emedicine.medscape.com Open in urlscan Pro
172.64.150.155 Public Scan

Back to summary

URL:
https://emedicine.medscape.com/article/183456-medication
Submission: On March 25 via manual (March 25th 2024, 7:56:17 pm UTC) from US — Scanned from US

Form analysis
3 forms found in the DOM

Name: SearchFormHeader — GET javascript:subsearchheadertrack('en');

<form name="SearchFormHeader" class="search-form-header" method="get" aria-label="Search" action="javascript:subsearchheadertrack('en');">
  <input type="hidden" value="news" name="searchSrc">
  <input id="search-input" class="search-input search-input-text" aria-label="Search" type="text" autocomplete="off" autocorrect="off" name="q" maxlength="500" placeholder="Search">
  <button type="submit" class="search-submit-button" aria-label="Search" title="search">
    <svg xmlns="https://www.w3.org/2000/svg" viewBox="0 0 21 21">
      <path fill="#005B80" fill-rule="evenodd"
        d="M12.018 12.468c-2.155 2.156-5.63 2.156-7.784 0-2.155-2.154-2.155-5.63 0-7.784 2.154-2.155 5.63-2.155 7.784 0 2.155 2.155 2.133 5.63 0 7.784M2.54 2.99c-3.078 3.08-3.078 8.07 0 11.15 2.727 2.727 6.927 3.035 10.006.946l5.168 5.167c.572.572 1.517.572 2.09 0 .57-.572.57-1.517 0-2.09l-5.17-5.166c2.09-3.08 1.782-7.28-.945-10.006-3.08-3.077-8.07-3.077-11.15 0">
      </path>
    </svg></button>
  <button type="button" class="search-open-button js-expand-button" data-section=".header-search" aria-label="Search">
    <svg xmlns="https://www.w3.org/2000/svg" viewBox="0 0 21 21">
      <path fill="#005B80" fill-rule="evenodd"
        d="M12.018 12.468c-2.155 2.156-5.63 2.156-7.784 0-2.155-2.154-2.155-5.63 0-7.784 2.154-2.155 5.63-2.155 7.784 0 2.155 2.155 2.133 5.63 0 7.784M2.54 2.99c-3.078 3.08-3.078 8.07 0 11.15 2.727 2.727 6.927 3.035 10.006.946l5.168 5.167c.572.572 1.517.572 2.09 0 .57-.572.57-1.517 0-2.09l-5.17-5.166c2.09-3.08 1.782-7.28-.945-10.006-3.08-3.077-8.07-3.077-11.15 0">
      </path>
    </svg></button>
  <div class="ilfulllist">
    <p class="il_combo_nor">No Results</p>
    <ul class="combolist"></ul>
  </div>
</form>

Name: SearchFormHeader — GET javascript:subsearchheadertrack('en');

<form name="SearchFormHeader" class="search-form-header" method="get" aria-label="Search" action="javascript:subsearchheadertrack('en');">
  <input type="hidden" value="news" name="searchSrc">
  <input id="layer-search-input" class="search-input search-input-text" aria-label="Search" type="text" autocomplete="off" autocorrect="off" name="q" maxlength="500" placeholder="Search">
  <button type="submit" class="search-submit-button" aria-label="Search" title="search">
    <svg xmlns="https://www.w3.org/2000/svg" viewBox="0 0 21 21">
      <path fill="#005B80" fill-rule="evenodd"
        d="M12.018 12.468c-2.155 2.156-5.63 2.156-7.784 0-2.155-2.154-2.155-5.63 0-7.784 2.154-2.155 5.63-2.155 7.784 0 2.155 2.155 2.133 5.63 0 7.784M2.54 2.99c-3.078 3.08-3.078 8.07 0 11.15 2.727 2.727 6.927 3.035 10.006.946l5.168 5.167c.572.572 1.517.572 2.09 0 .57-.572.57-1.517 0-2.09l-5.17-5.166c2.09-3.08 1.782-7.28-.945-10.006-3.08-3.077-8.07-3.077-11.15 0">
      </path>
    </svg></button>
  <div class="ilfulllist">
    <p class="il_combo_nor">No Results</p>
    <ul class="combolist"></ul>
  </div>
</form>

