Matenda a Crigler-Najjar: ndi chiyani, mitundu yayikulu ndi chithandizo

Zamkati

• Mitundu yayikulu ndi zizindikilo
• Mtundu wa Crigler-Najjar mtundu 1
• Mtundu wa Crigler-Najjar mtundu 2
• Momwe mungatsimikizire matendawa
• Momwe mankhwalawa amachitikira

Matenda a Crigler-Najjar ndimatenda amtundu wa chiwindi omwe amachititsa kuti bilirubin ipangidwe mthupi, chifukwa cha kusintha kwa enzyme komwe kumasintha izi kuti zithetsedwe kudzera mu bile.

Kusinthaku kumatha kukhala ndi madigiri osiyanasiyana komanso mawonekedwe a mawonekedwe, chifukwa chake, matendawa amatha kukhala mtundu 1, wowopsa, kapena mtundu wa 2, wopepuka komanso wosavuta kuchiza.

Chifukwa chake, bilirubin yomwe singathetsedwe ndikudziunjikira mthupi imayambitsa jaundice, kuyambitsa khungu ndi maso achikaso, komanso chiwopsezo cha kuwonongeka kwa chiwindi kapena kuledzera kwaubongo.

Mitundu yayikulu ndi zizindikilo

Matenda a Crigler-Najjar amatha kugawidwa m'magulu awiri, omwe amasiyanitsidwa ndi kuchuluka kwa kusagwira ntchito kwa enzyme ya chiwindi yomwe imasintha bilirubin, yotchedwa glucoronyl transferase, komanso ndi zizindikilo ndi chithandizo.

Mtundu wa Crigler-Najjar mtundu 1

Ndiwo mtundu woopsa kwambiri, popeza kulibe chiwindi chilichonse pakusintha kwa bilirubin, yomwe imapezeka m'magazi ochulukirapo ndipo imayambitsa zizindikilo ngakhale pakubadwa.

• Zizindikiro: jaundice yayikulu chibadwire, kukhala chimodzi mwazomwe zimayambitsa hyperbilirubinemia ya wakhanda, ndipo pali chiopsezo chakuwonongeka kwa chiwindi ndi poyizoni wamaubongo wotchedwa kernicterus, momwe mumakhala kusokonezeka, kuwodzera, kusokonezeka, kukomoka komanso kufa.

Dziwani zambiri pazomwe zimayambitsa matendawa komanso momwe mungachiritsire mitundu ya hyperbilirubinemia ya wakhanda.

Mtundu wa Crigler-Najjar mtundu 2

Pankhaniyi, enzyme yomwe imasintha bilirubin ndiyotsika kwambiri, ngakhale idakalipobe, ndipo ngakhale ilinso yoopsa, jaundice siyolimba kwambiri, ndipo pali zizindikilo ndi zovuta zochepa kuposa matenda amtundu wa 1. ubongo ndiwocheperako, womwe ungachitike magawo a bilirubin okwera.

• Zizindikiro: jaundice yamitundu yosiyanasiyana, yomwe imatha kukhala yofatsa mpaka yayikulu, ndipo imatha kuwonekera zaka zina m'moyo wonse. Zitha kuyambitsanso pambuyo povutika m'thupi, monga matenda kapena kuchepa kwa madzi m'thupi, mwachitsanzo.

Ngakhale kuwopsa kwa thanzi la mwana ndi moyo wake chifukwa cha mitundu yamatendawa, ndizotheka kuchepetsa kuchuluka ndi kuuma kwa mawonetseredwe ndi chithandizocho, ndi phototherapy, kapena ngakhale kuziika chiwindi.

Momwe mungatsimikizire matendawa

Kuzindikira kwa matenda a Crigler-Najjar kumapangidwa ndi dokotala wa ana, gastro kapena hepatologist, kutengera kuyezetsa thupi ndi kuyesa magazi, zomwe zikuwonetsa kuchuluka kwa milingo ya bilirubin, kuphatikiza pakuwunika kwa chiwindi, ndi AST, ALT ndi albumin, ya Mwachitsanzo.

Matendawa amatsimikiziridwa ndi kuyesa kwa DNA kapena chiwindi cha chiwindi, chomwe chimatha kusiyanitsa mtundu wa matenda.

Momwe mankhwalawa amachitikira

Chithandizo chachikulu pakuchepetsa ma bilirubin mthupi, mu matenda a Crigler-Najjar mtundu 1, ndi phototherapy yokhala ndi kuwala kwa buluu kwa maola osachepera 12 patsiku, omwe amatha kusiyanasiyana kutengera zosowa za munthu aliyense.

Phototherapy ndiyothandiza chifukwa imaphwanya ndikusintha bilirubin kuti izitha kufikira bile ndikuchotsedwa ndi thupi. Mankhwalawa amathanso kutsatiridwa ndi kuthiridwa magazi kapena kugwiritsa ntchito mankhwala osokoneza bongo a bilirubin, monga cholestyramine ndi calcium phosphate, kuti agwire bwino ntchito, nthawi zina. Dziwani zambiri za zomwe zikuwonetsa komanso momwe phototherapy imagwirira ntchito.

Ngakhale zili choncho, mwana akamakula, thupi limayamba kugonjetsedwa ndi chithandizo, chifukwa khungu limayamba kugonjetsedwa, kumafuna maola ochulukirapo a phototherapy.

Pochiza matenda amtundu wa Crigler-Najjar mtundu wachiwiri, Phototherapy imachitika m'masiku oyamba amoyo kapena, mibadwo ina, kokha ngati njira yothandizirana, chifukwa matenda amtunduwu amathandizidwa ndi mankhwala a Fenobarbital, omwe atha kuonjezera ntchito ya enzyme ya chiwindi yomwe imachotsa bilirubin kudzera mu bile.

Komabe, chithandizo chotsimikizika cha mtundu uliwonse wamatendawa chimatheka kokha ndikumuika chiwindi, momwe ndikofunikira kupeza woperekayo woyenera ndikukhala ndi mikhalidwe yochita opareshoni. Dziwani nthawi yomwe ikuwonetsedwa komanso momwe kuchira kumayambira chiwindi.