Wilson’s disease is an autosomal recessive disorder of copper metabolism associated with deposition in various organs including the heart and brain. Hepatic manifestations are commonest but extrahepatic known which include neuropsychiatric features, haemolytic anaemia, arthropathy etc. Knowledge all these result early clinical suspicion diagnosis confirmed by increased urinary levels decreased ...

BACKGROUND Wilson's disease (WD) evolves rapidly and is fatal if untreated. The treatment of WD patients with mild liver disease is not clearly defined. To address this issue, we evaluated long-term outcomes of three treatment regimens (D-penicillamine, zinc or both) in patients diagnosed in childhood. METHODS We retrospectively evaluated efficacy, compliance and reasons for treatment discont...

Introduction: The Wilson disease is an autosomal recessive disease in which the liver, central nervous system, eyes, blood and other parts of the body involved. Timely diagnosis and appropriate treatment of the disease requires awareness of the clinical presentations of this disease in children.Methods: This case series study included 62 patients with Wilson disease who admitted to children's M...

Dense, granular immunoglobulin deposits have been identified at the epidermo-dermal junction in 4 out of 10 patients who developed toxic reactions to D-penicillamine therapy for rheumatoid arthritis. Three of 4 patients developing a lupus-like syndrome while on penicillamine had similar findings on skin biopsy. Serum immunoglobulin and complement levels decreased significantly in patients treat...

A study was made in rabbits of the effect of topical dexamethasone ointment, .05%, and either subconjunctival or intraperitoneal D-penicillamine on the duration of function of glaucoma filtering surgery. The drugs were tested alone and in combination. Intraperitoneal D-penicillamine was not effective, either alone or combined with topical dexamethasone. Topical dexamethasone ointment alone and ...

OBJECTIVES To investigate whether the therapeutic response of rheumatoid arthritis (RA) patients to D-penicillamine is associated with polymorphisms in genes of the glutathione S-transferase (GST) supergene family. METHODS Disease activity in 81 patients with RA treated with D-penicillamine monotherapy was assessed using the Stoke Index, a validated index of disease activity, prior to treatme...

Two drugs, D-penicillamine and daunorubicin, were tested for their effect on proliferation and collagen synthesis of cultured conjunctival fibroblasts. This cell type is likely responsible for scar formation and ultimate filter surgery failure in glaucoma patients. Both drugs were antiproliferative; however, D-penicillamine required 2000 times the concentration of daunorubicin to achieve a simi...

Intraperitoneal gelatin sponge implants were used in guinea-pigs to examine the effect of D-penicillamine on the delayed hypersensitivity reaction in vivo. When it was administered daily in a dose of 200 mg/kg for 14 days before sensitisation or for 14 or 30 days before challenge, D-penicillamine increased the number of exudate cells during the onset of the delayed hypersensitivity reaction. Ab...

D-penicillamine is not generally considered to cause hepatic damage. Cholestatic jaundice developed in a patient with rheumatoid arthritis 4 weeks after penicillamine was added to his regimen, and he died in acute renal failure. The probability that penicillamine caused the cholestasis is discussed.

D-penicillamine (D-pen) has gained wide-spread use as a potent suppressor of disease activity in rheumatoid arthritis (RA) and juvenile chronic arthritis (JCA). The drug has been established for antirheumatotd therapy on the basis o f a considerable number o f comparative clinical studies o f D-pen treatment versus placebo or other disease modifying antirheumatic drugs (DMARDs). Despite the pos...