Trientine induced colitis during therapy for Wilson disease: a case report and review of the literature

BMC Clinical Pharmacology

Michael L. Schilsky¹  Dhanpat Jain²  Salih Boga¹ 

[1]Division of Digestive Diseases and Section of Transplantation and Immunology, Department of Medicine and Surgery, Yale University School of Medicine, Yale University Medical Center, 333 Cedar Street, LMP 1080, New Haven 06520, CT, USA

[2]Department of Pathology, Yale University School of Medicine, 310 Cedar Street, New Haven 06520-8023, CT, USA

关键词: Wilson disease;    Colitis;    Trientine;   

Others  :  1233823
DOI  :  10.1186/s40360-015-0031-z

 received in 2015-05-14, accepted in 2015-11-12,  发布年份 2015

【 摘 要 】

Background

Wilson disease (WD) is an autosomal recessive disorder of human copper metabolism characterized by copper accumulation in the liver due to impaired excretion of copper into the bile. Brain accumulation of copper may cause neuropsychiatric symptoms. Trientine (triethylenetetramine dihydrochloride) is a copper-chelating agent used to treat patients with WD. Trientine has been considered an option for initial treatment and maintentance therapy of WD due to its safety profile.

Case presentation

A 40 year old female with a recent diagnosis of WD was started on treatment with trientine for her WD. Within one month she developed profound bloody diarrhea unresponsive to medical treatment. Trientine was discontinued and a colonoscopy with biopsy showed moderately active ileitis and moderate to severe pancolitis, consistent with a drug induced mucosal injury. The colitis improved immediately upon withdrawal of trientine, and recurred when medication was rechallenged because of worsened WD symptoms. After second compulsory discontinuation of trientine, she remained on zinc therapy for her WD and her colitis resolved by time.

Conclusion

Drug induced colitis is a very rare side effect of trientine. Although trientine therapy is well tolerated and less side effects are reported with this medication than penicillamine, colitis can occur during trientine treatment. Zinc therapy may be an effective alternative for treatment of WD in patients experiencing side effects from chelation therapy.

【 授权许可】

   
2015 Boga et al.

【 图 表 】

Fig. 1.

【 参考文献 】

• [1]Scheinberg IH. Wilson’s disease. J Rheumatol Suppl. 1981; 7:90-93.
• [2]Waslhe JM. Management of penicillamine nephropathy in Wilson’s disease: A new chelating agent. Lancet. 1969; 2:1401-1402.
• [3]Waslhe JM. Wilson’s disease; new oral therapy. Lancet. 1956; 270:25-26.
• [4]Scheinberg IH, Jaffe ME, Sternlieb I. The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson’s disease. N Eng J Med. 1987; 317:209-213.
• [5]EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012; 56:671-685.
• [6]Roberts EA, Schilsky ML. American Association for Study of Liver Diseases (AASLD), Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008; 47:2089-2111.
• [7]Product Information. Syprine (trientine). Aton Pharma, Lawrenceville, NJ; 2013
• [8]Gibbs KR, Walshe JM. Orphan Diseases. The Metabolism of Trientine: Animal Studies. Orphan Drugs JH, Scheimberg Walshe JM, editors. Manchester University Press, Manchester, UK; 1986.
• [9]Dahlman T, Hartvig P, Löfholm M, Nordlinder H, Lööf L, Westermark K. Long-term treatment of Wilson’s disease with triethylene tetramine dihydrochloride (trientine). Q J Med. 1995; 88:609-616.
• [10]Członkowska A, Litwin T, Karliński M, Dziezyc K, Chabik G, Czerska M. D-penicillamine versus zinc sulfate as first-line therapy for Wilson's disease. Eur J Neurol. 2014; 21:599-606.
• [11]Wiernicka A, Jańczyk W, Dądalski M, Avsar Y, Schmidt H, Socha P. Gastrointestinal side effects in children with Wilson's disease treated with zinc sulphate. World J Gastroenterol. 2013; 19:4356-4362.