BACKGROUND Portal vein thrombosis (PVT) is a rare condition associated with serious morbidity and mortality. The objective of this study was to determine the frequency, clinical presentations, and risk factors of PVT from the set of data firstly collected among the Southeast Asian population. METHODS A retrospective study was undertaken to identify patients diagnosed with thrombosis of the po...

Immunodeficiency and disseminated mycobac-terial infection associated with homozygous nonsense mutation of IKKb To the Editor: Nuclear factor kappa B (NF-kB) signaling is known to be important for host protection against infection. For activation, proteins of the NF-kB transcription factor must be released from constitutive interaction with inhibitory IkB proteins (IkBa, IkBb, and IkBε), which ...

Leukocyte adhesion deficiency-I (LAD-I) is a rare disorder of leukocyte adhesion and migration, resulting from mutations in the ITGB2 gene encoding for the b2-integrin component CD18. Deficiencies in CD18 prevent normal integrin dimerization and leukocyte adhesion to endothelial surfaces, processes essential to extravasation and antimicrobial activity. Severe LAD-I (predominantly classified as ...

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