<form id="formGenerator">
  <div class="mdscp-form-generator">
    <div class="mdscp-form-generator-each-row  mdscp-regEmail">
      <div class="mdscp-textfield" file-data="[object Object]" component="TextField" validations="[object Object]" show="true" externalurl=""><label for="regEmail" class="mdscp-label-field--regular"> Email </label>
        <div class="input-group"><input type="text" name="regEmail" placeholder="Enter your email address" tabindex="" autocapitalize="none" maxlength="50" class="form-control mdscp-text-field--regular"><!----></div>
      </div><label class="mdscp-error-slot mdscp-error-reg-default" style="display: none;"><img
          src="data:image/png;base64,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"
          class="mdscp-error-slot-icon">
      </label><!---->
    </div>
    <div class="mdscp-form-generator-each-row  mdscp-isSocialEnable" style="display: none;"><input type="hidden" name="isSocialEnable" value="true" file-data="[object Object]" component="HiddenField" class=""><label
        class="mdscp-error-slot mdscp-error-reg-default" style="display: none;"><img
          src="data:image/png;base64,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"
          class="mdscp-error-slot-icon">
      </label><!----></div>
    <div class="mdscp-form-generator-each-row  mdscp-linkedSites" style="display: none;"><input type="hidden" name="linkedSites" file-data="[object Object]" component="HiddenField" class=""><label class="mdscp-error-slot mdscp-error-reg-default"
        style="display: none;"><img
          src="data:image/png;base64,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"
          class="mdscp-error-slot-icon">
      </label><!----></div>
  </div>
  <div class="mdscp-button reg-default"><button data-event-tracking="" class="mdscp-button--submit mdscp-button--medium"><!----><!----><span data-event-tracking=""> Continue </span></button></div>
</form>

Text Content

News & Perspective
Drugs & Diseases
CME & Education
Academy
Video
Decision Point
Edition: English

Medscape

English
Deutsch
Español
Français
Português
UKNew

Univadis

Français New
Italiano New
Log In
Sign Up It's Free!
English Edition

Medscape

* English
* Deutsch
* Español
* Français
* Português
* UKNew

Univadis

* Français New
* Italiano New

X
Univadis from Medscape

No Results

News & Perspective Drugs & Diseases CME & Education Academy Video Decision Point

close
Please confirm that you would like to log out of Medscape. If you log out, you
will be required to enter your username and password the next time you visit.
Log out Cancel

https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTgzNDU2LW1lZGljYXRpb24=

processing....

Drugs & Diseases > Gastroenterology

WILSON DISEASE MEDICATION

Updated: Feb 14, 2019
* Author: Richard K Gilroy, MD, FRACP; Chief Editor: Praveen K Roy, MD, MSc
more...

* 31
* Share
* Print
* Feedback

Close
* Facebook
* Twitter
* LinkedIn
* WhatsApp
* Email

Sections
Wilson Disease

* Sections Wilson Disease
* Overview

* Practice Essentials
* Background
* Etiology
* Epidemiology
* Prognosis
* Show All
* Presentation

* History
* Physical Examination
* Show All
* DDx
* Workup

* Approach Considerations
* Serum Ceruloplasmin
* Urinary Copper Excretion and Hepatic Copper Concentration
* Genetic Testing
* Radiolabeled Copper
* Cranial CT Scanning
* Brain MRI
* PET Scanning
* Electron Microscopy
* Histologic Findings
* Show All
* Treatment

* Approach Considerations
* Long-Term Monitoring
* Molecular Adsorbents Recirculating System (MARS)
* Show All
* Medication

* Medication Summary
* Chelators
* Nutrients
* Show All
* Questions & Answers
* Media Gallery
* Tables
* References

Medication

MEDICATION SUMMARY

The mainstay of therapy for Wilson disease is the use of chelating agents and
medications that block copper absorption from the gastrointestinal (GI) tract.

