Abstract:
:We describe a patient with leucocyte adhesion deficiency (LAD). Clinically, the patient had delayed umbilical cord detachment, omphalitis, impaired wound healing and persistent leucocytosis. The patient had the severe form of LAD, with a total absence of leucocyte cell adhesion molecules (LeuCAMs) and undetectable mRNA for the beta chain, the common subunit of the LeuCAMs. In vitro neutrophil chemotaxis, aggregation and oxygen consumption were severely impaired. In vitro incubation of neutrophils with recombinant human interferon-gamma (rIFN-gamma) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the beta chain mRNA. In vivo treatment with IFN-gamma was started. The Fc gamma RI receptor appeared on the neutrophils, the LeuCAMs remained undetectable, while the neutrophil functions remained disturbed. The patient died of surgical complications after 10 weeks of rIFN-gamma treatment. No new infections or side-effects due to rIFN-gamma were observed.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Weening RS,Bredius RG,Vomberg PP,van der Schoot CE,Hoogerwerf M,Roos Ddoi
10.1007/BF01958952keywords:
subject
Has Abstractpub_date
1992-02-01 00:00:00pages
103-7issue
2eissn
0340-6199issn
1432-1076journal_volume
151pub_type
杂志文章abstract:UNLABELLED:The aim of this study was to obtain comprehensive data on clinical presentation, microbiology, computed tomography, surgical findings and histology in acute, sub-acute and chronic mastoiditis. We performed a prospective, observational study in children under 16 years of age presenting to our institution duri...
journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00540255
更新日期:1984-09-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-003-1351-3
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:1983-04-01 00:00:00
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
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journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
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