ʻO ka hoʻohaʻahaʻa Hepatocerebral

Hepatocerebral degeneration kahi maʻi lolo e kū i ka poʻe me ka hōʻino o ke ake.

Hiki i kēia ʻano ke loaʻa i kekahi hihia o ka loaʻa ʻole o ke ake, e like me ka maʻi hepitis nui.

Hiki i ka hōʻino o ke ake ke alakaʻi i ke kūkulu ʻana o ka amonia a me nā mea ʻona ʻē aʻe i loko o ke kino. Hana kēia inā ʻaʻole holo pono ke ake. ʻAʻole ia e wāwahi a hoʻopau i kēia mau kemika. Hiki i nā mea ʻona ke hoʻopōʻino i kaʻiʻo o ka lolo.

ʻO nā ʻāpana kikoʻī o ka lolo, e like me ka ganglia basal, e hōʻeha ʻia no ka holo ʻole o ke ake. Kōkua ka ganglia basal i ka kaohi ʻana i ka neʻe. ʻO kēia ʻano ke ʻano "non-Wilsonian". ʻO ke kumu o ka hōʻino ʻia o ke akepaʻa ʻaʻole ia e kumu ʻia e nā waihona keleawe i ke ake. ʻO kēia kahi hiʻohiʻona nui o ka maʻi ʻo Wilson.

Pākuʻi ʻia paha nā ʻōuli:

• Ke hele wāwae paʻakikī
• Ka hana naʻau ʻino ʻole
• ʻO Jaundice
• Pā spasm (myoclonus)
• ʻOʻoleʻa
• Lulu o nā lima, poʻo (haʻalulu)
• Pākuʻi
• Nā neʻe ʻana o ke kino i kāohi ʻole ʻia (chorea)
• Hele wāwae ʻole ʻole (ataxia)

Aia nā hōʻailona me:

• Koma
• Fluid i loko o ka ʻōpū i kumu i ka pehu (ascites)
• ʻO ke kahe Gastrointestinal mai nā veins i hoʻonui ʻia i ka paipu meaʻai (varises esophageal)

Hiki i kahi ʻōnaehana ʻōnaehana (neurological) ke hōʻike i nā hōʻailona o:

• Dementia
• Nā neʻe manawaleʻa
• Ke hele paʻa ʻole

Hōʻike paha nā hoʻokolohua Laboratory i kahi pae amonia kiʻekiʻe i ke kahe o ke koko a me ka hana ate ʻole maʻamau.

Hiki i nā hoʻokolohua ʻē aʻe ke:

• MRI o ke poʻo
• EEG (e hōʻike i ka lohi o ka nalu ʻana o ka lolo)
• CT scan o ke poʻo

Kōkua ka lāʻau i ka hoʻohaʻahaʻa ʻana i nā kemika ʻino e kūkulu aʻe mai ka holo pono ʻole o ke ake. Pākuʻi ʻia paha ia i nā antibiotic a i ʻole kahi lāʻau e like me ka lactulose, kahi e hoʻohaʻahaʻa ai i ke kiʻekiʻe o ka amonia i loko o ke koko.

ʻO kahi lapaʻau i kapa ʻia ʻo ka chanched-chain amino acid therapy kekahi:

• Hoʻonui i nā hōʻailona
• Poino lolo hoʻohuli

ʻAʻohe lāʻau lapaʻau kūikawā no ka maʻi neurologic maʻi, no ka mea, he kumu ia no ka hōʻino ʻia o ke ake. Hiki ke hoʻōla i ke akepaʻa i ka maʻi ate. Eia naʻe, ʻaʻole hiki i kēia hana ke hoʻohuli i nā ʻōuli o ka lolo.

ʻO kēia kahi wā lōʻihi (mau) i hiki ai ke alakaʻi i nā ʻōuli ʻōnaehana i hiki ʻole ke hoʻololi ʻia (neurological).

E hoʻomau paha ke kanaka i ka ʻoi aku ka maikaʻi a make me ka loli ʻole o ke ake. Inā hana wili ʻia kahi transplant, hiki ke hoʻihoʻi ʻia ka maʻi neurological.

Komo pū nā pilikia:

• Koma Hepatic
• Poino lolo nui

Kāhea i kāu mea mālama ola inā loaʻa iā ʻoe nā ʻōuli o ka maʻi ate.

ʻAʻole hiki ke pale aku i nā ʻano maʻi āpau. Eia nō naʻe, hiki ke pale ʻia ka hepatitis ʻona a me viral.

E hoʻemi i kou makaʻu i ka loaʻa ʻana i ka wai ʻona a i ʻole viral hepatitis:

• Hōʻalo i nā hana hoʻoweliweli, e like me ka hoʻohana ʻana i ka lāʻau IV a i ʻole ka wahine pale ʻole ʻia.
• Mai inu, a inu paha i ka hoʻohaʻahaʻa.

Loaʻa kūlohelohe (Non-Wilsonian) hepatocerebral degeneration; Hepatic encephalopathy; ʻO ka encephalopathy Portosystem

• Anatomia akepau

Garcia-Tsao G. Cirrhosis a me kona hopena. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 153.

Haq IU, Tate JA, Siddiqui MS, Okun MS. ʻO ka nānā ākea o nā maʻi neʻe.I: Winn HR, hoʻoponopono. ʻO Youmans a me Winn Neurological Surgery. ʻEpekema 7 Piladelapia, PA: Elsevier; 2017: chap 84.