qanastek/EMEA-V3 huggingface dataset:
The leucocyte cystine level reaches its minimum (mean (± sd) value:
Il- livell ta ’ cystine fil- lewkoċiti jilħaq l- aktar livell baxx tiegħu (valur medju (± sd):
1.8 ± 0.8 hours) slightly later than the peak plasma cysteamine concentration (mean (± sd) value:
1. 8 ± 0. 8 siegħat) ftit aktar tard minn l- ogħla konċentrazzjoni ta ’ cysteamine fil- plażma (valur medju (± sd):
1.4 ± 0.4 hours) and returns to its baseline level as the plasma cysteamine concentration decreases at 6 hours post-dose.
1. 4 ± 0. 4 siegħat) u jerġa ’ jiġi lura fil- livell bażi ta 'referenza hekk kif il- konċentrazzjoni ta' cysteamine fil - plażma tonqos fis- 6 siegħat wara d- doża.
In one clinical study, baseline white cell cystine levels were 3.73 (range 0.13 to 19.8) nmol hemicystine/ mg protein and were maintained close to 1 nmol hemicystine/ mg protein with a cysteamine dose range of 1.3 to 1.95 g/ m2/ day.
Fi studju kliniku, il- livelli bażi ta 'referenza ta ’ cystine fiċ- ċelloli bojod kienu ta ’ 3. 73 (medda 0. 13 sa 19. 8) nmol hemicystine/ mg ta ’ proteina u nżammu viċin il- 1 nmol hemicystine/ mg ta ’ proteina permezz ta ’ firxa fid- doża ta ’ cysteamine minn 1. 3 sa 1. 95 g/ m2/ jum.
An earlier study treated 94 children with nephropathic cystinosis with increasing doses of cysteamine to attain white cell cystine levels of less than 2 nmol hemicystine/ mg protein 5 to 6 hours post-dose, and compared their outcome with an historical control group of 17 children treated with placebo.
Studju li sar qabel ġew ikkurati 94 tifel u tifla b’ ċistinożi nefropatika b’ dożi jiżdiedu ta ’ cysteamine biex jintlaħqu livelli ta ’ cystine fiċ- ċelloli bojod ta ’ anqas minn 2 nmol hemicystine/ mg ta ’ proteina 5 sa 6 siegħat wara t- teħid tad- doża u mqabbel ma 'riżultat miksub fuq grupp ta' 17- il tifel u tifla bi storja li ngħataw trattament permezz ta 'plaċebo bħala kontroll.
The principal efficacy measurements were serum creatinine and calculated creatinine clearance and growth (height).
Il- kejl prinċipali ta ’ l- effikaċja kienu l - krejatinina fis- serum u l- kalkolu tat- tneħħija ta 'krejatinina u t- tkabbir (tul).
The mean white cell cystine level attained during treatment was 1.7 + 0.2 nmol hemicystine/ mg protein.
Il- medja tal- għadd ta 'cystine fiċ- ċelloli bojod miksuba matul it- trattament kienet ta ’ 1. 7 + 0. 2 nmol hemicystine/ mg ta ’ proteina.
Among cysteamine patients, glomerular function was maintained over time.
Fost il- pazjenti li ngħataw cysteamine, il- funzjoni glomerulari nżammet tul il- perijodu.
Placebo treated patients, in contrast, experienced a gradual rise in serum creatinine.
Min - naħa l- oħra, pazjenti trattati permezz ta 'plaċebo, kellhom żjieda gradwali ta' krejatinina fis- serum.
However, growth velocity did not
Il - pazjenti li ngħataw trattament żammew iż- żjieda fit- tul meta mqabbla ma ’ dawk mhux ikkurati.
5 increase enough to allow patients to catch up the normal for their age.
Madankollu, ir- rata ta 'tkabbir ma żdiedetx biżżejjed biex tippermetti li l- pazjenti jilħqu t- tul normali għall- età tagħhom.
Renal tubular function was not affected by treatment.
Il- funzjoni tubulari renali ma kienetx affettwata mit- trattament.
Two other studies have shown similar results.
Żewġ studji oħra wrew riżultati bħal dawn.
In all studies, patient response was better when treatment was started at an early age with good renal function.
Fl- istudji kollha, ir- rispons mill- pazjenti kien aħjar meta t- trattament inbeda f’ età bikrija b’ funzjoni renali tajba.
Following a single oral dose of cysteamine bitartrate equivalent to 1.05 g of cysteamine free base in healthy volunteers, the mean (± sd) values for the time to peak and peak plasma concentration are 1.4 (± 0.5) hours and 4.0 (± 1.0) µg/ ml, respectively.
Wara doża orali waħda ta ’ cysteamine bitartrate ekwivalenti għal 1. 05 g ta ’ bażi ħieles ta 'cysteamine fuq voluntiera b' saħħithom, il- valuri medji (±sd) għall- ħin biex tintlaħaq l- ogħla konċentrazzjoni u l - ogħla konċentrazzjoni tal- plażma huwa ta ’ 1. 4 (± 0. 5) siegħat u 4. 0 (± 1. 0) µg/ ml, rispettivament.
In patients at steady state, these values are 1.4 (± 0.4) hours and 2.6 (± 0.9) µg/ ml, respectively, after a dose ranging from 225 to 550 mg.
F’ pazjenti fi stat fiss, dawn il- valuri huma ta '1. 4 (± 0. 4) siegħat u 2. 6 (± 0. 9) µg/ ml, rispettivament, wara doża li tvarja minn 225 sa 550 mg.
Cysteamine bitartrate (CYSTAGON) is bioequivalent to cysteamine hydrochloride and phosphocysteamine.
Cysteamine bitartrate (CYSTAGON) hu bioekwivalenti għal cysteamine hydrochloride u phosphocysteamine.
The in vitro plasma protein binding of cysteamine, which is mostly to albumin, is independent of plasma drug concentration over the therapeutic range, with a mean (± sd) value of 54.1% (± 1.5).
L- irbit ta ’ cysteamine mal- proteini tad- demm in vitro, prinċipalment ma ’ albumin, huwa indipendenti mill- konċentrazzjoni tal- mediċina fil- plażma fuq il- firxa terapewtika, b’ valur medju (± sd) ta ’ 54. 1% (± 1. 5).
The plasma protein binding in patients at steady state is similar:
L- ammont li jintrabat mal- proteini tad- demm f’ pazjenti fi stat fiss hu simili:
53.1% (± 3.6) and 51.1% (± 4.5) at 1.5 and 6 hours post-dose, respectively.
53. 1% (± 3. 6) u 51. 1% (± 4. 5) f’ 1. 5 u 6 siegħat wara d- doża, rispettivament.
In a pharmacokinetic study performed in 24 healthy volunteers for 24 hours, the mean estimate (± sd) for the terminal half-life of elimination was 4.8 (± 1.8) hours.
Fi studju farmakokinetiku mwettaq fuq 24 voluntiera b’ saħħithom għal 24 siegħa, l- istima medja (± sd) għall- half- life terminali ta ’ l- eliminazzjoni kienet ta ’ 4. 8 (± 1. 8) siegħat.
The elimination of unchanged cysteamine in the urine has been shown to range between 0.3% and 1.7% of the total daily dose in four patients; the bulk of cysteamine is excreted as sulphate.