Zinc and penicillamine are lifelong medications for patients with Wilson
disease. Dosages vary with the severity of the disorder. Another chelating agent
is trientine, which may be more easily tolerated than penicillamine. [21]
Patients who do not respond to zinc therapy and who have increased activities of
liver enzymes should be identified so that chelating agents may be added to the
therapeutic regimen. [22, 23]

Other medications used to treat Wilson disease include anticholinergics,
baclofen, gamma-aminobutyric acid (GABA) antagonists, and levodopa, to treat
parkinsonism and dystonia symptoms; antiepileptics to treat seizures; and
neuroleptics to treat psychiatric symptoms. In addition, protein restriction,
lactulose, or both are used to treat hepatic encephalopathy.

Next: Chelators

CHELATORS

CLASS SUMMARY

Chelating agents bind excess copper. Ammonium tetrathiomolybdate is an
investigational chelating drug used at the University of Michigan as an initial
treatment for patients who present with neurologic or psychiatric
manifestations. This drug works as a chelating agent and as an inhibitor of
copper absorption from the GI tract. [24]

PENICILLAMINE (CUPRIMINE, DEPEN)

* View full drug information

Penicillamine forms soluble complexes with metals excreted in urine. It was the
drug of choice before newer regimens were available. Because of extensive
toxicities, alternative agents are used. It must be administered with pyridoxine
25 mg by mouth daily.

TRIENTINE (SYPRINE)

* View full drug information

Trientine is an effective oral chelator used to induce cupruresis. It is useful
for patients who cannot tolerate penicillamine. It is indicated in Wilson
disease if the initial presentation is hepatic. It should be administered with
zinc.

DIMERCAPROL (BAL IN OIL)

* View full drug information

Dimercaprol is for refractory cases of Wilson disease that are not responding to
first- or second-line chelation treatment.

Previous
Next: Chelators

NUTRIENTS

CLASS SUMMARY

Nutrients are essential to normal growth and development, and they play a role
in many metabolic processes.

ZINC (GALZIN)

* View full drug information

Zinc is a cofactor for more than 70 types of enzymes. It is approved for
patients initially treated with a chelating agent. It should be used for
maintenance after initial chelation therapy. Zinc acetate is the drug of choice
in presymptomatic, pregnant, pediatric populations, and in some instance for
maintenance in compliant patients who have undergone copper chelation therapy.
It is a second-line therapy in patients with neurologic manifestations who do
not tolerate chelation as a consequence of deterioration on this therapy.

PYRIDOXINE (AMINOXIN, PYRI-500)

* View full drug information

Pyridoxine is involved in synthesis of GABA within the CNS.

Questions

REFERENCES

1. Dusek P, Roos PM, Litwin T, Schneider SA, Flaten TP, Aaseth J. The
neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's
diseases. J Trace Elem Med Biol. 2015 Jul. 31:193-203. [QxMD MEDLINE Link].

2. Rodriguez-Castro KI, Hevia-Urrutia FJ, Sturniolo GC. Wilson's disease: A
review of what we have learned. World J Hepatol. 2015 Dec 18.
7(29):2859-70. [QxMD MEDLINE Link].

3. Schilsky ML. Wilson disease: current status and the future. Biochimie. 2009
Oct. 91(10):1278-81. [QxMD MEDLINE Link].

4. Kieffer DA, Medici V. Wilson disease: at the crossroads between genetics
and epigenetics-A review of the evidence. Liver Res. 2017 Sep. 1(2):121-30.
[QxMD MEDLINE Link].

5. Bowcock AM, Farrer LA, Hebert JM, et al. Eight closely linked loci place
the Wilson disease locus within 13q14-q21. Am J Hum Genet. 1988 Nov.
43(5):664-74. [QxMD MEDLINE Link]. [Full Text].

6. Stapelbroek JM, Bollen CW, van Amstel JK, et al. The H1069Q mutation in
ATP7B is associated with late and neurologic presentation in Wilson
disease: results of a meta-analysis. J Hepatol. 2004 Nov. 41(5):758-63.
[QxMD MEDLINE Link].

