Matenda a impso a Autosomal ofala kwambiri

Matenda a impso a Autosomal omwe ali ndi kachilombo koyambitsa matendawa (ADTKD) ndi gulu lazikhalidwe zomwe zimakhudzidwa zomwe zimakhudza ma tubules a impso, zomwe zimapangitsa impso kutaya mwayi wawo wogwira ntchito.

ADTKD imayambitsidwa ndi kusintha kwa majini ena. Mavuto amtunduwu amapitilira m'mabanja (obadwa nawo) mofanana. Izi zikutanthauza kuti jini yosazolowereka imafunika kuchokera kwa kholo limodzi kuti athe kutenga matendawa. Nthawi zambiri, mamembala ambiri amakhala ndi matendawa.

Ndi mitundu yonse ya ADTKD, matendawa akamakula, ma tubules a impso awonongeka. Izi ndizida mu impso zomwe zimalola kuti madzi ambiri m'magazi azisefedwa ndikubwezeretsedwanso m'magazi.

Mitundu yawo yachilendo yomwe imayambitsa mitundu yosiyanasiyana ya ADTKD ndi iyi:

• UMOD jini - imayambitsa ADTKD-UMOD, kapena matenda a impso a uromodulin
• MUC1 jini - imayambitsa ADTKD-MUC1, kapena matenda a impso a mucin-1
• REN jini - imayambitsa ADTKD-REN, kapena banja lachinyamata la hyperuricemic nephropathy mtundu wa 2 (FJHN2)
• HNF1B jini - imayambitsa ADTKD-HNF1B, kapena kukula kwa matenda ashuga a mtundu wachinyamata 5 (MODY5)

Ngati chifukwa cha ADTKD sichikudziwika kapena kuyesa kwa majini sikunachitike, amatchedwa ADTKD-NOS.

Kumayambiriro kwa matendawa, kutengera mtundu wa ADTKD, zizindikilo zimatha kuphatikiza:

• Kukodza kwambiri (polyuria)
• Gout
• Kulakalaka mchere
• Kukodza usiku (nocturia)
• Kufooka

Matendawa akamakulirakulira, zizindikilo za impso zitha kuyamba, monga:

• Kuvulaza kosavuta kapena kutuluka magazi
• Kutopa, kufooka
• Ma hiccups pafupipafupi
• Mutu
• Mtundu wowonjezera wa khungu (khungu limawoneka lachikaso kapena lofiirira)
• Kuyabwa
• Malaise (kudwala)
• Minofu kugwedezeka kapena kukokana
• Nseru
• Khungu lotumbululuka
• Kuchepetsa kumva kwa manja, mapazi, kapena madera ena
• Kusanza magazi kapena magazi mu chopondapo
• Kuchepetsa thupi
• Kugwidwa
• Chisokonezo, kuchepa kwa chidwi, kukomoka

Wothandizira zaumoyo adzakufunsani ndikufunsani za matenda anu. Muyenera kuti mudzafunsidwa ngati abale ena ali ndi ADTKD kapena matenda a impso.

Mayeso omwe angachitike ndi awa:

• Vuto la mkodzo wamaola 24 ndi ma electrolyte
• Magazi urea asafe (BUN)
• Kuwerengera kwathunthu kwa magazi (CBC)
• Kuyesa magazi kwa Creatinine
• Chilolezo cha Creatinine - magazi ndi mkodzo
• Uric acid kuyesa magazi
• Mphamvu yokoka (idzakhala yochepa)

Mayesero otsatirawa angathandize kuzindikira vutoli:

• M'mimba mwa CT scan
• M'mimba ultrasound
• Kusokoneza impso
• Impso ultrasound

Palibe mankhwala a ADTKD. Poyamba, chithandizo chimayang'ana kuwongolera zizindikilo, kuchepetsa zovuta, ndikuchepetsa kukula kwa matendawa. Chifukwa madzi ndi mchere wambiri watayika, muyenera kutsatira malangizo akumwa zakumwa zamadzimadzi ambiri ndikumwa mankhwala owonjezera amchere kupewa kuperewera kwa madzi m'thupi.

Matendawa akamakula, matenda a impso amakula. Chithandizochi chingaphatikizepo kumwa mankhwala ndi kusintha kwa zakudya, kuchepetsa zakudya zomwe zili ndi phosphorous ndi potaziyamu. Mungafunike dialysis ndi kumuika impso.

Zaka zomwe anthu omwe ali ndi ADTKD amafika kumapeto kwa matenda a impso amasiyana, kutengera mtundu wa matendawa. Atha kukhala achichepere monga achinyamata kapena achikulire. Chithandizo cha moyo wonse chitha kuwongolera zizindikilo za matenda a impso.

ADTKD ingayambitse mavuto awa:

• Kuchepa kwa magazi m'thupi
• Bone kufooka ndi fractures
• Tamponade yamtima
• Kusintha kwa kagayidwe ka shuga
• Kulephera kwa mtima
• Matenda omaliza a impso
• Kutuluka m'mimba, zilonda
• Kutaya magazi (kutaya magazi kwambiri)
• Kuthamanga kwa magazi
• Hyponatremia (magazi otsika kwambiri)
• Hyperkalemia (potaziyamu wambiri m'magazi), makamaka ndimatenda a impso
• Hypokalemia (potaziyamu wochepa kwambiri m'magazi)
• Kusabereka
• Mavuto akusamba
• Kupita padera
• Matenda a m'mapapo
• Matenda a m'mitsempha
• Kulephera kwa ma Platelet ndi mabala osavuta
• Mtundu wa khungu umasintha

Itanani msonkhano ndi omwe amakupatsani ngati muli ndi vuto lililonse la kwamikodzo kapena impso.

Matenda a impso a medullary cystic ndi matenda obadwa nawo. Sizingatheke kupezeka.

ADTKD; Matenda a impso a medullary cystic; Matenda a impso a Renin; Wodziwika bwino wachinyamata wa hyperuricemic nephropathy; Matenda a impso a Uromodulin

• Matenda a impso
• Impso chotupa ndi ma gallstones - CT scan
• Impso - kutuluka magazi ndi mkodzo

Bleyer AJ, Kidd K, Živná M, Kmoch S. Autosomal wamkulu wa matenda a impso a tubulointerstitial. Adv Chronic Impso Dis. 2017; 24 (2): 86-93. PMID: 28284384 www.ncbi.nlm.nih.gov/pubmed/28284384. (Adasankhidwa)

Eckardt KU, Alper SL, Antignac C, ndi al. Matenda a impso a Autosomal opambana kwambiri: kuzindikira, kugawa, ndi kuwongolera - lipoti logwirizana la KDIGO. Impso Int. 2015; 88 (4): 676-683. PMID: 25738250 www.ncbi.nlm.nih.gov/pubmed/25738250.

Masewera a Guay-Woodford LM. Matenda ena a impso. Mu: Feehally J, Floege J, Tonelli M, Johnson RJ, olemba. Chachikulu Chachipatala Nephrology. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2019: mutu 45.