X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been a paucity of reports of noninfectious inflammatory arthritis in children with XLA. This case ill...

The close anatomical relationship between the immune system, estrogen deficiency and bone loss has been recognized for centuries but the existence of a functional relationship has emerged only recently. The role of the immune system in the development of senile osteoporosis, which arises primarily through the effects of estrogen deficiency and secondary hyperparathyroidism, is slowly being unra...

Patients may be referred to the immunology clinic for investigation of recurrent superficial abscess formation. In the majority of adult patients this clinical presentation does not equate with an underlying primary immune deficiency. Nevertheless, recurrent mucocutaneous abscesses can be associated with significant morbidity and long-term complications, including scarring and fistula formation...

Dietary methionine (met) is reported to enhance antibody production and boost cell-mediated immunity in chickens. Methionine deficiency has been shown to affect the development of the lymphoid organs and the generation of antibodies in chickens. This study is designed to investigate the effects of met deficiency on IgA+ B cells and immunoglobulins (sIgA, IgA, IgG and IgM) for a 6 wee...

The current review evaluates how inflammasomes and immune checkpoints are regulated in pre-eclampsia (PE) associated with tuberculosis (TB) Human Immune Deficiency Virus (HIV). Studies indicate that such as (NRLP3, NEK7, AIM2) (CLT4, PD-1, TIM3, LAG-3) dysregulated TB- HIV-infected individuals, also pre-eclamptic pregnancies, which explains why pregnant women who either infected TB or HIV have ...

Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension (PAH) in the setting of portal hypertension, and is associated with a poor prognosis [1]. The underling etiological cause of POPH is poorly understood [1]. Previously we have reported T cell-mediated immune deficiency or compromise in patients with chronic thromboembol-ic pulmonary hypertension (CTEPH), idiopathic p...

Hemophilia B is a rare X-linked recessive disorder with plasma factor IX (FIX) deficiency. 1-3% of patients treated with exogenous FIX-containing products develop inhibitors (i.e. polyclonal high affinity immunoglobulins) that neutralize the procoagulant activity of a specific coagulation factor. Although the incidence of inhibitors in hemophilia B patients is low, most are "high titer" and fre...

Acquired immune deficiency syndrome (AIDS) is a newly recognized disease of unknown etiology, characterized by deregulation of the cell-mediated immune function system and manifested by opportunistic infections, unusual neoplasms (particularly Kaposi's sarcoma) in previously healthy persons. Male homosexuals, drug addicts, Haitian immigrants, and hemophiliacs constitute the group at high risk o...

The first descriptions of the acquired immune deficiency syndrome (AIDS) were from the USA in 19811-3 although it is probable the first cases occurred in 1979.2 In Great Britain the first case occurred in 1981.4 In both countries there has been an exponential rise in the numbers of cases. The syndrome is defined by the Center for Diseases Control, Atlanta, Georgia, as characterised by opportuni...