Mob ntshav qab zib insipidus: cov tsos mob, kho mob, ua rau, yam tshwm sim
Ntshav qab zib insipidus yog ib qho mob tshwm sim los ntawm mob polyuria ntau vim qhov ua haujlwm ntawm lub raum tsis tuaj yeem tso zis zoo vim yog qhov ua haujlwm tsis haum lossis tsis ua haujlwm ntawm ADH.
Ib qho kev txo qis hauv kev zais cia lossis kev nqis tes ntawm ADH yog nrog cov kua dej tawm hauv lub cev ntau dua (ND), uas yog cov laj thawj rau qhov tseem ceeb ntawm tus kabmob.
Mob ntshav qab zib insipidus (ND) yog ib txoj kab mob pathological uas ua rau kev tso zis ntau thiab txo cov zis hypotonic poob.
Ua rau mob ntshav qab zib insipidus
Thawj qhov chaw rau cov neeg laus yog tus mob craniocerebral raug mob thiab kev tiv thaiv neurosurgical, thaum nyob hauv menyuam yaus CNS cov qog ua kom kub hnyiab (craniopharyngioma, germinoma, glioma, pituitary adenoma). Lwm qhov laj thawj yuav yog metastases ntawm malignant neoplasms, vascular lesions (plawv nres, hemorrhages, aneurysms), mob txhab rau hauv (histiocytosis, tuberculosis, sarcoidosis), kis kab mob (toxoplasmosis, cytomegalovirus infection, meningitis, encephalitis). Lub qhov txhab txhab txhawm rau mob rau daim nqaij neurohypophysis nyob rau hauv daim ntawv ntawm lymphocytic infundibulohypophysitis yog tsawg.
Txog 5% ntawm cov neeg mob muaj ib daim ntawv txheeb ze ntawm cov ntshav qab zib neurogenic insipidus nrog tus kabmob autosomal ncaus. Tus kab mob no tshwm sim los ntawm kev hloov pauv ntawm vasopressin txheej txheem ua ntej, prepropressophysin, nyob rau ntawm 20 chromosome.
Nruab nrab mob ntshav qab zib insipidus yav dhau los suav hais tias yog qhov tseem ceeb ntawm DIDMOAD syndrome, lossis Tungsten syndrome. Raws li cov ntaub ntawv niaj hnub no, qhov tsis tshua muaj caj ces tshwm sim vim hloov pauv ntawm WFS1 lub cev ntawm tus thib 4 chromosome encoding transmembrane protein tungstamine, uas koom rau hauv kev thauj khoom ntawm calcium ions hauv endoplasmic network ntawm neurons thiab β-hlwb ntawm pancreatic islets. Cov tsos mob tseem ceeb yog insulin-mob ntshav qab zib thiab nce zuj zus hauv lub zeem muag. Kev puas tsuaj rau lub hauv nruab nrab cov hlab hlwb, suav nrog cov ntshav qab zib insipidus, txhim kho tom qab hnub tom qab (20-30 xyoo) thiab tsis nyob hauv txhua tus neeg mob.
Kev swb ntawm hypothalamic-pituitary thaj av nrog kev txhim kho ntshav qab zib insipidus qee zaum tuaj yeem pom nrog cov kab mob tsis tshua muaj mob xws li Lawrence-Moon-Barde-Beadle syndrome (luv luv, rog, mob hlwb tsis txaus, retinal pigment degeneration, polydactyly, hypogonadism thiab urogenital anomalies) Hesxl qhov ntsuas hloov pauv.
Gestagenic ntshav qab zib insipidus nws tus kheej thaum cev xeeb tub. Hauv qhov no, cov placenta nce qhov puas tsuaj ntawm ADH, ua cov enzyme cysteine aminopeptidase, uas yog tsim los rhuav tshem oxytocin, tab sis kuj rhuav tshem vasopressin.
