Matenda a Alport

Matenda a Alport ndi matenda obadwa nawo omwe amawononga mitsempha yaying'ono ya impso. Zimayambitsanso kumva ndi mavuto amaso.

Matenda a Alport ndi mtundu wa impso yotupa (nephritis). Zimayambitsidwa ndi chilema (kusintha) mu jini la puloteni munyama yolumikizana, yotchedwa collagen.

Matendawa ndi osowa. Pali mitundu itatu ya majini:

• X-linked Alport syndrome (XLAS) - Uwu ndiye mtundu wofala kwambiri. Matendawa amakula kwambiri mwa amuna kuposa akazi.
• Autosomal recessive Alport syndrome (ARAS) - Amuna ndi akazi ali ndi matenda ofananiranso.
• Autosomal dominant Alport syndrome (ADAS) - Uwu ndiye mtundu wosowa kwambiri. Amuna ndi akazi ali ndi matenda oopsa mofanana.

MAFUPA

Ndi mitundu yonse ya matenda a Alport impso zimakhudzidwa. Mitsempha ing'onoing'ono yamagazi mu glomeruli ya impso yawonongeka. Glomeruli imasefa magazi kuti apange mkodzo ndikuchotsa zonyansa m'magazi.

Poyamba, palibe zisonyezo. Popita nthawi, ma glomeruli akucheperachepera, magwiridwe antchito a impso amatayika ndipo zinthu zotayika ndi madzi zimakhazikika mthupi. Vutoli limatha kupitilira mpaka kumapeto kwa matenda a impso (ESRD) ali aang'ono, pakati paunyamata ndi zaka 40. Pakadali pano, dialysis kapena impso kumuika kumafunika.

Zizindikiro za mavuto a impso ndi monga:

• Mtundu wosadziwika wa mkodzo
• Magazi mkodzo (omwe amatha kukulitsa matenda opuma opuma kapena kuchita masewera olimbitsa thupi)
• Kumva kupweteka
• Kuthamanga kwa magazi
• Kutupa mthupi lonse

MAKUTU

Popita nthawi, matenda a Alport amathandizanso kuti asamve bwino. Pofika zaka zoyambirira, zimakhala zachilendo mwa amuna omwe ali ndi XLAS, ngakhale mwa akazi, kumva kwakumva sikofala ndipo kumachitika akakula. Ndi ARAS, anyamata ndi atsikana ali ndi vuto lakumva ali ana. Ndi ADAS, zimachitika pambuyo pake m'moyo.

Kumva kwakanthawi kochepa kumachitika impso zisanachitike.

MASO

Matenda a Alport amachititsanso mavuto amaso, kuphatikiza:

• Maonekedwe osazolowereka a mandala (anterior lenticonus), omwe amatha kubweretsa kuchepa pang'onopang'ono kwa masomphenya komanso mathithi.
• Kukokoloka kwa m'mimba komwe kumataya gawo lakunja la chophimba cha diso, kumabweretsa kupweteka, kuyabwa, kapena kufiyira kwa diso, kapena kusawona bwino.
• Mitundu yachilendo ya diso, vuto lotchedwa dot-and-fleck retinopathy. Sizimayambitsa mavuto a masomphenya, koma zitha kuthandiza kuzindikira matenda a Alport.
• Macular hole pomwe pali kupatulira kapena kupumula kwa macula. Macula ndi gawo la diso lomwe limapangitsa kuti masomphenya akuthwa ndikulongosola bwino. Phokoso laling'ono limayambitsa masomphenya apakati kapena osokonekera.

Wothandizira zaumoyo adzakufunsani ndikufunsani za matenda anu.

Mayesero otsatirawa akhoza kuchitika:

• BUN ndi serum creatinine
• Kuwerengera kwathunthu kwa magazi
• Zosokoneza bongo
• Kupenda kwamadzi

Ngati wothandizira wanu akukayikira kuti muli ndi matenda a Alport, mudzakhalanso ndi mayesero a masomphenya ndi makutu.

Zolinga zamankhwala zimaphatikizapo kuwunika ndikuwongolera matendawa ndikuchiza matendawa.

Wopezayo angakulimbikitseni izi:

• Zakudya zomwe zimachepetsa mchere, madzi, ndi potaziyamu
• Mankhwala oletsa kuthamanga kwa magazi

Matenda a impso amayang'aniridwa ndi:

• Kutenga mankhwala kuti achepetse kuwonongeka kwa impso
• Zakudya zomwe zimachepetsa mchere, madzi, ndi mapuloteni

Kutaya kwakumva kumatha kuyang'aniridwa ndi zothandizira kumva. Mavuto amaso amathandizidwa pakufunika. Mwachitsanzo, mandala osazolowereka chifukwa cha lenticonus kapena ng'ala amatha kusinthidwa.

Upangiri wa chibadwa ungalimbikitsidwe chifukwa matendawa adachokera.

Izi zimapereka chidziwitso chambiri pa matenda a Alport:

• Alport Syndrome Foundation - www.alportsyndrome.org/about-alport-syndrome
• National Impso Foundation - www.kidney.org/atoz/content/alport
• National Organisation for Rare Disways - rarediseases.org/rare-diseases/alport-syndrome

Amayi nthawi zambiri amakhala ndi moyo wathanzi popanda zisonyezo za matendawa kupatula magazi mkodzo. Nthawi zambiri, azimayi amakhala ndi kuthamanga kwa magazi, kutupa, komanso kugontha kwamitsempha ngati vuto la mimba.

Amuna, ogontha, osawona, komanso matenda am'mapeto kumapeto kwake ali ndi zaka 50.

Pamene impso zikulephera, dialysis kapena kumuika kudzafunika.

Itanani kuti mudzakumane ndi wokuthandizani ngati:

• Muli ndi zizindikiro za matenda a Alport
• Muli ndi mbiri ya banja la matenda a Alport ndipo mukukonzekera kukhala ndi ana
• Kutulutsa kwanu kwamkodzo kumachepa kapena kuyima kapena mukawona magazi mumkodzo wanu (ichi mwina ndi chizindikiro cha matenda amisempha)

Kudziwitsa za zoopsa, monga mbiri ya banja la vutoli, kumatha kulola kuti vutoli lipezeke msanga.

Cholowa nephritis; Hematuria - nephropathy - kugontha; Hemorrhagic banja nephritis; Chibadwa chakumva ndi nephropathy

• Matenda a impso

Gregory MC. Matenda a Alport ndi zovuta zina. Mu: Gilbert SJ, Weiner DE, olemba., Eds. Primer pa Matenda a Impso a National Kidney Foundation. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 42.

Radhakrishnan J, Appel GB, D'Agati VD. Matenda achiwiri a glomerular. Mu: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, olemba. Brenner ndi Rector a Impso. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 32.

Rheault MN, Kashtan CE. Matenda a Alport ndi ma syndromes ena am'banja. Mu: Feehally J, Floege J, Tonelli M, Johnson RJ, olemba. Chachikulu Chachipatala Nephrology. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2019: mutu 46.