Hemolytic anaemia-Chauffard Minkowski: nchoputa na ọgwụgwọ

Na anaemia, ọbara ọtụtụ hemoglobin tụlee sharply. Na ọrịa a n'oge ndụ ha nwere na ihu a ọtụtụ ndị. Ọzọkwa, ọ na-adịghị zere akuku ọbụna obere ụmụaka. Kasị iche nke ọrịa na-akpata ogbenye nri, enweghị vitamin, ma ọ bụ na-ewere ụfọdụ ọgwụ. Mgbe-ebibi ndị nile mgbaàmà akpali iche ihe na-akpata cropped. Otu n'ime ihe ndị dịgasị iche iche nke enweghị nchịkwa Filiks ekenyela otu siri ike ma na dị nnọọ ize ndụ. Nke a hemolytic anaemia-Chauffard Minkowski. Nke ahụ bụ banyere ya ga-atụle taa isiokwu.

Gịnị bụ anaemia?

N'okpuru anaemia ọtụtụ ghọtara dị ka ala nke ahụ, e ji a nkọ iju egosi nke mkpụrụ ndụ ọbara uhie na haemoglobin. Ụfọdụ iche nke ọrịa na-eduga ná mgbanwe ke udi nke mkpụrụ ndụ ọbara uhie. Ha ejighi ha bụ isi ọrụ karịrị oge.

Anaemia a na-esonyere a dịgasị iche iche nke ọrịa, ma ọ dịghị mgbe isi. Ọ bụ ya mere ị ga-ahapụ na-enweghị uche aghara. Ọ dị mkpa ka anya dị ka o kwere omume ga-ahụ na-akpata ma na-agbalị iji kpochapụ.

Atụmatụ hemolytic anaemia

Okwu ahụ bụ "hemolytic anaemia" na-agụnye a dịgasị otu ọrịa. All nke ha na-ji a nkịtị pathogenesis. Mụbara mbibi nke mkpụrụ ndụ ọbara uhie na-eme na-abawanye na ha decomposition ngwaahịa na-abawanye na erythropoiesis. Loop guzobere na-akwusila mkpụrụ ndụ ọbara uhie. Filiks nke mbibi ha na-eji nwayọọ nwayọọ amalite merie ndị usoro nke ntoputa na maturation.

All haemolytic anaemia dabere kewara abụọ dị iche iche: butere n'aka na enwetara. N'isiokwu a, anyị nwere ihe ọmụma zuru ezu anya na mbụ version. Na-kpọmkwem, anyị na-atụle na bụ a butere n'aka anaemia-Chauffard Minkowski.

Na ọgwụ akwụkwọ i nwere ike ịhụ a ole na ole aha na-akọwa enweghị nchịkwa usoro. Nke a microspherocytic anaemia na butere n'aka spherocytosis, na ọrịa na-Chauffard Minkowski. Ọtụtụ mgbe na-eji aha ikpeazụ site na aha ndị ọkà mmụta sayensị, achọpụta.

The ọrịa a na-atụle ga-nnọọ nkịtị (1 na 5 ọ bụla puku. Population). Ọ na-chọpụtara tumadi na ndị mmadụ nke Northern Europe. The mbụ ihe ịrịba ama pụtara ìhè na ụmụ na nwata. Ọ bụrụ na ị na-amalite na-emeso ọrịa a-adọ ha n'ụzọ nke ya ezighị ezi mmetụta na ịrụ ọrụ nke dum organism.

Ndị na-akpata na usoro nke ọrịa

Anaemia Minkowski-Chauffard esonyere nsogbu nke cellular membranes nke erythrocytes owuwu na ọrụ. The N'ihi nke a Filiks, ha na-agbanwe ha udi ka a okirikiri, na-aghọ kenkuwa. The mbụ ihe ịrịba ama nke hemolysis - mbibi nke mkpụrụ ndụ ọbara uhie na nke imekotaotuugbo ntọhapụ nke haemoglobin.

Na a ike mmadụ mkpụrụ ndụ ọbara uhie na ha udi reminiscent nke a biconcave disk, nke mere na ha akpali unu kpamkpam site na arịa. Na anaemia, ndị akpụkpọ ahụ, ndị nke protein njikọ a kpaghasịrị ya. Nke a na-eduga ná penetration nke liquids n'ime mkpụrụ ndụ. N'ihi nke a, ha na-agbanwe ha udi. Agafe ọbara arịa, mkpụrụ ndụ ọbara uhie na-idiọk nkwarụ, na mgbe a mgbe na-amalite imebi ala. Against ndabere nke na-aga n'ihu Filiks, larịị nke mkpụrụ ndụ ọbara uhie tụlee n'ụzọ dị ịrịba ama, na-emepe emepe hemolytic anaemia.