F’ erba ’ pazjenti nstab li l- eleminazzjoni ta ’ cysteamine mhux mibdula fl- awrina tvarja bejn 0. 3% u 1. 7% tad- doża totali; il- parti l- kbira ta 'cysteamine hi mneħħija bħala sulphate.
Very limited data suggest that cysteamine pharmacokinetic parameters may not be significantly modified in patients with mild to moderate renal insufficiency.
6 Informazzjoni limitata ħafna turi li l- parametri farmakokinetiċi ta 'cysteamine m’ għandhomx jinbidlu b’ mod sinifikanti f’ pazjenti b’ insuffiċjenza renali minn ħafifa sa moderata.
No information is available for patients with severe renal insufficiency.
M’ hemm l- ebda informazzjoni dwar pazjenti b’ insuffiċjenza renali gravi.
Genotoxicity studies have been performed: although in published studies using cysteamine, induction of chromosome aberrations in cultured eukaryotic cell lines has been reported, specific studies with cysteamine bitartrate did not show any mutagenic effects in the Ames test or any clastogenic effect in the mouse micronucleus test.
Saru studji dwar il- ġenotossiċità: għalkemm fi studji ppubblikati dwar l- użu ta ’ cysteamine, kienet irrapportat żjieda ta ’ aberazzjoni tal- kromożoma f’ razza ta 'ċelloli ewkarjotiċi mkabbra, studji speċifiċi b’ cysteamine bitartrate ma wrew l- ebda effetti mutaġeniċi fit- test ta ’ Ames u l- ebda effett klastoġeniku fit- test fuq il- mikronukleju tal- ġurdien.
Reproduction studies showed embryofoetotoxic effects (resorptions and post-implantation losses) in rats at the 100 mg/ kg/ day dose level and in rabbits receiving cysteamine 50 mg/ kg/ day.
Studji fuq ir- riproduzzjoni wrew effetti tossiċi fuq l- embriju u l- fetu (assorbiment mill- ġdid u telf ta ’ wara l- impjantazzjoni) fil- firien f’ livell ta ’ doża ta ’ 100 mg/ kg/ jum u fi fniek li rċevew 50 mg/ kg/ jum ta ’ cysteamine.
Teratogenic effects have been described in rats when cysteamine is administered over the period of organogenesis at a dose of 100 mg/ kg/ day.
Effetti teratoġeniċi ġew irrapportati fil- firien meta cysteamine ġie mogħti fuq il - perijodu ta ’ organoġeneżi b’ doża ta ’ 100 mg/ kg/ jum.
This is equivalent to 0.6 g/ m2/ day in the rat, which is less than half the recommended clinical maintenance dose of cysteamine, i. e.
Dan hu ekwivalenti għal 0. 6 g/ m2/ jum fil- far, li hu anqas min- nofs tad- doża ta ’ manteniment kliniku rrakkomandata ta 'cysteamine, i. e.
1.30 g/ m2/ day.
1. 30 g / m2/ jum.
A reduction of fertility was observed in rats at 375 mg/ kg/ day, a dose at which body weight gain was retarded.
Kien osservat tnaqqis fil- fertilità tal- firien b’ 375 mg/ kg/ jum, doża li biha iddewwem iż- żjieda tal- piż tal- ġisem.
At this dose, weight gain and survival of the offspring during lactation was also reduced.
B’ din id- doża, kien hemm tnaqqis ukoll fiż- żjieda fil- piż u fin- numru ta ’ frieħ li baqgħu ħajjin waqt it- treddigħ.
High doses of cysteamine impair the ability of lactating mothers to feed their pups.
Dożi għolja ta ’ cysteamine inaqqsu l- kapaċità ta ’ ommijiet li jreddgħu lill- frieħ tagħhom.
Single doses of the drug inhibit prolactin secretion in animals.
Dożi singoli tal- mediċini jwaqqfu it- tnixxija ta 'prolactin fl- annimali.
Administration of cysteamine in neonate rats induced cataracts.
L- għotja ta ’ cysteamine fil- firien li għadhom kif jitwieldu kkaġunalhom il- katarretti.
High doses of cysteamine, either by oral or parenteral routes, produce duodenal ulcers in rats and mice but not in monkeys.
Dożi qawwija ta ’ cysteamine, kemm jekk mogħtija fil- ħalq u kemm jekk mogħtija b’ injezzjoni, ipproduċew ulċeri duwodenali fil- firien u fil- ġrieden iżda mhux fix- xadini.
Experimental administration of the drug causes depletion of somatostatin in several animal species.
Għotja sperimentali tal - prodott mediċinali wasslet għal nuqqas ta 'somatostatina f’ diversi speċi ta ’ annimali.
The consequence of this for the clinical use of the drug is unknown.
Il- konsegwenza ta ’ dan għall- użu kliniku ta ’ dan il- mediċinali mhiex magħrufa.
No carcinogenic studies have been conducted with CYSTAGON.
Ma saru l- ebda studji karċinoġeniċi b’ CYSTAGON.
Capsule content: microcrystalline cellulose, pregelatinized starch, magnesium stearate/ sodium lauryl sulfate, colloidal silicon dioxide, croscarmellose sodium Capsule shell: gelatin, titanium dioxide,
Il- kapsula tikkonsisti minn: microcrystalline cellulose, pregelatinized starch, magnesium stearate/ sodium lauryl sulfate, colloidal silicon dioxide, croscarmellose sodium Il- qoxra tal- kapsula tikkonsisti minn: gelatin, titanium dioxide,
black ink on hard capsules containing E172
Il- linka sewda fuq kapsuli iebsa fiha E172.
Do not store above 25°C.
7 Taħżinx f’ temperatura `l fuq minn 25°C.
Keep the container tightly closed in order to protect from light and moisture.
Żomm il- kontenitur magħluq sewwa sabiex tilqa ’ mid- dawl u l- umdità.
HDPE bottles of 100 and 500 hard capsules.
Fliexken HDPE ta ’ 100 u 500 kapsuli iebsin.
A desiccant unit containing black activated carbon and silica gel granules is included in the bottle.
Desikkant tal- karbonju iswed attivat u granijiet tas- silika gel huma inklużi fil- flixkun.
6.6 Special precautions for disposal and other handling
6. 6 Prekawzjonijiet speċjali li għandhom jittieħdu meta jintrema
Orphan Europe SARL Immeuble “ Le Wilson” F-92058 Paris La Défense France
Orphan Europe SARL Immeuble “ Le Wilson ” F- 92058 Paris La Défense Franza
EU/ 1/ 97/ 039/ 001 (100 hard capsules per bottle), EU/ 1/ 97/ 039/ 002 (500 hard capsules per bottle).
EU/ 1/ 97/ 039/ 001 (100 kapsula iebsa kull flixkun), EU/ 1/ 97/ 039/ 002 (500 kapsula iebsa kull flixkun).
Date of latest renewal:
23 ta ’ Ġunju 1997 Data ta ’ l- aħħar tiġdid:
23 June 2007.
23 ta ’ Ġunju 2007
Detailed information on this product is available on the website of the European Medicines Agency (EMEA) http: / /www. emea. europa. eu
Informazzjoni ddettaljata dwar dan il- prodott tinsab fuq il- website ta ’ l- Aġenzija Ewropeja dwar il - mediċini (EMEA) http: // www. emea. europa. eu /
CYSTAGON 150 mg hard capsules
CYSTAGON 150 mg Kapsuli Iebsin
Each hard capsule contains 150 mg of cysteamine (as mercaptamine bitartrate) For a full list of excipients, see section 6.1.
