Immunological aspects of immunodeficiency diseases part 67

Immune 3


These patients typically present in early childhood with recurrent pyogenic infection of skin, respiratory, and gastrointestinal tracts as well as mucous membranes. Poor wound healing and delayed umbilical cord separation are typical. Because of impaired neutrphil migration, these patients develop a lecocytosis and pus fails to form at sites of infection. These inherited disorders are typicallya ssociated with severe ginggivitis and periodontal disease, again indicating the particular importance of normal neutrophil function for maintenance of the health of the dental crevice. Outcome in both coondition is poor, with early death. BMT is curative in LAD1, and oral fucose supplementation is beneficial in LAD2.

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