One of the only
treatments for this disorder is removal of the tumors which is done like the
removal of any other tumor. New advancements are made everyday toward finding
the secret behind this disorder. Taking this fact into consideration, doctors
must always be kept up to date concerning new procedures and treatment of this
disorder. Even though there have been huge advances in understanding this
disorder there is no medical therapy available. The diagnosis of NF1 and NF2 are
still largely based on clinical criteria. The diagnosis for NF1 was established
by the NIH Consensus Development Conference. They stated specifically that 2 or
more of the following must be present: (1) 6 or more Café-au-lait macules (brown
spots) are present, (2) 2 or more neurofibromas (tumors), (3) freckling in the
axillary or inguinal regions, (4) an optic pathway tumor, (5) 2 or more Lisch
nodules, (6) a distinctive, osseous lesion, such as sphenoid wing dysplasia or
thinning of the cortex of the long bones, and (7) a first-degree relative
(parent, sibling, or offspring) with NF1 by the above criteria. To be diagnosed
with NF2 the following must be included: (1) bilateral eight-nerve masses
visualized by MR imaging or (2) a first degree relative with NF2 and either
inilateral eigth-nerve masses or two of the following: neurofibroma, meninggioma,
glioma, schwannoma, or jeuvanile posterior subcapsular lenticular opacity.
Neurofibromatosis used to be just another disease that only doctors knew about.
One man and his life changed this forever, the Elephant Man. This disease became
most well known after the broadway production of his life. The elephant man
spent most of his known life as a profecional circus friek. He first appeared
in1884 and his real name was Joseph Merrick. Because of his willingness to let
people see him, and the play that was created around his life, the amount of
reasearch done to find everything possible about this disease was certainly
increased. He had numerous tumors an the right side of his body and face. His
right hand was 3 to 4 times larger that the average man. His life had certainly
spread the knowlage and increased the knowledge we have today of
Neurofibromatosis.
Bibliography
Cook, Robert. 1994. The Gene Wars: Science, Politics, and Human Genome. W.W.
Norton Co., New York. Howell, Michael and Peter Ford. 1980. The True History of
the Elephant Man. Penguin Books, New York. Lewis, Ricki. 1994. Human Genetics,
Concepts and Applications. Wm. C. Brown Publishers, Dubuque. Lyon, Jeff. 1995.
Altered Fates. W.W. Norton Co., New York Milunsky, Aubrey MD. 1992. Heredity and
Your Family’s Health. John Hopkins University Press, Baltimore. Side, Lucy MB.
1997. “Homozygous Inactivation of the NF1 Gene in Neurofibromatosis Type 1 and
Myeloid Disorders.” The New England Journal of Medicine. 336, 1713-19.
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