Tus Sau: William Ramirez
Hnub Kev Tsim: 24 Lub Cuaj Hli Ntuj 2021
Hloov Hnub: 17 Lub Kaum Ib Hli Ntuj 2024
Anonim
Autosomal hom tubulointerstitial raum mob - Tshuaj Kho Mob
Autosomal hom tubulointerstitial raum mob - Tshuaj Kho Mob

Autosomal pawg tubulointerstitial lub raum mob (ADTKD) yog ib pab pawg neeg mob raws caj ces uas cuam tshuam rau cov leeg ntawm ob lub raum, ua rau lub raum maj mam poob qhov peev xwm ua haujlwm.

ADTKD tshwm sim los ntawm kev sib pauv hauv qee cov noob. Cov noob teeb meem no tau dhau los ntawm cov tsev neeg (tau txais los ntawm niam txiv) hauv kev siv tus qauv tshwj xeeb. Qhov no txhais tau tias qhov txawv txav yog xav tau los ntawm ib tug niam txiv thiaj li yuav tau tus kabmob. Feem ntau, ntau tsev neeg muaj tus kab mob.

Nrog rau txhua daim ntawv ntawm ADTKD, thaum cov kab mob zuj zus mus, lub raum ua rau lub raum puas ntsoog. Cov no yog cov qauv hauv lub raum uas tso cai rau cov dej hauv cov ntshav ntau thiab yuav rov qab los ua ntshav.

Lawv cov noob ntshawb uas ua rau sib txawv ntawm ADTKD yog:

  • UMOD noob - ua rau ADTKD-UMOD, lossis uromodulin mob raum
  • MUC1 noob - ua rau ADTKD-MUC1, lossis mob ntsws-1 mob raum
  • REN noob - ua rau ADTKD-REN, los yog tsev neeg cov hluas nephropathy mob ntshav siab hom 2 (FJHN2)
  • HNF1B noob - ua rau ADTKD-HNF1B, lossis loj hlob-pib kev mob ntshav qab zib mellitus ntawm cov tub ntxhais hluas hom 5 (MODY5)

Thaum qhov ua kom muaj ADTKD tsis tau paub lossis keeb kwm tsis tau sim, nws hu ua ADTKD-NOS.


Thaum nyuam qhuav pib mob, nyob ntawm tsab ntawv ADTKD, cov tsos mob yuav muaj xws li:

  • Kev tso zis ntau dhau (polyuria)
  • Gout
  • Kev ntshaw ntsev
  • Tso zis thaum hmo ntuj (nocturia)
  • Neeg tsis muaj zog

Raws li tus kab mob zuj zus, cov tsos mob ntawm lub raum tsis ua hauj lwm yuav tshwm sim, uas suav nrog:

  • Ua kom doog ntshav yooj yim lossis los ntshav
  • Qaug zog, qaug zog
  • Nquag ntsos
  • Mob taub hau
  • Cov tawv nqaij nce ntxiv (tawv nqaij yuav tshwm sim daj lossis xim av)
  • Khaus
  • Malaise (kev mob siab xwb)
  • Mob leeg ntswj lossis mob plab
  • Xeev siab
  • Tawv nqaij daj
  • Txo kev hnov ​​mob ntawm txhais tes, taw, lossis lwm thaj chaw
  • Ntuav ntshav los ntshav hauv cov quav
  • Lub cev yuag
  • Qaug dab peg
  • Tsis meej pem, tsis meej pem, tsis nco qab

Tus kws kho mob yuav kuaj xyuas koj thiab nug koj cov tsos mob. Yuav nug koj seb lwm tsev neeg puas muaj ADTKD lossis mob raum.

