I-spherocytosis eyifa: izimbangela, izimpawu nokwelashwa

-Delile

• Yini ebangela i-spherocytosis
• Izimpawu eziyinhloko
• Yelashwa kanjani

I-Hereditary spherocytosis yisifo sofuzo esibonakala ngokushintshwa kolwelwesi lwegazi olubomvu, oluthanda ukubhujiswa kwalo, ngakho-ke kubhekwa njenge-hemolytic anemia. Izinguquko kulwelwesi lwamangqamuzana abomvu egazi ziwenza abe mancane futhi amelane kancane kunokujwayelekile, abhujiswe kalula ubende.

I-Spherocytosis yisifo esizalwa nefa, esihambisana nomuntu kusukela ekuzalweni, kepha-ke, singathuthuka ngokushoda kwegazi okuhlukahlukene. Ngakho-ke, kwezinye izimo kungahle kungabikho zimpawu futhi kokunye, ukubukeka, ukukhathala, i-jaundice, ubende obukhulisiwe kanye nezinguquko zentuthuko, isibonelo, kungabonakala.

Yize lungekho ikhambi, i-spherocytosis inokwelashwa, okumele kuqondiswe yi-hematologist, futhi kufakwe i-folic acid esikhundleni futhi, ezimweni ezinzima kakhulu, kususwe ubende, olubizwa nge-splenectomy, ukuze kulawulwe lesi sifo .

Yini ebangela i-spherocytosis

I-hereditary spherocytosis idalwa ukuguqulwa kofuzo okuholela ekushintsheni kobuningi noma ikhwalithi yamaprotheni akha ulwelwesi lwamaseli abomvu egazi, aziwa kakhulu njengamaseli abomvu egazi. Izinguquko kulawa maprotheni zidala ukulahleka kobulukhuni nokuvikelwa kolwelwesi lwegazi elibomvu, okubenza babe ntekenteke kakhudlwana futhi babe nosayizi omncane, yize okuqukethwe kufana, kwakheka amangqamuzana abomvu amancane, anezici eziyindilinga futhi ezinombala ngaphezulu.

I-anemia ivela ngenxa yokuthi ama-spherocyte, njengoba kubizwa amaseli abomvu okhubazekile ku-spherocytosis, ngokuvamile abhujiswa ebuchosheni, ikakhulukazi uma izinguquko zibalulekile futhi kukhona ukulahleka kokuguquguquka nokumelana nokudlula ekujikelezeni kwegazi okuncane okuvela kulesi sitho.

Izimpawu eziyinhloko

I-Spherocytosis ingahlukaniswa njengemnene, elinganiselayo noma enzima. Ngakho-ke, abantu abane-spherocytosis emnene bangase bangabi nazimpawu, kuyilapho labo abane-spherocytosis elinganiselayo kuya kwelikhulu bangaba namazinga nezimpawu ezahlukahlukene ezinjenge:

• I-anemia eqhubekayo;
• Pallor;
• Ukukhathala nokungabekezeleli ukuzivocavoca umzimba;
• Ukwandiswa kwe-bilirubin egazini nase-jaundice, okuwumbala ophuzi wesikhumba kanye nolwelwesi lwamafinyila;
• Ukwakhiwa kwamatshe e-bilirubin ku-gallbladder;
• Usayizi wobende obandisiwe.

Ukuze kuhlolwe i-spherocytosis yefa, ngaphezu kokuhlolwa komtholampilo, i-hematologist inga-oda ukuhlolwa kwegazi okufana nokubalwa kwegazi, ukubala kwe-reticulocyte, isilinganiso se-bilirubin kanye ne-peripheral blood smear ekhombisa izinguquko ezisikisela lolu hlobo lwe-anemia.Ukuhlolwa kwe-osmotic fragility kukhonjisiwe, okulinganisa ukumelana nolwelwesi lwegazi elibomvu.

Yelashwa kanjani

I-hereditary spherocytosis ayinalo ikhambi, noma kunjalo, udokotela we-hematologist angancoma ukwelashwa okunganciphisa ukwanda kwesifo nezimpawu, ngokusho kwezidingo zesiguli. Endabeni yabantu abangazibonisi izimpawu zalesi sifo, akudingeki ukwelashwa okuthile.

Ukufakwa kwe-folic acid kunconyiwe ngoba, ngenxa yokwehla kwamangqamuzana abomvu egazi, le nto iyadingeka ukwakheka kwamaseli amasha emnkantsheni.

Indlela eyinhloko yokwelashwa ukususwa kobende ngokuhlinzwa, okuvame ukukhonjiswa ezinganeni ezineminyaka engaphezu kwemihlanu noma eyisithupha ubudala ezine-anemia enamandla, njengalezo ezine-hemoglobin engaphansi kuka-8 mg / dl esibalweni segazi, noma ngaphansi kuka-10 mg / dl uma kunezimpawu ezibalulekile noma izinkinga ezifana namatshe enyongo. Ukuhlinzwa kungenziwa nasezinganeni ezinokubambezeleka kokukhula ngenxa yalesi sifo.

Abantu abasuswa ubende banamathuba amaningi okuthola ukutheleleka okuthile noma ama-thromboses, ngakho-ke kudingeka imishanguzo, efana ne-pneumococcal, ngaphezu kokusetshenziswa kwe-ASA ukulawula ukuvalwa kwegazi. Bheka ukuthi ukuhlinzwa kokususwa kobende nokunakekelwa okudingekayo kwenziwa kanjani.