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Geneticdiseasesinartpaintings
Marfansyndrome
Marfansyndromeisanautosomaldominantconnectivetissuedisorder
characterizedbyacombinationofclinicalmanifestationsindifferentorgan
systems.PatientswithMarfansyndromewhoselifetimesareextendedmaybe
encounteredasacuteabdomen(appendicitis)casesapartfromtheobligatory
reasonsandemergenciesarisingnaturallyoutoftheirdisease[12].
FormaldiagnosisofMarfansyndromedemandscharacteristicskeletal,
ophthalmologicalandcardiacabnormalities,oralternativelytwoofthesethree
togetherwithaclearfamilyhistoryofMarfansyndrome.
“AgostaandRashatheFlugelMenschtheSchwazeTube”waspainted
in1929byaBavarianartistChristianSchad.Thetitleofthispainting,which
cancurrentlybeseeninthenewTateModerngalleryinLondon,hasbeen
incorrectlytranslatedasthe“Agostathepigeon-chestedmanandRasha
theblackdove”,whenthedominatingfeatureispectusexcavatumratherthan
pectuscarinatum(Fig.4).Inthepicture,bothAgostaandhiscompanion
arelookingoutatthespectatorwiththesereneexpressionofthoseused
topublicscrutiny.LookingatAgostawecanseedeeppectusexcavatumwith
outwarddeformitiesofthelowerhalvesoftheanteriorribcage.Alongandthin
thoraxandarelativelylongleftarmcanbeobserved.Inaddition,there
isanobviouskyphosisorkyphoscoliosisaffectinghisshouldergirdle.
Thesecondandthirdfingersofhisrighthandareawkwardlypositioned
withhyperextensionofproximalanddistalinterphalangealjointssuggesting
jointlaxity.Hisfaceisstraightwithslant–downeyes[13].