Kev ntsuam xyuas uas yuav ua tau muaj xws li:

  • 24-teev tso zis thiab electrolytes
  • Ntshav urea nitrogen (BUN)
  • Ua kom tiav cov ntshav suav (CBC)
  • Kev kuaj ntshav Creatinine
  • Creatinine tshem tawm - ntshav thiab zis
  • Kev kuaj ntshav Uric acid
  • Cov zis tshwj xeeb nqus (yuav qis)

Cov ntawv sim hauv qab no tuaj yeem pab txheeb xyuas tus mob no:


  • Mob plab hauv TV
  • Mob plab hauv ultrasound
  • Raum raum
  • Lub raum ultrasound

Tsis muaj ib txoj kev kho tau rau ADTKD. Thaum xub thawj, kev kho mob tsom rau kev tswj hwm cov tsos mob, txo cov kev mob hnyav, thiab ua kom qeeb ntawm kev muaj mob. Vim tias dej thiab ntsev ntau dhau, koj yuav tsum tau ua raws li cov lus qhia txog kev haus dej haus ntau thiab haus tshuaj ntsev kom tsis txhob muaj lub cev qhuav dej.

Thaum tus kabmob zuj zus ntxiv, lub raum tsis ua haujlwm. Txoj kev kho yuav suav nrog kev noj tshuaj thiab hloov pauv kev noj zaub mov, txwv cov zaub mov muaj phosphorus thiab potassium. Tej zaum koj yuav tsum lim ntshav thiab ua lub raum pauv.

Lub hnub nyoog uas cov neeg mob ADTKD mus txog rau theem kawg mob raum mob sib txawv, nyob ntawm tus qauv mob. Nws tuaj yeem ua tus me hauv cov hluas lossis hauv cov laus. Kev kho mob mus ntev yuav tswj cov tsos mob ntawm tus mob raum.

ADTKD tuaj yeem ua rau cov mob hauv qab no:

  • Mob Kev Plab
  • Cov leeg tsis muaj zog thiab pob txha tawg
  • Mob Lub Hlwb
  • Hloov cov metabolism hauv cov piam thaj
  • Congestive lub plawv tsis ua haujlwm
  • Mob raum-theem kab mob
  • Lub plab zom zaws txoj hnyuv ntshav, mob txhab
  • Hemorrhage (los ntshav ntau heev)
  • Ntshav siab
  • Hyponatremia (ntshav muaj ntsev tsawg)
  • Hyperkalemia (ntau poov tshuaj nyob rau hauv cov ntshav), tshwj xeeb tshaj yog nrog kawg-theem raum mob
  • Hypokalemia (muaj poov tshuaj me me hauv ntshav)
  • Ntxiv lawm tshob
  • Teeb meem khaub ncaws
  • Kev Txij Nkawm
  • Mob rau hlwv
  • Mob paj hlwb peripheral
  • Platelet kawg nrog yooj yim bruising
  • Daim tawv xim hloov pauv

Hu teem sijhawm nrog koj tus kws khomob yog tias koj muaj cov tsos mob tso zis lossis mob raum.


Kab mob hauv lub raum Medullary yog kev muaj mob tsis sib haum. Nws yuav tsis tuaj yeem tiv thaiv.

ADTKD; Kab mob hauv lub raum Medullary; Renin mob raum mob; Tus kab mob ntshav siab hyperuricemic Familial; Uromodulin cuam tshuam lub raum mob

  • Raum anatomy
  • Lub raum cyst nrog gallstones - CT scan
  • Raum - ntshav thiab zis txaus

Bleyer AJ, Kidd K, Živná M, Kmoch S. Autosomal hom tubulointerstitial raum mob. Adv Ua Rog Kho raumCov. 2017; 24 (2): 86-93. PMID: 28284384 www.ncbi.nlm.nih.gov/pubmed/28284384.

Eckardt KU, Alper SL, Antignac C, li al. Autosomal tseem ceeb tubulointerstitial lub raum mob: kev kuaj mob, kev faib tawm, thiab kev tswj hwm - daim ntawv ceeb toom KDIGO pom zoo. Raum IntCov. Xyoo 2015; 88 (4): 676-683. PMID: 25738250 www.ncbi.nlm.nih.gov/pubmed/25738250.

Guay-Woodford LM. Lwm cov mob raum. Hauv: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Cov Chaw Kho Mob Tshaj LojCov. 6 tus ed. Philadelphia, PA: Elsevier; 2019: chap 45.

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