7. Stuehler B, Reichert J, Stremmel W, Schaefer M. Analysis of the human
homologue of the canine copper toxicosis gene MURR1 in Wilson disease
patients. J Mol Med (Berl). 2004 Sep. 82(9):629-34. [QxMD MEDLINE Link].

8. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation,
diagnosis and long-term outcome of Wilson's disease: a cohort study. Gut.
2007 Jan. 56(1):115-20. [QxMD MEDLINE Link]. [Full Text].

9. Manolaki N, Nikolopoulou G, Daikos GL, et al. Wilson disease in children:
analysis of 57 cases. J Pediatr Gastroenterol Nutr. 2009 Jan. 48(1):72-7.
[QxMD MEDLINE Link].

10. Roberts EA, Schilsky ML; American Association for Study of Liver Diseases
(AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology.
2008 Jun. 47(6):2089-111. [QxMD MEDLINE Link].

11. Yoshitoshi EY, Takada Y, Oike F, et al. Long-term outcomes for 32 cases of
Wilson's disease after living-donor liver transplantation. Transplantation.
2009 Jan 27. 87(2):261-7. [QxMD MEDLINE Link].

12. Schilsky ML. Wilson disease: diagnosis, treatment, and follow-up. Clin
Liver Dis. 2017 Nov. 21(4):755-67. [QxMD MEDLINE Link].

13. Li WJ, Chen C, You ZF, Yang RM, Wang XP. Current drug managements of
Wilson's disease: from west to east. Curr Neuropharmacol. 2016.
14(4):322-5. [QxMD MEDLINE Link]. [Full Text].

14. Walshe JM. Copper: its role in the pathogenesis of liver disease. Semin
Liver Dis. 1984 Aug. 4(3):252-63. [QxMD MEDLINE Link].

15. Dastych M, Prochazkova D, Pokorny A, Zdrazil L. Copper and zinc in the
serum, urine, and hair of patients with Wilson's disease treated with
penicillamine and zinc. Biol Trace Elem Res. 2010 Mar. 133(3):265-9. [QxMD
MEDLINE Link].

16. Langwinska-Wosko E, Litwin T, Dziezyc K, Czlonkowska A. The sunflower
cataract in Wilson's disease: pathognomonic sign or rare finding?. Acta
Neurol Belg. 2016 Sep. 116(3):325-8. [QxMD MEDLINE Link].

17. Soni D, Shukla G, Singh S, Goyal V, Behari M. Cardiovascular and sudomotor
autonomic dysfunction in Wilson's disease--limited correlation with
clinical severity. Auton Neurosci. 2009 Dec 3. 151(2):154-8. [QxMD MEDLINE
Link].

18. Dziezyc K, Litwin T, Chabik G, Czlonkowska A. Measurement of urinary copper
excretion after 48-h d-penicillamine cessation as a compliance assessment
in Wilson's disease. Funct Neurol. 2015 Oct-Dec. 30(4):264-8. [QxMD MEDLINE
Link].

19. Tarnacka B, Szeszkowski W, Golebiowski M, Czlonkowska A. Metabolic changes
in 37 newly diagnosed Wilson's disease patients assessed by magnetic
resonance spectroscopy. Parkinsonism Relat Disord. 2009 Sep. 15(8):582-6.
[QxMD MEDLINE Link].

20. Sen S, Felldin M, Steiner C, et al. Albumin dialysis and Molecular
Adsorbents Recirculating System (MARS) for acute Wilson's disease. Liver
Transpl. 2002 Oct. 8(10):962-7. [QxMD MEDLINE Link].

21. Weiss KH, Thurik F, Gotthardt DN, et al. Efficacy and safety of oral
chelators in treatment of patients with Wilson disease. Clin Gastroenterol
Hepatol. 2013 Aug. 11(8):1028-1035.e2. [QxMD MEDLINE Link].

22. Weiss KH, Gotthardt DN, Klemm D, et al. Zinc monotherapy is not as
effective as chelating agents in treatment of Wilson disease.
Gastroenterology. 2011 Apr. 140(4):1189-98.e1. [QxMD MEDLINE Link].

