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377

Orlandi et al.

(Continues)

subclasses should be part of the evaluation of patients with refractory RS. CRS, bronchitis, otitis media, and chronic diarrhea are conditions associated with

immunodeficiency syndromes. A scoring system coupled with specific clinical

indicators may provide a useful guide to the identification of immunodeficient patients in the outpatient setting. Moin 939 2004 4 Case series Iranian XLA patients; age 2 months - 30 years; n = 33 Serum Ig levels (IgG, IgM, IgA) Circulating T- and B-lymphocyte levels Prevalence of co-existing infection in patients with XLA It is important to consider hypogammaglobulinemia in any pediatric Chee 493 2001 4 Retrospective review patient with a history of recurrent infections at different organ systems. Plebani 944 2002 4 Case series Italian patients with XLA; age 2-33; n = 73 Serum Ig levels % of circulating B cells BTK mutation analysis

Despite early diagnosis and appropriate Ig replacement, CLD and CRS are common

long-term complications in patients with XLA.

Study Year LOE Study Design Study Groups Clinical Endpoint Conclusions Vanlerberghe 940 2006 4 Case series / Retrospective review Belgian patients with humoral immunodefi ciency (261 adults, 46 children) Serum Ig levels Humoral immunodeficiency is present in a significant proportion of patients with refractory RS. The majority of these Yarmohammadi 926 2006 4 Retrospective Case control 113 patients with immune

deficiencies are subtle IgG subclass deficits. Measurement of IgA, total IgG, and IgG

There is a high incidence of immune dysfunction in patients with CRS.

Immune deficiency-related scores

Pneumococcal vaccine response

Duration of IVIG therapy Quantitative serum Ig

Allergy skin testing T-cell function

Adult patients with CRS; n = 79

deficiency, 124

patients without immunodefi ciency; age 1-8

TABLE IX-18 (Continued)