ABSTRACT. IgA nephropathy (IgA-N) that comprises Berger disease and Henoch-Schönlein Purpura (HSP) nephritis is defined by mesangial IgA deposits. Recently, this group has characterized a new receptor for IgA, the transferrin receptor (CD71), expressed on mesangial cells. To assess whether CD71 was involved in the pathogenesis of IgA-N, its expression was analyzed together with IgA deposits on ...

There is a need for neonatal screening tools to improve the long-term clinical outcome of patients with primary immunodeficiency diseases (PID). Recently, a PCR-based screening method for both TRECs and KRECs using Guthrie card samples has been developed. However, the applicability of these excision circle assays is limited to patients with severe T or B cell lymphopenia (SCID, XLA and A-T), wh...

M cells of intestinal epithelia overlying lymphoid follicles endocytose luminal macromolecules and microorganisms and deliver them to underlying lymphoid tissue. The effect of luminal secretory IgA antibodies on adherence and transepithelial transport of antigens and microorganisms by M cells is unknown. We have studied the interaction of monoclonal IgA antibodies directed against specific ente...

BACKGROUND IgA nephropathy is defined by the presence of IgA-dominant glomerular deposits. Within this definition, there is variation in the location of IgA and the presence of other immunoglobulins. The Oxford classification of IgA nephropathy identifies four histological features that are independent predictors of clinical outcome but does not include immunostains. Here, we investigate the po...

IgA is one of the five classes of immunoglobulin found in biological fluids. It is the major secretory immunoglobulin and is widely distributed in all mucosal secretions as a dimeric molecule linked by a joining chain and a third molecule, the secretory piece. The dimeric form is resistant to proteolytic digestion and IgA antibodies are important in the protection of mucous membranes against in...

IgA欠損患者に対する血液製剤の使用に際しては，IgAを含まない製剤の使用が推奨されているが，抗IgA抗体を有するIgA非欠損患者に対する輸血実施の対応は確立されていない．今回我々は，抗IgA抗体を有するIgA非欠損例において，洗浄血小板製剤の使用と骨髄濃縮により非血縁者間同種造血幹細胞移植を重篤な有害事象なく施行し得た1例を経験した．症例は48歳男性，基礎疾患は原発性骨髄線維症で，アレルギー・輸血・手術歴はない．初回輸血から9日後の血小板輸血中にアナフィラキシーが出現した．IgA欠損はなかったが，初回輸血前検体でIgG型抗IgA抗体を認め，抗体獲得機序は不明であった．洗浄血小板製剤に切り替えて対応し，有害事象なく経過した．また，ABO血液型一致非血縁者間同種移植であったが，輸注前に骨髄濃縮を実施することで輸注関連有害事象を認めることなく施行できた．移植後はドナー型造血となり，抗Ig...

Antineutrophil cytoplasmic antibodies (ANCA) of IgG class have been described at high prevalence in autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). Data on IgA class ANCA in these diseases are limited. The aim of this study was to determine the prevalence and fluorescence patterns of IgA class ANCA in AIH and PSC and to examine a relationship between the presence of IgA ANC...

INTRODUCTION Celiac disease (CD) prevalence is increasing but the disorder remains undiagnosed. The study compares CD serology markers requested by General Practitioners (GPs) over time and geographical areas. The aim of the current research is to assess the inter-practice and temporal variability in the request of CD serology markers by GPs in Spain, and the differences between regions. MATE...