Hemolytic anaemia-Chauffard Minkowski: ukuxilongwa kanye nokwelashwa

Ngo kwegazi, izinga igazi elithwala umoya-mpilo lehla kakhulu. Nalesi sifo ukuphila kwabo kufanele abhekane eziningi abantu. Ngaphezu kwalokho, lokho akusho ukugwema ohlangothini ngisho izingane ezincane. izinhlobonhlobo Iningi isifo zibangelwa ukudla okungenamsoco, ukuntuleka kwamavithamini, noma ukuthatha imithi ethile. Ngemva ukuqedwa kwazo zonke izimpawu ingqondo isici ukunqampunwa. Phakathi ezihlukahlukene izinqubo sokugembula labelwe omunye kunzima futhi kuyingozi kakhulu. Lokhu anaemia-Chauffard hemolytic Minkowski. Yilokho ngakho kuzoxoxwa ngakho esihlokweni lanamuhla.

Kuyini anaemia?

Ngaphansi anaemia elivame ukuqondwa ngayo njengoba isimo umzimba, elinesimo ukwehla izinkomba abukhali abomvu egazi ashaye futhi athwala umoya-mpilo. Ezinye izinhlobo zesifo kuholela ushintsho esime kwamangqamuzana egazi abomvu. Balahlekelwa umsebenzi wabo oyinhloko ngokuhamba kwesikhathi.

Isifo sokulahlekelwa yigazi uvame ukuhambisana ezihlukahlukene izifo, kodwa ungalokothi eyinhloko. Yingakho kufanele sebehamba ukunakwa sifo. Kuyadingeka ngokushesha ngangokunokwenzeka ukuze uthole imbangela futhi ukuzama ukuqeda.

Izici hemolytic anaemia

Igama elithi "hemolytic anaemia" kuhlanganisa iqembu ebanzi izifo. Bonke elinesimo pathogenesis ezivamile. ukubhujiswa Ukwandiswa kwamangqamuzana egazi abomvu kubangela ukwenyuka imikhiqizo yabo noqhekeko kanye nokwanda erythropoiesis. Loop kumiswa iphazamisekile amangqamuzana abomvu egazi. Izinqubo ngokubhujiswa kwabo kancane kancane ekuqaleni BUSA izindlela ukuvela kanye nokuvuthwa.

Zonke anaemia haemolytic kunemibandela ngamaqembu amabili: zenani elikhulu eziyifa kanye kuluthola. Kulesi sihloko, thina abe look eningiliziwe kweyesi-inguqulo yokuqala. Ukuze enembile, sicabanga ukuthi kuyinto lezinceku anaemia-Chauffard Minkowski.

Ngo yezokwelapha izincwadi ungathola amagama ambalwa achaza inqubo sokugembula. Lokhu anaemia microspherocytic futhi spherocytosis ngofuzo, futhi isifo-Chauffard Minkowski. Ngokuvamile wasebenzisa igama lokugcina ngamagama ososayensi, discoverers.

Lesi sifo ubhekwa evame kakhulu (1 kuzo zonke 5 izinkulungwane. Population). It sitholakale ikakhulukazi kubantu aseNyakatho Yurophu. Izimpawu zokuqala ezibonakala izingane zisencane. Uma ungaqali ukwelapha isifo ngendlela esifike ngesikhathi umthelela walo omubi ekusebenzeni wonke umzimba.

Izimbangela indlela lesi sifo

Isifo sokulahlekelwa yigazi Minkowski-Chauffard ephelezelwa ukuphazamiseka ulwelwesi yeselula zama-erythrocyte izakhiwo kanye nemisebenzi. Umphumela wale izinqubo, bayashintsha ukuma kwabo isekhula, abe buthakathaka. Izimpawu zokuqala hemolysis - ukubhujiswa abomvu egazi ashaye ukukhululwa ngesikhathi esisodwa-hemoglobin.

Ngendlela enempilo abomvu egazi lomuntu isesimweni yabo okusikhumbuza idiski biconcave, ukuze ukuhambisa ngokukhululekile ngokusebenzisa izitsha. Ngo kwegazi, izakhi ulwelwesi wamaprotheni zamagama kuyaphazamiseka. Lokhu kuholela kwema- uketshezi amaseli. Ngenxa yalesi sizathu, ashintsha ukuma kwabo. Kudlulwa imithambo yegazi, amaseli abomvu kanzima sikhubazekile, futhi ngemva kwesikhashana ziqala ukuyeka ukusebenza. Against isizinda izinqubo eziqhubekayo, ezingeni kwamangqamuzana egazi abomvu lwehla, ukuthuthukisa anaemia hemolytic.

