natgillin/translations-raw huggingface dataset:
Trial 7 evaluated subjects with lung clearance index (LCI2.5) ≥ 7.5 at the initial screening visit (mean LCI2.5 10.28 at baseline [range: 6.55 to 16.38]) and ppFEV1 ≥ 70 at screening (mean ppFEV1 89.8 at baseline [range: 48.6 to 119.6]). 
V preskušanju 7 so ocenjevali osebe z indeksom pljučnega očistka (LCI2,5) ≥ 7,5 pri začetnem presejalnem pregledu (povprečni LCI2,5 10,28 v izhodišču [razpon: 6,55 do 16,38]) in ppFEV1 ≥ 70 ob presejanju (povprečni ppFEV1 89,8 ob presejanju [razpon: 48,6 do 119,6]).
Primary Endpoint Absolute change in lung clearance index (LCI2.5) from baseline through Week 24 
Primarni cilj preskušanja Absolutna sprememba indeksa pljučnega očistka (LCI2,5) od izhodišča do konca
In Trial 8 in patients homozygous for F508del-CFTR mutation aged 2 to 5 years, the mean absolute within-group change in sweat chloride from baseline at Week 24 was -31.7 mmol/L (95% CI: -35.7, -27.6). 
V preskušanju 8 pri bolnikih, homozigotnih za mutacijo F508del-CFTR, starih 2 do 5 let, je bila povprečna absolutna sprememba kloridov v znoju znotraj skupine od izhodišča v 24. tednu -31,7 mmol/l (95 % IZ: -35,7, -27,6).
• Increase in blood pressure 
• porast krvnega tlaka,
MARKETING AUTHORISATION NUMBER(S) EU/1/15/1059/001 112 film-coated tablets (4 packs of 28 tablets) EU/1/15/1059/002 56 film-coated tablets (2 packs of 28 tablets) 
ŠTEVILKA(E) DOVOLJENJA (DOVOLJENJ) ZA PROMET EU/1/15/1059/001 112 filmsko obloženih tablet (4 pakiranja po 28 tablet) EU/1/15/1059/002 56 filmsko obloženih tablet (2 pakiranji po 28 tablet)
The exposure (AUC) of lumacaftor is approximately 2-fold higher in healthy adult volunteers compared to exposure in patients with CF. 
Izpostavljenost (AUC) lumakaftorju je približno dvakrat večja pri zdravih odraslih prostovoljcih kot pri bolnikih s CF. Izpostavljenost ivakaftorju je pri zdravih odraslih prostovoljcih in bolnikih s CF
Mean ivacaftor (SD) AUCss (μg/mL*h) 
Povprečna (SD) AUCss ivakaftorja (μg/ml*h)
Orkambi granules are indicated for the treatment of cystic fibrosis (CF) in children aged 2 years and older who are homozygous for the F508del mutation in the CFTR gene (see sections 4.2, 4.4 and 5.1). 
Zrnca zdravila Orkambi so indicirana za zdravljenje cistične fibroze (CF) pri otrocih, starih 2 leti ali več, ki so homozigotni za mutacijo F508del na genu CFTR (glejte poglavja 4.2, 4.4 in 5.1).
EU/1/15/1059/007 
EU/1/15/1059/006 EU/1/15/1059/007
The primary efficacy endpoint in both studies was the absolute change from baseline in ppFEV1 at week 24. 
Primarni učinkovitostni cilj v obeh študijah je bila absolutna sprememba ppFEV1 od izhodišča v 24. tednu.
Ivacaftor is a weak inhibitor of CYP3A when given as monotherapy. 
Ivakaftor je šibek zaviralec CYP3A, kadar ga dajemo kot monoterapijo.
There have been reports of false positive urine screening tests for tetrahydrocannabinol (THC - an active component in cannabis) in patients receiving Orkambi. 
Pri bolnikih, ki so jemali zdravilo Orkambi, so poročali o lažno pozitivnih urinskih presejalnih testih za tetrahidrokanabinol (THC - aktivna sestavina kanabisa).
Your child's doctor may monitor your child's blood pressure during treatment with Orkambi. 
Mogoče bo zdravnik otroku med zdravljenjem z zdravilom Orkambi spremljal krvni tlak.
