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Nephrogenic Systemic Fibrosis Symptoms
Nephrogenic Systemic Fibrosis (NSF) is a rare but
potentially fatal degenerative disease of the skin and
internal organs. First identified in 1997, it occurs
only in a few patients -- those who have a serious kidney
disease and have undergone magnetic resonance imaging
(MRI) or magnetic resonance angiography (MRA) tests.
Many scientists believe NSF is caused by exposure to
gadolinium, a chemical used as a dye in MRI/MRA tests.
In fact, one study showed that more than 95 percent
of NSF patients had been exposed to some type of gadolinium
within three months of contracting the disease. In response,
the FDA has already recommended that doctors avoid gadolinium,
and MRI/MRAs in general, in patients with kidney failure.
Researchers in the United States and Europe are working
to determine whether the substance should be banned
altogether.
Scientists believe gadolinium may cause NSF because
it reacts badly to high levels of acid in the body.
Because patients with kidney failure aren't able to
process impurities out of their blood as well as healthy
people, they have a much higher level of acid in their
bodies, a condition known as acidosis. The brand
names of gadolinium-containing dyes that are approved
for use in the United States with MRIs include:
* Magnevist
* MultiHance
* Omniscan
* OptiMARK
* ProHance
Brand names of gadolinium-containing dyes used in other
countries include Dotarim, Gadovist, Primovist and Vasovist.
Painful, Debilitating Symptoms
NSF was originally called nephrogenic fibrosing
dermopathy, because its first and most obvious symptoms
are lesions or rough patches on the patient's skin.
Over a period of days to weeks, patients notice a swelling
and tightening of the skin, especially the skin on the
arms, hands, legs and feet. This is often symmetrical,
meaning that a spot on the patient's right arm will
appear in a similar place on the left arm. The skin
develops lesions -- areas where it gets redder or darker,
which burn, itch or send sharp pains through the area.
On a microscopic level, scientists have found that these
lesions are caused by a buildup of too much skin and
connective tissue -- essentially, scar tissue.
Over time, the lesions harden and become "woody,"
resembling an orange peel. Eventually, this hardening
makes it impossible for patients to bend their fingers,
elbows, knees or other joints -- robbing patients of
their ability to walk, perform daily tasks or even care
for themselves. And the disease attacks other organs
in the same way, leading to a buildup of scar tissue
that could eventually stop internal organs from working
as well, causing death.
* In addition to these symptoms, NSF patients have
also reported: Blister-like spots on the hands
* Muscle weakness
* Bone pain in the hips and ribs
* Yellow lesions on or near the eyes
* Sudden development of hypertension (high blood
pressure)
Confounding Diagnoses
Because NSF is only found in patients who already
have kidney failure, its symptoms are often disguised
by existing symptoms of a serious disease. Indeed,
some scientists believe that NSF is underdiagnosed in
kidney patients. In addition, NSF is often found about
two weeks after the patient underwent a serious medical
problem or a surgery, such as failure of a transplanted
kidney, deep-vein thrombosis, angioplasty or another
traumatic event that affects the blood vessels and the
heart. Researchers are currently investigating whether
these traumas cause NSF or are some of its effects.
Many of them would be treated or diagnosed with MRI/MRA
tests, suggesting that the tests -- and their use of
gadolinium -- could be the problem.
Crippling Effects
Even if it didnt affect patients' independence,
NSF would be disabling because of its painful and disfiguring
effects on the skin. But because it also causes
muscle weakness, joint pain and physical inflexibility
in patients who already have serious kidney problems,
NSF can be literally crippling, putting some patients
in a wheelchair within weeks of diagnosis. In severe
cases, patients may need assistance just to move, care
for themselves and perform the daily tasks of living.
For people who have fought hard to live independent
lives after renal failure, this can be emotionally devastating
as well. And NSF presents a very real threat of death:
directly, through buildup of scar tissue on vital internal
organs, or indirectly, through falls and accidents caused
by the patient's loss of mobility. Thats just
too high a price to pay for a simple diagnostic test.
As of December 21, 2006, the U.S. Food and Drug
Administration knew of at least 215 cases of NSF worldwide.
Of these, all of the patients whose medical histories
were reviewed in detail had been exposed to gadolinium
in an MRI or MRA. In addition, tests have found gadolinium
in the skin lesions of people with NSF. While more research
is needed to confirm these findings, this very strong
correlation between gadolinium and NSF has already led
to lawsuits across the country. As researchers begin
to confirm the link between NSF and gadolinium, observers
expect many more.
Author: Peter Kent
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