نتایج جستجو برای: adhesion deficiency syndrome

تعداد نتایج: 816125  

Journal: :Blood 2007
Yumi Tone Taizo Wada Fumie Shibata Tomoko Toma Yoko Hashida Yoshihito Kasahara Shoichi Koizumi Akihiro Yachie

Leukocyte adhesion deficiency type 1 (LAD-1) is an autosomal recessive disorder caused by mutations in the ITGB2 (CD18) gene and characterized by recurrent severe infections, impaired pus formation, and defective wound healing. We describe an unusual case of severe phenotypic LAD-1 presenting with somatic mosaicism. The patient is a compound heterozygote bearing 2 different frameshift mutations...

Journal: :Revista medica de Chile 2009
Hermann M Wolf Claudia Stöllberger Josef Finsterer

The association of Down syndrome with mannose-binding lectin (MBL)-deficiency, recurrent infections and vasculitis has not been reported. We report a 30 year-old female with Down-syndrome associated with MBL-deficiency with the genotype LXA/HYD, IgG-deficiency, recurrent uro-genital infections, cutaneous vasculitis, G20.210A prothrombin mutation, deep venous thrombosis, and pulmonary embolism. ...

Journal: :Archives of Iranian medicine 2008
Reem Dababneh Adel M Al-Wahadneh Shamekh Hamadneh Antwan Khouri Nabil F Bissada

BACKGROUND Leukocyte adhesion deficiency type I (LAD-I) is an autosomal recessive immunodeficiency disorder characterized by defects in the integrin receptors of white blood cells that lead to impaired adhesion and chemotaxis. Affected patients are susceptible to recurrent bacterial and fungal infections, impaired pus formation, delayed wound healing, and periodontitis. METHODS A case of gene...

Journal: :Gut 1996
I D D'Agata K Paradis Z Chad Y Bonny E Seidman

CD11/CD18 leucocyte glycoprotein deficiency is a rare, congenital adhesion molecule disorder which, in its severe form, is usually fatal. Leucocytes in affected subjects have abnormal migration and adherence, rendering patients susceptible to life threatening infections. The CD11/CD18 integrins, and other adhesion molecules, are considered essential to the normal inflammatory response. It has b...

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