Diipona insipidus: hōʻailona, ​​mālama, kumu, nā hōʻailona

ʻO ka maʻi maʻi maʻi insipidus ka maʻi i hōʻike ʻia e ka polyuria koʻikoʻi ma muli o ka hiki ʻole o nā pūpū me ka nānā ʻana i nā urine ma muli o kahi mea i nele a hana paha i ka hana ADH.

ʻO ka hōʻemi ʻana i ka mea huna a i ʻole ka hana a ADH me ka piʻi nui ʻana o ke kahe (ND), ʻo ia ke kumu no nā hōʻike nui o ka maʻi.

ʻO ka maʻi maʻi maʻi indipidus (ND) kahi maʻi pathological kahi i nalowale ka nui o nā huehue a me nā hypotonic urine.

ʻO nā kumu o ka maʻi insipidus maʻi

ʻO ka wahi mua i ka pākeke ka nona nā hōʻeha craniocerebral a me ka wawa o ka neurosurgical interventions, aʻo ia hoʻi i ka wā kamaliʻi CNS tumor predominate (craniopharyngioma, germinoma, glioma, pituitary adenoma). ʻO nā kumu ʻē aʻe paha ka metastases o ka neoplasms malignant, lesions vascular (hōʻeha o ka naʻau, hemorrhages, aneurysms), lesi infiltrative (histiocytosis, tuberkulosis, sarcoidosis), nā maʻi hopuna (toxoplasmosis, infection cytomegalovirus, meningitis, encephalitis). ʻO kahi lesion autoimmune o ka neurohypophysis ma ke ʻano o ka lymphocytic infundibulohypophysitis, ʻaʻole loaʻa.

Ma kahi o 5% o nā mea maʻi e loaʻa i kahi ʻano familial o ka insipidus maʻi neurogen me ka hoʻoilina aliʻi autosomal. Hoʻopilikia ʻia kēia maʻi ma ka mutation o ka gen vasopressin precursor, prepropressophysin, aia ma ka chromosome 20.

ʻO ka insipidus diabetes kikowaena i mua ua manaʻo ʻia he mea pono ia o ka DIDMOAD syndrome, a i ʻole syndrome syndrome. Wahi a nā ʻike hou, ʻike ʻia kēia maʻi genetic loa nā maʻi a ka huaʻehā o ka hua WFS1 ma ka 4 chromosome e hoʻopili ana i ka tungstamine protein transembrane, ka mea i pili i ka halihali o nā kaʻa o ka calcium ma ka pūnaewele endoplasmic o nā neurons a me nā cells-cell o nā pulupulu pancreatic. ʻO nā mea nui ka maʻi maʻamau e pili i ka insulin a me ka hoʻohaʻaha nui ʻana i ka hihio. Hōʻino ʻia i ka pūnaehana neʻe i loko o ka pokela, me ka diabetes insipidus, hoʻomohala i kahi lā ma hope (20-30 mau makahiki) ʻaʻole i nā maʻi āpau.

ʻO ka pohō ʻana o ka wahi hypothalamic-pituitary me ka hoʻomohala ʻana i ka maʻi diabetes insipidus i kekahi mau manawa ke ʻike ʻia me nā maʻi ʻano genetic ʻole e like me Lawrence-Moon-Barde-Beadle syndrome (pōkole pōkole, huʻihuli, ka lalo o ka noʻonoʻo, ka ululāʻau manuahi retinalation, polydactyly, hypogonadism a me anomalies urogenital) Hesxl transcription kumu.

Hōʻike ka maʻi maʻi diabetes gestagenic i ka wā hāpai. Ma kēia hihia, hoʻonui ka plasenta i ka wāwahi o ADH, e hoʻopuka ana i ka cysteine ​​aminopeptidase enzim, i hoʻolālā ʻia e luku aku i ka oxytocin, akā e hoʻopau pū i ka vasopressin.

