Immunizing deficit severe compound related to X

The immunizing deficit severe compound related to the X is a cellular immunity humorale and overdrawn by absence of Lymphocyte T, NK and absence of Lymphocyte B functional. This Genetic disease touches only the boys and the first signs appear between three and six months of life.

The signs include/understand a defect of growth, an absence of amygdala, candidoses oral, recurring and opportunist infections like the pneumocystis and the absence of cure in spite of a well led treatment. The diarrhea is a usual sign. The little boys with an average deficit have a diarrhea, disorders of malabsorption, a weak growth and autoimmune demonstrations.

Other names

Etiology

Incidence & prevalence

Description

This pathology must with a change of gene coding the protein gamma C which is a receiver for the cytokines IT 15 and IT 7. IT 7 allows the differentiation of the lymphocytes T in the thymus and IL15 allows that of the NK.

Diagnosis

Treatment & Assumption of responsibility

The genetic Council

Mode of transmission

Sources

Online Mendelian Inheritance in Man, OMIM (TM). Johns Hopkins University, Baltimore, MANDELEVIUM. MIM Number: 300400 * GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2005

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