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Home > West Medicine > Pediatrics > Pediatric Internal Med

Classroom classification

百科

X-linked hyper-IgM syndrome

小儿内科

Common symptoms

口炎、甲状腺功能低下、腹泻、口腔溃疡、免疫缺陷、血小板减少、反复上呼吸道感染

The pathology of disease

X-linked hyper-IgM syndrome is CD40 ligand defect; low IgG/IgA/IgE with elevated IgM; recurrent bacterial infections; neutropenia; Pneumocystis; IVIG; bone marrow transplant.

Symptoms

Recurrent bacterial infections; Pneumocystis pneumonia; neutropenia; boys; low IgG high IgM; chronic cryptosporidiosis

Causes

CD40LG mutation; X-linked; class-switch recombination defect.

Treatment

IVIG; prophylactic antibiotics; PCP prophylaxis; bone marrow transplant; avoid live vaccines; immunology

Dept

Immunology / pediatrics / infectious disease.

Warm reminder: Health education reference only; seek care at regular hospital.

Family room

小儿内科Overview of the Western Medical Room