natgillin/translations-raw huggingface dataset:
The pharmacokinetic data of homocystinuric patients on long-term betaine supplementation are very similar to those of healthy volunteers.
Farmakokinetični podatki bolnikov s homocistinurijo, ki dolgoročno prejemajo dodatek betaina, so zelo podobni kot pri zdravih prostovoljcih.
This demonstrates that differences in betaine kinetics are most probably due to betaine depletion in untreated homocystinuria and are only meaningful for the initial treatment.
To kaže, da so razlike v kinetiki betaina najverjetneje posledica izčrpanja betaina pri nezdravljeni homocistinuriji in so pomembne samo za začetno zdravljenje.
Long-term carcinogenicity and reproductive toxicity studies have not been conducted on betaine.
Dolgoročnih študij kancerogenosti in vpliva na sposobnost razmnoževanja z betainom niso opravili.
6.2 Incompatibilities
5 6. 2 Inkompatibilnosti
5 After the first opening:
Po prvem odpiranju:
1 month.
1 mesec.
HDPE bottles with a child resistant closure.
Steklenice iz HDPE s pokrovčkom z za otroke varno zaporko.
Each pack contains 1 bottle with 180 g of powder.
Vsaka škatlica vsebuje 1 steklenico s 180 g praška.
Three measuring spoons are included in each pack.
V vsaki škatlici so priložene tri merilne žličke.
Orphan Europe SARL Immeuble “ Le Wilson” F - 92058 Paris-La Défense France
Orphan Europe SARL Immeuble “ Le Wilson ” F - 92058 Paris- La Défense Francija
Immeuble "Le Wilson" 70 avenue du Général de Gaulle F-92800 Puteaux France
Orphan Europe SARL Immeuble “ Le Wilson ” 70 avenue du Général de Gaulle F - 92800 Puteaux Francija
Medicinal product subject to restricted medical prescription (see annex I:
Izdaja zdravila je le pod omejenimi pogoji in na recept (glejte Dodatek I:
The MAH must ensure that the system of pharmacovigilance is in place and functioning before the product is placed on the market and for as long as the marketed product remains in use.
Imetnik dovoljenja za promet z zdravilom mora zagotoviti, da je sistem farmakovigilance vzpostavljen in deluje, preden da zdravilo v promet in toliko časa, kolikor bo zdravilo v uporabi.
The Marketing Authorisation Holder commits to performing the studies as detailed in the Pharmacovigilance Plan.
Imetnik dovoljenja za promet z zdravilom se zavezuje, da bo izvajal študije in dodatne farmakovigilančne dejavnosti, ki so opisane v Farmakovigilančnem načrtu.
10 PARTICULARS TO APPEAR ON THE OUTER PACKAGING
10 PODATKI NA ZUNANJI OVOJNINI
Cystadane 1 g oral powder Betaine anhydrous
Zdravilo Cystadane 1 g peroralni prašek brezvodni betain
Three measuring spoons dispense 1 g, 150 mg and 100 mg of betaine anhydrous.
Priložene so tri merilne žličke, s katerimi odmerimo 1 g, 150 mg in 100 mg brezvodnega betaina.
Lightly shake the bottle before opening.
Preden steklenico odprete, jo rahlo pretresite.
EXP {month/ year} Shelf life after the first opening:
EXP {mesec/ leto} Rok uporabnosti po prvem odpiranju:
Cystadane 1 g oral powder
zdravilo Cystadane 1 g peroralni prašek
12 PARTICULARS TO APPEAR ON THE THE IMMEDIATE PACKAGING
12 PODATKI NA PRIMARNI OVOJNINI
EU/ 1/ 06/ 379/ 001
EU/ 1/ 06379/ 379/ 001
15 PACKAGE LEAFLET:
15 NAVODILO ZA UPORABO
What Cystadane is and what it is used for 2.
Kaj je zdravilo Cystadane in za kaj ga uporabljamo 2.