12 years and older 
12 let ali več
Table 1: Recommended dose of Orkambi in patients aged 2 years and older 
Preglednica 3: Priporočeni odmerek zdravila Orkambi za bolnike, stare 2 leti ali več
• Avocados, hummus, soy-based products (tofu) 
• avokado, humus, izdelki iz soje (tofu); • prehranske ploščice ali pijače.
Orkambi should be taken with fat-containing food. 
Zdravilo Orkambi je treba jemati z mastno hrano.
PHARMACEUTICAL FORM AND CONTENTS granules 
FARMACEVTSKA OBLIKA IN VSEBINA
Vascular disorders uncommon Hypertension 
Žilne bolezni občasni hipertenzija
Therefore, co-administration with strong CYP3A inducers (e.g., rifampicin, St. John's wort [Hypericum perforatum]) is not recommended (see section 4.5). 
Zato sočasno dajanje z močnimi induktorji CYP3A (npr. rifampicinom ali šentjanževko [Hypericum perforatum]) ni priporočljivo (glejte poglavje 4.5).
Description of selected adverse reactions for paediatric patients aged 2 to 11 years Hepatobiliary events 
Opis izbranih neželenih učinkov pri pediatričnih bolnikih, starih 2 do 11 let Dogodki, povezani z jetri in žolčevodom
During the 24-week, placebo-controlled, Phase 3 studies, a maximum decrease in mean heart rate of 6 beats per minute (bpm) from baseline was observed on Day 1 and Day 15 around 4 to 6 hours after dosing. 
Med 24-tedenskimi študijami 3. faze, kontroliranimi s placebom, so 1. in 15. dne okrog 4 do 6 ur po odmerjanju ugotovili največje zmanjšanje srednje srčne frekvence za 6 utripov na minuto (beats per minute, bpm) od izhodišča.
Safety data were evaluated in 60 patients aged 2 to 5 years, 161 patients aged 6 to 11 years (Trials 6, 7 and 8) and in 194 patients aged 12 to 17 years with CF who are homozygous for the F508del mutation and who received lumacaftor/ivacaftor in clinical studies. 
Varnostne podatke so ocenili pri 60 bolnikih, starih 2 do 5 let, 161 bolnikih, starih 6 do 11 let (preskušanja 6, 7 in 8), in pri 194 bolnikih, starih 12 do 17 let, s CF, ki so bili homozigotni za mutacijo F508del in ki so prejemali lumakaftor/ivakaftor v kliničnih študijah.
Abnormalities in liver function, including advanced liver disease, can be present in patients with CF. 
Pri bolnikih s CF so lahko prisotne nenormalnosti funkcije jeter, vključno z napredovalo boleznijo jeter.
Lumacaftor and ivacaftor work together to improve the function of the abnormal CFTR protein. 
Lumakaftor in ivakaftor skupaj izboljšata funkcijo nenormalne beljakovine CFTR.
The active substances are lumacaftor and ivacaftor. 
Učinkovini sta lumakaftor in ivakaftor.
Non-clinical data reveal no special hazard for humans based on conventional studies of safety pharmacology, repeated dose toxicity, genotoxicity, carcinogenic potential, and toxicity to reproduction and development. 
Predklinični podatki na osnovi običajnih študij farmakološke varnosti, toksičnosti pri ponavljajočih se odmerkih, genotoksičnosti, kancerogenega potenciala, toksičnega vpliva na sposobnost razmnoževanja in razvoja ne kažejo posebnega tveganja za človeka.
In patients who received placebo (mean baseline 114 mmHg systolic and 69 mmHg diastolic), the maximum increase from baseline in mean systolic and diastolic blood pressure was 0.9 mmHg and 0.9 mmHg, respectively. 
Pri bolnikih, ki so prejemali placebo (srednji izhodiščni sistolični tlak 114 mm Hg in srednji izhodiščni diastolični tlak 69 mm Hg), je bil največji porast srednjega sistoličnega in diastoličnega krvnega tlaka od izhodišča 0,9 mm Hg oziroma 0,9 mm Hg.
Interim Analysis: December 2022 
december 2022.
During Trials 1 and 2, the incidence of maximum transaminase (ALT or AST) levels > 8, > 5, and > 3 x ULN was 0.8%, 2.0%, and 5.2%; and 0.5%, 1.9%, and 5.1% in lumacaftor/ivacaftor- and placebo-treated patients, respectively. 
