Fanconi anemia

Fanconi anemia cuta ce mai saurin yaduwa ta hanyar dangi (gado) wanda ya fi shafar kashin ƙashi. Yana haifar da raguwar samar da kowane irin sel na jini.

Wannan shine mafi yawan nau'ikan cututtukan cututtukan jini na gado.

Fanconi anemia ya bambanta da ciwo na Fanconi, cuta mai saurin ciwan koda.

Fanconi anemia ya samo asali ne daga wata kwayar halitta da ba ta dace ba wacce ke lalata kwayoyin halitta, wanda ke hana su gyara DNA da ta lalace.

Don gadon karancin jini na Fanconi, dole ne mutum ya sami kwaya guda ta kwayar cutar da ba ta dace ba daga kowane mahaifa.

Ana yawan gano cutar a cikin yara tsakanin shekaru 3 zuwa 14.

Mutanen da ke da karancin jini na Fanconi suna da ƙananan-ƙwayoyin jini na jini, da jajayen jini, da kirinjin jini (ƙwayoyin da ke taimaka wa jini gudan).

Rashin isassun ƙwayoyin jinin jini na iya haifar da cututtuka. Rashin jajayen ƙwayoyin jini na iya haifar da gajiya (anemia).

Plateananan-yadda-al'ada na platelet na iya haifar da zubar jini da yawa.

Mafi yawan mutane masu fama da cutar Fanconi suna da wasu daga cikin wadannan alamun:

• Zuciya mara kyau, huhu, da hanyar narkewa
• Matsaloli na ƙashi (musamman kwatangwalo, kashin baya ko haƙarƙari) na iya haifar da lankwasa kashin baya (scoliosis)
• Canje-canje a launin fata, kamar wuraren duhu na fata, ana kiran su café au lait spots, da vitiligo
• Rashin ji saboda kunnuwan marasa kyau
• Matsalar ido ko fatar ido
• Kodan da basu yi daidai ba
• Matsaloli game da hannaye da hannaye, kamar ɓacewa, ƙari ko babban yatsun hannu, matsalolin hannaye da ƙashi a cikin ƙananan hannu, da ƙarami ko ɓataccen ƙashi a gaba
• Gajeren gajere
• Headananan kai
• Testananan ƙwayoyin cuta da canje-canje na al'aura

Sauran alamun bayyanar:

• Rashin cin nasara
• Illar karatu
• Weightananan nauyin haihuwa
• Rashin hankali

Gwaje-gwaje na yau da kullun don Fanconi anemia sun haɗa da:

• Gwajin kasusuwa
• Kammala ƙididdigar jini (CBC)
• Gwajin ci gaba
• Magunguna da aka kara wa samfurin jini don bincika lalacewar chromosomes
• Hanyar x-ray da sauran nazarin hoto (CT scan, MRI)
• Gwajin ji
• Rubutun nama na HLA (don nemo masu ba da gudummawar kasusuwa)
• Duban dan tayi

Mata masu juna biyu na iya samun amniocentesis ko samfur mara kyau don gano yanayin cikin ɗansu da ba a haifa ba.

Mutanen da ke da sauƙin matsakaicin matsakaitan ƙwayoyin jini waɗanda ba sa buƙatar ƙarin jini na iya buƙatar buƙatar bincike na yau da kullun da ƙididdigar jini kawai. Mai ba da kula da lafiyar zai lura da mutum sosai game da wasu cututtukan kansa. Wadannan na iya hada da cutar sankarar bargo ko cutar kansa, wuya, ko kuma tsarin fitsari.

Magungunan da ake kira abubuwan haɓaka (kamar su erythropoietin, G-CSF, da GM-CSF) na iya inganta ƙididdigar jini na ɗan gajeren lokaci.

Sanyawar kashin kashi zai iya magance matsalolin ƙididdigar jini na Fanconi anemia. (Mafi kyawun mai ba da kasusuwa na ɗan'uwana ne ko 'yar'uwa wacce nau'in kayan jikin ta yayi daidai da wanda cutar Fanconi ta shafa.)

Mutanen da suka sami nasarar ciyewar kasusuwan ƙashi har yanzu suna buƙatar dubawa na yau da kullun saboda haɗarin ƙarin ciwon daji.

Maganin Hormone hade tare da ƙananan ƙwayoyin steroid (kamar su hydrocortisone ko prednisone) an ba da umarnin ga waɗanda ba su da mai ba da kasusuwa mai ba da ƙashi. Yawancin mutane suna amsa maganin hormone. Amma duk wanda ke da cutar zai yi saurin lalacewa lokacin da aka dakatar da magunguna. A mafi yawan lokuta, waɗannan magungunan sun daina aiki.

Treatmentsarin jiyya na iya haɗawa da:

• Magungunan rigakafi (mai yuwuwa ana bayarwa ta wata jijiya) don magance cututtuka
• Karin jini don magance alamomin saboda karancin jini
• Alurar rigakafin cutar papilloma ta ɗan adam

Yawancin mutane da wannan yanayin suna ziyartar likita akai-akai, ƙwarewa wajen magance:

• Rikicin jini (masanin jini)
• Cututtukan da suka shafi gland (endocrinologist)
• Cututtukan ido (ophthalmologist)
• Cututtuka na ƙashi (orthopedist)
• Koda cuta (nephrologist)
• Cututtukan da suka shafi gabobin haihuwa da nono (likitan mata)

Matsayin rayuwa ya bambanta daga mutum zuwa mutum. Hangen nesa ba shi da kyau a cikin waɗanda ke da ƙarancin ƙidayar jini. Sababbin ingantattun jiyya, kamar su dashen kashi, wataƙila sun inganta rayuwa.

Mutanen da ke fama da cutar ƙarancin jini na Fanconi sun fi saurin haifar da nau'ikan cututtukan jini da cutar kansa. Wadannan na iya hada da cutar sankarar bargo, cutar myelodysplastic, da kansar kai, wuya, ko tsarin fitsari.

Mata masu fama da karancin jini na Fanconi waɗanda suka yi ciki ya kamata masaniya ta sa musu ido sosai. Irin wadannan matan galibi suna bukatar karin jini a duk lokacin da suke ciki.

Maza masu fama da karancin jini na jini sun rage haihuwa.

Matsalolin rashin jinin Fanconi na iya haɗawa da:

• Ciwon kashin baya
• Ciwon kansa
• Ciwon daji na hanta (duka marasa kyau da masu haɗari)

Iyalai da ke da tarihin wannan yanayin na iya samun nasiha game da kwayar halitta don fahimtar haɗarin su.

Alurar riga kafi na iya rage wasu rikice-rikice, gami da cututtukan pneumococcal pneumonia, hepatitis, da varicella infections.

Mutanen da ke da cutar shan jini ta Fanconi ya kamata su guji abubuwan da ke haifar da cutar kansa (carcinogens) kuma a riƙa duba su akai-akai don auna cutar kansa.

Fanconi ta rashin jini; Anemia - Fanconi's

• Abubuwan da aka kafa na jini

Dror Y. Ciwon cututtukan ɓarkewar kasusuwa. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 29.

Lissauer T, Carroll W. Rashin lafiyar Haematological. A cikin: Lissauer T, Carroll W, eds. Littafin rubutu na likitan yara. 5th ed. Philadelphia, PA: Elsevier; 2018: babi na 23.

Vlachos A, Lipton JM. Ciwon kashin baya. A cikin: Lanzkowsky P, Lipton JM, Kifi JD, eds. Littafin Lanzkowsky na ilimin likitan yara da Oncology. Na 6 ed. Philadelphia, PA: Elsevier; 2016: babi na 8.