Ciwon Progeria ko Hutchinson-Gilford

Ciwon Progeria ko Hutchinson-Gilford

Progeria cuta ce da ba a saba ganin irinta ba ta halin tsufa na yaro.

Ma'anar progeria

Progeria, wanda kuma aka sani da cutar Hutchinson-Gilford, cuta ce da ba a saba gani ba. An halin ta ƙara tsufa na jiki. Yaran da ke da wannan cutar suna nuna alamun farkon tsufa.

A lokacin haihuwa, yaron ba ya gabatar da wasu abubuwan da ba su dace ba. Yana bayyana “al'ada” har zuwa jariri. Sannu a hankali, ilimin halittar jikinsa da jikinsa suna haɓaka ba kamar yadda ya saba ba: yana girma ƙasa da sauri fiye da yadda aka saba kuma baya samun nauyin yaro na shekarunsa. A fuska, ci gaban kuma yana jinkirta. Yana gabatar da fitattun idanu (cikin walwala, ci gaba mai ƙarfi), hanci mai kauri da ƙugi, leɓan bakin ciki, ƙaramin ƙamshi da kunnuwa masu fitowa. Progeria kuma shine sanadin babban asarar gashi (alopecia), tsufa fata, raunin haɗin gwiwa, ko ma asarar yawan kitse na subcutaneous (kitse na subcutaneous).

Haɓaka hazaƙa da hazaƙan yaro gaba ɗaya baya shafar su. Ainihin rashi ne da sakamako akan haɓaka motsi, yana haifar da wahala a zaune, tsaye ko ma tafiya.

Marasa lafiya da ciwon Hutchinson-Gilford suma suna da taƙaitaccen wasu jijiyoyin, wanda ke haifar da ci gaban atherosclerosis (toshewar jijiyoyin jijiyoyin jini). Koyaya, arteriosclerosis shine babban abin da ke haifar da haɗarin haɗarin bugun zuciya, ko ma Hadarin Cerebral Vascular (bugun jini).

Yaduwar wannan cuta (adadin mutanen da abin ya shafa a cikin jimillar yawan jama'a) ya kai jarirai miliyan 1/4 a duk duniya. Saboda haka cuta ce da ba a saba gani ba.

Tunda progeria cuta ce da ta mamaye gadon sarauta, mutanen da ke cikin haɗarin kamuwa da irin wannan cutar sune waɗanda iyayen su ma ke da progeria. Hakanan haɗarin maye gurbi na kwayoyin halitta yana yiwuwa. Progeria na iya shafar kowane mutum, koda cutar ba ta cikin mahallin iyali.

Sanadin progeria

Progeria wata cuta ce da ba a saba gani ba da kuma kwayoyin halitta. Asalin wannan cututtukan ya samo asali ne saboda maye gurbi a cikin kwayar halittar LMNA. Wannan kwayar halittar tana da alhakin samar da furotin: Lamine A. Na karshen yana taka muhimmiyar rawa wajen samuwar kwayar halitta. Yana da mahimmanci a cikin samuwar ambulan nukiliya (membrane da ke kewaye da ƙwayoyin sel).

Halittar kwayoyin halitta a cikin wannan kwayar halittar tana haifar da samuwar Lamine A. Wannan furotin da ba a saba gani ba shine asalin rashin kwanciyar hankalin kwayar halitta, da kuma farkon mutuwar kwayoyin halittar kwayoyin halitta.

Masana kimiyya a halin yanzu suna tambayar kansu game da batun, domin samun ƙarin cikakkun bayanai kan shigar da wannan furotin cikin ci gaban halittar ɗan adam.

Kwayar cutar cututtukan Hutchinson-Gilford tana faruwa ne ta hanyar gado na autosomal. Ko dai watsa daya daga cikin kwafi biyu na takamaiman kwayar halitta (ko daga uwa ko daga uba) ya wadatar don cutar ta bunkasa a cikin yaron.

Bugu da ƙari, maye gurbi (ba sakamakon watsa kwayoyin halittar iyaye ba) na ƙwayar LMNA na iya kasancewa a asalin irin wannan cutar.

Alamomin progeria

Alamun gabaɗaya na progeria sun haɗa da:

  • farkon tsufa na jiki (tun yana ƙanana);
  • ƙarancin nauyi fiye da na al'ada;
  • ƙaramin girman yaro;
  • mabanbanta fuska: wani siriri, ƙugiya hanci, fitattun idanu, ƙaramin ƙamshi, kunnuwa masu fitowa, da sauransu;
  • jinkirin mota, yana haifar da wahala a tsaye, zaune ko ma tafiya;
  • raguwa da arteries, babban haɗarin haɗarin arteriosclerosis.

Abubuwan haɗari don progeria

Tun da progeria ba kasafai yake faruwa ba, kwayoyin halittu da cututtukan da suka mamaye autosomal, kasancewar cutar a cikin ɗayan iyayen biyu shine mafi mahimmancin haɗarin.

Wane magani ga progeria?

Alamomin da ke da alaƙa da progeria suna ci gaba kuma suna iya haifar da mutuwar mai haƙuri.

Babu maganin cutar a halin yanzu. Iyakar abin da ake sarrafa progeria shine na alamomi.

Sannan bincike yana cikin haske, domin gano maganin da ke ba da damar maganin irin wannan cuta.

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