Krabbe disease

Multinucleated macrophages with PAS-positive inclusions, so-called globoid cells

Krabbe disease (also Krabbe globoid leukodystrophy ) is a leukodystrophy . It is one of the inherited diseases caused by a congenital defect of lysosomal enzymes. The result is an unrestricted, non-tumor accumulation of mature adipose tissue in places where it does not normally occur. This is a galactocerebroside β-galactosidase deficiency. It affects CNS cells.

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related articles

• Leukodystrophy
• Lipidosis
• Hereditary metabolic disorders
• Hereditary small molecule metabolic disorders
• Hereditary metabolic disorders of complex molecules

Source

• • VOKURKA, Martin and Jan HUGO, et al. Great Medical Dictionary [online]  . 8th edition. Prague: Maxdorf, 2009. 1144 pp. Also available from < http://lekarske.slovniky.cz/ >.