Kull kapsula iebsa fiha 150 mg ta’ cysteamine bitartrate (bħala mercaptamine bitartrate.
Hard Capsule White, opaque hard capsules with CYSTAGON 150 on the body and MYLAN on the cap.
Kapsula Iebsa Kapsuli iebsin bojod, opaki b’ CYSTAGON 150 fuq il- korp u MYLAN fuq ir- ras.
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.
Cysteamine inaqqas l - akkumulazzjoni ta ’ cystine f’ xi ċelloli (eż. lewkoċiti, ċelloli tal- muskolu u tal- fwied) f’ pazjenti b’ ċistinożi nefropatika u, meta t- trattament jinbeda fi żmien bikri, dan idewwem l- iżvilupp ta ’ insuffiċjenza renali.
CYSTAGON treatment should be initiated under the supervision of a physician experienced in the treatment of cystinosis.
It- trattement b’ CYSTAGON għandu jinbeda taħt is- superviżjoni ta ’ tabib b’ esperjenza fit- trattament taċ- ċistinożi.
The goal of therapy is to keep leucocyte cystine levels below 1 nmol hemicystine/ mg protein.
L- iskop ta 'terapija hu li l- livell ta’ cystine fil- lewkoċiti jinżamm taħt il- 1 nmol ta ’ hemicystine/ mg ta ’ proteina.
White blood cell (WBC) cystine levels should therefore be monitored to adjust the dose.
L- ammont ta 'cystine fiċ- ċelloli bojod tad- demm għandu għalhekk jiġi osservat sabiex tkun tista ’ tiġi mibdula d- doża.
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.
L- ammont ta 'ċelloli bojod tad- demm għandhu jiġi mkejjel kull 5 sa 6 sigħat wara l- għoti tad- doża u għandu ikun ikkontrollat regolarment wara l- bidu tat- terapija (eż. kull xahar) u kull 3- 4 xhur meta d- doża tkun stabbli.
• For children up to age 12 years, CYSTAGON dosing should be on the basis of body surface area (g/ m2/ day).
• Għat- tfal li għandhom sa 12- il sena id- doża ta 'CYSTAGON għandha tingħata skond l- erja superfiċjali tal- ġisem (g/ m2/ kuljum).
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.
Id- doża rrakkomandata hi ta '1. 30 g/ m2/ kuljum tal- bażi libera mqassma f' erba' darbiet kuljum. • Għal pazjenti li għandhom aktar minn 12- il sena u għandhom piż ta 'aktar minn 50 kg id- doża rrakkomandata ta' CYSTAGON hi ta '2 g/ kuljum, li titqassam erba' darbiet kuljum.
Starting doses should be 1/ 4 to 1/ 6 of the expected maintenance dose, increased gradually over 4- 6 weeks to avoid intolerance.
Id- dożi inizjali għandhom ikunu ta ’ 1/ 4 sa 1/ 6 tad- doża ta ’ mantiment li mistennija tingħata, u tiżdied gradwalment fuq perijodu ta '4- 6 ġimgħat sabiex tiġi evitata l- intolleranza għall- prodott medicinali.
The dose should be raised if there is adequate tolerance and the leucocyte cystine level remains > 1 nmol hemicystine/ mg protein.
Id - doża għandha tiżdied jekk it- tolleranza tkun adegwata u l- livell ta ’ cystine fil- lewkoċiti jibqa ’ > 1 nmol hemicystine/ mg ta ’ proteina.
The maximum dose of CYSTAGON used in clinical trials was 1.95 g/ m2/ day.
L- ogħla doża ta ’ CYSTAGON użata fi provi kliniċi kienet ta ’ 1, 95 g/ m2/ jum.
Digestive tolerance of cysteamine is improved when the medicinal product is taken just after or with food.
It- tolleranza diġestiva ta ’ cysteamine hi aħjar meta l- prodott mediċinali jittieħed ma 'l- ikel jew eżatt wara jew waqt l- ikel.
9 Patients on dialysis or post-transplantation:
9 b’ bażi ta ’ lamtu huma xierqa biex jitħalltu ma ’ dan it- trab.
Dose adjustment is not normally required; however, leucocyte cystine levels should be monitored.
Ġeneralment mhux meħtieġ tibdil fid- doża, iżda għandha ssir osservazzjoni tal- livelli ta ’ cystine fil - lewkoċite.
CYSTAGON should not be used during pregnancy, particularly during the first trimester, unless clearly necessary (see section 4.6 and section 5.3), as it is teratogenic in animals.
CYSTAGON m’ għandux jittieħed waqt it- tqala, b' mod partikolari matul l- ewwel trimestru, sakemm ma jkunx meħtieġ bl- aktar mod evidenti (ara sezzjoni 4. 6 u sezzjoni 5. 3), minħabba li nstab li hu teratoġeniku fuq fl- annimali.
4.4 Special warnings and special precautions for use
CYSTAGON huwa kontra- indikat f ’ pazjenti li żviluppaw sensittività eċċessiva għal cysteamine jew peniċillamina.
CYSTAGON therapy must be initiated promptly after confirmation of the diagnosis of nephropathic cystinosis to achieve maximum benefit.
4. 4 Twissijiet speċjali u prekawzjonijiet speċjali għall- użu
These skin lesions were associated with skin striae and bone lesions first seen during an X-ray examination.
Dawn il- leżjonijiet fil- ġilda huma assoċjati ma ’ strije fil- ġilda u leżjonijiet fl- għadam li ntwerew l- ewwel darba waqt eżamijiet tar- raġġi X.
It is therefore recommended to monitor regularly skin and to consider X-ray examinations of the bone as necessary.
Għalhekk huwa rrakkomandat li wieħed jimmonitorja regolarment il- ġilda u li tikkonsidra eżamijiet tar- raġġi X għall- għadam bħala neċessarji.
Oral cysteamine has not been shown to prevent eye deposition of cystine crystals.
Instab li cysteamine meħud mill- ħalq ma waqqafx id- depożitu ta’ kristalli ta ’ cystine fil- għajn.
Therefore, where cysteamine ophthalmic solution is used for that purpose, its usage should continue.
Għalhekk, fejn tintuża soluzzjoni għal - għajn għal dak l- iskop, l- użu tagħha għandu jitkompla.
Intact CYSTAGON hard capsules should not be administered to children under the age of approximately 6 years due to risk of aspiration (see section 4.2).
Kapsuli iebsin ta ’ CYSTAGON shah m’ għandhomx jiġu mogħtija lit- tfal ta ’ taħt l- età ta ’ madwar 6 snin minħabba r- riskju ta ’ aspirazzjoni (ara sezzjoni 4. 2).
No interaction studies have been performed Interactions with other medicines have not been studied.
10 Ma sarux studji dwar l- effetti ta ’ mediċini oħrajn jew ta ’ affarijiet oħra fuq l- effett farmaċewtiku tal - prodott.
CYSTAGON can be administered with electrolyte and mineral replacements necessary for management of the Fanconi syndrome as well as vitamin D and thyroid hormones.
CYSTAGON jista ’ jiġi mogħti ma ’ elektroliti u sustanzi li jingħataw bħala sostitut ta ’ minerali meħtieġa għall- immaniġġar tas- sindromu ta ’ Fanconi kif ukoll ma ’ vitamina D u ormoni ta 'tirojde.