Nā neʻe a me ka maʻi neʻe i ka maʻi neʻe i ka wa neʻe. ʻO ka nefrogenic maʻi nephrogenic diabetes insipidus he maʻi ʻano genetic i kumu e ka insensitivity i ka ADH. Ka X-link recessive form, ka mea i hoʻowalewale ʻia e ka vasopressin type 2 receptor gene, i kaawale, a me ka autosomal recessive a me autosomal dominant o ka aquaporin-2 gene (transmembrane wai kai o ka apical membrane o ka hōʻiliʻili i nā cell epithelial) ʻoi aku ka liʻiliʻi o ka maʻamau.

Ka loaʻa i nā maʻi diabetes nephrogenic insipidus e hōʻike pinepine iā ia ma mua o ka congenital, akā i ka hōʻailona o ka kiʻi haukapila a me ka hoʻihoʻi hou ʻana o nā maʻi. ʻO ke kumu ʻoi aku ka pinepine ma mua o ka mākaukau lithium, hiki iā ia ke hōʻoki i ke alima hoʻoliʻelima intracellular mai nā vasopressin receptors. Gentamicin, metacyclin, isophosphamide, colchicine, vinblastine tolazamide, phenytoin, norepinephrine (norepinephrine), hika a me ka diuretika osmotic me ka hopena like me ka lōʻihi a me ka hoʻohana nui ʻana. Hiki ke nānā ʻia nā kumumana o ka maʻi nephrogenic i nā pilikia o ka electrolyte (hypokalemia, hypercalcemia), maʻi o ka pēpē (pyelonephritis, tubulo-interstitial nephritis, polycystic, postobstructive uropathy), amyloidosis, myeloma, ʻĀnitela cell anemia a me sarcoidosis.

ʻO nā pathogenesis o ka maʻi insipidus maʻi

Hoʻokaʻawale ʻia ka huna ʻia o ka vasopressin e ka osmoreceptors anterior hypothalamus, e pane ana i nā maʻi osmolality o ka liʻiliʻi ma mua o 1% o ka kumu. ʻO ka pohō wai kūlohelohe (urine a me ka huki ʻana, ka hanu) alakaʻi i kahi hoʻonui nui i ka osmolality o ke kaila koko. Me kona hoʻonui i ka 282-285 mosm / kg, ua hoʻomaka ka hoʻonui ʻia o ka vasopressin. Ka nui o ka waiwai me ka hoʻohaʻahaʻa ʻana i ka osmolality plasma, i ka ʻokoʻa, pale i ka hoʻoweliweli ʻana o ka ADH, e alakaʻi ana i kahi hōʻemi o ka wai me ka hoʻonui ʻana o ka hopena i ka urine.

Central (neurohypophysial) diabetes insipidus

Ma ka ND waena, ʻikea ka poli hypotonic ma muli o ke kuleana kū ʻole a pili ʻole paha i ka neʻe ADH huna, ʻoiai ke kūpono o ka hoʻoulu ʻia ʻana o ka ʻoni a me ka pane maʻamau i ADH. Ua hoʻokaʻawale ʻia ka ND Central i loko o nā subtypes.

Ke kuʻi nei i ka nui o ka nele o ADH:

• kahua piha ND piha i kahi ʻano piha piha ʻole e hana i ka synthesize a huna ʻole i ka ADH,
• piha ka ND kūhelu piha i ka hanana nui o ka synthesis a i ʻole ka huna ʻana o ka ADH.

Ma muli o ka hoʻoilina:

• ʻO ka papa waena ʻohana ND kahi papa līlī, kahi i hoʻoili ʻia e ke ʻano mana autosomal me nā ʻano kahe like ʻole a hoʻomohala i ka wā kamaliʻi, ʻo ka hapa nui o nā ʻano genetic e pili ana i ka hoʻololi ʻana o ke ʻano o ka molekene neurophysin, ka mea e hōʻole ʻia i ka halihali intracellular o prohormone.
• loaʻa ka ND waena e ala ana ma muli o kekahi mau kumu.

