Akubona abantu abaningi bazi ukuthi ngaphandle kwakho konke uhlobo 1 nohlobo 2 lwesifo sikashukela esijwayelekile, kukhona ne-insipidus yesifo sikashukela. Lesi yisifo sezindlala ze-endocrine, kuyisifo sohlelo lwe-hypothalamic-pituitary. Ngakho-ke, isifo esinjalo empeleni asihlangene nesifo sikashukela, ngaphandle kwegama nokoma okungapheli.
Ngoshukela we-insipidus, ukuvelela okuyingxenye noma okuphelele kwe-vasidiopathy ye-antidiuretic vasopressin kuyaphawulwa. Inqoba ingcindezi nezitolo ze-osmotic, bese isabalalisa uketshezi emzimbeni wonke.
Ngakho-ke, i-hormone inikeza inani lamanzi elidingakalayo, ivumela izinso ukuba zisebenze ngokujwayelekile. Ngakho-ke, i-vasopressin iyadingeka i-homeostasis yemvelo, ngoba iqinisekisa ukusebenza kwayo okujwayelekile ngisho nokuntuleka komswakama emzimbeni.
Esimweni esibucayi, ngokwesibonelo, ngesikhathi sokuphelelwa ngumzimba, ubuchopho buthola isibonakaliso elilawula ukusebenza kwezitho zomzimba. Lokhu kusiza ukunciphisa ukulahleka kukamanzi ngokunciphisa ukuhamba kwamathe nomchamo.
Ngakho-ke, i-insipidus yesifo sikashukela ihlukile kushukela ngoba lapho kwenzeka, inkomba ye-glucose esegazini ihlala ijwayelekile, kepha zombili lezi zifo zinophawu oluvamile - polydipsia (ukoma okunzima). Ngakho-ke, i-insipidus yesifo sikashukela, ebonakala ngokubuyisa okungamanzi okuvela ezingxenyeni zezinso, ithole leli gama.
Inkambo ye-ND imvama kakhulu. Ubhekwa njengesifo sabancane, yingakho isigaba seziguli sifika eminyakeni engama-25. Ngaphezu kwalokho, ukwephulwa kwezindlala ze-endocrine kungenzeka kwabesifazane nabesilisa.
Isifo sikashukela: izinhlobo
Kukhona i-insipidus ephakathi ne-nephrogenic. I-LPC yona ihlukaniswe izinhlobo ezimbili:
- ukusebenza;
- organic.
Uhlobo olusebenzayo luhlukaniswa njengefomu le-idiopathic. Izici ezithinta ukubonakala kwalolu hlobo lwenyama azikasungulwa ngokuphelele, kepha odokotela abaningi bakholelwa ukuthi ifa lidlala indima enkulu ekwenziweni kwalesi sifo. Futhi, izizathu zilele ekuphulekeni okuningana kokuhlanganiswa kwe-hormone neurophysin noma vasopressin.
Uhlobo lwe-organic yalesi sifo luvela ngemuva kokulimala okuhlukahlukene, ukuhlinzwa kanye neminye imihuzuko.
I-insulinidi yesifo sikashukela yeNephrogenic iba lapho ukusebenza kwemvelo kwezinso kukhubazekile. Kwezinye izimo, kukhona ukwehluleka ekucindezelweni kwe-osmotic ye-renal tubules, kwezinye izimo, ukutholakala kwama-tubules kuya kwe-vasopressin kuyehla.
Kukhona futhi uhlobo olunjenge-psychogenic polydipsia. Kungabangelwa ukusetshenziswa kabi kwezidakamizwa noma i-PP ingenye yokubonakaliswa kwe-schizophrenia.
Izinhlobo ezinjalo ezingandile ze-ND njengohlobo lwe-gestagen ne-polyuria yesikhashana nazo ziyahlukaniswa. Esimweni sokuqala, i-enzyme ye-placenta isebenza kakhulu, enomphumela omubi kwi-hormone ye-antidiuretic.
Uhlobo lwesikhashana sikashukela luba khona ngaphambi kweminyaka engu-1.
Lokhu kwenzeka lapho izinso zakhiwa ngaphansi, lapho ama-enzyme abandakanyeka ezinqubweni ze-metabolic eqala ukuziphatha kahle.
