Skewed Distribution of Circulating Activated Natural Killer T (NKT) Cells in Patients with Common Variable Immunodeficiency Disorders (CVID)

Common variable immunodeficiency disorder (CVID) is the commonest cause of primary antibody failure in adults and children, and characterized clinically by recurrent bacterial infections and autoimmune manifestations. Several innate immune defects have been described in CVID, but no study has yet investigated the frequency, phenotype or function of the key regulatory cell population, natural killer T (NKT) cells. We measured the frequencies and subsets of NKT cells in patients with CVID and compared these to healthy controls. Our results show a skewing of NKT cell subsets, with CD4+ NKT cells at higher frequencies, and CD8+ NKT cells at lower frequencies. However, these cells were highly activated and expression CD161. The NKT cells had a higher expression of CCR5 and concomitantly expression of CCR5+CD69+CXCR6 suggesting a compensation of the remaining population of NKT cells for rapid effector action

Educational paper: Primary antibody deficiencies

Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies and are characterized by a defect in the production of normal amounts of antigen-specific antibodies. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemias, in which the antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent respiratory tract infections, PADs are associated with a wide range of other clinical complications. This review will describe the pathophysiology, diagnosis, and treatment of the different PADs

Educational paper: The expanding clinical and immunological spectrum of severe combined immunodeficiency

Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency characterized by absence of functional T lymphocytes. It is a paediatric emergency, which is life-threatening when recognized too late. The clinical presentation varies from the classical form of SCID through atypical SCID to Omenn syndrome. In addition, there is a considerable immunological variation, which can hamper the diagnosis. In this educational review, we describe the immunopathological background, clinical presentations and diagnostic process of SCID, as well as the therapeutic possibilities

Antineutrophil cytoplasmic autoantibodies in uveitis

Antineutrophil cytoplasmic autoantibodies were detected in patients with some autoimmune and vascular disease such as Wegner’s granulomatosis polyarthritis nodosa and systemic lupus erythematosus. Indirect immunofluorescence technique was employed to detect these autoantibodies. By this method, two general patterns of antineutrophil cytoplasmic autoantibodies were seen: a cytoplasmic (C-ANCA) and a perinuclear form (P-ANCA). These antibodies also were observed in uveitis. In this study the presence of antineutrophil cytoplasmic autoantibodies in 25 patients with uveitis and its relationship with uveitis and its relationship with anatomical location of the disease is evaluated. According to the results antineutrophil cytoplasmic autoantibodies was detected in 16% (4 out of 25) of the patients all of them being C-ANCA type. The results also showed that there was not any significant correlation between the presence of antineutrophil cytoplasmic autoantibodies and anatomical location of the disease (P=0.65)

Effect of health disorders on the hazard of culling on the first or second lactation in Iranian dairy herds

We conducted a longitudinal retrospective study in order to assess the effect of health disorders (HDs) on culling in the 1st or 2nd lactation cows, in Iranian dairy herds. In total, 7067 first- and second-parity Holstein cows, from 32 Iranian daily herds, which calved from March 2007 to March 2008, were followed until the next calving or culling. Parametric survival models with time-dependent covariates were used to capture the effect of HDs with different duration and recurrence episodes on the risk of culling. Mastitis, locomotor disorders, ovarian cysts, abortion, diarrhea, rumen disorders, displaced abomasum and respiratory disorders were associated with an increased risk of culling. Hazard ratios and 95% confidence intervals were 2.78 (2.31; 335), 3.4(2.79; 4.13) and 1.62 (1.15; 2.78) for mastitis, locomotor disorders and ovarian cysts as common HDs, respectively. Parity and milk-yield were identified as confounder and effect modifier, respectively. The risk of culling in the presence of uterine infection or traumatic reticulo-peritonitis increased with decreasing milk yield. (C) 2012 Elsevier B.V. All rights reserved