Uma omunye umzali kwake kwatholakala nalesi sifo, kuyoba ngempela abadlula ifa nengane. Kakhulu akuvamile izingane ezigulayo zizalwa omama ephelele futhi obaba. Kulokhu, anaemia eba ngokumelene isizinda izinguquko DNA. Okuxhunyanwa isakhi sofuzo kuguquka kwenzeka ngisho phakathi kwengane esiswini. Okudingeka sokuthuthukiswa lesi sifo umthelela umzimba kamama lezi zinto ezilandelayo:

• ngemisebe, X-ray;
• ukudakwa nge heavy metal nosawoti, nezidakamizwa, i-nicotine;
• igciwane ukuhlaselwa.

Ngaphansi kwethonya lezi zici akufanele zokugula lubucayi kakhudlwana kuphela anaemia-Chauffard Minkowski, kodwa futhi. Ngakho-ke, ngesikhathi ekhulelwe, owesifazane kufanele azame ukuvikela umzimba wakho.

Izimpawu zokuqala

Isithombe emitholampilo buvezwa kobukhulu inqubo sokugembula kanye nenani erythrocyte ukushintshwa. izimpawu yakhe yokuqala kungabonakala nasemkhakheni abantwana zasenkulisa kanye ekuqaleni abasesikoleni. Ngalesi uhlobo anaemia ngokuvamile inamagquma. Ukuquleka hemolytic esibucayi uma kukhona inqwaba ekubhujisweni ngesikhathi esisodwa abomvu egazi, kulandele cwaka. Kulokhu, izimpawu ingase ihluke kancane.

Ngokwesibonelo, isikhathi interictal lesi sifo kubonakala izimpawu igazi. Phakathi kwazo kunabafundi pallor kwesikhumba, nolwelwesi lwamafinyila noma amehlo. Uma kunezinkinga hemolytic isithombe emtholampilo beshintsha futhi kuhambisana zimpawu ezilandelayo:

1. Ukwandisa izinga lokushisa degrees 38, ikhanda elibuhlungu, umkhuhlane.
2. Ukuthuthukiswa jaundice.
3. Ubuhlungu esiswini, ebonakala uhlamvu spastic.
4. Ukungakhululeki esibindini ngenxa izithelo zalo.
5. Ukuvuvukala ubende.

Ukubambezeleka hemolytic anaemia-Chauffard Minkowski etholakala kwabadala. Isizathu engicasula kakhulu ngokufuna usizo lodokotela kulokhu iyona yellowness kwesikhumba. Nokho, ezimweni eziningi, lesi sifo singumbulali asymptomatic. Iziguli ufunde khona ngengozi futhi ngokuvamile ngesikhathi kuhlolwa isimiso.

ngihlolwa

Ukuxilongwa anaemia-Chauffard Minkowski ulula. Uma usola isifo, futhi kubonakala izimpawu kokuqala kufanele afune usizo lodokotela. Pathology kohlelo hematopoietic kukhona ngaphansi kwekhono a eyisazi. Ngemva kokuhlola izikhalazo isiguli nomkhaya wakhe umlando Uchwepheshe kufanele ahlole isikhumba kanye sclera, ukuqhuba palpation besisu. Ayimpoqo ultrasonography ayabelwe sesibindi ubende, njengoba uphawu lesi sifo nokwanda ubukhulu kulezi zinhlangano.

Kanyekanye eyisazi unikeza isiqondiso eziningi zocwaningo. Hemolytic anaemia-Chauffard Minkowski waqinisekisa khona izinguquko ezilandelayo:

1. Umchamo: hemoglobinuria, kukhuphuke amaprotheni izinkomba kanye urobilin.
2. Biochemistry igazi: ekunciphiseni cholesterol, dehydrogenase lactate, ukukhula, ukwanda bilirubin engesobala.
3. Ucwaningo kwamangqamuzana egazi abomvu: reticulocytosis evelele, ukuncishiswa ngobukhulu cell, yehlisa azinze osmotic.
4. ukuhlaziywa Jikelele wegazi: ESR ukushesha, ukwehla okuncane platelet ne leukocyte ukunciphisa umbala inkomba.

Ekugcineni uqinisekise esiphethweni kwangaphambilini kusiza ukuziphatha electrophoresis amaprotheni we ulwelwesi erythrocyte ngokuhlanganyela quantitation yabo.

ukuxilongwa umehluko

Ngezinye izikhathi i-anemiya Hemolytic Minkowski-Chauffard izingane ukubangela izinkinga ngesikhathi uphethwe. Lesi sifo kuyafana nezinye pathologies sokuhlasela kwamasosha omzimba izimpawu. Ngakho-ke, odokotela kufanele bazazi ezinye ehlukile futhi isici kule uhlobo izimpawu igazi.

