TRIENTINE DIHYDROCHLORIDE Metabolic disorders

9.8 TRIENTINE DIHYDROCHLORIDE Metabolic disorders

Indications Wilson’s disease in patients intolerant of

9.8.1 Drugs used in metabolic disorders

penicillamine

9.8.2 Cautions Acute porphyrias see notes above; pregnancy (Appendix 4); interactions: Appendix 1 (trientine)

This section covers drugs used in metabolic disorders Side-effects nausea, rash; rarely anaemia and not readily classified elsewhere.

Dose . 1.2–2.4 g daily in 2–4 divided doses before food; CHILD

initially 0.6–1.5 g daily in 2–4 divided doses before food, adjusted according to response

Trientine Dihydrochloride (Univar) A

9.8.1 Drugs used in metabolic

Capsules , trientine dihydrochloride 300 mg. Label: 6,

disorders 22 ZINC ACETATE

Wilson’s disease Indications Wilson’s disease (initiated under specialist

supervision)

Penicillamine (see also section 10.1.3) is used in Cautions portal hypertension (risk of hepatic decom- Wilson’s disease (hepatolenticular degeneration) to aid

pensation when switching from chelating agent); the elimination of copper ions. See below for other

monitor full blood count and serum cholesterol; indications.

pregnancy (Appendix 4); interactions: Appendix 1 Trientine is used for the treatment of Wilson’s disease

(zinc)

only in patients intolerant of penicillamine; it is not an Contra-indications breast-feeding alternative to penicillamine for rheumatoid arthritis or

Side-effects gastric irritation (usually transient; may cystinuria.

be reduced if first dose taken mid-morning or with a erythematosus may not resolve on transfer to trientine.

Penicillamine-induced

systemic lupus

little protein); less commonly sideroblastic anaemia and Zinc prevents the absorption of copper in Wilson’s

leucopenia

disease. Symptomatic patients should be treated initially

Dose

with a chelating agent because zinc has a slow onset of Note Dose expressed as elemental zinc action. When transferring from chelating treatment to

. Wilson’s disease, 50 mg 3 times daily (max. 50 mg 5 zinc maintenance therapy, chelating treatment should

times daily), adjusted according to response; CHILD 1–

6 years, 25 mg twice daily; 6–16 years, body-weight maximal effect.

be co-administered for 2–3 weeks until zinc produces its

under 57 kg, 25 mg 3 times daily, body-weight over

9 PENICILLAMINE

57 kg, 50 mg 3 times daily; ADOLESCENT 16–18 years,

50 mg 3 times daily

Nutrition

Indications (Orphan Europe) see under Dose below

TA Cautions

Wilzin c

Capsules , zinc (as acetate) 25 mg (blue), net price see section 10.1.3

250-cap pack = £132.00; 50 mg (orange), 250-cap Contra-indications see section 10.1.3

pack = £242.00. Label: 23 Side-effects

and

see section 10.1.3 Dose

blood

. Wilson’s disease, 1.5–2 g daily in divided doses before food; max. 2 g daily for 1 year; maintenance 0.75–1 g daily; ELDERLY

20 mg/kg daily in divided doses, adjusted according to response; CHILD up to 20 mg/kg

Carnitine deficiency

daily in divided doses, minimum 500 mg daily Carnitine is available for the management of primary . Autoimmune hepatitis (used rarely; after disease

carnitine deficiency due to inborn errors of metabolism controlled with corticosteroids), initially 500 mg daily

or of secondary deficiency in haemodialysis patients. in divided doses increased slowly over 3 months; usual maintenance dose 1.25 g daily; ELDERLY not

recommended . Cystinuria, therapeutic, 1–3 g daily in divided doses

CARNITINE

before food, adjusted to maintain urinary cystine Indications primary and secondary carnitine defi- below 200 mg/litre; prophylactic (maintain urinary

ciency

cystine below 300 mg/litre) 0.5–1 g at bedtime; Cautions diabetes mellitus; renal impairment; moni- maintain adequate fluid intake (at least 3 litres daily);

toring of free and acyl carnitine in blood and urine CHILD and ELDERLY minimum dose to maintain urinary

recommended; pregnancy (Appendix 4) and breast- cystine below 200 mg/litre

feeding

. Severe active rheumatoid arthritis, section 10.1.3 Side-effects nausea, vomiting, abdominal pain, diarr- hoea, body odour; side-effects may be dose-related—

Preparations monitor tolerance during first week and after any dose Section 10.1.3

increase

9.8.1 Drugs used in metabolic disorders BNF 57 Dose

Gaucher’s disease

. Primary deficiency,

by mouth , up to 200 mg/kg daily in 2–4 divided doses; higher doses of up to 400 mg/

Imiglucerase, an enzyme produced by recombinant kg daily occasionally required; by intravenous

DNA technology, is administered as enzyme replace- injection over 2–3 minutes, up to 100 mg/kg daily in

ment therapy for non-neurological manifestations of 3–4 divided doses

type I or type III Gaucher’s disease, a familial disorder . Secondary deficiency, by intravenous injection over

affecting principally the liver, spleen, bone marrow, and 2–3 minutes, 20 mg/kg after each dialysis session

lymph nodes.

(dosage adjusted according to carnitine concentra- Miglustat, an inhibitor of glucosylceramide synthase, is tion); maintenance, by mouth , 1 g daily

licensed for the treatment of mild to moderate type I

c Carnitor Gaucher’s disease in patients for whom imiglucerase is (Sigma-Tau) A unsuitable; it is given by mouth. Oral liquid , L -carnitine 100 mg/mL (10%), net price

Paediatric solution , L -carnitine 300 mg/mL (30%), net price 20 mL = £21.00 Excipients include sorbitol

IMIGLUCERASE

Injection , L -carnitine 200 mg/mL. Net price 5-mL amp = £11.90

Indications (specialist use only) non-neurological manifestations of type I or type III Gaucher’s disease Cautions pregnancy (Appendix 4); breast-feeding (Appendix 5); monitor for imiglucerase antibodies; when stabilised, monitor all parameters and response to treatment at intervals of 6–12 months