- Dosages
- Side effects in children
- Warnings & precautions in children
- Contra-indications in children
-
Interactions - PK
- Renal impairment
- References
Pharmacokinetics in children
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dose recommendation of formulary compared to licensed use (on-label versus off-label)
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Available formulations
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Dosages
| Neuronal ceroid lipofuscinosis type 2 (CLN2) |
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| CAUTION: |
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Renal impaiment in children > 3 months
No information available on dose adjustment in renal impairment.
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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
Very common (> 10%): hypersensitivity. Upper respiratory tract infection. Irritability. Convulsions, headache. Vomiting. Fever. Increase or decrease in protein levels in cerebrospinal fluid, pleiocytosis in cerebrospinal fluid, ECG abnormalities.
Common (1-10%): anaphylactic reaction. Bradycardia. "Dropped head" syndrome. Blistering of oral mucosa and tongue, abdominal pain. Conjunctivitis, infection by the access system. Skin rash, urticaria. Nervousness.
In addition, meningitis has been reported.
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The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here
Contra-indications in children
- life-threatening anaphylactic reaction to cerliponase α, if re-administration is unsuccessful;
- ventriculoperitoneal shunt;
- signs of acute leakage or failure of the intracerebroventricular access system, or infection associated with the access system.
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
Administration Precautions: Use caution with risk factors for complications due to intracerebroventricular administration such as obstructive hydrocephalus. Inspect the scalp for skin integrity before each infusion to ensure that the access system has not been compromised. Before administration, rule out leakage and/or failure of the intracerebroventricular access system.
Monitoring: Monitor vital signs before, periodically during and after infusion. Post-administration observation may be required for an extended period, especially in children < 3 years of age. Monitor ECG during infusion in patients with a history of bradycardia, cardiac arrhythmia or structural heart disease. In cardiac normal patients, perform an ECG every 6 months. Send CSF samples routinely for laboratory testing to detect subclinical infections due to the access system.
Be prepared for any anaphylactic reaction; observe the patient closely during and after infusion. Immediately discontinue administration in the event of an anaphylactic reaction. Use extra caution during re-administration if anaphylaxis has occurred previously.
Infections associated with the access system have been observed, including meningitis (symptoms: fever, headache, neck stiffness, photosensitivity, nausea, vomiting and changes in mental status) and subclinical infections. In clinical trials, antibiotics were administered, the access system was replaced and treatment with cerliponase α continued.
Degradation access system: After long-term use, degradation of the reservoir material of the intracerebroventricular access system occurs. In two clinical cases, the access system showed no signs of failure at the time of infusion, but degradation was demonstrated after removal. Consider replacement of the access system within 4 years of (regular) use.
Research data: There are few data on use in patients >8 years of age. Efficacy and safety in children < 3 years of age have not yet been established. There are few data for children aged 2 years and no clinical data for children < 2 years. Patients with advanced disease progression have been excluded from clinical trials. In advanced CLN2 and neonates, the integrity of the blood-brain barrier may be impaired; the effects of potentially increased peripheral exposure to the drug are unknown
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Interactions
The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here
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| Amino acids and derivatives | ||
|---|---|---|
| A16AA06 | ||
| A16AA05 | ||
| A16AA01 | ||
| A16AA04 | ||
| Enzymes | ||
|---|---|---|
| A16AB03 | ||
| A16AB04 | ||
| A16AB07 | ||
| A16AB13 | ||
| A16AB22 | ||
| 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 | ||
| A16AA04 | ||
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Reference
- BioMarin International Limited, SmPC Brineura (EU/1/17/1192/001) Rev6, 16-8-2022, www.ema.europa.eu
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Changes
Changes
