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Minerva Medica 2006 October;97(5):437-42

Copyright © 2006 EDIZIONI MINERVA MEDICA

language: English, Italian

Decreased chemiluminescence in leukocyte adhesion deficiency presenting with recurrent sepsis, amoebiasis and Candida albicans urinary tract infection

Valentini P. ¹, De Sole P. ², De Luca D. ¹, Plaisant P. ³, Puggioni P. ⁴, Rossi M. C. ², Rumi C. ⁴, Ranno O. ¹

¹ Pediatric Infectious Disease Unit Department of Pediatrics Sacro Cuore Catholic University, Rome, Italy 2 Chemiluminescence Laboratory Department of Chemistry and Clinical Chemistry Sacro Cuore Catholic University, Rome, Italy 3 Department of Microbiology, Sacro Cuore Catholic University, Rome, Italy 4 1Flow-Cytofluorimetry Study Center Department of Haematology Sacro Cuore Catholic University, Rome, Italy

Leukocyte adhesion deficiency (LAD) is a rare disorder of cellular immunity, generally due to various mutations producing reduced or altered expression of membrane integrins. The authors report a case of LAD due to integrins expression imbalance. LAD was suspected after recurrent sepsis, fungine infection and amoebiasis with persistent leukocytosis. Neutrophils were studied with chemiluminescence showing decreased functional activity: up to now, this seems the first chemiluminescence study of neutrophil function and the first report of amoebiasis at the onset in LAD.