Progeria

Progeria ndi chibadwa chosowa chomwe chimabweretsa ukalamba mwachangu mwa ana.

Progeria sikupezeka kawirikawiri. Ndizodabwitsa chifukwa zizindikiro zake zimafanana kwambiri ndi ukalamba wamunthu, koma zimachitika mwa ana ang'onoang'ono. Nthawi zambiri, sizimaperekedwa kudzera m'mabanja. Simawoneka kawirikawiri mwa ana opitilira m'modzi m'banja.

Zizindikiro zake ndi izi:

• Kukula kwakukula mchaka choyamba cha moyo
• Nkhope yopapatiza, yopindika kapena makwinya
• Kusamala
• Kutaya nsidze ndi nsidze
• Msinkhu waufupi
• Mutu wawukulu kukula kwa nkhope (macrocephaly)
• Tsegulani malo ofewa (fontanelle)
• Nsagwada yaying'ono (micrognathia)
• Wouma, wansalu, khungu lowonda
• Kuyenda kocheperako
• Mano - kuchedwa kapena kusapezeka

Wopereka chithandizo chamankhwala ayesa thupi ndikukonzekera mayeso a labotale. Izi zitha kuwonetsa:

• Kukaniza kwa insulin
• Khungu limasintha mofanana ndi lomwe limawoneka mu scleroderma (minofu yolumikizana imakhala yolimba ndi yolimba)
• Nthawi zambiri cholesterol ndi triglyceride milingo

Kuyesedwa kwa kupsinjika kwa mtima kumatha kuwulula zizindikilo za atherosclerosis yoyambirira yamitsempha yamagazi.

Kuyesedwa kwa majini kumatha kuzindikira kusintha kwa jini (LMNA) zomwe zimayambitsa progeria.

Palibe mankhwala enieni a progeria. Mankhwala a aspirin ndi statin atha kugwiritsidwa ntchito kuteteza motsutsana ndi vuto la mtima kapena sitiroko.

Progeria Research Foundation, Inc. - www.progeriaresearch.org

Progeria imayambitsa kufa msanga. Anthu omwe ali ndi vutoli nthawi zambiri amakhala ndi moyo mpaka zaka zawo zaunyamata (zaka zapakati pazaka 14). Komabe, ena amatha kukhala ndi zaka zoyambira 20. Choyambitsa imfa nthawi zambiri chimakhudzana ndi mtima kapena sitiroko.

Zovuta zingaphatikizepo:

• Matenda a mtima (m'mnyewa wamtima infarction)
• Sitiroko

Itanani omwe akukuthandizani ngati mwana wanu sakuwoneka kuti akukula kapena kukula bwino.

Hutchinson-Gilford progeria matenda; HGPS

• Mitsempha ya Coronary imatseka

Gordon LB. Hutchinson-Gilford progeria matenda (progeria). Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 109.

Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford progeria matenda. Zowonjezera. 2015: 1. PMID: 20301300 www.ncbi.nlm.nih.gov/pubmed/20301300. Idasinthidwa pa Januware 17, 2019. Idapezeka pa Julayi 31, 2019.