natgillin/translations-raw huggingface dataset:
Hos patienter med NMOSD fører ukontrolleret terminal komplementaktivering forårsaget af autoantistoffer mod AQP4 til dannelsen af MAC- og C5a-afhængig inflammation, hvilket fører til astrocytnekrose og øget permeabilitet af blod-hjerne-barrieren samt død af omgivende oligodendrocytter og neuroner. 
In patients with NMOSD, uncontrolled terminal complement activation caused by autoantibodies against AQP4 leads to the formation of the MAC and C5a-dependent inflammation which results in astrocyte necrosis and increased permeability of the blood brain barrier, as well as death of the surrounding oligodendrocytes and neurons.
b Meningokokinfektion omfatter den følgende gruppe af foretrukne betegnelser: Meningokok-infektion, meningokok-sepsis, meningokok-meningitis, Neisseria-infektion. 
Meningococcal infection includes the following group of PTs: Meningococcal infection, Meningococcal sepsis, Meningitis meningococcal, Neisseria infection.
Eculizumabs farmakokinetik blev evalueret i studie M07-005 hos pædiatriske PNH-patienter (i alderen fra 11 år til under 18 år) og i studie C08-002, C08-003, C09-001r og C10-003 hos pædiatriske patienter med aHUS (i alderen fra 2 måneder til under 18 år) med et kropsvægtbaseret dosisregime. 
The pharmacokinetics of eculizumab was evaluated in Study M07-005 in PNH paediatric patients (aged from 11 to less than 18 years) and in Studies C08-002, C08-003, C09-001r and C10-003 in aHUS pediatric patients (aged 2 months to less than 18 years) with body-weight based dose regimen.
I alt 35 patienter fik PE/PI før eculizumab. 
A total of 35 patients received PE/PI prior to eculizumab.
Den anbefalede dosis overføres til en infusionspose. 
Transfer the recommended dose to an infusion bag.
På grund af virkningsmekanismen bør forsigtighed udvises ved anvendelse af Soliris til patienter med aktive systemiske infektioner. 
Due to its mechanism of action, Soliris therapy should be administered with caution to patients with active systemic infections.
Mediantiden fra aHUS-diagnose til første Soliris-dosis var 14 måneder (interval <1 til 110 måneder). 
The median time from aHUS diagnosis to first dose of Soliris was 14 months (range <1, 110 months).
• behovet for at patienter vaccineres mod Neisseria meningitidis-infektion to uger før de modtager eculizumab og/eller at de modtager profylaktisk antibiotisk behandling. 
• The need for patients to be vaccinated against Neisseria meningitidis two weeks prior to receiving eculizumab and/or to receive antibiotic prophylaxis.
2 Årvågenhed over for meningokokinfektion og andre Neisseria-infektioner), skal du straks informere lægen. 
If you experience any of the meningococcal infection symptoms (see section 2 Meningococcal and other Neisseria infections alert), you should immediately inform your doctor.
Ændring fra baseline efter 12 uger i LDHværdi (E/l) 
Change from baseline at 12 weeks of LDH Value (U/L)
Ved maternel eksponering for antistoffet under organogenesen blev der iagttaget to tilfælde af retinal dysplasi og ét tilfælde af umbilikalhernie blandt 230 unger af hunner, der blev eksponeret for den høje antistofdosis (ca. 4 gange den anbefalede maksimale humane Soliris-dosis, baseret på kropsvægt). 
When maternal exposure to the antibody occurred during organogenesis, two cases of retinal dysplasia and one case of umbilical hernia were observed among 230 offspring born to mothers exposed to the higher antibody dose (approximately 4 times the maximum recommended human Soliris dose, based on a body weight comparison); however, the exposure did not increase foetal loss or neonatal death.
Forholdet mellem den adjudikerede annualiserede recidivrate (ARR) i løbet af studiet (ARR) (95 % CI) for eculizumab sammenlignet med placebo var 0,045 (0,013; 0,151), hvilket udgør en 95,5 % relativ reduktion i adjudikeret ARR i løbet af studiet for patienter i behandling med eculizumab sammenlignet med placebo (p<0,0001) (tabel 13). 