Okokuqala sikhuluma yofuzo. Kuphela ezimweni ezehlukile kokubili abazali engumqemane. Ngakolunye uhlangothi, abe ingane egulayo usukela ushintsho letihlukile amathambo Yekhanda. Ezimweni ngabazeka ngaphezu kwalokho babelwe Coombs isampula. Uma ukuhlaziywa alivumi, isiguli kusekelwa anaemia-Chauffard Minkowski. Sifo ngalokhu igcwaliswe.

ukwelashwa okulondoloza

ukwelashwa anemia abakhethiwe ngenxa namandla aso. Phakathi ukusikhohlisa, njengoba umthetho, kokungenela liyadingeka. Ngesikhathi kuhlaselwa esilandelayo isiguli esibhedlela ngokushesha.

ukuphathwa okulondoloza igazi-Chauffard Minkowski ihlanganisa izinyathelo ezilandelayo:

1. replacement therapy erythrocyte mass, uma izinga igazi elithwala umoya-mpilo lehla ezingeni 70 g / l.
2. Albumin kuboniswa ukwelashwa yamazinga aphezulu okungabi bilirubin.
3. Ukuze ngokukhishwa kobuthi egazini esetshenziswa yokwelapha ukumnika.
4. In the ukungabikho express crisis hemolytic sibonisa reception izidakamizwa choleretic.

Isikhathi yokwelapha esinjalo, izidakamizwa ethize kanye umthamo wabo - konke lezi zinto ezinqunywa udokotela umuntu ngamunye.

kokungenela op

Uma hemolytic microspherocytic anaemia-Chauffard Minkowski ibe mibi kakhulu njengenkemba, ukwelashwa ngoludala akakwazi ukubhekana izinhloso kushiwo, isiguli wasikisela ukuba ngihlinzwe kukhishwe ubende. indlela enjalo akakwazi ukwelapha ngokuphelele ukugula. Ngakolunye uhlangothi, ngemva ukungenela inani wabhubhisa amangqamuzana egazi abomvu kakhulu ziyancipha futhi yinde umjikeleto wemphilo yabo.

Inkinga Hemolytic emva msebenzi okuphindaphindiwe, kodwa enezinto eziningi Izimo. Ngokwesibonelo, ukususwa ubende is hhayi Kunconywa izingane ezingaphansi kweminyaka 5 ngenxa okusezingeni eliphezulu lokushona kwabantwana bezalwa esikhathini postoperative. Yini embi ngalo inqubo ubhekwa wehlise ukumelana umzimba maqondana izifo ezibangelwa amagciwane kanye fungal.

I samuntu okunye kubhekwa ukususa ubende occlusion endovascular. Lena enye indlela yokwelashwa ukusiza ngokuvamile khonga ngaphakathi the ukuxilongwa "anaemia-Chauffard microspherocytic Minkowski." Ngesikhathi inqubo, udokotela ifaka emzimbeni umuthi kuphakamisa zibe, futhi ubende kuholela ngemuva kokuhlaselwa yisifo senhliziyo. Abanye ke silondoloza liningi igazi okugcwele akaphelelwa ikhono ukulwa nezifo.

yezinkinga kungenzeka

Hemolytic anaemia-Chauffard Minkowski izingane zasenkulisa ngokuvamile kuholela ukubambezeleka ekuthuthukisweni ngokwengqondo nangokomzimba. Ikakhulukazi uma abazali isikhathi eside akazange afune usizo lwezokwelapha noma iseluleko indiva kadokotela.

In iziguli omdala, izinkinga ezivamile kubhekwa ngokumelene isizinda isifo gallstone iziyaluyalu bilirubin umzimba. Into wukuthi ngokuvamile obumbi hemolytic ubonakala ekuqaleni ukuthuthukiswa jaundice, ngakho ukwelashwa okufanelekile imisiwe. Ephambi kwezinduna amatshe e-gallbladder Kunconywa ukuthi cholecystectomy nge splenectomy.

Isibikezelo ukutakula

Ngo isifo kancane ezinzima futhi ukuziphatha esifike ukusebenza ukususa ubende Izibikezelo evumayo. Ukuthethelelwa ngokuvamile kwenzeka ngokushesha ngemva kokuba Inkinga hemolytic. ubude bayo ziyahlukahluka, kodwa ezimweni eziningi kuba iminyaka ecishe ibe mibili.

izindlela zokunqanda

Isifo sokulahlekelwa yigazi Minkowski-Chauffard omkhulu sitholwa njengefa. Ngakho-ke ukuvimbela okuvela kuyo isifo akunakwenzeka. Ukuze uvimbele amafomu okunzima sifo iziguli unesifo uhlobo anaemia kunconywa ukuthi ngezikhathi ezithile ibhekane ukuhlolwa okugcwele ngesikhathi eyisazi.

Uma uhlela ukukhulelwa Kufanele kwaziwe kodwa ukuthi amathuba asathuthuka lesi sifo umntwana ongakazalwa ungumuntu 50%. Ngakho-ke ewusana olusanda kuzalwa futhi libonisa nokuqapha okuqhubekayo okwenziwa udokotela ukuhlonza pathologies ngendlela efanele usaqala.