Before you take Cystadane 3.
Kaj morate vedeti, preden boste vzeli zdravilo Cystadane 3.
How to take Cystadane 4.
Kako jemati zdravilo Cystadane 4.
Possible side effects 5 How to store Cystadane 6.
Možni neželeni učinki 5 Shranjevanje zdravila Cystadane 6.
WHAT CYSTADANE IS AND WHAT IT IS USED FOR
KAJ JE ZDRAVILO CYSTADANE IN ZA KAJ GA UPORABLJAMO
Cystadane contains betaine anhydrous which is intended to be an adjunctive treatment of homocystinuria, an inborn error of metabolism.
Zdravilo Cystadane vsebuje brezvodni betain, ki je namenjen dodatnemu zdravljenju homocistinurije, prirojene napake presnove.
Methionine is an amino acid which is present in regular food protein (e. g. meat, fish, milk, cheese, eggs).
Metionin je aminokislina, prisotna v običajnih beljakovinah v hrani (npr. mesu, ribah, siru, jajcih).
Homocystinuria is a disease caused by the accumulation of homocysteine which is not converted to cysteine and is characterized by formation of clots in the veins, bone weakness, and skeletal and crystalline lens abnormalities.
Zanjo je značilno nastajanje strdkov v venah, šibkost kosti in nenormalnosti okostja in očesne leče.
BEFORE YOU TAKE CYSTADANE
KAJ MORATE VEDETI, PREDEN BOSTE VZELI ZDRAVILO CYSTADANE
Do not take Cystadane If you or your child are allergic (hypersensitive) to betaine.
Ne jemljite zdravila Cystadane če ste vi ali vaš otrok alergični na (preobčutljivi za) betaine.
Take special care with Cystadane If you notice side effects like headaches, vomiting or a change in your vision, please contact your doctor immediately, they could be signs of a brain oedema.
Bodite posebno pozorni pri uporabi zdravila Cystadane Če opazite neželene učinke, kot so glavoboli, bruhanje ali spremembe vida, se nemudoma obrnite na svojega zdravnika, ker bi to lahko bili znaki možganskega edema.
Your treatment with betaine may need to be interrupted.
Mogoče bo treba vaše zdravljenje z betainom prekiniti.
If you are treated with Cystadane and with an amino-acid mixture and if you need to take other medicines at the same time, leave 30 minutes between the intake (see “ taking other medicines”).
Če se zdravite z zdravilom Cystadane in z mešanico aminokislin in morate istočasno jemati še druga zdravila, druga zdravila vzemite šele pol ure ali več po tistem, ko vzamete Cystadane in mešanico aminokislin (glejte » jemanje drugih zdravil «).
Taking other medicines If you are taking amino-acid mixture or medicines such as vigabatrin or Gaba analogues, please tell your doctor as they might interact with your treatment with betaine.
Jemanje drugih zdravil Če jemljete mešanico aminokislin ali zdravila, kakršna sta vigabatrin ali zdravila z zdravilnimi učinkovinami, podobnimi GABA, to povejte zdravniku, ker bi ta zdravila lahko vplivala na vaše zdravljenje z betainom.
16 Pregnancy and breast-feeding Please inform your doctor if you are pregnant or breast-feeding.
16 Nosečnost in dojenje Če ste noseči ali če dojite, to povejte zdravniku.
Your doctor will decide if the medicine may be used during pregnancy and breast-feeding.
Vaš zdravnik bo odločil, ali smete med nosečnostjo in dojenjem jemati to zdravilo.
HOW TO TAKE CYSTADANE
KAKO JEMATI ZDRAVILO CYSTADANE
Always take Cystadane exactly as your doctor has told you.
Pri jemanju zdravila Cystadane natančno upoštevajte zdravnikova navodila.
The daily dose in adults, adolescents and children over 10 years of age is 6 g per day divided in 2 doses of 3 g per day.
