Find information on thousands of medical conditions and prescription drugs.

Wolfram syndrome

Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness), is a rare genetic disorder, causing diabetes mellitus, optic atrophy, and deafness. It was first diagnosed in 1938 by a physician named Wolfram in four siblings. The disease affects both the brain (especially the brain stem) and the central nervous system. It is thought to be caused by both a malfunction of the mitochondria and of myelination, the latter in effect similar to multiple sclerosis. There is no known treatment. more...

Home
Diseases
A
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
Panniculitis
Waardenburg syndrome
Wagner's disease
WAGR syndrome
Waldenstrom...
Wallerian degeneration
Warkany syndrome
Warts
Waterhouse-Friderichsen...
Watermelon stomach
Wegener's granulomatosis
Weissenbacher Zweymuller...
Werdnig-Hoffmann disease
Werner's syndrome
Whipple disease
Whooping cough
Willebrand disease
Willebrand disease, acquired
Williams syndrome
Wilms tumor-aniridia...
Wilms' tumor
Wilson's disease
Wiskott-Aldrich syndrome
Wolf-Hirschhorn syndrome
Wolff-Parkinson-White...
Wolfram syndrome
Wolman disease
Wooly hair syndrome
Worster-Drought syndrome
Writer's cramp
X
Y
Z
Medicines

Life expectancy of people suffering from this syndrome is about 30 years.

See also : List of rare diseases

Read more at Wikipedia.org


[List your site here Free!]


Insulin-like growth factor-I can be helpful towards end of life - Brief Article - Letter to the Editor
From British Medical Journal, 3/17/01 by Aidan Bolger

EDITOR--In their editorial Smith et al review the literature relating high circulating concentrations of insulin-like growth factor-I to the risk of developing certain malignancies.[1] They warn of the dangers of the use of exogenous insulin-like growth factor-I by athletes, in whom the additional lifetime risk of developing an associated cancer may be increased. Towards the end of life, however, maybe the anabolic anti-apoptotic properties of insulinlike growth factor-I be should be considered in a different light.

Cardiac cachexia is a devastating phenotype of chronic heart failure associated with high morbidity and reduced survival.[2] High concentrations of growth hormone and inappropriately low concentrations of insulin-like growth factor-I are recognised features of this syndrome and suggest that a state of growth hormone resistance pervades.[3] The finding that skeletal muscle bulk also correlates inversely with concentrations of insulin-like growth factor-I hints at a pathophysiological link between these findings.[4] Furthermore, in dogs with heart failure, which have a high incidence of cachexia, those with raised concentrations of circulating insulin-like growth factor-I actually show an advantage in survival.[5] These findings perhaps reflect the possible advantages of higher concentrations of insulin-like growth factor-I in chronic disease alluded to by Smith et al.[1]

In patients with chronic wasting diseases approaching the end of life, when the theoretical risk of treatment associated malignancy can reasonably be ignored, higher concentrations of insulin-like growth factor-I may be beneficial. There is, therefore, a valid argument for the use of exogenous, anabolic, anti-apoptotic compounds, such as insulin-like growth factor-I, in catabolic states where mortality is high and even short term benefits are elusive. Such new approaches to the treatment of a crippling syndrome such as cardiac cachexia would be most welcomed.

Aidan Bolger research fellow a.bolger@ic.ac.uk

Wolfram Doehner research fellow

Stefan D Anker research associate Department of Clinical Cardiology, National Heart and Lung Institute, London SW3 6LY

[1] Smith GD, Gunnell D, Holly J. Cancer and insulin-like growth factor-I. BMJ 2000;321:847-8. (7 October.)

[2] Anker SD, Ponikowski P, Varney S, Chua TP, Clark AL, Webb-Peploe KM, et al. Wasting as independent risk factor for mortality in chronic heart failure. Lancet 1997;349:1050-3.

[3] Anker SD, Chua TP, Ponikowski P, Harrington D, Swan JW, Kox WJ, et al. Hormonal changes and catabolic/anabolic imbalance in chronic heart failure and their importance for cardiac cachexia. Circulation 1997;96:526-34.

[4] Niebauer J, Pflaum C-D, Clark AL, Strasburger CJ, Hooper J, Poole-Wilson PA, et al. Deficient insulin-like growth factor-1 in chronic heart failure predicts altered body composition, cytokine and neurohormonal activation. JAm Coil Cardiol 1998;32:393-7.

[5] Freeman LM, Rush JE, Kehayias JJ, Ross JN Jr, Meydani SN, Brown DJ, et al. Nutritional alterations and the effect of fish oil supplementation in dogs with heart failure. Intern Med 1998;12:440-8.

COPYRIGHT 2001 British Medical Association
COPYRIGHT 2001 Gale Group

Return to Wolfram syndrome
Home Contact Resources Exchange Links ebay