ʻO nā kumu o ka insipidus diabetes kikowaena

Palena ND (ʻaʻole i loaʻa)

Palapala ND (kūʻai)

Insipidus maʻi maʻa

Hōʻailona ia e ka hypotonic polyuria mau loa, ʻoiai kahi pae kūpono o ADH, a ʻaʻole i pili ka hoʻomohala o ka exogenous ADH i ka nui o ka urine i hoʻopuka a i kona osmolarity. Ua hoʻokaʻawale ʻia ka Nīhana neʻe i ka ʻĀpana.

Ke kau nei i ke kiʻekiʻe o ADH.

• Hōʻike ʻia ka nephrogenic ND piha i kahi hiki ʻole holoʻokoʻa e pane i ka vasopressin komo i nā kūmole pharmacological.
• Hōʻano ʻia ka nephrogen ND incomplete e ka hiki ke pane aku i nā dosis pharmacological o nā hoʻomākaukau vasopressin.

Mahalo i ka hoʻoilina.

• E kūlia ka nephrogenic ND neʻe e pili ana i nā mutations i nā ʻāpana ʻelua. I nā 90% o nā hihia, hoʻokūkū ka mutation i ka hana o vasopressin V₂mea hoʻokaʻawale i ka tubule renal. Inheritance method X-pili, recessive, he wahine he hemozygous mutation carrier loaʻa he mauʻeha haʻahaʻa o ka metabolism wai impaired me ka nocturia, noctidipsy a me subnormal spesialika o ka urine. Ma 10% o nā ʻohana me ka ND hoʻoilina, ʻike ʻia ka mutation i ka wai aquaporin-2 i loaʻa i ka chromosome 12, wahi q13. Hoʻopili ʻana me kēia mutation hiki i ka autosomal recessive a dominant paha.
• Loaʻa pinepine ʻia ka ND maʻamau ma muli o ka hyperkalemia a i ʻole ka hypercalcemia. I nā kūlana ʻelua, kāohi ʻia ka hana o ka aquaporin-2 i loko o nā keiki. He hopena like ka Lithium. ʻO ka hemahema o ka renal a me ka pōpoki pāpaʻi paha e paʻakikī paha e ka hoʻomohala ʻana o ka ND nephrogenic.

ʻO nā kumu no nā Nīneʻa Nānā Nepalake

Nā polydipsia mua

Me ka polydipsia mua, hoʻonui ʻia ka hana kaila i ka mua, hiki ke kapa ʻia ia "hōʻino" ka wai o ka wai, a ua hele lua ʻia i ka polyuria a me ka hoʻēmi ʻana i ka osmolality koko. Ua hoʻokaʻawale ʻia nā polydipsia mua i nā ʻano ʻelua.

• Dipsogen ND, ma kahi o ka osmotic thrushold no ka hoʻoulu ʻana i ka ʻōlelo huna ADH e mālama ʻia ma kahi pae maʻamau, ʻoiai ka pae ʻana osmotic anuʻu haʻahaʻa no ka hoʻōla i hoʻoweliweli. Ke alakaʻi nei kēia kuhihewa i ka polydipsia hypotonic mau loa, no ka mea mālama i ka osmolarity serum ma lalo o ka paepae no ka hoʻoulu ʻana o ka hoʻolaha ADH.
• ʻO ka polydipsia Psychogenic, kahi i hoʻonui ʻia ai ka nui o ka wai i kahi paroxysmal i mea e hoʻonāukiuki i nā mea noʻonoʻo a i ʻole ka naʻau noʻonoʻo. ʻAʻole i like me Ns dipsogenic, ma kēia mau hihia, ʻaʻohe emi ʻana i ka paʻu osmotic e hoʻoulu i ka makewai.

