Escolar et al developed a staging system for children with infantile Krabbe disease based on clinical progression. This can help to identify patients likely to benefit from umbilical cord blood transplantation (UCBT). The authors are from the University of North Carolina at Chapel Hill and Duke University.


Enzyme defect: galactocerebrosidase


Patient selection: infantile


Clinical Findings

Clinical Stage

normal development without neurologic signs

Stage 1

normal development with minor, inconclusive neurologic findings

Stage 1

definite neurologic findings that are mild

Stage 2

moderate to severe neurologic disease

Stage 3

advanced neurologic deterioration

Stage 4


An infant in Stage 1 or 2 is a candidate for UCBT and has a good chance for a good survival if the transplant is performed if transplanted during the first weeks of life.


An infant in Stage 3 or 4 is not because of the rate of severe disability or death.


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