Table 2

Acute metabolic and neurological crises: mortality rate and recurrences

Crisis numberPatients at riskAbsence of crisisOccurrence of crisisMedian age (months)Deaths/survivalMortality (%)
  • * Information concerning circumstances of death was not available for six patients who died several decades ago.

  • Two of the 23 survivors of a first crisis died in undocumented circumstances. They are excluded from the following rows of the table.

  • One surviving patient (the genetic compound of A354V and C1277Xdel8) experienced seven crises.

  • § Patients with no further crises all survived. The 10 survivors include the 9 shown in the above column plus the one patient who survived >4 crises.