Chronic granulomatous disease

Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome, is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens. This leads to the formation of granulomas in many organs. CGD affects about 1 in 200,000 people in the United States, with about 20 new cases diagnosed each year. This condition was first discovered in 1950 in a series of 4 boys from Minnesota, and in 1957 it was named "a fatal granulomatosus of childhood" in a publication describing their disease. The underlying cellular mechanism that causes chronic granulomatous disease was discovered in 1967, and research since that time has further elucidated the molecular mechanisms underlying the disease. Bernard Babior made key contributions in linking the defect of superoxide production of white blood cells, to the cause of the disease. In 1986, the X-linked form of CGD was the first disease for which positional cloning was used to identify the underlying genetic mutation. Classically, patients with chronic granulomatous disease will have recurrent bouts of infection due to the decreased capacity of their immune system to fight off disease-causing organisms. The recurrent infections they acquire are specific and are, in decreasing order of frequency: pneumonia abscesses of the skin, tissues, and organs septic arthritis osteomyelitis bacteremia/fungemia superficial skin infections such as cellulitis or impetigo Most people with CGD are diagnosed in childhood, usually, before age 5. Early diagnosis is important since these people can be placed on antibiotics to ward off infections before they occur. Small groups of CGD patients may also be affected by McLeod syndrome because of the proximity of the two genes on the same X-chromosome.

PD-1-cis IL-2R agonism yields better effectors from stem-like CD8(+) T cells

Douglas Hanahan, Mélanie Louise Tichet, Petra Catalina Schwalie, Stephan Wullschleger, Maria Karagianni

Expansion and differentiation of antigen-experienced PD-1(+)TCF-1(+) stem-like CD8(+) T cells into effector cells is critical for the success of immunotherapies based on PD-1 blockade(1-4). Hashimoto et al. have shown that, in chronic infections, administr ...

NATURE PORTFOLIO2022

Targeting the aging of tumor-associated neutrophils in lung cancer

Single-mitosis dissection of acute and chronic DNA mutagenesis and repair

PD-1-cis IL-2R agonism yields better effectors from stem-like CD8(+) T cells

PD-1-cis IL-2R agonism yields better effectors from stem-like CD8(+) T cells

Single-mitosis dissection of acute and chronic DNA mutagenesis and repair

Targeting the aging of tumor-associated neutrophils in lung cancer