Crigler-Najjar ciwo: menene menene, manyan nau'ikan da magani

Wadatacce

• Babban iri da bayyanar cututtuka
• Crigler-Najjar ciwo irin na 1
• Crigler-Najjar ciwo iri na 2
• Yadda za a tabbatar da ganewar asali
• Yadda ake yin maganin

Ciwon Crigler-Najjar cuta ce ta kwayar cutar hanta da ke haifar da tarin bilirubin a cikin jiki, saboda canje-canje a cikin enzyme wanda ke canza wannan abu don kawar da shi ta hanyar bile.

Wannan canjin na iya samun digiri daban-daban da kuma alamun bayyanar, saboda haka, cutar na iya zama nau'in 1, mafi tsanani, ko kuma rubuta 2, yana da sauƙi da sauƙin magani.

Don haka, bilirubin da ba za a iya kawar da shi ba kuma ya taru a cikin jiki yana haifar da jaundice, yana haifar da fata da idanu masu launin rawaya, da haɗarin lalacewar hanta ko buguwa ta kwakwalwa.

Babban iri da bayyanar cututtuka

Za a iya rarraba cututtukan Crigler-Najjar zuwa nau'ikan 2, waɗanda aka banbanta da matakin rashin aiki na enzyme na hanta wanda ke canza bilirubin, wanda ake kira glucoronyl transferase, da kuma ta alamomin da magani.

Crigler-Najjar ciwo irin na 1

Wannan shine nau'in mafi tsananin, saboda babu cikakken aikin hanta don canzawar bilirubin, wanda aka tara shi da yawa a cikin jini kuma yana haifar da alamomin koda a lokacin haihuwa.

• Kwayar cututtuka: jaundice mai tsanani tun daga haihuwa, kasancewarta daya daga cikin abubuwan da ke haifar da hyperbilirubinemia na jariri, kuma akwai hatsarin lalacewar hanta da gubar kwakwalwa da ake kira kernicterus, inda a ciki akwai rashin nutsuwa, bacci, tashin hankali, rashin lafiya da kuma barazanar mutuwa.

Ara koyo game da abin da ke haifar da shi da kuma yadda za a warkar da nau'ikan hyperbilirubinemia na jariri.

Crigler-Najjar ciwo iri na 2

A wannan yanayin, enzyme da ke canza bilirubin yana da rauni ƙwarai, duk da cewa har yanzu yana nan, kuma duk da cewa shima mai tsanani ne, jaundice ba shi da ƙarfi, kuma akwai alamun alamun da ke faruwa da rikice-rikice kaɗan fiye da cutar ta type 1. kwakwalwa kuma karami ce, wanda zai iya faruwa aukuwa na daukaka bilirubin.

• Kwayar cututtuka: jaundice na bambancin ƙarfi, wanda na iya zama mai laushi zuwa mai tsanani, kuma yana iya bayyana a wasu shekarun cikin rayuwa. Hakanan za'a iya haifar dashi bayan wasu damuwa a jiki, kamar kamuwa da cuta ko rashin ruwa a jiki, misali.

Duk da kasada ga lafiyar yaro da rayuwar da nau'ikan wannan ciwo ke haifarwa, yana yiwuwa a rage lamba da tsananin bayyanuwar tare da magani, tare da maganin foterapi, ko ma dashen hanta.

Yadda za a tabbatar da ganewar asali

Ganewar Crigler-Najjar ciwo ne wanda likitan yara, gastro ko hepatologist suka yi, dangane da gwajin jiki da gwajin jini, wanda ke nuna karuwar matakan bilirubin, ban da kimanta aikin hanta, tare da AST, ALT da albumin, don misali.

An tabbatar da cutar ta hanyar gwajin DNA ko ma ta hanyar biopsy na hanta, wanda ke iya banbanta nau'in ciwo.

Yadda ake yin maganin

Babban magani don rage yawan bilirubin a jiki, a cikin Crigler-Najjar ciwo irin na 1, shine maganin fototherapy tare da shuɗi mai haske a ƙalla awanni 12 a rana, wanda zai iya bambanta dangane da bukatun kowane mutum.

Phototherapy yana da inganci saboda yana lalacewa yana canza bilirubin ta yadda zai iya kaiwa bile kuma jiki ya cire shi. Wannan maganin zai iya kasancewa tare da ƙarin jini ko amfani da ƙwayoyin bilirubin na yin tawaye, kamar su cholestyramine da calcium phosphate, don inganta tasirinsa, a wasu lokuta. Ara koyo game da alamomi da kuma yadda phototherapy ke aiki.

Duk da wannan, yayin da yaron ya girma, jiki yakan zama mai jurewa magani, yayin da fatar ke ƙara zama mai tsayayya, yana buƙatar ƙarin awoyi na daukar hoto.

Don maganin Crigler-Najjar ciwo irin na 2, ana yin phototherapy a kwanakin farko na rayuwa ko, a wasu shekarun, kawai a matsayin ƙarin tsari, saboda irin wannan cutar tana da kyakkyawar amsa ga magani tare da maganin Fenobarbital, wanda zai iya kara aikin hanta enzyme wanda ke kawar da bilirubin ta bile.

Koyaya, tabbataccen magani ga kowane nau'in cututtukan yana samuwa ne kawai tare da dasa hanta, wanda ya zama dole a sami mai ba da gudummawa mai dacewa kuma yana da yanayin jiki don tiyatar. San lokacin da aka nuna shi kuma yaya ake murmurewa daga dasawar hanta.