Neesrogenic ntshav qab zib insipidus hauv nws daim ntawv nthuav dav yog ntau tsawg dua li hypothalamic-pituitary. Congenital nephrogenic ntshav qab zib insipidus yog ib hom mob muaj tsawg heev los ntawm qhov tsis txaus ntseeg mus rau ADH. X-txuas ntxiv daim ntawv tsim tawm, uas tshwm sim los ntawm kev hloov pauv ntawm vasopressin hom 2 receptor gene, yog raug rho tawm, thiab autosomal recessive thiab autosomal hloov pauv ntawm aquaporin-2 noob (transmembrane dej channel ntawm apical membrane ntawm kev sau cov leeg epithelial hlwb) txawm tias tsawg dua.
Tau txais nephrogenic mob ntshav qab zib insipidus ua tau nws tus kheej ntau dua li kev yug los, tab sis yog pom los ntawm cov duab tsis tshua zoo nkauj thiab muaj kev cuam tshuam ntawm kev cuam tshuam. Qhov ua kom tshwm sim ntau dua li lwm tus yog kev npaj lithium, uas tuaj yeem cuam tshuam cov teeb liab intracellular kis los ntawm vasopressin receptors. Gentamicin, metacyclin, isophosphamide, colchicine, vinblastine tolazamide, phenytoin, norepinephrine (norepinephrine), lub voj thiab osmotic diuretics muaj cov txiaj ntsig zoo li kev siv lub sijhawm ntev thiab loj. Cov ntsiab lus ntawm cov ntshav qab zib nephrogenic tuaj yeem pom nyob rau hauv cov kab mob electrolyte (hypokalemia, hypercalcemia), mob raum (pyelonephritis, tubulo-interstitial nephritis, polycystic, postobstructive uropathy), amyloidosis, myeloma, sickle cell anemia thiab sarcoidosis.
Pathogenesis ntawm ntshav qab zib insipidus
Kev zais cia ntawm vasopressin yog tswj hwm los ntawm anterior hypothalamus osmoreceptors, uas teb rau osmolality hloov pauv ntawm tsawg dua 1% ntawm qhov qub. Kev poob dej hauv lub cev (tso zis thiab tawm hws, ua pa) ua rau maj mam nce rau hauv osmolality ntawm ntshav ntshav. Nrog nws nce mus rau 282–285 mosm / kg, kev zais ntawm vasopressin pib nce ntxiv. Kev haus dej ntau dhau thiab txo qis ntshav plasma osmolality, qhov tsis sib thooj, txwv tsis pub tso cai ntawm ADH, uas ua rau kom cov dej rov qab tau txais thiab txo cov zis ntxiv.
Nruab nrab (neurohypophysial) mob ntshav qab zib insipidus
Hauv nruab nrab ND, hypotonic polyuria tau pom los ntawm kev tsis muaj lossis tsis muaj qhov txheeb ze ntawm ADH tso pa tawm, txawm hais tias muaj kev tsim nyog tau zais kev zais cia thiab ib txwm lub raum teb rau ADH. ND Nruab Nrab tau muab faib ua subtypes.
Ua raws li cov neeg kawm ntawv ntawm ADH tsis muaj peev xwm:
- ua tiav hauv nruab nrab ND yog pom los ntawm ua tsis tiav tsis muaj peev xwm ua ke lossis ua kom sib luag ntawm ADH,
- Qhov tsis txaus ntawm hauv nruab nrab ND yog qhov pom los ntawm kev tsis sib txuas ua ke lossis zais cia ntawm ADH.
Nyob ntawm cov cuab yeej cuab tam:
- tsev neeg nruab nrab ND yog cov kab mob uas tsis tshua muaj, tau txais los ntawm ib qho hom autosomal nrog ntau cov qauv ntws thiab kev loj hlob hauv menyuam yaus, feem ntau cov caj ces tsis txaus ntseeg tau cuam tshuam nrog kev hloov kho ntawm cov qauv ntawm cov neurophysin molecule, uas tej zaum cuam tshuam kev thauj mus los ntawm prohormone,
- nrhiav lub hauv paus ND tshwm sim vim qee qhov laj thawj.
