ʻO ka maʻi maʻi Ectopic Cushing

ʻO ka Ectopic Cushing syndrome kahi ʻano o Cushing Syndrome kahi tumo ma waho o ka pituitary gland e hana i kahi hormone i kapa ʻia he adrenocorticotropic hormone (ACTH).

ʻO Cushing Syndrome kahi maʻi e kū i ka wā i kiʻekiʻe ai kou kino ma mua o ka pae maʻamau o ka hormone cortisol. Hana ʻia kēia hormona i loko o nā adrenal glands. Hiki i ka nui o ka cortisol ma muli o nā pilikia like ʻole. ʻO kekahi o nā kumu inā nui ka nui o ka hormone ACTH i loko o ke koko. Hana ʻia ʻo ACTH e ka pituitary i nā mea liʻiliʻi a laila hōʻailona i nā gland adrenal e hana i ka cortisol. I kekahi manawa hiki i nā hunaola ʻē aʻe ma waho o ka pituitary ke hana i nā nui he nui o ACTH. Kapa ʻia kēia ʻo ectopic Cushing Syndrome. Ectopic ʻo ia hoʻi ke hana nei kekahi mea i kahi wahi kūpono ʻole i ke kino.

Hoʻokumu ʻia ka Ectopic Cushing syndrome e nā tumors e hoʻokuʻu iā ACTH. Hiki i nā tumors ke, i nā manawa maʻamau, hoʻokuʻu iā ACTH ke:

• ʻO nā tumors carcinoid Benign o ka māmā
• ʻO nā kūpuna pūnaewele Islet o ka pancreas
• Ka maʻi kanesa o ka thyroid
• ʻO nā tumele pūnaewele liʻiliʻi o ka akemāmā
• Nā Tumors o ka gland timus

Hiki i ka Ectopic Cushing maʻi ke kumu i nā ʻōuli like ʻole. Loaʻa i kekahi poʻe nā ʻōuli he nui a ʻo kekahi he kakaikahi wale nō. ʻO ka hapanui o ka poʻe me kekahi ʻano o Cushing syndrome:

• Poepoe, ʻulaʻula, a helehelena piha (maka maka)
• Lohi ka ulu ʻana o nā keiki
• Loaʻa kaupaona me ka hōʻiliʻili momona ma ke kumu, akā lilo ka momona mai nā lima, nā wāwae, a me nā puʻupuʻu (ka momona nui)

Hoʻololi ka ʻili i ʻike pinepine ʻia:

• Nā maʻi ʻili
• ʻO nā māka poni (1/2 iniha 1 kenimika a ʻoi ākea paha) i kapa ʻia ʻo striae ma ka ʻili o ka ʻōpū, nā ʻūhā, nā lima luna, a me nā umauma
• ʻIli lahilahi me ka palapū maʻalahi

Hoʻololi kaʻiʻo a me ka iwi i:

• Backache, i kū me nā hana maʻamau
• ʻEha iwi a palupalu paha
• ʻOhi o ka momona ma waena o nā poʻohiwi a ma luna o ka iwi kola
• Nā ʻiwi a me nā iwi o ka iwi i hana ʻia e ka lahilahi o nā iwi
• Nā nāwaliwali nāwaliwali, ʻo ka puhaka a me nā poʻohiwi

Hiki i nā pilikia ākea o ke kino (ʻōnaehana) ke:

• ʻAno 2 diabetes mellitus
• Piʻi koko kiʻekiʻe
• Kolesterol kiʻekiʻe a me nā triglycerides
  

Loaʻa paha i nā wahine:

• ʻO ka ulu nui o ka lauoho ma ka maka, ka ʻāʻī, ka umauma, ʻōpū, a me nā ʻūhā
• Nā wā i kūleʻa a kū hoʻi

Loaʻa paha i nā kāne:

• Hoemi a ʻaʻohe makemake i ka moekolohe
• Impotence

ʻO nā ʻōuli ʻē aʻe e kū pū paha me:

• Hoʻololi manaʻo, e like me ke kaumaha, hopohopo, a i ʻole nā ​​loli i ka lawena
• Ka luhi
• ʻEha poʻo
• Hoʻonui i ka make wai a me ka urination

E hana ka mea mālama ola i kahi hoʻokolohua kino a nīnau e pili ana i kāu mau hōʻailona.

ʻO nā hoʻāʻo e hana ʻia ana paha:

• 24-hola mimi laʻana e ana i ka pae o ka cortisol a me ka creatinine
• Nā ho'āʻo koko e nānā iā ACTH, cortisol, a me nā pae potassium (haʻahaʻa pinepine i ka ectopic Cushing Syndrome)
• ʻO ka hoʻāʻo suppression Dexamethasone (ʻo ke kiʻekiʻe a me ka haʻahaʻa.
• ʻImi haʻahaʻa petrosal sinus sampling (kahi hoʻāʻo kūikawā e ana iā ACTH mai nā veins kokoke i ka lolo a me ka umauma)
• Hoʻouka kōmiʻo
• MRI a me nā kiʻekiʻe kiʻekiʻe CT scan e ʻike ai i ka tumo (i kekahi manawa pono pono ʻia kahi scans lāʻau nukelea)

ʻO ka lapaʻau maikaʻi loa no ka ectopic Cushing Syndrome ka ʻoki kino e hemo ai i ka tumo. Hiki ke ʻoki kino i ka manawa noncancerous o ke tumo (benign).

I kekahi mau hihia, maʻi ʻaʻai ka tumo a pāhola ʻia i nā wahi ʻē aʻe o ke kino ma mua o ka hiki ʻana i ke kauka ke ʻike i ka pilikia me ka hana ʻana o cortisol. ʻAʻole hiki ke kaha ʻana ma kēia mau hihia. Akā hiki i ke kauka ke kuhikuhi i nā lāʻau e ālai i ka hana cortisol.

I kekahi manawa pono ka hemo ʻana o nā kaha adrenal ʻelua inā ʻaʻole hiki ke ʻike ʻia ka tumo a ʻaʻole paʻa pono nā lāʻau i ka hana cortisol.

ʻO ke ʻoki ʻana e hemo ai i ka tumo hiki i ke ola piha. Akā aia kahi manawa e hoʻi hou ka tumo.

Hoʻolahalaha a hoʻi paha ka tumo ma hope o ke kahakaha ʻana. Hiki ke hoʻomau i kahi pae cortisol kiʻekiʻe.

Kāhea i kāu mea hāʻawi inā hoʻomōhala ʻoe i nā ʻōuli o Cushing syndrome.

ʻO ka mālama koke ʻana i nā tumors e hōʻemi paha i ka makaʻi i kekahi mau hihia. ʻAʻole hiki ke pale ʻia nā hihia he nui.

Cushing syndrome - ectopic; ʻO ka maʻi maʻi ectopic ACTH

• Nānā Endocrine

Nieman LK, Biller BM, Findling JW, a me al. Hoʻomaʻamaʻa ʻana i ka maʻi o Cushing: kahi papa hana hoʻomaʻamaʻa ʻo Endocrine Society. ʻO J Clin Endocrinol Metab. 2015; 100 (8): 2807-2831. PMID 26222757 www.ncbi.nlm.nih.gov/pubmed/26222757.

ʻO Stewart PM, Newell-Kumukūʻai JDC. ʻO ka Cortex adrenal. I: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. ʻO Williams Textbook of Endocrinology. 13th ed. Piladelapia, PA: Elsevier; 2016: chap 15.