Dear Tim,
You may find this recent article useful.
Muller T, Koppikar S, Taylor RM, Carragher F, Schlenck B, Heinz-Erian
P, Kronenberg F, Ferenci P, Tanner S, Siebert U, Staudinger R, Mieli-Vergani
G, Dhawan A.
Related Articles, Links
Re-evaluation of the penicillamine challenge test in the diagnosis of
Wilson's disease in children.
J Hepatol. 2007 Aug;47(2):270-6. Epub 2007 Apr 4.
PMID: 17449133 [PubMed - indexed for MEDLINE]
Best regards,
Chloe
**************************
Dr Chloe Mak
Chemical Pathologist
MBBS, FRCPA, FHKAM
Division of Clinical Biochemistry
Queen Mary Hospital
Hong Kong
----- Original Message -----
From: "Nick Miller" <[log in to unmask]>
To: <[log in to unmask]>
Sent: Thursday, October 18, 2007 9:16 PM
Subject: Re: Penicillamine challenge in Wilson's disease
> Tim,
>
> I don't often do penicillamine challenge tests as it is a poorly
> tolerated chelating agent, especially in children.
>
> When we do, the protocol is to do 2 baseline measurements of 24h
> urinary copper before starting the test, then at 9.00 am on the day of
> the test give 500 mg of penicillamine, take blood for copper and
> caeruloplasmin, and start a new 24 hour urine copper collection.. A
> second dose of 500 mg of penicillamine is given at 9.00 pm at night
> and the urine collection for copper terminated at 9.00 am the next
> morning. This makes it an in-patient procedure, but if the patient is
> going home I would accept the second dose of penicillamine at 6.00 pm.
>
> The interpretation is a that in Wilson's you expect a serum copper of
> less than 12 mmol/L with an inappropriately low or undetectable
> caeruloplasmin and an incremental excretion of urine copper of greater
> than 20 mmol/L.
>
>
> Reference: da Costa DM, Baldwin D, Portmann B, Lolin Y, Mowat A,
> Mieli-Vergani G. Value of urinary copper excretion after penicillamine
> challenge in the diagnosis of Wilson's disease. Hepatology
> 1992;15:609-615
>
> Glad to see you are using dynamic testing to make your diagnoses of
> metal toxicity!
>
> Nick Miller
> London
>
> On 18/10/2007, Reynolds Tim <[log in to unmask]> wrote:
>> I need to organise a penicillamine challenge test for a patient with
>> probable Wilson's disease there are lots of references to this test on
>> the net but they all give instructions like this:
>>
>> Following penicillamine 0.5 g 12 hourly x 2, urine copper exceeds 25
>> micromol/24 hour in 88% patients with WD and 2% with other liver
>> disorders.
>>
>> Does anyone know the precise time schedule relating the penicillamine
>> should be taken and the time the urine collection starts?
>>
>> i.e. is it start both penicillamine and collection at the same time, or
>> is there a lag between taking the drug and starting th eurine
>> collection?
>>
>> TIM
>>
>> ************************************************************************
>> *************
>> Prof. Tim Reynolds,
>> Queen's Hospital,
>> Belvedere Rd,
>> Burton-on-Trent,
>> Staffordshire,
>> DE13 0RB
>>
>> work tel: 01283 511511 ext. 4035
>> work fax: 01283 593064
>> work email: [log in to unmask]
>> home email: [log in to unmask]
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