X-linked Hyper IgM (HIGM1) in an African kindred: the first report from South Africa

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X-linked Hyper IgM (HIGM1) in an African kindred: the first report from South Africa

BACKGROUND The objective of this study was to describe the clinical and molecular features of the first South African family with X-linked hyper-IgM syndrome (HIGM1). METHODS Diagnoses were based on immunoglobulin results and the absence of CD40 ligand (CD40L) expression on activated T-cells. Complete molecular characterisation involved CD40L cDNA sequencing, and genomic DNA analysis by polym...

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The X-linked deafness of Nance is present in a South African kindred. Recognition of the familial pattern of the disorder, together with the characteristic clinical and audiometric features, permits diagnostic precision, thereby facilitating accurate genetic counselling and rational management. Linkage studies indicated that the loci for the Xg blood group and the deafness gene are unlikely to ...

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X-linked hyper-IgM syndrome with eosinophilia in a male child: A case report

The hyper-IgM syndromes (HIGMs) are a group of primary immune deficiency diseases characterized by a normal or elevated serum level of IgM and low or absent serum levels of IgG, IgA and IgE. Here, we report a case of X-linked HIGM with a new CD40L gene mutation presenting with eosinophilia. The patient experienced recurrent pneumonia and acute respiratory distress syndrome (ARDS) from 4 months ...

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ژورنال

عنوان ژورنال: BMC Pediatrics

سال: 2003

ISSN: 1471-2431

DOI: 10.1186/1471-2431-3-12