Igazi Sideroblastic: iimpawu, unyango

Abantu abaninzi bayazi ukuba kwegazi sisifo kuyingozi kakhulu. Kukho iintlobo ezininzi kwesi sifo, yaye ngamnye kubo kuyingozi kwimpilo yabantu. igazi Sideroblastic i ngezifo eyingozi ezinxulumene kakuhle Micro yokuqaphela. Kulo sifo wamathambo usebenzisa intsimbi umbutho ihemoglobin, ngoko lifakwa nezibilini. Eyona nto ibalulekileyo - ukuthintela iingxaki, yaye ngenxa yale njongo kufuneka ngokukhawuleza bafune uncedo lobungcali.

ukuba nombono

igazi Sideroblastic yohlukile kwezinye iintlobo zezifo kuncitshiswa yoxinaniso yesinyithi iiseli ezibomvu. Isibakala sokuba ukuba umongo akasebenzisi le element kuyondelelaniswe mpilo. Kaninzi, ngenxa yokugula iphuhlisa njengoko iziphene okanye lufundiwe. Kwinqanaba yemfuza, ianemia kwenzeka kakhulu amakhwenkwe.

Isifo sinokusulela njengento autosomal ngendlela lubalaseleyo. Oku kuthiwa anemia syndrome Pearson. Xa umzimba yakhiwe tenxileyo wendele intsimbi, oko lifakwa enyama yangaphakathi, leyo igazi sideroblastic. Ukuba ndlala kuba kakhulu, umsebenzi uwaphule isibindi, izintso kunye umsipha.

iintlobo zezifo

Olu hlobo kwegazi wahlulwe iqondo elihlabayo, kodwa ngenxa imbonakalo kunye nomfanekiso clinical. Zininzi iintlobo eyodwa anemia sideroblastic:

1. Njengelifa. Le izifo njengelifa ngenxa gene wezakhi. Oku kwenza i inkqubo yokucolwa anomaly ngezifo ebandakanya vitamin B6 kunye aminolevulinic acid. Isifo yenza ngokwayo waziva emva kokuzalwa okanye ngexesha lokufikisa.
2. Bekhubazekile. Le fomu ayinabantu ngokwahlukileyo, nangona othile edibana ngayo ifomu amafa. Apha, lubonakala ngokuba isiqulatho eliphezulu erythrocyte coproporphyrin.
3. Ezifuniweyo. Kubonakala ngenxa iziphumo ezibi wemichiza. Phakathi kwazo kukho ethanol, ilothe, tsiklosirin.

Iingxaki kunye ezwi yentsimbi kwakhona zenzeka ngenxa yeenkqubo ithumba emzimbeni. Malunga 1/10 ezigulane igazi sideroblastic unesifo wegazi oyingozi.

Izinto ezibangela izifo

Oyena nobangela uphambili esi sifo na protoporfiirina ukungabikho, leyo yenye yeendima ezibalulekileyo le Yindibanisela haemoglobin element. Ukongeza, izinto inxaxheba ekudalweni protein kunye nentsimbi.

kuvela uhlobo Acquired anemia sideroblastic ngenxa yokuba umzimba ngenisa izinto eziyimfuneko imali eyaneleyo. Kukho iimeko apho compound oyifunayo zicinezelwa ngamayeza esithatha abantu.

Umzimba olusele ngenxa yokuba sesichengeni utywala. Anaemia kunokwenzeka ngenxa Ityhefu yelothe okanye ulwamkelo antibiotics olomeleleyo. Le fom lelifa wacanda chromosome ababhinqileyo ufuzo yonakele. Kwakhona, unobangela sifo inokuba ukuphazamiseka kwenkqubo omzimba kunye nophuhliso lweenkqubo neoplastic.

Diagnosis anemia sideroblastic

Inkqubo yokuchonga esi sifo entsonkothileyo kakhulu, ekubeni uyaqhubeka phantse nazimpawu. Ngaphezu koko, kukho umfanekiso abahlukene lwezonyango, onokuwatshintsha uthembele. Ukuba ujonga kuphela iimpawu zangaphandle, uxilongo kwesi sifo ibingakholeleki. Noko ke, kukho enye indlela ukubona anemia sideroblastic - uvavanyo lwegazi.

