Immunodeficiency with thymoma was described by R.A. Good in 1954 and is also named after him. The syndrome is characterized by hypogammaglobulinemia associated with thymoma and recurrent infections, bacterial but also viral, fungal and parasitic. Autoimmune diseases, mainly pure red cell aplasia, other hematological disorders and erosive lichen planus are a common finding. We describe here a typical case exhibiting all these clinical features and report a detailed immunophenotypic assessment, as well as the positivity for autoantibodies against three cytokines (IFN-alpha, IL-6 and GM-CSF), which may add to known immune abnormalities. A review of the published literature, based on case series and immunological studies, offers some hints on the still unsolved issues of this rare condition.
RATIONALE: Atopic eczema and food allergy are common during the first 2 years of life. The relationship between eczema onset after infancy and allergen sensitizationand atopic diseases was investigated. METHODS: We investigated 186 children followed up regularly for 4 years in a birth cohort study. Early-onset eczema was defined to occur symptoms before age of 2. Specific IgE antibodies against food (egg white, milk, and wheat) and inhalant allergens (mite) were measured at 6 months as well as 1, 1.5, 2, 3 and 4 years of age. The associations between onsets of eczema and atopic indices were assessed. RESULTS: A significantly higher prevalence of sensitization to food, especially milk was found in children with early-onset eczema compared to children without eczema at age 1, 1.5, 2, 3 and 4. A significantly higher number of eosinophils was found in children with early or late-onset eczema compared to children without eczema at age of 1.5 years. Both early and late-onset eczema were not only significantly associated with higher prevalence of allergic rhinitis at 2, 3, and 4 years of age, but also asthma at age 2. Furthermore, early-onset eczema appeared to show a significantly increased risk of allergic rhinitis [odds ratio (OR), 3.71; 95% confidence interval (CI), 1.37-10.02; P 5 0.010] and asthma (OR, 3.80; 95% CI, 1.12-12.85; P 5 0.032) at an age of 4 years. CONCLUSIONS: Early-onset eczema appears not only to be associated with an increase in the prevalence of milk sensitization but also the risk of rhinitis and asthma in early childhood.
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