Nā hōʻailona a me nā hōʻailona o ka maʻi insipidus maʻi

E like me ka mea i hōʻike ʻia, ʻo nā mākaʻikaʻi nui o ka maʻi insipidus o ka make wai, ka polyuria a me ka polydipsia (me ka manaʻo o ka manawa o ka lā). Hoʻomaʻamaʻa pinepine nā mea maʻi e inu i ka wai anuanu a i nā mea i kuke ʻia. Hōʻalo ka makewai pō, a me ka polyuria i ka hiamoe, a me ka hoʻohaʻahaʻa ʻana a me nā hana i ka noʻonoʻo. ʻO ka hoʻohana mau ʻana i ka nui o ka nui o ka wai i hiki i ke kaohi ʻana i ka ʻōpū a me ka hoʻohaʻahaʻa ʻana i ka mea huna o kāna mau kōlapa, ka hoʻomanū a ka maʻi gastrointestinal.

ʻO nā makahiki o ka hoʻomaka ʻana o ka maʻi insipidus i loaʻa i nā mea āpau, ʻoiai i loko o nā ʻano kongenital aia kekahi mau hiʻohiʻona.

No ka maʻi ʻaki o ka maʻi insipidus

ʻO ka koho ʻana i ke kumu o ka polyuria pinepine ʻole i kahi pilikia i kekahi mau pilikia, akā i kekahi manawa he hana paʻakikī ia. Pēlā, ʻo ka ʻike ʻia o ka maʻi diabetes i ka mea maʻi me ka polyuria hōʻike maopopo i ke kumu. ʻO ka hele ʻana o kahi maʻi noʻonoʻo i ka mea maʻi i hui pū me ka polyotonic polyuria e hōʻike i ka polydipsia kumu (psychogenic). Ma kekahi ʻaoʻao, hypotonic polyuria e kū nei i ka mua o ka hoʻonui i ka osmolarity plasma a me ka koni sima kiʻekiʻe ka haʻalele ʻana i ka maʻi ma nā polydipsia mua. Ke hele mai ʻo polyuria ma hope o ka hana ʻana a i ʻole ka trauma i ka wahi hypothalamic-pituitary, ʻike maopopo loa ka maʻi ka maʻi kumu o ka ND kikowaena. I nā hihia ʻike ʻole, makemake ʻia nā hōʻike kūikawā.

Ma hope o ka hōʻeha ʻana i ka wahi hypothalamic-pituitary a i ʻole kona hōʻeha, ʻo ka hihia o ka hoʻemi ʻana o ka wai ma ka maʻamau i ʻekolu mau hanana.

• ʻO ka hana mua o ka ND transient e pili ana me ka pīhoihoi axonal a me ka hiki ʻole o nā sela nerve e hana i ka hiki ke hana. Ua hōʻike ʻia ʻo ia iho i nā hola 24 mua ma hope o kahi hōʻeha a hoʻoholo i loko o kekahi mau lā.
• Hōʻike ʻia ka lua o ka lua e ka ADH hypersecretion syndrome, ka manawa o 5-7 mau lā ma hope o ka hōʻeha a pili pū me ka hoʻokuʻu ʻana i ka ADH mai nā sela nerve a synthesizing ADH i luku ʻia ma muli o ka trauma (hana trophic, hemorrhage).
• ʻO ke kolu o ke kolu ʻo ia ka hoʻomohala ʻana o ka Central ND, i ka ʻoi aku o ka 90% o nā pūpuna e hana ana i ka ADH ua luku ʻia e ka trauma.

Ma ka mea maopopo, ʻaʻole i mālama ʻia nā kaukani ʻekolu i hōʻike ʻia i nā mea āpau - ma kekahi mau mea maʻi wale nō i hiki i ka hoʻomaka mua ke hoʻomohala, ma loko ʻē aʻe - ʻo ka mua a me ka lua, a ma kekahi mau mea maʻi, ʻo ka hōʻeha o ka lolo me ka center ND.