Ua rau hauv nruab nrab ntawm ntshav qab zib insipidus
Thawj ND (tsis xav tau)
Secondary ND (muas)
Kev poob plig | Kev raug mob hauv tsev |
Kev raug mob Iatrogenic (lag luam) | |
Lub pob hlav | Craniopharyngioma |
Thawj Pob Tawb Yog Dab | |
Tumor metastases (qog qog caj ces, ntsws) | |
Mob caj dab mob ntshav dawb | |
Lymphomatoid granulomatosis | |
Hnab tshos Cyst | |
Mob qog ua rau cov qog cell (tsis tshua muaj) | |
Granulomatosis | Sarcoidosis |
Keebkwm Daviocytosis | |
Tub Ntshaw | |
Kab mob | Ua mob rau daim tawv nqaij |
Encephalitis | |
Mob Vascular | Aneurysm |
Sheehan cov tsos mob | |
Hypoxic encephalopathy | |
Tshuaj Kho Mob / Tshuaj Kom Raug Mob | Cawv |
Diphenylhydantion | |
Autoimmune genesis | Lymphocytic pituitary caj pas (tsis tshua muaj, feem ntau cuam tshuam rau sab xub ntiag) |
Neesrogenic ntshav qab zib insipidus
Nws yog tus cwj pwm los ntawm tas li hypotonic polyuria, txawm hais tias muaj qib tsim nyog ntawm ADH, thiab kev tswj hwm ntawm exogenous ADH tsis cuam tshuam rau qhov ntim ntawm cov zis tso tawm lossis nws osmolarity. Neesrogenic ND tau faib ua subtypes.
Ua raws li cov neeg kawm ntawv ntawm ADH tsis muaj peev xwm.
- Ua kom tiav nephrogenic ND yog qhov pom los ntawm qhov ua tsis tiav los teb rau vasopressin txawm tias hauv cov tshuaj pharmacological.
- Ua tsis tiav nephrogenic ND yog tus cwj pwm los ntawm kev muaj peev xwm los teb rau pharmacological koob tshuaj vasopressin npaj.
Nyob ntawm cov kab mob siab.
- Hereditary nephrogenic ND tshwm sim vim kev hloov hauv ob thaj chaw sib txawv. Hauv 90% ntawm cov neeg mob, qhov hloov pauv ua rau ua txhaum ntawm txoj haujlwm ntawm vasopressin V2receptor ntawm hlaus tubule. Txoj kev ncaus Tus kab mob X-txuas, tsis kam ua, tus poj niam lub cev sib hloov pauv tuaj yeem muaj cov tsos mob me me ntawm lub cev tsis ua haujlwm nrog lub cev hu ua nocturia, noctidipsy thiab lub cev nqus ntawm cov zis. Hauv 10% ntawm cov tsev neeg muaj mob ND, muaj kev hloov pauv tau tshawb pom hauv aquaporin-2 gene nyob rau ntawm chromosome 12, thaj av q13. Qub txeeg qub teg nrog rau qhov kev sib pauv tuaj yeem yog tus kheej autosomal recessive lossis pawg thawj coj.
- Tau ND tshwm sim feem ntau vim yog hyperkalemia los yog hypercalcemia. Hauv ob qho tib si, kev ua ntawm aquaporin-2 hauv lub raum yog suppressed. Lithium muaj cov nyhuv zoo li no. Mob raum tsis ua hauj lwm thiab tso zis tsis tso zis yuav nyuaj los ntawm kev txhim kho nephrogenic ND.
Cov Kev Vim Li Cas Xav Tau Cov Neeg Nerogenrogenic ND
Neeg muaj keeb | |
---|---|
Familial X-txuas tsis ua si (hloov chaw hauv V2txais) | |
Autosomal recessive (hloov mus rau hauv aquaporin gene) | |
Autosomal tseem ceeb (hloov mus rau hauv aquaporin gene) | |
Nrhiav | |
Cov tshuaj noj | Kev npaj lithium |
Demeclocycline | |
Methoxyflurane | |
Kev zom zaub mov | Kev Hypokalemia |
Hypercalcemia / Hypercalciuria | |
Qhov tshwm sim ntawm ob sab urethral kev khaum | Benign prostatic hyperplasia |
Neurogenic zais zis (mob ntshav qab zib visceral neuropathy) | |
Mob leeg | Sickle cell anemia |
Infiltrative | Mob Amyloidosis |
Kev noj zaub mov muaj protein tsawg |
Thawj polydipsia
Nrog cov tshuaj polydipsia thawj zaug, cov kua dej kom tsawg yog qhov pib nce, uas tuaj yeem hu ua "kev tsim txom" ntawm cov kua dej, uas yog tus thib ob tau nrog polyuria thiab txo qis ntshav osmolality. Thawj polydipsia tau muab faib ua ob hom.
