Glycerolfenylbutyraat
Generic name
Glycerolfenylbutyraat
Brand name
ATC Code
A16AX09
- Dosages
- Side effects in children
- Warnings & precautions in children
- Contra-indications in children
-
Interactions - PK
- Renal impairment
- References
Pharmacokinetics in children
| < 2 year | 3-5 year | 6-11 year | 12-17 year | |
| PAA Clearance (l/hour) | 7,1 | 10,9 | 16,4 | 24,4 |
dose recommendation of formulary compared to licensed use (on-label versus off-label)
On-label
Licensed use Licensed useAvailable formulations
Drank 1,1 g/ml
Dosages
| Chronic Urea Cycle Disorders |
|---|
|
Renal impaiment in children > 3 months
No information available on dose adjustment in renal impairment.
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
< 2 months: anemia, thrombocytosis, hypophagia, gastroesophageal reflux, diarrhea, constipation, flatulence, skin rash, decrease in amino acid levels, increase in liver enzyme levels (transaminases, γ-GT).
> 2 months to 2 years of age: constipation, diarrhea, skin rash, eczema, nail rash.
The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here
Adult Side Effects
Vaak (1-10%): verandering in eetlust, voedselaversie. Duizeligheid, hoofdpijn, tremoren. Oraal ongemak, misselijkheid, braken, kokhalzen, dyspepsie, buikpijn, opgezette buik, flatulentie, obstipatie, diarree. Abnormale lichaamsgeur, acne. Metrorragie. Vermoeidheid, perifeer oedeem. Stijging van ASAT en ALAT, toegenomen anion gap, afname lymfocytenaantal, daling vitamine D-concentratie.
Soms (0,1-1%): gastro-intestinale virale infectie. Hypothyreoïdie. Hypoalbuminemie, hypokaliëmie. Dysgeusie, lethargie, paresthesie, psychomotorische hyperactiviteit, slaperigheid, spraakstoornis. Verwardheid, gedeprimeerde stemming. Ventriculaire aritmie. Opvliegers. Dysfonie, epistaxis, verstopte neus, orofaryngeale pijn, irritatie van de keel. Droge mond, oprispingen, stomatitis, verandering in ontlasting, pijnlijke stoelgang, steatorroe. Galblaaspijn, blaaspijn. Alopecia, hyperhidrose, jeukende huiduitslag. Rugpijn, zwelling van de gewrichten, spierspasmen, pijn in de ledematen, fasciitis plantaris. Amenorroe, onregelmatige menstruatie. Gewichtsverandering, koorts. Stijging kaliumconcentratie en triglyceridenconcentratie, abnormaal ECG, stijging LDL-concentratie, verlengde protrombinetijd, stijging leukocytenaantal.
The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here
Contra-indications in adults
behandeling van acute hyperammoniëmie.
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
The dose for children with mild, moderate or severe hepatic impairment should be started at the low end of the recommended dose range (5.3 g / m2 / day) and maintained at the lowest dose required to control the patient's ammonia levels.
In unexplained drowsiness, confusion, nausea and lethargy in children with normal or low ammonia levels, there may be high phenylacetic acid (PAA) levels (especially in children younger than 2 months). If the PAA level exceeds 500 µg / l and the ratio of PAA to phenylacetyl glutamine (PAGN) in the plasma exceeds 2.5, consideration should be given to reducing the dose of glycerol phenyl butyrate or increasing the frequency of dosing.
The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here
Alerts And Precautions Adults
Gebruik in combinatie met een eiwitarm dieet en soms met voedingssupplementen (bijv. essentiële aminozuren, arginine, citrulline, eiwitvrije caloriesupplementen), afhankelijk van de dagelijkse eiwitconsumptie van voeding die nodig is om groei en ontwikkeling te bevorderen.
Tijdens de behandeling kan acute hyperammoniëmie, waaronder hyperammoniëmische encefalopathie, optreden.
Een lage concentratie of afwezigheid van pancreasenzymen of darmaandoeningen die resulteren in slechte vetopname, kunnen leiden tot verminderde of afwezige omzetting van glycerolfenylbutyraat en/of absorptie van fenylbutyraat; controleer de ammoniakspiegels nauwlettend bij patiënten met pancreasinsufficiëntie of intestinale malabsorptie.
De veiligheid en werkzaamheid bij patiënten met N-acetylglutamaatsynthase (NAGS)- en CITRIN (citrullinemie type 2)-deficiëntie. Wees voorzichtig bij ernstige nierinsufficiëntie wegens het ontbreken van gegevens. Er zijn weinig gegevens bij ouderen ≥ 65 jaar.
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 | ||
| A16AA04 | ||
| Enzymes | ||
|---|---|---|
| A16AB03 | ||
| A16AB04 | ||
| A16AB07 | ||
| A16AB13 | ||
| A16AB22 | ||
| A16AB17 | ||
| A16AB08 | ||
| A16AB09 | ||
| A16AB02 | ||
| A16AB05 | ||
| A16AB14 | ||
| A16AB10 | ||
| Various alimentary tract and metabolism products | ||
|---|---|---|
| A16AX10 | ||
| A16AX | ||
| A16AX18 | ||
| A16AX06 | ||
| A16AX04 | ||
| A16AX07 | ||
| A16AX | ||
| A16AX08 | ||
| A16AX07 | ||
| A16AX05 | ||
| AMINO ACIDS AND DERIVATIVES | ||
|---|---|---|
| A16AA06 | ||
| A16AA05 | ||
| A16AA01 | ||
| A16AA04 | ||
Reference
- Horizon Pharma Ireland Limited, SmPC Ravicti (EU/1/15/1062/001-4) 29-01-2019, www.geneesmiddeleninformatiebank.nl
Changes
Therapeutic Drug Monitoring
Overdose
