Meckel-Gruber ciwo - wanda aka fi sani da dysencephalia splanchnocystica, ciwon Meckel, da ciwon gurasar Gruber - shine cututtukan kwayoyin cutar da ke haifar da mummunan cututtukan jiki. Saboda wadannan cututtuka masu tsanani, waɗanda ke tare da ciwon Meckel kusan mutuwa kafin ko jimawa bayan haihuwa.
Halittar Halitta
Ƙungiyar Meckel tana hade da maye gurbi a akalla daya daga cikin jinsin takwas.
Hanyoyi a cikin wadannan jinsin guda takwas suna kimanin kusan kashi 75 cikin 100 na lokuta na Meckel-Gruber. Sauran kashi 25 cikin dari suna haifar da maye gurbin da ba'a gano ba.
Domin yaron ya sami ciwo na Meckel-Gruber, dole ne iyaye biyu su ɗauki nau'i na ɓarna mara kyau. Idan iyaye biyu suna dauke da kwayar maras kyau, akwai damar kashi 25 cikin dari da yaro zai sami yanayin. Akwai damar kashi 50 cikin dari na yaro zai sami gado daya daga cikin jinsin. Idan yaro ya gaji ɗaya daga cikin jinsin, za su kasance mai ɗaukar yanayin. Ba za su sami yanayin kansu ba.
Tsarin jima'i
Halin da ke faruwa na nakasar Meckel-Gruber ya bambanta daga 1 a cikin 13,250 zuwa 1 a cikin haihuwa 140,000. Bincike ya gano cewa wasu mutane, kamar wadanda suka fito daga Finnish (1 a cikin mutane 9,000) da kuma zuriya na Belgium (kimanin 1 a cikin mutane 3,000), zasu iya samun rinjaye. Sauran halayen haɓaka da yawa sun samo a cikin 'yan makiyaya a Kuwait (1 cikin 3,500) kuma daga cikin mutanen Indiyawa (1 cikin 1,300).
Wadannan mutane suna da matsayi masu yawa, tare da ko'ina daga 1 a cikin 50 zuwa 1 a cikin mutane 18 da ke ɗauke da kwafin kwayoyin cutar. Duk da yawan wadannan nauyin, yanayin zai iya tasiri ga kowane kabila, da kuma genders.
Cutar cututtuka
An gano cutar ciwo Meckel-Gruber don haifar da lalacewa ta jiki, ciki har da:
- Wani babban launi mai laushi (fontanel), wanda ya ba da damar kwakwalwa da ƙwayar asalin ƙasa don tasowa (encephalocele)
- Zuciyar zuciya
- Babban kodan cike da cysts (ƙwayoyin polycystic)
- Ƙarin yatsunsu ko yatsun kafa ( polydactyly )
- Hanyoyin daji (hanta fibrosis)
- Cikakken ciwon huhu ba shi da cikakke (pulmonary hypoplasia)
- Sulhu mai laushi da shinge
- Abanormalities na al'ada
Kodan karyar sune alamar da ta fi kowa, bin ta polydactyly. Mafi yawan Meckel-Gruber fatalities ne saboda hypoplasia pulmonary, cike da ciwon daji ci gaba.
Sanin asali
Yara da ƙwayar cutar Meckel-Gruber za a iya bincikar su bisa ga bayyanar su a haife su, ko kuma ta hanyar duban dan tayi kafin haihuwa. Yawancin lokutta da aka gano ta hanyar duban dan tayi suna bincikar su a karo na biyu. Duk da haka, mai fasaha mai fasaha zai iya gane yanayin a farkon farkon watanni. Bayanan chromosome, ko dai ta hanyar samfurin vilusic sampling ko amniocentesis, za'a iya aiwatar da shi don yin sarauta daga Trisomy 13 , wanda ya kasance daidai da mummunar yanayin da yake da alamun bayyanar.
Jiyya
Abin takaici, babu magani ga ciwo na Meckel-Gruber saboda ƙwayar da ke cikin ƙananan yara da kuma kodan da ke ciki ba zai iya taimakawa rayuwa ba. Halin yana da kashi 100 cikin 100 na mace-mace a cikin kwanakin haihuwa.
Idan an gano Meckel-Gruber ciwo a lokacin daukar ciki, wasu iyalai zasu iya zaɓar su gama da ciki.
Sources:
Carter, SM (2015). Meckel-Gruber ciwo. eMedicine.
Hanyar NIH na Gidan Gida. Meckel Syndrome (2016)