The adjudicated on-trial annualized relapse rate (ARR) ratio (95% CI) for eculizumab compared with placebo was 0.045 (0.013, 0.151), representing a 95.5% relative reduction in adjudicated On-trial ARR for patients treated with eculizumab compared with placebo (p<0.0001) (Table 13).
Når behandlinger med immunsuppressive midler og anticholinesterase reduceres eller seponeres, skal patienterne overvåges nøje for tegn på sygdomsforværring. 
When immunosuppressant and anticholinesterase therapies are decreased or discontinued, patients should be monitored closely for signs of disease exacerbation.
Tabel 9: Patientdemografi og karakteristika i studie ECU-MG-301 
Table 9: Patient Demographic and Characteristics in Study ECU-MG-301
• risikoen for reaktioner som følge af infusion, inklusive anafylaktisk shock, og behovet for klinisk post-infusion monitorering. 
• The risk of infusion reactions with eculizumab, including anaphylaxis, and the need for clinical monitoring post-infusion.
Tidligere ventilatorstøtte siden diagnose, n 
Any prior ventilator support since diagnosis,
• Den anbefalede dosis overføres til en infusionspose. 
• Transfer the recommended dose to an infusion bag.
Der er ikke udført dyrereproduktionsstudier med eculizumab (se pkt. 
Animal reproduction studies have not been conducted with eculizumab (see section 5.3).
• alvorlig allergisk reaktion, der forårsager vejrtrækningsbesvær eller svimmelhed (anafylaktisk reaktion), overfølsomhed 
• serious allergic reaction which causes difficulty in breathing or dizziness (anaphylactic reaction),
Seponering af behandlinger med immunsuppressive midler og anticholinesterase under behandling med Soliris for refraktær gMG blev ikke vurderet i de placebokontrollerede studier. 
Withdrawal of immunosuppressant and anticholinesterase therapies during Soliris treatment for refractory gMG was not assessed in the placebocontrolled studies.
Hos patienter med NMOSD angribes og beskadiges øjennerverne og rygmarven af kroppens eget immunsystem, hvilket kan føre til blindhed i det ene eller i begge øjne, svaghed eller lammelse i arme og ben, smertefulde spasmer, følelsesløshed og markant hæmning af dagligdags aktiviteter. 
In patients with NMOSD, their eye nerve and spinal cord are attacked and damaged by the immune system which can lead to blindness in one or both eyes, weakness or paralysis in the legs or arms, painful spasms, loss of sensation, and markedly impaired activities of daily living.
Kontakt lægen, hvis du får nogen af disse symptomer. 
If you have any of these symptoms, contact your doctor.
I alle kliniske studier var den alvorligste bivirkning meningokoksepsis, som er et almindeligt symptom på meningokokinfektioner hos patienter behandlet med Soliris (se pkt. 
In all clinical studies, the most serious adverse reaction was meningococcal sepsis which is a common presentation of meningococcal infections in patients treated with Soliris (see section 4.4).
Når behandlingen blev fortsat ud over 26 uger, opnåede og opretholdt yderligere to patienter et fuldstændigt TMA-respons på grund af normalisering af LDH (1 patient) og reduktion i serum-kreatinin (2 patienter). 
When treatment was continued for more than 26 weeks, two additional patients achieved and maintained Complete TMA response due to normalization of LDH (1 patient) and a decrease in serum creatinine (2 patients).
Studie ECU-NMO-301 var et dobbeltblindet, randomiseret, placebokontrolleret fase 3 multicenterstudie af Soliris hos patienter med NMOSD. 
Study ECU-NMO-301 was a double-blind, randomized, placebo-controlled, multi-center, Phase 3 study of Soliris in patients with NMOSD.
Soliris (eculizumab) er ikke blevet undersøgt til behandling af akutte recidiver hos NMOSD-patienter. 
Soliris (eculizumab) has not been studied for the treatment of acute relapses in NMOSD patients.
Proteiner kan hos nogle fremkalde allergiske reaktioner. 
Allergic reactions Soliris contains a protein and proteins can cause allergic reactions in some people.