Indomethacin and CYSTAGON have been used simultaneously in some patients.
Indomethacin u CYSTAGON intużaw flimkien f ’ xi pazjenti.
10 4.6 Pregnancy and lactation
4. 6 Tqala u Treddigħ
There are no adequate data from the use of cysteamine bitartrate in pregnant women.
M’ hemmx tagħrif adegwat dwar l- użu ta ’ cysteamine bitartrate fuq nisa tqal.
Studies in animals have shown reproductive toxicity, including teratogenesis (see section 5.3).
Studji fuq annimali wrew tossiċità riproduttiva, inkluż teratoġenesi (ara sezzjoni 5. 3).
If a pregnancy is diagnosed or planned, the treatment should be carefully reconsidered and the patient must be advised of the possible teratogenic risk of cysteamine.
Jekk tkun hemm dijanjosi ta 'tqala jew tqala ppjanata, it- trattament għandu jkun jerġa khunsiderat b' reqqa u l- pazjent għandu jiġi infurmat dwar ir- riskju tal- possibiltà teratoġenika ta 'cysteamine.
However, due to the results of animal studies in breast-feeding mothers and neonates (see section 5.3), breast-feeding is contra-indicated in women taking CYSTAGON.
Madankollu, minħabba r- riżultati ta ’ studji fuq annimali fuq ommijiet li qed ireddgħu u fuq frieħ li għadhom kif jitwieldu (ara sezzjoni 5. 3), it- treddigħ hu kontra- indikat f ''nisa li qed jieħdu CYSTAGON.
CYSTAGON has minor or moderate influence on the ability to drive ans use machines CYSTAGON may cause drowsiness.
CYSTAGON jista ’ jikkawża n- ngħas.
Liver function tests abnormal Uncommon:
Rimettar, dardir, dijarrea.
Headache, encephalopathy Uncommon:
Uġigħ addominali, nifs bir - riħa, dispepsja, gastroenterite Mhux komuni:
Somnolence, convulsions
Ulċera gastro- intestinali
Gastrointestinal disorders
awrina Disturbi tal- ġilda u tat- tessut subkutaneji
Renal and urinary disorders Skin and subcutaneous tissue disorders
Mhux Mhux
Uncommon:
komuni:
Nephrotic syndrome Common:
Riħa tal- ġilda, anormali, raxx komuni:
Skin odour abnormal, rash Uncommon:
Disturbi muskolo- skeletali u tat- tessut
Hair colour changes, skin striae, skin fragility (molluscoid pseudotumor on elbows)
11 faġilità fil- ġilda (molluscoid pseudotumor fil - minkbejn)
Musculoskeletal and connective tissue disorders Metabolism and nutrition disorders
konnettiv Disturbi marbuta mal- metaboliżmu u n -
11 Two cases of nephrotic syndrome have been reported within 6 months of starting therapy with progressive recovery after treatment discontinuation.
Żewġ każi ta ’ sindromu nefrotiku kienu rrapurtati sa 6 xhur mill- bidu ta 'terapija b’ fejqan progressiv wara t- twaqqif ta' trattament.
A few cases of Ehlers-Danlos like syndrome on elbows have been reported in children chronically treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/ m2/ day.
Xi ftit każijiet li jixbhu s- sindromu ta ’ Ehlers- Danlos fuq il- minkbejn ġew irrapportati fi tfal ittrattati kronikament b’ dożi għoljin ta ’ mediċini b’ tipi differenti ta ’ cysteamine (cysteame chlorhydrate jew cystamine jew cysteamine bitartrate) li ħafna minnhom huma ’ l fuq mid- doża massima ta ’ 1. 95 g/ m2/ kuljum).
One patient subsequently died of acute cerebral ischemia with marked vasculopathy.
Pazjent wieħed sussegwentament miet minn iskemija ċerebrali akuta b’ vaskulopatija qawwija.
In some the patients, the skin lesions on elbows regressed after CYSTAGON dose reduction.
F’ xi pazjenti, il- lezjonijiet fil- ġilda fuq il- minkbejn irrigressaw wara tnaqqis fid- doża ta ’ CYSTAGON.
Cysteamine mechanism of action by interfering with the cross-linking of collagen fibers has been postulated (see section 4.4).
Hu maħsub li cysteamine jista 'jtellef li ssir il- ħolqa ta ’ katina tal- fibri tal- kollaġen (ara sezzjoni 4. 4).
Normal individuals and heterozygous subjects for cystinosis have white cell cystine levels of < 0.2, and usually below 1 nmol hemicystine/ mg protein, respectively.
Individwi normali u pazjenti eterożigotiċi għal ċistinożi għandhom livelli ta ’ cystine fiċ- ċelluli bojod ta ’ < 0, 2 u ġeneralment anqas minn 1 nmol ta ’ hemicystine/ mg ta ’ proteina, rispettivament.
Individuals with nephropathic cystinosis have elevations of white cell cystine above 2 nmol hemicystine/ mg protein
Individwi b’ ċistinożi nefropatika jkollhom żjieda ta 'cystine fiċ- ċelluli bojod ta ’ aktar minn 2 nmol ta ’ hemicystine/ mg ta ’ proteina.
Cysteamine reacts with cystine to form the mixed disulfide of cysteamine and cysteine, and cysteine.
12 Cysteamine tirreaġixxi ma 'cystine biex tifforma disulfide ta' cysteamine u cysteine, u cysteine.
The decrease in leucocyte cystine levels is correlated to the cysteamine plasma concentration over the six hours following the administration of CYSTAGON.
It- tnaqqis fl- għadd ta ’ cystine fil- lewkoċiti hu marbut mal- konċentrazzjoni ta ’ cysteamine fil- plażma matul is- sitt sigħat wara l- ghoti ta ’ CYSTAGON.
In one clinical study, baseline white cell cystine levels were 3.73 (range 0.13 to 19.8) nmol hemicystine/ mg protein and were maintained close to 1 nmol hemicystine/ mg protein with a cysteamine dose range of 1.3 to 1.95 g/ m2/ day.
Fi studju kliniku, il- livelli bażi ta 'referenza ta ’ cystine fiċ- ċelloli bojod kienu ta ’ 3. 73 (medda 0. 13 sa 19. 8) nmol hemiemicystine/ mg ta ’ proteina u nżammu viċin il- 1 nmol hemicystine/ mg ta ’ proteina permezz ta ’ firxa fid- doża ta ’ cysteamineminn1. 3 sa 1. 95 g/ m2/ jum.
12 increase enough to allow patients to catch up the normal for their age.
Madankollu, ir- rata ta 'tkabbir ma żdiedetx biżżejjed biex tippermetti li l- pazjenti jilħqu t- tul normali għall- età tagħhom.
Following a single oral dose of cysteamine bitartrate equivalent to 1.05 g of cysteamine free base in healthy volunteers, the mean (± sd) values for the time to peak and peak plasma concentration are 1.4 (± 0.5) hours and 4.0 (± 1.0) µg/ ml, respectively.
Wara doża orali waħda ta ’ cysteamine bitartrate ekwivalenti għal 1, 05 g ta ’ bażi ħieles ta 'cysteamine fuq voluntiera b' saħħithom, il- valuri medji (±sd) għall- ħin biex tjintlaħaq l- ogħla konċentrazzjoni u l - ogħla konċentrazzjoni tal- plażma huwa ta ’ 1. 4 (± 0. 5) siegħat u 4. 0 (± 1. 0) µg/ ml, rispettivament.
