ʻO ka maʻi Hyperimmunoglobulin E

He maʻi laha ʻole ʻo Hyperimmunoglobulin E maʻi, hoʻoilina hoʻoilina. ʻO ia ke kumu o nā pilikia me ka ʻili, nā lawehala, nā mumū, nā iwi, a me nā niho.

ʻO ka maʻi ʻo Hyperimmunoglobulin E i kapa ʻia ʻo Job syndrome. Ua kapa ʻia ʻo ia ma muli o ke ʻano Baibala ʻo Ioba, nona ka ʻoiaʻiʻo i hoʻāʻo ʻia e ka pilikia me ka hoʻokahe ʻana i ka ʻili a me nā pustules. Loaʻa nā kānaka me kēia ʻano i nā wā lōʻihi, nā ʻili ʻino loa.

Aia pinepine nā ʻōuli i ka wā kamaliʻi, akā no ka laha ʻole o ka maʻi, pinepine ia i mau makahiki ma mua o ka hōʻike pololei ʻana.

Hōʻike nā noiʻi hou e pili pinepine ʻia ka maʻi e ka loli (hoʻololi) i loaʻa i loko o ka Kānāwai3ʻāpana ma ka chromosome 17. Pehea e hoʻomaopopo ʻole ʻia ai kēia ʻano ʻokoʻa o nā ʻenekua. Eia nō naʻe, ʻo ka poʻe me ka maʻi i loaʻa ka pae kiʻekiʻe ma mua o ka maʻamau o kahi antibody i kapa ʻia ʻo IgE.

ʻO nā hōʻailona me:

• ʻO nā kīnā iwi a me nā niho, me nā haki a me ka nalo ʻana o nā niho pēpē
• Eczema
• Pau nā ʻili a me nā maʻi
• Hoʻopili hou i nā maʻi sinus
• Hoʻopili hou i nā maʻi lolo

E hōʻike paha kahi hōʻike kino:

• Ka ʻōwili ʻana o ke kuamoʻo (kyphoscoliosis)
• Osteomyelitis
• E hana hou i nā maʻi sinus

Ua hoʻohana ʻia nā hoʻāʻo e hōʻoia i ka ʻike.

• Helu eosinophil pau loa
• CBC me ke hoʻokaʻawale koko
• Serum globulin electrophoresis e nānā no ke kiʻekiʻe IgE koko kiʻekiʻe
• Ke hoʻāʻo genetic o Kānāwai3 ʻāpana

Hōʻike kahi hoʻokolohua maka i nā hōʻailona o ka maʻi maka maloʻo.

Hōʻike kahi x-ray umauma i nā abscesses o ka lung.

Nā hoʻokolohua ʻē aʻe i hiki ke hana ʻia:

• CT scan o ka umauma
• Nā moʻomeheu o ka pūnaewele i loaʻa i ka maʻi
• Nā hōʻike koko kūikawā e nānā i nā ʻāpana o ka ʻōnaehana pale
• X-ray o nā iwi
• CT scan o nā sinus

Hiki ke hoʻohana ʻia kahi ʻōnaehana helu e hoʻohui i nā pilikia like ʻole o Hyper IgE syndrome e kōkua i ka hana ʻana i ke kuhi.

ʻAʻohe lāʻau i ʻike ʻia no kēia maʻi. ʻO ka pahuhopu o ka mālama ʻana ka mālama ʻana i nā maʻi. Loaʻa i nā lāʻau:

• Nā Antibiotics
• Antifungal a me nā lāʻau antiviral (ke kūpono)

I kekahi manawa pono ke ʻoki kino e hoʻokahe i nā abscesses.

Hāʻawi ʻia ʻo Gamma globulin ma o kahi aa (IV) i ke kūkulu ʻana i ka ʻōnaehana pale inā loaʻa nā maʻi koʻikoʻi.

ʻO ka maʻi hyper IgE kahi ola mau loa. Pono kēlā me kēia maʻi hou i ka lāʻau.

Komo pū nā pilikia:

• Nā maʻi i hana hou ʻia
• Sepsis

Kāhea i kāu mea mālama ola inā loaʻa nā ʻōuli o Hyper IgE Syndrome.

ʻAʻohe ala kūpono e pale aku ai i ka maʻi ʻo Hyper IgE. Kōkua ka hoʻomaʻemaʻe laulā maikaʻi i ka pale ʻana i nā maʻi ʻili.

Hōʻike paha kekahi mau mea hoʻolako i nā lāʻau ʻaloʻalo pale no nā poʻe e hoʻomohala i nā maʻi he nui, keu pū me Staphylococcus aureus. ʻAʻole hiki i kēia lapaʻau ke hoʻololi i ke ʻano, akā hiki ke hoʻēmi i kāna mau pilikia.

ʻOihana maʻi; ʻO ka maʻi ʻo Hyper IgE

Chong H, Green T, Larkin A. Allergy a me nā pale maʻi. I: Zitelli BJ, McIntire SC, Nowalk AJ, eds. ʻO Zitelli lāua ʻo Davis 'Atlas o ka Pediatric Physical Diagnosis. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 4.

Hōlani SM, Gallin JI. Ka loiloi o ka mea maʻi me ka manaʻo ʻia he immunodeficiency. I: Bennett JE, Dolin R, Blaser MJ, hua pā. ʻO Mandell, Douglas, a me Bennett's Principle a me ka hoʻomaʻamaʻa ʻana i nā maʻi kūpilikiʻi. 9th ed. Piladelapia, PA: Elsevier; 2020: chap 12.

Hsu AP, Davis J, Puck JM, Holland SM, Freeman AF. ʻO ka Autosomal mana nui hyper IgE maʻi maʻi. Nā Manaʻo Gene. 2012; 6. PMID: 20301786 www.ncbi.nlm.nih.gov/pubmed/20301786. Hoʻohou ʻia Iune 7, 2012. Loaʻa iā Iulai 30, 2019.