Med preskušanjima 1 in 2 je bila incidenca najvišjih ravni transaminaz (ALT ali AST) > 8, > 5 oziroma > 3 x ULN 0,8 %, 2,0 % oziroma 5,2 %; in 0,5 %, 1,9 % oziroma 5,1 % pri bolnikih, ki so prejemali lumakaftor/ivakaftor oziroma placebo.
• Dark urine 
• temen urin;
PARTICULARS TO APPEAR ON THE OUTER PACKAGING INNER CARTON FOR MULTIPACK NO BLUE BOX 
PODATKI NA ZUNANJI OVOJNINI NOTRANJA ŠKATLA ZA SKUPNO PAKIRANJE BREZ »MODREGA OKENCA«
Trial 8: Safety and tolerability study in pediatric patients with CF aged 2 to 5 years homozygous for the F508del mutation in the CFTR gene 
Preskušanje 8: Študija varnosti in prenašanja pri pediatričnih bolnikih s CF, starih 2 do 5 let, homozigotnih za mutacijo F508del na genu CFTR
The treatment difference (LS mean) in sweat chloride for the average absolute change at Day 15 and at Week 4 as compared to placebo was -20.8 mmol/L (95% CI: -23.4, -18.2; nominal P < 0.0001). 
Razlika zaradi zdravljenja (povprečje najmanjših kvadratov) kloridov v znoju za povprečno absolutno spremembo v 15. dnevu in v 4. tednu v primerjavi s placebom je bila -20,8 mmol/l (95 % IZ: -23,4; -18,2; nominalna P < 0,0001).
Your doctor may monitor your blood pressure during treatment with Orkambi. 
Mogoče vam bo zdravnik med zdravljenjem z zdravilom Orkambi spremljal krvni tlak.
Key secondary endpoints included average absolute change from baseline in sweat chloride at Day 15 and Week 4 and at Week 24 (see Pharmacodynamic effects), absolute change from baseline in BMI at Week 24, absolute change from baseline in CFQ-R Respiratory Domain through Week 24. 
Ključni sekundarni cilji študije so vključevali povprečno absolutno spremembo kloridov v znoju od izhodišča do 15. dne in v 4. tednu in v 24. tednu (glejte Farmakodinamični učinki), absolutno spremembo ITM od izhodišča do 24. tedna in absolutno spremembo respiratorne domene CFQ-R od izhodišča do 24. tedna.
Lumacaftor is a strong inducer of CYP3A and ivacaftor is a weak inhibitor of CYP3A when given as monotherapy. 
Lumakaftor je močan induktor CYP3A, ivakaftor pa šibek zaviralec CYP3A, kadar ju dajemo kot monoterapijo.
• Stuffy or runny nose 
• kongestija nosu,
Patients with CF who have a gating (Class III) mutation in the CFTR gene Lumacaftor/ivacaftor has not been studied in patients with CF who have a gating (Class III) mutation in the CFTR gene on one allele, with or without the F508del mutation on the other allele. 
Bolniki s CF, ki imajo mutacijo spreminjanja prehodnosti (skupina III) na genu CFTR Lumakaftorja/ivakaftorja niso proučevali pri bolnikih s CF, ki imajo mutacijo spreminjanja prehodnosti (skupina III) na genu CFTR na enem alelu in bodisi mutacijo F508del na drugem alelu, bodisi te mutacije na drugem alelu nimajo.
PARTICULARS TO APPEAR ON THE OUTER PACKAGING OUTER CARTON - MULTIPACK - WITH BLUE BOX 
PODATKI NA ZUNANJI OVOJNINI ŠKATLA - SKUPNO PAKIRANJE - Z MODRIM OKENCEM
NAME AND ADDRESS OF THE MARKETING AUTHORISATION HOLDER Vertex Pharmaceuticals (Ireland) Limited 
IME IN NASLOV IMETNIKA DOVOLJENJA ZA PROMET Z ZDRAVILOM Vertex Pharmaceuticals (Ireland) Limited
• Immunosuppressants (used after an organ transplantation) for example: ciclosporin, everolimus, sirolimus, tacrolimus 
• zdravila za zaviranje imunske odzivnosti (uporabljajo se po presaditvi organa), na primer: ciklosporin, everolimus, sirolimus, takrolimus;
No serious respiratory events, dose reductions or discontinuations were seen in patients who were initiated at the half dose (see section 4.4). 
