Hemolytic anemia-Chauffard Minkowski: tātaritanga me te maimoatanga

I roto i te anemia, makaka koi te taumata toto pūtoto. Ki tenei mate puta noa to ratou oraraa i ki te kanohi i te rota o te iwi. Ano, e kore e karo i te reira taha noa tamariki iti. E meinga e kai rawakore, te kore o te huaora, tango etahi rongoā ranei te nuinga momo o te mate. I muri i te whakakorenga o ngā tohu katoa whakapataritari tauwehe Katohia. I roto i te momo o ngā tukanga pathological tohaina kotahi uaua, me te tino kino. Tenei anemia-Chauffard hemolytic Minkowski. Ko te pā ana ki ka e kōrero reira i roto i te tuhinga o tenei ra.

He aha te mea anemia?

I raro i anemia kei te matau rite te āhua o te tinana, āhuatanga e te tohu heke koi o ngā pūtau toto whero, me te pūtoto. Ētahi momo o te mate arata'i ki te huringa i roto i te āhua o ngā pūtau toto whero. ngaro ratou ratou mahi tuatahi i runga i te wā.

Kei te maha haere tahi anemia i te momo o mate, engari e kore tuatahi. Ko Ko te aha e kore e waiho e koe kahore whakarongo te whakararuraru,. E mea titauhia rite wawe tonu ki te kitea te take, me te ngana ki te whakakore.

Āhuatanga hemolytic anemia

Ko te wā "anemia hemolytic" ngā te whānui o mate rōpū. Kua āhuatanga katoa o ratou i te pathogenesis noa. ai te whakangaromanga o ngā pūtau toto whero nui haere te hua i roto i o ratou hua whāwehe me ki te hua i roto i te erythropoiesis. fakahohaasi Koromeke hanganga te pūtau toto whero. Kei te āta tīmata tukanga o ratou whakangaromanga ki taea i runga i nga huarahi o te putanga me te matuatanga.

wehea herenga anemia haemolytic katoa ki e rua ngā rōpū: tuku, me te riro. I roto i tenei tuhinga, to tatou he titiro āmiki i te putanga tuatahi. Hei kia pau, whakaaro tatou e ko te tuku anemia-Chauffard Minkowski.

I roto i te pukapuka tohutoro hauora ka taea e kitea e koe i te ingoa torutoru e whakaahua te tukanga pathological. Tenei anemia microspherocytic me spherocytosis tuku, me te mate-Chauffard Minkowski. Nuinga o ngā wā e whakamahia ana te ingoa whakamutunga e te ingoa o pūtaiao, discoverers.

whakaaro te mate te ki kia tino noa (1 i roto i nga 5 mano. Taupori). Kei te pāngia te reira i te nuinga i roto i te iwi o Northern Europe. E kitea i roto i ngā tamariki te tohu tuatahi i te wawe tau. Ki te kore koe e timata ki te hamani mate i roto i te wā mo tona pānga kino i runga i te mahi o te rauropi katoa.

Ko te take, me te tikanga o te mate

Anemia Minkowski-Chauffard tahi e te ngangau o kiriuhi pūtau o ngā hanganga erythrocytes me ngā mahi. Ko te hua o enei tukanga, huri ratou i to ratou āhua ki te porowhita, hei pakarukaru. Ko te tohu tuatahi o te hemolysis - te whakangaromanga o ngā pūtau toto whero ki te tuku tukutahi o pūtoto.

I roto i te hauora tangata pūtau toto whero i roto i te haamana'oraa o te kōpae biconcave, kia kia neke noa ratou i roto i te oko ratou āhua. I roto i te anemia, ohorere nga mea timatanga membrane o te kōtuitanga pūmua te. arata'i tenei ki, kătahi wai ki ngā pūtau. Hoki tenei take, huri ratou i to ratou āhua. E haere ana i te oko toto, he pūtau toto whero tino haka, a i muri i te ia timata ki te wahi i raro. Ki te whakamārama o ngā tukanga haere tonu, makaka te taumata o ngā pūtau toto whero tino, te whakawhanake anemia hemolytic.

