Hemolytic anemia-Chauffard Minkowski: mob thiab kev kho mob

Nyob rau hauv anemia, cov ntshav hemoglobin theem dauv sharply. Nrog tus kab mob no thoob plaws hauv lawv lub neej muaj mus fim ib tug ntau ntawm cov neeg. Ntxiv mus, nws tsis txhob sab txawm me me cov me nyuam. Feem ntau cov ntau ntau yam ntawm tus kab mob yog tshwm sim los ntawm cov neeg pluag noj cov zaub mov, tsis muaj cov vitamins, los yog noj tej yam tshuaj. Tom qab tshem tawm ntawm tag nrho cov tsos mob provoking tau cropped. Ntawm ntau yam ntawm pathological dab faib ib tsis yooj yim thiab heev txaus ntshai. Qhov no hemolytic anemia-Chauffard Minkowski. Uas yog hais txog nws yuav tau los sib tham nyob rau hauv hnub no tus tsab xov xwm.

Yuav ua li cas yog anemia?

Nyob rau hauv anemia yog feem ntau to taub raws li hauv lub xeev ntawm lub cev, yus muaj los ntawm ib tug ntse poob ntsuas cov ntshav liab thiab qe ntshav liab. Ib txhia ntau ntau yam ntawm tus kab mob ua rau yus qhov kev hloov nyob rau hauv qhov zoo ntawm cov ntshav liab. Lawv tsis tau lawv thawj muaj nuj nqi thaum lub sij hawm.

Anemia yog feem ntau nrog los ntawm ib tug ntau yam ntawm cov kab mob, tab sis yeej tsis thawj. Uas yog vim li cas koj yuav tsum tsis txhob tawm tsis paub tsis meej. Nws yog tsim nyog kom sai li sai li sai tau mus nrhiav qhov ua rau thiab ua kom tshem tawm.

Nta hemolytic anemia

Lub sij hawm "hemolytic anemia" xws li muaj ib tug ntau pab pawg neeg ntawm cov kab mob. Tag nrho cov ntawm lawv yog yus muaj los ntawm ib qho pathogenesis. Nce kev puas tsuaj ntawm cov ntshav liab ua rau ib qho kev nce rau hauv lawv cov decomposition khoom thiab mus rau ib qho kev nce rau hauv erythropoiesis. Loop tsim yog cuam tshuam cov ntshav liab. Dab ntawm lawv kev puas tsuaj yog maj pib yeej tshaj lub mechanisms ntawm rov tshwm sim thiab maturation.

Tag nrho haemolytic anemia conditionally muab faib ua ob pawg: raws roj ntsha thiab mas yuav kis tau. Nyob rau hauv no tsab xov xwm, peb muaj ib tug ncauj lus kom ntxaws saib tus thawj version. Yuav kom leej, peb xav tias yog ib tug hereditary anemia-Chauffard Minkowski.

Nyob rau hauv kev kho mob reference cov phau ntawv koj yuav nrhiav tau ib tug ob peb npe uas piav txog cov pathological txheej txheem. Qhov no microspherocytic anemia thiab raws roj ntsha spherocytosis, thiab tus kab mob-Chauffard Minkowski. Feem ntau cov feem ntau siv lub npe los ntawm lub npe ntawm kws tshawb fawb, discoverers.

Tus kab mob yog pom tias yuav muaj coob (1 nyob rau hauv txhua txhua 5 txhiab. Pejxeem). Nws thiaj paub tias yog mas nyob rau hauv cov neeg ntawm sab qaum teb teb chaws Europe. Cov thawj cov cim qhia yog tshwm nyob rau hauv cov me nyuam nyob rau ntawm ib thaum ntxov lub hnub nyoog. Yog hais tias koj tsis pib kho tus kab mob nyob rau hauv ib tug raws sijhawm rau tej yam tsis zoo nyob rau hauv cov hauj lwm ntawm tag nrho cov kab mob.

Cov ua thiab mechanism ntawm tus kab mob

Anemia Minkowski-Chauffard nrog disturbance ntawm cellular daim nyias nyias ntawm erythrocytes lug thiab kev khiav dej num. Cov tshwm sim ntawm cov dab, lawv hloov lawv zoo mus rau ib tug yeej, ua nkig. Cov thawj cov cim qhia ntawm hemolysis - kev puas tsuaj ntawm cov ntshav liab nrog lub simultaneous kev tso tawm ntawm hemoglobin.

