Marfan ciwo da ciki: abin da kuke bukatar ku sani

Marfan ciwo a cututtukan da ba kasafai ba, tare da rinjayen watsawa na autosomal, wanda ke shafar mata da maza. Wannan nau'in watsa kwayoyin halitta yana nufin cewa, "lokacin da aka shafi iyaye, haɗarin kowane yaro da abin ya shafa shine 1 cikin 2 (50%), ba tare da la'akari da jinsi ba", Yayi bayanin Dr Sophie Dupuis Girod, wanda ke aiki a Cibiyar Kula da Cututtuka ta Marfan da Rare Vascular Disease Competence Center, a cikin CHU de Lyon. An kiyasta cewa daya daga cikin mutane 5 ya shafa.

"Cuta ce da ake kira connective tissue, wato goyon bayan tissues, tare da nakasu wanda zai iya shafar kyallen jikin da dama da gabobin jiki da dama.”, in ji Dr Dupuis Girod. Yana rinjayar kyallen da ke tallafawa jiki, waɗanda ke musamman a ciki fata, da manyan arteries, ciki har da aorta, wanda zai iya karuwa a diamita. Hakanan zai iya rinjayar zaruruwan da ke riƙe da ruwan tabarau, da haifar da tarwatsewar ruwan tabarau.

Mutanen da ke fama da ciwon Marfan ba koyaushe ne ake gane su ba, kodayake an gano cewa galibi ana samun su dogo, tare da dogayen yatsu kuma sai fata. Suna iya nuna babban sassauci, ligament da hyperlaxity na haɗin gwiwa, ko ma alamun shimfiɗa.

Duk da haka, akwai masu ɗauke da maye gurbi waɗanda ke da alamun kaɗan, da wasu waɗanda ke nuna alamun da yawa, wani lokaci a cikin iyali ɗaya. Ana iya isa ga mutum tare da tsananin muguwar canji.

Za mu iya la'akari da ciki tare da Marfan ciwo?

"Muhimmin abin da ke tattare da cutar Marfan shi ne fashewar aorta: lokacin da aorta ya yi nisa sosai, kamar balloon da aka hura da yawa, akwai haɗarin cewa bangon zai yi sirara sosai. da karyawa”, in ji Dr Dupuis-Girod.

Saboda karuwar jini da sauye-sauyen hormonal da yake haifarwa, ciki lokaci ne mai haɗari ga duk matan da abin ya shafa. Domin waɗannan canje-canje na iya kasancewa tare da suƘara haɗarin dilation na aorta ko ma rarraba aorta a cikin uwa mai ciki.

Lokacin da diamita na aortic ya fi 45 mm, an hana ciki ciki saboda haɗarin mutuwa daga ruptured aorta yana da yawa, in ji Dokta Dupuis-Girod. Sannan ana ba da shawarar tiyata aortic kafin yiwuwar ciki.

A ƙasa da 40 mm a diamita na aortic, an yarda da ciki, yayin datsakanin 40 zuwa 45 mm a diamita, dole ne ku yi hankali sosai.

A cikin shawarwarin da suka bayar na kula da ciki a cikin mace mai fama da ciwon Marfan, Hukumar Kula da Magungunan Halittu (Biomedicine Agency) da Kwalejin Kwalejin Gynecologists da Obstetricians ta Faransa (CNGOF) sun bayyana cewa. hadarin aortic dissection akwai"komai diamita na aortic"Amma wannan hadarin"Ana la'akari da ƙananan lokacin da diamita ya kasance ƙasa da 40mm, amma ana la'akari da babba a sama, musamman sama da 45mm".

Takardar ta ƙayyade cewa ciki yana contraindicated idan mai haƙuri:

• An gabatar da shi tare da ɓarna aortic;
• Yana da bawul ɗin inji;
• Yana da diamita na aortic fiye da 45 mm. Tsakanin 40 da 45 mm, za a yanke shawara a kan yanayin da ya dace.

Yaya ciki ke tafiya lokacin da kake da ciwon Marfan?

Idan mahaifiyar ita ce mai ɗaukar Marfan ciwo, an yi amfani da duban dan tayi ta hanyar likitan zuciya wanda ya saba da ciwon a ƙarshen farkon farkon trimester, a karshen na biyu trimester, da kowane wata a cikin uku trimester, da kuma game da wata daya bayan haihuwa.

Dole ne ciki ya ci gaba a kan beta-blocker far, a cikin cikakken kashi idan zai yiwu (bisoprolol 10 MG alal misali), tare da shawarwari tare da likitan mahaifa, ya lura da CNGOF a cikin shawarwarinsa. Wannan maganin beta-blocker, wanda aka wajabta don kare aorta, kada a daina, ciki har da lokacin haihuwa. Shayarwa ba zai yiwu ba saboda wucewar beta blocker a cikin madara.

Ya kamata a lura cewa magani tare da mai hana enzyme mai canzawa (ACE) ko sartans an hana shi yayin daukar ciki.

Idan ma'auratan kawai abin ya shafa, za a bi ciki kamar yadda aka saba.

Menene haɗari da rikitarwa na ciwon Marfan yayin daukar ciki?