The in vitro plasma protein binding of cysteamine, which is mostly to albumin, is independent of plasma drug concentration over the therapeutic range, with a mean (± sd) value of 54.1% (± 1.5).
L- irbit ta ’ cysteamine mal- proteini tad- demm in vitro, prinċipalment ma ’ albumin, huwa indipendenti mill- konċentrazzjoni tal - mediċina fil- plażma fuq il- firxa terapewtika, b’ valur medju (± sd) ta ’ 54. 1% (± 1. 5).
Very limited data suggest that cysteamine pharmacokinetic parameters may not be significantly modified in patients with mild to moderate renal insufficiency.
Informazzjoni limitata ħafna turi li l- parametri farmakokinetiċi ta 'cysteamine m’ għandhomx jinbidlu b’ mod sinifikanti f’ pazjenti b’ insuffiċjenza renali minn ħafifa sa moderata.
5.3 Preclinical safety data
13 5. 3 Tagħrif ta 'qabel l- użu kliniku dwar is- sigurtà tal- mediċina
Genotoxicity studies have been performed: although in published studies using cysteamine, induction of chromosome aberrations in cultured eukaryotic cell lines has been reported, specific studies with cysteamine bitartrate did not show any mutagenic effects in the Ames test or any clastogenic effect in the mouse micronucleus test.
Saru studji dwar il- ġenotossiċità: għalkemm fi studji ppubblikati dwar l- użu ta ’ cysteamine, kien irrapportat zjieda ta ’ aberazzjoni tal- kromożoma f’ razza ta 'ċelloli ewkarjotiċi mkabbra, studji speċifiċi b ’ cysteamine bitartrate ma wrew l- ebda effetti mutaġeniċi fit- test ta ’ Ames u l- ebda effett klastoġeniku fit- test fuq il- mikronukleu tal- ġurdien.
This is equivalent to 0.6 g/ m2/ day in the rat, which is less than half the recommended clinical maintenance dose of cysteamine, i. e.
Dan hu ekwivalenti għal 0. 6 g/ m2/ jum fil- far, li hu anqas min- nofs tad- doża ta ’ manteniment kliniku rakkomandata ta 'cysteamine, i. e.
At this dose, weight gain and survival of the offspring during lactation was also reduced.
B’ din id- doża, kien hemm tnaqqis ukoll fiż- żjieda fil- piż u fin- numru ta ’ frieħ li baqgħu ħajjin waqt it- treddigh.
High doses of cysteamine impair the ability of lactating mothers to feed their pups.
Dożi għolja ta ’ cysteamine inaqqsu l- kapċità ta ’ ommijiet li jreddgħu lill- frieħ tagħhom.
Experimental administration of the drug causes depletion of somatostatin in several animal species.
Għotja sperimentali tal - prodott medicinali wasslet għal nuqqas ta 'somatostatina f’ diversi speċi ta ’ annimali.
Capsule content: microcrystalline cellulose, pregelatinized starch, magnesium stearate/ sodium lauryl sulfate, colloidal silicon dioxide, croscarmellose sodium Capsule shell: gelatin, titanium dioxide,
Il- kapsula tikkonsisti minn: microcrystalline cellulose, pregelatinized starch, magnesium stearate/ sodium lauryl sulfate, colloidal silicon dioxide, croscarmellose sodium, Il- qoxra tal- kapsula tikkonsisti minn: gelatin, titanium dioxide, Il- linka sewda fuq kapsuli iebsa fiha E172.
6.2 Incompatibilities
6. 3 Inkompatibilitajiet
Do not store above 25°C.
Taħżinx f’ temperatura `l fuq minn 25°C.
HDPE bottles of 100 and 500 hard capsules.
14 Fliexken HDPE ta ’ 100 u 500 kapsuli iebsin.
A desiccant unit containing black activated carbon and silica gel granules is included in the bottle.
Dessikant tal- karbonju iswed attivat u granijiet tas- silika gel huma inklużi fil- flixkun.
EU/ 1/ 97/ 039/ 003 (100 hard capsules per bottle), EU/ 1/ 97/ 039/ 004 (500 hard capsules per bottle).
EU/ 1/ 97/ 039/ 003 (100 kapsula iebsa kull flixkun), EU/ 1/ 97/ 039/ 004 (500 kapsula iebsa kull flixkun).
23 June 2007
23 ta ’ Ġunju 2007
Detailed information on this product is available on the website of the European Medicines Agency (EMEA) http: / /www. emea. europa. eu
Informazzjoni dettaljata dwar dan il- prodott tinsab fuq il- website ta ’ l- Aġenzija Ewropeja dwar il - mediċini (EMEA) http: // www. emea. europa. eu /
Orphan Europe SARL, 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, Franza
Summary of Product Characteristics, section 4.2).
Sommarju tal- Karatteristiċi tal- Prodott fil- Qosor, sezzjoni 4. 2)
OUTER CARTON CYSTAGON 50 mg x 100 hard capsules OUTER CARTON CYSTAGON 50 mg x 500 hard capsules
19 TAGĦRIF LI GĦANDU JIDHER FUQ IL- PAKKETT TA ’ BARRA KARTUNA TA ’ BARRA TA ’ CYSTAGON 50 mg x 100 kapsula iebsa KARTUNA TA 'BARRA TA' CYSTAGON 50 mg x 500 kapsula iebsa
CYSTAGON 50 mg hard capsules Cysteamine
CYSTAGON 50 mg kapsuli iebsin Cysteamine
Each hard capsule contains 50 mg of cysteamine (as mercaptamine bitartrate).
Kull kapsula iebsa fiha 50 mg ta ’ cysteamine (bħala mercaptamine bitartrate)
100 hard capsules (with a desiccant unit in the bottle) 500 hard capsules (with a desiccant unit in the bottle)
100 kapsula iebsa (b’ junit desskikanti fil- flixkun) 500 kapsula iebsa (b’ junit dessikanti fil- flixkun)
Oral use
Użu għal ħalq
Read the package leaflet before use.
Aqra l- fuljett ta 'tagħrif qabel l- użu
Do not store above 25°C.
Taħżinx f’ temperatura 'l fuq minn 25°C.
Keep the container tightly closed in order to protect from light and moisture
Żomm il- kontenitur magħluq sewwa sabiex tilqa ’ mid- dawl u mill- umdità.
EU/ 1/ 97/ 039/ 001 – 100 hard capsules EU/ 1/ 97/ 039/ 002 – 500 hard capsules
EU/ 1/ 97/ 039/ 001 – 100 kapsula iebsa EU/ 1/ 97/ 039/ 002 - 500 kapsula iebsa
Batch {number}
Lott [numru]
OUTER CARTON CYSTAGON 150 mg x 100 hard capsules OUTER CARTON CYSTAGON 150 mg x 500 hard capsules
21 TAGĦRIF LI GĦANDU JIDHER FUQ IL- PAKKETT TA ’ BARRA KARTUNA TA ’ BARRA TA ’ CYSTAGON 150 mg x 100 kapsula iebsa KARTUNA TA ’ BARRA TA ’ CYSTAGON 150 mg x 500 kapsula iebsa
CYSTAGON 150 mg hard capsules Cysteamine
CYSTAGON 150 mg kapsuli iebsin Cysteamine
Each hard capsule contains 150 mg of cysteamine (as mercaptamine bitartrate).
