Nhaka spherocytic kupererwa neropa

Hereditary spherocytic anemia chirwere chisingawanzoitika chepamusoro pevhu (membrane) yemasero matsvuku eropa. Inotungamira kumasero matsvuku eropa ayo akaumbwa kunge ma sphere, uye kukurumidza kuparara kwemasero matsvuku eropa (hemolytic anemia).

Ichi chirwere chinokonzerwa neyakaremara jeni. Kuremara kunoguma mune isina kujairika tsvuku sero membrane. Maseru akakuvara ane nzvimbo diki yepasi yehuwandu hwavo kupfuura zvakajairika masero matsvuku eropa, uye anogona kuvhurika nyore.

Iyo anemia inogona kusiyanisa kubva pane yakapfava kusvika kune yakaoma. Muzviitiko zvakakomba chirwere ichi chinogona kuwanikwa muhudiki. Muzviitiko zvinyoro zvinogona kuenda zvisingaonekwe kusvika munhu mukuru.

Dambudziko iri rinowanzoitika kuvanhu vanobva kuchamhembe kweEurope, asi rakawanikwa mumarudzi ese.

Vacheche vanogona kuve nekutsvukirira kweganda nemaziso (jaundice) uye kuchena kupenda (pallor).

Zvimwe zviratidzo zvinogona kusanganisira:

• Kuneta
• Kushatirwa
• Kufema kwekufema
• Kushaya simba

Muzviitiko zvakawanda, iyo spleen inowedzerwa.

Rabhoritari bvunzo dzinogona kubatsira kuongorora mamiriro aya. Miedzo inogona kusanganisira:

• Ropa smear kuratidza masero akaumbwa zvisina kujairika
• Chikamu cheBilirubin
• Pedzisa kuverenga ropa kuti utarise kushaya ropa
• Coombs bvunzo
• Chikamu cheLDH
• Osmotic fragility kana yakasarudzika kuyedza kuongorora iyo tsvuku sero masero chirema
• Reticulocyte kuverenga

Kuvhiya kubvisa iyo spleen (splenectomy) kunorapa kushaya anemia asi hakugadzirise iyo isina kujairika maseru chimiro.

Mhuri dzine nhoroondo yespherocytosis dzinofanirwa kuti vana vadzo vavhenekwe chirwere ichi.

Vana vanofanira kumirira kusvika zera mashanu kuti vave ne splenectomy nekuda kwehutachiona hutachiona. Muzviitiko zvinyoro-nyoro zvakawanikwa muvakuru, zvinogona kunge zvisiri kudikanwa kubvisa iyo spleen.

Vana nevakuru vanofanirwa kupihwa pneumococcal jekiseni pamberi pekuvhiya kwepleen. Ivo zvakare vanofanirwa kugamuchira folic acid zvinowedzera. Mamwe majekiseni anogona kudikanwa zvichienderana nenhoroondo yemunhu.

Izvi zvinotevera zviwanikwa zvinogona kupa rumwe ruzivo nezve nhaka nhaka spherocytic anemia:

• Genetic uye Rare Zvirwere Ruzivo Ruzivo - rarediseases.info.nih.gov/diseases/6639/hereditary-spherocytosis
• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/anemia-hereditary-spherocytic-hemolytic

Mhedzisiro yacho inowanzo kuve yakanaka nekurapwa. Mushure mekunge spleen yabviswa, hurefu hwehupenyu hwesero tsvuku reropa hunodzokera pane zvakajairwa.

Zvinetso zvinogona kusanganisira:

• Matombo
• Zvakawanda zvakadzika tsvuku masero eropa kugadzirwa (aplastic dambudziko) inokonzerwa nehutachiona hutachiona, izvo zvinogona kuita kuti anemia iwedzere

Fonera wako mupi wezvehutano kana:

• Zviratidzo zvako zvinowedzera.
• Zviratidzo zvako hazviite nekurapa kutsva.
• Unovandudza zviratidzo zvitsva.

Ichi chirwere chakagarwa nhaka uye chingangodzivirirwa. Kuziva njodzi yako, senge nhoroondo yemhuri yechirwere ichi, zvinogona kukubatsira kuti uongororwe nekurapwa nekukurumidza.

Koberekwa spherocytic hemolytic kupererwa neropa; Spherocytosis; Hemolytic kupererwa neropa - spherocytic

• Masero matsvuku eropa - akajairwa
• Masero matsvuku eropa - spherocytosis
• Masero eropa

Gallagher PG. Tsvuku dzeropa maseru membrane kusagadzikana. Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chits.

Merguerian MD, Gallagher PG. Nhaka spherocytosis. Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chitsipa 485.