Nyob rau hauv ib tug noj qab nyob zoo human cov ntshav liab nyob rau hauv lawv cov zoo reminiscent ntawm ib tug biconcave disk, yog li ntawd lawv tsiv mus nyob dawb do los ntawm cov hlab ntsha. Nyob rau hauv anemia, daim nyias nyias ntsiab ntawm cov protein synthesis yog ntxhov. Qhov no ua rau cov allergic kua rau hauv lub hlwb. Vim li no, lawv hloov lawv zoo lawm. Dhau los ntawm cov hlab ntsha, cov ntshav liab muaj mob deformed, thiab tom qab ib tug thaum pib mus ua txhaum down. Tawm tsam tom qab ntawm kev tu ncua dab, cov theem ntawm cov ntshav liab dauv dramatically, tsim hemolytic anemia.

Yog hais tias ib tug niam txiv tau yav tas los lawm thiaj paub hais tias nrog tus kab mob no, nws yuav tas kis tau los ntawm qub txeeg qub teg rau tus me nyuam. Heev tsis tshua muaj mob cov me nyuam yug los txig noj qab nyob zoo niam thiab dads. Nyob rau hauv cov ntaub ntawv no, anemia npaj tiv thaiv lub keeb kwm ntawm kev hloov nyob rau hauv lub DNA. Lub hom noob hloov tshwm sim txawm thaum lub sij hawm me nyuam hauv plab kev loj hlob. Yuavtsum tau kawm uantej rau txoj kev loj hlob ntawm tus kab mob yog tej yam nyob rau hauv leej niam lub cev yam nram qab no:

• tawg, X-rays;
• intoxication nrog hnyav hlau salts, narcotics, nicotine;
• tus kab mob no nres.

Nyob rau hauv tus ntawm cov yam tseem ceeb yuav tsis tau tsuas yog anemia-Chauffard Minkowski, tab sis kuj loj pathology. Yog li ntawd, thaum lub sij hawm cev xeeb tub, ib tug poj niam yuav tsum tau sim los tiv thaiv koj lub cev.

Tus thawj cov tsos mob

Cov soj ntsuam daim duab yog lom zem ntau los ntawm tus heev ntawm lub pathological txheej txheem thiab tus nqi ntawm hloov erythrocytes. Nws thawj thawj cov tsos mob yuav tsum tau cai nyob rau hauv cov me nyuam ntawm preschool thiab thaum ntxov lub tsev kawm ntawv uas muaj hnub nyoog. Thaum lub sij hawm no hom ntawm anemia feem ntau undulating. Qaug dab peg hemolytic ntsoog thaum muaj ib tug loj tus naj npawb ntawm simultaneous kev puas tsuaj ntawm cov ntshav liab, raws li los ntawm lub sij hawm ntawm silence. Nyob rau hauv cov ntaub ntawv no, cov tsos mob tej zaum yuav txawv me ntsis.

Piv txwv li, lub interictal lub sij hawm ntawm tus kab mob yog manifested tej yam tshwm sim ntawm anemia. Ntawm lawv yog cov lub pallor ntawm daim tawv nqaij, txheej week los yog ob lub qhov muag. Yog hais tias hemolytic ntsoog soj ntsuam daim duab yog hloov thiab yog nrog los ntawm cov nram qab no cov tsos mob:

1. Ua lub kub rau 38 degrees, mob taub hau, tsis xis nyob.
2. Cov kev loj hlob ntawm daj ntseg.
3. Mob nyob rau hauv lub plab mog, yus spastic cim.
4. Tsis xis nyob rau hauv lub siab vim nws nce.
5. O ntawm tus po.