Kull kapsula iebsa ta ’ CYSTAGON 150 mg ta ’ cysteamine (bħala mercaptamine bitartrate)
100 hard capsules (with a desiccant unit in the bottle) 500 hard capsules (with a desiccant unit in the bottle)
100 kapsula iebsa (b’ junit desikkanti fil- flixkun) 500 kapsula iebsa (b’ junit dessikanti fil- flixkun)
EU/ 1/ 97/ 039/ 003 – 100 hard capsules EU/ 1/ 97/ 039/ 004 – 500 hard capsules
EU/ 1/ 97/ 039/ 003 – 100 kapsula iebsa EU/ 1/ 97 039/ 004 - 500 kapsula iebsa
23 PARTICULARS TO APPEAR ON THE IMMEDIATE PACKAGING
23 TAGĦRIF LI GĦANDU JIDHER FUQ IL- PAKKETT LI JMISS MAL- PRODOTT
BOTTLE LABEL CYSTAGON 50 mg x 100 hard capsules BOTTLE LABEL CYSTAGON 50 mg x 500 hard capsules
TIKKETTA TAL- FLIXKUN TA ’ CYSTAGON 50 mg x 100 kapsula iebsa TIKKETTA TAL- FLIXKUN TA ’ CYSTAGON 50 mg x 500 kapsula iebsa
CYSTAGON 50 mg hard capsules Cysteamine
CYSTAGON 50 mg kapsuli iebsa Cysteamine
Each hard capsule contains 50 mg of cysteamine (as mercaptamine bitartrate).
Kull kapsula iebsa fiha 50 mg ta ’ cysteamine (bħala mercaptime bitartrate)
NAME AND ADDRESS OF THE MARKETING AUTHORISATION HOLDER
ISEM U INDIRIZZ TA TAD- DETENTUR TA ’ L- AWTORIZZAZZJONI GĦAT - TQEGĦID FIS- SUQ
25 PARTICULARS TO APPEAR ON THE IMMEDIATE PACKAGING
25 TAGĦRIF LI GĦANDU JIDHER FUQ IL- PAKKETT FUQ IL- PAKKETT LI JMISS MAL - PRODOTT
BOTTLE LABEL CYSTAGON 150 mg x 100 hard capsules BOTTLE LABEL CYSTAGON 150 mg x 500 hard capsules
TIKKETTA TAL- FLIXKUN TA ’ CYSTAGON 150 mg x 100 kapsula iebsa TIKKETTA TAL- FLIXKUN TA ’ CYSTAGON 150 mg x 500 kapsula iebsa
Each hard capsule contains 150 mg of cysteamine (as mercaptamine bitartrate).
Kull kapsula iebsa fiha 150 mg ta ’ cysteamine (bħala mercaptamine bitartrate)
100 hard capsules (with a desiccant unit in the bottle) 500 hard capsules (with a desiccant unit in the bottle)
100 kapsula iebsa (b’ unità desikkanti fil- flixkun) 500 kapsula iebsa (b’ junit dessikanti fil- flixkun)
SPECIAL WARNING THAT THE MEDICINAL PRODUCT MUST BE STORED OUT OF THE REACH AND SIGHT OF CHILDREN
TWISSIJA SPEĊJALI LI L- PRODOTT MEDIĊINALI GĦANDU JINŻAMM FEJN MA JINTLAĦAQX U MA JARAWHX IT- TFAL
EU/ 1/ 97/ 039/ 003 – 100 hard capsules EU/ 1/ 97/ 039/ 004 – 500 hard capsules
EU/ 1/ 97/ 039/ 003 – 100 kapsula iebsa EU/ 1/ 97 039/ 004 – 500 kapsula iebsa
28 PACKAGE LEAFLET:
28 FULJETT TA ’ TAGĦRIF:
CYSTAGON 50 mg Hard Capsules CYSTAGON 150 mg Hard Capsules Cysteamine bitartrate (mercaptamine bitartrate)
CYSTAGON 50 mg Kapsuli Iebsin CYSTAGON 150 mg Kapsuli Iebsin Cysteamine bitartrate (mercaptamine bitartrate)
Read all of this leaflet carefully before you start using this medicine.
Aqra dan il- fuljett kollu qabel tibda tuża din il- mediċina
- This medicine has been prescribed for you.
- Din il- mediċina ġiet mogħtija lilek għall- użu personali tiegħek.
It may harm them, even
Tgħaddihiex lil persuni oħra.
if their symptoms are the same as yours.
Tista ’ tagħmlilhom il- ħsara, anki jekk ikollom l- istess sintomi bħal tiegħek.
please tell your doctor or pharmacist.
m’ humiex imsemmijin f’ dan il- fuljett, jekk jogħġbok, għid lit- tabib jew lill- ispiżjar tiegħek
What CYSTAGON is and what it is used for 2.
X’ inhu CYSTAGON u għalxiex jintuża 2.
Before you use CYSTAGON 3.
Qabel ma tieħu CYSTAGON 3.
How to use CYSTAGON 4.
Kif tuża CYSTAGON 4.
How to store CYSTAGON 6.
Kif taħżen CYSTAGON 6.
WHAT CYSTAGON IS AND WHAT IT IS USED FOR
X’ INHU CYSTAGON U GĦALXIEX JINTUŻA
Cystinosis is a metabolic disease called ‘ nephropathic cystinosis’ which is characterized by an abnormal accumulation of the amino acid cystine in various organs of the body such as the kidney, eye, muscle, pancreas, and brain.
Ic- cistanosi hija marda marbuta mal- metaboliżmu li tissejjaħ ‘ ċistanosi nefropatika ’ ikkaratterizzata b’ akkumulazzjoni anormali ta ’ l- aċidu amminiku cystine f’ diversi organi tal- ġisem bħall- kliewi, l- għajnejn, il- muskoli, il- frixa u l- moħħ.
Cystine build up causes kidney damage and excretion of excess amounts of glucose, proteins and electrolytes.
It- tiġmigħ jikkaġuna ħsara fil- kliewi u l- eliminazzjoni ta ’ ammonti eċċessivi ta ’ glukows, proteini u elettroliti.
Different organs are affected at different ages.
Organi differenti huma affettwati f’ etajiet differenti.
CYSTAGON is prescribed to manage this rare inherited disorder.
CYSTAGON jiġi preskritt biex jiġġestixxi dan id- disturb ereditarju rari.
CYSTAGON is a medicine that reacts with cystine to decrease its level within the cells.
CYSTAGON hi mediċina li tirreaġixxi ma ’ cystine biex tnaqqas il- livelli tagħha fiċ- ċelloli.
BEFORE YOU USE CYSTAGON
QABEL MA TUŻA CYSTAGON
Do not use CYSTAGON
Tużax CYSTAGON:
- if you -or your child- are allergic (hypersensitive) to cysteamine bitartrate or penicillamine or
- jekk int jew it- tifel/ tifla tiegħek għandkom allerġija (sensittività eċċessiva) cysteamine bitartrate
any of the other ingredients of Cystagon.
jew penicillamine jew xi wieħed mis- sustanzi ta ’ Cystagon.
