RAVICTI 1.1 GML

Country: Israel

Language: English

Source: Ministry of Health

Active ingredient:

GLYCEROL PHENYLBUTYRATE

Available from:

MEDISON PHARMA LTD

ATC code:

A16AX09

Pharmaceutical form:

LIQUID

Composition:

GLYCEROL PHENYLBUTYRATE 1.1 G / 1 ML

Administration route:

GASTROENTERAL, ORAL

Prescription type:

Required

Manufactured by:

HORIZON PHARMA IRELAND LTD, IRELAND

Therapeutic area:

GLYCEROL PHENYLBUTYRATE

Therapeutic indications:

RAVICTI is indicated for use as adjunctive therapy for chronic management of adult and paediatric patients ≥2 months of age with urea cycle disorders (UCDs) including deficiencies of carbamoyl phosphate-synthase-I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g., essential amino acids, arginine, citrulline, protein-free calorie supplements).

Authorization date:

2019-10-07

Summary of Product characteristics

1
1.
NAME OF THE MEDICINAL PRODUCT
RAVICTI 1.1 g/ml
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each ml of liquid contains 1.1 g of glycerol phenylbutyrate. This
corresponds to a density of 1.1 g/ml.
3.
PHARMACEUTICAL FORM
liquid.
Clear, colourless to pale yellow liquid.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
RAVICTI is indicated for use as adjunctive therapy for chronic
management of patients with urea
cycle disorders (UCDs) including deficiencies of carbamoyl phosphate
synthetase I (CPS), ornithine
carbamoyltransferase (OTC), argininosuccinate synthetase (ASS),
argininosuccinate lyase (ASL),
arginase I (ARG) and ornithine translocase deficiency
hyperornithinaemia-hyperammonaemia
homocitrullinuria syndrome (HHH) who cannot be managed by dietary
protein restriction and/or
amino acid supplementation alone.
RAVICTI must be used with dietary protein restriction and, in some
cases, dietary supplements (e.g.,
essential amino acids, arginine, citrulline, protein-free calorie
supplements).
4.2
POSOLOGY AND METHOD OF ADMINISTRATION
RAVICTI should be prescribed by a physician experienced in the
management of UCDs.
Posology
RAVICTI must be used with dietary protein restriction and sometimes
dietary supplements (e.g.,
essential amino acids, arginine, citrulline, protein-free calorie
supplements) depending on the daily
dietary protein intake needed to promote growth and development.
The daily dose should be individually adjusted according to the
patient’s protein tolerance and the
daily dietary protein intake needed.
RAVICTI therapy may be required life long unless orthotopic liver
transplantation is elected.
_Adults and children _
The recommended dose for patients naïve to phenylbutyric acid and for
patients switching from
sodium phenylbutyrate or from sodium phenylacetate/sodium benzoate
injection to RAVICTI are
different.
The recommended total daily dose of RAVICTI is based on body surface
area and ranges from 4.5
ml/m
2
/day to 11.2 ml/m
2
/day (5.3 g/m
2
/day to 12.4 g/m
2
/day) and should take into acco