- Dipsogenic ND, nyob rau hauv uas lub osmotic pib rau stimulating ADH tso pa tawm yog tswj nyob rau hauv ib txwm tus kheej, thaum ib tug tsis xws li qis osmotic pib rau nqhis dej kev txhim kho. Qhov kev ua txhaum no ua rau muaj cov kab mob hypotonic polydipsia tas mus li, txij li cov ntshav osmolarity raug khaws cia hauv qab no qhov pib rau kev txhawb ntawm ADH secretion.
- Cov tshuaj tiv thaiv kev mob hlwb (psychogenic polydipsia), nyob rau hauv uas muaj paroxysmal nce siv dej ntau, uas ua rau muaj kev puas siab puas ntsws lossis mob hlwb. Tsis zoo li dipsogenic ND, hauv cov rooj plaub no tsis muaj qhov txo qis ntawm qhov pib osmotic kom txhawb kev nqhis dej.
Cov tsos mob thiab cov tsos mob ntawm tus mob ntshav qab zib insipidus
Raws li tau hais, cov tsos mob tseem ceeb ntawm cov ntshav qab zib insipidus yog nqhis dej, polyuria thiab polydipsia (tsis hais lub sijhawm nruab hnub). Cov neeg mob feem ntau nyiam haus dej txias lossis haus dej txias. Hmo ntuj nqhis dej thiab polyuria cuam tshuam kev pw tsaug zog, thiab kev ua haujlwm ntawm lub hlwb thiab lub hlwb ua haujlwm tsawg. Kev siv cov kua ntau tas mus li ua rau lub plab thiab qhov qis ntawm lub qog ntawm nws cov qog, lub plab ua kom lub cev tsis zoo.
Lub hnub nyoog pib ntawm kev kis mob ntshav qab zib insipidus tuaj yeem yog ib qho, thaum nyob hauv nws cov ntawv yug hauv plab muaj qee cov qauv.
Kev kuaj mob ntshav qab zib insipidus
Kev txiav txim siab qhov ua rau polyuria feem ntau tsis ua rau muaj teeb meem tshwj xeeb, tab sis qee zaus nws yog ib txoj haujlwm nyuaj. Yog li, kev kuaj pom cov ntshav qab zib hauv tus neeg mob uas mob polyuria pom tseeb nws qhov ua rau. Lub xub ntiag ntawm kev puas siab puas ntsws nyob rau hauv tus neeg mob hauv kev sib xyaw nrog hypotonic polyuria qhia thawj hom tshuaj (psychogenic) polydipsia. Ntawm qhov tod tes, hypotonic polyuria tawm tsam keeb kwm yav dhau los ntawm nce ntshav osmolarity thiab ntshav dej siab sodium tshem tawm kev kuaj mob ntawm cov thawj polydipsia. Thaum polyuria tshwm sim tom qab kev phais mob los yog xwm txheej hauv thaj chaw hypothalamic-pituitary, kev kuaj mob ntawm ND nruab nrab yog yuav luag pom tseeb. Hauv qhov tsis muaj qhov tseeb, qhov kev ntsuam xyuas tshwj xeeb yog qhov tsim nyog.
Tom qab kev phais mob hauv thaj av hypothalamic-pituitary lossis nws qhov kev raug mob, qhov kev ua txhaum ntawm dej tsis sib thooj feem ntau tshwm sim hauv peb qib.