Dnevni odmerek pri odraslih, mladostnikih in otrocih, starejših od 10 let, je 6 g na dan, razdeljenih v 2 odmerka po 3 g na dan.
In children less than 10 years of age, the daily dose will be calculated from the child’ s body weight by your doctor.
Pri otrocih, mlajših od 10 let, bo vaš zdravnik izračunal dnevni odmerek iz otrokove telesne mase.
You or your child will therefore need regular blood tests to determine the correct daily dose.
Vi ali vaš otrok boste zato potrebovali redne krvne preiskave za določanje pravilnega dnevnega odmerka.
The use of this medicine will be supervised by a physician experienced in the treatment of patients with homocystinuria.
Uporabo tega zdravila bo nadzoroval zdravnik, izkušen v zdravljenju bolnikov s homocistinurijo.
Cystadane should be taken orally (by mouth).
Zdravilo Cystadane morate jemati peroralno (skozi usta).
To measure the dose: • shake the bottle lightly before opening • take the correct measuring spoon:
Odmerek odmerite takole: • Rahlo pretresite steklenico, preden jo odprete. • Vzemite pravilno merilno žličko:
the small spoon measures 100 mg of betaine anhydrous powder; the middle size spoon measures 150 mg of betaine anhydrous powder; the large spoon measures 1 g of betaine anhydrous powder.
z malo žličko odmerite 100 mg betaina v obliki brezvodnega praška; s srednjo žličko odmerite 150 mg betaina v obliki brezvodnega praška; z veliko žličko odmerite 1 g betaina v obliki brezvodnega praška.
• pass the flat back of a knife over the top of the spoon • the powder left in the spoon is one spoonful • take the correct number of spoonfuls of powder from the bottle
• Vzemite zvrhano merilno žličko praška iz steklenice. • Potegnite čez merilno žličko z noževim rezilom. • V merilni žlički ostane za eno žličko praška. • Vzemite pravilno število žličk praška iz steklenice.
If you forget to take Cystadane:
Če ste pozabili vzeti zdravilo zdravilo Cystadane:
Like all medicines, Cystadane can cause side effects, although not everybody gets them.
Kot vsa zdravila ima lahko tudi zdravilo Cystadane neželene učinke, ki pa se ne pojavijo pri vseh bolnikih.
The following side effects were reported as follows: very common (more than 1 out of 10 persons), common (more than 1 out of 100 persons and less than 1 out of 10 persons), uncommon (more than 1 out of 1,000 persons and less than 1 out of 100 persons), rare (more than 1 out of 10,000 persons and less than 1 out of 1,000 persons), very rare (less than 1 out of 10,000 persons).
Poročali so o naslednjih neželenih učinkih: zelo pogosti (pri več kot 1 od 10 oseb), pogosti (pri več kot 1 od 100 oseb in pri manj kot 1 od 10 oseb), občasni (pri več kot 1 od 1. 000 oseb in pri manj kot 1 od 100 oseb), redki (pri več kot 1 od 10. 000 oseb in pri manj kot 1 od 1. 000 oseb), zelo redki (pri manj kot 1 od 10. 000 oseb).
Very common: blood methionine increased.
17 Zelo pogost: zvišan metionin v krvi.
17 Uncommon: decreased appetite, agitation, depression, irritability, personality disorder, sleep disturbance, oedema of the brain (see heading “Take special care with Cystadane” in section 2), dental disorders, diarrhoea, inflammation of the tongue, nausea, stomach discomfort, vomiting, hair loss, hives, skin odour abnormality and urinary incontinence.
Občasni: zmanjšan tek, hud nemir, depresivnost, razdražljivost, osebnostna motnja, motnja spanja, možganski edem (glejte podnaslov “ Bodite posebno pozorni pri uporabi zdravila Cystadane ” v poglavju 2), zobne bolezni, driska, vnetje jezika, siljenje na bruhanje, bolečina v želodcu, bruhanje, izpadanje las, koprivnica, nenormalen vonj kože in nezmožnost zadrževanja urina.
