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Primary Immunodeficiency

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29 Table 8. Assessing Serotype-Specific Responses to Pneumococcal Capsular Polysaccharides Phenotype Age <6 y Age >6 y Mild Concentration >1.3 μg/mL for >50% of types with a 2-fold increase for <50% of serotypes Concentration >1.3 μg/mL for >70% of types with a 2-fold increase for <70% of serotypes Moderate Concentration >1.3 μg/mL for <50% of serotypes Concentration >1.3 μg/mL for <70% of serotypes Severe Concentration >1.3 μg/mL for ≤2 serotypes Memory Loss of response within 6 mo Adapted from Orange et al. J Allerg y Clin Immunol. 2012;130(suppl):S1-S24. Transient hypogammaglobulinemia of infancy (THI) Î SS 107. Infants and young children with frequent viral and bacterial respiratory illnesses and low IgG levels with normal vaccine responses should be given a diagnosis of THI. (C) Î SS 108. The principles of management of THI should follow those for antibody deficiency. (C) Immunoglobulin class-switch defects Î SS 109. Patients with immunoglobulin class-switch defects should be clearly differentiated from those with other forms of CID with similar screening laboratory findings. (C) Î SS 110. The principles of management of immunoglobulin class-switch defects should follow those for antibody deficiency. (C) Î SS 111. Autoimmune, lymphoproliferative, or malignant diseases associated with immunoglobulin class-switch defects are treated as they would be in other clinical settings. (C) Unspecified hypogammaglobulinemia Î SS 112. Any patient with primary hypogammaglobulinemia and normal cellular immunity who does not fulfill the diagnostic criteria for the above disorders should be given a diagnosis of unspecified hypogammaglobulinemia. (D) Î SS 113. Management of unspecified hypogammaglobulinemia should adhere to the general principles presented for antibody deficiency. (D)

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