Babban haɗari ga mahaifiyar mai zuwa shine ta sami a rarrabawar aortic, da kuma yin tiyatar gaggawa. Ga tayin, idan mahaifiyar da za ta kasance tana da irin wannan matsala mai tsanani, akwai kasadar ciwon tayi ko mutuwa. Idan duban duban dan tayi ya nuna babban hadarin wargajewar aortic ko tsagewa, yana iya zama dole a yi sashin cesarean kuma a haifi jaririn da wuri.

Marfan ciwo da ciki: menene hadarin da yaron ya shafa?

"Lokacin da aka shafi iyaye, haɗarin kowane yaro da za a shafa (ko aƙalla mai ɗaukar maye gurbin) shine 1 cikin 2 (50%), ba tare da la'akari da jima'i ba.”, in ji Dr Sophie Dupuis Girod.

Halin maye gurbi da ke da alaƙa da cutar Marfan shine ba lallai ba ne iyaye su yada shi, Hakanan yana iya fitowa a lokacin haihuwa, a cikin yaron da babu ɗayan iyayen da ba ya ɗaukarsa.

Shin za a iya yin ganewar asali don gano ciwon Marfan a cikin mahaifa?

Idan an san maye gurbi kuma an gano shi a cikin iyali, yana yiwuwa a yi bincike na prenatal (PND), don sanin ko tayin ya shafa, ko ma da ganewar asali (PGD) bayan in vitro hadi (IVF).

Idan iyaye ba sa son ɗaukar ciki har zuwa lokacin da yaron ya shafa, kuma suna son samun damar zuwa ƙarshen ciki (IMG) a cikin wannan yanayin, ana iya gudanar da ganewar asali na haihuwa. Amma wannan DPN ana bayar da ita ne kawai bisa bukatar ma'auratan.

Idan ma'auratan suna la'akari da IMG idan yaron da ba a haifa ba yana da ciwon Marfan, za a bincika fayil ɗin su a cikin Cibiyar Bincike na Prenatal Diagnostic (CDPN), wanda zai buƙaci amincewa. Duk da yake nasan cewaba zai yiwu a san yawan lalacewar da jaririn da ke ciki zai kasance ba, kawai idan shi mai ɗaukar hoto ne ko ba na maye gurbin kwayoyin halitta ba.

Shin za a iya yin ganewar asali kafin a dasa don hana kamuwa da tayin?

Idan daya daga cikin ma'auratan biyu ya kasance mai dauke da kwayoyin maye gurbi da ke da alaka da cutar Marfan, za a iya samun hanyar da za a tabbatar da ganewar asali, domin a dasa amfrayo a mahaifar da ba zai zama mai dauke da shi ba.

Duk da haka, wannan yana nufin samun komawa zuwa hadi na in vitro don haka zuwa tsarin haihuwa na taimakon likita (MAP), hanya mai tsawo kuma mai nauyi ga ma'aurata.

Ciki da Marfan ciwo: yadda za a zabi haihuwa?

Ciki tare da ciwon Marfan yana buƙatar bibiya a asibitin haihuwa inda ma'aikata ke da kwarewa wajen kula da mata masu ciki masu wannan ciwo. Akwai duka jerin sunayen masu haihuwa, wanda aka buga akan gidan yanar gizon marfan.fr.

"A cikin shawarwari na yanzu, dole ne a sami cibiyar da ke da sashin tiyata na zuciya a wurin idan diamita na aortic a farkon ciki ya fi 40 mm.”, Dokta Dupuis-Girod ya bayyana.

Lura cewa wannan ƙayyadaddun ba shi da alaƙa da nau'in haihuwa (I, II ko III), wanda ba shine ma'auni na zabar haihuwa a nan ba. A hakikanin gaskiya, Ma'aurata don Marfan syndrome Gabaɗaya suna cikin manyan birane, don haka matakin II ko ma III.

Ciki da ciwon Marfan: shin za mu iya samun epidural?

"Wajibi ne a gargadi masu maganin sa barci da yiwuwar su shiga tsakani, domin za a iya samun ko dai scoliosis ko dural ectasia, wato dilation na jakar (dural) wanda ke dauke da kashin baya. Kuna iya buƙatar yin MRI ko CT scan don tantance yiwuwar ko rashin samun maganin sa barci”, in ji Dr Dupuis-Girod.

Ciwon ciki da ciwon Marfan: shin dole ne a haihu ko ta hanyar cesarean?

Nau'in bayarwa zai dogara, a tsakanin sauran abubuwa, a kan diamita na aortic kuma ya kamata a sake yin magana akai-akai.

“Idan yanayin zuciya na mahaifa ya tabbata, bai kamata a dauki haihuwa a matsayin ka'ida kafin makonni 37 ba. Ana iya aiwatar da haihuwa a farji idan diamita na aortic ya tabbata, kasa da 40 mm, idan har epidural zai yiwu. Za a iya ba da taimakon korar ta hanyar tilastawa ko ƙoƙon tsotsa don iyakance ƙoƙarin korar. In ba haka ba za a gudanar da bayarwa ta hanyar cesarean, ko da yaushe kulawa don kauce wa bambancin hawan jini.”, in ji ƙwararren.

Tushen da ƙarin bayani:

• https://www.marfan.fr/signes/maladie/grossesse/
• https://www.agence-biomedecine.fr/IMG/pdf/recommandations-pour-la-prise-en-charge-d-une-grossesse-chez-une-femme-presentant-un-syndrome-de-marfan-ou-apparente.pdf
• https://www.assomarfans.fr