Tabel 5: Patientdemografi og karakteristika i C08-002A/B og C08-003A/B Parameter 
Table 5: Patient Demographics and Characteristics in C08-002A/B and C08-003A/B
Et hundrede og atten (118) af de 125 (94 %) patienter gennemførte den 26ugers behandlingsperiode, og 117 (94 %) patienter indgik efterfølgende i studie ECU-MG-302, et åbent multicenter-forlængelsesstudie til vurdering af langtidssikkerhed og -virkning, hvor alle patienterne fik behandling med Soliris. 
One hundred and eighteen (118) of the 125 (94%) patients completed the 26-week treatment period and 117 (94%) patients subsequently enrolled in Study ECU-MG-302, an open-label, multi-center long-term safety and efficacy extension study in which all patients received Soliris treatment.
I alt (N = 143) 
(N = 143)
Hæmolyse*, abnorm koagulationsfaktor, agglutination af røde blodlegemer, koagulopati 
Haemolysis*, Abnormal clotting factor, Red blood cell agglutination, Coagulopathy
Wilcoxon Signed Rank 
P - Value Mean (SD) Wilcoxon Signed Rank
Årvågenhed over for meningokok- og andre Neisseria-infektioner Soliris kan svække din naturlige modstandskraft over for infektioner, især med visse organismer, der er årsag til meningokokinfektion (svær hjernehindebetændelse og blodforgiftning) og andre Neisseriainfektioner, herunder dissemineret gonorré. 
Meningococcal and other Neisseria infections alert Soliris treatment may reduce your natural resistance to infections, especially against certain organisms that cause meningococcal infection (severe infection of the linings of the brain and sepsis) and other Neisseria infections including disseminated gonorrhea.
Baseline-karakteristika er vist i tabel 2. 
Baseline characteristics are shown in Table 2.
Tabel 4: Effektresultater (LDH-niveau og FACIT-træthedsscore) hos PNH-patienter uden transfusion i anamnesen i PNH-register M07-001 
Table 4: Efficacy Outcomes (LDH level and FACIT-Fatigue) in Patients with PNH with No History of Transfusion in M07-001
2 til <12 år (n=10) 
2 to <12 years (n=10)
I det ikke-kontrollerede studie C04-002 (SHEPHERD) blev PNH-patienter, der havde fået mindst én transfusion de foregående 24 måneder og havde et trombocyttal på mindst 30 000/μl, behandlet med Soliris i en periode på 52 uger. 
In the non-controlled study C04-002 (SHEPHERD), PNH patients with at least one transfusion in the prior 24 months and at least 30,000 platelets/microliter received Soliris over a 52-week period.
Hvis du kommer ud for nogen af nedenstående symptomer, skal du straks henvende dig til lægen: hovedpine med kvalme eller opkastning hovedpine med nakke- eller rygstivhed feber udslæt omtågethed stærk muskelømhed samtidig med influenzalignende symptomer lysfølsomhed. 
If you experience any of the following symptoms, you should immediately inform your doctor: - headache with nausea or vomiting - headache with a stiff neck or back - fever - rash - confusion - severe muscle aches combined with flu-like symptoms
Det primære endepunkt for studie ECU-MG-301 var ændringen fra baseline i MG-Activities of Daily Living-profilen (MG-ADL - en patientrapporteret udfaldsmåling valideret i gMG) total score ved uge 26. 
The primary endpoint for Study ECU-MG-301 was the change from baseline in the MG Activities of Daily Living Profile (MG-ADL - a patient reported outcome measure validated in gMG) total score at Week 26.
Soliris produceres i et ekspressionssystem for murint myelom (NS0 cellelinje) og renses ved affinitetsog ionbytningskromatografi. 
Soliris is produced in a murine myeloma (NS0 cell line) expression system and purified by affinity and ion exchange chromatography.
Før iværksættelse af Soliris-behandling anbefales det, at PNH-, aHUS-, refraktær gMG- og NMOSDpatienter påbegynder vaccination i henhold til de gældende retningslinjer. 
Prior to initiating Soliris therapy, it is recommended that PNH, aHUS, refractory gMG and NMOSD patients initiate immunizations according to current immunization guidelines.
I alt 15 pædiatriske patienter (i alderen 2 måneder til 12 år) fik Soliris i aHUS-studiet C09-001r. 
A total of 15 paediatric patients (aged 2 months to 12 years) received Soliris in aHUS Study C09-001r.
• Opløsningen inspiceres visuelt for partikler og misfarvning. 
