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trots behandling kan akut hyperammonemisk encefalopati inträffa hos ett antal patienter.
even on therapy, acute hyperammonaemic encephalopathy may occur in a number of patients.
Last Update: 2017-04-26
Usage Frequency: 3
Quality:
Även under behandling med glycerolfenylbutyrat kan akut hyperammonemi, inklusive hyperammonemisk encefalopati, uppstå hos en viss andel patienter.
even while on treatment with glycerol phenylbutyrate, acute hyperammonaemia including hyperammonaemic encephalopathy may occur in a proportion of patients.
Last Update: 2017-04-26
Usage Frequency: 1
Quality:
om patienten är i hyperammonemisk koma ges carbaglu genom att snabbt pressa dosen genom en spruta via den sond som lagts in för näringstillförsel.
when the patient is in hyperammonaemic coma, carbaglu is administered by fast push through a syringe via the tube set up and used to feed you.
Last Update: 2017-04-26
Usage Frequency: 3
Quality:
det är också indicerat hos patienter med sen sjukdomsdebut (partiell enzymbrist, som debuterar efter första levnadsmånaden), som uppvisar hyperammonemisk encefalopati.
it is also indicated in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonaemic encephalopathy.
Last Update: 2017-04-26
Usage Frequency: 3
Quality:
hos patienter som har diagnostiserats under fostertiden och som har behandlats innan någon episod av hyperammonemisk encefalopati har inträffat var överlevnaden 100 %, men även flertalet av dessa patienter uppvisade kognitiva brister eller andra neurologiska rubbningar.
in patients diagnosed during gestation and treated prior to any episode of hyperammonaemic encephalopathy, survival was 100 %, but even in these patients, many subsequently demonstrated cognitive impairment or other neurologic deficits.
Last Update: 2017-04-26
Usage Frequency: 3
Quality:
hos patienter med sen debut av bristtillståndet, inklusive kvinnliga patienter som är heterozygota för ornitintranskarbamylasbrist, vilka har återhämtat sig från hyperammonemisk encefalopati och sedan fått kronisk behandling med natriumfenylbutyrat och proteinreducerad kost var överlevnaden 98 %.
in late-onset deficiency patients, including females heterozygous for ornithine transcarbamylase deficiency, who recovered from hyperammonaemic encephalopathy and were then treated chronically with dietary protein restriction and sodium phenylbutyrate, the survival rate was 98 %.
Last Update: 2017-04-26
Usage Frequency: 3
Quality:
av 51 vuxna patienter som deltog i studie 2 rapporterade 7 patienter (14 %) totalt 10 hyperammonemiska kriser under behandling med glycerolfenylbutyrat, jämfört med 9 patienter (18 %) som hade rapporterat totalt 15 kriser under de 12 månaderna före inskrivning i studien medan de behandlades med natriumfenylbutyrat.
of 51 adult patients participating in study 2, 7 patients (14%) reported a total of 10 hyperammonaemic crises during treatment with glycerol phenylbutyrate as compared with 9 patients (18 %) who had reported a total of 15 crises in the 12 months prior to study entry while they were being treated with sodium phenylbutyrate.
Last Update: 2017-04-26
Usage Frequency: 1
Quality: