Sickle cell anemia: yintoni, iimpawu, oonobangela kunye nonyango

Umxholo

• Iimpawu eziphambili
• Indlela yokuqinisekisa ukuxilongwa
• Unobangela onokubakho we-sickle cell anemia
• Unyango lwenziwa njani
• Iingxaki ezinokwenzeka

I-Sickle cell anemia sisifo esibonakaliswa kukutshintsha kobume beeseli ezibomvu zegazi, ezinesimo esifana nerhengqa okanye isiqingatha senyanga. Ngenxa yolu tshintsho, iiseli ezibomvu zegazi ziye zingakwazi ukuthwala ioksijini, ukongeza ekunyukeni komngcipheko wokuthintelwa kwenqanawa yegazi ngenxa yemeko eguqulweyo, enokuthi ikhokelele kwintlungu ebanzi, ubuthathaka kunye nokungakhathali.

Iimpawu zolu hlobo lwe-anemia zinokulawulwa ngokusetyenziswa kwamachiza ekufuneka ethathwe kubo bonke ubomi ukunciphisa umngcipheko weengxaki, nangona kunjalo unyango luyenzeka kuphela ngokufakelwa kweeseli ze-hematopoietic stem.

Iimpawu eziphambili

Ukongeza kwiimpawu eziqhelekileyo zalo naluphi na olunye uhlobo lwe-anemia, njengokudinwa, ukugcwala kunye nokulala, iarhente yeseli anemia inokubangela ezinye iimpawu ezinje:

• Ubuhlungu emathanjeni nakumalungu kuba ioksijini ifika ngesixa esincinci, ikakhulu kwiziphelo, ezinjengezandla neenyawo;
• Iingxaki zentlungu kwisisu, esifubeni nakwingingqi ye-lumbar, ngenxa yokufa kweeseli zomongo wethambo, kwaye inokuthi inxulunyaniswe nomkhuhlane, ukugabha kunye nomchamo omnyama okanye onegazi;
• Rhoqo usulelokuba iiseli ezibomvu zegazi zinokonakalisa udakada, olunceda ukulwa nosulelo;
• ​Ukudodobala kokukhula kunye nokulibaziseka ukufikisakuba iiseli ezibomvu zegazi ezivela kwi-sickle cell anemia zibonelela ngeoksijini encinci kunye nezakhamzimba ukuze umzimba ukhule kwaye uphuhle;
• Amehlo amnyama kunye nolusu ngenxa yokuba iiseli ezibomvu zegazi "ziyafa" ngokukhawuleza kwaye, ke, i-bilirubin pigment iqokelela emzimbeni obangela umbala otyheli kulusu namehlo.

Ezi mpawu zihlala zivela emva kweenyanga ezi-4 ubudala, kodwa isifo eso sihlala senziwa kwiintsuku zokuqala zobomi, okoko umntwana osandul 'ukuzalwa esenza uvavanyo lwenyawo losana. Fumanisa ngakumbi malunga nokuvavanywa kwesithende kunye nezifo esizifumanayo.

Indlela yokuqinisekisa ukuxilongwa

Ukuchongwa kwe-sickle cell anemia kuhlala kwenziwa ngokuvavanya unyawo losana kwiintsuku zokuqala zobomi bosana. Olu vavanyo luyakwazi ukwenza uvavanyo olubizwa ngokuba yi-hemoglobin electrophoresis, ejonga ubukho be-hemoglobin S kunye noxinaniso lwayo. Kungenxa yokuba ukuba kufunyaniswe ukuba umntu uneseli enye kuphela ye-S, oko kukuthi, ihemoglobin yohlobo lwe-AS, oko kuthetha ukuthi ungumntu ophethe i-sickle cell anemia gene, ehlelwa njenge-sickle cell trait. Kwiimeko ezinjalo, umntu usenokungabonakalisi zimpawu, kodwa kufuneka alandelwe ngokuvavanywa rhoqo kwilebhu.

Xa umntu efunyaniswe ene-HbSS, oko kuthetha ukuba umntu une-sickle cell anemia kwaye kufuneka anyangwe ngokweengcebiso zonyango.

Ukongeza kwi-hemoglobin electrophoresis, ukuxilongwa kolu hlobo lwe-anemia kungenziwa ngomlinganiso we-bilirubin ehambelana nokubalwa kwegazi kubantu abangakhange benze uvavanyo lwesithende ngexesha lokuzalwa, kunye nobukho beeseli ezibomvu ezinjengeirhengqa, ubukho bee-reticulocytes, basophilic speckles kunye ne-hemoglobin value engezantsi kwexabiso eliqhelekileyo lereferensi, ngesiqhelo phakathi kwe-6 kunye ne-9.5 g / dL.

