Sickle cell anemia: kuyini, izimpawu, izimbangela kanye nokwelashwa

-Delile

• Izimpawu eziyinhloko
• Ungakuqinisekisa kanjani ukuxilongwa
• Izimbangela ezingaba khona ze-sickle cell anemia
• Yelashwa kanjani
• Izinkinga ezingenzeka

I-Sickle cell anemia yisifo esibonakala ngokushintsha kwesimo samangqamuzana abomvu egazi, anomumo ofana nesikela noma inyanga yenyanga. Ngenxa yalolu shintsho, amangqamuzana abomvu egazi ahluleka ukuphatha i-oksijini, ngaphezu kokukhulisa ubungozi bokuvinjelwa kwemithambo yegazi ngenxa yesimo esishintshiwe, okungaholela ebuhlungwini obandile, ebuthakathaka kanye nokunganaki.

Izimpawu zalolu hlobo lwe-anemia zingalawulwa ngokusetshenziswa kwezidakamizwa okufanele zithathwe impilo yonke ukunciphisa ubungozi bezinkinga, kepha ukwelashwa kwenzeka kuphela ngokufakelwa kwamaseli we-hematopoietic stem.

Izimpawu eziyinhloko

Ngaphezu kwezimpawu ezivamile zanoma yiluphi olunye uhlobo lwe-anemia, njengokukhathala, i-pallor nokulala, i-sickle cell anemia nayo ingadala ezinye izimpawu zesici, njenge:

• Ubuhlungu emathanjeni nasemalungeni ngoba umoya-mpilo ufika ngobuncane, ikakhulukazi emaphethelweni, njengezandla nezinyawo;
• Izinkinga zobuhlungu endaweni yesisu, isifuba kanye ne-lumbar, ngenxa yokufa kwamangqamuzana omnkantsha, futhi kungahle kuhlotshaniswe nomkhuhlane, ukuhlanza nomchamo omnyama noma onegazi;
• Ukutheleleka njalongoba amangqamuzana abomvu egazi angalimaza ubende, olusiza ukulwa nezifo;
• ​Ukubambezeleka kokukhula nokubambezeleka kokuthombangoba amaseli abomvu egazi avela ku-sickle cell anemia ahlinzeka nge-oksijeni nezakhi zomzimba ezincane ukuze umzimba ukhule futhi uthuthuke;
• Amehlo aphuzi nesikhumba ngenxa yokuthi amangqamuzana abomvu egazi "ayafa" ngokushesha okukhulu, ngakho-ke, i-bilirubin pigment iqoqa emzimbeni ibangele umbala ophuzi esikhumbeni nasemehlweni.

Lezi zimpawu zivame ukuvela ngemuva kwezinyanga ezine ubudala, kepha ukuxilongwa kuvame ukwenziwa ezinsukwini zokuqala zokuphila, inqobo nje uma usana lusavivinya unyawo lwengane. Thola okuningi mayelana nokuhlolwa kwesithende nokuthi kuthola ziphi izifo.

Ungakuqinisekisa kanjani ukuxilongwa

Ukuxilongwa kwe-sickle cell anemia kuvame ukwenziwa ngokuhlola unyawo lwengane ezinsukwini zokuqala zempilo yengane. Lokhu kuhlolwa kuyakwazi ukwenza isivivinyo esibizwa nge-hemoglobin electrophoresis, esihlola ubukhona be-hemoglobin S kanye nokugxila kwayo. Lokhu kungenxa yokuthi uma kutholakala ukuthi umuntu unesakhi sofuzo esisodwa S, okungukuthi, i-hemoglobin yohlobo lwe-AS, kusho ukusho ukuthi ungumphathi wesakhi sofuzo se-sickle cell anemia, ehlukaniswa njenge-sickle cell trait. Ezimweni ezinjalo, umuntu kungenzeka angakhombisi izimpawu, kepha kufanele alandelwe ngokuhlolwa okujwayelekile kwelabhorethri.

Lapho umuntu kutholakala ukuthi une-HbSS, kusho ukuthi lowo muntu une-sickle cell anemia futhi kufanele elashwe ngokweseluleko sezokwelapha.

Ngaphezu kwe-hemoglobin electrophoresis, ukuxilongwa kwalolu hlobo lwe-anemia kungenziwa ngokulinganisa kwe-bilirubin ehambisana nokubalwa kwegazi kubantu abangazange benze ukuhlolwa kwesithende lapho bezalwa, kanye nokuba khona kwamangqamuzana egazi abomvu amise okwegazi, ukutholakala kwama-reticulocytes, ama-basophilic speckles nenani le-hemoglobin elingaphansi kwenani elijwayelekile lesethenjwa, imvamisa liphakathi kuka-6 no-9.5 g / dL.