Izimbangela kanye nezimpawu zesifo
Kunezici eziningi eziholela ekuthuthukisweni kwesifo sikashukela:
- ukwakheka kwesimila;
- izifo ezingalapheki futhi ezinzima kakhulu (i-postpsum sepsis, umkhuhlane, i-syphilis, i-typhoid, umkhuhlane obomvu kanye nabanye);
- ukwelashwa ngemisebe;
- i-jade;
- ukulimala kwemithambo yegazi nezingxenye zobuchopho;
- ukulimala kwengqondo noma ukuhlinzwa;
- i-amyloidosis;
- i-granulomatosis;
- hemoblastosis.
Izifo ze-Autoimmune kanye nokukhubazeka kwengqondo nakho kunomthelela ekwenzekeni kwe-ND. Futhi ngesimo se-idiopathic yalesi sifo, imbangela yalokho kwenzeka ukubukeka okubukhali kwama-antibodies ngokumelene namaseli akhiqiza ama-hormone.
Isimo somtholampilo soshukela i-insipidus sehlukahlukene, siqala ngekhanda futhi siphela ngokuphelelwa ngamanzi ngenxa yokungabikhona kokudla kwenani elilinganayo lamanzi. Ngakho-ke, ngaphezu kokuhlolwa, kwenziwa ukuhlolwa okuhlukahlukene kwesifo sikashukela.
Izimpawu eziphambili zalesi sifo zibandakanya:
- ukuphazamiseka endaweni yokugaya ukudla - ukuqunjelwa, i-gastritis, i-colitis, isifiso sokudla;
- ukoma okukhulu;
- ukungasebenzi ngocansi;
- ukuphazamiseka kwengqondo - ukulala kabi, ukungasebenzi, ikhanda elibi, ukukhathala;
- ukuchama kaningi ngenani eliningi lamanzi akhutshiwe (amalitha ayi-6-15);
- ukomisa ulwelwesi lwama-mucous nesikhumba;
- ukungahambi kahle kokubuka ushukela;
- ukuncipha;
- i-anorexia;
- i-asthenic syndrome.
Imvamisa, i-insipidus yesifo sikashukela ihambisana nengcindezi yangaphakathi futhi yehlise ukujuluka. Ngaphezu kwalokho, uma isiguli singaphuzi amanzi anele, isimo sakhe sizothuthuka. Ngenxa yalokhu, isiguli singaba nezimpawu ezifana nokuopha igazi, ukugabha, isicanucanu, i-tachycardia, umkhuhlane nokuwohloka komzimba kuvela ngemuva kokuphelelwa ngamandla emzimbeni. Kwabesifazane abane-ND, umjikelezo wokuya esikhathini uyaphambuka, futhi amadoda awanalo i-potency emfushane.
Ezinganeni, inkambo yalesi sifo ingaholela ekwehlekeni kokukhula ngokocansi nangokomzimba.
Ukuxilonga
Ukubona ubukhona be-ND, kuhlolwa izigaba ezintathu zokuxilonga:
- ukutholwa kwe-hypotonic polyuria (i-urinalysis, ukuhlolwa kweZimnitsky, ukuhlolwa kwegazi nge-biochemical);
- izivivinyo ezisebenzayo (ukuhlolwa kwe-desmopressin, ukoma);
- ukutholwa kwezimbangela ezacunula ukuthuthukiswa kwalesi sifo (MRI).
Isigaba sokuqala
Ekuqaleni, uma kusolakala ukuthi isifo sikashukela, isifo sikashukela siyahlolwa ukuthola ukuthi umchamo ungakanani. Ngemuva kwakho konke, ngalesi sifo, ukusebenza kwezinso kuya ngokuya kukhula, ngenxa yalokho, izinkomba zokuxinana komchamo zingaphansi kwe-1005 g / l.
Ukuthola izinga lobuningi phakathi nosuku, kwenziwa ucwaningo eZimnitsky. Ukuhlaziywa okunjalo kwenziwa njalo emahoreni amathathu amahora angama-24. Ngalesi sikhathi, kuthathwa amasampula womchamo ayi-8.
Imvamisa, imiphumela inqunywa ngale ndlela: inani lokujwayelekile lomchamo wansuku zonke akumele lidlule amalitha ama-3, ubuningi bawo buyi-1003-1030, kanti isilinganiso sokuphuma komchamo ubusuku nemini ngu-1: 2, kanti inani lamanzi akhishwe futhi adakiwe yi-50-80-100%. Umchamo osmolarity - 300 mosm / kg.
