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Wilson’s disease – how do i manage dr. ashish bavdekar
- 1. Wilson’s Disease – How do I manage ? Dr. Ashish Bavdekar Associate Professor Consultant Ped. Gastroenterologist K.E.M. Hospital, Pune bavdekar@vsnl.com
- 2. Zn + penicillamine Zn + trientine Zn sulfate Zn acetate trientine penicillamine transplanted EuroWilson: initial treatment Why? “Available in our country Cheap Tried and tested What we’ve always used “Not available in our country Kept as second line Not as effective? “expensive”
- 3. Wilson’s disease – management - Low copper diet - Serving size (< 0.1mg, 0.1-0.2, > 0.2mg)
- 5. Wilson’s disease – management - Low copper diet - Serving size (< 0.1mg, 0.1-0.2, > 0.2mg) - Medications - D-Penicillamine - Trientine - Zinc - Ammonium molybdate
- 6. Wilson’s Disease - therapy 1) Reduce Cu to sub-toxic threshold - takes 6-12 months - DP, Trientine 2) Maintain slightly negative Cu balance - life long therapy - DP, Trientine, Zn
- 7. DP Trientine Zinc Chelator Chelator Induces MT Easy availability Patient named basis Easy availability Reasonable cost V Expensive Cheap Side effects Neurological Minimal SE Gastric discomfort
- 8. Treatment: Hepatic cases acute liver failure with encephalopathy acute liver failure without encephalopathy intermediate severity Asymptomatic transaminitis Asymptomatic and normal LFTs Neonate detected by screening List for Tx Trientine + zinc ‘bridge’ Modified Kings score Tx if >11 Trientine + zinc
- 9. Score Bilirubin mol/Lɥ INR AST IU/L WCC x 109 /L Albumin g/L 0 0-100 0-1.29 0-100 0-6.7 >45 1 101-150 1.3-1.6 101-150 6.8-8.3 34-44 2 151-200 1.7-1.9 151-300 8.4-10.3 25-33 3 201-300 2.0-2.4 301-400 10.4-15.3 21-24 4 >301 >2.5 >401 >15.4 <20 Modified King’s score A score > 11 = urgent need for transplantation Validated in other centres; better than PELD
- 10. Treatment: Hepatic cases acute liver failure with encephalopathy acute liver failure without encephalopathy intermediate severity Asymptomatic transaminitis Asymptomatic and normal LFTs Neonate detected by screening List for Tx Trientine + zinc ‘bridge’ Modified Kings score Tx if >11 Trientine + zinc Zinc Zinc – when to start?
- 11. Treatment: Hepatic cases acute liver failure with encephalopathy acute liver failure without encephalopathy intermediate severity Asymptomatic transaminitis Asymptomatic and normal LFTs Neonate detected by screening List for Tx Trientine + zinc ‘bridge’ Modified Kings score Tx if >11 Trientine + zinc Zinc Zinc – when to start? Trials needed
- 12. DP Trientine Zinc Chelator Chelator Induces MT Easy availability Patient named basis Easy availability Reasonable cost V Expensive Cheap Side effects Neurological Minimal SE Gastric discomfort Initial therapy Initial therapy Initial Rx / co-Rx Maintenance Rx All except Severe t-penia DP intolerance Neurological Initial co-Rx Maintenance Rx Presympt. cases
- 13. Treatment of WD in pregnancy • Treatment should not be stopped • DP, Trientine, Zn allowed • Zinc preferable, no dosage change • DP, Trientine reduce dose to 25-50% esp in last trimester
- 14. Monitoring in WD ? • To determine clinical and biochemical improvement/deterioration • Ensure compliance • To identify adverse effects of medications • To review diagnosis if necessary
- 15. Monitoring plan (chelators) • Clinical – Liver status, neuro-psychiatric worsening – KF ring annually • Biochemical (USG) – CBC, LFTs, Urine – 3, 6, 9, 12 days initially – Weekly, biweekly, 1 mo, 3 mo, 6mo • Urinary Cu, Serum free copper – Initially 4 times per year – Later 1-2 times
- 16. DP Trientine Zinc Early Fever, Rash BM suppression, Proteinuria, LNpathy Avoid iron + T Rashes Haem. Gastritis Sideroblastic A Loss of taste Gastritis Leucopenia Increased lipase and amylase Late Nephrotoxicity Lupus like S EPS Loss of taste V Late Myasthenia, Polymyositis
- 18. Zinc DP / Trientine Initial Rx U Cu 100-500 ug/d S free Cu > 25 ug/dL U Cu > 500ug/d S free Cu > 25 ug/dL Good control U Cu < 75ug/d S free Cu < 15 ug/dL U Cu 200-500 ug/d S free Cu < 15ug/dL Non-compliance/ Inadequate dose U Zn < 2mg/d U Cu < 200 ug/d U Cu > 200 ug/d S free Cu > 15ug/dL Over-treatment U Cu < 25 ug/d S. free Cu < 5 ug/dL Anemia, leucopenia Increased ferritin U Cu < 200 ug/d S. free Cu < 5 ug/dL Anemia, leucopenia Increased ferritin Urinary copper in Wilson’s disease
- 19. Zinc DP / Trientine Initial Rx U Cu 100-500 ug/d S free Cu > 25 ug/dL U Cu > 500ug/d S free Cu > 25 ug/dL Good control U Cu < 100ug/d S free Cu < 15 ug/dL U Cu 200-500 ug/d S free Cu < 15ug/dL Non-compliance/ Inadequate dose U Zn < 2mg/d U Cu < 200 ug/d U Cu > 200 ug/d S free Cu > 15ug/dL Over-treatment U Cu < 25 ug/d S. free Cu < 5 ug/dL Anemia, leucopenia Increased ferritin U Cu < 200 ug/d S. free Cu < 5 ug/dL Anemia, leucopenia Increased ferritin Urinary copper in Wilson’s disease
- 20. Summary • Chelators are mainstay of treatment • Diet has adjunct role • Zinc has role in long-term Rx • Monitoring is crucial • Interpretation of UCu important
Editor's Notes
- DP – epty stomach, pyridoxine Trientine not with iron,
- Imovement in sythetic function and clinical imrovement 2-6mo but upto 1 year Deterioration between 1mo to 1 year after discontinuation
- Ucu &lt; 100 after 2 days of stopping DP – good terapeutic effect, &gt;100 non compliance