Nephrogenic lefu la tsoekere insipidus
Nephrogenic lefu la tsoekere insipidus (NDI) ke bokuli boo ho bona sekoli methapong e menyenyane (li-tubules) liphio se etsang hore motho a fete moroto o mongata mme a lahleheloe ke metsi a mangata.
Ka tloaelo, li-tubules tsa liphio li lumella metsi a mangata maling hore a tlhotloe ebe a khutlisetsoa maling.
NDI e etsahala ha lipeipi tsa liphio li sa arabele hormone e 'meleng e bitsoang antidiuretic hormone (ADH), eo hape e bitsoang vasopressin. Hangata ADH e etsa hore liphio li etse hore moroto o be matla haholo.
Ka lebaka la ho se arabele lets'oao la ADH, liphio li ntša metsi a mangata haholo ka morong. Sena se etsa hore 'mele o hlahise moroto o moholo haholo.
NDI ha e fumanehe hangata. Congenital nephrogenic lefu la tsoekere insipidus le teng ha a hlaha. Ke ka lebaka la phoso e fetisitsoeng ke malapa. Hangata banna baa ameha, leha basali ba ka fetisetsa mofuta ona ho bana ba bona.
Hangata, NDI e ba teng ka lebaka la mabaka a mang. Sena se bitsoa boloetse bo fumanoeng. Lintho tse ka hlahisang sebopeho se fumanoeng sa boemo bona li kenyelletsa:
- Thibelo ea pampitšana ea ho ntša metsi
- Maemo a phahameng a calcium
- Maemo a potasiamo a tlase
- Ts'ebeliso ea lithethefatsi tse itseng (lithium, demeclocycline, amphotericin B)
U kanna oa ba le lenyora le leholo kapa le sa laoleheng, 'me u lakatsa metsi a leqhoa.
U tla hlahisa moroto o mongata, hangata ho feta lilithara tse 3, le ho fihlela lilithara tse 15 ka letsatsi. Moroto o hlapolotse haholo mme o shebahala joalo ka metsi. U kanna ua hloka ho ntša metsi hora e 'ngoe le e' ngoe kapa le ho feta, esita le bosiu ha u sa je kapa u sa noe haholo.
Haeba u sa noe maro a lekaneng, ho ka fella ka ho felloa ke metsi 'meleng. Matšoao a ka kenyelletsa:
- Lera le omileng
- Letlalo le ommeng
- Ponahalo e soabileng mahlong
- Sunken fontanelles (sebaka se bonolo) ho masea
- Liphetoho mohopolong kapa ho leka-lekana
Matšoao a mang a ka hlahang ka lebaka la khaello ea maro, a bakang ho felloa ke metsi 'meleng, a kenyelletsa:
- Mokhathala, ho ikutloa o fokola
- Ho opeloa ke hlooho
- Ho teneha
- Mocheso oa 'mele o tlase
- Bohloko ba mesifa
- Ho otla ha pelo ka potlako
- Ho fokotsa boima ba 'mele
- Phetoho ea ho falimeha, esita le ho akheha
Mofani oa tlhokomelo ea bophelo o tla u hlahloba 'me a botse ka matšoao a hau kapa a ngoana oa hau.
Tlhahlobo ea 'mele e ka senola:
- Khatello ea mali e tlase
- Pelo e potlakileng
- Ho tshoha
- Matšoao a ho felloa ke metsi 'meleng
Liteko li ka senola:
- Serum osmolality e phahameng
- Phallo e phahameng ea moroto, ho sa tsotelehe hore na u noa mokelikeli o kae
- Liphio ha li tsepamise mohopolo ha u fuoa ADH (hangata moriana o bitsoang desmopressin)
- Motsoako o tlase oa moroto
- Maemo a tloaelehileng kapa a phahameng a ADH
Liteko tse ling tse ka etsoang li kenyelletsa:
- Teko ea mali ea sodium
- Moroto oa lihora tse 24
- Teko ea khatello ea moroto
- Motsoako o itseng oa khoheli
- Teko e hlokolosi ea ho hloka metsi
Morero oa kalafo ke ho laola maemo a mokelikeli oa 'mele. Ho tla fuoa metsi a mangata. Chelete eo e lokela ho lekana le palo ea metsi a lahleheloang ke moroto.
Haeba boemo bo bakoa ke moriana o itseng, ho emisa sethethefatsi ho ka ntlafatsa matšoao. Empa, U SE KE UA emisa ho noa moriana o fe kapa o fe pele u bua le mofani oa litšebeletso.
Meriana e ka fanoa ho ntlafatsa matšoao ka ho fokotsa tlhahiso ea moroto.
Haeba motho a noa metsi a lekaneng, boemo bona bo ke ke ba e-ba le phello e kholo tekanyong ea mokelikeli kapa elektrolyte ea 'mele. Ka linako tse ling, ho fetisa moroto o mongata nako e telele ho ka baka mathata a mang a electrolyte.
Haeba motho a sa noe maro a lekaneng, tlhahiso e phahameng ea moroto e ka baka ho felloa ke metsi 'meleng le maemo a phahameng a sodium maling.
NDI e teng ha motho a hlaha ke boemo ba nako e telele bo hlokang kalafo ea bophelo bohle.
Ha e sa alafshoa, NDI e ka baka eng kapa eng ea tse latelang:
- Ho senyeha ha li-ureters le senya
- Sodium e phahameng ea mali (hypernatremia)
- Ho felloa ke metsi 'meleng haholo
- Ho tshoha
- Coma
Bitsa mofani oa hau haeba uena kapa ngoana oa hau u na le matšoao a lefu lena.
Congenital NDI e ke ke ea thibeloa.
Ho sebetsana le mathata a ka lebisang mofuteng oa boemo bo ka e thibela ho hola maemong a mang.
Nephrogenic lefu la tsoekere insipidus; Fumaneha nephrogenic lefu la tsoekere insipidus; Congenital nephrogenic lefu la tsoekere insipidus; NDI
- Tsamaiso ea monna ea ho ntša metsi
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