ʻAʻole nui ka poʻe i ʻike he ʻokoʻa ka maʻamau o nā ʻano ka maʻi maʻamau a me nā lākiʻi 2 mellitus, aia nō kekahi maʻi diabetes insipidus. ʻO kēia kahi maʻi o nā kelopa endocrine, ʻo ia kahi maʻi o ka mana o ka hypothalamic-pituitary. No laila, ʻaʻole e pili ka maʻi like ʻole i ka maʻi maʻi, koe wale nō ka inoa a me ka makewai mau.
Me ka diabetes insipidus, ua ʻike ʻia kahi hapa ʻole a me ke kūpaʻa piha o ka antidiuretic hormone vasopressin. Hoʻopili i kēlā me ke kaha o ka osmotic a me nā hale kūʻai, a laila hoʻomoʻe i ka wai ma loko o ke kino holoʻokoʻa.
No laila, hāʻawi ka hormone i ka nui o ka wai e pono ai, e ʻae ana i nā keiki e hana maʻamau. No laila, pono ʻo vasopressin no ka homeostasis kūlohelohe, no ka mea e hōʻoia ana i kāna hana maʻamau a me ka nele o ka māla ʻana i ke kino.
Ma kahi kūlana koʻikoʻi, no ka laʻana, i ka wā dehydration, loaʻa i ka lolo kahi hōʻailona e hoʻoponopono i ka hana ʻana o nā huina. Hāʻawi kēia i ka hōʻemi o ka neʻe ma ka hoʻemi ʻana i ke kahe o ka lawena a me ka urine.
No laila, ʻokoʻa ka diabetes insipidus mai ka kō i loko o ia ka neʻe ʻana o kona kaila o ka glucose i ke koko, akā ke ʻano mau nā maʻi ʻelua - polydipsia (makehewa nui). No laila, loaʻa i ka diabetes insipidus, ka mea i hōʻailona ʻia e ka hoʻoliʻi ʻana o ka wai mai loko o nā ʻoloka o nā keiki, ua loaʻa kēia inoa.
Hoʻomaʻamaʻa pinepine ke papa o ND. Ua manaʻo ʻia ʻo ia he maʻi o nā ʻōpio, no laila ʻo ka makahiki o nā mea maʻi a hiki i ka 25 makahiki. Eia nō naʻe, hiki ke hoʻokuʻi ʻia i nā lepa endocrine i nā kāne a me nā kāne.
Hoʻopili ʻana i nā maʻi mellitus: ʻano
Aia ka maʻi a me ka nephrogenic diabetes insipidus. ʻO LPC, ma ka huli ʻana, ua māhele ʻia i 2 mau ʻano:
- hana;
- kūlohelohe.
ʻO ka ʻano hana hana i helu ʻia he ʻano idiopathic. ʻO nā mea i hoʻopilikia ʻia i ka nānā ʻana o kēia ʻano ʻano ʻaʻole i hoʻokū piha ʻia, akā nui ka poʻe kauka e manaʻo nei he nui ka mana o ka moʻomeheu i ka hoʻomohala ʻia o ka maʻi. Eia kekahi, ʻo nā kumu ke waiho i kahi ʻāpana ʻokoʻa o ka synthesis o ka neurophysin hormone a i ʻole vasopressin.
Hōʻike ʻia ke ʻano pilikino o ka maʻi ma hope o nā hōʻeha ʻana, nā hana ʻeha a me nā mea ʻē aʻe.
Hoʻokomo ka diabetes insipidus diabetes i ka wā o ka hana maoli o ke keiki i ka kīnā. I kekahi mau hihia, aia kahi hemahema o ka hoʻonaninani o ka osmotic o nā tubula renal, ma nā kūlana ʻē aʻe, i ka emi ʻana o ka tubula e hele i ka vasopressin.
Aia kekahi ʻano hewa e like me ka polydipsia psychogenic. Hiki ke hoʻowalewale ʻia i ka hōʻino ʻana i ka lāʻau lapaʻau a i ʻole PP kekahi o nā hōʻike o ka schizophrenia.
Kēia mau ʻano leʻia o ND e like me ke ʻano gestagen a me nā polyuria transient. I ka hihia mua, he ikaika loa ka enzyme pletenta, ka hopena i ka hopena antidiuretic.
Kūkulu ʻia kahi ala o ka maʻi maʻi ma mua o ka makahiki o ka makahiki 1.
Hana ʻia kēia i ka wā e hoʻomau ʻia ka lolo, i ka wā i hoʻomaka ai nā mākaʻikaʻi e hoʻopili i ka hana pānaʻi.
