Dib-u-eegis ku saabsan xad-dhaafka birta ee ka yimaada gudbin fara badan
Xaddiga birtu waa xaalad halka ay jirto bir badan oo jirka ah marka loo eego jidhka. Ereyga farsamada ee xad-dhaafka birtu waa hemochromatosis. Waxaa jira laba sababood oo waaweyn oo birta xad dhaaf ah: 1) hemochromatosis ama 2) hemochromatosis ku dhalata. Cudurka Hemochromatosis waa cudur xaalad qoys oo loo soo gudbiyo. Xanuunku wuxuu ku dhacaa marka qofku u baahan yahay unugyo badan oo unugyada dhiigga cas (RBC).
Birta waxaa laga helaa gudaha hemoglobin , borotiin gudaha gudaha RBC. Shaqada birta ayaa ka caawisa gaadiidka gaadiidka in ay u gudbiyaan dhammaan unugyada. Caadi ahaan jidhku wuxuu ka soo baxaa birta oo ku filan cuntadaada si aad ula socoto wax soo saarka RBC. Haddii aadan cunin cuntooyin ku filan oo bir leh birta, heerarka birtaagu wuxuu noqonayaa mid hooseeya taasoo keeneysa dhiig yaraanta birta . Marka RBCs ay gaarto dhammaadka noloshooda, birta gudaha ku jirta ayaa dib loo warshadeeyaa in loo isticmaalo RBC cusub oo cusub.
Maxay u Qabsoomaan Raadinta Badan ee RBC Sababta Birta?
Birta waxaa laga helaa gudaha dhammaan RBC-yada ee qaybta dhiigga lagu wareejiyo. Sidaa daraadeed waa in qof kasta oo lagu helo shuban B RBC uu yahay asal ahaan faleebo faleebo (IV) oo birta ah. Dhibaatadu waxay tahay in jidhku uu xadidan yahay awooda ay u leedahay in laga takhaluso macdanta xad-dhaafka ah. Waxaa intaa dheer, dadka qaba thalassaemia waxay u nuugaan bir bir ah inta ay u baahan yihiin cuntadooda oo ka sii dareysa dhibaatada.
Yaa Khatar U Baahan Yahradda Dheeraadka Dheeraadka ah?
Qofkasta oo qaata maadooyin badan oo RBC ah ayaa halis ugu jira, laakiin dadka ku tiirsan xawilaadda waa khatarta ugu sareysa.
Tani waxay ku jiri doontaa dadka qaba cudurka sickle cell ee lahaa istaroog (ama khatarta sare ee istaroogga ), beta thalassaemia major , Diamond Blackfan Anemia , dhiig yaraanta dhiigga , iyo cudurka myelodysplastic syndrome iyo kuwa kale. Dadka qaba kansarka u baahan baahida badan ee mudada daaweynta daaweynta kemotherabi ama lafo-beelka lafo-beelka ayaa sidoo kale halis u ah in xad-dhaafka birta ee is-beddelka.
Sidee Bixiyeha Daryeelkeyga Kormeeray Xadida Birta?
Dadka u baahan in la sii wado inta lagu guda jiro ayaa guud ahaan loola socdaa si xad-dhaaf ah oo birta ah. Bukaankan, xad-dhaafka xad-dhaafka ayaa la arki karaa ka dib 12 illaa 15 raajisyada RBC. Birta xad dhaafka ah waxaa sida caadiga ah la socdaa baaritaanka dhiigga loo yaqaan ferritin markii hore. Ferritin waxay ka dhigan tahay wadarta birta lagu keydiyo jirka. Ferritin waxaa sida caadiga ah lagu sameeyaa jadwal, midba saddex ilaa saddex bilood, sidaa daraadeed dhakhtarkaagu wuxuu arki karaa sida ay u socoto (taas oo ah, qiimaha kor u kacay?). Nadiifinta, mudada daawooyinka loo isticmaalo in laga saaro birta, ayaa sida caadiga ah la bilaabaa marka ferritinku ka weyn yahay 1000 ng / mL. Nasiib darro, heerarka ferritinka waxaa saameeya waxyaabo aan ka ahayn xad-dhaaf xad-dhaaf ah. Heerka ferritin ee sareeya ayaa la arki karaa inta lagu jiro jirada iyo bararka.
Sababtoo ah dhibaatooyinka ferritin, hababka kale ee lagu qiimeeyo xadiga xad-dhaafka ah ayaa la horumariyey. Markii hore, xaddi badan oo birta ah ayaa la kormeeray kaashka beerka halkaasoo qayb ka yar beerka laga saaray oo lagu qiimeeyay birta. Waqtigan xaadirka ah, dadka badidooda waxaa lagu kormeeri karaa sawirada magnetka (MRI) ee beerka iyo / ama wadnaha. MRI waxay xisaabin kartaa naqshadda birta beerka (LIC) oo la mid ah natiijooyinka beerka lagaga jaray.
Gawaarida waxaa la bilaabay markii LIC ay ka weyn tahay 3 mg per galka beerka culus ee qalalan. Sidoo kale, MRI ee wadnaha ayaa qiyaasi kara xadiga birta ku yaal muruqa wadnaha.
Waa maxay Dhibaatooyinka ay ku dhici karaan xad-dhaafka birta?
Marka dhammaan goobaha caadiga ah ee lagu keydiyo birta aan la heli karin, birta ayaa markaa lagu keydin karaa beerka, wadnaha, beeryarada, iyo xubnaha unugyada gaduudan (guud ahaan loo yaqaan qanjidhada). Marka birta lagu keydiyo meelahaas, waxay dhaawici kartaa xubnaha. Tani waxay keeni kartaa fibrosis ama cirridka beerka, kiniiniyada wadnaha (cudurada muruqa wadnaha), sonkorow mellitus (macdanta birta ee xameetida), hypothyroidism (heerarka hoormoonka tayroodhka hoose) iyo hypogonadism (oo keena libido yar iyo awood la'aan la'aan raga iyo la'aanta caadada caadada ee haweenka).
Si looga hortago dhibaatooyinkan halista ah ee halista ah, birta xad dhaafka ah waxaa lagu daweeyaa si taxadar leh.
Sidee Looga Daawan Yahay Xayiraadda Birta?
Xaddiga birta ee ka yimaadda gudbinta waxaa lagu daaweeyaa daaweynta chelation, daawada la siiyo si looga saaro birta jirka. Waxaa jira saddex dawo oo la heli karo.
- Deferoxamine (Desferal)
- Deferasirox (Jadenu ama Exjade)
- Deferiprone (Ferriprox)
Qalabka faafa ee loo yaqaan 'Therapeutic phlebotomy' : Haddii marxaladda aad joojiso kudhawaansho, xad-dhaafka birta waxaa lagu daaweyn karaa phlebotomy series. Phlebotomy waxay la mid tahay deeq dhiig ah halkaasoo dhiig badan oo jirka laga saaro. Gudaha RBC waxaa laga saaraa birta iyo marka kuwan lagu beddelo dhuuxa lafta wuxuu isticmaali doonaa birta xad-dhaafka ah ee lagu keydiyo jirkaada.
> Ilo:
> Schrier SL iyo Bacon BR. Daweynta Chelation for Thalassaemia iyo Wadamada kale ee Dareemada Birta ah iyo Cudurka Dabaysha Xanuunada kale oo ka baxsan Hemochromatosis. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.