Fanconi Anemia wani nau'i ne mai gagarumar ciwo mai karfin zuciya wanda ake kira pancytopenia (ƙananan jini, jini mai launin jini, da takarda) da kuma abubuwan da ke cikin jiki. Yawancin yawanci ya fi girma a cikin Ashkenazi Yahudawa, Romawa na Spain, da kuma 'yan Afirka ta kudu ta Kudu. Bai kamata a dame shi ba tare da ciwo na Fanconi, cuta na kodan. Yawancin marasa lafiya an gano su a gaban shekaru 10.
Alamai da cututtuka na Fanconi Anemia
Fiye da kashi 50 cikin dari na marasa lafiya suna da nakasar jiki. Wadannan sun haɗa da:
- Sauyewar fata - raunuka masu tsinkaye (haske fiye da kewaye da fata) ko wuraren café-au-lait (wurare masu launin kofi)
- Kwanan lokaci
- Matsalar ƙyama ta Thumb
- Abun daji na ƙwayar cuta - ƙananan girman kai, ƙara yawan ruwa cikin kwakwalwa
- Rashin kodaya ko maras lafiya
- Abubuwa masu yawa na genitalia
- Ba da jinkiri ba
Yayin da jini yayi la'akari da shi, alamun alamun pancytopenia na iya bunkasa.
- Ƙara ƙusarwa ko zub da jini (ƙananan plalet count ko thrombocytopenia )
- Fatigue, kodadde fata ( anemia )
- Fever, cututtuka (low count count ko neutropenia )
Sanin asalin ciwon daji zai iya zama farkon gabatarwa. Mutane da ke fama da Fanconi Anemia sun kara yawan haɗari na tasowa:
- Murar cutar sankarar myeloid mai tsanani
- Myelodysplastic ciwo
- Ƙunƙarar kai da wuyansa
- Ciwon ciwon zuciya
- Wilms tumo (ciwon daji na koda)
- Gastrointestinal (GI) cututtuka)
- Vulvar da ciwon daji
Sanin asali
Hanyar da ake bincikarwa zai iya zama mai jinkiri, kamar yadda gabatarwar yakan haifar da lokaci.
Ƙididdigar ƙwararrun ƙwararrun yawanci shine alamar farko na matsalolin. Sa'an nan kuma a tsawon lokaci, ƙwayar jinin jini na ƙididdigar da ake biyo bayan anemia. Ana danganta anemia a matsayin ma'anar macrocytic cewa jinin jini ya fi girma fiye da al'ada. Wadannan binciken binciken da aka haɗu tare da ciwo na jiki wanda aka gwada a sama yana nuna Fanconi Anemia.
Magunguna tare da Fanconi Anemia suna cikin haɗarin ƙwayar cutar ciwon daji. Ciwon daji ke nunawa a lokacin tsufa na iya zama farkon gabatarwa a wasu marasa lafiya. Har ila yau, ana iya ɗaukar shi idan mutane da yawa daga wannan iyali su ci gaba da ciwon daji.
Lokacin da ake zargi da jinin cutar Fanconi, ana aika jini don gwajin gwajin gwaji na musamman da ake kira raunin chromosomal. Halin kwayoyin cutar a Fanconi Anemia ya hana chromosomes su iya gyara kansu yadda ya kamata don cin zarafin kasusuwa da ciwon daji a lokacin tsufa. Idan jarrabawar ɓarkewar chromosomal ta dace da Fanconi Anemia, ana iya aika gwajin kwayoyin. Baya ga tabbatar da ganewar asali, wannan bayanin yana ba wa iyalin ƙarin bayani game da hadarin samun ƙarin yara tare da wannan yanayin.
Gida
Fanconi Abun da aka shawo sau da yawa an saukar da shi a cikin wani abin da ya dace. Wannan yana nufin iyaye biyu su zama mai ɗaukar hoto don yaron ya kamu da cutar. A cikin wannan labari, iyaye biyu masu sufuri ne kuma suna da 1 a 4 damar samun yara tare da Fanconi Anemia. Mafi mahimmanci, an sauke shi a cikin hanyar X-linked wanda ke nufin ma'anar canji a kan X chromosome. A wannan yanayin, mahaifiyar tana iya maganin cutar ga 'ya'yanta maza.
Jiyya
Harkokin jini: Ana amfani da kwayar jini ta Red blood da kuma platinlet don sake samar da shi kamar yadda kasusuwan kasusuwa ba zai iya samar da yawan kuɗi ba. Ana amfani da karfin jini don hana bayyanar cututtuka na anemia (gajiya, gajiya) ko thrombocytopenia (zub da jini).
Oxymetholone: Oxymetholone shine maganin steroid na anabolic maganin da za'a iya amfani dashi don inganta yawan ƙwayoyin jini. An yi amfani da wannan don marasa lafiya wadanda ba su da 'yan uwantaka don su kasance masu ba da gudummawa.
Gwangwadon saɓo mai lalacewa: Tsarkewar hawan gwal na iya zama curative ga pancytopenia, amma ba ya rage yawan ciwon daji na rayuwa. Ana ganin sakamakon mafi kyau lokacin da 'yan uwan da suka dace su zama masu bayarwa.
> Source:
> Bertuch A. An ɗauke da anemia aplastic a cikin yara da matasa. A cikin: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.