inborn-errors-of-metabolism63

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THE USUAL COURSE OF NEONATAL HYPERAMMONEMIA-5• Pharmacological“cocktail”Starts (continues) PharmacologicalTreatment• Waiting for…RESPONSENO RESPONSE• Dialysis• Have we beensuccessful?RE-FEEDINGDIALYSISOUTCOME ANALYSIS
PROGNOSTIC INDICATORS: SURVIVALMcBryde,2006Bachman,2003Uchino,1998Schaefer,1999Picca,2001•pNH 4at admission<180 µmol/L•Time to RRT<24 hrs•Medical treatment<24 hrs•BP> 5%ile at RRT initiation•HD initial RRT (trend)•pNH 4at admission<300 µmol/L•Peak pNH 4<480 µmol/L•pNH 4at admission<180 µmol/L•50% pNH 4decay time < 7 hrs•(catheter > 5F)•pre-treatment coma duration < 33 hrs (no influence of post-treatment duration)•responsiveness to pharmacological therapyPela,2008• pre-treatment coma duration < 10 hrs
Page 1 and 2: 5th International Conference on Ped
Page 3 and 4: KEY POINTS FACING TO A HYPERAMMONEM
Page 5 and 6: THE USUAL COURSE OF NEONATAL HYPERA
Page 11 and 12: 100 CAVHD patientsNH4p (percent of
Page 13: Dialysis in hyperammonemia:the “b
Page 17 and 18: 65n= 484patients32101986 1988 1990
Page 19 and 20: DESCRIPTIVE (2)Non continuous varia
Page 21 and 22: 10080Ammonia Decay (%)604020PD00 8
Page 23 and 24: DEP. VARIABLE 2: DEVELOPMENT AT 2 Y
Page 25 and 26: CONCLUSIONS-DIALYSIS• PD provides
Page 27 and 28: Outcome Neonatal Onset ptsShort-ter

inborn-errors-of-metabolism63

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