23. da Costa Mdo D, Spitz M, Bacheschi LA, Leite CC, Lucato LT, Barbosa ER.
Wilson's disease: two treatment modalities. Correlations to pretreatment
and posttreatment brain MRI. Neuroradiology. 2009 Oct. 51(10):627-33. [QxMD
MEDLINE Link].

24. Brewer GJ, Askari F, Dick RB, et al. Treatment of Wilson's disease with
tetrathiomolybdate: V. control of free copper by tetrathiomolybdate and a
comparison with trientine. Transl Res. 2009 Aug. 154(2):70-7. [QxMD MEDLINE
Link].

25. Chaudhry HS, Anilkumar AC. Wilson Disease. StatPearls [Internet]. 2019 Jan
11. 2018:[QxMD MEDLINE Link]. [Full Text].

26. Gerosa C, Fanni D, Congiu T, et al. Liver pathology in Wilson's disease:
from copper overload to cirrhosis. J Inorg Biochem. 2019 Jan 15.
193:106-11. [QxMD MEDLINE Link].

Media Gallery

* Computed tomography (CT) scan in a 15-year-old boy who presented with central
nervous system findings consistent with Wilson disease. The CT scan reveals
hypodense regions in the basal ganglia (caudate nucleus, putamen, globus
pallidus). The differential diagnosis based on this image alone included
leukodystrophy, vasculitis, and, less likely, infection. Ventricular
enlargement and posterior fossa atrophy may also be seen on brain CT scans in
a patient with Wilson disease. The extent of involvement as depicted on CT
scans does not provide prognostic information.

* Approach to the diagnosis of Wilson disease (WD) in a patient with
unexplained liver disease. KF = Kayser-Fleischer ring; CPN = ceruloplasmin.
From the American Association for the Study of Liver Diseases Practice
Guidelines.

* In this particular case, there is abundant Mallory hyaline. Another notable
finding is the moderate to marked chronic inflammation which involved most
portal tracts and periportal/perinodular areas.

* Prismaflex eXeed II adds citrate anticoagulation with integrated calcium
management. Image courtesy of Gambro.

* Molecular adsorbents recirculating system (MARS) circuit.

* Biopsy specimen showing hepatocellular injury in an explant specimen from a
patient transplanted for Wilson Disease.

* Biopsy specimen showing a more detailed image of the cellular injury in acute
Wilson disease.

* Wilson disease biopsy specimen with rhodanine stain.

* Wilson disease biopsy specimen with rhodanine stain (stain specific for
copper deposition).

of 9

TABLES

* Table. Prognostic Index in Fulminant Wilsonian Hepatitis

Table. Prognostic Index in Fulminant Wilsonian Hepatitis

Score

Serum bilirubin (reference range, 3-20 mmol/L)

< 100

100-150

151-200

201-300

>300

Serum aspartate transaminase (reference range, 7-40 IU/L)

< 100

100-150

151-200

201-300

>300

Prothrombin time prolongation (seconds)

< 4

4-8

9-12

13-20

>30

Back to List

CONTRIBUTOR INFORMATION AND DISCLOSURES

Author

Richard K Gilroy, MD, FRACP Gastroenterologist, Intermountain Healthcare

Disclosure: Received salary from gilead, NPS pharmaceuticals, salix
pharmaceuticals, AbbVie for speaking and teaching.

Coauthor(s)

Rahil Shah, MD Consulting Staff, Lebanon Endoscopy Center

Rahil Shah, MD is a member of the following medical societies: American College
of Gastroenterology, American Society for Gastrointestinal Endoscopy

Disclosure: Received consulting fee from Takeda for speaking and teaching.

Michael H Piper, MD Clinical Assistant Professor, Department of Internal
Medicine, Division of Gastroenterology, Wayne State University School of
Medicine; Consulting Staff, Digestive Health Associates, PLC

Michael H Piper, MD is a member of the following medical societies: Alpha Omega
Alpha, American College of Gastroenterology, American College of Physicians,
Michigan State Medical Society