Ciwon Crigler-Najjar

Ciwon Crigler-Najjar cuta ce ta gado da ba a cika samun ta ba wacce ba za a iya farfasa bilirubin ba. Bilirubin wani sinadari ne da hanta ke hadawa.

Wani enzyme yana canza bilirubin zuwa sifar da za a iya cire ta cikin sauki. Crigler-Najjar ciwo yana faruwa lokacin da wannan enzyme ɗin baya aiki daidai. Idan ba tare da wannan enzyme ba, bilirubin na iya ginawa cikin jiki ya kai ga:

• Jaundice (launin rawaya da fata da idanu)
• Lalacewa ga kwakwalwa, tsokoki, da jijiyoyi

Nau'in I Crigler-Najjar shine nau'in cutar da ke farawa da wuri. Nau'in II Crigler-Najjar na iya fara daga baya a rayuwa.

Ciwon yana gudana a cikin dangi (wanda aka gada). Yaro dole ne ya karɓi kwafin kwayar cutar da ta lalace daga iyaye biyu don haɓaka mummunan yanayin yanayin. Iyaye masu ɗauka (tare da ɗayan lalatattun ƙwayoyi) suna da kusan rabin aikin enzyme na baligi na al'ada, amma KADA ku da alamun bayyanar.

Kwayar cutar na iya haɗawa da:

• Rikicewa da canje-canje a cikin tunani
• Fata mai launin rawaya (jaundice) da rawaya a cikin fararen idanuwa (icterus), wanda zai fara bayan fewan kwanaki bayan haihuwa kuma ya zama mafi muni a kan lokaci
• Rashin nutsuwa
• Rashin ciyarwa
• Amai

Gwajin aikin hanta sun hada da:

• Haɗuwa (daure) bilirubin
• Jimlar matakin bilirubin
• Bilirubin da ba a haɗa shi ba (wanda ba shi da iyaka).
• Enzyme yayi gwaji
• Gwajin hanta

Ana buƙatar magani mai sauƙi (phototherapy) a duk rayuwar mutum. A cikin jarirai, ana yin wannan ta amfani da fitilun bilirubin (bili ko ‘blue’ lights). Phototherapy baya aiki sosai bayan shekaru 4, saboda fata mai kauri tana toshe haske.

Ana iya yin dashen hanta a cikin wasu mutanen da ke da cuta irin ta I.

Karin jini na iya taimakawa wajen sarrafa yawan bilirubin a cikin jini. Wasu lokuta ana amfani da sinadarin Calcium don cire bilirubin a cikin hanji.

Ana amfani da magungunan phenobarbitol a wasu lokuta don magance cutar ta II Crigler-Najjar.

Hanyoyin cutar mai laushi (nau'in II) baya haifar da lahani ga hanta ko sauyin tunani yayin yarinta. Mutanen da cutar ta shafa da taushi mara nauyi har yanzu suna da cutar jaundice, amma suna da ƙananan alamun cututtuka da ƙananan lalacewar gabobi.

Yaran da ke dauke da mummunar cutar (nau'ikan I) na iya ci gaba da cutar jaundice har zuwa girma, kuma suna iya buƙatar magani na yau da kullun. Idan ba ayi magani ba, wannan mummunan nau'in cutar zai haifar da mutuwa a yarinta.

Mutanen da ke da wannan yanayin waɗanda suka balaga za su ci gaba da lalacewar ƙwaƙwalwa saboda jaundice (kernicterus), ko da tare da magani na yau da kullun. Tsammani na rayuwa ga nau'in I na shekaru 30.

Matsalolin da ka iya faruwa sun hada da:

• Wani nau'i na lalacewar kwakwalwa ta hanyar jaundice (kernicterus)
• Kullum fata / idanu rawaya

Nemi shawara kan kwayoyin halitta idan kuna shirin samun yara kuma kuna da tarihin iyali na Crigler-Najjar.

Kira mai ba da sabis na kiwon lafiya idan ku ko jaririn da aka haifa yana da cutar jaundice da ba za ta tafi ba.

Ana ba da shawara game da kwayar halitta don mutanen da ke da tarihin iyali na cutar Crigler-Najjar waɗanda ke son samun yara. Gwajin jini na iya gano mutanen da ke ɗauke da bambancin jinsin.

Glucuronyl rashi transferase (rubuta I Crigler-Najjar); Ciwon Arias (nau'in II Crigler-Najjar)

• Hanta jikin mutum

Kaplan M, Wong RJ, Burgis JC, Sibley E, Stevenson DK. Yonatal jaundice da cututtukan hanta. A cikin: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff da Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020: babi 91.

Lidofsky SD. Jaundice. A cikin: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger da Cututtukan Cutar hanta da na Fordtran. 11th ed. Philadelphia, PA: Elsevier; 2021: babi na 21.

Peters AL, Balistreri WF. Cututtuka na rayuwa na hanta. A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 384.