Hereditary spherocytosis: zvinokonzera, zviratidzo uye kurapwa

Zvemukati

• Chii chinokonzera spherocytosis
• Zviratidzo zvikuru
• Kurapwa kunoitwa sei

Hereditary spherocytosis chirwere chemajini chinoratidzwa neshanduko reropa tsvuku reropa, iro rinofarira kuparadzwa kwaro, uye rinoonekwa sehemolytic anemia. Shanduko mu membrane yemasero matsvuku eropa dzinovaita madiki uye asashomeka kupfuura zvakajairwa, zvichiparadzwa zviri nyore nespleen.

Spherocytosis chirwere chekugara nhaka, icho chinoperekedza munhu kubva pakuzvarwa, zvisinei, icho chinogona kufambira mberi neanemia yekusiyana kwakakomba. Nekudaro, mune zvimwe zviitiko panogona kunge pasina zviratidzo uye mune mamwe, pallor, kuneta, jaundice, yakawedzera spleen uye shanduko dzekuvandudza, semuenzaniso, dzinogona kuonekwa.

Kunyangwe pasina mushonga, spherocytosis ine kurapwa, iyo inofanirwa kutungamirirwa nachiremba wehematologist, uye folic acid kutsiva inogona kuratidzwa uye, mumatambudziko akaipisisa, kubviswa kwepleen, iyo inonzi splenectomy, kuitira kudzora chirwere ..

Chii chinokonzera spherocytosis

Hereditary spherocytosis inokonzerwa nekuchinja kwemajini kunokonzeresa shanduko muhuwandu kana mhando yemapuroteni anoumba membreni yemasero matsvuku eropa, anonyanya kuzivikanwa semasero matsvuku eropa. Shanduko mumapuroteni aya inokonzeresa kuomarara uye kudzivirirwa kwesero dzvuku reropa, rinovaita kuti vawedzere kusimba uye nehukuru hushoma, kunyangwe zvirimo zvakafanana, zvichiumba masero matsvuku madiki, ane chimiro chakakomberedzwa uye ane pigmented yakawanda.

Anemia inomuka nekuti ma "spherocyte", semasero matsvuku akaremara mu spherocytosis anonzi, anowanzoparadzwa mu spleen, kunyanya kana shanduko dzakakosha uye paine kurasikirwa kwekuchinja uye kuramba kupfuurira kuburikidza neiyo microcirculation yeropa kubva kunhengo iyi.

Zviratidzo zvikuru

Spherocytosis inogona kusarudzwa seyakareruka, akaenzana kana akaomarara. Nekudaro, vanhu vane hunyoro spherocytosis vanogona kunge vasina chero zviratidzo, nepo avo vane mwero kusvika kune yakaoma spherocytosis vanogona kunge vane akasiyana madhivhosi ezviratidzo nezviratidzo se:

• Kuramba uchishaya ropa;
• Pallor;
• Kuneta uye kusashivirira kune kurovedza muviri;
• Yakawedzera bilirubin muropa uye jaundice, inova yero yeruvara rweganda uye mucous membranes;
• Kuumbwa kwematombo ebilirubin muguruva;
• Yakawedzera spleen saizi.

Kuti uwane hutachiona spherocytosis, pamusoro pekuongororwa kwekiriniki, iyo hematologist inogona kuraira kuongororwa kweropa senge kuwanda kweropa, reticulocyte kuverenga, bilirubin kuyera uye peripheral yeropa smear inoratidza shanduko inoratidzira yerudzi urwu rweanemia.Kuongororwa kweosmotic fragility inoratidzwa zvakare, iyo inoyera kupokana kweiyo tsvuku yeropa cell membrane.

Kurapwa kunoitwa sei

Hereditary spherocytosis haina mushonga, zvisinei, iyo hematologist inogona kukurudzira kurapwa kunogona kudzikisira kuwedzera kwechirwere nezviratidzo, zvinoenderana nezvinodiwa nemurwere. Panyaya yevanhu vasingaratidze zviratidzo zvechirwere, hapana chaiyo kurapwa kunodiwa.

Iko kutsiviwa kwe folic acid inokurudzirwa nekuti, nekuda kwekuwedzera kwekuparara kwemasero matsvuku eropa, chinhu ichi chinonyanya kudikanwa pakuumbwa kwemasero matsva mumongo.

Nzira huru yekurapa kubviswa kwepleen nekuvhiya, iyo inowanzo kuratidzwa muvana vanopfuura makore mashanu kana matanhatu ekuberekwa vane chirwere chakanyanya chekushaya ropa, sevaya vane hemoglobin iri pasi pe8 mg / dl muhuwandu hweropa, kana pazasi pe10 mg / dl kana paine zvakakosha zviratidzo kana kunetseka kwakadai sematombo enduru. Kuvhiya kunogona kuitwawo pavana vane kunonoka kukura nekuda kwechirwere.

Vanhu vanobviswa spleen vane mukana wekuvandudza hutachiona kana maronda, saka majekiseni, akadai sepneumococcal, anodikanwa, kuwedzera pakushandiswa kweASA kudzora kugwamba kweropa. Tarisa uone kuti kuvhiyiwa kwekubviswa kwepleen uye kutarisirwa kwakakodzera kunoitwa sei.