- Thawj theem ntawm kev hloov pauv ND cuam tshuam nrog kev ua axonal poob siab thiab qhov tsis muaj peev xwm ntawm cov hlab ntshav ntawm daim ntawv tsim kom muaj peev xwm ua tau. Nws qhia tau nws tus kheej hauv thawj 24 teev tom qab raug mob thiab daws tsis dhau li ntawm ob peb hnub.
- Qhov theem ob yog pom los ntawm ADH qhov mob siab tsis haum, tshwm sim 5-7 hnub tom qab raug mob thiab cuam tshuam nrog kev tso tawm ntawm ADH los ntawm cov leeg hlwb tso tawm cov tshuaj tiv thaiv ADH uas tau rhuav tshem vim kev mob tshwm sim (kev puas tsuaj, mob ntshav qab zib, hemorrhage).
- Qeb thib peb yog kev txhim kho hauv nruab nrab ntawm ND, thaum ntau tshaj 90% ntawm cov hlwb ua ADH raug pov tseg los ntawm kev ua kom raug mob.
Pom tseeb, qhov piav qhia peb-qib kev ua tsis tau pom muaj nyob hauv txhua tus neeg mob - hauv qee tus neeg mob tsuas yog thawj theem tuaj yeem txhim kho, hauv lwm tus - thawj thiab theem thib ob, thiab hauv qee cov neeg mob, kev raug mob rau lub hlwb xaus nrog qhov nruab nrab ND.
Lub hauv paus ntsiab lus ntawm kev txheeb xyuas mob ntawm ND hauv nruab nrab yog txo mus rau kev cais tawm ntawm txhua lwm yam ua tau rau ND. Hauv tshwj xeeb, qhov kev txo qis hauv cov zis uas coj nrog vasopressin tsis lees paub qhov kev kuaj mob hauv nruab nrab ntawm ND, vim tias qhov kev tawm tsam no tshwm sim hauv thawj polydipsia, hauv cov neeg mob tom qab phais mob hlwb, thiab hauv cov neeg mob uas muaj dej zoo, tom kawg, kev tuav dej kuj tseem tuaj yeem. dej ua qaug cawv. Ib qho kev kuaj mob sib xyaw rau nruab nrab ND yog kev sib xyaw ntawm hypotonic polyuria tawm tsam ib txwm los sis nce me ntsis ntawm cov ntshav osmolarity thiab qhov ntshav tsawg kawg ntawm ADH hauv cov ntshav. Tsis zoo li thawj polydipsia, nyob rau hauv uas tsis muaj ib qho nce osmolarity ntawm cov ntshav, thiab qee zaum nws tseem txo.
Kuaj kev txwv dej
Thaum lub sijhawm kuaj nrog kev txwv dej, kev noj haus tsis yog dej nkaus xwb, tabsis tseem muaj lwm yam kua hauv lub nruab nrog kom ua kom lub cev qhuav dej thiab ua rau lub cev muaj zog txaus rau kev txhawb siab tshaj plaws ntawm ADH. Lub sijhawm nyob hauv kev txwv cov kua dej yog nyob ntawm tus nqi ntawm cov kua dej los ntawm lub cev, thiab feem ntau qhov ntsuas tau ntev txog 4 txog 18 teev, nws raug nquahu kom ua qhov kev ntsuas nyob hauv chav hauv qhov tsis muaj dej. Ua ntej pib ntsuas, tus neeg mob yuav tsum tso zis, tom qab ntawd nws yuav tsum ntsuas kom hnyav. Txij lub sijhawm no, tus neeg mob lub cev qhov hnyav tau saib xyuas txhua teev, qhov ntim ntawm cov zis tawm yog kaw, thiab cov zis osmolarity tau txiav txim siab teev. Kev xeem yog txiav nyob rau hauv cov xwm txheej hauv qab no:
- poob phaus nce mus txog 3%,
- tus neeg mob qhia pom tias tsis muaj kev ua haujlwm ntawm kev ua haujlwm ntawm cov hlab plawv,
- zis osmolarity stabilized (osmolarity fluctuation hauv peb ntu ntawm cov zis tsis ntau tshaj 30 mOsm / kg),
- hypernatremia tsim (ntau dua 145 mmol / l).