ʻO ka palena o ke kuhikuhi ʻana i ke kikowaena ND i hōʻemi ʻia i ka haʻalele ʻana i nā kumu ʻē aʻe a pau o ND. No ka mea, ʻo ka hōʻemi o ka nui o ka urine i lawe ʻia me vasopressin ʻaʻole ia e hōʻoia i ka hōʻike ʻana o ke kikowaena ND, ʻoiai ʻo ia ʻano ʻano e hiki mai ana i ka polydipsia kumu, i loko o nā mea maʻi ma hope o ka hana ʻana o ka lolo, a ma nā mea maʻi me ka kaila wai maikaʻi, ma ka hihia hope loa, hiki ke mālama ʻia i ka wai inu wai. ʻO kahi hui diagnostic kūikawā no ke kikowaena ND kahi hui o ka polyuria hypotonic e kūʻē i ka osmolarity maʻamau a i ʻole hoʻi ke kiʻekiʻe o ka ADH i ke koko. ʻAʻole e like me polydipsia kumu, kahi i hoʻonui ʻole ʻia ai ka osmolarity o ke koko, a i kekahi manawa ke emi iki ia.

ʻO ka hoʻohiolo wai

I ka wā o ka hoʻāʻo me ka palena wai, ka ʻai ʻana ʻaʻole wale nō o ka wai, akā ke hoʻokaʻawale ʻia nā ʻano wai ʻē aʻe i kumu e hoʻoheheʻe ʻia i ke kino a pēlā ke ʻano o ka hoʻoulu ʻia ʻana no ka hoʻoulu loa ʻana i ka ADH. Hoʻololi ka lōʻihi i ka hoʻolilo ʻana i ka inu kahe e pili ana i ka neʻe ʻana o ka wai ma o ke kino, a ʻo ka manawa maʻamau ka lōʻihi o ka hōʻike ʻana mai ka hola 4 a hiki i 18 mau hola. Hoʻolaha ʻia e mālama i ka hōʻike ma loko o kahi kīnā ʻole o nā wai. Ma mua o ka hoʻomaka ʻana i ka hoʻāʻo, e hoʻomanawanui ka mea maʻi, ma hope o ke kaupaona ʻia. Mai kēia manawa ke nānā ʻia ka paona o ka mea maʻi i kēlā me kēia hola, ua hoʻopaʻa ʻia ka nui o nā urine e hoʻokaʻawale ʻia, a ua hoʻoholo ʻia ka nui o ka urine. Hoʻopau ʻia ka hōʻike ʻana ma nā hihia penei:

• Ua hōʻemi ke kaumaha i ka 3%,
• hōʻike ka mea maʻi i nā hōʻailona o ka hikiʻole i ka hana ʻana o ka ʻōnaehana cardiovascular,
• urine osmolarity paʻa (osmolarity fluctuation i ʻekolu mau manawa o ka urine ʻaʻole i oi aku i 30 mOsm / kg),
• hooulu ia ka hypernatremia (ma mua o 145 mmol / l).

I ka hoʻomaka ʻana o ka osmolarity a i ʻole ua nalowale paha ka mea maʻi ma mua o ka 2% o ka paona o ke kino, ke ʻano nei ka ʻike ʻana i ke koko:

• kaʻona sodium
• kahulialaha
• vasopressin kūlia.

Ma hope o kēlā, ua hopu ʻia ka mea maʻi me ka arginine-vasopressiner (5 mau pūʻulu) a i ka desmopressin (1 mg) subcutaneously, a me ka urm osinoline a me kona helu he ninaninau 30, 60 a me 120 mau minuke ma hope o ka maʻi. Hoʻohana ʻia ka ʻenehana o ka osmolarity kiʻekiʻe e hoʻomaʻamaʻa i ka pane ʻana i ka hoʻokele o ka arginine-vasopressinar. No ka hoʻokō pono ʻana o ka hoʻokolokolo, pono ia e aʻo i ka plasma osmolarity i ka hoʻomaka ʻana o ka hoʻokolokolo, a laila - ma mua o ka hoʻolaha ʻana o ka arginine-vasopressin a i ka desmopressin a ma hope o ka hoʻokele ʻana o ka lāʻau.

