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INTRODUCTION IgA deficiency (IgAD) is the most common primary immunodeficiency, which can cause frequent infections. The association of IgA deficiency with systemic lupus erythematosus (SLE) is very important because of the high morbidity and mortality rates of infections in patients with this disease. OBJECTIVES To study the prevalence of IgA deficiency in SLE patients from southern Brazil a...

Selective IgA deficiency (IgAD) is the most common primary immunodeficiency disorder in Caucasians and is defined as serum IgA concentrations below or equal to 0.07 g/l, with normal serum concentrations of IgM and IgG, in individuals 4 years of age or older. The prevalence of IgAD is approximately 1:600 in the general population and recent results have shown that patients with IgAD have signifi...

eliac disease (CD) results from an abnormal response of the small bowel mucosa to gluten peptides derived from wheat. Classically, affected individuals have abdominal distension, malabsorption and diarrhea but in the last few years occult forms of the disease presenting with isolated iron deficiency anemia have been recognized.1 The disorder is more common in women, is familial and is associate...

A 13 year old school boy presented with seronegative inflammatory polyarthritis after a flu-like illness. Four months later clinical features of eosinophilic fasciitis became apparent. After histological diagnosis treatment was started with prednisone 40 mg daily, with a good response. Routine investigations showed persistent selective IgA deficiency.

The following conditions and diseases that are associated with primary immunodeficiency disorder include, Combined variable immunodeficiency disease, Ataxia-telangiectasia, Chediak-Higashi syndrome, Complement deficiencies, DiGeorge syndrome, Hypogammaglobulinemia, Job syndrome, Leukocyte adhesion defects, Bruton disease, Congenital agammaglobulinemia, Selective deficiency of IgA, Wiscott-Aldri...

A 24-year-old man with primary Sjögren's syndrome presented with xerophthalmia, xerostomia, and marked parotid swelling. He had a previous history of selective IgA deficiency and ulcerative colitis treated with sulphasalazine. Immunosuppression and withdrawal of sulphasalazine resulted in rapid resolution of the parotitis and disappearance of autoantibodies. A possible role for sulphasalazine i...

Selective IgA deficiency (IgAD) is the most common primary immunodeficiency in Caucasians. Although it is often asymptomatic, selected patients show an increased frequency of infections, allergies and autoimmune manifestations. Common variable immunodeficiency (CVID) is a primary antibody deficiency disease that shares many clinical features with IgAD. A common genetic basis for IgAD and CVID h...

Antibodies to nucleic acid antigens were measured in symptomatic and asymptomatic IgA-deficient individuals, non-IgA-deficient blood donors, and patients with systemic lupus erythematosus (SLE). There was no increase in mean levels of antibodies to nucleic acid antigens (native or denatured DNA, transfer RNA) in the IgA-deficient group, although 4 of 100 IgA-deficient blood donors had persisten...

Monosomy 18p is a relatively frequent deletion syndrome with an estimated frequency of one in 50,000 liveborns. Most frequent findings consist of mild to moderate growth deficiency, intellectual disability, microcephaly, and facial dysmorphic features including ptosis, epicanthic folds, low nasal bridge, hypertelorism and large protruding ears. Anomalies of other systems may accompany. A 31-yea...