Mob muaj keeb hemolytic anemia-Chauffard Minkowski pom nyob rau hauv cov neeg laus. Qhov feem ntau heev yog vim li cas rau nrhiav kev kho mob nyob rau hauv cov ntaub ntawv no yog lub yellowness ntawm daim tawv nqaij. Txawm li cas los, nyob rau hauv Feem ntau, tus kab mob no yog asymptomatic. Cov neeg mob kawm txog nws lub neej los ntawm lub caij thiab feem ntau thaum lub sij hawm ib tug niaj hnub soj ntsuam.

kev xeem

Mob ntawm anemia-Chauffard Minkowski yog heev yooj yim. Yog hais tias koj xav tias ib tug kab mob, thiab nws zoo nkaus li thawj zaug tsos mob yuav tsum nrhiav kev kho mob. Pathology ntawm lub hematopoietic system yog nyob rau hauv lub rab peev xwm ntawm ib tug hematologist. Tom qab tshuaj ntsuam kev tsis txaus siab ntawm tus neeg mob thiab nws tsev neeg zaj keeb puam yuav tsum tshawb xyuas ntawm daim tawv nqaij thiab lub sclera, kev mob plab palpation. Yuav tsum muab ultrasonography ntawm daim siab thiab tus po, raws li ib tug cov tsos mob ntawm tus kab mob no yog ib qho kev nce nyob rau hauv lub loj ntawm cov lub cev.

Ib txhij hematologist coj mus rau ib tug xov tooj ntawm laboratory kev ntsuam xyuas. Hemolytic anemia-Chauffard Minkowski paub tseeb hais tias muaj cov nram qab no hloov:

1. Cov Kev Kuaj Zis: hemoglobinuria, nce protein ntsuas thiab urobilin.
2. Biochemistry cov ntshav: txo cov roj cholesterol, lactate dehydrogenase, kev loj hlob, nce nyob rau hauv indirect bilirubin.
3. Ib txoj kev tshawb ntawm cov ntshav liab: reticulocytosis pronounced, yuav txo tau nyob rau hauv cell loj, me, txo lawv cov osmotic stability.
4. General tsom xam ntawm cov ntshav: ESR acceleration, ib tug me ntsis txo nyob rau hauv platelet thiab leukocyte yuav txo tau cov xim qhia.

Thaum kawg paub meej tias cov ua ntej xaus pab coj electrophoresis ntawm cov nqaijrog ntawm lub erythrocyte membrane nyob rau hauv nrog lawv quantitation.

differential mob

Hemolytic anemia Minkowski-Chauffard cov me nyuam tej zaum yuav ua rau teeb meem thaum lub sij hawm kuaj mob. Tus kab mob no yog zoo li lwm yam autoimmune pathologies cov tsos mob. Yog li ntawd, cov kws kho mob yuav tsum tau paub txog ib co ntawm cov yus thiab yam ntxwv rau no hom ntawm anemia cov tsos mob.

Ua ntej ntawm tag nrho cov peb tham txog ib tug kev tshuaj ntsuam genetic predisposition. Tsuas yog nyob rau hauv exceptional mob leej niam leej txiv yog kiag li noj qab nyob zoo. Nyob rau lwm cov tes, muaj ib tug me nyuam mob yuav tau twb tsis siv ib cov hloov nyob rau hauv cov pob txha ntawm cov pob txha taub hau. Nyob rau hauv doubtful mob ntxiv muab Coombs qauv. Yog hais tias tus tsom xam yog tsis zoo, tus neeg mob yog txaus siab anemia-Chauffard Minkowski. Kuaj cog lus ua kom tiav.

conservative kev kho mob

Anemia txoj kev kho xaiv vim hais tias ntawm nws cov heev. Thaum lub sij hawm ntxias, raws li ib tug txoj cai, kev pab yog yuav tsum tau. Thaum lub sij hawm tom ntej no nres ntawm tus neeg mob pw tsev kho mob tam sim ntawd.

Conservative kev kho mob ntawm anemia-Chauffard Minkowski muaj xws li cov nram qab no:

1. Hloov txoj kev kho erythrocyte loj, yog tias cov ntshav hemoglobin theem dauv mus rau theem ntawm 70 g / l.
2. Albumin yog qhia rau kev kho mob ntawm muaj cov bilirubin.
3. Rau detoxification siv Txoj kev lis ntshav txoj kev kho.
4. Nyob rau hauv lub qhaj ntawv ntawm express hemolytic ntsoog tso txais tos choleretic tshuaj.

