Menene cutar Wilson?

Kwayar halittar kwayoyin halitta tana haifar da lalacewa mai tsabta

Maganin Wilson, wanda aka fi sani da ciwon ƙwayar cuta, shine cututtukan kwayoyin halitta wanda zai haifar da haɗuwa mai yawa na jan ƙarfe a jikin. Wannan mummunar cuta ce wanda yake shafi mutum a cikin mutane 30,000. Don mutum ya shafi, dole ne ya kasance dole ne ya sami wani maye gurbin kwayoyin daga ba daya banda iyaye.

Wannan haɗakar tarawar jan karfe mafi rinjaye yana rinjayar hanta, kwakwalwa, koda, da idanu amma yana iya tasiri zuciya da kuma endocrin tsarin.

Kwayar cututtuka da cutar Wilson ta nuna a fili a farkon rayuwarsu, yawancin shekarun shekaru biyar da 35. Abubuwa da cutar zasu iya haɗawa da rashin cin hanta, matsalolin koda, kuma wani lokacin magungunan cutar neuropsychiatric.

Dalilin

Cutar cutar Wilson ita ce cutar da aka gada a cikin tsarin da aka yi wa juna . Abinda wannan ke nufi shi ne cewa iyaye biyu suna kasancewa masu sada zumunta don maye gurbin kwayoyin halitta, ko da yake bazai iya samun alamun bayyanar cutar ba ko tarihin iyali na cutar. Mutanen da suke sufuri suna iya samun hujjoji na ƙaƙafun ƙarfe na metabolism amma yawanci bai isa ya ba da izini ba.

Maganin Wilson shine daya daga cikin cututtukan kwayoyin halitta wanda ƙarfin jan jiki yake ginawa a cikin tsarin, mafi yawancin hanta a cikin hanta. Ya ƙunshi wani nau'in da ake kira ATP7B wanda jiki yayi amfani da shi don ya janye jan karfe a cikin bile . Rashin maye gurbin wannan rukuni yana hana wannan tsari kuma yana tsangwama tare da haɓakar jan karfe daga jiki.

Yayin da matakan jan ƙarfe suka fara haɗuwa da hanta, jiki zai yi kokarin karya su ta hanyar ɓoye albarkatun hydrochloric da ƙarfe mai laushi don yin amfani da kwayoyin halitta. Yawancin lokaci, wannan zai iya haifar da hanta (fibrosis), hepatitis , da kuma cirrhosis.

Saboda jan ƙarfe yana da mahimmanci ga kafawar collagen da ɗaukar baƙin ƙarfe, duk wani lalacewar wannan tsari zai iya haifar da rauni a lokacin da ya fara.

Wannan shine dalilin da ya sa cutar Wilson ta iya haifar da hepatitis a farkon shekaru uku na rayuwa da kuma cirrhosis (yanayin da yafi dacewa da tsofaffi) a matasa da matasa.

Abun hankalin cututtukan da suka shafi cutar

Alamar cututtukan cutar Wilson ta bambanta ta wurin wurin lalacewar nama. Tun da jan ƙarfe yana tarawa a cikin hanta da kuma kwakwalwa na farko, alamun cututtukan cututtuka sun kasance sun fi bayyana a cikin wadannan kwayoyin halitta.

Sakamakon farko na cutar rashin hanta yana da kama da wadanda aka gani tare da hepatitis . Ci gaban ci gaba na fibrosis zai iya haifar da yanayin da aka sani da hauhawar jini wanda tasirin jini a cikin hanta ya fara tashi. Yayin da lalacewan hanta ya ƙaru, mutum zai iya fuskanci abubuwa masu ban mamaki da kuma yiwuwar rayuwa, ciki har da zubar da ciki da hanta .

Daga cikin mafi yawan hanta-halayen bayyanar cututtuka da aka gani a cutar Wilson:

• Wulo
• Jiɗa
• Vomiting
• Rashin ci
• Muscle cramps
• Samun fata da fata ( jaundice )
• Haɗakar ruwa a kafafu ( edema )
• Haɗuwa da ruwa a ciki ( ascites )
• Gizo-gizo gizo-gizo kamar nau'i a kan fata (gizo-gizo gizo-gizo)
• Pain ko cikakke a cikin hagu na hagu na sama saboda karamin girma

• Yau da jini ko dakatar da tsabta saboda bambancin jinsin halitta

Yayin da cirrhosis yakan kasance a cikin mutanen da ke fama da cutar mai tsanani, rashin lafiya Wilson, yana da ci gaba da ci gaba da ciwon ciwon huhu (ba kamar cirrhosis da ke hade da kofiya ko ciwon hauka).

Magungunan cututtuka na jiki

Raunin hanta mai haɗari yana haɓaka da ci gaba da wani nau'i na anemia da ake kira anemia wanda yake dauke da kwayoyin cutar jini wanda za a rushe da kuma mutu. Tun da jini kwayoyin jini ya ƙunshi sau uku adadin ammonia kamar plasma (maɓallin jini), lalata wadannan kwayoyin halitta zai iya haifar da ƙaddamar da ammoniya da sauran gubobi a cikin jini.

