Beckwith-Wiedemann ciwo

Ciwon Beckwith-Wiedemann cuta ce ta ci gaban da ke haifar da girman jiki, manyan gabobi, da sauran alamomi. Yanayi ne na haihuwa, wanda ke nufin yana nan lokacin haihuwa. Alamu da alamomin cutar sun dan bambanta daga yaro zuwa yaro.

Rashin haihuwa na iya zama lokaci mai mahimmanci ga jarirai masu wannan yanayin saboda yiwuwar:

• Sugararancin sukarin jini
• Wani nau'i na hernia da ake kira omphalocele (lokacin da yake)
• Harshen da aka faɗaɗa (macroglossia)
• Increasedara yawan ci gaban tumo. Ciwon kututtukan Wilms da hepatoblastomas sune ciwace ciwan yara gama gari a cikin yara masu wannan ciwo.

Beckwith-Wiedemann ciwo yana haifar da lahani a cikin kwayoyin halittar chromosome 11. Kusan kashi 10 cikin 100 na cututtukan ana iya yada su ta hanyar dangi.

Alamu da alamomin cutar Beckwith-Wiedemann sun haɗa da:

• Girman girma ga jariri
• Alamar haihuwar ja akan goshi ko fatar ido (nevus flammeus)
• Halitta a cikin kunnen kunne
• Babban harshe (macroglossia)
• Sugararancin sukarin jini
• Launin bangon ciki (hernia ko omphalocele)
• Fadada wasu gabobi
• Cunkushewar gefe ɗaya na jiki (hemihyperplasia / hemihypertrophy)
• Ciwon ƙari, irin su ciwan Wilms da hepatoblastomas

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki don neman alamu da alamomin cutar Beckwith-Wiedemann. Sau da yawa wannan yana isa don yin ganewar asali.

Gwaje-gwajen cutar sun hada da:

• Gwajin jini don ƙaran sukarin jini
• Nazarin chromosomal don rashin daidaituwa a cikin chromosome 11
• Duban dan tayi

Jarirai da ke da ƙananan sikarin jini za a iya kula da su da ruwan da aka bayar ta jijiya (intravenous, IV). Wasu jarirai na iya buƙatar magani ko wani kulawa idan ƙarancin sukarin jini ya ci gaba.

Ana iya gyara lahani a cikin bangon ciki. Idan karin girman harshe yana wahalar numfashi ko ci, ana iya buƙatar tiyata. Yaran da ke da girma a gefe ɗaya na jiki ya kamata a kula da su don karkatar da lanƙwasa (scoliosis). Yaro kuma dole ne a sa masa ido sosai don ci gaban ciwace-ciwace. Tumor binciken ciki har da gwajin jini da kuma ultrasounds na ciki.

Yaran da ke da cutar Beckwith-Wiedemann yawanci suna rayuwa ta yau da kullun. Ana buƙatar ci gaba da nazari don haɓaka bayanan biye na dogon lokaci.

Wadannan rikitarwa na iya faruwa:

• Ci gaban ciwace-ciwace
• Matsalar ciyarwa saboda girman harshe
• Matsalar numfashi saboda girman harshe
• Scoliosis saboda hemihypertrophy

Idan kana da ɗa mai cutar Beckwith-Wiedemann kuma alamun damuwa na ɓullowa, kira likitan yara yanzunnan.

Babu sanannun rigakafin cutar Beckwith-Wiedemann. Bayar da shawara game da kwayar halitta na iya zama da amfani ga iyalai waɗanda suke son su sami yara da yawa.

• Beckwith-Wiedemann ciwo

Devaskar SU, Garg M. Rashin lafiya na carbohydrate metabolism a cikin sabon. A cikin: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff da Martin's Neonatal-Perinatal Medicine. 10 ed. Philadelphia, PA: Elsevier Saunders; 2015: babi na 95.

Madan-Khetarpal S, Arnold G. Rashin lafiyar kwayoyin halitta da yanayin dysmorphic. A cikin: Zitelli, BJ, McIntire SC, Nowalk AJ, eds. Zitelli da Davis 'Atlas na Ciwon Lafiyar Jiki na Yara. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 1.

Sperling MA. Hypoglycemia. 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 111.