Lefu la Wilson

Lefu la Wilson ke lefu le futsitsoeng leo ho lona ho nang le koporo e ngata haholo linthong tsa 'mele. Koporo e feteletseng e senya sebete le sistimi ea methapo.

Lefu la Wilson ke lefu le sa tloaelehang le futsitsoeng. Haeba batsoali ka bobeli ba na le liphatsa tsa lefutso tse sa sebetseng bakeng sa lefu la Wilson, ho na le monyetla oa 25% hoimana ka 'ngoe eo ngoana a tla ba le eona.

Lefu la Wilson le etsa hore 'mele o kene le ho boloka koporo e ngata haholo. Koporo e beha sebeteng, bokong, liphio le mahlo. Sena se baka tšenyo ea lisele, lefu la lisele, le lesapo. Litho tse amehileng li emisa ho sebetsa ka mokhoa o tloaelehileng.

Boemo bona bo atile haholo ho batho ba Europe Bochabela, ba Sicilians le ba ka boroa ho Mataliana, empa bo ka etsahala sehlopheng sefe kapa sefe. Lefu la Wilson hangata le hlaha ho batho ba ka tlase ho lilemo tse 40. Ho bana, matšoao a qala ho bonahala ka lilemo tse 4.

Matšoao a ka kenyelletsa:

• Boemo bo sa tloaelehang ba matsoho le maoto
• Ramatiki
• Pherekano kapa pherekano
• Dementia ea kelello
• Ho thata ho tsamaisa matsoho le maoto, ho satalla
• Ho thata ho tsamaea (ataxia)
• Liphetoho maikutlong kapa boitšoarong
• Ho atolosoa ha mpa ka lebaka la ho bokella mokelikeli (ascites)
• Botho boa fetoha
• Phobias, mahlomola (neuroses)
• Ho tsamaea butle
• Ho sisinyeha kapa ho fokotseha ha motsamao le ponahalo ya sefahleho
• Ho senyeha ha puo
• Ho thothomela ha matsoho kapa matsoho
• Motsamao o sa laoleheng
• Motsamao o sa lebelloang le o makatsang
• Ho hlatsa madi
• Bofokoli
• Letlalo le mosehla (jaundice) kapa 'mala o mosehla oa' mala o mosoeu oa leihlo (icterus)

Tlhahlobo ea leihlo la lebone e ka bonts'a:

• Motsamao o lekanyelitsoeng oa mahlo
• Lesale le bosootho kapa le 'mala o sootho ho potoloha iris (masale a Kayser-Fleischer)

Tlhahlobo ea 'mele e ka bontša matšoao a:

• Tšenyo ea methapo ea methapo, ho kenyelletsa tahlehelo ea khokahano, tahlehelo ea taolo ea mesifa, ho thothomela ha mesifa, tahlehelo ea monahano le IQ, tahlehelo ea mohopolo le pherekano (delirium kapa dementia)
• Mathata a sebete kapa a spleen (ho kenyelletsa hepatomegaly le splenomegaly)

Liteko tsa Lab li ka kenyelletsa:

• Palo e felletseng ea mali (CBC)
• Serum ceruloplasmin
• Koporo ea serum
• Serum uric acid
• Koporo ea moroto

Haeba ho na le mathata a sebete, liteko tsa lab li ka fumana:

• Phahameng AST le ALT
• Bilirubin e phahameng
• PT e phahameng le PTT
• Albumin e tlase

Liteko tse ling li ka kenyelletsa:

• Teko ea koporo ea lihora tse 24
• X-ray ea mpeng
• MRI ea mpeng
• CT scan ea mpa
• Hlooho ea CT scan
• Hlooho ea MRI
• Biopsy ea sebete
• Endoscopy e kaholimo

Ho fumanoe liphatsa tsa lefutso tse bakang lefu la Wilson. E bitsoa EA-7B. Tlhahlobo ea DNA ea fumaneha bakeng sa lefutso lena.Bua le mofani oa hao oa tlhokomelo ea bophelo kapa moeletsi oa liphatsa tsa lefutso haeba u ka rata ho etsa tlhahlobo ea liphatsa tsa lefutso.

Morero oa kalafo ke ho fokotsa palo ea koporo ka har'a lisele. Sena se etsoa ka ts'ebetso e bitsoang chelation. Ho fanoa ka meriana e meng e tlamang koporo le ho e thusa ho e ntša ka liphio kapa mala. Kalafo e tlameha ho ba bophelo bohle.