I nā mea maʻi me ka polyuria koʻikoʻi (ʻoi aku ma mua o 10 l / lā), pono e hoʻomaka i ka hoʻomaʻamaʻa ʻana ma kahi ʻōpū ʻole i ke kakahiaka, a ke lawe ʻia nei ma lalo o ka mālama pono ʻana o ka kūlana o ka mea maʻi e nā limahana lapaʻau. Inā maʻalahi ka polyuria, a laila hiki ke hoʻomaka ka hoʻāʻo ʻana mai ka hola 22, me ka mea e pono ai ka hoʻomaha ʻana no nā hola 12-18.

Ma mua o ka hoʻāʻo, inā hiki, nā lāʻau lapaʻau e pili pū ana i ka synthesis a me nā mea huna o ADH e hoʻihoʻi. ʻO nā kīʻaha caffeinated, me ka waiʻona a me ka uahi hoʻi i hoʻopau ʻia ma kahi o 24 mau hola ma mua o ka hoʻāʻo. I ka wā o ka hoʻokolohua, pono pono ka nānā pono ʻana i ka mea maʻi, ʻoi loa ka hōʻike ʻana o nā hōʻailona hiki ke hoʻoikaika i ka hūnā ʻia o ka vasopressin e kū nei i kahi kūlana o ka osmolarity maʻamau (no ka laʻana, ka nausea, ka hypotension arterial, a i ʻole nā ​​hopena vasovagal).

Olakino. I ka poʻe olakino, hiki i ka palena o ka wai ke hoʻopiha nei i ka mea huna o ADH a hoʻoulu i ka nui o ka urine. I ka hopena, ʻaʻole ka alakaʻi o ka ADH i nā mea hou aʻe i ka hoʻonui ʻana i ka osmolarity ma mua o 10% i loko o ka urine i hoʻokūpaʻa ʻia.

Nā polydipsia mua. Ke kū ʻole ka osmolarity o ka urine i kahi kiʻekiʻe iʻoi aʻe ma mua o ka osmolarity o ke koko, ke kū ʻole ka polydipsia mua, ke ʻole ma ka hoʻokaʻawale ʻia ke kahe o ka wai e ka mea maʻi i ka wā o ka hoʻāʻo. I kēia hihia hope loa, ʻaʻohe osmolarity koko a i ʻole urine osmolarity e hoʻonui kūpono i ka wā o ka hoʻoweliweli wai wai.ʻO kekahi mea hōʻailona i ka nānā ʻole ʻana me ka pauku hoʻokolokolo ʻo ia ka ʻae ʻana ma waena o ka dinamika o ke koʻikoʻi o ke kino a me ka nalowale o ka nui o ka wai e ke kino - ka pakeneka o ka wai nui o ka wai me ka paona o ka mea maʻi ma mua o kēlā me kēia.

Piha ND. Ma nā kikowaena ʻelua a me ka nephrogenic ND, ma kahi o ND piha, i ka osmolarity o ka urine ʻaʻole iʻoi aku i ka osmolarity plasma i ka hopena o ka hōʻike me ka kāohi wai. E like me ke ʻano o ka hoʻokele ʻana o ka arginine-vasopressin a i ka desmopressin, hiki ke ʻano ʻokoʻa kēia mau ʻano ʻelua o ND. Me ka nephrogenic ND, hiki i ke kiʻi liʻiliʻi ke osmolarity ma hope o ka hoʻokele ʻana o ka arginine-vasopressin a i ka desmopressin, akā ʻaʻole iʻoi aku ma mua o 10% i loaʻa i ka hopena o ka wā hoʻomāliko. Me ke kikowaena ND, ʻo ka hoʻokele o ka arginine-vasopressin e hoʻoulu ai i ka osmolarity i ka urine ma mua o 50%.

