Matenda a Crigler-Najjar

Matenda a Crigler-Najjar ndimatenda achilendo omwe bilirubin sangathe kuwonongeka. Bilirubin ndi chinthu chopangidwa ndi chiwindi.

Enzyme imasintha bilirubin kukhala mawonekedwe omwe amatha kuchotsedwa mthupi mosavuta. Matenda a Crigler-Najjar amapezeka ngati enzyme iyi sigwira ntchito bwino. Popanda enzyme iyi, bilirubin imatha kukula mthupi ndikupangitsa kuti:

• Jaundice (khungu lamaso ndi khungu)
• Kuwonongeka kwa ubongo, minofu, ndi mitsempha

Mtundu I Crigler-Najjar ndiye mtundu wa matenda omwe amayamba adakali aang'ono. Matenda amtundu wa II Crigler-Najjar amatha kuyamba m'moyo.

Matendawa amakhala m'mabanja (obadwa nawo). Mwana ayenera kulandira mtundu wa cholakwika kuchokera kwa makolo onse kuti apange mawonekedwe ovutawo. Makolo omwe amanyamula (ali ndi jini imodzi yokha yolakwika) amakhala ndi theka la michere ya achikulire, koma ALIBE zizindikiro.

Zizindikiro zimaphatikizapo:

• Kusokonezeka komanso kusintha kwa malingaliro
• Khungu lachikaso (jaundice) ndi chikasu mwa azungu azungu (icterus), omwe amayamba masiku angapo pambuyo pobadwa ndikuwonjezeka pakapita nthawi
• Kukonda
• Kudya moperewera
• Kusanza

Kuyesedwa kwa chiwindi kumagwira ntchito:

• Conjugated (womangidwa) bilirubin
• Mulingo wonse wa bilirubin
• Bilcubin yosakanizidwa (yopanda malire) m'magazi.
• Kuyesa mavitamini
• Chiwindi

Chithandizo chopepuka (phototherapy) chimafunikira pamoyo wamunthu. Kwa makanda, izi zimachitika pogwiritsa ntchito magetsi a bilirubin (bili kapena 'buluu' magetsi). Phototherapy imagwiranso ntchito usanakwanitse zaka 4, chifukwa khungu lolimba limaletsa kuwalako.

Kuika chiwindi kumatha kuchitidwa mwa anthu ena omwe ali ndi matenda amtundu wa I.

Kuikidwa magazi kumatha kuthandizira kuwongolera kuchuluka kwa bilirubin m'magazi. Mankhwala a calcium nthawi zina amagwiritsidwa ntchito pochotsa bilirubin m'matumbo.

Mankhwala a phenobarbitol nthawi zina amagwiritsidwa ntchito pochiza mtundu wachiwiri wa Crigler-Najjar.

Mitundu yocheperako yamatenda (mtundu wachiwiri) siyimayambitsa kuwonongeka kwa chiwindi kapena kusintha kwa malingaliro paubwana. Anthu omwe amakhudzidwa ndi mawonekedwe ofatsa amakhalabe ndi jaundice, koma amakhala ndi zizindikilo zochepa komanso kuwonongeka kwa ziwalo zochepa.

Makanda omwe ali ndi matendawa (mtundu wa I) amatha kupitiliza kukhala ndi jaundice mpaka atakula, ndipo angafunike chithandizo chatsiku ndi tsiku. Ngati sanalandire chithandizo, matendawa amapha ali ana.

Anthu omwe ali ndi vutoli omwe amakula msinkhu amatha kudwala ubongo chifukwa cha jaundice (kernicterus), ngakhale atalandira chithandizo chamankhwala. Kutalika kwa moyo wa matenda amtundu wa I ndi zaka 30.

Mavuto omwe angakhalepo ndi awa:

• Mtundu wakuwonongeka kwaubongo komwe kumayambitsidwa ndi jaundice (kernicterus)
• Khungu lachikaso / maso achikaso

Funsani upangiri wamtundu ngati mukukonzekera kukhala ndi ana ndikukhala ndi mbiri yabanja ya Crigler-Najjar.

Itanani yemwe akukuthandizani ngati inu kapena mwana wanu wakhanda ali ndi matenda a jaundice omwe samatha.

Upangiri wa chibadwa umalimbikitsidwa kwa anthu omwe ali ndi mbiri yabanja ya matenda a Crigler-Najjar omwe akufuna kukhala ndi ana. Kuyezetsa magazi kumatha kuzindikira anthu omwe ali ndi mitundu yosiyanasiyana.

Kulephera kwa Glucuronyl transferase (mtundu wa I Crigler-Najjar); Matenda a Arias (mtundu wachiwiri Crigler-Najjar)

• Matenda a chiwindi

Kaplan M, Wong RJ, Burgis JC, Sibley E, Stevenson DK. Matenda a neonatal jaundice ndi matenda a chiwindi. Mu: Martin RJ, Fanaroff AA, Walsh MC, olemba., Eds. Fanaroff ndi Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 91.

Lidofsky Sd. Jaundice. Mu: Feldman M, Friedman LS, Brandt LJ, olemba. Sleisenger ndi Matenda a Mimba ndi a Fordtran Amatenda a Chiwindi. 11th ed. Philadelphia, PA: Elsevier; 2021: mutu 21.

Peters AL, Balistreri WF. Matenda amadzimadzi pachiwindi. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 384.