{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T04:03:21Z","timestamp":1773288201178,"version":"3.50.1"},"reference-count":37,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2004,3,30]],"date-time":"2004-03-30T00:00:00Z","timestamp":1080604800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,3,30]]},"abstract":"<jats:title>SUMMARY<\/jats:title><jats:p>Blood samples were collected over a 4-year period from 335 children (aged 1\u201316\u2003years) suffering from recurrent respiratory infections and 78 controls. The patients were subdivided into four groups: I, children with no immune system defects detected (n\u2003=\u2003101); II, children with allergies (n\u2003=\u200394); III, children with humoral response defects (n\u2003=\u200393); and IV, children with disturbances of cellular immunity (n\u2003=\u200366). Nineteen patients had both humoral and cellular abnormalities. All patients and controls were investigated to determine the exon 1 and promoter region variants of the mbl-2 gene. MBL serum concentrations were also determined in samples from 291 patients and 75 controls. The proportion of O (B, D or C) alleles was significantly higher in the patient group compared to controls, and this association was strongest for subgroup III. The promoter LX variant frequency was also commoner in the patients as a whole, and significantly so in subgroups II and IV. Genotypes markedly influenced MBL concentrations in all groups, and correlated with ability to activate the lectin pathway of complement activation. The strongest and most significant inverse correlations between serum MBL and respiratory disease were found in patient group III and in 17 patients with multiple humoral and\/or cellular abnormalities. Among nine patients with unexpectedly low LP activity in view of their MBL concentrations, one person was found to be MASP-2 deficient. Our results indicate that mannan-binding lectin insufficiency, with or without a coexisting immune defect, is associated with the occurrence of recurrent respiratory infections in childhood, and this relationship is particularly strong and statistically significant in children with concomitant impairments of humoral immunity.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2004.02453.x","type":"journal-article","created":{"date-parts":[[2004,4,8]],"date-time":"2004-04-08T08:00:40Z","timestamp":1081411240000},"page":"304-311","source":"Crossref","is-referenced-by-count":123,"title":["Mannan-binding lectin insufficiency in children with recurrent infections of the respiratory system"],"prefix":"10.1093","volume":"136","author":[{"given":"M","family":"CEDZYNSKI","sequence":"first","affiliation":[{"name":"Laboratory of Immunobiology of Infections, Centre of Medical Biology and Microbiology, Polish Academy of Sciences"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"SZEMRAJ","sequence":"additional","affiliation":[{"name":"Department of Biochemistry"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A S T","family":"SWIERZKO","sequence":"additional","affiliation":[{"name":"Laboratory of Immunobiology of Infections, Centre of Medical Biology and Microbiology, Polish Academy of Sciences"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"BAK-ROMANISZYN","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Gastroenterology, Institute-Polish Mothers\u2019 Memorial Hospital"},{"name":"Department of Paediatrics and Clinical Immunology, Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M","family":"BANASIK","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology, Institute-Polish Mothers\u2019 Memorial Hospital, Lodz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K","family":"ZEMAN","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Clinical Immunology, Medical University"},{"name":"Department of Clinical Immunology, Institute-Polish Mothers\u2019 Memorial Hospital, Lodz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D C","family":"KILPATRICK","sequence":"additional","affiliation":[{"name":"Scottish National Blood Transfusion Service, National Science Laboratory, Edinburgh, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2004,3,30]]},"reference":[{"key":"2022011223184779000_b1","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1006\/smim.1998.0141","article-title":"The serum mannose-binding protein and the macrophage mannose receptor are pattern recognition molecules that link innate and adaptive 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