Izimbangela ezingaba khona ze-sickle cell anemia

Izimbangela ze-sickle cell anemia ziyizakhi zofuzo, okungukuthi, izalwa nengane futhi idluliselwa isuka kubaba iye endodaneni.

Lokhu kusho ukuthi noma nini lapho kutholakala ukuthi umuntu unalesi sifo, unofuzo lwe-SS (noma i-hemoglobin SS) aluthole kunina nakubaba wakhe. Yize abazali bengabukeka bephilile, uma ubaba nomama benohlobo lwe-AS (noma i-hemoglobin AS), okukhomba othwala lesi sifo, obizwa nangokuthi isikela yesici, kunethuba lokuthi ingane izoba nalesi sifo ( 25% ithuba) noma ube othwala (50% ithuba) lesifo.

Yelashwa kanjani

Ukwelashwa kwe-sickle cell anemia kwenziwa ngokusetshenziswa kwemithi futhi kwezinye izimo ukumpontshelwa igazi kungadingeka.

Izidakamizwa ezisetshenzisiwe ikakhulukazi iPenicillin ezinganeni ezisukela ezinyangeni ezimbili kuya eminyakeni emihlanu ubudala, ukuvimbela ukuqala kwezinkinga ezifana nenyumoniya, ngokwesibonelo. Ngaphezu kwalokho, izidakamizwa ze-analgesic kanye ne-anti-inflammatory nazo zingasetshenziswa ukudambisa izinhlungu ngesikhathi senhlekelele futhi zisebenzise ngisho imaskhi yomoya-mpilo ukukhulisa inani lomoya-mpilo egazini nokwenza lula ukuphefumula.

Ukwelashwa kwe-sickle cell anemia kufanele kwenziwe impilo yonke ngoba lezi ziguli zingahle zitheleleke kaningi. Imfiva ingakhombisa ukutheleleka, ngakho-ke uma umuntu one-sickle cell anemia enomkhuhlane, kufanele aye kudokotela ngokushesha ngoba angaba ne-septicemia emahoreni angama-24 kuphela, angabulala. Izidakamizwa ezinciphisa umkhuhlane akufanele zisetshenziswe ngaphandle kolwazi lwezokwelapha.

Ngaphezu kwalokho, ukufakelwa umnkantsha wohlobo oluthile kuyindlela yokwelapha, ekhonjiswa ezimweni ezithile ezibucayi futhi ekhethwe ngudokotela, engahle izelapha lesi sifo, noma kunjalo inezingozi ezithile, njengokusetshenziswa kwemithi enciphisa ukuzivikela komzimba. Thola ukuthi ukufakelwa umongo wethambo kwenziwa kanjani nezingozi ezingaba khona.

Izinkinga ezingenzeka

Izinkinga ezingathinta iziguli ezine-sickle cell anemia kungaba:

• Ukuvuvukala kwamalunga wezandla nezinyawo okuwashiya evuvukile futhi ebuhlungu kakhulu futhi ekhubazekile;
• Ukwanda kwengozi yokutheleleka ngenxa yokubandakanyeka kobende, okungeke kuhlunge igazi ngokufanele, ngaleyo ndlela kuvumele ukuba khona kwamagciwane kanye namagciwane emzimbeni;
• Ukukhubazeka kwezinso, ngokuvama kokuchama kaningi, kujwayelekile nokuthi umchamo ube mnyama futhi ingane ichame embhedeni ize ifinyelele ebusheni;
• Amanxeba emilenzeni okunzima ukuwelapha futhi adinga ukugqoka kabili ngosuku;
• Ukukhubazeka kwesibindi okuzibonakalisa ngezimpawu ezifana nombala ophuzi emehlweni nasesikhumbeni, kodwa okungesona isifo sokusha kwesibindi kohlobo;
• Gall amatshe;
• Ukwehla kokubona, izibazi, izisihla nokwelulwa emehlweni, kokunye kungaholela ekungaboni;
• Stroke, ngenxa yobunzima begazi ekuniseleni ubuchopho;
• Ukwehluleka kwenhliziyo, nge-cardiomegaly, infarctions kanye nokububula kwenhliziyo;
• I-Priapism, okuyi-erection ebuhlungu, engajwayelekile futhi ephikelelayo engahambisani nesifiso sobulili noma ukuvusa inkanuko, okuvamile emadodeni amasha.

Ukumpontshelwa igazi nakho kungaba yingxenye yokwelashwa, ukwandisa inani lamangqamuzana abomvu egazi ekusakazeni, futhi ukufakelwa kabusha kwamaseli we-hematopoietic kuphela okunikeza ikhambi elilodwa elingaba yikhambi le-sickle cell anemia, kodwa ngezinkomba ezimbalwa ngenxa yezingozi ezihambisana inqubo.