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BENTA Disease; Other names: B-cell expansion with NF-kB and T-cell anergy disease: For each child an affected parent has, there is a 50% chance of passing on the mutation, regardless of the sex of the child autosomal dominant: Specialty: Immunology
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Sickle cell-Hb Lepore Boston syndrome is a type of sickle cell disease (HbS) that differs from homozygous sickle cell disease where both parents carry sickle hemoglobin. In this variant one parent has the sickle cell hemoglobin the second parent has Hb Lepore Boston, the only one of the three variants described in association with HbS. [7]
In hematology, plasma cell dyscrasias (also termed plasma cell disorders and plasma cell proliferative diseases) are a spectrum of progressively more severe monoclonal gammopathies in which a clone or multiple clones of pre-malignant or malignant plasma cells (sometimes in association with lymphoplasmacytoid cells or B lymphocytes) over-produce and secrete into the blood stream a myeloma ...
I-cell disease is associated with various clinical features that affect physical appearance, organ function, and growth development. The severity of these symptoms varies between individuals, though the prognosis is poor due to the disease’s systemic nature. I-Cell Disease patients may also experience impaired cognitive and motor development.
Environmental enteropathy (EE or tropical enteropathy or environmental enteric dysfunction or EED) is an acquired small intestinal disorder characterized by gut inflammation, reduced absorptive surface area in small intestine, and disruption of intestinal barrier function.
[2] [9] A wide range of stimuli cause cells to ubiquinate proteins that promote the movement of NF-κB from its inactive location in the cell's cytosol to the cell's nucleus. NF-κB is a transcription factor which when located in the nucleus stimulates the expression of various genes that in turn promote cell death by apoptosis and necroptosis ...
Shigellosis was a sporadic disease in South Korea for many years, until 1998. Beginning in 1998 South Korea experienced a sudden epidemic of shigellosis among school children. Contaminated school meals were identified as the major source of infection, and after several years, the infection rate declined significantly. [8]