modelImiglucerase
Extends from Pharmacolibrary.Drugs.ATC.A.A16AB02.
Information
| name: | Imiglucerase | |
| ATC code: | A16AB02 | route: | intravenous |
| compartments: | 2 | |
| dosage: | 60 | mg |
| volume of distribution: | 0.07 | L |
| clearance: | 6.7 | mL/min/kg |
| other parameters in model implementation | ||
Imiglucerase is a recombinant enzyme replacement therapy used for the treatment of Gaucher disease type 1, a rare genetic lysosomal storage disorder. It is a modified form of the human enzyme β-glucocerebrosidase, and it helps reduce the accumulation of glucocerebroside in macrophages. Imiglucerase is approved and widely used as a standard care for Gaucher disease type 1.
Pharmacokinetics
Pharmacokinetic parameters reported for adult patients with type 1 Gaucher disease following intravenous infusion. Data represent average PK values from published studies.
References
Berger, J, et al., & Berger, MG (2019). Intra-monocyte Pharmacokinetics of Imiglucerase Supports a Possible Personalized Management of Gaucher Disease Type 1. Clinical pharmacokinetics 58(4) 469–482. DOI:10.1007/s40262-018-0708-8 PUBMED:https://pubmed.ncbi.nlm.nih.gov/30128966
Morris, JL (2012). Velaglucerase alfa for the management of type 1 Gaucher disease. Clinical therapeutics 34(2) 259–271. DOI:10.1016/j.clinthera.2011.12.017 PUBMED:https://pubmed.ncbi.nlm.nih.gov/22264444
Sekijima, Y, et al., & Fukushima, Y (2010). Successful pregnancy and lactation outcome in a patient with Gaucher disease receiving enzyme replacement therapy, and the subsequent distribution and excretion of imiglucerase in human breast milk. Clinical therapeutics 32(12) 2048–2052. DOI:10.1016/j.clinthera.2010.11.008 PUBMED:https://pubmed.ncbi.nlm.nih.gov/21118740
Parameters
| Type | Name | Default | Description |
|---|---|---|---|
| Modelica.Units.SI.Mass | weight (from PK_1C) | 75 | patient weight (kg) |
| Modelica.Units.SI.SpecificVolume | VdPerKg (from PK_1C) | 0.9 | Volume of distribution (L/kg) |
| Modelica.Units.SI.MassFraction | F (from PK_1C) | 0.8 | bioavailiability (0-1) |
| Pharmacolibrary.Types.Clearance | Cl (from PK_1C) | 20 | clearance |
| Modelica.Units.SI.Time | adminTime (from PK_1C) | 60 | first administration time (s) |
| Modelica.Units.SI.Time | adminDuration (from PK_1C) | 600 | administration duration (s) |
| Modelica.Units.SI.Time | adminPeriod (from PK_1C) | 8*60*60 | period of administration (default 8 hours)(s) |
| Pharmacolibrary.Types.Mass | adminMass (from PK_1C) | 1000 | administration mass (mg) |
| Integer | adminCount (from PK_1C) | 8 | number of dose administered (1) |
| Pharmacolibrary.Types.Volume | Vd (from PK_1C) | VdPerKg*weight | Volume of distribution (m3) |
| Pharmacolibrary.Types.MassConcentration | Cmin (from PK_1C) | 0.004 | minimal therapeutic range |
| Pharmacolibrary.Types.MassConcentration | Cmax (from PK_1C) | 0.008 | minimal therapeutic range |
| Pharmacolibrary.Types.MassConcentration | Ctox_peak (from PK_1C) | 0.012 | toxicity peak level |
| Pharmacolibrary.Types.MassConcentration | Ctox_trough (from PK_1C) | 0.006 | toxicity trough level |
| Pharmacolibrary.Types.Volume | Vdp (from PK_2C) | VdpPerKg*weight | Volume of distribution (m3) |
| Modelica.Units.SI.SpecificVolume | VdpPerKg (from PK_2C) | 0.9 | Volume of distribution peripheral(l/kg) |
| Pharmacolibrary.Types.Clearance | k12 (from PK_2C) | 1 | intercompartmental C-P clearance |
| Pharmacolibrary.Types.Clearance | k21 (from PK_2C) | 1 | intercompartmental P-C clearance |
Connectors
| Type | Name | Default | Description |
|---|---|---|---|
| Types.ConcentrationOutput | C_central (from PK_1C) | ||
| Interfaces.ConcentrationPort_b | centralCPort (from PK_1C) | ||
| Interfaces.ConcentrationPort_b | peripheralCPort (from PK_2C) | ||
| Pharmacolibrary.Types.ConcentrationOutput | C_peripheral1 (from PK_2C) |
Components
| Type | Name | Default | Description |
|---|---|---|---|
| Pharmacokinetic.NoPerfusedTissueCompartment | central (from PK_1C) | ||
| Pharmacokinetic.ClearanceDrivenElimination | elim (from PK_1C) | ||
| Sources.PeriodicDose | periodicDose (from PK_1C) | ||
| Modelica.Units.SI.Time | t1_2 (from PK_1C) | elimination half-life | |
| Pharmacolibrary.Pharmacokinetic.TransferFirstOrderNonSym | transfer (from PK_2C) | ||
| Pharmacolibrary.Pharmacokinetic.NoPerfusedTissueCompartment | peripheral (from PK_2C) |
Revisions
- 06/2025 Tomas Kulhanek, generated model from data extracted from PUBMED, DrugBank and LLM(GPT4.1)