Kwakhona benza Ukuhlolisisa amalungu angaphakathi umguli, ukufumana iidipozithi zentsimbi. Kodwa ngexesha element, ngoko ke ibangela iimpawu hematological. Ukuze aphephe iimpazamo, kuyimfuneko ukuqinisekisa uxilongo. Oku kwenziwa ngoncedo isifundo kokukhulu le umongo wethambo.

Ukuze ukuba enobungozi evela kumchiza, oko kuyimfuneko ukuze aqhube biopsy. Yiyo le nkqubo yeyona ndlela iphumelelayo ukumisela anemia sideroblastic. Kuliwa ngolu hlobo lulandelayo: biopsy pre-nebala izinto ezikhethekileyo, kwaye xa ufunyaniswe non-kuhlelwe laa mfanekiso wenziwe ngentsimbi, iikhompawundi ummeli eyahluklleyo.

yintoni ingcali ukuzilungisa ukuze?

Njengoko sele sibonile, iimpawu kwegazi sideroblastic phantse uzimisele. Lo mfanekiso kwezonyango Okungekhoyo kwakhona, yaye ngenxa ukuba kukho ubunzima obuthile. Ukuba umntu uziva ukudinwa, ubuthathaka emzimbeni, oko kudla ibhekisela therapist. Ugqirha for kwegazi okusolwayo iyithumele isigulane ukuba segazi umntu oqhuba uphando.

Okokuqala, le ngcali ifumana nemeko ngokubanzi isigulana unomdla yokuphila, imikhwa emibi, uluhlu kwezifo kunye t elinye ixesha. D. Ukuba gqirha libona kuyimfuneko, liya kubamba izifundo ezininzi. Ezi ziquka:

• igazi (kwaye ezinto);
• isibindi biopsy;
• analysis of bone umongo cell kuqamba.

Segazi uyakwazi ukukhetha ukwalathisa isigulana nezinye iingcali ukuze axilongwe epheleleyo ngakumbi. Umzekelo, ukuba ukufumana uhlobo kwegazi, kuyimfuneko ukuba babuyele imizila yemfuza. Le ugqirha kugqiba enoba kukho kubakho uhlobo ilifa anemia sideroblastic. Kwakhona kufuneka adibane ugqirha, womchamo okanye proctologist.

Iindlela zonyango

Phambi kokuba uqale unyango, kufuneka ubone enoba kukho ngenene isimemezelo yentsimbi. Ukulungiselela le njongo, desferalovuyu isampula. It ilawulwa ngenaliti intramuscular, kunye nomchamo iziphumo kufuneka kwabelwa malunga 0.5-1.1 mg zesinyithi, yaye hypochromic, gipersideremicheskoy, sideroahrestical, igazi sideroblastic - 5.10 mg.

Kubalulekile ukuphawula ukuba uhlobo ilifa zesi sifo asinyangeki. Ukuze gqiba sofuzo waqala lokuziguqula unyango lasebenzisa rhoqo ngeedosi eziphezulu vitamin B6. It ibhalwa isixa 100 mg ngamnye ngosuku. Nangona kunjalo, indlela abasabela ngayo umzimba phantse engalindelekanga. Xa unyango zenqanaba haemoglobin anemia sideroblastic kufuneka linyuke lifike kwinqanaba eziqhelekileyo kwisithuba seenyanga ezintathu. Ukuba ngeli thuba ixesha swi nga pfuna, unyango ngakumbi yanto.

Prevention and abane

Lidla esi sifo kwenzeka ngenxa yityhefu phambili. Ukuthintela oku, kufuneka kuthathwe ukujongana nalo mba. Ngexesha kuvuselelwa izindlu ubudala kufuneka athathe amanyathelo kangangoko ukuba resettle okwexeshana abantwana. Musa ukulahla okanye kungcwaba ipeyinti equlethe ukhokelo. Kungcono bawuphose umhlaba abawuphalileyo okanye ukususa kubo afana. Ukongeza, imfuneko yokuba kubekwe iliso rhoqo ikhaya kwizakhiwo, ukuthobela isakhiwo kunye nempilo nemigangatho.

Ukumphilisa ifomu ilifa kwesi sifo akunakwenzeka. Ukuze kuqinisekiswe imiphumo eyingenelo kufuneka esweni rhoqo sobalo igazi, ingakumbi kwinqanaba mpilo. Oku kuphunyezwa ngokulandela sicatshulwa rhoqo kwikhondo unyango, engaquki ukuba esi sifo ukuphuhlisa nokugcina imeko eqhelekileyo emntwini.