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SCID- SEVERE COMBINED IMMUNODEFICIENCES - Cuddles Child Clinic

Combined immunodeficiency involves malfunctioning of multiple components of the immune system, including both humoral immunity and cell mediated immunity. •    It is also called as “bubble boy disease” •    It is X-linked genetic disorder •    It characteristically lead to death from overwhelming infection in the first year of life unless they have undergone successful bone marrow transplantation. •    In it neither T cells nor the B cells work properly Types : •    Typical SCID •    Leaky SCID •    Variant SCID Clinical presentation of SCID : •    Children with SCID may develop infections caused by organisms or vaccines •    Organisms which are most dangerous to cause fatal diseases are: pneumonia, fungal infection, viral infections like chickenpox, CMV, influenza, measles etc. •    Patients with SCID having rash are mistakenly diagnosed as eczema, but is actually caused by reaction of the mother’s T-cells. Diagnosis : •    Low lymphocyte count <1500 per cubic millimeter •