Ciwon Marfan

Ciwon Marfan cuta cuta ce ta kayan haɗi. Wannan shine tsokar da ke karfafa sifofin jiki.

Rikitarwa na kayan haɗi suna shafar tsarin ƙashi, tsarin zuciya da jijiyoyin jini, idanu, da fata.

Ciwon Marfan yana faruwa ne sakamakon lahani a cikin kwayar halitta wacce ake kira fibrillin-1. Fibrillin-1 na taka muhimmiyar rawa a matsayin tubalin gini ga kayan haɗin kai a cikin jiki.

Raunin kwayar halitta yana haifar da dogayen kasusuwa na jiki suyi girma sosai. Mutanen da ke fama da wannan ciwo suna da tsayi da tsayi da ƙafa da ƙafa. Ba a fahimci yadda wannan karuwar ta faru ba.

Sauran sassan jikin da abin ya shafa sun hada da:

• Naman huhu (wataƙila akwai cutar pneumothorax, wanda iska zata iya tserewa daga huhun zuwa kogon kirji ya faɗo huhun)
• Aorta, babban jijiyar jini da ke ɗauke da jini daga zuciya zuwa jiki na iya miƙawa ko kuma ya zama mai rauni (wanda ake kira diba aortic ko aortic aneurysm)
• Zuciyar zuciya
• Idanu, masu haifar da cutar ido da sauran matsaloli (kamar ɓarkewar ruwan tabarau)
• Fata
• Naman dake rufe layin kashin baya
• Abubuwan haɗin gwiwa

A mafi yawan lokuta, cutar Marfan tana yaduwa ne ta hanyar dangi (wadanda aka gada). Koyaya, har zuwa 30% na mutane ba su da tarihin iyali, wanda ake kira "mai saurin zuwa". A cikin wasu lokuta, ana jin cewa cutar na faruwa ne ta wani sabon canjin yanayi.

Mutanen da ke da cutar Marfan galibi suna da tsayi tare da dogaye, sirara hannu da ƙafa da yatsu kamar na gizo-gizo (wanda ake kira arachnodactyly). Tsawon hannayen ya fi girma lokacin da aka miƙa hannaye.

Sauran cututtukan sun hada da:

• Kirjin da yake nitsewa ko ya fita waje, ana kiransa kirjin mazurari (pectus excavatum) ko nonon tantabara (pectus carinatum)
• Flat ƙafa
• Hannun baki mai ƙarfi da haƙoran mutane
• Hypotonia
• Abubuwan haɗin gwiwa waɗanda suke da sassauƙa sosai (amma gwiwar hannu na iya zama mai sauƙi)
• Illar karatu
• Motsiwar tabarau na ido daga matsayinta na yau da kullun (rarrabuwa)
• Dubawa
• Lowerananan ƙananan muƙamuƙi (micrognathia)
• Spine wanda ke lankwasawa zuwa gefe ɗaya (scoliosis)
• Siriri, kunkuntar fuska

Mutane da yawa da ke fama da cutar Marfan suna fama da ciwon tsoka da na haɗin gwiwa.

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki. Abubuwan haɗin gwiwa na iya motsawa fiye da yadda aka saba. Hakanan akwai alamun alamun:

• Rashin abinci
• Huhu ya tarwatse
• Matsaloli na bugun zuciya

Gwajin ido na iya nuna:

• Launin ruwan tabarau ko cornea
• Rage ganuwa
• Matsalar hangen nesa

Za a iya yin gwaje-gwaje masu zuwa:

• Echocardiogram
• Gwajin maye gurbin Fibrillin-1 (a cikin wasu mutane)

Ya kamata ayi echocardiogram ko wani gwaji a kowace shekara don a duba gindin aorta kuma da yiwuwan zuciya.

Ya kamata a magance matsalolin hangen nesa idan ya yiwu.

Kulawa don scoliosis, musamman a lokacin samartaka.

Magani don rage bugun zuciya da rage saukar karfin jini na iya taimakawa hana damuwa a cikin aorta. Don kaucewa cutar da aorta, mutanen da ke cikin yanayin na iya canza ayyukansu. Wasu mutane na iya buƙatar tiyata don maye gurbin tushen jijiyoyin jiki da bawul.

Dole ne a kula da mata masu juna biyu masu fama da cutar Marfan sosai saboda tsananin damuwa akan zuciya da aorta.

Gidauniyar Marfan ta Kasa - www.marfan.org

Rikice-rikicen da suka shafi zuciya na iya rage tsawon rayuwar mutanen da ke dauke da wannan cutar. Koyaya, mutane da yawa suna rayuwa zuwa shekaru 60 zuwa sama. Kyakkyawan kulawa da tiyata na iya ƙara tsawanta rayuwa.

Matsaloli na iya haɗawa da:

• Saukewar Aortic
• Rushewar azaba
• Cutar endocarditis
• Rarraba ƙwayoyin cuta
• Ara girman tushe na aorta
• Ajiyar zuciya
• Rushewar bawan mitral
• Scoliosis
• Matsalar hangen nesa

Ma'auratan da ke da wannan yanayin kuma suna shirin haihuwar yara na iya so su yi magana da mai ba da shawara kan kwayar halitta kafin su fara iyali.

Ba za a iya hana sabbin maye gurbi da ke haifar da Marfan (kasa da kashi ɗaya cikin uku na shari'o'in). Idan kana da cutar Marfan, duba mai bayarwa a kalla sau daya a kowace shekara.

Aortic aneurysm - Marfan

• Pectus excavatum
• Ciwon Marfan

Doyle JJ, Doyle AJ, Dietz HC. Ciwon Marfan. 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 722.

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 Ilimin Yara. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 1.

Pyeritz RE. Cututtukan gado na kayan haɗi. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 244.