Ki te mea kua mua i pāngia kotahi matua ki tenei mate, ka tika haere i te reira na roto i te tupu ki te tamaiti. Tino uaua whanau tamariki mate e mau mama maitai hauora me Dads. I roto i tenei take, whakawhanake anemia ki te whakamārama o ngā rerekētanga i roto i te DNA. puta te irakētanga ira tuatahi noa i roto i te whanaketanga fetal. Tikanga matua mō te whanaketanga o te mate ko te pānga i runga i te tinana o te whaea i te āhuatanga e whai ake nei:

• radiation, X-hihi;
• haurangi ki tai whakarewa taimaha, raau taero, nikotīni;
• whakaeke huaketo.

I raro i te mana o enei āhuatanga e kore kia anake anemia-Chauffard Minkowski, engari ano hoki māuiuitanga atu nui. Na reira, i roto i hapūtanga, kia tamata i te wahine ki te tiaki i to koutou tinana.

Ko te tohu tuatahi

Kei te nuinga o whakaritea te pikitia haumanu i te pakeke o te tukanga pathological me te nui o erythrocytes whakarerekē. Ka taea te kite ana tohu tuatahi i roto i ngā tamariki o te kōhungahunga, me te tīmatanga o tau kura. I roto i tenei momo o te anemia te tikanga tāwakawaka. Haurangi hemolytic raruraru ka reira te mea he maha nui o whakangaromanga tukutahi o ngā pūtau toto whero, aru e wā o puku. I roto i tenei take, kia rerekē te tohu paku.

Hei tauira, whakakitea te wā interictal o te mate e tohu o anemia. I roto ia ratou Ko te ataahua o te kiri, kiriuhi mucous kanohi ranei. Ki te whakarerekētia raruraru hemolytic pikitia haumanu te me e tahi e te tohu e whai ake nei:

1. Te whakanui ake i te pāmahana ki te 38 nga nekehanga, ānini, ahuatanga.
2. Ko te whanaketanga o te jaundice.
3. Mamae i roto i te puku, te āhuatanga pūāhua mate hukihuki.
4. Hūhi i roto i te ate e tika ana ki tona hua.
5. Mumura o te kōateate.

Tuku anemia hemolytic, kitea Chauffard Minkowski i roto i ngā pakeke. Ko te take tino auau mō rapu aro hauora i roto i tenei take ko te yellowness o te kiri. Heoi, i roto i te nuinga o te wā, he asymptomatic te mate. ako Tūroro o tona oraraa i te tupono noa, me te nuinga o te wā i roto i te tirohanga ngā.

whakamātautau Medical

Tātaritanga o te anemia-Chauffard Minkowski he tino ohie. Ki te whakapae koe he mate, a puta reira kia rapu i te tohu tuatahi tohutohu hauora. Māuiuitanga o te pūnaha hematopoietic he i roto i te mātau o te hematologist. I muri ui nga amuamu o te manawanui, me tona mātanga te aamu utuafare e tirotiro te kiri, me te mā karu, whakahaere palpation puku. Herea ultrasonography whakaritea o te ate, me te kōateate, rite he tohumate o te mate ko te hua i roto i te rahi o enei tinana.

Wä hematologist homai aronga ki te maha o ngā whakamātautau taiwhanga. whakapumautia Hemolytic anemia-Chauffard Minkowski te aroaro o te huringa e whai ake nei:

1. Mimi: hemoglobinuria, nui haere tohu pūmua me te urobilin.
2. toto Biochemistry: tukupu cholesterol, hua dehydrogenase lactate, te hua i roto i te bilirubin autaki.
3. He ako o ngā pūtau toto whero: reticulocytosis korerotia, whakaiti i roto i te rahi pūtau, raro ratou mau osmotic.
4. tātari General o te toto: ESR whakaterenga, he hekenga iti i roto i te mōtepe me leukocyte whakaiti tohu tae.

Hei whakamutunga whakau tauturu te mutunga tuatahi whakahaere electrophoresis o pūmua o te membrane ngeniwhero i roto i te taha ki to ratou quantitation.

tātaritanga pārōnaki

anemia Hemolytic Minkowski-Chauffard tamariki ētahi wā ai fifi i roto i te tātaritanga. He rite ki ētahi atu pathologies autoimmune tohu tenei mate. Na reira, kia mōhio o etahi o te motuhake me te āhuatanga mō tenei momo o tohu anemia kaiwhakaako.