- If you are pregnant, this is particularly relevant during the first trimester
- Jekk inti tqila dan hu partikolarment importanti waqt l- ewwel tlett xhur
Take special care with CYSTAGON
Oqgħod attent ħafna b’ CYSTAGON:
- When your or your child’ s disorder has been confirmed by leucocyte cystine measurements, the
- Meta l- kundizzjoni tiegħek jew tal- wild tiegħek tkun ġiet ikkonfermata b’ kejl ta ’ leucocyte
therapy with CYSTAGON must be started as soon as possible.
cystine, it- terapija b’ Cystagon għandha tinbeda minnufih.
- A few cases of skin lesions on elbows like little hard lumps have been reported in children
- Xi ftit każijiet ta 'leżjonijiet fuq il- ġilda fil- minkbejn, bħal gundalli iebsin żgħar, ġew irraportati
treated with high doses of different cysteamine preparations.
fit- tfal li ġew ittrattati b' dożi għoljin ta 'suriet ta ’ tipidifferenti ta ’ cysteamine.
These lesions were associated with skin striae and bone lesions such as fracture and bone deformities, and with laxity of joints.
Dawn il- leżjonijiet kienu marbutin ma striji tal- ġilda u leżjonijiet tal- għadam bħal ksur u deformitajiet, u ma 'laxkezza tal- ġogi.
Your doctor could require a regular physical and X-ray examination for the skin and the bones to control the effects of the medicinal product.
It- tabib tiegħek jew it- tabib tat- tifel/ tifla tiegħek jistgħu jirranġaw sabiex isiru eżamijiet fiżiċi regolari u għar- raġġi- X tal- ġilda u tal- għadam biex jikkontrollaw l- effetti tal - prodott mediċinali.
Self examination of your or your child’ s skin is recommended.
Huwa rrakkomandat li jsiru eżamijiet minnek stess tal- ġilda tiegħek jew tat - tifel/ tifla tiegħek.
If any skin or bone abnormalities appear, please inform your doctor immediately.
Jekk jibdew jidhru xi anormalitajiet fuq il- ġilda jew fl- għadam, jekk jogħġbok għarraf lit- tabib minnufih.
- Your doctor could require controlling the blood cell count on a regular basis.
- It- tabib tiegħek għandu mnejn ikun jeħtieġ li jikkontrolla l- għadd ta ’ ċelloli tad- demm fuq bażi
Where
- CYSTAGON ma ntweriex li jimpedixxi l- kristalli tas- cystine milli jakkumulaw fl- għajnejn.
cysteamine ophthalmic solution has been used for that purpose, its usage should continue.
Għalhekk, fejn soluzzjoni oftalmika ta ’ cysteamine hija użata għal dak l- iskop, l- użu tagħha għandu jitkompla.
- In contrast to phosphocysteamine, another active substance close to cysteamine bitartrate,
- B’ kuntrast ma 'phosphocysteamine, sustanza attiva oħra viċin cysteamine bitartrate,
CYSTAGON does not contain phosphate.
CYSTAGON ma fihx fosfat.
29 and the dose of these may need to be changed when CYSTAGON is substituted for phosphocysteamine.
Jista ’ jkun li diġà qed tingħata phosphate żejjed u d- doża jista ’ jkollha bżonn tinbidel meta CYSTAGON jingħata minflok phosphocysteamine.
- To avoid any risk of aspiration in the lungs, the capsules should not be given to children under
- Sabiex tevita kull riskju ta ’ aspirazzjoni fil- pulmun, il- kapsuli m’ għandhomx jingħataw lil tfal li
the age of approximately 6 years.
għandhom inqas minn madwar 6 snin.
Using other medicines Please inform your doctor or pharmacist if you are taking or have recently taken any other medicines, even those not prescribed.
Meta tieħu mediċini oħra Jekk jogħġbok għid lit- tabib jew lill- ispiżjar tiegħek jekk qiegħed tieħu jew ħadt dan l- aħħar xi mediċini oħra, anki dawk mingħajr riċetta.
Using CYSTAGON with food and drink For children under approximately six years of age, the hard capsule may be opened and the contents sprinkled on food (e. g. milk, potatoes or starch based foods) or mixed in formula.
Għal tfal li għandhom inqas minn madwar 6 snin, il- kapsuli iebsin jistgħu jinfetħu u l- kontenut tagħhom jitraxxax fuq l- ikel f’ temperatura xierqa (eż. ħalib, patata u prodotti oħrajn magħmulin mill - lamtu) jew imħallta fil- formula.
Do not add to acidic drinks e. g. orange juice.
Iżżidux ma ’ xorb aċiduż, bħal meraq tal- larinġ.
Consult the doctor for complete directions.
Ikkonsulta mat- tabib tiegħek għal struzzjonijiet sħaħ.
Pregnancy You should not use CYSTAGON if you are pregnant.
Tqala M’ għandekx tuża CYSTAGON jekk inti tqila.
Please consult your doctor if you plan to become pregnant.
Jekk jogħġbok ikkonsulta mat- tabib tiegħek jekk qed tippjana li toħroġ tqila.
Breast-feeding CYSTAGON must not be used during breast-feeding.
Jekk qed tredda CYSTAGON m’ għandux jintuża waqt li qed tredda ’.
Driving and using machines CYSTAGON may cause some drowsiness.
CYSTAGON jista ’ jikkawża n- ngħas.
When starting therapy, you or your child should not engage in potentially hazardous activities until the effects of the drug are well known.
Meta tinbeda t- terapija int jew it- tifel jew it- tifla tiegħek m’ għandkomx twettqu attivitajiet li jistgħu jkunu perikolużi sakemm l- effetti tal- mediċina jkunu magħrufa sew.
HOW TO USE CYSTAGON
KIF JITTIEĦED CYSTAGON
Always use CYSTAGON exactly as your doctor or your child’ s doctor has told you.
Dejjem għandek tieħu CYSTAGON skond il- parir tat- tabib.
The dose of CYSTAGON prescribed for you or your child will depend on your or your child's age and weight.
Id- doża ta 'CYSTAGON li ġiet preskritta lilek jew lit- tifel/ tifla tiegħek ser tiddependi fuq l- età u l- piż tiegħek jew tat- tifel/ tifla tiegħek.
For children up to age 12 years, the dose will be based on the body size (surface area), the usual dose being 1.30 g/ m2 of body surface area per day.
Għal tfal li għandhom sa 12- il sena ta 'età, id- doża ser tkun bażata fuq id- daqs tal- ġisem (erja superfiċjali), id- doża li ġeneralment tingħata hi ta' 1. 30 g/ m2 ta 'erja korporali superfiċjali kuljum.
For patients over age 12 and over 50 kg weight, the usual dose is 2g/ day.
Għal pazjenti li għandhom aktar minn 12- il sena, id- doża li ġeneralment tingħata hi ta '2g/ kuljum.
In any case the usual dose should not exceed 1.95 g/ m2/ day.
Fi kwalunkwe każ id- doża m' għandhiex taqbeż 1. 95 g/ m2/ kuljum.
CYSTAGON should be taken or given only by mouth and exactly as your or your child's doctor directs.
CYSTAGON għandu jittieħed jew jingħata biss mill- ħalq u eżatt kif it- tabib tiegħek jew tat- tifel jew tifla tiegħek jispjegalek.
In order for CYSTAGON to work correctly, you must do the following:
Biex CYSTAGON jaħdem sew, int għandek tagħmel dan li ġej:
- Follow your doctor's directions exactly.
