Hepatic Vascular Involvement in Adenosine Deaminase 2 Deficiency (DADA2): Case Reports and Literature Review [PDF]
Background and Clinical Significance: Deficiency of Adenosine Deaminase 2 (DADA2) is a rare monogenic vasculopathy characterised by systemic inflammatory and immunodeficiency features.
Mihaela Sparchez +4 more
doaj +2 more sources
Nodular lymphoid hyperplasia with hypogammaglobulinaemia [PDF]
A patient with nodular lymphoid hyperplasia, who manifested defects in cellular immunity, is presented. The clinical course is discussed and a review included of the 23 patients thus far reported with nodular lymphoid hyperplasia and hypogammaglobulinaemia.
A B, Ajdukiewicz +2 more
exaly +3 more sources
Obinutuzumab versus Rituximab to maintain remission in children with steroid-dependent and frequently relapsing nephrotic syndrome: the OBIRINS study protocol, a double-blind randomised controlled trial [PDF]
Introduction There is an unmet clinical need for the development of novel treatment strategies to improve the outcome of children with frequent relapsing or steroid-dependent nephrotic syndrome.
Florentia Kaguelidou +17 more
doaj +2 more sources
Gamma gap and albumin-globulin ratio show poor sensitivity for monoclonal gammopathy screening in South Africa [PDF]
Background: Monoclonal gammopathies, including multiple myeloma, present significant challenges in sub-Saharan Africa. Diagnosis is often missed because of limited screening tools.
Njabulo Khumalo +3 more
doaj +2 more sources
Demographic, clinical, and immunological features in combined immunodeficiency patients: a comparative analysis of those with and without pulmonary manifestations – a multicenter study from Iran [PDF]
Background Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.
Ghamartaj Khanbabaee +24 more
doaj +2 more sources
Long-term safety and efficacy of the combination of belimumab and rituximab in the treatment of severe and refractory SLE: a preliminary report [PDF]
Objective Combination therapy with rituximab and belimumab is a novel treatment strategy for severe SLE and lupus nephritis. Phase II studies have shown promising results, although long-term data are currently lacking.
Tineke Kraaij +11 more
doaj +2 more sources
This article reviews the primary immunodeficiencies that result in hypogammaglobulinemia or predominantly antibody deficiency disorders. This group makes up the largest proportion of patients with primary immunodeficiency. Significant advances have been made in understanding the molecular basis and clinical characteristics of patients with the more ...
Patrick F K, Yong +2 more
+6 more sources
Objectives: Chemotherapy, including bendamustine, usually causes lymphocytopaenia and hypogammaglobulinaemia as side effects in patients with haematological malignancies.
Akie Hirata +11 more
doaj +1 more source
Chronic Giardiasis in a Case of Common Variable Immunodeficiency (CVID): A Case Report [PDF]
Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by low antibody levels and recurrent infections. This makes an individual more prone to recurrent respiratory and gastrointestinal tract infections. In cases where there
Supriya M. Paranjpe +2 more
doaj +1 more source
Good's syndrome presenting with CMV pneumonitis and oesophageal candidiasis: A case report
Good's syndrome is an acquired combined T‐ and B‐cell immunodeficiencies and patients are prone to opportunistic infections. The diagnosis is based on a characteristic immunoglobulin and lymphocyte subset profile, with clinical features of thymoma ...
Chin Tong Kwok, Yiu Cheong Yeung
doaj +1 more source

