ʻO Glanzmann thrombasthenia

ʻO Glanzmann thrombasthenia kahi maʻi laha ʻole o nā pā koko. ʻO Platelet kahi ʻāpana o ke koko e kōkua i ke kāpili ʻana o ke koko.

Hoʻokumu ʻia ʻo Glanzmann thrombasthenia e ka nele o kahi protein i maʻa mau ma luna o ka platelet. Pono kēia mea no nā platelet e hoʻopili pū e hana i nā clots koko.

ʻO ke ʻano he congenital, ʻo ia hoʻi aia ia mai ka wā hānau. Nui a hewahewa nā genetics i hiki ke kumu i ke ʻano.

Pākuʻi ʻia paha nā ʻōuli i kekahi o kēia mau mea:

• Kahe nui ke kahe i ka wā a ma hope o ke kaha ʻana
• Pepeiao koko
• Māmā maʻalahi
• Kahe nui ka haehae
• Nā Nosebleeds e kū mālie ʻole
• ʻO ke kahe lōʻihi ʻana me nā ʻeha liʻiliʻi

Hiki ke hoʻohana ʻia nā hoʻokolohua aʻe e kuhikuhi ai i kēia ʻano:

• Helu piha koko (CBC)
• Nā hoʻāʻo hōʻuluʻulu platelet
• Nānā i ka hana platelet (PFA)
• Ka manawa Prothrombin (PT) a me ka manawa thromboplastin hapa (PTT)

Pono paha nā hoʻokolohua ʻē aʻe. Pono paha e hoʻāʻo ʻia nā lālā o ka ʻohana.

ʻAʻohe mālama kūikawā no kēia maʻi. Hiki ke hāʻawi ʻia i nā transfusions platelet i ka poʻe e nui ana ke kahe ʻana o ke kahe.

ʻO nā hui aʻe nā kumuwaiwai maikaʻi no ka ʻike ma Glanzmann thrombasthenia:

• Ka Pūnaewele ʻIke Genetic and Rare Diseases (GARD) - rarediseases.info.nih.gov/diseases/2478/glanzmann-thrombasthenia
• National Organization for Rare Disorder (NORD) - rarediseases.org/rare-diseases/glanzmann-thrombasthenia

ʻO Glanzmann thrombasthenia kahi ola āpau, a ʻaʻohe lāʻau. Pono ʻoe e hana i nā hana kūikawā e hoʻāʻo ai e hōʻalo i ke kahe ʻana o ke koko inā loaʻa iā ʻoe kēia ʻano

ʻO kēlā me kēia mea me ka maʻi koko e hōʻalo i ka lawe ʻana i ka aspirin a me nā lāʻau anti-inflammatory nonsteroidal (NSAIDs) e like me ibuprofen a me naproxen. Hiki i kēia mau lāʻau ke hoʻolōʻihi i nā manawa kahe ma ke kaohi ʻana i nā platelet mai ka ʻōpala ʻana.

Komo pū nā pilikia:

• Kahe nui ke koko
• ʻO ka anemia hemahema o ka hao ma nā wahine menstruating ma muli o ke kahe koko kaumaha

Kāhea i kāu kahu mālama ola inā:

• Loaʻa ʻoe i ke kahe a i ʻole ka ʻeha o kahi kumu ʻike ʻole
• ʻAʻole kū ka holo ʻana ma hope o ka hoʻomaʻamaʻa maʻamau

ʻO Glanzmann thrombasthenia kahi kūlana hoʻoilina. ʻAʻohe pale e ʻike ʻia.

Maʻi ʻo Glanzmann; ʻO Thrombasthenia - Glanzmann

ʻO Bhatt MD, Ho K, Chan AKC. Nā maʻi o ka coagulation i ka neonate. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 150.

ʻO Nichols WL. ʻO ka maʻi ʻo Von Willebrand a me nā hemorrhagic abnormalities o ka platelet a me ka vascular function. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 173.