1863 liter three years before admission. Instead of providing immediate immune globulin replacement on admission, his clinicians remeasured the serum IgG five days later (130 mg per deciliter), and immune globulin replacement was given seven days after admission. The patient died on the 12th hospital day. There is an extensive body of literature supporting the value of intravenous immune globul...

CD40 ligand (CD40L) is a membrane molecule that plays a key role in T cell-B cell cooperation, providing B cells the helper signals needed for activation, proliferation, differentiation and prevention of apoptosis. Patients with B-cell chronic lymphocytic leukemia (B-CLL) were studied to verify the following hypotheses: a) whether defective CD40L expression on activated T cells could account fo...

The question of whether viruses persist after apparent clearance of infection remains unanswered. Here, we describe a patient with hypogammaglobulinemia whose acute hepatitis C virus (HCV) infection appeared to resolve after receipt of interferon therapy, relapse immediately, and then clear spontaneously--only to relapse after receipt of corticosteroid therapy, and clear again, 8.5 years later....

The present case is a 5-month-old female with atopic dermatitis who was brought to hospital for growth failure noted upon regular health examination. Laboratory examinations revealed hyponatremia, hyperkalemia, hypoproteinemia, hypogammaglobulinemia, elevated plasma renin activity and hyperaldosteronemia. Immune function was normal. Composition of the exudate collected from the skin lesions of ...

We describe a child with congenital hypogammaglobulinemia that was diagnosed at 13 months of age. When he was 4 years old, gait disturbances began. The main neurological manifestations were progressive spastic tetraparesis and intellectual and speech deterioration. No infectious agent was identified. A magnetic resonance imaging scan of the central nervous system revealed periventricular demyel...

Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder caused by inherited defects in the nicotinamide adenine dinucleotide phosphate oxidase complex. The neutrophils of patient with CGD can ingest bacteria normally, but the oxidative processes that lead to superoxide anion formation, hydrogen peroxide production, nonoxidative pathway activation, and bacterial killing are im...

Introduction: Antibody production defects may predispose children to inflammatory pathologies and therefore we hypothesized that this group of immune deficiencies may be associated with food allergy. Objective of the study: To better characterize the interrelated pathomechanisms of food allergy coexisting with hypogammaglobulinemia in children and to define the relationship between clinical man...