Pri bolnikih, ki so jim uvedli polovični odmerek, ni bilo resnih respiracijskih dogodkov, zmanjšanj odmerkov ali prekinitev zdravljenja (glejte poglavje 4.4).
Adverse reactions identified from the 24-week, placebo-controlled, Phase 3 studies (Trials 1 and 2) in patients aged 12 years and older and from a 24-week, placebo-controlled study in patients aged 6 to 11 years (Trial 7), who are homozygous for the F508del mutation in the CFTR gene are presented in Table 4 and are listed by system organ class, frequency, and adverse reactions. 
Neželeni učinki, ugotovljeni v 24-tedenskih, s placebom kontroliranih študijah faze 3 (preskušanji 1 in 2) pri bolnikih, starih 12 let ali več, in v 24-tedenski, s placebom kontrolirani študiji pri bolnikih, starih 6 do 11 let (preskušanje 7), ki so bili homozigotni za mutacijo F508del na genu CFTR, so navedeni v preglednici 4 po organskih sistemih, pogostnosti in neželenih učinkih.
Changes in sweat chloride in response to lumacaftor alone or in combination with ivacaftor were evaluated in a double-blind, placebo-controlled, Phase 2 clinical trial in patients with CF age 18 years and older. 
Spremembe kloridov v znoju v odziv na lumakaftor sam ali v kombinaciji z ivakaftorjem so ovrednotili v dvojno slepem kliničnem preskušanju 2. faze, kontroliranem s placebom, pri bolnikih s CF, starih 18 let ali več.
Co-administration of lumacaftor/ivacaftor with itraconazole, a strong CYP3A inhibitor, did not impact the exposure of lumacaftor, but increased ivacaftor exposure by 4.3-fold. 
Sočasno dajanje lumakaftorja/ivakaftorja z itrakonazolom, ki je močan zaviralec CYP3A, ni vplivalo na izpostavljenost lumakaftorju, je pa zvečalo izpostavljenost ivakaftorju za 4,3-krat.
Skin and subcutaneous tissue disorders common 
Bolezni kože in podkožja pogosti izpuščaj
Dizziness has been reported in patients receiving ivacaftor, a component of Orkambi, which could influence the ability to drive or use machines. 
Pri bolnikih, ki so jemali ivakaftor, ki je ena od sestavin zdravila Orkambi, so poročali o omotici, ki bi lahko vplivala na sposobnost uporabe zdravil ali strojev.
Patients who had 2 or more abnormal liver function tests (ALT, AST, AP, GGT ≥ 3 times the ULN), or ALT or AST > 5 times ULN, or total bilirubin > 2 times ULN were excluded. 
2 ali več nenormalnih testov funkcije jeter (ALT, AST, AP, GGT ≥ 3-kratnika ULN), ali ALT ali AST > 5-kratnika ULN, ali celotni bilirubin > 2-kratnika ULN, so bili izključeni.
It is unknown whether lumacaftor and/or ivacaftor and metabolites are excreted in human milk. 
Ni znano, ali se lumakaftor in/ali ivakaftor in presnovki izločajo v materino mleko.
• Pack containing 28 film-coated tablets. 
• pakiranje, ki vsebuje 28 filmsko obloženih tablet;
No dose adjustment is necessary when CYP3A inhibitors are initiated in patients currently taking lumacaftor/ivacaftor. 
Prilagajanje odmerka ni potrebno, kadar uvajamo zaviralce CYP3A bolnikom, ki trenutno jemljejo lumakaftor/ivakaftor.
Patients received either lumacaftor/ivacaftor (n = 62) or placebo (n = 63) in addition to their prescribed CF therapies. 
Bolniki so poleg svojih predpisanih terapij CF prejemali bodisi lumakaftor/ivakaftor (n = 62) ali placebo (n = 63).
Patients received either lumacaftor 200 mg/ivacaftor 250 mg every 12 hours (n = 103) or placebo (n = 101) in addition to their prescribed CF therapies. 
Bolniki so prejemali poleg svojih predpisanih terapij CF bodisi lumakaftor 200 mg/ivakaftor 250 mg vsakih 12 ur (n = 103) ali placebo (n = 101).
