ʻO Paroxysmal anuanu hemoglobinuria (PCH)

ʻO Paroxysmal cold hemoglobinuria (PCH) kahi maʻi koko laha ʻole kahi e hana ai ka ʻōnaehana pale kino i nā mea ʻino e luku ai i nā hunaola ʻulaʻula. Hana ʻia ia ke hōʻike ʻia ke kanaka i nā mahana anuanu.

Kū wale ʻo PCH i ke anuanu, a pili pinepine i nā lima a me nā wāwae. Hoʻopili nā Antibodies (hoʻopaʻa) i nā hunaola ʻulaʻula. ʻAe kēia i nā protein ʻē aʻe i loko o ke koko (i kapa ʻia ʻo ke kākoʻo) e hoʻopaʻa pū hoʻi. Hoʻopau nā mea ʻino i nā hunaola ʻulaʻula ke neʻe lākou i loko o ke kino. I ka luku ʻia ʻana o nā hunaola, hoʻokuʻu ʻia ka hemoglobin, ka ʻāpana o nā hunaola ʻulaʻula e lawe ana i ka oxygen, i loko o ke koko a hala i ka mimi.

Ua hoʻopili ʻia ʻo PCH i nā syphilis ʻelua, syphilis tertiary, a me nā maʻi maʻi viral a bacteria paha. I kekahi manawa ʻaʻole maopopo ke kumu.

Kākaʻikahi ka maʻi.

Pākuʻi ʻia paha nā ʻōuli:

• ʻO Chills
• Fiva
• ʻEha kua
• ʻEha wāwae
• Abdominal eha
• ʻEha poʻo
• General discomfort, uneasiness, a maʻi maʻi paha (malaise)
• Koko i ka mimi (mimi ʻulaʻula)

Hiki i nā hoʻokolohua Laboratory ke kōkua i ke kuhi ʻana i kēia ʻano.

• ʻO ke kiʻekiʻe Bilirubin kiʻekiʻe i ke koko a me ka mimi.
• Kuhi piha ke koko (CBC) hōʻike i ka anemia.
• ʻAʻole maikaʻi ka hoʻāʻo ʻana o Coombs.
• He maikaʻi ka hoʻāʻo Donath-Landsteiner.
• He kiʻekiʻe ka pae dehydrogenase lactate.

Hiki ke kōkua i ka mālama ʻana i ke kūlana kumu. ʻO kahi laʻana, inā pili ka PCH e syphilis, hiki ke hoʻomaikaʻi ʻia nā ʻōuli ke mālama ʻia ka syphilis.

I kekahi mau hihia, hoʻohana ʻia nā lāʻau e kāohi i ka ʻōnaehana pale.

ʻO ka poʻe me kēia maʻi e hoʻomaikaʻi wikiwiki a loaʻa ʻole nā ​​hōʻailona ma waena o nā hanana. I ka hapanui o nā hihia, pau nā hoʻouka ke pau nā neʻe o nā hunaola i ka neʻe ʻana i loko o ke kino.

Komo pū nā pilikia:

• Nā hoʻouka hoʻomau
• Hewa ʻole nā ​​hakuʻala
• Anemia ʻaʻano koʻikoʻi

Kāhea i kāu mea mālama ola inā loaʻa nā ʻōuli o kēia maʻi. Hiki i ka mea hāʻawi ke kāpae i nā kumu ʻē aʻe o nā hōʻailona a hoʻoholo inā pono ʻoe i ka lāʻau.

ʻO ka poʻe i ʻike ʻia me kēia maʻi hiki ke pale i nā hoʻouka kaua e hiki mai ana ma ka noho ʻana i waho o ke anuanu.

PCH

• Nā pūnaewele koko

ʻO Michel M. Autoimmune a me nā anemias hemolytic intravascular. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 151.

Lanakila N, Richards SJ. Loaʻa i nā anaemias haemolytic. I: Bain BJ, Bates I, Laffan MA, eds. ʻO Dacie lāua ʻo Lewis Hematology Pono. 12th ed. Piladelapia, PA: Elsevier; 2017: chap 13.