Progeria

Progeria waa xaalad dhif ah oo hidde ah oo soo saarta gabow dhaqso leh carruurta.

Progeria waa xaalad naadir ah. Waa wax cajiib ah maxaa yeelay astaamaheeda ayaa si aad ah ugu eg gabowga caadiga ah ee aadanaha, laakiin waxay ku dhacdaa carruurta yaryar. Xaaladaha badankood, looma sii gudbiyo qoysaska. Waa dhif in lagu arko in kabadan hal cunug oo qoys ah.

Calaamadaha waxaa ka mid ah:

• Kobaca koritaanka inta lagu jiro sanadka koowaad ee nolosha
• Weji cidhiidhi ah, cidhiidhi ah ama duuduuban
• Bidaarta
• Lumidda sunnayaasha iyo baarkooda
• Dherer Gaaban
• Madax weyn oo cabirka wejiga ah (macrocephaly)
• Meel jilicsan fur (fontanelle)
• Daanka yar (micrognathia)
• Qallalan, xoqan, maqaarka khafiifka ah
• Dhaqdhaqaaq xadidan
• Ilkaha - habsaamid ama maqnaansho

Bixiyaha xanaanada caafimaadka wuxuu sameyn doonaa baaritaan jireed wuxuuna amri doonaa baaritaano shaybaar. Tani waxay muujin kartaa:

• Caabbinta insulin
• Isbeddelada maqaarku waxay la mid yihiin tan lagu arkay scleroderma (unugyada isku xirnaanta ayaa adkaada oo adkaada)
• Heerarka guud ahaan caadiga ah ee kolestaroolka iyo triglyceride

Tijaabada buufiska wadnaha ayaa laga yaabaa inay muujiso calaamadaha atherosclerosis ee xididdada dhiigga ee hore.

Tijaabinta hiddo-wadaha ayaa lagu ogaan karaa isbeddelada hidda-wadahaLMNA) oo sababa progeria.

Ma jiro daaweyn gaar ah oo loogu talagalay progeria. Dawada Aspirin iyo statin ayaa loo isticmaali karaa si looga hortago wadno qabad ama istaroog.

Aasaaska Cilmi-baarista Progeria, Inc. - www.progeriaresearch.org

Progeria wuxuu sababa geeri hore. Dadka xaaladdan qaba badiyaa waxay noolaadaan oo keliya sannadaha dhallinyarnimada (celcelis ahaan cimrigooda oo ah 14 sano). Si kastaba ha noqotee, qaarkood waxay ku noolaan karaan da'da 20-aad. Sababta dhimashada badanaa waxay la xiriirtaa wadnaha ama istaroog.

Dhibaatooyinka waxaa ka mid noqon kara:

• Wadne qabad (wadnaha oo yaraada)
• Faalig

Wac daryeel bixiyahaaga haddii ilmahaagu uusan u muuqan inuu korayo ama u kobcayo si caadi ah.

Hutchinson-Gilford progeria syndrome; HGPS

• Xididdada halbowlayaasha wadnaha

Gordon LB. Hutchinson-Gilford progeria syndrome (progeria). Gudaha: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Buugga Buugga Caafimaadka Caruurta. 21aad ed. Philadelphia, PA: Elsevier; 2020: cutubka 109.

Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford progeria cillad. GeneReviews. 2015: 1. PMID: 20301300 www.ncbi.nlm.nih.gov/pubmed/20301300. La cusbooneysiiyay Janaayo 17, 2019. La helay July 31, 2019.