Marketing Authorisation Holder Vertex Pharmaceuticals (Ireland) Limited 28-32 Pembroke Street Upper Dublin 2, D02 EK84 
IMETNIK DOVOLJENJA ZA PROMET Z ZDRAVILOM Vertex Pharmaceuticals (Ireland) Limited 28-32 Pembroke Street Upper
There was negligible urinary excretion of ivacaftor as unchanged drug. 
V urinu se je v obliki nespremenjenega zdravila izločila zanemarljiva količina ivakaftorja.
Ivacaftor did not cause developmental defects in the offspring of pregnant rats dosed orally from pregnancy through parturition and weaning at 100 mg/kg/day. 
Peri- in postnatalni razvoj Ivakaftor ni povzročil razvojnih okvar pri potomstvu brejih podgan, ki so peroralno prejemale ves čas brejosti, kotitve in odstavljanja od prsi odmerek po 100 mg/kg/dan.
NAME OF THE MARKETING AUTHORISATION HOLDER Vertex Pharmaceuticals (Ireland) Limited 
IME IMETNIKA DOVOLJENJA ZA PROMET Z ZDRAVILOM Vertex Pharmaceuticals (Ireland) Limited
Age, weight 
Starost, telesna masa
Patients may start taking Orkambi on any day of the week. 
Bolniki lahko začnejo jemati zdravilo Orkambi kateri koli dan v tednu.
Give Orkambi to your child for as long as your child's doctor recommends. 
Če ste prenehali otroku dajati zdravilo Orkambi Otroku dajajte zdravilo toliko časa, dokler to priporoča otrokov zdravnik.
Potential for other medicinal products to affect lumacaftor/ivacaftor Inhibitors of CYP3A 
Možnost, da bodo druga zdravila vplivala na lumakaftor/ivakaftor Zaviralci CYP3A
Pack size of 56 sachets (contains 4 individual wallets with 14 sachets per wallet). 
Velikost pakiranja 56 vrečic (vsebuje 4 posamezne zloženke s po 14 vrečicami na zloženko).
The treatment difference between lumacaftor 400 mg q12h alone and placebo evaluated as mean absolute change in ppFEV1 was -4.6 percentage points (95% CI: -9.6, 0.4) from baseline to Day 28, 4.2 percentage points (95% CI: -1.3, 9.7) from baseline to Day 56, and 7.7 percentage points (95% CI: 2.6, 12.8; statistically significant) from Day 28 to Day 56 (following the addition of ivacaftor to lumacaftor monotherapy). 
Razlika zaradi zdravljenja med lumakaftorjem 400 mg na 12 ur samim in placebom, ocenjena kot povprečna absolutna sprememba ppFEV1, je bila -4,6 odstotne točke (95 % IZ: -9,6, 0,4) od izhodišča do 28. dne, 4,2 odstotne točke (95 % IZ: -1,3, 9,7) od izhodišča do 56. dne in 7,7 odstotne točke (95 % IZ: 2,6, 12,8; statistično signifikantno) od 28. dne do 56. dne (po dodatku ivakaftorja monoterapiji z lumakaftorjem).
Patients with a history of colonisation with organisms such as Burkholderia cenocepacia, Burkholderia dolosa, or Mycobacterium abscessus or who had 3 or more abnormal liver function tests (ALT, AST, AP, GGT ≥ 3 times the ULN or total bilirubin ≥ 2 times the ULN) were excluded. 
kot so Burkholderia cenocepacia, Burkholderia dolosa ali Mycobacterium abscessus, ali ki so imeli 3 ali več nenormalnih testov funkcije jeter (ALT, AST, AP, GGT ≥ 3-krat ULN ali celotni bilirubin ≥ 2krat ULN), so izključili.
Ivacaftor impaired fertility and reproductive performance indices in male and female rats. 
Ivakaftor je zmanjšal indekse plodnosti in reproduktivne uspešnosti pri samcih in samicah podgan.
The most common adverse reactions experienced by patients aged 12 years and older who received lumacaftor/ivacaftor in the pooled, placebo-controlled, Phase 3 studies were dyspnoea (14.0% versus 7.8% on placebo), diarrhoea (11.0% versus 8.4% on placebo), and nausea (10.2% versus 7.6% on placebo). 