Ọ bụrụ na otu nne na nna mbụ e chọpụtara na ọrịa a, ọ ga-bụchaghị agafe nketa nwa. Nnọọ adịkarịghị na-arịa ọrịa na a mụrụ ụmụaka zuru oke mma Nne na Iruo. Na nke a, anaemia amalite megide ndabere nke mgbanwe ndị na-DNA. The isi site n'usoro mutation emee ọbụna n'oge nwa ebu n'afọ na mmepe. Mkpa maka mmepe nke ọrịa bụ mmetụta na nne ahụ na-esonụ ihe:

• radieshon, X-ìhè;
• igbu egbu na-arọ metal salts, ọgwụ ọjọọ, nicotine;
• virus agha.

N'okpuru nduzi nke ihe ndị a nwere ike ọ bụghị naanị na anaemia-Chauffard Minkowski, ma njọ daa ọrịa. Ya mere, n'oge ime, a nwaanyị kwesịrị-agbalị ichebe gị ahu.

The mbụ mgbaàmà

The adakarị picture na-n'ụzọ dị ukwuu kpebisiri ike site ogo-enweghị nchịkwa na usoro na ego nke gbanwetụrụ erythrocytes. Ya mbụ mgbaàmà pụrụ hụrụ na ụmụ ụlọ akwụkwọ ọta akara na n'oge ụlọ akwụkwọ afọ. N'oge a ụdị anaemia emekarị undulating. Ọdịdọ hemolytic nsogbu mgbe e nwere ọnụ ọgụgụ buru ibu nke imekotaotuugbo mbibi nke mkpụrụ ndụ ọbara uhie, ndị oge nke idere duu. Na nke a, ihe mgbaàmà nwere ike ịdị iche ubé.

Ka ihe atụ, interictal oge nke ọrịa na-egosipụta ihe ịrịba ama nke anaemia. Otu n'ime ha bụ pallor nke anụ ahụ, mucous membranes ma ọ bụ anya. Ọ bụrụ na hemolytic nsogbu adakarị picture na-gbanwetụrụ na na-esonyere ndị na-esonụ mgbaàmà:

1. Ịba okpomọkụ 38 degrees, isi ọwụwa, malaise.
2. Mmepe nke jaundice.
3. Mgbu na afo, ji spastic agwa.
4. Erughị ala na imeju ruru ya na-abawanye.
5. Mbufụt nke splin.

Butere n'aka hemolytic anaemia-Chauffard Minkowski hụrụ okenye. Ndị kasị Ugboro ihe mere na-achọ ọgwụ na ntị na nke a bụ yellowness nke anụ. Otú ọ dị, ọtụtụ mgbe, ọrịa bụ asymptomatic. Ọrịa na-amụta nke ya adị site ohere na-emekarị n'oge a na-eme nnyocha.

Medical ule

Diagnosis nke anaemia-Chauffard Minkowski bụ nnọọ mfe. Ọ bụrụ na ị na-enyo a ọrịa, na ọ na-egosi na mbụ mgbaàmà na-achọ ọgwụ ndụmọdụ. Pathology nke hematopoietic usoro bụ n'ime iru eru nke a hematologist. Mgbe ha nyochasịrị mkpesa nke onye ọrịa na ezinụlọ ya akụkọ ihe mere eme ọkachamara kwesịrị inyocha na-anụ na sclera, eduzi abdominal palpation. Manye kenyere ultrasonography nke imeju na-na splin, dị ka a mgbaàmà nke ọrịa bụ onye na-abawanye na size nke ndị a na ozu.

N'out oge hematologist enye ntụziaka a ọnụ ọgụgụ nke laabu ule. Hemolytic anaemia-Chauffard Minkowski enen ọnụnọ nke ndị na-esonụ mgbanwe:

1. Mmamịrị: hemoglobinuria, mụbara protein egosi na urobilin.
2. N'ihi mmiri ọgwụ ọbara: mbenata cholesterol, lactate dehydrogenase, ibu, na-abawanye na anọ bilirubin.
3. A na-amụ mkpụrụ ndụ ọbara uhie: reticulocytosis akpọ, Mbelata ke cell size, belata ha osmotic kwụsie ike.
4. General analysis of ọbara: ESR osooso, a nta ọnụ ke platelet na leukocyte Mbelata agba egosi.

N'ikpeazụ n'ịkwado mbido ọgwụgwụ enyere omume electrophoresis nke ndi na-edozi nke erythrocyte akpụkpọ ahụ na njikọ na ha quantitation.

esi nchoputa

Hemolytic anaemia Minkowski-Chauffard ụmụ mgbe ụfọdụ na-akpata ihe isi ike mgbe nchoputa. Nke a na ọrịa bụ yiri ọzọ autoimmune pathologies mgbaàmà. Ya mere, ndị dọkịta kwesịrị maara nke ụfọdụ nke pụrụ iche na e ji mara maka nke a ụdị anaemia mgbaàmà.

