Dysautonomia, Familial
Definition
Dysautonomia, Familial: An autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of Ashkenazic Jewish descent. Clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension (HYPOTENSION, ORTHOSTATIC), fixed pupils, excessive SWEATING, loss of pain and temperature sensation, and absent reflexes. Pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons. (From Adams et al., Principles of Neurology, 6th ed, p1348; Nat Genet 1993;4(2):160-4)
Annotations
PRIMARY DYSAUTONOMIAS is also available
Other Entries
HSAN Type III
Hereditary-Sensory and Autonomic Neuropathy Type III
Neuropathy, Hereditary and Autonomic, Type III
Riley-Day Syndrome
Dominant Hereditary Sensory Neuropathy, Type III
HSAN (Hereditary Sensory and Autonomic Neuropathy) Type III
HSAN 3
Hereditary Sensory Neuropathy Type 3
Hereditary Sensory Neuropathy, Dominant, Type 3
Hereditary Sensory Neuropathy, Dominant, Type III
Hereditary Sensory Neuropathy, Type 3, Dominant
Hereditary Sensory and Autonomic Neuropathy 3
Type 3 Hereditary Sensory Neuropathy, Dominant
Type III Hereditary Sensory Neuropathy, Dominant
Familial Dysautonomia
HSAN Type IIIs
Hereditary Sensory and Autonomic Neuropathy Type III
Riley Day Syndrome
Allowable Qualifiers
blood cerebrospinal fluid chemically induced classification complications diagnosis diet therapy drug therapy economics embryology enzymology epidemiology ethnology etiology genetics history immunology metabolism microbiology mortality nursing parasitology pathology physiopathology prevention & control psychology radiography radionuclide imaging radiotherapy rehabilitation surgery therapy ultrasonography urine veterinary virology
Categories
Entry Date
01-01-1999
Common Chronic Illnesses
Coronary Disease
An imbalance between myocardial functional requirements and the capacity of the CORONARY VESSELS to supply sufficient blood flow. It is a form of MYOCARDIAL ISCHEMIA (insufficient blood supply to the heart muscle) caused by a decreased capacity of the coronary vessels.
Diabetes Mellitus, Type 1
A subtype of DIABETES MELLITUS that is characterized by INSULIN deficiency. It is manifested by the sudden onset of severe HYPERGLYCEMIA, rapid progression to DIABETIC KETOACIDOSIS, and DEATH unless treated with insulin. The disease may occur at any age, but is most common in childhood or adolescence.
Diabetes Supplies | Diabetic Groups
Diabetes Mellitus, Type 2
A subclass of DIABETES MELLITUS that is not INSULIN-responsive or dependent (NIDDM). It is characterized initially by INSULIN RESISTANCE and HYPERINSULINEMIA; and eventually by GLUCOSE INTOLERANCE; HYPERGLYCEMIA; and overt diabetes. Type II diabetes mellitus is no longer considered a disease exclusively found in adults. Patients seldom develop KETOSIS but often exhibit OBESITY.
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Hypertension
Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.
Mesothelioma
A tumor derived from mesothelial tissue (peritoneum, pleura, pericardium). It appears as broad sheets of cells, with some regions containing spindle-shaped, sarcoma-like cells and other regions showing adenomatous patterns. Pleural mesotheliomas have been linked to exposure to asbestos. (Dorland, 27th ed)
Osteoarthritis
A progressive, degenerative joint disease, the most common form of arthritis, especially in older persons. The disease is thought to result not from the aging process but from biochemical changes and biomechanical stresses affecting articular cartilage. In the foreign literature it is often called osteoarthrosis deformans.