Hoʻopau ʻole ND. I nā mea maʻi me ka ND piha piha, ma ka hihia o ke kolamu a me nephrogenic ND, hiki i ka osmolarity urine ke keu aku i ka osmolarity o ke koko ma ka hope o ka hōʻike me ka paʻa ʻana o ka wai. Ma ka manawa like, me ka Central ND, ʻoi aku ka pae o ka ADH plasma ma lalo o ka mea i manaʻo ʻia me ka pae haʻahaʻa o ka osmolarity, ʻoiai me ka nephrogenic ND ua lawa lākou i kēlā me kēia.

Hypertonic Sodium Chloride Infusion

Hiki i kēia ala ke hoʻokaʻawale i ka ND piha mai ka polydipsia mua.

Ke ʻano a me ka wehewehe ʻana

I loko o kēia hōʻike hoʻonāukiuki, ua hōʻeha ʻia kahi hopena 3% sodium klorida ma ka nui o 0.1 ml / kg i hoʻokahi minuke no 1-2 mau hola. A laila e hoʻoholo ʻia ka ʻike ADH i ka wā e hiki ʻole ai i ka pae o ka osmolarity a me ka sodium plasma e hōʻea i ka> 295 mOsm / l a me 145 mEq / l, kekekahi.

I ka poʻe maʻi me ka nephrogenic ND a i ʻole polydipsia mua, he piʻi nui i loko o ka serum ADH i pane i ka hoʻonui ʻana i ka osmolarity, he maʻamau, a i loko o nā mea maʻi me ke kikowaena ND, he hoʻonui hoʻonui subnormal i loko o ka hoʻopaʻa ʻia ʻana o ADH a i ʻole a i ʻole.

Hoʻolaha hoʻokolohua

Hiki i kēia ala ke hoʻokaʻawale i kahi kikowaena ND piha piha mai kahi ND nephrogenic piha.

Ke ʻano a me ka wehewehe ʻana

E hāʻawi i kahi hoʻokolokolo hoʻokolokolo me desmopressin no 2-3 mau lā. Hoʻopili a hōʻoluʻolu paha kēia hoʻomaʻamaʻa i nā hōʻike o ke kikowaena ND a ʻaʻole i pā i ka papa o ka nephrogenic ND. Ma nā polydipsia kumu mua, ʻaʻole i pili ka maikaʻi o ka hoʻāʻo ʻana i ka inu ʻana i ka wai, ʻoiai i kekahi manawa me ND waena, hiki i ka mea maʻi ke hoʻomau i ka nui o ka wai.
ʻO ka mea mua, pono ʻoe e ʻoluʻolu e loaʻa ka polyuria i ka mea maʻi.

Haʻalele ka mea maʻi i ka lawe ʻana mai a hiki i ke kaumaha o ke kino e hoʻoneʻe ana ma mua o 5% o ka hoʻomaka a i ʻole ka makewai ʻaʻole hiki ke loaʻa. No kēia mea, ʻo ka hapanui o nā manawa, ua lawa nā hola 8-12. Ma nā kānaka olakino, ma lalo o kēia mau ʻano, ka emi iki ʻana o ka nui a me ka hoʻonui ʻana mai o ka ʻike / l ʻO ka hoʻonui i ka osmolality urine a hiki i ka 750 mosm / l e hōʻike ana i ka insipidus maʻi neurogen.

Ke ʻike ana i ka maʻi nephrogenic diabetes insipidus e nānā i ka loiloi piha ʻana i ke kūlana o nā keiki, ka wehe ʻana o nā mea hoʻonā electrolyte.