Since some of these side effects are serious, ask your doctor to explain their warning signs.
Ker so nekateri od teh neželenih učinkov resni, prosite zdravnika, naj vam razloži njihove svarilne znake.
If any of the side effects gets serious, or if you notice any other side effects not listed in this leaflet, please tell your doctor or pharmacist.
Če katerikoli neželeni učinek postane resen ali če opazite katerikoli drug neželeni učinek, ki ni omenjen v tem navodilu, obvestite svojega zdravnika ali farmacevta.
HOW TO STORE CYSTADANE
SHRANJEVANJE ZDRAVILA CYSTADANE
Do not use Cystadane after the expiry date which is stated on the bottle label and the carton after EXP.
Zdravila Cystadane ne smete uporabljati po datumu izteka roka uporabnosti, ki je naveden na nalepki steklenice in škatli za EXP.
After the first opening of the bottle, the product should be used within 1 month.
Zdravilo morate porabiti v 1 mesecu od takrat, ko steklenico prvič odprete.
Medicine should not be disposed of via wastewater or household waste.
Zdravila ne smete odvreči v odpadne vode ali med gospodinjske odpadke.
What Cystadane contains
Kaj vsebuje zdravilo Cystadane
- The active substance is betaine anhydrous.
- Zdravilna učinkovina je brezvodni betain.
1 g of oral powder contains 1 g of betaine anhydrous.
1 g peroralnega praška vsebuje 1 g brezvodnega
Three measuring spoons dispense 1 g, 150 mg and 100 mg of betaine anhydrous.
S tremi merilnimi žličkami lahko odmerite 1 g, 150 mg ali 100 mg brezvodnega betaina.
- There is no other ingredient.
- Zdravilo ne vsebuje pomožnih snovi.
What Cystadane looks like and contents of the pack Cystadane is a white crystalline oral powder.
Izgled zdravila Cystadane in vsebina pakiranja Zdravilo Cystadane je bel kristalen peroralni prašek.
It is presented in bottles with child resistant closures.
Na voljo je v steklenicah s pokrovčkom z za otroke varno zaporko.
Each bottle contains 180 g of betaine anhydrous powder.
Vsaka steklenica vsebuje 180 g betaina v obliki brezvodnega praška.
Three measuring spoons are enclosed in the carton to assist you in measuring out the dose prescribed by your doctor.
V škatlici so priložene tri merilne žličke, ki vam pomagajo odmeriti odmerek, ki vam ga je predpisal zdravnik.
Marketing Authorisation Holder and Manufacturer Orphan Europe SARL Immeuble “Le Wilson” F - 92058 Paris-La Défense France
Imetnik dovoljenja za promet z zdravilom in izdelovalec Orphan Europe SARL Immeuble “ Le Wilson ” F - 92058 Paris- La Défense Francija
18 For any information about this medicinal product, please contact the local representative of the Marketing Authorisation Holder.
Za vse morebitne nadaljnje informacije o tem zdravilu se lahko obrnete na predstavništvo imetnika dovoljenja za promet z zdravilom.
Belgique/België/Belgien Orphan Europe Benelux Koning Albert I, Iaan 48 bus 3 B-1780 Wemmel (Brussels) Tél/Tel: +32 2 46101 36
18 Belgique/ België/ Belgien Orphan Europe Benelux Koning Albert I, Iaan 48 bus 3 B- 1780 Wemmel (Brussels) Tél/ Tel: +32 2 46101 36
Gran via de les Cortes Catalanes, 649 Despacho, n°1 E-08010 Barcelona Espanha Tel: +34 93 342 51 20
Espanha Tel: +34 93 342 51 20
If you need more information about your medical condition or your treatment, read the Package Leaflet (also part of the EPAR) or contact your doctor or pharmacist.