• Inspect visually Soliris solution for particulate matter and discolouration.
Sammenlignet med patienter, der fik placebo, havde patienter, der fik Soliris, reducerede annualiserede rater af indlæggelse (0,04 for Soliris vs. 0,31 for placebo) af intravenøse kortikosteroidadministrationer for at behandle akutte recidiver (0,07 for Soliris vs. 0,42 for placebo), og for behandling med plasmaudskiftning (0,02 for Soliris vs. 0,19 for placebo). 
Compared to placebo-treated patients, Soliris-treated patients had reduced annualized rates of hospitalizations (0.04 for Soliris versus 0.31 for placebo), of intravenous corticosteroid administrations to treat acute relapses (0.07 for Soliris versus 0.42 for placebo), and of plasma exchange treatments (0.02 for Soliris versus 0.19 for placebo).
4 af de 5 patienter, der havde behov for dialyse ved studiets begyndelse, kunne ophøre med dialysebehandling under Soliris-behandlingen; 1 patient udviklede behov for dialyse. 
Four of the five patients who required dialysis at study entry were able to discontinue dialysis for the duration of Soliris treatment, and one patient developed a new dialysis requirement.
Primære endepunkter omfattede ændring af trombocyttallet i forhold til baseline i studie C08-002A/B og TMA-hændelsesfri tilstand i studie C08-003A/B. 
Primary endpoints included platelet count change from baseline in study C08-002A/B and thrombotic microangiopathy (TMA) event-free status in study C08-003A/B.
I alt 10 patienter fik PE/PI før eculizumab. 
A total of 10 patients received PE/PI prior to eculizumab.
Varighed af MG (år), 
Age at MG Diagnosis (years),
Længerevarende behandling med Soliris (median: 52 uger; interval: fra 15 uger til 126 uger) var forbundet med en øget forekomst af klinisk betydningsfulde forbedringer hos voksne patienter med aHUS. 
Longer term treatment with Soliris (median 52 weeks ranging from 15 to 126 weeks) was associated with an increased rate of clinically meaningful improvements in adult patients with aHUS.
• Den fortyndede opløsning skal henstå, til den er nået op på rumtemperatur (18 °C - 25 °C), før den administreres. 
• The diluted solution should be allowed to warm to room temperature [18°C - 25°C] prior to administration by exposure to ambient air.
PE/PI-hændelsesfri tilstand, n (%) 'Ny dialyse'-hændelsesfri tilstand, n (%) 
PE/PI Event-Free Status, n (%) New Dialysis Event-Free Status, n (%)
Normalisering af trombocyttal Alle patienter, n (%) (95 % CI) Patienter med unormal baseline, n/n (%) TMA-hændelsesfri tilstand, n (%) (95 % CI) Forekomst af TMAintervention Daglige værdier før eculizumab, median (min., maks.) 
Normalization of platelet count All patients, n (%) (95% CI) Patients with abnormal baseline, n/n (%) TMA event-free status, n (%) (95% CI) TMA intervention rate Daily preeculizumab rate, median (min, max) Daily duringeculizumab rate, median (min, max) P-value
Tidspunkt for supplerende Soliris-dosis 
Supplemental Soliris Dose
Tabel 14: Effektresultater i det pædiatriske PNH-studie M07-005 
Table 14: Efficacy Outcomes in Paediatric PNH Study M07-005
Den gennemsnitlige clearance var 0,31 ± 0,12 ml/h/kg, det gennemsnitlige fordelingsvolumen 110,3 ± 17,9 ml/kg og den gennemsnitlige halveringstid 11,3 ± 3,4 dage. 
Mean clearance was 0.31 ± 0.12 mL/hr/kg, mean volume of distribution was 110.3 ± 17.9 mL/kg, and mean elimination half-life was 11.3 ± 3.4 days.
Børn og unge med PNH eller aHUS, som vejer under 40 kg, kræver en mindre dosis, baseret på deres vægt. 
Children and adolescents with PNH or aHUS and who are under 40 kg weight require a lower dose based on how much they weigh.
Virkningen af eculizumab-behandling hos pædiatriske PNH-patienter synes at være i overensstemmelse med, hvad der er observeret hos voksne PNH-patienter, der indgik i de pivotale PNH-studier (C04-001 og C04-002) (tabel 3 og 14). 
