Hōʻike koko Porphyrins

Kōkua ʻo Porphyrins i nā mea nui i loko o ke kino. ʻO kekahi o kēia he hemoglobin. ʻO kēia ka protein i nā hunaola ʻulaʻula e lawe i ka oxygen i loko o ke koko.

Hiki ke ana ʻia nā porphyrins i ke koko a i ka mimi paha. Kūkākūkā kēia ʻatikala i ka hoʻāʻo ʻana i ke koko.

Pono kahi laʻana koko.

Hoʻokomo ʻia ka laʻana i loko o ka hau a lawe koke ʻia i ke keʻena hoʻokolohua. ʻEkolu mau porphyrins hiki ke ana ʻia i nā mea liʻiliʻi i ke koko kanaka. O lākou:

• ʻO Coproporphyrin
• Protoporphyrin (PROTO)
• Uroporphyrin

ʻIke ʻia ʻo Protoporphyrin i ka nui loa. Pono nā ho'āʻo hou aʻe e hōʻike i nā pae o nā porphyrins kikoʻī.

ʻAʻole ʻoe e ʻai no 12 a 14 mau hola ma mua o kēia hōʻike. Hiki paha iā ʻoe ke inu wai ma mua pono o ka hoʻāʻo. E loli paha kāu mau hopena hōʻike inā ʻaʻole ʻoe e mālama i kēia mau ʻōlelo aʻo.

Ke hoʻokomo ʻia ke kui kele e huki i ke koko, manaʻo kekahi poʻe i ka ʻeha kaulike. ʻIke wale kekahi i kahi ʻōniʻu a i ʻole kui. Ma hope, loaʻa paha i kahi kūpikipiki a i ʻole ʻeha iki. Hele koke kēia.

Hoʻohana ʻia kēia hōʻike e hōʻike ai i nā porphyrias. ʻO kēia kahi hui o nā maʻi maʻamau i hele pinepine ʻia i waena o nā lālā o ka ʻohana.

Hiki nō ke hoʻohana pū ʻia me nā hoʻokolohua ʻē aʻe e kuhikuhi i ka lāʻau make kēpau a me kekahi ʻōnaehana hopohopo a me nā maʻi o ka ʻili.

Ana kēia kikoʻī i nā pae porphyrin āpau. Akā, nā helu kūmole (kahi laulā o nā waiwai i ʻike ʻia i kahi hui o nā kānaka olakino) no nā mea ponoʻī pū kekahi.

• Huina pae porphyrin: 0 a 1.0 mcg / dL (0 a 15 nmol / L)
• Papa Coproporphyrin: 2 mcg / dL (30 nmol / L)
• Pākuʻi Protoporphyrin: 16 a 60 mcg / dL (0.28 a 1.07 µmol / L)
• Pae Uroporphyrin: 2 mcg / dL (2.4 nmol / L)

Hiki ke loli iki nā pae waiwai maʻamau i waena o nā hale hana ʻokoʻa. Hoʻohana kekahi mau labs i nā ana like ʻole a i ʻole nā ​​hoʻāʻo i nā laʻana like ʻole. E kamaʻilio me kāu kauka e pili ana i ke ʻano o kāu mau hopena hōʻike kikoʻī.

ʻO nā pae i hoʻonui ʻia o nā coproporphyrins he hōʻailona paha o:

• ʻO porphyria erythropoietic Congenital
• Hepatic coproporphyria
• Anemia Sideroblastic
• Pepeia porphyria

ʻO kahi pae protoporphyrin hoʻonui ʻia he hōʻailona paha o:

• Anemia o ka maʻi maʻi
• Protoporphyria erythropoietic Congenital
• Hoʻonui i nā erythropoiesis
• Maʻi kīnā
• Anemia hemahema hao
• Lāʻau make kēpau
• Anemia Sideroblastic
• Thalassemia
• Pepeia porphyria

ʻO kahi pae uroporphyrin i hoʻonui ʻia he hōʻailona paha ia o:

• ʻO porphyria erythropoietic Congenital
• ʻO Porphyria cutanea tarda

ʻAʻohe pilikia iki i ka lawe ʻia ʻana o kou koko. ʻOkoʻa nā aʻa a me nā aʻa i ka nui mai kekahi kanaka a i kekahi, a mai kekahi ʻaoʻao o ke kino i kekahi ʻē aʻe. ʻOi aku ka paʻakikī o ka loaʻa ʻana o kahi laʻana koko mai kekahi poʻe ma mua o nā poʻe ʻē aʻe.

ʻO nā pilikia ʻē aʻe e pili ana i ke kau ʻana o ke koko he liʻiliʻi, akā hiki ke hoʻopili ʻia:

• Kahe nui ke koko
• Pāleʻale a ʻoluʻolu paha
• Nā punctures he nui e ʻimi i nā uaua
• Hematoma (hōʻiliʻili koko ma lalo o ka ʻili)
• Maʻiʻaʻai (i iki kamepiula i kēlā me kēia manawa ka ʻili i haki)

Nā pae Protoporphyrin; Porphyrins - huina; Nā pae Coproporphyrin; Hōʻike PROTO

• Hōʻaʻo koko

Chernecky CC, Berger BJ. Porphyrins, helu - koko. I: Chernecky CC, Berger BJ, hua. Nā Hoʻokolohua Laboratory a me nā kaʻina hana Diagnostic. ʻElua huaʻōlelo St Louis, MO: Elsevier Saunders; 2013: 891-892.

ʻO Fuller SJ, Wiley JS. Heme biosynthesis a me kāna mau maʻi: porphyrias a me sideroblastic anemias. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 38.