Lokacin da waɗannan abubuwa ke cutar da kwakwalwa, mutum zai iya ci gaba da kwantar da hankali a cikin mahaifa , rashin asarar kwakwalwar aiki saboda cutar cutar hanta. Kwayar cututtuka na iya haɗawa da:

• Migraines
• Insomnia
• Rashin ƙwaƙwalwa
• Maganar zance
• Canje-canje a hangen nesa
• Matsalar motsi da asarar ma'auni
• Rashin tsoro ko damuwa
• Canje-canje na mutum (ciki har da rashin aiki da rashin adalci)
• Parkinsonism (rigidity, tashin hankali, raguwar motsi)
• Psychosis

Saboda mawuyacin tasirin wadannan bayyanar cututtuka sune yawa, rashin lafiyar Wilson ba shi da alamun bincike ne kawai a kan siffofin neuropsychiatric kawai.

Sauran cututtuka

Abun magungunan ƙarfe na jan karfe a cikin jiki zai iya shafar kai tsaye da kuma kai tsaye a shafi wasu tsarin kwayoyin halitta, da.

• Lokacin da yake faruwa cikin idanu, cutar Wilson zai iya haifar da alamar bayyanar da ake kira Kayser-Fleisher zobba. Wadannan su ne jimlar launin ruwan zinari na launin zinariya a kusa da kewaye da iris da aka lalacewa ta hanyar kwalliya na jan ƙarfe. Yana faruwa a kusan kashi 65 cikin dari na mutanen da ke fama da cutar Wilson.
• Lokacin da ke faruwa a kodan, cutar Wilson zai iya haifar da gajiya, rauni na tsoka, rikice-rikice, duwatsu na koda, da jini a cikin fitsari saboda kwayoyi masu yawa a jini. Halin zai iya haifar da ajiyar kima na kullun a cikin kodan kuma, a cikin ɓarna, raunana kasusuwa saboda redistribution da asarar alli.

Duk da yake ba a sani ba, cutar Wilson zai iya haifar da cardiomyopathy (rauni na zuciya) da kuma rashin haihuwa da kuma sake maimaitawa sakamakon sakamakon rashin lafiyar thyroid.

Sanin asali

Saboda bambancin cututtuka, cutar Wilson ta iya zama da wuya a gano asalin. Musamman ma idan bayyanar cututtuka ba ta da kyau, za a iya kuskure cutar ta hanyar kuskure ga dukan abu daga guba mai tsanani da kuma hepatitis C zuwa lupus da kwayar cutar.

Idan ana zargin cutar ta Wilson, binciken zai hada da nazari game da bayyanar cututtukan jiki tare da wasu gwaje-gwajen gwaji, ciki har da:

• Gwajin gwajin enzyme
• Yin gwajin jini don duba manyan matakan jan ƙarfe da ƙananan matakan ceruloplasmin (furotin wanda ke dauke da jan karfe tawurin jini)
• Glucose na jini don gwaje-gwaje don matakan jini
• Hanya 24 na hutawa don gwaji don matakan acidity da ƙwayoyin calcium
• Labaran da ke da tausayi don auna ma'aunin ƙarfin jan ƙarfe
• Nazarin kwayoyin gwajin don tabbatar da kasancewa na maye gurbin ATB7B

Jiyya

Sanarwar farko da cutar Wilson ta ba da ita ga mafi kyawun sakamakon. Mutanen da aka bincikar da cutar suna yawan bi da su a matakai guda uku:

1. Magunguna sukan fara ne tare da amfani da kwayoyi masu tayar da jan ƙarfe don kawar da jan ƙarfe daga tsarin. Penicillamine ne yawancin miyagun ƙwayoyi na farko. Yana aiki ne ta hanyar ɗaure da jan ƙarfe, yana barin ƙwayar ta fi sauƙi a cikin fitsari. Hanyoyi na wasu lokuta suna da muhimmanci kuma zasu iya haɗawa da rauni na tsoka, rash, da kuma haɗin gwiwa. Daga cikin wadanda ke fama da bayyanar cututtuka, kashi 50 cikin 100 zasu fuskanci mummunar damuwa da bayyanar cututtuka. A irin wannan hali, ana iya tsara kwayoyi na biyu.
2. Da zarar matakan jan ƙarfe suna da kyau, zinc za'a iya tsara shi a matsayin hanyar farfadowa. Zinc da aka yi magana a hankali yana hana jiki daga shawan jan ƙarfe. Abun ciwo shine sakamako mafi rinjaye.
3. Shirye-shiryen abinci na yau da kullum yana tabbatar da cewa ka guji yin amfani da jan ƙarfe marar kyau. Wadannan sun haɗa da irin albarkatun mai-mai-gurasar nama kamar kifi, hanta, kwayoyi, namomin kaza, 'ya'yan itatuwa masu sassauci, man shanu, da cakulan cakulan. Abubuwan da ke dauke da gwangwani, irin su multivitamins da waɗanda aka yi amfani da su na osteoporosis, na iya bukatar maye gurbin.

Mutanen da ke fama da cutar hanta mai tsanani wadanda basu kasa amsa maganin zasu iya buƙatar haɗin hanta .

> Sources:

> Borges Pinto, R .; Reis Schneider, A .; da kuma Silveira, T. "Cirrhosis a cikin yara da matasa: Wani bayyani." Jaridar Duniya na Hepatology. 2015; 7 (): 392-405.

> Patil, M .; Sheth, K .; Krishnamurthy, A. et al. "Wani Binciken da Hannun Kan Gaggawa akan Lafiya Wilson." Journal of Clinical and Experimental Hepatology. 2013; 3 (4): 321-336.