Mercaptamine

Generic name
Mercaptamine
Brand name
ATC Code
A16AA04

Mercaptamine

Dosages
Side effects in children
Warnings & precautions in children
Contra-indications in children

Interactions
PK
Renal impairment
References

Pharmacokinetics in children

No information on PK in children (source SmPC Cystagon)

dose recommendation of formulary compared to licensed use (on-label versus off-label)

No information is present at this moment.

Available formulations

No information is present at this moment.

Dosages

Nephropathic cystinosis
  • Oral
    • 1 month up to 12 years
      • Initial dose: 0.2 - 0.3 g/m²/day in 4 doses.
      • Maintenance dose: Increase initial dose weekly based on tolerance and leukocyte cystine level (> 1 nmol hemicystine/mg protein)  to 1.3 g/m²/day in 4 doses. Max: 1.95 g/m²/day.
      • When applying the gastro-resistant tablet, divide the daily dose into 2 doses.
        Treatment by or after consultations with a paediatric specialist (metabolic disorders) who has experience using mercaptamine for this indication.

    • 12 years up to 18 years
      • Initial dose: 0.3 - 0.5 g/day in 4 doses.
      • Maintenance dose: Increase initial dose weekly based on tolerance and leukocyte cystine level (> 1 nmol hemicystine/mg protein)  to 2 g/day in 4 doses. Max: 1.95 g/m²/day.
      • When applying the gastro-resistant tablet, divide the daily dose into 2 doses
        Treatment by or after consultations with a paediatric specialist (metabolic disorders) who has experience using mercaptamine for this indication.

Renal impaiment in children > 3 months

GFR ≥10 ml/min/1.73m2: Dose adjustment not required.

GFR <10 ml/min/1.73m2: A general recommendation on dose adjustment cannot be provided.

The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here

Side effects in children

Ehlers-Danlos syndrome-like cases on the elbows have been reported. These skin lesions are in some cases accompanied by vascular proliferation, stretch marks and bone lesions. The results of histopathological examination suggest angio-endotheliomatosis. This side effect has been reported in children chronically treated with high doses (usually above the max dose of 1.95 g/m²/day).

The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here

Contra-indications

No information available on specific contra indications in children.

The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here

Warnings & precautions in children

Treatment of cystinosis is executed in specialized pediatric kidney centers.

In children, treatment with high doses of various cysteamine preparations (cysteamine chlorohydrate or cystamine or cysteamine bitartrate), usually exceeding the maximum daily dose of 1.95 g/m2, was reported to cause a syndrome similar to Ehlers Danlos syndrome and vascular disorders in the elbow area. These skin lesions were associated with vascular proliferation, skin streaks and bone lesions. It is therefore recommended to perform regular skin examinations and to consider bone X-rays if necessary. If any similar skin or bone abnormalities appear, it is recommended to decrease the dose.

[SmPC Cystagon 50 mg hard capsules]

Interactions

The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here

OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS

This pages provides a list of drugs from the same ATC class for comparison. This does not necessarily mean that these drugs are interchangeable.

Amino acids and derivatives
A16AA06
A16AA05
A16AA01
Enzymes
A16AB03
A16AB04
A16AB07
A16AB13
A16AB22
A16AB17
A16AB08
A16AB09
A16AB02
A16AB05
A16AB14
A16AB10
Various alimentary tract and metabolism products
A16AX10
A16AX09
A16AX
A16AX18
A16AX06
A16AX04
A16AX07
A16AX
A16AX08
A16AX07
A16AX05
AMINO ACIDS AND DERIVATIVES
A16AA06
A16AA05
A16AA01

References

  1. Rademaker C.M.A. et al, Geneesmiddelen-Formularium voor Kinderen, 2007
  2. Blau, Hoffmann, Leonard and Clarke, Physicians guide to the treatment and follow-up of metabolic diseases., Springer, 2006
  3. Recordati Rare Diseases, SmPC Cystagon (EU/1/97/039/001) Rev 19, 25-07-2024, www.ema.europa.eu

Changes

Therapeutic Drug Monitoring


Overdose