Mafi yawancin rinjayar jarirai
A cikin Blackfan Diamond (ko Diamond Blackfan) anemia, ɓawon jiki na jiki yana samar da ƙwayoyin jini kadan ko a'a. Blackfan Diamond anemia yana rinjayar kusan 600 zuwa 700 mutane a dukan duniya. Ba'a sani dalili ba, kodayake kuskuren kwayoyin halitta a cikin kwayar halittar da ake kira RPS19 a kan chromosome 19 an hade da kimanin kashi 25 cikin dari. A cikin kimanin 10% zuwa 20% na lokuta, akwai tarihin iyali na rashin lafiya.
Cutar cututtuka
Blackfan Diamond anemia ya kasance a lokacin haihuwar amma zai iya zama da wuya a gano. A kusan kashi ɗaya cikin uku na yara da aka haifa tare da cutar, akwai lahani na jiki irin su nakasar hannu ko lahani na zuciya, amma ba a gano alamun alamun bayyanannu ba. Kwayoyin cututtuka na iya bambanta ƙwarai, daga mummunan hali mai tsanani da kuma barazanar rai.
Kwayoyin jini suna ɗauke da oxygen a cikin jiki, don haka yaro tare da Blackfan Diamond na iya samun alamun bayyanar da ba su da isasshen jini oxygen (anemia):
- pallor (kariya)
- ƙwaƙwalwar ajiyar zuciya, saboda zuciya yana ƙoƙarin kiyaye oxygen motsi cikin jiki
- gajiya, rashin jin daɗi, da kuma raunana .
Sanin asali
Blackfan Diamond anemia yawanci ana bincikarsa a cikin shekaru biyu na rayuwa, wani lokaci har ma a haihuwar, bisa ga alamun bayyanar. Alal misali, ana iya ɗaukakar jaririn da ciwon anemia idan yana ko da yaushe kodayake kuma ya rage numfashi lokacin shan kwalban ko jinya.
Iyaye suna tsammanin akwai "wani abu ba daidai ba" tare da yaro. Ba za a gane ganewar asali na anjamau na Blackfan Diamond ba, saboda cutar ta zama rare sosai kuma 'yan likitoci sun saba da shi.
Kwayoyin jini (CBC) cikakke don jariri zai nuna yawan ƙananan jinin jini da kuma rashin haemoglobin low.
Wani gwajin jini zai nuna adenosine deaminase (ADA). Wani samfurin kasusuwa (biopsy) zai nuna cewa an halicci wasu sabon jini a jikin jini.
Jiyya
Hanya na farko na magani don cutar ta Blackfan Diamond shi ne ya ba da yarinyar maganin maganin steroid, yawancin lokaci. Kimanin kashi 70% na yara tare da Blackfan Diamond anemia za su amsa wannan magani, inda magani ya zuga samar da ƙarin jinin jini. Duk da haka, wannan yana nufin cewa yaron zai dauki magani na steroid don sauran rayuwarsa, wanda yake da mummunan illa mai lalacewa irin su ciwon sukari , glaucoma , rauni na kasuwa ( osteopenia ), da hawan jini . Har ila yau, magani zai iya ba da daɗewa ba da aiki ga mutumin a kowane lokaci.
Idan wani bai amsa maganin steroid ba ko kuma yana buƙatar tsayin daka don kiyaye kwayar cutar jini ta jini, magani zai zama jini. Hanyoyin jini na yau da kullum zai samar da jini mai yaduwar jini amma kuma zai kai ga baƙin ƙarfe a jiki . Yawanci, jiki yana amfani da baƙin ƙarfe yayin yin sabon jini, amma tun da mutumin da ke dauke da Blackem Diamond anemia ba sa yawancin sel, ƙarfin yana ƙaruwa. Mutumin yana buƙatar shan magani wanda yake dauke da baƙin ƙarfe daga jiki.
Gurasar da ake samu don Blackfan Diamond anemia shine kututture ne kawai , wanda ya maye gurbin sashin jiki na ɓarkewar mutumin da mai laushi lafiya. Duk da haka, aikin dasa shi ne hanya mai wuyar tafiya ta hanyar kuma bata koyaushe aiki ba. Yawanci ana adanawa ne ga mutanen da masu maganin magunguna da jini ba su taimaka ba.
> Sources:
> Guinan, EC. "Sanin asali da Gudanarwa na Aplastic Anemia." ASH Education Book , 2011, 76-81.
> Ƙungiyar Ƙungiya ta Ƙananan Raunuka. "Blackfan Diamond Anemia." Rahoton Raunuka Rare, 2008.
> Birtaniyar Birtaniya Black Black. "Mene ne Diamond Blackfan Muriya?" 2008.