Če potrebujete več informacij o vašem zdravstvenem stanju ali zdravljenju, preberite Navodilo za uporabo (ki je prav tako del EPAR) ali se posvetujete z vašim zdravnikom ali s farmacevtom.
What is CYSTAGON?
Kaj je zdravilo CYSTAGON?
CYSTAGON is a medicine containing the active ingredient cysteamine bitartrate (also known as mercaptamine bitartrate).
CYSTAGON je zdravilo, ki vsebuje zdravilno učinkovino cisteamin.
It is available as capsules (50 and 150 mg).
Na voljo je v 50 in 150 mg kapsulah.
What is CYSTAGON used for?
Za kaj se zdravilo CYSTAGON uporablja?
CYSTAGON is used to manage patients who have nephropathic (kidney) cystinosis.
Zdravilo CYSTAGON se uporablja za zdravljenje bolnikov, ki imajo nefropatsko (ledvično) cistinozo.
Cystinosis is a rare inherited disorder where cystine builds up within body cells, preventing them from working correctly.
Cistinoza je redka dedna bolezen, pri kateri se cistin kopiči znotraj telesnih celic, kar preprečuje njihovo pravilno delovanje.
This leads to kidney problems and spreads to other parts of the body, including the eyes, some glands (thyroid, gonads, pancreas), the central nervous system, muscles and the liver.
Zaradi tega se pojavijo težave z ledvicami, ki se razširijo tudi na druge dele telesa, med drugim na oči, nekatere žleze (žlezo ščitnico, spolne žleze, trebušno slinavko), osrednji živčni sistem, mišice in jetra.
Stunted growth is a symptom of the condition.
Simptom te bolezni je okrnjena rast.
How is CYSTAGON used?
Kako se zdravilo CYSTAGON uporablja?
CYSTAGON treatment should be started by a doctor who has experience in the treatment of cystinosis.
Zdravljenje z zdravilom CYSTAGON mora začeti zdravnik, ki ima izkušnje z zdravljenjem cistinoze.
Cystine levels in white blood cells should be monitored to adjust the dose.
Zaradi prilagajanja odmerka je treba spremljati stopnjo cistina v belih krvničkah.
For children up to the age of 12 years, the recommended dose is 1.30 g/ m2/ day divided into 4 daily doses.
Pri otrocih do 12. leta starosti znaša priporočeni odmerek 1, 30 g/ m2/ dan, porazdeljen na štiri odmerke preko dneva.
For patients over the age of 12 and over 50 kg in weight, the recommended dose is 2 g/ day, divided into 4 daily doses.
Pri bolnikih, starejših od 12 let in s telesno maso nad 50 kg, znaša priporočeni odmerek 2 g/ dan, porazdeljen na štiri odmerke preko dneva.
Starting doses should be one quarter to one sixth of the final expected doses.
Začetni odmerki znašajo 1/ 4 do 1/ 6 končnega pričakovanega odmerka.
The starting dose should be increased gradually over 4-6 weeks.
Začetni odmerek se povečuje postopoma skozi 4 do 6 tednov.
How does CYSTAGON work?
Kako zdravilo CYSTAGON deluje?
CYSTAGON reacts with cystine to form cysteine and a cysteine-cysteamine salt (mixed disulfide).
CYSTAGON v reakciji s cistinom tvori cistinovo in cistin- cisteaminovo sol (mešani disulfid).
This salt is then removed from the cells by a system that transports another amino acid (lysine).
Sol nato iz celic odstrani sistem, ki prenaša drugo aminokislino (lizin).
The amount of cystine in the organs is reduced and this limits the damage to these organs.
Količina cistina v organih se zmanjša, zaradi česar je manj okvar teh organov.
How has CYSTAGON been studied?
Kako je bilo zdravilo CYSTAGON raziskano?
CYSTAGON has been investigated in three clinical studies including 234 patients over a 12-year period.
