira pokumbononotswa hwepfungwa uye kwomuviri. Kunyanya kana vabereki kwenguva refu haana kutsvaka rubatsiro kana zano kuteerera chiremba wacho.

In varwere mukuru, vakawanda kuomesa ichaongororwa tikafunga gallstone chirwere kusagadzikana bilirubin muviri. The ndechokuti hemolytic dambudziko Kazhinji sezvo kutanga kukura jaundice, saka kurapwa kuri kunonoka. In pamberi matombo ari gallbladder inokurudzirwa kuti cholecystectomy pamwe splenectomy.

The for kupora

In zvishoma kwazvo chirwere uye nenguva mufambiro hwokushanda kubvisa chirwatata zvakanaka fungidziro. Kuregererwa Kazhinji kunoitika pakarepo mushure hemolytic dambudziko. Its nguva unogona siyana, asi kazhinji kacho anenge makore maviri.

nzira kudzivirira

Kupererwa Minkowski-Chauffard ndiyo nhaka. Naizvozvo kutadzisa panowanikwa chirwere hazvibviri. Kuitira kudzivisa chakakomba siyana hosha pavarwere nechirwere muchimiro kupererwa neropa Zvinokurudzirwa kuti nguva nenguva chaidzo zvizere rokuongorora panguva hematologist.

Kana kuronga pamuviri anofanira kunzwisiswa kuti mukana wokuti kukura hosha iri mwana asati aberekwa 50%. Saka achangobva rinoratidzawo kuramba zvichiongororwa nachiremba kuziva pathologies yekare kare yemagwaro.