Suppl. Table 3. Evidence table of systematic literature review.doc

++,1+,1-,2++. NoneLevel2+. Evidencefromwell-conductedcase-controlorcohortstudieswithalowriskofconfounding,bias,ölkeretal(2006)PediatrRes;59:840-,e,andtreatmentefficacyinchildrenandadultswithglutaryl-:-Questionaire-basedevaluationofn=279patientsfromEurope,America,-dependentand–independentvariables.-Non-parametricstatisticalanalysis;-n=61asymptomaticpatients(neonatalscreening,high-riskscreening,siblings,macrocephaly);n=218symptomaticpatients(risis,insidious-onset)risis-urreduntilage3years;nocrisiswasreportedafterage6years;-Mostpatients(70%)-Approximately20%ofallpatientsdied.-Thecauseofdeathremainedoftenunknown,pneumoniawasthemostfrequentlyconsideredcauseofdeath;-:-Earlydiagnosis(<3monthsofage),-Oralcarnitinesupplementation-Lysine-restricteddietNoeffect:-Riboflavin-Protein-restricteddietNotevaluated:-Emergencytreatmentcouldnotbeevaluatedbythisapproach,sinceimportantparameters(hoursofdelay,exactprotocolsused)--Natureofinsidious-onsettype(distinctdiseasecourseorabortivecrisis);--. Evidencefromcase-controlorcohortstudieswithahighriskofconfounding,bias,(2000)JPediatr137 :681-:-Meta-analysisofpreviouslypublishedcasereportsandcohortstudies(n