I-Nephrogenic isifo seswekile insipidus

I-Nephrogenic diabetes insipidus (NDI) sisifo apho isiphene kwimibhobho encinci (ityhubhu) kwizintso sibangela ukuba umntu adlule umchamo omkhulu kwaye aphulukane namanzi amaninzi.

Ngokwesiqhelo, ii-tubules zezintso zivumela amanzi amaninzi egazini ukuba acocwe kwaye abuyiselwe egazini.

I-NDI yenzeka xa izibilini zezintso zingaphenduli kwihomoni emzimbeni obizwa ngokuba yi-antidiuretic hormone (ADH), ekwabizwa ngokuba yi-vasopressin. I-ADH ibangela ukuba izintso zenze umchamo ugxile ngakumbi.

Ngenxa yokungaphenduli kwisiginali ye-ADH, izintso zikhupha amanzi amaninzi kumchamo. Oku kubangela ukuba umzimba uvelise umchamo omkhulu kakhulu.

I-NDI inqabile kakhulu. I-congenital nephrogenic diabetes insipidus ikhona xa izalwa. Sisiphumo sesiphene esidluliselwe kwiintsapho. Amadoda ahlala echaphazeleka, nangona abantu basetyhini bengayidlulisela le gene kubantwana babo.

Ngokuqhelekileyo, i-NDI ikhula ngenxa yezinye izizathu. Oku kubizwa ngokuba sisifo esifunyenwe. Izinto ezinokubangela ifom efunyenweyo yale meko ibandakanya:

• Ukuvalwa kwendlela yokuchama
• Amanqanaba aphezulu e-calcium
• Amanqanaba aphantsi e-potassium
• Ukusetyenziswa kwamachiza athile (i-lithium, demeclocycline, amphotericin B)

Unokuba nonxano olunzulu okanye olungalawulekiyo, kwaye unqwenela amanzi e-ice.

Uya kuvelisa izixa ezikhulu zomchamo, zihlala ngaphezulu kwe-3 yeelitha, kunye ne-15 yeelitha ngosuku. Umchamo uhlanjululwe kakhulu kwaye ujongeka ngathi ngamanzi. Kuya kufuneka uchame iyure nganye okanye nangaphezulu, nokuba kusebusuku xa ungatyi okanye ungaseli kakhulu.

Ukuba awuseli manzi aneleyo, ukuphelelwa ngamanzi emzimbeni kunokubangela. Iimpawu zingabandakanya:

• Inwebu eyomileyo eyomileyo
• Ulusu olomileyo
• Ukubonakala kwe-Sunken emehlweni
• I-Sunken fontanelles (indawo ethambileyo) kwiintsana
• Utshintsho kwimemori okanye kwibhalansi

Ezinye iimpawu ezinokuthi zenzeke ngenxa yokungabikho kwamanzi, okubangela ukungcola, kubandakanya:

• Ukudinwa, ukuziva ubuthathaka
• Intloko ebuhlungu
• Ukucaphuka
• Ubushushu obuphantsi bomzimba
• Intlungu yezihlunu
• Inqanaba lentliziyo elikhawulezayo
• Ukuhla ukusinda
• Utshintsho kulumko, nditsho nokudideka

Umboneleli wezempilo uya kukuvavanya kwaye abuze malunga neempawu zakho okanye zomntwana.

Uvavanyo lomzimba lunokubonisa:

• Uxinzelelo lwegazi oluphantsi
• Ukubetha ngokukhawuleza
• Umothuko
• Iimpawu zokuphelelwa ngamanzi emzimbeni

Uvavanyo lunokutyhila:

• Iserum ephezulu yesmolality
• Iziphumo eziphezulu zomchamo, nokuba ungasela kangakanani ulwelo
• Izintso musa ukugxila kumchamo xa unikwa i-ADH (ngesiqhelo iyeza elibizwa ngokuba yi-desmopressin)
• Umchamo osmolality osezantsi
• Amanqanaba aqhelekileyo okanye aphezulu e-ADH

Olunye uvavanyo olunokwenziwa lunokubandakanya:

• Uvavanyo lwegazi
• Umchamo weeyure ezingama-24
• Uvavanyo lokujonga umchamo
• Umchamo othile womxhuzulane
• Uvavanyo lokuncitshiswa kwamanzi
  

Injongo yonyango kukulawula amanqanaba omzimba womzimba. Kuya kunikwa isixa esikhulu samanzi. Isixa kufanele ukuba silingane nesixa samanzi alahlekileyo kumchamo.

Ukuba imeko ibangelwa liyeza elithile, ukumisa iyeza kunokuphucula iimpawu. Kodwa, musa ukuyeka ukuthatha nawaphi na amayeza ngaphandle kokuqala uthethe nomboneleli wakho.

Amayeza anokunikwa ukuphucula iimpawu ngokunciphisa ukuphuma komchamo.

Ukuba umntu usela amanzi aneleyo, le meko ayizukuchaphazela kakhulu ulwelo okanye i-electrolyte balance yomzimba. Ngamanye amaxesha, ukuhambisa umchamo omde ixesha elide kunokubangela ezinye iingxaki ze-electrolyte.

Ukuba umntu akaseli manzi ngokwaneleyo, ukuphuma komchamo okuphezulu kunokubangela ukomisa emzimbeni kunye namanqanaba aphezulu esodiyam egazini.

I-NDI ekhoyo ekuzalweni yimeko yexesha elide efuna unyango lobomi bonke.

Ukunganyangwa, i-NDI inokubangela nayiphi na kwezi zinto zilandelayo:

• Ukujikeleza kwe-ureters kunye ne-bladder
• Isodiyam yegazi ephezulu (hypernatremia)
• Ukuphelelwa ngamandla emzimbeni
• Umothuko
• Coma

Tsalela umnikezeli wakho ukuba wena okanye umntwana wakho uneempawu zesi sifo.

I-NDI ye-Congenital ayinakuthintelwa.

Ukunyanga ukuphazamiseka okungakhokelela kwifomu efunyenweyo yemeko kunokuthintela ekubeni kuphuhle kwezinye iimeko.

I-Nephrogenic isifo seswekile insipidus; Ukufumana i-nephrogenic diabetes insipidus; I-nephrogenic yesifo seswekile i-insipidus; NDI

• Inkqubo yomchamo wamadoda

I-Bockenhauer D. I-Fluid, i-electrolyte, kunye nokuphazamiseka kwesiseko seasidi ebantwaneni. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ii-eds. Brenner kunye noMphathi weZintso. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2020: isahl 73.

Ukuqhekeka kwe-DT, Majzoub JA. Isifo seswekile. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 574.

UHannon MJ, uThompson CJ. IVasopressin, isifo seswekile i-insipidus, kunye nesifo se-antidiuresis esingafanelekanga. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, ii-eds. Endocrinology: Abantu abadala kunye nabantwana. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; 2016: isahl 18.

Scheinman SJ. Ukuphazamiseka kwezithuthi kwizintso. Ku: Gilbert SJ, Weiner DE, ii-eds. Isiseko seZintso seSizwe seZintso. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 38.