Sai li lub osmolarity tau ruaj khov lossis tus neeg mob tau poob ntau dua 2% ntawm lub cev qhov hnyav, kuaj ntshav hauv qab no tau ua:
- sodium cov ntsiab lus
- osmolarity
- vasopressin concentration.
Tom qab ntawd, tus neeg mob tau txhaj nrog arginine-vasopressiner (5 units) lossis desmopressin (1 mg) subcutaneously, thiab cov ntshav osmolarity thiab nws qhov ntim tau kuaj 30, 60 thiab 120 feeb tom qab txhaj tshuaj. Qhov siab tshaj plaws osmolarity tus nqi (lub ncov siab) yog siv los ntsuas cov lus teb rau kev tswj hwm ntawm arginine-vasopressinar. Kev ua tiav kev tshuaj xyuas, nws yog qhov yuav tsum tau kawm cov ntshav ntshav osmolarity thaum pib xeem, tom qab ntawd - ua ntej kev qhia ntawm arginine-vasopressin lossis desmopressin thiab tom qab kev tswj hwm tshuaj.
Hauv cov neeg mob uas muaj mob polyuria ntau dua (ntau dua 10 l / hnub), nws raug nquahu kom pib qhov kev kuaj rau ntawm lub plab khoob thaum sawv ntxov, thiab nws tau coj los saib xyuas tus neeg mob tus mob los ntawm cov neeg ua haujlwm kho mob. Yog tias polyuria muaj ntsis, ces qhov kev sim pib tuaj yeem pib los ntawm 22 teev, raws li kev txwv dej rau 12-18 teev yuav tsum muaj.
Ua ntej kev kuaj mob, yog tias ua tau, cov tshuaj uas cuam tshuam rau cov synthesis thiab kev zais ntawm ADH yuav tsum tsis ua mus ntxiv. Cov dej haus muaj caffeine, nrog rau dej cawv thiab haus luam yeeb, raug tso tseg tsawg kawg 24 teev ua ntej ntsuas. Thaum qhov kev kuaj mob, nws yuav tsum ua tib zoo saib xyuas tus neeg mob, tshwj xeeb tshaj yog cov tsos mob uas tuaj yeem txhawb lub zais vasopressin tawm tsam keeb kwm ntawm ib txwm osmolarity (piv txwv li, xeev siab, mob ntshav hypotension, lossis vasovagal tshua).
Noj qab nyob zoo. Hauv cov neeg muaj kev noj qab haus huv, kev txwv tsis pub cov dej yuav ua rau muaj kev tso siab ntau ntawm ADH thiab ua rau cov zis ntau dua. Raws li qhov tshwm sim, kev qhia txog ADH ntxiv lossis nws cov qauv tshuaj yuav tsis ua rau muaj qhov nce ntawm osmolarity ntau dua 10% hauv cov zis twb muaj lawm.
Thawj polydipsia. Thaum lub osmolarity ntawm cov zis tsis nce rau theem siab dua lub osmolarity ntawm cov ntshav, thawj polydipsia raug tshem tawm, tshwj tsis yog cov kua dej tau zais los ntawm tus neeg mob thaum lub sijhawm kuaj xyuas yog cais tsis tau kiag li. Hauv qhov teeb meem tom kawg no, tsis tuaj ntshav osmolarity tsis tso zis ntshav nce siab thaum ntsuas kev txwv dej.Lwm qhov taw qhia ntawm kev tsis ua raws li hom kev ntsuas no yog qhov tsis sib xws ntawm lub zog ntawm lub cev qhov hnyav thiab poob cov dej hauv lub cev los ntawm lub cev - qhov feem pua ntawm cov dej hauv lub cev muaj feem cuam tshuam nrog tus neeg mob lub cev yuav tsum hnyav dua lossis tsawg dua qhov feem pua ntawm lub cev yuag thaum lub sijhawm kuaj.