Tuatahi o te katoa e korero tatou e pā ana ki te predisposition ira. Anake i roto i te wā rawa e tino hauora mātua e rua. I te tahi atu te ringa, kua taea te ata he tamaiti mate he huringa motuhake i roto i te wheua o te angaanga. I roto i te wā whakaaro ato'a whakaritea Coombs tauira. Ki te he kino te tātari, tautoko te manawanui te anemia-Chauffard Minkowski. kua oti te tātaritanga.

maimoatanga momo

Anemia therapy whiriwhiri no te mea o tona pakeke. I roto i te mariri, rite ki te tikanga, e hiahiatia ana wawaotanga te. I roto i te whakaekenga i muri o te manawanui falemahaki tonu.

maimoatanga Conservative Chauffard Minkowski-anemia ngā te mehua e whai ake nei:

1. Whakakapinga therapy ngeniwhero papatipu, ki te makaka te taumata toto pūtoto ki te taumata o te 70 g / l.
2. tohua albumin te mo te maimoatanga o reiti tiketike o bilirubin.
3. Hoki detoxification whakamahia e whaowhia therapy.
4. I roto i te ngaro o te raruraru hemolytic faaite whakaatu fariiraa taero choleretic.

Ko te roanga o taua therapy, nga taero motuhake me ratou inenga - e faaoti enei take katoa e te rata i runga i te pūtake takitahi.

wawaotanga operative

Ki te kia nui hemolytic microspherocytic anemia-Chauffard Minkowski, e kore e taea e te maimoatanga momo akakoromaki i te whāinga kī, tohutohu te manawanui te pokanga ki te tango i te kōateate. he huarahi taua he kore tino whakaora te mate. I te tahi atu te ringa, i muri i te wawaotanga o te maha o huna pūtau toto whero tino heke, ka roa to ratou huringa ora.

e kore te raruraru Hemolytic i muri i te mahi toutou, engari te mea he maha o contraindications. Hei tauira, e kore e tūtohu tango o te kōateate mō ngā tamariki i raro i te 5 tau e tika ana ki te auau te tahuti nei tiketike i roto i te wā postoperative. whakaaro te mate o te tukanga e ki te whakaiti ātete o te tinana ki te mate huaketo me harore.

whakaaro He rerekē ahua te ki te tango i te kōateate occlusion endovascular. Ko tenei te maha rapu tetahi tikanga o te maimoatanga ki te āwhina i roto i te tātaritanga o "anemia-Chauffard microspherocytic Minkowski." I roto i te tukanga, kōkuhu te tākuta ki te tinana o te tarukino e whakaoho mamae, me arata'i kōateate ki te whakaeke ngakau. Ētahi o reira ka whakaora i te supply toto tonu, me te kore e ngaro i te kaha ki te whawhai mate.

pōauautanga taea

maha arata'i Hemolytic anemia-Chauffard Minkowski kōhungahunga tamariki ki takaroa i roto i te whanaketanga hinengaro, me te tinana. Rawa, ki te kahore i nga matua mo te wa roa e rapu tauturu a te taote tohutohu tākuta o tukunoa'i ranei.

I roto i te tūroro pakeke, whakaaro te pōauautanga tino noa te ki te papamuri o disorders mate gallstone o bilirubin pākia. Ko te mea, ko te e te maha i te raruraru hemolytic kite rite te timatanga o te whanaketanga o te jaundice, na whakaroa maimoatanga tika te. I roto i te aroaro o kohatu i roto i te gallbladder tūtohu te e te cholecystectomy ki splenectomy.

Ko te matapae mō te whakaora

I roto i te mate iti kino, me te whakahaere wā o te mahi ki te tango i te kōateate waitohunga pai. te tikanga puta murunga tonu i muri i te raruraru hemolytic. Ka taea e tōna roanga rerekē, engari i roto i te nuinga o te wā, ko te pā ana ki e rua nga tau.

ara ki te aukati i

he tuku anemia Minkowski-Chauffard. Na reira ārai kore e taea te takanga o te mate. I roto i te tikanga ki te ārai i ngā momo nui o te mate i roto i te tūroro pāngia ki te ahua o te anemia e tūtohu ana ki te wā mamae te tirohanga tonu i te hematologist.

A, no te whakamahere i te hapūtanga kia matau e te tūponotanga o te whakawhanake i te mate i roto i te tamaiti e parauhia ko 50%. Na reira tamarii fanau whakaatu hoki aroturuki tonu i te tākuta ki te tāutu i ngā pathologies i te wā wawe.