The efficacy of eculizumab treatment in paediatric PNH patients appears to be consistent with that observed in adult PNH patients enrolled in PNH pivotal Studies (C04-001 and C04-002) (Table 3 and
For voksne patienter med Ahus, refraktær gMG og NMOSD og pædiatriske patienter med aHUS er supplerende Soliris-doser påkrævet ved samtidig plasmaferese/plasmaudskiftning eller infusion af frisk frosset plasma (PE/PI): 
For adult aHUS, refractory gMG and NMOSD patients and paediatric aHUS patients supplemental dosing of Soliris is required in the setting of concomitant PE/PI (plasmapheresis or plasma exchange, or fresh frozen plasma infusion):
Tid fra første kliniske præsentation af NMOSD til den første dosis studielægemiddel (år) 
Time from NMOSD initial clinical presentation to first dose of study drug (years)
Længerevarende behandling med Soliris (median: 55 uger; interval: fra 1 dag til 107 uger) var forbundet med en øget forekomst af klinisk betydningsfulde forbedringer hos pædiatriske og unge patienter med aHUS. 
Longer term treatment with Soliris (median 55 weeks ranging from 1day to 107 weeks) was associated with an increased rate of clinically meaningful improvements in paediatric and adolescent patients with aHUS.
Dosisregimet til voksne patienter med aHUS, refraktær gMG og NMOSD (≥ 18 år) består af en 4-ugers startfase efterfulgt af en vedligeholdelsesfase: 
The aHUS, refractory gMG and NMOSD dosing regimen for adult patients (≥18 years of age) consists of a 4 week initial phase followed by a maintenance phase:
Trods genopstart af Soliris efter behandlingsafbrydelse oplevede én patient progression til dialysekrævende nyresygdom i slutstadiet. 
Despite Soliris re-initiation following discontinuation, progression to end stage renal disease occurred in one patient.
Til og med data-afslutning (12. oktober, 2012), med median Soliris-behandlingsvarighed på ca. 44 uger (interval: 1 dosis til 88 uger). 
Through data cut-off (October 12, 2012), with median duration of Soliris therapy of 44 weeks (range: 1 dose to 88
Det anbefales, at behandlingen med Soliris fortsættes i hele patientens levetid, medmindre der er klinisk indikation for afbrydelse (se pkt. 
Soliris treatment is recommended to continue for the patient's lifetime, unless the discontinuation of Soliris is clinically indicated (see section 4.4).
C5-ekspressionen i de undersøgte humane væv er forenelig med publicerede beskrivelser af C5-ekspression, da C5 er beskrevet både i glatmuskulatur, tværstribet muskulatur og epitel fra de proksimale nyretubuli. 
C5 expression in the human tissue panel examined in this study is consistent with published reports of C5 expression, as C5 has been reported in smooth muscle, striated muscle, and renal proximal tubular epithelium.
Soliris er et rekombinant, humaniseret, monoklonalt IgG2/4κ-antistof, der bindes til humant komplementprotein C5 og hæmmer den terminale komplementaktivering. 
Soliris is a recombinant humanised monoclonal IgG2/4k antibody that binds to the human C5 complement protein and inhibits the activation of terminal complement.
Tabel 12: Patientens anamnese og baseline-karakteristika i studie ECU-NMO-301 Variabel 
Table 12: Patient Disease history and Baseline Characteristics in Study ECU-NMO-301 Variable
I studie ECU-MG-301 blev en person med klinisk respons i QMG total score defineret som en person med en forbedring på mindst 5 point. 
In Study ECU-MG-301, a clinical responder in the QMG total score was defined as having at least a 5point improvement.
PNH-registret viste, at de patienter, der blev behandlet med Soliris, oplevede en reduktion i hæmolyse og dermed forbundne symptomer. 
In the PNH Registry, patients treated with Soliris were observed to have a reduction in haemolysis and associated symptoms.
• spontan peniserektion 
• spontaneous penile erection
Obstipation, dyspepsi, abdominal distension 
Constipation, Dyspepsia, Abdominal distension
Data fra 143 patienter i et kontrolleret studie (studie ECU-NMO-301) og fra 119 patienter, som fortsatte i et åbent forlængelsesstudie (studie ECU-NMO-302), blev anvendt til at evaluere virkning og sikkerhed ved Soliris ved behandling af patienter med NMOSD. 