Mbụ niile anyị na-ekwu okwu banyere mkpụrụ ndụ ihe nketa pụrụ ịrịa. Naanị na ahụkebe ikpe ma nne ma nna na-nnọọ ahụ. N'aka nke ọzọ, nwere a na-arịa ọrịa na nwa ike deere a iche mgbanwe ke ọkpụkpụ nke okpokoro isi. Na echeghi ikpe Ọzọkwa kenyere Coombs sample. Ọ bụrụ na ndị analysis bụ na-ezighị ezi, ọrịa a na-akwado anaemia-Chauffard Minkowski. Diagnosis a na ugbu dechara.

Conservative ọgwụgwọ

Anaemia ọgwụ họọrọ n'ihi na nke ya ntaramahụhụ. N'oge lull, dị ka a na-achị, aka a chọrọ. N'oge ọzọ agha nke onye ọrịa n'ụlọ ọgwụ ozugbo.

Conservative ọgwụgwọ anaemia-Chauffard Minkowski agụnye ndị na-esonụ jikoro:

1. Nnọchi ọgwụ erythrocyte uka, ọ bụrụ na ọbara haemoglobin larịị tụlee ka larịị nke 70 g / l.
2. Albumin gosiri maka ọgwụgwọ nke elu udu nke bilirubin.
3. N'ihi na detoxification eji infusion ọgwụ.
4. Na enweghị awara awara hemolytic nsogbu egosi n'ikuku choleretic ọgwụ ọjọọ.

Na oge nke ndị dị otú ahụ ọgwụ, ụfọdụ ọgwụ ọjọọ na ha onunu ogwu - niile ndị a okwu na-kpebiri site dibịa na n'otu n'otu.

ịkpa aka

Ọ bụrụ na microspherocytic hemolytic anaemia-Chauffard Minkowski enwe oké ike, mgbanwe ọgwụgwọ ike ịnagide kwuru ebumnobi, onye ọrịa na-gwara ịwa ahụ iji wepụ splin. Otú ahụ nwere ike ọ bụghị kpamkpam gwọọ ndị ọrịa. N'aka nke ọzọ, mgbe ndị na aka nke ọnụ ọgụgụ nke-ebibi mkpụrụ ndụ ọbara uhie budata belata na-lengthened ha toruru na ndụ.

Hemolytic nsogbu mgbe ime ihe na-adịghị ugboro ugboro, ma ọ nwere ọtụtụ contraindications. Ka ihe atụ, mwepụ nke splin adịghị atụ aro maka ụmụaka n'okpuru 5 afọ ruru akwa enwe udu na postoperative oge. The downside nke usoro a na-ewere ka ibelata ahụ na-eguzogide na-akwanyere malitere ịrịa na fungal-efe efe.

Otu ọzọ n'onwe a na-ewere iji wepụ splin endovascular occlusion. Nke a bụ ihe ọzọ na usoro ọgwụgwọ na-enyere na-achọkarị na nchoputa nke "microspherocytic anaemia-Chauffard Minkowski." N'oge usoro, dọkịta inserts n'ime ahu nke a ọgwụ na-akpalite apịajighị apịaji, na splin na-eduga ná a obi ọgụ. Ụfọdụ n'ime ya mgbe azọpụta zuru ọbara ọkọnọ na anaghị ida ikike na-alụ ọgụ na-efe efe.

kwere omume nsogbu

Hemolytic anaemia-Chauffard Minkowski ụlọ akwụkwọ ọta akara ụmụaka na-akpatakarị igbu oge na uche na nke anụ development. Karịsịa ma ọ bụrụ na nne na nna maka a ogologo oge na-achọ ọgwụ na enyemaka ma ọ bụ leghara ihe dọkịta gwara.

Na okenye ọrịa, ọtụtụ nkịtị sikwuoro a na-ewere megide ndabere nke gallstone ọrịa anaghị ekwe nke bilirubin metabolism. The ihe bụ na hemolytic nsogbu mgbe hụrụ dị ka mmalite nke mmepe nke jaundice, otú kwesịrị ekwesị ọgwụgwọ a na-egbu oge. Na ọnụnọ nke nkume na gallbladder na-atụ aro na a cholecystectomy na splenectomy.

The amụma mgbake

Na obere oké ọrịa na-adọ omume nke na-arụ ọrụ iji wepụ splin mma prognosis. Ngbaghara na-emekarị emee ozugbo hemolytic nsogbu. Ya oge nwere ike ịdị iche iche, ma ọtụtụ mgbe bụ banyere afọ abụọ.

ụzọ gbochie

Anaemia Minkowski-Chauffard bụ butere n'aka. Ya mere gbochie omume nke ọrịa bụ ekwe omume. Iji gbochie oké forms nke ọrịa na ọrịa chọpụtara na a ụdị nke anaemia ọ na-atụ aro oge ata full nnyocha na a hematologist.

Mgbe atụmatụ a ime ga-aghọta na likelihood nke na-emepe emepe na ọrịa na nwa e bu n'afọ bụ 50%. Ya mere a mụrụ ọhụrụ na-egosikwa na-aga n'ihu nlekota site a dọkịta mata pathologies na nzọụkwụ mbụ.