Ke maʻi mau nei kāu keiki? Hiki paha iā ia ke ʻano laha ʻole o ka WHIM Syndrome?

Loaʻa pinepine anei i kāu keiki ke anu, ka ʻū, ka maʻi pepeiao, a i ʻole nā ​​maʻi ʻili? E hoʻomaikaʻi anei kekahi maʻi ma mua o kekahi? ʻO ka maʻamau, manaʻo mākou ua haʻahaʻa iki ka pale ʻana o ke keiki a ke loaʻa nei iā ia nā maʻi ma ke kula a ma nā wahi pāʻani. Eia nō naʻe, i nā hihia kakaikahi loa, hiki ke hana ʻia kēia e kahi ʻano genetic kikoʻī i kapa ʻia ʻo WHIM Syndrome . ʻOiai ʻaʻole i kamaʻilio nui ʻia kēia, he mea nui loa ia e makaʻala i ia mea.

He aha kēia WHIM syndrome?

I ka ʻōlelo maʻalahi, he maʻi genetic kakaikahi loa ka WHIM syndrome e hoʻopilikia ana i ka ʻōnaehana pale o ko mākou kino. Ua loaʻa ka inoa WHIM mai nā leka mua o nā hōʻailona nui ʻehā o ka maʻi.

• Nā warts (nā warts)
• Hypogammaglobulinemia (haʻahaʻa nā pae o nā antibodies i loko o ke koko)
• Nā maʻi (nā maʻi pinepine)
• ʻO ke kahe koko Myeloalveolar (ka paʻa ʻana o nā keʻena koko keʻokeʻo i loko o ka iwi iwi)

E noʻonoʻo i ko mākou kino he pūʻali koa o nā hunaola koko keʻokeʻo. ʻO ka hana a kēia mau koa, ʻo ia ke kaua aku i nā ʻenemi e like me nā bacteria a me nā virus e hoʻoulu ai i nā maʻi. I loko o kekahi me ka maʻi WHIM, ʻo nā koa koʻikoʻi loa o kēia pūʻali koa, ʻo nā hunaola koko keʻokeʻo i kapa ʻia ʻo neutrophils , e paʻa i loko o ko lākou kumu, ka iwi iwi. ʻAʻole hiki iā lākou ke puka i waho, i loko o ke koko, e hakakā i nā maʻi. No laila, ua nāwaliwali ka ʻōnaehana pale o ke kino, a hoʻomaka lākou e maʻi pinepine.

He aha nā hōʻailona o ka WHIM syndrome?

Hiki ke ʻokoʻa loa kēia mau hōʻailona mai kēlā kanaka a kēia kanaka. Loaʻa i kekahi poʻe nā hōʻailona palupalu loa. Hiki i kekahi ke loaʻa i nā maʻi koʻikoʻi e hiki ke hoʻoweliweli i ke ola. Hoʻomaka pinepine kēia mau hōʻailona i ka wā kamaliʻi, akā i kekahi manawa hiki ke ʻike ʻole ʻia ka maʻi a hiki i ka wā makua.

ʻOi loa aku e pili ana i nā warts a me ka pilikia o ka maʻi kanesa

Hiki i nā poʻe he nui a puni mākou ke loaʻa i ka maʻi papillomavirus kanaka (HPV), ka mea e hoʻoulu ai i nā warts. Eia nō naʻe, i loko o ke kanaka me kahi ʻōnaehana pale olakino, nalowale koke kēia maʻi mai ke kino. Eia nō naʻe, no ka mea ua nāwaliwali ka ʻōnaehana pale o ke kanaka me ka WHIM syndrome, he paʻakikī ke hakakā i kēia maʻi HPV. No laila, noho ka maʻi i loko o ke kino no ka manawa lōʻihi, e hoʻoulu ai i nā warts, a i kekahi mau hihia, e hoʻonui ana i ka pilikia o ka maʻi kanesa.

He aha ke kumu o kēia? He mea laha anei kēia?

ʻO ke kumu nui o ka maʻi WHIM he mutation i loko o ka gene i kapa ʻia ʻo `CXCR4` i loko o ko mākou kino. E noʻonoʻo i kēia gene ma ke ʻano he polokalamu e kuhikuhi ana i kā mākou mau cell. Ke loli kēia gene, ua pau nā cell koko keʻokeʻo i ʻōlelo ʻia ma luna (Neutrophils) i ka puka ʻana mai loko mai o ka iwi iwi.

No ka mea, he maʻi hoʻoilina kēia, ʻaʻole hiki ke laha mai ke kanaka a i ke kanaka ma o ka ʻū ʻana a i ʻole ka hoʻopā ʻana . Eia nō naʻe, hiki ke hoʻoili ʻia mai nā mākua a i nā keiki. Inā loaʻa i kekahi o nā mākua kēia hoʻololi ʻana o ka gene, loaʻa i kēlā me kēia keiki a lākou he 50% ka manawa e hoʻoilina ai i ke maʻi .