ND. Hauv ob lub hauv nruab nrab thiab nephrogenic ND, nyob rau hauv rooj plaub ntawm ua tiav ND, osmolarity ntawm cov zis tsis dhau lub plasma osmolarity qhov kawg ntawm kev sim nrog kev txwv dej. Raws li kev tawm tsam rau kev tswj hwm ntawm arginine-vasopressin lossis desmopressin, ob hom ntawv ntawm ND no tuaj yeem sib txawv. Nrog rau nephrogenic ND, qhov nce me ntsis ntawm osmolarity yog qhov ua tau tom qab kev tswj hwm ntawm arginine-vasopressin lossis desmopressin, tab sis tsis ntau tshaj 10% ua tiav thaum xaus ntawm lub sijhawm lub cev qhuav dej. Nrog rau hauv nruab nrab ND, kev tswj hwm ntawm arginine-vasopressin ua rau kom tso zis osmolarity ntau dua 50%.
Ua tsis tiav ND. Hauv cov neeg mob uas tsis muaj qhov tsis txaus ND, ob qho tib si nyob rau hauv nruab nrab thiab nephrogenic ND, tso zis osmolarity yuav dhau ntshav osmolarity thaum kawg ntawm kev sim nrog kev txwv dej. Nyob rau tib lub sijhawm, nrog ND hauv nruab nrab, plasma ntshav ADH qib qis dua qhov yuav tsum tau nrog qhov qib tau pom ntawm osmolarity, thaum nrog nephrogenic ND lawv tau txaus rau ib leeg.
Hypertonic Sodium Chloride Txoj Kev Yooj Yim
Txoj kev no tso cai rau qhov sib txawv tsis tiav ND los ntawm thawj polydipsia.
Txoj Kev thiab Kev Txhais Lus
Thaum lub caij sim no, qhov dej siab 3% sodium chloride tov txhaj koob rau ntawm tus nqi ntawm 0.1 ml / kg ib feeb rau 1-2 teev. Tom qab ntawv ADH cov ntsiab lus tau txiav txim siab thaum theem ntawm osmolarity thiab ntshav sodium tsis ncav cuag> 295 mOsm / l thiab 145 mEq / l, feem.
Hauv cov neeg mob nrog nephrogenic ND lossis thawj polydipsia, kev nce ntxiv ntawm cov ntshav ADH teb rau kev nce osmolarity yuav zoo li qub, thiab hauv cov neeg mob nrog ND nruab nrab, qhov nce tsis txaus hauv ADH kev zais cia tau sau lossis tsis tuaj ib qho.
Kev kho mob sim
Hom kab ke no ua rau sib txawv ntawm qhov tsis txaus hauv nruab nrab ND los ntawm qhov ua tsis tiav nephrogenic ND.
Txoj Kev thiab Kev Txhais Lus
Ua rau kev sim kho mob nrog desmopressin li 2-3 hnub. Qhov kev kho no tshem tawm lossis txo cov kev pom ntawm central ND thiab tsis cuam tshuam rau chav kawm ntawm nephrogenic ND. Hauv cov tshuaj polydipsia, kev teem sijhawm kho lub sijhawm tsis cuam tshuam rau kev haus dej, txawm hais tias qee zaum nrog ND nruab nrab, tus neeg mob yuav txuas ntxiv haus cov dej ntau ntxiv.
Ua ntej tshaj, koj yuav tsum paub tseeb tias tus neeg mob tau polyuria.
Tus neeg mob tsis koom nrog kev haus dej kom txog thaum lub cev hnyav dua li 5% ntawm thawj zaug lossis kev nqhis dej ua tsis tau. Rau qhov no, feem ntau, 8-12 teev yog txaus. Hauv cov neeg muaj kev noj qab haus huv, nyob rau hauv cov kev mob no, maj mam txo qhov ntau thiab nce ntawm qhov muaj peev xwm nce thiab qhov txheeb ze ntawm cov zis tau tshwm sim, thaum nyob hauv cov neeg mob ntshav qab zib insipidus, qhov ntim ntawm cov zis tawm tsis hloov pauv tsis tau, thiab nws osmolality tsis tshaj 300 mosm / l Kev nce hauv cov ntshav osmolality txog li 750 mosm / l qhia tias neurogenic mob ntshav qab zib insipidus.
