wilson disease 8 ut body. only neds. aur (O75) ing/ doy 5 fyrta Cuts excreted 9% Bile » Fecal Cu Udine aa wit ikon ditcase . there & genetic defect that tesult ia the excess epee bet. Kepe 7 TEA _Keop O:45 mq + Excess Cu tn the body and deposited fn vacinus Hesse (igh ger pode) YOu Hse - Free copper (Cu)* an Cu". OH + OH ; Free raditle : tfssue domge ser_(hepato cyte Jyrhelp tn get rid of eicess copper s= copper from diet absorbed fn the smal itestine Va enterocyte ond posed aff portal vein to. the liver min Iner ts sent to | ATP#B. "transport pratia® @Binds Cu to __“apocestloplasmin” (casey 4S of copper in blood ) @ Gather Ga tte vesicles for eceeys TY ‘into btle canaliculi? —» Bileom_.. Ga tras porter Cu Excretion wes ver - portal vein Heparscyte + cancaity 6 'mokcules of copper at once | Enterocy te (ofS here fs auakomalitecessive defect in ATP 3B. transporter ; : Le se fr can’t bind Cu with Ceriloplasmin or excrer it_fare bile K, So. the copper bilad up! fn “hepatscyte and srt produce Feee radiels.—» i wl dawg paises causty fr Copper pil cut foto. rnerssvial ppace blood sup other tissue Oke bloodot Frqwcobaal SyMPIM feos wilson etl « brain tt poo, Cu {Sao ne dlisease camplt cation rr sid» basal ganglia $ cause! par Ktnsonfem p cerebral cortex 3 cause! cell death 4 dementia iid descemets membran of cornea 2 at Merron berween. stroma. and exdeheial layer of crex diagnosis 3 (ee Ob. OS ANAS Sym proms. 8 sLivecdamge: _[ Acute hepatit _» etalnests nl neurological. Symptoms, x. ead} ceruloplasmin Aoceruleplamin t uasable dower las ven4 lang in Slouna free Cat Otter conplcarion s*enlargedtiver_atalspeen * Renal disease Hemolytic anemiaTreatments BD Acute de-coppecing thempy, BB rraintaince therapy. Keep Cu fn normal range = o¢ aminoacid metabolite of gencilin = No anti biote prepares. + otis tn SS wf Terra thiomalypdate AZinc salts. sais at protinsin__intestinal cell canboid to Cul) } pind with Free copper fn Sbload and tissue excretion inucine mepllorbionein teres treaieal cets Op excreted in feces Biver vansplant