Familial dysautonomia (Riley et al., 1949) is an autosomal recessive disease affecting Jews of Eastern European ancestry (Brunt and McKusick, 1970). As the name implies the autonomic nervous system is defective, and this is seen clinically as defective control of temperature, blood pressure, and swallowing, by skin blotching, excessive sweating, lack of tearing, and bowel disturbance. Abnormali...

A 63-year-old male with history of prostate cancer treated with radiation presented for a colonoscopy for small volume hematochezia. The colonoscopy revealed numerous polyps, which were found to be ganglioneuromas on histological examination. He was referred to medical genetics with suspicion for hamartomatous polyposis syndrome and was found to have a mutation in the PTEN gene. Based on this a...

We use medium resolution optical spectra of 3CR radio galaxies to estimate their black hole masses and accretion rates. Black hole masses are found from central stellar velocity dispersions, and accretion rates are derived from narrow emission-line luminosities. The sample covers both Fanaroff-Riley (FR) classes; the more powerful FRIIs and the less powerful FRIs. We find that FRIs and FRIIs se...

The first report of replication of Riley's plasma enzyme-elevating virus (Riley virus) in tissue culture was by Yaffe (1), 2 years after the discovery of the virus in 1960 (2). Yaffe found that Riley virus multiplied in primary mouse embryo cultures until the death of the cultures through overcrowding (3 to 4 weeks), without producing cytopathic or other detectable changes, and could be seriall...

In mice infected with Riley virus (1), the activities of a number of plasma enzymes are permanently elevated (2, 3). Similar, although temporary, increases in plasma enzyme activity have been produced in mice by the administration of certain substances which reduce the phagocytic activity of the reticuloendothelial system (RES), and so it was suggested that in both cases the increased plasma en...

Familial dysautonomia, also known as Riley-Day syndrome, is a disorder of autonomic nervous system with an autosomal recessive mode of inheritance. Reduction and/or loss of unmyelinated and small myelinated fibers is found, as reduction of dopamine beta-hydroxylase in blood. The diagnosis is based on clinical features: diminished lacrimation, insensitivity to pain, poor temperature control, abo...