Diagnosis of Wilson’s Disease – A 20 year audit

Rathnayake, Geetha; Saad, Mirette; Choy, Kay Weng; Doery, James C.G.

doi:10.1016/j.pathol.2015.12.251

Cited by 1 publication

(1 citation statement)

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“…The diagnosis of WD was based on our previous standard [ 20 ], including characteristic clinical manifestations, positive family history, low serum ceruloplasmin (<0.2 g/l), elevated 24-h urinary copper (24-h UC) excretion (>100 µg/24 h), elevated liver copper (>250 µg/g dry weight), presence of a Kayser–Fleischer (K–F) ring, elevated 24-h UC excretion following the administration of 2× 500-mg doses of DPA (>1600 µg/24 h) and magnetic resonance imaging (MRI) of brain [ 21 , 22 ], and met the WD diagnostic criteria formed at the 8th International Meeting on Wilson’s disease [ 23 , 24 ].…”

Section: Methodsmentioning

confidence: 99%

Combined dimercaptosuccinic acid and zinc treatment in neurological Wilson’s disease patients with penicillamine-induced allergy or early neurological deterioration

Zhu

Yang

et al. 2020

Bioscience Reports

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The clinical data of safety and efficacy of a combined treatment with dimercaptosuccinic acid (DMSA) and Zinc with two years’ follow-up in 60 neurological Wilson’s disease (WD) patients was retrospectively analyzed. All the patients included in this study were newly diagnosed and initialized with D-penicillamine (DPA) treatment but were found to have either neurological deterioration or allergy, and their treatment was switched to a combined treatment of DMSA and Zinc. Fifty one patients (85%) had the neurological symptoms improved one year and two years after treatment, 7 (11.67%) experienced a stable neurological condition, and 2 (3.33%) suffered deterioration of neurological symptoms. No early neurological deterioration was observed in all patients. 25% patients experienced mild adverse reactions which did not require a discontinuation of the DMSA and Zinc treatment. Our study confirmed the safety and efficacy of the combined DMSA and Zinc therapy as an initial and probably long-term treatment in neurological WD patients.

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