Potential Hepatotoxicity of Penicillamine Treatment in Three Patients with Wilson's Disease

Deutscher, J; Kieß, Wieland; Scheerschmidt, G; Willgerodt, H

doi:10.1097/00005176-199911000-00031

Cited by 19 publications

(9 citation statements)

References 4 publications

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“…Very late side effects include nephrotoxicity, severe allergic response upon restarting the drug after it has been discontinued, myasthenia gravis, polymyositis, loss of taste, immunoglobulin A depression, and serous retinitis. Hepatotoxicity has been reported 169. Hepatic siderosis has been reported in treated patients with reduced levels of serum ceruloplasmin and non–ceruloplasmin bound copper 170…”

Section: Treatmentmentioning

confidence: 99%

Diagnosis and treatment of Wilson disease: An update

Roberts¹,

2008

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Section: Treatmentmentioning

confidence: 99%

Diagnosis and treatment of Wilson disease: An update

Roberts¹,

2008

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“…Hepatotoxicity has been reported. 143 Hepatic siderosis has been reported in association with treated patients with reduced levels of serum ceruloplasmin and nonceruloplasmin-bound copper. 144 Tolerability of pencillamine may be enhanced by starting with incremental doses, 250 to 500 mg/d, increased by 250 mg increments every 4 to 7 days to a maximum of 1,000 to 1,500 mg/d in 2 to 4 divided dosages.…”

Section: Available Treatmentsmentioning

confidence: 99%

A practice guideline on Wilson disease

2003

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PreambleThis guideline is intended for use by physicians. It describes preferable up-to-date approaches to the diagnosis and treatment of patients with Wilson disease. As their purpose is to direct patient care, these guidelines should not be considered inflexible mandates. They have been developed in a manner consistent with the American Association for the Study of Liver Diseases Policy Statement on Development and Use of Practice Guidelines.These guidelines provide data-supported approaches to the diagnosis and management of patients with Wilson disease. They are based on, first, broad-based review of the published literature in pediatrics and medicine including Medline searches on hepatolenticular degeneration and related subjects; and second, 40 accumulated years of personal experience of the authors. In order to standardize recommendations as much as possible, each has been characterized with Roman numerals I through IV to indicate the quality of evidence on which the recommendation is based (Table 1). 1 A significant problem with the literature on Wilson disease is that patients are sufficiently rare to preclude large cohort studies or randomized controlled trials; moreover, most treatment modalities were developed at a time when conventions for drug assessment were less stringent than currently.

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“…In many, this worsening was transient and improved with continued administration of this drug, but some patients developed irreversible and severe neurologic deficits. With respect to liver disease, reports indicate that worsening hepatic inflammation detected on blood testing in some patients is due to penicillamine because these symptoms were alleviated following its withdrawal [11]. Although this effect appears to be infrequent, it could also be under-reported, because treatment is often begun at a time when liver function tests are abnormal, and initial worsening of the disease is often thought to be disease progression.…”

Section: Penicillaminementioning

confidence: 99%

Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation?

Schilsky

2001

Curr Gastroenterol Rep

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New options are available for the medical treatment of patients with Wilson's disease. Penicillamine is no longer the treatment of choice, as there is a growing experience with safer and more effective alternatives. Trientine may be the best choice for initial therapy in symptomatic patients requiring chelation therapy, and it may be even more effective when used in combination with zinc, which is recommended for maintenance therapy. Further studies are needed to determine the best therapy for pregnant patients with Wilson's disease, and whether combination therapy using trientine and zinc will be the next treatment of choice for all symptomatic patients with liver or neurologic disease.

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Potential Hepatotoxicity of Penicillamine Treatment in Three Patients with Wilson's Disease

Cited by 19 publications

References 4 publications

Diagnosis and treatment of Wilson disease: An update

Diagnosis and treatment of Wilson disease: An update

A practice guideline on Wilson disease

Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation?

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