Wilson's disease: the 60th anniversary of Walshe's article on treatment with penicillamine

Teive, Hélio Afonso Ghizoni; Barbosa, Egberto Reis; Lees, Andrew

doi:10.1590/0004-282x20160166

Cited by 5 publications

(5 citation statements)

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“…Its main mechanism of action is by the inhibition of HO activity (Juckett et al, 1998 ). Still there are risks of fatalities associated with this drug, along with the onset of aplastic anemia, thrombocytopenia, myasthenia gravis and Goodpasture syndrome (Louie et al, 1986 ; Peces et al, 1987 ; Fishel et al, 1989 ; Kaufman et al, 1996 ; Narayanan and Behari, 1999 ; Teive et al, 2017 ).…”

Section: Ethnopharmacological Approaches For Management Of Jaundice: mentioning

confidence: 99%

Ethnopharmacological Approaches for Therapy of Jaundice: Part I

et al. 2017

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Jaundice is a very common symptom especially in the developing countries. It is associated with several hepatic diseases which are still major causes of death. There are many different approaches to jaundice treatment and the growing number of ethnomedicinal studies shows the plant pharmacology as very promising direction. Many medicinal plants are used for the treatment of jaundice, however a comprehensive review on this subject has not been published. The use of medicinal plants in drug discovery is highly emphasized (based on their traditional and safe uses in different folk medicine systems from ancient times). Many sophisticated analytical techniques are emerging in the pharmaceutical field to validate and discover new biologically active chemical entities derived from plants. Here, we aim to classify and categorize medicinal plants relevant for the treatment of jaundice according to their origin, geographical location, and usage. Our search included various databases like Pubmed, ScienceDirect, Google Scholar. Keywords and phrases used for these searches included: “jaundice,” “hyperbilirubinemia,” “serum glutamate,” “bilirubin,” “Ayurveda.” The first part of the review focuses on the variety of medicinal plant used for the treatment of jaundice (a total of 207 medicinal plants). In the second part, possible mechanisms of action of biologically active secondary metabolites of plants from five families for jaundice treatment are discussed.

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Section: Ethnopharmacological Approaches For Management Of Jaundice: mentioning

confidence: 99%

Ethnopharmacological Approaches for Therapy of Jaundice: Part I

et al. 2017

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“…It is most commonly diagnosed between the ages of 5 and 35 and can be fatal if not treated in time. [1][2][3][4] In the 1940s, the WD diagnosis was based on typical neurologic and hepatic findings, the presence of Kayser-Fleischer (KF) rings detected on the biomicroscopic examination with the slit-lamp, family history, and postmortem liver histology. In the 1950s, WD diagnosis was confirmed by hypoceruloplasminemia, increased 24-hour urinary Cu excretion, and increased Cu content in biopsy-derived liver tissue.…”

Section: Introductionmentioning

confidence: 99%

“…The identification of the WD gene in 1993 6,7 opened up a new approach that is also helpful in the screening of asymptomatic relatives. [4][5][6] Medications to be taken orally include Cu chelating agents and metallothionein inducers. Liver transplantation is appropriate in patients with decompensated liver disease who do not respond to oral medications and do not have severe neurological impairment, or in patients with severe acute liver failure.…”

Section: Introductionmentioning

confidence: 99%

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Wilson disease: the diagnostic challenge and treatment outcomes in a series of 262 cases

Deguti,

Araujo,

Terrabuio

et al. 2024

Arq Neuropsiquiatr

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Background Wilson disease (WD) is an autosomal recessive disorder that leads to organ toxicity due to copper overload. Early diagnosis is complicated by the rarity and diversity of manifestations. Objective To describe the diagnostic features and response to treatment in our cohort of WD patients. Methods This was a retrospective analysis of 262 WD patients stratified by clinical presentation, complementary exams, ATP7B genotyping, and response to treatment. Results Symptoms occurred at an average age of 17.4 (7–49) years, and patients were followed up for an average of 9.6 (0–45) years. Patients presented mainly with hepatic (36.3%), neurologic (34.7%), and neuropsychiatric (8.3%) forms. Other presentations were hematologic, renal, or musculoskeletal, and 16.8% of the patients were asymptomatic. Kayser-Fleischer rings occurred in 78.3% of the patients, hypoceruloplasminemia in 98.3%, and elevated cupruria/24h in 73.0%, with an increase after D-penicillamine in 54.0%. Mutations of the ATP7B gene were detected in 84.4% of alleles. Brain magnetic resonance imaging showed abnormalities in the basal ganglia in 77.7% of patients. D-penicillamine was the first choice in 93.6% of the 245 patients, and 21.1% of these patients were switched due to adverse effects. The second-line therapies were zinc and trientine. The therapeutic response did not differ significantly between the drugs (p = 0.2). Nine patients underwent liver transplantation and 82 died. Conclusion Wilson disease is diagnosed at a late stage, and therapeutic options are limited. In people under 40 years of age with compatible manifestations, WD could be considered earlier in the differential diagnosis. There is a need to include ATP7B genotyping and therapeutic alternatives in clinical practice.

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