Zdravilo CYSTAGON so raziskovali v treh kliničnih študijah, v katerih je sodelovalo 234 bolnikov v obdobju 12 let.
These studies included children and newly recruited patients in whom two different doses were tested.
V študijah so sodelovali otroci in novi bolniki, na katerih so testirali dva različna odmerka.
Because the disease is very serious, it was not possible for ethical reasons to compare CYSTAGON directly to a dummy treatment.
Namesto tega je bila narejena primerjava s skupino bolnikov, ki so prejemali zdravilo brez zdravilne učinkovine kot del druge, nepovezane študije.
The comparison was done instead with a group of
V študijah so preučevali delovanje ledvic, stopnjo preživetja in rast pri bolnikih.
(44-20) 74 18 84 00 Fax (44-20) 74 18 84 16 E-mail: mail@emea. europa. eu http: / /www. emea. europa. eu ©EMEA 2007 Reproduction and/ or distribution of this document is authorised for non commercial purposes only provided the EMEA is acknowledged patients who had been treated with a dummy treatment as part of another, unrelated trial.
(44- 20) 74 18 84 00 Fax (44- 20) 74 18 74 18 84 16 E- mail: mail@ emea. europa. eu http: // www. emea. europa. eu ©EMEA 2007 Reproduction and/ or distribution of this document is authorised for non commercial purposes only provided the EMEA is acknowledged Kakšne koristi je zdravilo CYSTAGON izkazalo med raziskavami?
The three studies have shown that CYSTAGON delays kidney problems, and the need for dialysis or renal transplantation when treatment is started at an early age in patients with good kidney function.
Tri študije so pokazale, da zdravilo CYSTAGON odloži težave z ledvicami in potrebo po dializi ali presaditvi ledvic, če se zdravljenje prične v zgodnji starosti pri bolnikih z dobrim delovanjem ledvic.
What is the risk associated with CYSTAGON?
Kakšna tveganja so povezana z zdravilom CYSTAGON?
The side effects of CYSTAGON are mainly connected with the digestive system.
Neželeni stranski učinki zdravila CYSTAGON so večinoma povezani s prebavnim sistemom.
The most common side effects (seen in more than 1 patient in 10) are anorexia (loss of appetite), vomiting, nausea (feeling sick), diarrhoea, lethargy (lack of energy) and pyrexia (fever).
Najpogostejši stranski učinki (opaženi pri več kot 1 od 10 bolnikov) so anoreksija (izguba teka), bruhanje, navzeja (občutek slabosti), driska, letargija (pomanjkanje energije) in pireksija (povišana telesna temperatura).
For the full list of all side effects reported with CYSTAGON, see the Package Leaflet.
Za celoten seznam neželenih učinkov, o katerih so poročali pri uporabi zdravila CYSTAGON, glejte Navodilo za uporabo.
CYSTAGON should not be used in people who may be hypersensitive (allergic) to cysteamine or any of the other ingredients, or to penicillamine.
Zdravila CYSTAGON ne smejo uporabljati osebe, ki utegnejo biti preobčutljive za (alergične na) cisteamin, na katero koli drugo sestavino ali na penicilamin.
It should also not be used in women who are breast- feeding, or who are or may be pregnant (particularly during the first three months) unless it is clearly necessary.
Zdravila se ne sme uporabljati pri ženskah, ki dojijo, ki so ali bi utegnile biti noseče (zlasti v prvih treh mesecih) razen, kadar je nujno potrebno.
Why has CYSTAGON been approved?
Zakaj je bilo zdravilo CYSTAGON odobreno?
Cystinosis is a rare, fatal disease, and CYSTAGON is considered a useful medicine for this disease, for which no alternative treatments are available.
Cistinoza je redka, smrtna bolezen in zdravilo CYSTAGON velja kot koristno zdravilo za to bolezen, za katero ne obstaja nobeno drugo alternativno zdravljenje.