Nā kumu a me nā hōʻailona o ka maʻi
Aia nā mea he nui e alakaʻi i ka hoʻomohala ʻana i ka maʻi insipidus maʻi:
- nā hana ʻōpū;
- nā maʻi a me nā maʻi maʻi (postpartum sepsis, flu, syphilis, typhoid, scarlet fever a me nā mea ʻē aʻe);
- radiation;
- jade;
- hana ʻino i nā kīʻaha koko a me nā ʻāpana o ka lolo;
- ʻeha a hōʻeha paha ma ka lolo;
- amyloidosis;
- granulomatosis;
- hemoblastosis.
ʻO nā maʻi Autoimmune a me nā maʻi psychogenic e kōkua pū i ka hoʻomakaʻana o ND. A me ke ʻano o ka idiopathic o ka maʻi, ke kumu o ka loaʻa ʻana i ka helehelena o ka helehelena ka nānā ʻana o nā antibodies e kū ana i nā ʻenehana hoʻohālikelike.
He ʻokoʻa nā kiʻi lapaʻau ʻo ka maʻi insipidus o ka maʻi maʻi, e hoʻomaka me ka hōʻeha a me ka hopena me ka dehydration i ka loaʻa ʻole o ka hoʻokomo o ka nui o ka nui o ka wai. No laila, ʻokoʻa i ka nānāʻana, ua hoʻokō ʻia nā ʻano hōʻike like ʻole no ka maʻi insipidus maʻi.
ʻO nā hōʻailona nui o ka maʻi ka:
- ka hanaʻole i ka digestive tract - constipation, gastritis, colitis, ʻona maikaʻi ʻole;
- makewai ikaika;
- hana hoʻopili;
- nā manaʻo noʻonoʻo - maikaʻi ka hiamoe, huhū, hōʻeha, hōʻeha;
- ka hoʻomehana pinepine ʻana me ka nui kope kope o ka wai excreted (6-15 lita);
- ka maloʻo ʻana o nā membrane a me ka ʻili;
- ka ʻike maka ʻana i ka maʻi diabetes;
- lilo ka kaumaha;
- anorexia;
- ʻālaʻaka aseic.
Hoʻohui pinepine ʻia nā mea maʻi diabetes insipidus i ka hoʻonui ʻia ʻana o ka naʻau a me ka hoʻohaʻahaʻa ʻana i ka luhi. Eia nō hoʻi, inā ʻaʻole inu nui ka mea maʻi i ka wai e nui, a laila e pōʻino nō kona kūlana. A ke hopena, hiki i ka mea maʻi ke hoʻomohala i kēlā mau hōʻike e like me ke uhui ʻana o ke koko, ka luaʻi ʻana, ka ʻōpū, ke tachycardia, ke ʻala, a me ka hiwa o ka maʻi o ka maʻi. I nā wahine me ND, hele hewa ke alualu ʻana i nā kāne.
I nā keiki, hiki i ka papa o ka maʻi ke alakaʻi i ka hoʻolohi ʻana i ka ulu kino a me ke ʻano.
Naʻi hoʻolika
No ka ʻike ʻana i ka hiki ʻana o ND, ua hōʻike ʻia kahi hōʻike diagnostic ʻekolu paleka.
- ka ʻike ʻana i ka polyuria hypotonic (urinalysis, hoʻokolohua Zimnitsky, ʻike ʻia ke koko biochemical);
- nā ho'āʻo kūpono (hoʻokolohua desmopressin, maloʻo);
- ka ʻike o nā kumu i hoʻonāukiuki i ka hoʻomakeʻe ʻana o ka maʻi (MRI).
Hana mua
I ka mua, inā ʻaihue ʻia ka maʻi diabetes, e hoʻāʻo ʻia ka maʻi diabetes e hoʻoholo i ka kūlike o ka urine. ʻOiaʻiʻo, me ka maʻi o ka maʻi, ke hana nei ka hana o nā keiki, ma muli o ka hopena, nā hōʻailona ma ke ʻano o ka urine ma mua o 1005 g / l.
E ʻike ai i ka pae o ka hoʻonaʻauao ma ke ao, aʻo ʻia kahi hōʻike ma Zimnitsky. Hoʻopili ʻia kēlā ʻano hōʻike i kēlā me kēia ʻekolu mau hola no 24 mau hola. I kēia manawa hana, ua lawe ʻia nā hiʻohiʻona 8 o ka urine.
Ma ka maʻamau, ʻo nā hualoaʻa i kuhi ʻia i kēia ala: ʻaʻole ka nui o nā maʻamau o ka urine i kēlā me kēia lā he 3 mau lita, ʻo kona kū pono loa he 1003-1030, aʻo ka pālahalaha o ka pō a me ka hopena o ka urine he 1: 2, a ʻo ka nui o ka wai i hoʻopuka a inu i ka 50-80-100%. Pāʻai urine - 300 mosm / kg.
