Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ Key Features ++ Recurrent serious infections "Cold" abscesses without pus formation Poor wound healing Gingival or periodontal disease (or both) ++ Clinical Findings ++ Patients present with variably severe phenotypes, including Recurrent serious infections Lack of pus formation Poor wound healing Gingival and periodontal disease The hallmark is little inflammation and absent neutrophils on histopathologic evaluation of infected sites (ie, "cold" abscesses), especially when concurrent with neutrophilia, and expression of poor adherence to vessel walls The most severe phenotype manifests with infections in the neonatal period, including delayed separation of the umbilical cord with associated omphalitis ++ Diagnosis ++ Striking neutrophilia demonstrated on laboratory evaluation Diagnosis is confirmed by flow cytometry analysis for CD18 (type I) or CD15s (type II) ++ Treatment ++ Aggressive antibiotic therapy Fucose supplementation in type II has been reported with some success GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options