Progeria

Progeria o se seasea tupuʻaga kenera tulaga e maua ai vave matutua i tamaiti.

Progeria o se tulaga seasea tupu. E ofoofogia aua o ona faʻailoga e foliga tutusa lava ma le masani o tagata matutua, ae e tupu i tamaiti laiti. I le tele o tulaga, e le pasiina i totonu o aiga. E seasea vaʻaia i le sili atu ma le tasi tamaititi i se aiga.

Faʻailoga aofia ai:

• Tuputupu aʻe le lelei i le taimi o le muamua tausaga o le olaga
• Vaapiapi, maanuminumi pe maanuminumi foliga
• Sasae
• Leiloa o mata ma mata mata
• Tulaga puupuu
• Ulu lapoʻa mo le tele o foliga (macrocephaly)
• Tatala vaivai nofoaga (fontanelle)
• Auvae laiti (micrognathia)
• Paʻu mago, paʻu, manifinifi
• Faatapulaʻa tulaga o lafo
• Nifo - tolopo pe le toe faʻavaeina

O le tausi soifua maloloina o le a faia se faʻataʻitaʻiga tino ma okaina falesuesue suʻega. Atonu e faʻaalia lenei:

• Teteʻe inisalini
• Paʻu suia tutusa ma lena vaaia i scleroderma (o le fesoʻotaʻiga aʻa avea maʻaʻa ma faʻamaʻaʻaina)
• Masani masani kolesterol ma triglyceride tulaga

Ole faʻamaʻi ole fatu ole fatu e ono faʻaalia ai ni faʻailoga o le amataga o le atherosclerosis o le toto.

Ole suʻega ole kenera e mafai ona iloa ai suiga ile gafa (LMNA) e mafua ai progeria.

E leai se faʻapitoa togafitiga mo progeria. Aspirin ma statin vailaʻau mafai ona faʻaaogaina e puipuia ai mai se faʻamaʻi fatu po o le stroke.

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

Progeria mafua ai le vave maliu. O tagata e maua i le maʻi e masani ona naʻo o latou tausaga talavou e ola ai (averesi le umi o le 14 tausaga). Ae ui i lea, o nisi e mafai ona ola i le amataga o le 20s. O le mafuaʻaga o le oti e masani ona fesoʻotaʻi ma le fatu poʻo le faʻamaʻi.

Faʻafitauli e ono aofia ai:

• Fatu fatu (myocardial infarction)
• Pa

Valaʻau lau tautua pe a fai o lau tama e le foliga mai o loʻo tupuola pe faʻasolosolo masani.

Hutchinson-Gilford progeria syndrome; HGPS

• O le poloka o alatoto toto

Gordon LB. Hutchinson-Gilford progeria syndrome (progeria). I le: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Tusitusiga o Pediatrics. 21 ed. Filatelefia, PA: Elsevier; 2020: chap 109.

Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford progeria syndrome. GeneReviews. 2015: 1. PMID: 20301300 www.ncbi.nlm.nih.gov/pubmed/20301300. Faʻafou Ianuari 17, 2019. Avanoa ia Iulai 31, 2019.