Eia kekahi, hoʻomaʻamaʻa ʻia kahi hōʻike koko biochemical e hōʻike ai i ka ND. I kēia hihia, helu ʻia ke osmolarity o ke koko. Inā he kiʻi nui o nā paʻakai ma ka plasma o nā mea o ka 292 mosm / l a me kahi kikoʻi nui o ka sodium (mai ka 145 nmol / l), kahi maʻi maʻi maʻi insipidus.
Lawe ʻia ke koko mai kahi vein ma kahi ʻōpū ʻole. Ma mua o ke kaʻina (6-12 mau hola) hiki iā ʻoe ke inu i ka wai wale nō. ʻO ke kānāwai, pono ke kali i nā hopena o nā hoʻokolokolo.
Eia kekahi, me ka noiʻi ʻana biochemical o ke koko, i ka nui o:
- kekuhi
- paʻakai a me ka sodium;
- huina piha protein, e komo ai i ka hemoglobin;
- pākana ʻula;
- mea hana;
- hormonalin parathyroid;
- aldosterone.
ʻO ka ʻōlelo holi koko o ka maʻamau a hiki i ka 5.5 mmol / l. Eia nō naʻe, me ka ND, pinepine ka piʻi ʻana o ka glucose. Akā hiki ke hoʻomaopopo i kona mau liuliu me ke kaumaha a i ke kaumaha paha i ke kino, nā maʻi o ka pancreas, pheochromocytoma a me nā maʻi maʻi o ka aʻalolo a me nā hemahema. ʻO ka emi ʻana o ke kō o ka honi e loaʻa me ka mau hana hewa ma ka hana o nā kipi endocrine, ka pōloli, ka ʻūhā a me ka hihia o ka hoʻohāinu koʻikoʻi.
ʻO ka paʻakai a me ka sodium nā mea ʻenemi i hoʻopau i nā membran cell me nā waiwai uila. ʻO ka umauma potassium maʻamau ʻo 3.5 - 5.5 mmol / L. Inā kiʻekiʻe loa kona hōʻailona, a laila ʻike kēia i nā kakiki a me ka adrenal insufficiency, poʻomanaʻo a me ka dehydration. ʻIke ʻia nā papa pākaha haʻahaʻa i ka wā wikiwiki, pilikia pilikia, ʻoi aku ka nui o kekahi mau hormones, dehydration, a me ka cystic fibrosis.
ʻO ke kumu o ka sodium i loko o ke kahawai koko mai 136 a 145 mmol / l. Hoʻokomo ka Hypernatremia me ka hana nui o ka paʻakai paʻakai, nele i ka pauna wai-paʻakai, hyperfunction o ka adrenal cortex. A hele mai ʻo hyponatremia me ka hoʻohana ʻana i ka nui o ka wai o ka nui a ma ka hihia o nā pathologies o ke akeʻa a me nā kelepaʻi adrenal.
Hōʻike ka loiloi no ka huina piha i ka pae o ka albumin a me ka globulin. ʻO ka hōʻailona maʻamau o ka nui o ka protein i loko o ke koko no nā pākeke he 64-83 g / l.
ʻO ka mea nui nui ma ka maʻi maʻi o ka maʻi insipidus e pili ana i ka hemoglobin glycosylated. Hōʻike ka Ac1 i ka awelika koko koko ma mua o 12 mau hebedoma.
ʻO Hemoglobin kahi mea i loaʻa i loko o nā kōpona koko ʻula e hāʻawi i ka oxygen i nā iwi āpau a me nā ʻōnaehana. I loko o nā poʻe ʻaʻole e ʻeha nei i ka maʻi diabetes, ka glycosylated hemoglobin i ke koko ʻaʻole iʻoi aku i ka nui o 4-6%, ʻo ia hoʻi kahi ʻano o ka maʻi insipidus maʻi. No laila, hiki i nā ʻōmua Ac1 overestimated hiki ke hoʻokaʻawale i kēia mau maʻi.
Eia nō naʻe, hiki i nā lūlū i nā pae he hemoglobin me ka anemia, ka hoʻohana ʻana o ka hoʻohui i nā meaʻai, ka hoʻokomo ʻana o nā huaora E, C a me ka nui o ka kolamu. Eia kekahi, loaʻa i ka hemoglobin glycosylated e loaʻa i nā hōʻailona like ʻole i nā maʻi o ka pūpū a me nā pūpū.
ʻO ka pae o ka calcium o ka ionized he hōʻailona e pili ana i ka minuke metabolism. Loaʻa kona mau helu waiwai mai ka 1.05 a i ka 1.37 mmol / L.
Eia kekahi, nā hoʻokolohua no ka maʻi maʻi maʻi insipidus pili i kahi hōʻike o ke koko no ka ʻike o ald testosterone. ʻO ke kaohi o kēia hāneʻe hōʻike pinepine i ka hele mai o ka maʻi insipidus maʻi.