Futhi, ukuhlolwa kwegazi kwe-biochemical kwenziwa ukuze kutholakale i-ND. Kulokhu, i-osmolarity yegazi ibalwa. Uma kunokugcwala okukhulu kukasawoti ku-plasma engaphezu kwama-292 mosm / l nokuqukethwe okweqile kwe-sodium (kusuka ku-145 nmol / l), kutholakala ukuthi isifo sikashukela siyatholakala.
Igazi lithathwa emthanjeni esiswini esingenalutho. Ngaphambi kwenqubo (amahora ayi-6 kuya kwele-12) ungaphuza kuphela amanzi. Njengomthetho, imiphumela yezivivinyo idinga ukulinda usuku olulodwa.
Ngaphezu kwalokho, ngokuhlaziywa kwegazi okubandakanya izinto eziphilayo, amanani anjengokuthi:
- ushukela
- potassium nesodium;
- inani lamaprotheni, kufaka phakathi i-hemoglobin;
- i-ionized calcium;
- i-creatinine;
- i-parathyroid hormone;
- i-aldosterone.
Inkomba kashukela wegazi imvamisa ifinyelela ku-5.5 mmol / l. Kodwa-ke, nge-ND, ukugxila kwe-glucose kuvame ukwanda. Kepha ukuguquguquka kwayo kungabhekwa ngokuxineka okuqinile ngokomzwelo noma ngokomzimba, izifo zamanyikwe, i-pheochromocytoma kanye nokuhluleka kwesibindi nokuqina kwezinso. Ukwehla kokuhlushwa ushukela kwenzeka ngokuphulwa kokusebenza kwezindlala ze-endocrine, indlala, isimila kanye nokuphuza kakhulu.
I-Potassium ne-sodium yizinto zamakhemikhali ezivumela ulwelwesi lwamaseli ngezinto zikagesi. Okuqukethwe pot potum okujwayelekile ngu-3.5 - 5.5 mmol / L. Uma inkomba yayo iphezulu kakhulu, khona-ke lokhu kukhombisa ukungasebenzi kahle kwesibindi ne-adrenal, ukulimala kweseli nokuphelelwa ngamandla komzimba. Amazinga aphansi we-potassium abonwa ngesikhathi sokuzila ukudla, izinkinga zezinso, ukweqiwa kwama-hormone athile, ukuphuma komzimba, ne-cystic fibrosis.
Isimo se-sodium emfuleni wegazi sisuka ku-136 siye ku-145 mmol / l. I-Hypernatremia yenzeka ngokudla usawoti ngokweqile, ukwehluleka ekulinganiselweni kukasawoti wamanzi, ukuqina kwethambo lendlala kwe-adrenal cortex. Futhi i-hyponatremia yenzeka ngokusebenzisa umthamo omkhulu we-fluid futhi esimweni se-pathologies yezinso nezindlala ze-adrenal.
Ukuhlaziywa kwamaprotheni ephelele kuveza izinga le-albhamuin ne-globulin. Inkomba ejwayelekile yamaprotheni agcwele egazini kubantu abadala yi-64-83 g / l.
Okubaluleke kakhulu ekutholakaleni kwesifo sikashukela i-hemoglobin ye-glycosylated. I-Ac1 ikhombisa ushukela ojwayelekile weshukela ngaphezulu kwamasonto ayi-12.
IHemoglobin iyinto ekhona kumaseli ebomvu akhipha umoya-mpilo kuzo zonke izitho nezinhlelo. Kubantu abangenaso isifo sikashukela, i-glycosylated hemoglobin esegazini ayidluli i-4-6%, okubuye kube yisimpawu sokuthola isifo sikashukela. Ngakho-ke, ama-indices we-ac1 we-opverestimated angahlukanisa lezi zifo.
Kodwa-ke, ukuguquguquka kwamazinga we-hemoglobin kungenzeka nge-anemia, ukusetshenziswa kwezithasiselo zokudla, ukudla kwamavithamini E, C kanye ne-cholesterol eyeqile. Ngaphezu kwalokho, i-glycosylated hemoglobin ingaba nezinkomba ezihlukile ezifweni zesibindi nezinso.
Izinga le-ionized calcium liyinkomba ebhekene ne-mineral metabolism. Amanani wayo aphakathi nendawo aqala ku-1,05 kuya ku-1.37 mmol / L.
Futhi, ukuhlolwa kwesifo sikashukela i-insipidus kubandakanya ukuhlolwa kwegazi kokuqukethwe kwe-aldosterone. Ukushoda kwale-hormone kuvame ukukhombisa ubukhona besifo sikashukela.