The Committee for Medicinal products for Human Use (CHMP) decided that CYSTAGON’ s benefits are greater than its risks and recommended that CYSTAGON be given marketing authorisation.
Odbor za zdravila za uporabo v humani medicini (CHMP) je odločil, da so koristi zdravila CYSTAGON večje od z njim povezanih tveganj, zato je zanj priporočil odobritev dovoljenja za promet.
CYSTAGON was originally authorised under "Exceptional Circumstances", because, as the disease is rare, limited information was available at the time of approval.
Dovoljenje za promet z zdravilom CYSTAGON je bilo sprva odobreno v „ izjemnih okoliščinah “, saj so bile zaradi redkosti bolezni ob odobritvi na voljo le omejene informacije.
As the company had supplied the additional information requested, the "Exceptional Circumstances" ended on 17 April 2007.
Podjetje je predložilo dodatne zahtevane informacije, zato so „ izjemne okoliščine “ prenehale 17. aprila 2007.
Other information about CYSTAGON:
Druge informacije o zdravilu CYSTAGON:
The European Commission granted a marketing authorisation valid throughout the European Union for CYSTAGON to Orphan Europe on 23 June 1997.
Evropska komisije je dovoljenje za promet z zdravilom CYSTAGON, veljavno po vsej Evropski uniji, odobrila družbi Orphan Europe dne 23. junija 1997.
The marketing authorisation was renewed on 23 June 2002 and on 23 June 2007.
Dovoljenje je bilo podaljšano dne 23. junija 2002 in 23. junija 2007.
The full EPAR for CYSTAGON can be found here.
Celotno Evropsko javno poročilo o oceni zdravila (EPAR) je na voljo tukaj.
This summary was last updated in 06-2007.
Ta povzetek je bil nazadnje posodobljen 06- 2007.
Each hard capsule contains 50 mg of cysteamine (as mercaptamine bitartrate).
Ena trda kapsula vsebuje 50 mg cisteamina (v obliki merkaptaminijevega bitartrata).
4.1 Therapeutic indication
4. 1 Terapevtske indikacije
Cysteamine reduces cystine accumulation in some cells (e. g. leukocytes, muscle and liver cells) of nephropathic cystinosis patients and, when treatment is started early, it delays the development of renal failure.
Cisteamin zmanjšuje nabiranje cistina v nekaterih celicah (npr. levkocitih, mišičnih in jetrnih celicah) pri bolnikih s cistinozo in, če se zdravljenje začne dovolj zgodaj, zavira nastanek ledvične odpovedi.
The goal of therapy is to keep leucocyte cystine levels below 1 nmol hemicystine/ mg protein.
Cilj zdravljenja je vzdrževati koncentracijo cistina v levkocitih pod vrednostjo 1 nmol hemicistina/ mg beljakovin.
The WBC levels should be measured 5 to 6 hours after dosing and should be checked frequently when initiating therapy (e. g. monthly) and every 3-4 months when on a stable dose.
Koncentracijo v L je treba določati 5 do 6 ur po odmerjanju ter jo v začetnem obdobju zdravljenja pogosto preverjati (npr. enkrat mesečno), ob že ustaljenem odmerjanju pa vsake 3 – 4 mesece.
• For children up to age 12 years, CYSTAGON dosing should be on the basis of body surface area (g/ m2/ day).
• Pri otrocih do 12. leta starosti je treba CYSTAGON odmerjati na podlagi telesne površine (g/ m2/ dan).
The recommended dose is 1.30 g/ m2/ day of the free base divided four times daily. • For patients over age 12 and over 50 kg weight, the recommended CYSTAGON dose is 2 g/ day, divided four times daily.
Priporočeni odmerek je 1, 30 g/ m2/ dan proste baze, porazdeljeno v 4 odmerke na dan. • Pri bolnikih starejših od 12 let in telesno maso nad 50 kg znaša priporočeni odmerek CYSTAGONa 2 g/ dan, porazdeljeno v 4 odmerke na dan.