Pehea ʻoe e ʻike pololei ai i kēia maʻi?

No ka mea he maʻi laha ʻole kēia, he paʻakikī ke ʻike me hoʻokahi wale nō hoʻāʻo. E nānā pono kāu kauka iā ʻoe a me nā hōʻailona o kāu keiki, pehea ka pinepine o ka loaʻa ʻana o nā maʻi, a laila e paipai paha i kekahi mau hoʻāʻo.

• Helu Koko Piha (CBC): Nānā kēia i nā hunaola koko keʻokeʻo haʻahaʻa, ʻoiai nā neutrophils, i loko o ke koko.
• Hoʻāʻo antibody (hoʻāʻo koko Immunoglobulin G - IgG):Hana ʻia kēia hoʻāʻo e ʻike inā haʻahaʻa nā pae o nā antibodies e hakakā nei i ka maʻi.
• Biopsy o ka iwi iwi: Ma kēia kaʻina hana, lawe ʻia kahi hāpana liʻiliʻi o ka iwi iwi mai ka iwi ʻūhā ma lalo o ka anesthesia māmā a nānā ʻia ma lalo o kahi microscope. Hiki i kēia ke hoʻoholo pololei inā ua paʻa nā hunaola koko keʻokeʻo i loko o ka iwi iwi (myelokathexis).
• Hoʻāʻo ʻana i ka Genetic: Hiki i kēia hoʻāʻo ke hōʻoia inā he mutation i loko o ka gene `CXCR4` e hoʻoulu ai i ka WHIM syndrome.

He aha nā lāʻau lapaʻau no ka WHIM syndrome?

ʻAʻohe lāʻau lapaʻau no kēia maʻi i kēia manawa. Eia nō naʻe, aia kekahi mau lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, ​​​​pale i nā maʻi, a kōkua iā ʻoe e ola i kahi ola maʻamau.

Nā ʻano lāʻau lapaʻau

• Xolremdi: He lāʻau lapaʻau hou kēia i ʻāpono ʻia i ka makahiki 2024. Kōkua ia i ka hoʻokuʻu ʻana i nā hunaola koko keʻokeʻo i paʻa i loko o ka iwi iwi. Hiki i kēia ke hōʻemi i ka pilikia o ka maʻi ma kahi o 40%.
• Lapaʻau G-CSF: He lāʻau pana kēia. Hoʻoulu ia i ka iwi iwi a kōkua iā ia e hana i nā hunaola koko keʻokeʻo hou aʻe.
• Lapaʻau IgG: No ka poʻe me nā pae antibody haʻahaʻa, hoʻoikaika ʻia ka ʻōnaehana pale ma o ka lawelawe ʻana i nā antibodies mai waho.
• Nā lāʻau antibiotic: Ke ulu mai nā maʻi bacteria (e like me ka pneumonia, nā maʻi ʻili), e kuhikuhi kāu kauka i kēia mau mea e kāohi iā lākou.

Lapaʻau no nā warts

Inā loaʻa pinepine nā ʻūlū, hiki iā ʻoe ke ʻike i kahi kauka ʻili e wehe iā lākou. Aia kekahi mau ʻano hana no ka hana ʻana pēlā.

• ʻO ke ʻoki kino
• ʻOki uila
• Lapaʻau laser
• Nā ʻano o nā uhi kūikawā

Pehea e ola olakino ai me ka WHIM syndrome?

I ke ola ʻana me ia ʻano maʻi laha ʻole, pono ʻoe e noʻonoʻo hou aku e pili ana i kou olakino.

• ʻAi maikaʻi: He mea nui loa ka ʻai momona no ka hoʻoikaika ʻana i kou ʻōnaehana pale. E hoʻokomo i nā mea kanu, nā huaʻai, nā palaoa piha, ka iʻa, a me ka moa i kāu papaʻai.
• Hoʻoikaika Kino: Kōkua ka hoʻoikaika kino i ka hoʻoikaika kino e hoʻoikaika i ke kino a hoʻonui i ka palekana. E hana i kahi mea āu e makemake ai, e like me ka haʻuki, ka hele wāwae, a i ʻole ka ʻauʻau ʻana, i kēlā me kēia lā.
• Ka palekana mai nā maʻi:
• E hōʻalo i nā wahi piha kanaka i ka hiki.
• E holoi pinepine i kou mau lima me ke kopa.
• E kamaʻilio me kāu kauka a hoʻoholo i nā lāʻau pale e pono ai ʻoe a me kou ʻohana.E kamaʻilio kikoʻī e pili ana i nā mea e like me ka lāʻau pale HPV, ka lāʻau pale niumonia, a me ka lāʻau pale maʻi palū makahiki.

Ma waho aʻe o kāu kauka ʻohana, pono paha ʻoe i ke kōkua o nā kauka like ʻole, e like me kahi immunologist, he hematologist, a me kahi dermatologist, e mālama iā ʻoe.