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Replagal


producent: Shire mÀnskliga genetiska terapier

innehÄller

Replagal Àr ett lÀkemedel mot Fabrys sjukdom.

Aktiva Àmnen

agalsidas alfa


ansökan

Replagal anvÀnds för att behandla:

  • en mycket sĂ€llsynt Ă€rftlig sjukdom, Fabrys sjukdom pĂ„ grund av enzymbrist.

AnvÀnds endast pÄ sjukhuset.


dos

Finns som koncentrat för infusionsvÀtska, efter utspÀdning, i en ven.

Vuxna. Vanligen 0,2 mg per kg kroppsvikt en gÄng varannan vecka.


Eventuella biverkningar

Mycket vanlig (över 10%)

Feber, frossa, skada och obehag, trötthet

illamÄende

huvudvÀrk

Vanlig (1-10%)

Influensaliknande symtom, Powerlessness

Diarré, buksmÀrta, krÀkningar

Akut illamÄende, blÄaktig hud, högt blodtryck, förkylningssymptom, halsont, hjÀrtklappning, hosta, snabb hjÀrtfrekvens, Sammandragning halsen, Nasofaryngit, tryck över bröstet, vÀtskeansamling i t.ex. armar och ben, Andnöd

bröstsmÀrta

Svullnad, ledsmÀrta, muskel- och ledvÀrk, muskelsmÀrta, ryggsmÀrta, ben i ben och ben

FörlÀngd sömn, nedsatt kÀnsla, nervinflammation, krÀkningar, yrsel, förÀndring i hudens kÀnsla

Akne, Skelett, HududslÀtt, RÞdme

Minskad kÀnslighet mot hornhinnan, Tinnitus, TÄrflod

Mindre vanliga (0,1-1%)

Minskad syremÀttnad

Allergisk svullnad i ansikte och mun och struphuvud, anafylaktisk chock - en akut allvarlig allergisk tillstĂ„nd ÖverkĂ€nslighet

Ej kÀnt

HjÀrtarytmi, hjÀrtarytmi, hjÀrtsvikt



graviditet

Bör inte anvÀndas pÄ grund av bristande kunskap. Denna text skiljer sig frÄn företagets godkÀnda produktinformation (produktsammanfattning). AnvÀnds inte pÄ grund av bristande kunskap. Denna text skiljer sig frÄn företagets godkÀnda produktinformation (produktsammantrÀde).

amning

Skall inte anvÀndas pÄ grund av bristande kunskap. Denna text skiljer sig frÄn företagets godkÀnda produktinformation (produktsammanfattning).

trafik

Ingen varning.

givarblod

Falla inte. Givaren mÄste tillkÀnnages av donatorpoolen.

effekt

  • Fungerar genom att bryta ner nĂ„gra Ă€mnen i organismen pĂ„ samma sĂ€tt som enzymet saknar sjukdomen. Detta minskar risken för skador pĂ„ bland annat hjĂ€rta och njurar.
  • Halveringstiden i blodet (TÂœ) Ă€r ungefĂ€r 120 minuter.

farmaceutiska former

Koncentrat till infusionsvÀtska, lösning. 1 ml innehÄller 1 mg agalsidas alfa.



AnvÀndning av andra lÀkemedel

Det Àr viktigt att informera din lÀkare om alla lÀkemedel du tar.
  • Effekten av Replagal reduceras med:
    • Klorokin (malaria och reumatoid artrit)
    • Amiodaron (antihypertensivt medel)
    • Gentamicin (antibiotikum).

Grants, leverans, förpackning och priser

subventionerutlÀmningDoseringsform och styrkapackningPris i kr.
TillhandahÄlls endast till sjukhus. Om inte annat följer av lÀkemedel i doserings grupp Akoncentrera till infusionsvÀtska, lösning 1 mg / ml3,5 ml20.125,60
TillhandahÄlls endast till sjukhus. Om inte annat följer av lÀkemedel i doserings grupp Akoncentrera till infusionsvÀtska, lösning 1 mg / ml3,5 ml (Orifarm)20.665,20
TillhandahÄlls endast till sjukhus. Om inte annat följer av lÀkemedel i doserings grupp Akoncentrera till infusionsvÀtska, lösning 1 mg / ml3,5 ml (EuroPharmaDK)UtgÄr 29-01-2018
TillhandahÄlls endast till sjukhus. Om inte annat följer av lÀkemedel i doserings grupp Akoncentrera till infusionsvÀtska, lösning 1 mg / ml4 x 3,5 ml80.530,05



Replagal

FAQ - 💬

❓ What is Replagal used for?

