Krabbe disease (galactocerebrosidase deficiency) is an inherited leukodystrophy that results in severe neurological defects due to altered myelination. Classically, disease onset is within the first year of life. Juvenile and adult-onset cases may have less classic presentations, making diagnosis difficult and often delayed. Here, we review the literature to demonstrate the hetereogeneity of presenting symptoms across all age groups. We also discuss diagnostic approach, emphasizing variation in biochemical, functional, and genetic results among Krabbe phenotypes. Better understanding of the various Krabbe disease phenotypes is critical to facilitate timely diagnosis and appropriate treatment of this clinically heterogeneous disorder.

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Historically, HIV-positive individuals have not been considered to be candidates for liver transplantation due to the need for further immunosuppresion of these patients post-transplant, as well as other factors such as pharmacokinetic interactions between the necessary antiretroviral and immunosuppressant drugs. However, HIV-positive individuals with end-stage liver disease are now eligible for liver transplantation in British Columbia. The purpose of this study was to summarize the outcomes of HIV-positive individuals referred for liver transplanation in British Columbia.

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