Maʻi Alström

ʻO ka maʻi Alström kahi maʻi ʻano ʻole loa. Hoʻoholo ʻia i loko o nā ʻohana (hoʻoilina). Hiki i kēia maʻi ke alakaʻi i ka makapō, ke kuli, ka maʻi diabetes, a me ka momona.

Ua hoʻoili ʻia ka maʻi Alström ma kahi ʻano recessive autosomal. ʻO kēia ke kumu o ka hala ʻana o kou mau mākua i kahi kope o ka ʻāpana hemahema (ALMS1) i mea e loaʻa ai kēia maʻi iā ʻoe.

ʻAʻole maopopo pehea ka ʻāpana hemahema ke kumu o ka maʻi.

Kākaʻikahi loa ke kūlana.

ʻO nā ʻōuli maʻamau o kēia ʻano:

• ʻO ka makapō a hōʻino paha i ka ʻike maka i ka wā kamaliʻi
• Nā ʻāpana pouli o ka ʻili (acanthosis nigricans)
• Kuli ke kuli
• Ka hana naʻau hōʻino (cardiomyopathy), i hiki ai i ka puʻuwai puʻuwai
• Ka momona
• Holomua hakuʻala holomua
• Hoʻonui lohi
• Nā ʻōuli o ka hoʻomaka ʻana o ka wā kamaliʻi a i ʻole he ʻano diabetes ʻelua

I kekahi manawa, hiki i kēia ke hana:

• ʻO reflux Gastrointestinal
• Hypothyroidism
• Hewa ke ake
• Pālolo liʻiliʻi

E nānā ke kauka maka (ophthalmologist) i nā maka. Ua hōʻemi paha ke kanaka i ka hihiʻo.

E hana ʻia nā hoʻokolohua e nānā ai:

• Nā kō kō kō (e kuhikuhi i hyperglycemia)
• Lohe ʻana
• Hana puʻuwai
• Hana thyroid
• Nā pae Triglyceride

ʻAʻohe mālama kūikawā no kēia maʻi maʻi. ʻO ka mālama ʻana i nā ʻōuli e pili pū paha:

• ʻO ka lāʻau diabetes
• Kōkua hoʻolohe
• Lapaʻau naʻau
• Pani hone thyroid

ʻO Alström Syndrome International - www.alstrom.org

E hoʻomohala paha kēia mau mea:

• Kuli ke kuli
• Makapō paʻa paʻa
• ʻAno 2 diabetes

ʻOi aku paha ka hōʻino ʻana o ka hakuʻala a me ke ake.

ʻO nā pilikia kūpono:

• Nā pilikia mai ka maʻi kō
• ʻO ka maʻi o ke aʻa coronary (mai ka maʻi diabetes a me ke kiʻekiʻe o ke kolesterol)
• Ka luhi a me ka liʻiliʻi o ka hanu (inā ʻaʻole mālama ʻia ka hana naʻau maikaʻi ʻole)

Kāhea i kāu kahu mālama ola inā loaʻa a ʻike paha kāu keiki i nā ʻōuli o ka maʻi kō. ʻO nā ʻōuli maʻamau o ka maʻi kō e hoʻonui i ka make wai a me ka urination. E ʻimi koke i ka lāʻau inā ʻoe e manaʻo ʻaʻole hiki i kāu keiki ke ʻike a lohe paha i maʻamau.

ʻO Farooqi IS, O'Rahilly S. Genetic syndromes e pili ana i ka momona. I: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Makua a Pediatric. ʻEpekema 7 Piladelapia, PA: Elsevier Saunders; 2016: chap 28.

Freund KB, Sarraf D, Mieler WF, Yannuzzzi LA. Nā ʻōiwi chorioretinal hereditary. I: Freund KB, Sarraf D, Mieler WF, Yannuzzi LA, eds. ʻO Retina Atlas. ʻElua ed. Piladelapia, PA: Elsevier; 2017: chap 2.

ʻO Torres VE, ʻo Harris PC. Nā maʻi Cystic o ka hakuʻala. I: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. ʻO Brenner a me Rector's The Kidney. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 45.