Izinga elikhulayo le-creatinine ne-parathyroid hormone lingakhombisa nokuba khona kwalesi sifo.
Isigaba sesibili
Kulesi sigaba, kuyadingeka ukuthi udwebe umthetho olandelwayo wohlolo olomile. Isigaba sokuphelelwa ngamanzi sihlanganisa:
- isampula yegazi ukuhlola ama-osmolality kanye namazinga e-sodium;
- ukuthatha umchamo ukuthola ubungako bawo kanye nokuhlukahluka;
- isineke;
- ukukalwa kwesilinganiso senhliziyo nomfutho wegazi.
Kodwa-ke, nge-hypernatremia, ukuhlolwa okunjalo kufakwa ku-contraindified.
Kuyaqapheleka ukuthi ngesikhathi sokuhlolwa awukwazi ukudla ukudla okune-carbohydrate okusheshayo okunenkomba ye-glycemic ephezulu. Okuncamelayo kufanele kunikezwe kunhlanzi, inyama ebusayo, amaqanda abilisiwe, isinkwa sokusanhlamvu.
Ukuhlolwa okwomile kuyamiswa uma: izinga le-osmolality ne-sodium lidlula okujwayelekile, kuvela ukoma okungabekezeleleki futhi kube nokunciphisa isisindo okungaphezulu kwe-5%.
Ukuhlolwa kwe-desmopressin kwenziwa ukuhlukanisa phakathi kwe-insipidus ephakathi ne-nephrogenic. Kususelwa ekuhlolweni kokuzwela kwesiguli nge-desmopressin. Ngamanye amagama, ukusebenza okusebenzayo kwama-V2 receptors kuyahlolwa. Lolu cwaningo lwenziwa ngemuva kovivinyo olomile olunokuvezwa okuphezulu kakhulu kuma-WUA endolo.
Ngaphambi kokuhlaziywa, isiguli kufanele sichame. Ngemuva kwalokho unikezwa i-desmopressin, kuyilapho akwazi ukuphuza futhi adle, kodwa ngokulinganisela. Ngemuva kwamahora we-2-4, umchamo uthathwa ukunquma i-osmolality yayo nevolumu.
Imvamisa, imiphumela yocwaningo ingu-750 mOsm / kg.
Esimweni se-NND, ama-indices akhuphuka afinyelela kuma-300 mOsm / kg, kuthi esimweni se-LPC ngemuva kokuphelelwa amanzi emzimbeni, angama-300, kanye ne-desmopressin - 750 mOsm / kg.
Isigaba sesithathu
Imvamisa, i-MRI yenziwa ukuthola isifo sikashukela. Kumuntu ophilile ku-pituitary gland, umehluko ocacile phakathi kwamagobongo angaphandle nangaphandle uyabonakala. Ngaphezu kwalokho, lokhu okugcina esithombeni se-T1 kunophawu olukhulu kakhulu. Lokhu kungenxa yobukhona bayo yemiganu eyimfihlo equkethe ama-phospholipids nama-WUA.
Ebukhoneni be-LPC, isignali ekhishwe yi-neurohypophysis ayikho. Lokhu kungenxa yokungasebenzi kahle ekwakhiweni nasekuthuthweni nasekugcinweni kwama-granules we-neurosecretory.
Futhi, ngesifo sikashukela i-insipidus, ukuhlolwa kwe-neuropsychiatric, eyethalmological and radiological kungenziwa. Futhi ngefomu le-renal lalesi sifo, i-ultrasound ne-CT yezinso ziyenziwa.
Inketho ehamba phambili yokwelashwa kwe-NND ukuthatha ama-synthes vasopressin analog (Desmopressin, Chlorpropamide, Adiuretin, Minirin). Ngefomu le-renal, ama-diuretics nama-NSAID anqunywe.
Noma yiluphi uhlobo lwesifo sikashukela lubandakanya ukwelashwa kokungena okususelwa kusawoti. Lokhu kuyadingeka ukulungisa umzimba wamasawoti-usawoti.
Ukuhambisana nokudla okuthile akubalulekile kangako, kufaka phakathi ukuthamba usawoti (4-5 g) namaprotheni (kuze kufike ku-70 g). Lezi zidingo zihambelana nokudla uNo. 15, 10 no-7.
Yiziphi izivivinyo okufanele zithathwe uma usola ukuthi i-insipidus yesifo sikashukela ichazwe kuvidiyo kule ndatshana.