ʻO kahi hoʻonui nui o ka creatinine a me nā hormone parathyroid hiki ke hōʻike i ka noho o ka maʻi.
Hana ʻelua
I kēia papa hana, pono e huki i kahi protocol hoʻokolohua me ka hōʻike maloʻo. Pākuʻi ʻia ke ʻano o ka huhū:
- hoʻāʻo ʻia ke koko e nānā ai i nā pae osmolality a me ka paʻakai;
- lawe i ka urine e hoʻoholo i kona nui a me ka osmolality;
- ke kaupaona ʻana;
- ka helu ʻana o ka puʻuwai naʻau a me ke koko.
Eia nō naʻe, me ka hypernatremia, ua hoʻohālikelike ʻia nā hōʻike.
He mea kūpono ia i ka wā o ka hoʻāʻo, ʻaʻole hiki iā ʻoe ke ʻai i ka meaʻai wikiwiki-calcium me kahi kiʻekiʻe glycemic index. Hāʻawi ʻia ka makemake i ka iʻa, ka ʻiʻo momona, nā hua i kuke ʻia, ka palaoa palaoa.
Ua hooki ʻia ka maloʻo inā: ʻo ka osmolality a me ka pae o ka sodium ma mua o ka pākaukau, he makewai nui ʻole e loaʻa a loaʻa ke kaumaha o ka nui o ka 5% e kū nei.
Hana ʻia ka hoʻokolohua desmopressin e hoʻokaʻawale i waena o ke kinipona a me ka maʻi diabetes nexrogen. Kūkulu ʻia ia i ka hoʻāʻo ʻana i ka naʻau o ka mea maʻi i ka desmopressin. I nā huaʻōlelo hoʻokahi, ua hoʻāʻo ʻia ka hana kūpono o nā receptor V2. Hana ʻia ka aʻo ʻana ma hope o kahi hōʻike maloʻo me ka hōʻike kiʻekiʻe loa i nā WUA endogenous.
Ma mua o ka nānā ʻana, pono i ka maʻi hoʻomehana. A laila ua hāʻawiʻia ʻo ia ʻo desmopressin, ʻo ia e hiki ke inu a ʻai loa, akā, i ka hoʻohālike. Ma hope o nā hola 2-4, lawe ʻia ka urine e hoʻoholo i kāna osmolality a me ka nui.
Ma ka maʻamau, ʻo nā hopena noiʻi o 750 mOsm / kg.
Me NND, hoʻonui nā indices i 300 mau mOsm / kg, a ma kahi kekaʻi o LPC ma hope o ka hoʻoheheʻeʻana, he 300 lākou, a me desmopressin - 750 mOsm / kg.
ʻEkolu hana
Hoʻomaʻo pinepine, MRI ka ʻike ʻana i ka maʻi maʻi diabetes. I loko o kekahi kanaka olakino i loko o ka puʻu pituitary, ʻike maopopo ʻia nā ʻokoʻa ma waena o nā anterior a me nā posterior. Eia kekahi, ʻo ka mea hope loa i ke kiʻi T1 he hōʻailona hōʻailona koʻikoʻi. ʻO kēia ma muli o ka hiki i loko o nā mea i hoʻopaʻa ʻia i loko o nā mea i ʻike ʻia i nā phospholipids a me WUA.
Ma ke alo o LPC, ka hala o ka hōʻailona e hoʻopili ʻia e ka neurohypophysis. ʻO kēia ma muli o ka hana hewa ʻole i ka hoʻopili a me ka halihali a me ka waiho ʻana o nā hale lole neurosecretory.
Eia kekahi, me ka maʻi maʻamau o ka maʻi insipidus, hiki i kahi neuropsychiatric, ophthalmological a me nā hoʻokolohua radiological. A me kahi ʻano o ka maʻi, ua hana ʻia ka ultrasound a me ka CT o nā keiki.
ʻO ka koho alakaʻi aʻoaʻo no NND e lawe i ka analogs vasopressin analogs (Desmopressin, Chlorpropamide, Adiuretin, Minirin). Ma ke kumu hoʻohālikelike, ua kuhikuhi ʻia nā diuretics a me NSAID.
Kekahi ʻano maʻi maʻi maʻi insipidus pili i ka hana infusion e pili ana i ka paʻakai. Pono kēia e hoʻoponopono i ka metabolism-paʻakai wai.
ʻO ka minamina me kekahi mau meaʻai kīnā ʻole i ka liʻiliʻi nui, ʻo ke kau ʻana ma ka paʻakai o ka paʻakai (4-5 g) a me ka protein (a hiki i 70 g). Ua kūpono kēia mau koi i ka papa Helu 15, 10 a me 7
He aha nā hōʻike e pono ai e lawoʻa inā e kānalua ʻo ʻoe i ka maʻi insipidus i wehewehe ʻia i loko o ke wikiō i kēia ʻatikala.
