Displaying all 10 publications

    Med J Malaya, 1956 Dec;11(2):134-8.
    PMID: 13417937
    Matched MeSH terms: Hepatolenticular Degeneration*
  2. Low QJ, Siaw C, Lee RA, Cheo SW
    QJM, 2020 Sep 01;113(9):693-694.
    PMID: 31917404 DOI: 10.1093/qjmed/hcaa005
    Matched MeSH terms: Hepatolenticular Degeneration*
  3. Greer KE, Askew FC, Richardson DR
    Arch Dermatol, 1976 Sep;112(9):1267-9.
    PMID: 136925
    A 41-year-old patient with hepatolenticular degeneration (Wilson disease), who had been treated for 15 years with penicillamine, developed small white papules at sites of venipuncture in the antecubital fossae and at surgical suture sites. Histologically, these papules showed focal areas of connective tissue degeneration in the dermis, but there was no evidence of inclusion cysts. The changes most likely resulted from the effect of penicillamine on new connective tissue formation at the sites of injury. The patient also developed crinkling of the skin of her face and neck while on the penicillamine regimen, and these changes were attributed, at least in part, to the effects of this drug on connective tissue.
    Matched MeSH terms: Hepatolenticular Degeneration/drug therapy*; Hepatolenticular Degeneration/pathology
  4. Loh KY, Kew ST
    Aust Fam Physician, 2010 Sep;39(9):647-8.
    PMID: 20877768
    Matched MeSH terms: Hepatolenticular Degeneration/complications*; Hepatolenticular Degeneration/genetics; Hepatolenticular Degeneration/metabolism; Hepatolenticular Degeneration/ultrasonography
  5. Mohamed R, Tan CT, Wong NW
    Med J Malaysia, 1994 Mar;49(1):49-52.
    PMID: 8057991
    The clinical course of 18 patients with Wilson's disease is reported. There were 13 males and five females of whom one is Malay. The prevalence of Wilson's disease in Malaysia is probably the same as elsewhere. Being a genetic syndrome, the genetic carrier rate for Wilson's disease is probably lower amongst the Malays. At diagnosis, the clinical signs were predominantly hepatic in 10 patients, neurological in five patients with three asymptomatic cases. All patients were commenced on penicillamine but poor compliance was observed in many patients. Two patients defaulted follow-up and seven patients died. Out of the nine surviving patients, only four are well with no clinical symptoms.
    Matched MeSH terms: Hepatolenticular Degeneration/diagnosis; Hepatolenticular Degeneration/drug therapy; Hepatolenticular Degeneration/genetics; Hepatolenticular Degeneration/epidemiology*
  6. Xu J, Jiang H, Li J, Cheng KK, Dong J, Chen Z
    PLoS One, 2015;10(4):e0119654.
    PMID: 25849323 DOI: 10.1371/journal.pone.0119654
    Wilson's disease (WD), also known as hepatoleticular degeneration (HLD), is a rare autosomal recessive genetic disorder of copper metabolism, which causes copper to accumulate in body tissues. In this study, rats fed with copper-laden diet are used to render the clinical manifestations of WD, and their copper toxicity-induced organ lesions are studied. To investigate metabolic behaviors of 'decoppering' process, penicillamine (PA) was used for treating copper-laden rats as this chelating agent could eliminate excess copper through the urine. To date, there has been limited metabolomics study on WD, while metabolic impacts of copper accumulation and PA administration have yet to be established.
    Matched MeSH terms: Hepatolenticular Degeneration/drug therapy*; Hepatolenticular Degeneration/metabolism*; Hepatolenticular Degeneration/pathology
  7. Nazariah Aiza, H., Aisah, A.R., Anita, C., Yeoh, S.H., Ng, C.G.
    Wilson disease is an inherited metabolic disorder. It is an autosomal recessive disorder caused by mutation of ATP7B gene, which results in excessive accumulation of copper in the body and deposition in various organs. The clinical presentation varies and neuropsychiatric manifestations are common. It is a diagnostic challenge in the initial phase where it mimics other psychiatric conditions and the diagnosis of Wilson disease is based on a combination of laboratory tests and clinical features. Wilson disease treatment comprises of copper chelating therapy such as D-Penicillamine and zinc sulphate wheras the behavior and mood symptoms response well with atypical antipsychotic treatment. The present report illustrates two cases of Wilson disease in middle-aged patients. The first presentation involved changes in behavior and personality. There was some delay in making the diagnosis in the initial stage. Both cases were diagnosed to have Wilson disease after further investigations. Their condition improved with the combination of copper chelating agent and atypical antipsychotic. In conclusion, it emphasizes the awareness of psychiatric manifestations as the initial presentation of Wilson disease.
    Matched MeSH terms: Hepatolenticular Degeneration
  8. Tan SS, Latif SA, Poh WY
    Med J Malaysia, 2012 Jun;67(3):323-5.
    PMID: 23082426 MyJurnal
    Penicillamine toxicity in Wilson's disease has been well reported but rarely seen now as newer agents are being used. We present a case who developed multiple rare complications of Penicillamine concurrently. Our patient is one of three siblings on Penicillamine, she was the only one who developed massive breast enlargement four months after commencing Penicillamine therapy, as well as dermatological adverse reactions and myasthenia gravis three more months later. All the adverse effects improved soon after substitution of the offending agent with Trientine.
    Matched MeSH terms: Hepatolenticular Degeneration/drug therapy
  9. Ping CC, Hassan Y, Aziz NA, Ghazali R, Awaisu A
    J Clin Pharm Ther, 2007 Feb;32(1):101-7.
    PMID: 17286794
    To report a case of early-decompensated liver cirrhosis secondary to discontinuation of penicillamine therapy in a patient with Wilson's disease.
    Matched MeSH terms: Hepatolenticular Degeneration/drug therapy*
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