Najpogostejši neželeni učinki pri bolnikih, starih 12 let ali več, ki so prejemali lumakaftor/ivakaftor v združenih študijah 3. faze, kontroliranih s placebom, so bili dispneja (14,0 % proti 7,8 % pri placebu), driska (11,0 % proti 8,4 % pri placebu), in navzea (10,2 % proti 7,6 % pri placebu).
Treatment with lumacaftor/ivacaftor significantly decreased the risk for exacerbations requiring hospitalisation versus placebo by 61% (rate ratio=0.39, P < 0.0001; event rate per 48 weeks 0.17 for lumacaftor/ivacaftor and 0.45 for placebo) and reduced exacerbations requiring treatment with intravenous antibiotics by 56% (rate ratio = 0.44, P < 0.0001; event rate per 48 weeks 0.25 for lumacaftor/ivacaftor and 0.58 for placebo). 
Zdravljenje z lumakaftorjem/ivakaftorjem je značilno zmanjšalo tveganje poslabšanj, zaradi katerih je bila potrebna hospitalizacija, v primerjavi s placebom za 61 % (razmerje pogostnosti = 0,39, P < 0,0001; pogostnost dogodkov na 48 tednov 0,17 za lumakaftor/ivakaftor in 0,45 za placebo) in zmanjšalo poslabšanja, pri katerih je bilo potrebno zdravljenje z intravenskimi antibiotiki, za 56 % (razmerje pogostnosti = 0,44, P < 0,0001; pogostnost dogodkov na 48 tednov 0,25 za lumakaftor/ivakaftor in 0,58 za placebo).
A decline in ppFEV1 at initiation of therapy was observed during serial post dose spirometry assessments. 
Med serijskim spirometričnim testiranjem po prejemu odmerka so med uvajanjem zdravljenja ugotovili zmanjšanje ppFEV1.
Decrease in heart rate 
Zmanjšanje srčne frekvence
The exposure of lumacaftor increased approximately 2.0-fold when given with fat-containing food relative to fasted conditions. 
Izpostavljenost lumakaftorju se je v primerjavi s stanjem na tešče zvečala približno za dvakrat, ko so ga dajali z mastno hrano.
Orkambi 100 mg/125 mg granules in sachet One sachet in the morning; One sachet in the evening 
Orkambi 100 mg/125 mg zrnca v vrečici ena vrečica zjutraj; ena vrečica zvečer
In the pooled analysis, the rate ratio of exacerbations through week 24 in subjects treated with lumacaftor/ivacaftor (lumacaftor 400 mg/ivacaftor 250 mg q12h; n = 369) was 0.61 (P < 0.0001), representing a reduction of 39% relative to placebo. 
V združeni analizi je bilo razmerje pogostnosti poslabšanj do 24. tedna pri bolnikih, zdravljenih z lumakaftorjem/ivakaftorjem (lumakaftor 400 mg/ivakaftor 250 mg na 12 ur; n = 369) 0,61 (P < 0,0001), kar predstavlja zmanjšanje za 39 % glede na placebo.
In patients treated with lumacaftor/ivacaftor (mean baseline 114 mmHg systolic and 69 mmHg diastolic), the maximum increase from baseline in mean systolic and diastolic blood pressure was 3.1 mmHg and 1.8 mmHg, respectively. 
Pri bolnikih, zdravljenih z lumakaftorjem/ivakaftorjem (srednji izhodiščni sistolični tlak 114 mm Hg in srednji izhodiščni diastolični tlak 69 mm Hg), je bil največji porast srednjega sistoličnega in diastoličnega krvnega tlaka od izhodišča 3,1 mm Hg oziroma 1,8 mm Hg.
* Adverse reactions and frequencies observed in patients in clinical studies with ivacaftor monotherapy (a component of 
*Neželeni učinki in njihova pogostnost, ki so jih ugotovili pri bolnikih v kliničnih študijah z monoterapijo z ivakaftorjem (sestavina zdravila Orkambi).
Patients with CF who are heterozygous for the F508del mutation in the CFTR gene Lumacaftor/ivacaftor is not effective in patients with CF who have the F508del mutation on one allele plus a second allele with a mutation predicted to result in a lack of CFTR production or that is not responsive to ivacaftor in vitro (see section 5.1). 