👉 Replagal is used to treat patients who have Fabry disease, a rare inherited disorder. Patients with Fabry disease do not have enough of an enzyme called alpha-galactosidase A. This enzyme normally breaks down a fatty substance called globotriaosylceramide (Gb3 or GL-3).

❓ Who manufactures Replagal?

👉 Replagal is a treatment for Fabry disease patients ages 7 and older. Shire (now owned by Takeda) developed it.

❓ How is Replagal administered?

👉 Replagal is administered at a dose of 0.2 mg/kg body weight every other week by intravenous infusion over 40 minutes. Studies in patients over the age of 65 years have not been performed and no dosage regimen can presently be recommended in these patients as safety and efficacy have not yet been established.

❓ What is Fabry's disease?

👉 Fabry disease is an inherited disorder that results from the buildup of a type of fat, called globotriaosylceramide, in the body's cells. Beginning in childhood, this buildup causes signs and symptoms that affect many parts of the body.

❓ How is Replagal made?

👉 It is produced by switching on the gene for α-galactosidase A in cells. The enzyme is then removed from the cells and made into a sterile concentrate for solution for infusion. Before REPLAGAL is given to you, it is mixed with 0.9% sodium chloride intravenous solution (saline).

❓ Is Replagal available in the US?

👉 It is being developed by the biotechnology company Shire (now owned by Takeda). Although in use in many countries, including Canada, Russia, the United Kingdom, Mexico, Israel, and many members of the European Union, Replagal has not been approved by the U.S. Food and Drug Administration (FDA).

❓ How is Fabry disease treated?

👉 Enzyme replacement therapy (ERT) is the only specific treatment for Fabry disease. The beneficial effect of ERT on different organs/systems has been extensively evaluated. Quality of life of patients receiving ERT is improved.

❓ Is Replagal in America available?

👉 It is being developed by the biotechnology company Shire (now owned by Takeda). Although in use in many countries, including Canada, Russia, the United Kingdom, Mexico, Israel, and many members of the European Union, Replagal has not been approved by the U.S. Food and Drug Administration (FDA).

❓ How long is the average lifespan of a person with Fabry disease?

👉 Published data from the Fabry registry indicates that male Fabry disease patients live an average of about 58 years, compared to about 75 years for men in the general population in the U.S. For women with Fabry disease, the average life expectancy is around 75 years compared to 80 years for women in the U.S. general ...

❓ What triggers Fabry disease?

👉 What causes Fabry disease? Children inherit a mutation (change) in the galactosidase alpha (GLA) gene on the X chromosome from a parent. The GLA gene produces the alpha-GAL enzyme that helps break down fatty substances (sphingolipids). People who inherit a defective GLA gene don't produce enough alpha-GAL enzyme.

❓ Is Neflamapimod available?

👉 At this time, our investigational drug neflamapimod is only available through participation in one of our ongoing clinical trials.

👉 Replagal is medicine that contains the active substance agalsidase alfa. It is available as a concentrate to be made into solution for infusion (drip) into a vein. What is Replagal used for? Replagal is used to treat patients who have Fabry disease, a rare inherited disorder.

👉 Clinical particulars Replagal is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry Disease (α-galactosidase A deficiency). Replagal treatment should be supervised by a physician experienced in the management of patients with Fabry Disease or other inherited metabolic diseases.

👉 The pharmaceutical company Shire manufactures agalsidase alfa (INN) under the trade name Replagal as a treatment for Fabry disease, and was granted marketing approval in the EU in 2001. FDA approval was applied for the United States.

👉 Replagal is a concentrated form of the enzyme alpha-galactosidase A that is administered via infusion into the bloodstream. The formulation, which includes sodium phosphate, monohydrate, polysorbate 20, sodium chloride, and sodium hydroxide, allows the enzyme to enter the cells easily where it can break down Gb3 and clear it out of the cell.


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