- Segwi bl- eżatt l- istruzzjonijiet tat- tabib tiegħek.
Do not increase or decrease the amount of medicine
Iżżidx jew tnaqqas l- ammont ta 'mediċina
without your doctor's approval.
mingħajr l- approvazzjoni tat- tabib tiegħek.
- Hard capsules should not be given to children under approximately six years of age because
- Kapsuli iebsin m’ għandhomx jingħataw lil tfal sa inqas madwar 6 snin, għaliex jistgħu jbatu
they may not be able to swallow them and they may choke.
biex jibilgħuhom u jistgħu jifgaw.
For children under approximately six years of age, the hard capsule may be opened and the contents sprinkled on food (e. g. milk, potatoes or starch based foods) or mixed in formula.
Għal tfal taħt is- sitt snin, il- kapsula iebsa tista ’ tinfetaħ u l - kontenut tagħha jitraxxax fuq l- ikel (eż. ħalib, patata u prodotti oħrajn magħmulin mill- lamtu) jew mħallat fil- formula.
Consult the doctor for complete directions.
Ikkonsulta lit- tabib tiegħek għal struzzjonijiet kompluti.
- Your or your child's medical treatment may include, in addition to CYSTAGON, one or more
- It- trattament mediku tiegħek jew tat- tifel jew tifla tiegħek jista ’ jinkludi, minbarra CYSTAGON,
supplements to replace important electrolytes lost through the kidneys.
suppliment jew aktar biex iżidu l- elektroliti importanti mitlufa mill- kliewi.
It is important to take or give these supplements exactly as instructed.
Hu importanti li tieħu jew tagħti dawn is- supplimenti eżatt kif spjegat.
If several doses of the supplements are missed or weakness or drowsiness develops, call the doctor for instructions.
Jekk taqbeż diversi dożi tas- supplimenti jew tħossok bla saħħa jew għajjien, kellem lit- tabib tiegħek għal struzzjonijiet.
- Regular blood tests to measure the amount of cystine inside white blood cells are necessary to
- Eżamijiet regolari tad- demm biex tkejjel l- ammont ta ’ cystine fiċ- ċelloli bojod tad- demm huma
help determine the correct dose of CYSTAGON.
meħtieġa biex tiġi stabbilita d- doża korretta ta ’ CYSTAGON.
Your or your child's doctor will arrange for the blood tests to be done.
It- tabib tiegħek jew tat- tifel/ tifla
Regular blood and urine tests to measure the levels of the body's important electrolytes are also necessary to help your or your child's doctor correctly adjust the doses of these supplements.
Eżamijiet regolari tad- demm u ta ’ l - awrina biex ikejjlu l- livelli ta ’ l- elektroliti importanti tal- ġisem huma meħtieġa wkoll biex jgħinu lit- tabib tiegħek, jew it- tabib tat- tifel/ tifla tiegħek jibdel id- dożi ta ’ dawn is- supplimenti kif meħtieġ.
CYSTAGON should be taken 4 times a day, every 6 hours, preferably just after or with food.
CYSTAGON għandu jittieħed 4 darbiet kuljum, kull 6 siegħat, preferibbilment wara jew ma ’ l- ikel.
It is important to take the dose as close to every 6 hours as possible.
Hu importanti li possibbilment tieħu d- doża kemm jista 'jkun viċin kull 6 siegħat.
30 Treatment with CYSTAGON should continue indefinitely, as instructed by your doctor.
It- trattament b’ CYSTAGON għandu jkompli indefinitivament, skond kif jgħidlek it- tabib tiegħek.
If you use more CYSTAGON than you should:
Jekk tuża CYSTAGON aktar milli suppost għandek:
You should contact your or your child's doctor or the hospital emergency department immediately if more medicine has been taken than has been prescribed, drowsiness develops.
Għandek tagħmel kuntatt mat- tabib tiegħek jew tat- tarbija tiegħek jew mad- dipartiment ta ’ l - emerġenza ta ’ l- isptar immedjatament jekk tkun ħadt doża akbar minn dik li ngħatat lilek fuq ir- riċetta, jekk tħossok għajjien.
If you forget to take CYSTAGON:
Jekk tinsa tieħu CYSTAGON:
If a dose of medicine is missed, it should be taken as soon as possible.
Jekk tinsa tieħu doża ta ’ mediċina, din għandha tittieħed kemm jista ’ jkun malajr.
However if it is within two hours of the next dose, skip the missed dose and go back to the regular dosing schedule.
Madankollu, jekk fadal anqas minn sagħtejn għad- doża li jmiss, aqbez id- doża li tkun tlift u erġa ’ lura għall- iskeda regolari tad- doża.
Do not take a double dose to make up for a forgotten dose.
Tieħux doża doppja biex tpatti għad- doża li tkun insejt tieħu.
Like all medicines, CYSTAGON can cause side effects, although not everybody gets them.
Bħal kull mediċina oħra, CYSTAGON jista ’ jkollu effetti oħra, għalkemm ma jidhrux fuq kulħadd
CYSTAGON may cause some people to become drowsy or less vigilant than they are normally.
CYSTAGON jista ’ jwassal biex ċertu persuni jħossuhom għajjenin jew anqas żvelti milli huma s - soltu.
Make sure you know how you or your child reacts to this medicine before doing anything that could be dangerous if not alert.
Kun ċert li taf kif int jew it- tifel jew it- tifla tiegħek tirreaġġixxu għal din il- mediċina qabel tagħmel xi ħaġa li tista ’ tkun perikoluża jekk ma tkunx pront.
The following side effects were reported as follows: very common (occurring in at least one in 10 patients), common (occurring in at least one in 100 patients), uncommon (occurring in at least one in1,000 patients), rare (occurring in at least one in 10,000 patients), very rare (occurring in at least one in 100,000 patients).
L- effetti kollaterali li ġejjin ġew irrapportati kif ġej: komuni ħafna (li jseħħu f’ ta ’ l- anqas wieħed minn kull 10 pazjenti), komuni (li jseħħu f’ ta ’ l- anqas wieħed minn kull 100 pazjent), mhux komuni (li jseħħu f’ ta ’ l- anqas wieħed minn kull 1, 000 pazjent), rari (li jseħħu f’ ta ’ l- anqas wieħed minn kull 10, 000 pazjent), rari ħafna (li jseħħu f’ ta ’ l- anqas wieħed minn kull 100, 000 pazjent).
- Very common: vomiting, nausea, diarrhoea, loss of appetite, fever and sensation of sleep
- Komuni ħafna: rimettar, dardir, dijarrea, nuqqas ta ’ aptit, deni u tħossok trid torqod.
- Common: abdominal pain or discomfort, unpleasant breath and body odour, skin eruption,
- Komuni: uġigħ jew skumdità addominali, riħa ħażina tan- nifs u tal- ġisem, raxx fil- ġilda,
gastroenteritis, fatigue, headache, encephalopathy (brain disorder) and liver function test abnormalities.
gastroenterite, għejja, uġigħ ta ’ ras, enċefalopatija (kundizzjoni tal- moħħ) u abnormalitajiet fit - testijiet tal- funzjoni tal- kliewi.
- Uncommon: skin striae, skin lesion (little-hard lumps on elbows), joint laxity, leg pain, bone
- Mhux komuni: striji tal- ġilda, leżjonijiet fuq il- ġilda (ġundalli kemmxejn iebsin fuq il -