Ho ka sebelisoa meriana e latelang:

• Penicillamine (joalo ka Cuprimine, Depen) e tlama koporo mme e lebisa ho lokolloeng ha koporo ka hara moroto.
• Trientine (joalo ka Syprine) e tlama (chelates) koporo mme e eketsa ho tsoa ha eona ka moroto.
• Zinc acetate (joalo ka Galzin) e thibela koporo hore e se kenelle ka mpeng ea mala.

Lisebelisoa tsa vithamine E le tsona li ka sebelisoa.

Ka linako tse ling, litlhare tse chelate koporo (joalo ka penicillamine) li ka ama tšebetso ea boko le tsamaiso ea methapo (neurological function). Meriana e meng e ntseng e hlahlojoa e ka tlama koporo ntle le ho ama tšebetso ea methapo.

Lijo tse nang le koporo e tlase le tsona li ka buelloa. Lijo tse lokelang ho qojoa li kenyelletsa:

• Tsokolate
• Litholoana tse omisitsoeng
• Sebete
• Li-mushroom
• Linate
• Litlhapi

U kanna ua batla ho noa metsi a silafalitsoeng hobane metsi a mang a pompo a phalla liphaepheng tsa koporo. Qoba ho sebelisa lijana tsa ho pheha ka koporo.

Matšoao a ka laoloa ka boikoetliso kapa kalafo ea 'mele. Batho ba ferekaneng kapa ba sitoang ho itlhokomela ba ka hloka mehato e khethehileng ea ts'ireletso.

Ho kenyelletsa sebete ho ka nahaneloa maemong ao sebete se senyehileng hampe ke lefu lena.

Lihlopha tsa ts'ehetso ea lefu la Wilson li ka fumanoa ho www.wilsonsdisease.org le www.geneticalliance.org.

Kalafo ea bophelo bohle e ea hlokahala ho laola lefu la Wilson. Boloetse bona bo ka baka litlamorao tse bolaeang, joalo ka tahlehelo ea sebete. Koporo e ka ba le litlamorao tse chefo tsamaisong ea methapo. Maemong ao lefu lena le sa bolaeeng, matšoao a ka ba a holofatsa.

Mathata a ka kenyelletsa:

• Phokolo ea mali (hemolytic anemia ha e fumanehe hangata)
• Mathata a tsamaiso ea methapo e bohareng
• Cirrhosis
• Ho shoa ha lisele tsa sebete
• Sebete se mafura
• Lefu la sebete
• Monyetla o eketsehileng oa ho robeha ha masapo
• Palo e eketsehileng ea tšoaetso
• Kotsi e bakoang ke ho oa
• Jaundice
• Likonteraka tse kopanetsoeng kapa bofokoli bo bong
• Ho lahleheloa ke bokhoni ba ho itlhokomela
• Ho lahleheloa ke bokhoni ba ho sebetsa mosebetsing le lapeng
• Ho felloa ke matla a ho sebelisana le batho ba bang
• Ho lahleheloa ke mesifa (mesifa ea matla)
• Mathata a kelello
• Litla-morao tsa penicillamine le meriana e meng e sebelisetsoang ho phekola lefu lena
• Mathata a spleen

Ho hloleha ha sebete le ho senya tsamaiso ea methapo e bohareng (boko, lesapo la mokokotlo) ke litlamorao tse tloaelehileng le tse kotsi tsa bokuli. Haeba lefu lena le sa tšoaroe le ho phekoloa kapele, le ka bolaea.

Bitsa mofani oa hau haeba u na le matšoao a lefu la Wilson. Bitsa moeletsi oa liphatsa tsa lefutso haeba u na le nalane ea lefu la Wilson lelapeng la hau mme o ntse o rera ho ba le bana.

Ho khothaletsoa tlhabollo ea lefutso bakeng sa batho ba nang le nalane ea malapa ea lefu la Wilson.

Lefu la Wilson; Ho senyeha ha hepatolenticular

• Sisteme ea methapo e bohareng le methapo ea methapo
• Teko ea moroto oa koporo
• Sebopeho sa sebete

Setsi sa Naha sa lefu la tsoekere le sebaka sa marang-rang sa Maloetse a Phepo le Liphio. Lefu la Wilson. www.niddk.nih.gov/health-information/liver-disease/wilson-disease. E ntlafalitsoe ka Pherekhong 2018. E fihlile ka la 3 Pulungoana, 2020.

Roberts EA. Lefu la Wilson. Ka: Feldman M, Friedman LS, Brandt LJ, bahlophisi. Sleisenger le Fordtran's Mpeng le Mafu a Sebete. 11th ed. Philadelphia, PA: Elsevier; 2021: khaolo ea 76.

Mokha oa Schilsky. Lefu la Wilson. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 200.