Data from 143 patients in one controlled study (Study ECU-NMO-301) and from 119 patients who continued in one open-label extension trial (Study ECU-NMO-302) were used to evaluate the efficacy and safety of Soliris in the treatment of patients with NMOSD.
• unormal udsivning af det infunderede lægemiddel fra blodåren, unormal fornemmelse på infusionsstedet, varmefornemmelse 
• abnormal leakage of the infused drug out of the vein, infusion site abnormal sensation, feeling hot
Afbrydelse af aHUS-behandlingen 
Treatment Discontinuation for aHUS
Soliris reducerede tegn på komplementmedieret TMAaktivitet, vist ved en stigning i det gennemsnitlige trombocyttal fra baseline til 26 uger. 
Soliris reduced signs of complement-mediated TMA activity, as shown by an increase in mean platelet counts from baseline to 26 weeks.
• reaktioner i forbindelse med infusionen 
• infusion related reaction
Paroksystisk nokturn hæmoglobinuri (PNH): 
In Paroxysmal Nocturnal Haemoglobinuria (PNH):
- hvis du ikke er vaccineret mod meningokok-infektion, medmindre du tager antibiotika i 2 uger efter, du er blevet vaccineret, for at nedsætte risikoen for infektion. 
- If you have not been vaccinated against meningococcal infection unless you take antibiotics to reduce the risk of infection until 2 weeks after you have been vaccinated .
LDH-niveau før behandling (median, E/l) Fri hæmoglobin ved baseline (median, mg/dl) 
Pre-treatment LDH levels (median, U/L) Free Haemoglobin at baseline (median, mg/dL)
Ændring fra baseline efter 12 uger i frit hæmoglobin i plasma (mg/dl) Ændring fra baseline i type III RBCklonstørrelse (procent abnorme celler) Ændring fra baseline efter 12 uger i PedsQLTM4.0 Generic Core-skala (patienter) 
Change from baseline at 12 weeks in Plasma Free Haemoglobin (mg/dL) Change from baseline Type III RBC clone size (Percent of aberrant cells) Change from baseline at 12 weeks of PedsQLTM4.0 Generic Core scale (patients) 10.5 (6.66)
Hos patienter med refraktær gMG forårsager ukontrolleret terminal komplementaktivering membranangrebskompleks (MAC)-afhængig lysis og C5a-afhængig inflammation i de neuromuskulære synapser, hvilket fører til svigtende neuromuskulær transmission. 
In refractory gMG patients, uncontrolled terminal complement activation causes membrane attack complex (MAC) dependent lysis and C5a-dependent inflammation at the Neuromuscular Junction (NMJ) leading to failure of neuromuscular transmission.
Hæmatologiske normalisering blev defineret som normalisering af trombocyttal og LDH-niveauer i ≥ 2 på hinanden følgende målinger og opretholdt i ≥ 4 uger. 
Haematologic normalization was defined as normalization of platelet counts and LDH levels sustained for ≥2 consecutive measurements for ≥4 weeks.
Trafik- og arbejdssikkerhed Soliris påvirker ikke eller kun i ubetydelig grad evnen til at føre motorkøretøj og betjene maskiner. 
Driving and using machines Soliris has no or negligible influence on the ability to drive and use machines.
Tabel 9 viser baseline-karakteristika for patienterne med refraktær gMG, der indgik i studie ECU-MG 
Table 9 presents the baseline characteristics of the refractory gMG patients enrolled in Study ECU-MG
Læger, som påtænker at ordinere Soliris, skal læse instruktionsfolderen vedrørende ordination. 
All physicians who intend to prescribe Soliris must ensure they are familiar with the physician's guide to prescribing.
For at mindske infektionsrisikoen skal alle patienter vaccineres mindst 2 uger, før de får Soliris, medmindre risikoen ved at udskyde behandling med Soliris opvejer risici for udvikling af meningokokinfektion. 
To reduce the risk of infection, all patients must be vaccinated at least 2 weeks prior to receiving Soliris unless the risk of delaying Soliris therapy outweighs the risks of developing a meningococcal infection.