Bolniki s CF, ki so heterozigotni za mutacijo F508del na genu CFTR Lumakaftor/ivakaftor ni učinkovit pri bolnikih s CF, ki imajo mutacijo F508del na enem alelu, na drugem alelu pa mutacijo, za katero je napovedano, da bo povzročila pomanjkanje izdelave CFTR, ali ki ni odzivna na ivakaftor in vitro (glejte poglavje 5.1).
Orkambi 150 mg/188 mg granules in sachet One sachet in the morning; One sachet in the evening 
Orkambi 150 mg/188 mg zrnca v vrečici ena vrečica zjutraj; ena vrečica zvečer
It is unknown if lumacaftor or ivacaftor are found in human milk. 
Ni znano, ali se lumakaftor ali ivakaftor izloča v materino mleko.
• Anticoagulants (used to prevent blood clots from forming or growing larger in blood and blood vessels) for example: warfarin, dabigatran 
• antikoagulanti (uporabljajo se za preprečevanje nastajanja ali rasti krvnih strdkov v krvi in krvnih žilah), na primer:
• Pack size of 56 sachets (contains 4 individual wallets with 14 sachets per wallet) 
• Velikost pakiranja 56 vrečic (vsebuje 4 posamezne zloženke s po 14 vrečic na zloženko).
MARKETING AUTHORISATION NUMBER(S) EU/1/15/1059/001 112 film-coated tablets EU/1/15/1059/002 56 film-coated tablets 
ŠTEVILKA(E) DOVOLJENJA (DOVOLJENJ) ZA PROMET EU/1/15/1059/001 112 filmsko obloženih tablet EU/1/15/1059/002 56 filmsko obloženih tablet
If your child has poor lung function your child's doctor may monitor your child more closely when she/he starts Orkambi. 
Če ima vaš otrok slabše delovanje pljuč, ga bo zdravnik mogoče skrbneje spremljal, ko bo začel jemati zdravilo
The treatment difference (LS mean) in sweat chloride for the absolute change at Week 24 as compared to placebo was -24.9 mmol/L (nominal P < 0.0001). 
kloridov v znoju za absolutno spremembo v 24. tednu v primerjavi s placebom -24,9 mmol/l (nominalna P < 0,0001).
No dose adjustment is recommended when used with moderate or weak CYP3A inducers. 
Pri uporabi z zmernimi ali šibkimi induktorji CYP3A prilagajanja odmerka ne priporočajo.
MARKETING AUTHORISATION NUMBER(S) EU/1/15/1059/003 28 film-coated tablets 
ŠTEVILKA(E) DOVOLJENJA (DOVOLJENJ) ZA PROMET EU/1/15/1059/003 28 filmsko obloženih tablet
See section 4.5 for interactions with immunosuppressants. 
Glede medsebojnega delovanja z imunosupresivi glejte poglavje 4.5.
Absolute change from baseline in stature-for-age z-score n = 57 95% CI: 0.02, 0.15; P = 0.0104 
Absolutna sprememba telesne višine za starost od izhodišča z-vrednost n = 57 95 % IZ: 0,02, 0,15; P = 0,0104
Lumacaftor/ivacaftor will decrease the exposure of these immunosuppressants, which may reduce the efficacy of these immunosuppressants. 
Lumakaftor/ivakaftor bo zmanjšal izpostavljenost tem imunosupresivom, kar lahko zmanjša učinkovitost teh imunosupresivov.
After oral administration of lumacaftor 400 mg q12h/ivacaftor 250 mg q12h in a fed state, the steady-state mean (± SD) for AUC0-12h and Cmax were 198 (64.8) µg∙hr/mL and 25.0 (7.96) µg/mL for lumacaftor, respectively, and 3.66 (2.25) µg∙hr/mL and 0.602 (0.304) µg/mL for ivacaftor, respectively. 
Po peroralnem dajanju lumakaftorja 400 mg na 12 ur/ivakaftorja 250 mg na 12 ur po obroku hrane sta bili povprečji (±SD) AUC0-12h oziroma Cmax v stanju dinamičnega ravnovesja 198 (64,8) µg∙h/ml oziroma 25,0 (7,96) µg/ml za lumakaftor in 3,66 (2,25) µg∙h/ml oziroma 0,602 (0,304) µg/ml za ivakaftor.