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Wolfram Syndrome
Wolfram SyndromeA syndrome comprising diabetes insipidus, a mild form of diabetes mellitus, optic atrophy, and deafness. It is an autosomal recessive inherited disorder, with the chromosomal abnormality on the short arm of chromosome 4. Also called DIDMOAD, for diabetes insipidus, diabetes mellitus, optic atrophy and deafness. RELATED TERMS-------------------------------------- Syndrome A grouping of signs and symptoms, based on their frequent co-occurrence, that may suggest a common underlying pathogenesis, course, familial pattern, or treatment selection. Diabetes A condition in which blood glucose is not well controlled. Type I diabetics make no insulin, whereas type 2 diabetics are characterized by the overproduction of insulin, but the inability of the target cells to respond to the insulin. Atrophy A defect or failure of cell nutrition manifested as decrease in size or healthiness of an organ or tissue. Deafness A general term for the complete or partial loss of the ability to hear from one or both ears. Deafness may result from ear diseases; vestibulocochlear nerve diseases; or brain diseases. Autosomal "Pertaining to a chromosome that is not a sex chromosome; relating to any one of the chromosomes save the sex chromosomes. People normally have 22 pairs of autosomes (44 autosomes) in each cell together with two sex chromosomes (X and Y in the male and XX in the female). " Chromosome A structural unit within a eukaryotic nucleus that carries genes. A chromosome consists of a long, continuous strand of DNA and associated proteins. DIDMOAD An acronym that stands for Diabetes Insipidus (inability to concentrate the urine), Diabetes Mellitus, Optic Atrophy (degeneration of the nerve to the eye), and Deafness. These features are characteristic of this genetic disease which is also called the Wolfram syndrome. Patients usually also suffer from severe abnormalities of the nervous system that can be accompanied by behavior problems, psychiatric hospitalizations and, in about a quarter of cases, suicide attempts. SIMILAR TERMS-------------------------------------- Wolff Parkinson White Syndrome (WPW) WPW is a form of supraventricular tachycardia (fast heart rate originating above the ventricles). When you have WPW, along with your normal conduction pathway, you have extra pathways called accessory pathways. These electrical impulses set up a short circuit causing the heart to beat rapidly and conduct impulses in both directions. The impulses travel through the extra pathway (short cut) as well as the normal AV-HIS Purkinje system. The impulses can travel around the heart very quickly, in a circular pattern, causing the heart to beat unusually fast. This is called re-entry tachycardia. Wolff-Parkinson-White syndrome An extra electrical pathway that connects the atria and ventricles and causes rapid heartbeat. Wolffian duct structures The embryonic structures that develop into the internal reproductive anatomy of the male, attached to the testicles; named for Kaspar F. Wolff, German embryologist (1733-1794). Wolfina Wolfina is a prescription or over-the-counter drug which is (or once was) approved in the United States and possibly in other countries. Active ingredient(s): rauwolfia serpentina. PREVIOUS AND NEXT TERMS-------------------------------------- Wheeze A whistling or squeaky breathing sound caused by the narrowing (constriction) or blocking of the airway. Withdrawal The act or process of giving up the use of a drug to which one has become addicted or dependent. Wrist The joint or part of the arm between the hand and the forearm. Well-being (of trial subjects) The physical and mental integrity of the subjects participating in a clinical trial. Within-patient differences In a crossover trial, variability in each patient is used to assess treatment difference. Wolfram Syndrome Waiting List After evaluation by the transplant physician, and after committee presentation, a patient is added to the national waiting list by the transplant center. Lists are specific to both geographic area and organ type: heart, lung, kidney, pancreas, intestine, heart-lung, kidney-pancreas. Each time a donor organ becomes available, the computer generates a list of potential recipients based on factors that include blood type, organ size, medical urgency and time on the waiting list. A "new" United Network for Organ Sharing (UNOS) waiting list is generated each time an organ becomes available. There are many factors that go into the actual decision of donor selection and recipient matching. Although your place on the list and the amount of time you have been waiting for transplant are important factors, the transplant surgeon ultimately is the one to make the final decision. Wolff Parkinson White Syndrome (WPW) WPW is a form of supraventricular tachycardia (fast heart rate originating above the ventricles). When you have WPW, along with your normal conduction pathway, you have extra pathways called accessory pathways. These electrical impulses set up a short circuit causing the heart to beat rapidly and conduct impulses in both directions. The impulses travel through the extra pathway (short cut) as well as the normal AV-HIS Purkinje system. The impulses can travel around the heart very quickly, in a circular pattern, causing the heart to beat unusually fast. This is called re-entry tachycardia. We thank you for using the Medical Glossary to search for Wolfram Syndrome. If you have a better definition for Wolfram Syndrome than the one presented here, please let us know by making use of the suggest a term option. This definition of Wolfram Syndrome may be disputed by other professionals. Our attempt is to provide easy definitions on Wolfram Syndrome and any other medical topic for the public at large.
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