Thaum kuaj pom nephrogenic mob ntshav qab zib insipidus yuav tsum muaj kev tshuaj xyuas txhua yam ntawm kev mob ntawm ob lub raum, tshem tawm cov cuam tshuam nrog electrolyte cuam tshuam.
Kev ua tib zoo sau keeb kwm ntawm tsev neeg, kev tshuaj ntsuam xyuas ntawm tus neeg mob cov neeg txheeb ze pub rau peb txheeb xyuas thiab cais cov qauv mob ntshav qab zib muaj ntshav qab zib insipidus.
Kev kho mob ntshav qab zib insipidus
Kev nqus dej kom txaus
Cov neeg mob uas muaj cov tsos mob me me ntawm ND (txhua hnub diuresis tsis pub tshaj 4 l) thiab lub cev muaj cov kev nqhis dej xav tias tsis tas yuav sau cov tshuaj kho mob, nws txaus los tsis txwv cov kua dej txaus.
ND Nruab Nrab. Qhia cov tshuaj pleev ib qho vasopressin - desmopressin.
Cov haujlwm feem ntau ntawm V2-receptors hauv lub raum thiab cov nyhuv me ntawm V receptors1 vasopressin hauv cov hlab ntsha. Yog li ntawd, cov tshuaj muaj txo cov ntshav siab ua haujlwm thiab antidiuretic yog kho kom zoo. Tsis tas li ntawd xwb, nws tau nce ib nrab-lub neej.
Cov tshuaj yuav tuaj yeem kho 2 zaug ib hnub hauv vaj huam sib luag, thiab cov tshuaj zoo hauv cov neeg mob sib txawv nws muaj ntau qhov sib txawv:
- qhov ncauj koob ntawm 100-1000 mcg / hnub,
- intranasal koob tshuaj 10-40 mcg / hnub,
- subcutaneous / intramuscular / leeg koob ntawm 0.1 mus 2 mcg / hnub.
Neesrogenic ND
- Lub hauv paus chiv keeb ntawm tus kab mob (metabolic lossis tshuaj) yog tshem tawm.
- Kev txhaj tshuaj siab ntawm desmopressin qee zaum muaj txiaj ntsig (piv txwv li, txog 5 mcg intramuscularly).
- Kev siv txaus ntawm cov kua dej txaus.
- Thiazide diuretics thiab prostaglandin inhibitors, xws li indomethacin, yuav pab tau zoo.
Kev mob hlwb psychogenic polydipsia yog qhov nyuaj rau kev kho thiab xav tau kev kho mob los ntawm tus kws kho mob hlwb.
Yog tias mob ntshav qab zib feem ntau insipidus tawm tsam keeb kwm yav dhau los ntawm kev hloov pauv hauv thaj chaw hypothalamic-pituitary, kev sim yuav tsum tau ua rau kev kho mob etiotropic (kev kho mob phais lossis hluav taws xob thiab tshuaj tua cov qog ntshav, los tiv thaiv kev kho mob rau sarcoidosis, mob rau daim npluag qhwv, thiab lwm yam).
Kev kho mob sib luag rau cov ntshav qab zib nephrogenic insipidus tseem tsis tau tsim los. Yog tias ua tau, qhov ua rau yuav kis tus kab mob yuav tsum tau muab tshem tawm (piv txwv li, txo qhov koob tshuaj ntawm lithium npaj). Cov neeg mob tau pom tias tsim nyog them nyiaj tsawg, ua kom ntsev ntau.
Tshuaj ntsuam tus kab mob ntshav qab zib insipidus
Ntshav qab zib insipidus tom qab kev ua haujlwm neurosurgical thiab raug mob hlwb feem ntau yog hloov, muaj kev kho txhua yam ntawm idiopathic cov ntaub ntawv ntawm tus kabmob tau piav qhia.
Cov kev soj ntsuam ntawm cov neeg mob uas tau txais neurogenic ntshav qab zib insipidus, raws li txoj cai, yog txiav txim siab los ntawm cov kab mob hauv qab los ua kev puas tsuaj rau lub hypothalamus lossis neurohypophysis, thiab concomitant adenohypophysis tsis txaus.