Unobangela onokubakho we-sickle cell anemia

Unobangela we-sickle cell anemia yimfuza, oko kukuthi, izalwa kunye nomntwana kwaye idluliselwa isuka kutata iye kunyana.

Oku kuthetha ukuba nanini na xa umntu efunyaniswa enesi sifo, une-SS gene (okanye ihemoglobin SS) awayeyifumana kumama notata wakhe. Nangona abazali benokubonakala besempilweni, ukuba utata nomama bane-gene ye-AS (okanye ihemoglobin AS), ebonisa umntu ophethe esi sifo, ekwabizwa ngokuba ligciwane lesirhengqa, likho ithuba lokuba umntwana abe nesi sifo ( 25% ithuba) okanye ube ngumntu ophetheyo (ama-50% amathuba) esi sifo.

Unyango lwenziwa njani

Unyango lwe-sickle cell anemia lwenziwa ngokusetyenziswa kwamayeza kwaye kwezinye iimeko kungafuneka utofelo-gazi.

Amachiza asetyenzisiweyo ikakhulu yiPenicillin ebantwaneni ukusuka kwiinyanga ezi-2 ukuya kwiminyaka emi-5 ubudala, ukuthintela ukuqala kweengxaki ezinje nge-pneumonia, umzekelo. Ukongeza, iziyobisi ze-analgesic kunye ne-anti-inflammatory zingasetyenziselwa ukunciphisa iintlungu ngexesha lobunzima kwaye zisebenzise imaski yeoksijini ukunyusa inani leoksijini egazini kunye nokwenza lula ukuphefumla.

Unyango lwe-sickle cell anemia kufuneka lwenziwe ubomi kuba ezi zigulana zinokuba nosulelo rhoqo. Umkhuhlane unokubonisa ukosuleleka, ke ukuba umntu one-sickle cell anemia unefiva, kufuneka aye kwagqirha ngoko nangoko kuba angafumana i-septicemia kwiiyure nje ezingama-24, ezinokubulala. Amachiza okunciphisa umkhuhlane akufuneki asetyenziswe ngaphandle kolwazi lonyango.

Ukongeza, ukufakelwa umongo wethambo ikwaluhlobo lonyango, oluboniswe kumatyala athile kwaye lukhethwe ngugqirha, elinokuthi lize kunyanga isifo, nangona kunjalo libonisa umngcipheko othile, njengokusebenzisa amayeza anciphisa ukhuseleko. Fumanisa ukuba kwenziwa njani ukutsalwa komongo wethambo kunye nobungozi obunokubakho.

Iingxaki ezinokwenzeka

Iingxaki ezinokuchaphazela abaguli abane-sickle cell anemia kunokuba:

• Ukudumba kwamalungu ezandla kunye neenyawo ezibashiya bedumbe kwaye bebuhlungu kakhulu kwaye bekhubazekile;
• Ukwanda komngcipheko wosulelo ngenxa yokubandakanyeka kwepeni, engayi kucoca igazi ngokufanelekileyo, ngaloo ndlela ivumela ubukho beentsholongwane kunye neebhaktiriya emzimbeni;
• Ukukhubazeka kwezintso, ngokuhamba rhoqo komchamo, kuqhelekile ukuba umchamo ube mnyama kwaye umntwana achame ebhedini kude kube kwinqanaba lokufikisa;
• Amanxeba emilenzeni ekunzima ukuphilisa kwaye afuna ukunxiba kabini ngemini;
• Ukuphazamiseka kwesibindi okuzibonakalisa ngeempawu ezinjengombala otyheli emehlweni naseluswini, kodwa ingeyoyo i-hepatitis;
• Amatye e-Gall;
• Ukwehla kombono, amanxeba, amabala kunye neempawu zokolula amehlo, kwezinye iimeko kungakhokelela kukungaboni;
• Stroke, ngenxa yobunzima begazi ngokunkcenkceshela ingqondo;
• Ukungaphumeleli kwentliziyo, kunye ne-cardiomegaly, infarctions kunye nokumbombozela kwentliziyo;
• Ukubaluleka kwangaphambili, okuqaqanjiswayo, okungaqhelekanga kunye nokuzingisa okungahambelaniyo nomnqweno wesini okanye ukuvusa inkanuko, okuxhaphakileyo emadodeni amancinci.

Utofelo-gazi lunokuba yinxalenye yonyango, ukwandisa inani leeseli ezibomvu ekujikelezeni, kwaye kukufakwa nje kweeseli zehematopoietic stem kunika kuphela unyango olunokubakho lwearomy cell anemia, kodwa zimbalwa izibonakaliso ngenxa yomngcipheko onxulumene Inkqubo.