ʻO ka hōʻiliʻili pono ʻana o ka mōʻaukala ʻohana, ka nānā ʻana i nā hoahānau o ka mea maʻi e ʻae iā mākou e hoʻomaopopo a ʻano hou i nā ʻano congenital o ka maʻi insipidus maʻi.

Ka hoʻomaʻemaʻe ʻana i ka maʻi maʻi maʻi insipidus

Ke lawa nei ka ʻike wai ʻana

ʻO nā mea maʻi me nā hōʻike haʻahaʻa o ND (ka diuresis i kēlā me kēia lā ʻaʻole iʻoi aku ma mua o 4 l) a me ka hana i mālama ʻia i ka makewai ʻaʻole pono e kuhikuhi i ka lāʻau lapaʻau, ua lawa ia ʻaʻole e kaupalena i ka hoʻoilo wai.

Kikowaena ND. E kau i kahi kiko o vasopressin - desmopressin.

ʻO nā hana ma ka V₂-taiahi i loko o ka pūpū a me ka hopena pākahi ma V receptors₁ vasopressin i loko o nā moku. A ke hopena, ua hōʻemi ʻia ka lāʻau lapaʻau i ka hopena hypertensive a hoʻonui ʻia ka antidiuretic. Eia kekahi, ua hoʻonui i ka hapalua-ola.

Hiki ke kau ʻia ka lāʻau lapaʻau i nā manawa 2 i ka lā i nā kaulike like ʻole, a ʻo ka hopena hopena i nā maʻi maʻi like ʻole i ka laulā ākea.

• waha waha o 100-1000 mcg / lā,
• intranasal dosis o 10-40 mcg / lā,
• subcutaneous / intramuscular / intravenous dosis o 0.1 a 2 mcg / lā.

Neʻīpīhai ND

• ʻO ke kumu kumu o ka maʻi (metabolic a i ʻole lāʻau lapaʻau) e hoʻopau ʻia.
• ʻO nā wā nui o ka desmopressin i ka manawa kūpono (no ka laʻana, a hiki i ka 5 mcg intramuscularly).
• ʻO ka noʻonoʻo o ka nui o ka wai o ka wai.
• ʻO ka maʻi a me nā wailele o Thiazide me ka inhibitor o ka prostaglandin e like me ka indomethacin.

He paʻakikī i ka polydipsia Psychogenic ke hoʻomaʻamaʻa a e pono ai i ka mālama ʻana e kahi psychiatrist.

Inā ulu ka maʻi diabetes intipidus e pili ana i ka mua o ka hiki ke hoʻololi i ka ulu ʻana o ka wahi hypothalamic-pituitary, pono e hana ʻia nā hoʻāʻo ʻana e hana ai i ka etiotropic (hana lapaʻau a ʻano maʻi maʻi a me ka chemotherapy o nā maʻi maʻi, anti-inflammatory therapy no sarcoidosis, meningitis, etc.).

Hoʻolaha ʻia kahi hana kūpono no ka nephrogenic diabetes insipidus. Inā hiki, ke hoʻopau ʻia ke kumu o ka maʻi i loaʻa (no ka laʻana, e hōʻemi i ka nui o nā mākaukau lithium). Hōʻike ʻia ka poʻe maʻi i ka lawa o ka wai ʻauʻau, ka palena o ka paʻakai.

Hoʻopuka no ka maʻi maʻi indipidus

Hōʻike ʻia ka maʻi diabetes indipidus ma hope o nā hana neurosurgical a me nā hōʻeha o ka puʻuwai traumatic e hele pinepine, i hōʻike ʻia nā hala o nā ʻano idiopathic o ka maʻi.

ʻO ka prognosis o nā poʻe maʻi me ka maʻi insipidus maʻi neurogen, i mea e hoʻoholo ai, e hoʻoholo ʻia e nā maʻi lalo e alakaʻi ana i ka hōʻino o ka hypothalamus a i ʻole neurohypophysis, a me ka hoʻopiʻi adenohypophysis inscomitant.