Lub caij ntawm xws txoj kev kho, cov kev tshuaj thiab lawv ntau npaum - tag nrho cov teeb meem no yog txiav txim siab los ntawm tus kws kho mob rau ib tug neeg zus.

hauj lwm kev pab

Yog hais tias microspherocytic hemolytic anemia-Chauffard Minkowski ua tau mob hnyav, conservative kev kho mob yuav tsis tau tiv nrog lub teev cov hom phiaj, tus neeg mob yuav tsum tau kev phais kom tshem tawm tus po. Xws li ib tug mus kom ze tsis tau kiag li kho tau tus mob. Nyob rau lwm cov tes, tom qab cov kev pab ntawm tus xov tooj ntawm kev puas tsuaj ntshav liab ho txo thiab lengthened lawv lub neej voj voog.

Hemolytic ntsoog tom qab lub lag luam yog tsis dua, tiam sis nws muaj ib tug xov tooj ntawm contraindications. Piv txwv li, tshem tawm ntawm tus po yog tsis pom zoo rau cov me nyuam nyob rau hauv 5 xyoo vim lub siab lub neej no nqi nyob rau hauv lub postoperative lub sij hawm. Lub downside ntawm cov txheej txheem yog pom tias yuav txo tau lub cev kuj muaj respect rau tus kab mob thiab fungal kab mob.

Ib lwm embodiment yog pom tias yuav tshem tawm cov po endovascular occlusion. Qhov no yog lwm txoj kev kho mob los pab yog feem ntau nrhiav nyob rau hauv lub mob ntawm "microspherocytic anemia-Chauffard Minkowski." Thaum lub sij hawm txoj kev, tus kws kho mob inserts mus rau hauv lub cev ntawm ib tug tshuaj uas provokes chua, thiab tus po ua tau kom lub plawv nres. Ib txhia ntawm nws ces yam tag nrho cov ntshav mov thiab tsis poob lub peev xwm mus tua tus kab mob.

tau teeb meem

Hemolytic anemia-Chauffard Minkowski preschool cov me nyuam feem ntau ua rau zoo nyob rau hauv kev puas hlwb thiab lub cev txoj kev loj hlob. Tshwj xeeb tshaj yog yog hais tias tus niam txiv rau ib ntev lub sij hawm tsis nrhiav kev pab kho mob los sis tsis tus kws kho mob lub tswv yim.

Nyob rau hauv neeg laus cov neeg mob, feem ntau yam tab kaum yog suav tias yog tiv thaiv lub keeb kwm ntawm gallstone tus kab mob mob ntawm bilirubin metabolism. Qhov no yog hais tias lub hemolytic teeb meem yog feem ntau pom raws li qhov pib ntawm txoj kev loj hlob ntawm daj ntseg, yog li kom kev kho mob yog ncua. Nyob rau hauv lub xub ntiag ntawm lub pob zeb nyob rau hauv lub gallbladder yog pom zoo hais tias ib tug cholecystectomy nrog splenectomy.

Cov kev twv rau rov qab

Nyob rau hauv tsis mob hnyav kab mob thiab raws sij hawm coj ntawm lub lag luam kom tshem tawm cov po paaj raug. Kev so ib pliag feem ntau tshwm sim tam sim ntawd tom qab lub hemolytic ntsoog. Nws ntev yuav sib txawv, tab sis nyob rau hauv feem ntau yog hais txog ob lub xyoos.

txoj kev los mus tiv thaiv

Anemia Minkowski-Chauffard yog raws roj ntsha. Yog li ntawd tiv thaiv kom txhob muaj tshwm sim ntawm tus kab mob yog tsis tau. Nyob rau hauv thiaj li yuav tiv thaiv kom txhob mob loj heev ntaub ntawv ntawm tus kab mob nyob rau hauv cov neeg mob thiaj paub hais tias nrog ib daim ntawv ntawm anemia nws yog pom zoo kom tseg yauv tag nrho kev soj ntsuam ntawm ib tug hematologist.

Thaum npaj ib tug cev xeeb tub yuav tsum tau to taub hais tias qhov yuav ua rau kev tsim tus kab mob nyob rau hauv lub me nyuam hauv plab yog 50%. Yog li ntawd mos liab kuj qhia tau hais tias tas mus li xyuas los ntawm ib tug kws kho mob kom paub tias pathologies nyob rau ntawm ib thaum ntxov rau theem.