Zellweger ciwo ne mai wuya, gadon maye gurbi wanda ke rinjayar peroxisomes, kwayoyin da aka samu a kusan dukkanin kwayoyin jiki. Peroxisomes suna da alhakin ƙwayoyin salula masu yawa, ciki har da tsarin makamashi, wanda ke nufin cewa ciwo na Zellweger zai iya tasiri jiki sosai. Koyi game da yadda Zellweger ciwo yana rinjayar jiki, da magani da kuma tsarin shawarwari na kwayoyin halitta.
Zellweger Spectrum Disorders
Zellweger ciwo shi ne mafi tsanani daga rukuni na cuta da ake kira Zellweger bakan cuta. Duk da yake yanayin da ake yi a kan bakan da aka yi la'akari da zama ƙungiyoyi daban-daban, ba a ƙayyade su ba a matsayin bambancin daban-daban na tsari guda daya. Zellweger nau'i-nau'in bidiyon sun hada da:
- Cerebrohepatorenal Syndrome
- Hyperpipecolic acidmia
- Rawancin Refsum na Raba
- Neonatal Adrenoleukodystrophy
- Zellweger Syndrome
Kwayoyin cuta suna rarraba alamun alamun da yawa, amma ba dukkanin mutane zasuyi daidai da alamun sunadarai ko sakamakon sakamako ba dangane da inda suke fada akan bakan.
Cutar cututtuka
An kiyasta ciwo na Zellweger a cikin 1 daga kowace 50,000 zuwa 100,000 haihuwa. Dukkan maza da mata za a iya haife su tare da wannan yanayin. Yana rinjayar da yawa sassa na jiki, ciki har da:
- Head da fuska: girma girma; babban goshi; babban tsohuwar launi fontanelle ("launi mai laushi"); ƙananan kunne; fuskar fuska
- Ƙwayar cuta da kuma juyayi: rashin ci gaba da kwakwalwar kwakwalwa da ke haifar da tashe-tashen hankali ; jin da hankali da hangen nesa; raunin hankali mai tsanani da jinkirin jinkiri; ragewa ko kuskuren da ba ya nan
- Harkar: kara girman hanta tare da aiki mara kyau; jaundice
- Kodan: ƙananan kaya; hydronephrosis
- Waƙoƙi da ƙashi: ƙananan ƙwayar tsoka (hypotonia); kuskuren kasuwa a hannun, kafafu, da ƙafa
Sanin asali
Halin siffar kai da fuskar fuskar jaririn da aka haife shi tare da ciwon Zellweger yana ba da alamar ganewar asali. Zellweger ciwo yana haifar da gina tsararren sarkar mai karfi (VLCFA), saboda haka gwaji ga VLCFA zai taimaka wajen tabbatar da ganewar asali. Wannan kuma da sauran gwaje-gwajen biochemical da gwaje-gwaje na musamman za a iya yi a wasu wuraren gwaji.
Jiyya
Duk da binciken da aka samu a cigaba da fahimtar ƙwayar cutar Zellweger, babu magani ko wanzu, kuma jariran da aka haife su tare da cutar sukan mutu a cikin shekarar farko ta rayuwa. Gidajen kulawa na mayar da hankali kan magance bayyanar cututtuka da ke akwai, irin su ciwon hanta da haɗari. Canja yawan adadin VLCFA a cikin abinci ba a nuna su zama magani mai mahimmanci ba.
Bugu da ƙari, ta jiki, sana'a, da kuma maganganun maganganu na iya taimakawa wajen ciyarwa da ta'aziyya.
Nazarin Halitta
Sakamakon farko na ciwo na Zellweger da kuma sauran Zellweger cuta na bakan ya yiwu ta hanyar gwajin kwayoyin. Zellweger ciwon daji ya gaji a cikin wani motsa jiki ta hanzari, ma'anar cewa yayinda yara ke bunkasa idan iyaye biyu suna ɗaukar nauyin ɓarna. Idan wannan shine lamarin, kowane yaro na gaba yana da damar kashi 25 cikin dari na haihuwa da Zellweger syndrome.
Masana kimiyya zasu iya taimaka maka magana ta hanyar hadari.
Sources:
Chedrawi, A, & Clark, G. (2002). Hanyoyin maganin fata. eMedicine.
Grayer, J. (2005). Amfani da ciwo na Zellweger a lokacin haihuwa. Adv Neonatal Care, 5 (1), 5-13.