Dear conflating-ga;
Thank you for allowing me an opportunity to answer your interesting
question.
Here is the very wordy, academic description of “Chester-Erdheim
disease” sometimes called “Erdheim-Chester disease” or simply ECD, but
recognized in medical terminology as “Lipogranulomatosus
Erdheim-Chester”:
“Chester-Erdheim disease is a rare form of non-Langerhans cell
histiocytosis consisting of disseminated xanthogranulomatous
infiltration and fibrosis that primarily involves the bones, visceral
organs and systemic fatty spaces.”
“Advanced Chiropractic Neurology Center – module 9”
http://www.dendrites.com/module_09.htm
In layman’s terms, this disease, first recorded and studied by William
Chester, while working in the pathological institute in Vienna in
1931, is a rare condition (histiocytic disorder). The disease is
characterized by an abnormally excessive production and accumulation
of histiocytes (large “scavenger-like” cells that appear normally in
the system and are designed to hunt down and dispose of bacteria,
infection and cellular debris). Patients with ECD may have a number of
affected tissues such as the long bones, skin, tissues behind the
eyeballs, lungs, brain, pituitary gland, and/or other tissues and
organs. The accumulation of these histiocytes in the organs and issues
can, and sometimes does interfere with or inhibit the normal function
of the tissue or organ. If the disease has progressed to the point
that organs are not functioning properly, the prognosis is poor.
Mortality is 57%, usually from respiratory distress, cardiac failure,
or pulmonary fibrosis. The symptoms and treatment vary from one
patient to another depending on the area(s) affected and the extent of
involvement but some patients exhibit no symptoms at all.
Unfortunately, as for the potential heredity nature of the disease,
the disease is so rare (with less than 100 cases reported in the
literature) that the specific underlying cause(s) of ECD is unknown.
Jakob Erdheim, a German pathologist also studied the disease and the
eponym Erdheim-Chester was given to the disease some time later
although Chester actually identified the disease by himself.
Below you will find that I have carefully defined my search strategy
for you in the event that you need to search for more information. By
following the same type of searches that I did you may be able to
enhance the research I have provided even further. I hope you find
that that my research exceeds your expectations. If you have any
questions about my research please post a clarification request prior
to rating the answer. Otherwise, I welcome your rating and your final
comments and I look forward to working with you again in the near
future. Thank you for bringing your question to us.
Best regards;
Tutuzdad-ga
INFORMATION SOURCES
“Advanced Chiropractic Neurology Center – module 9”
http://www.dendrites.com/module_09.htm
WHONAMEDIT.COM
“Erdheim-Chester disease”
http://www.whonamedit.com/synd.cfm/2412.html
HEALTH LIBRARY – Erdheim Chester Disease
http://yalenewhavenhealth.org/library/healthguide/IllnessConditions/topic.asp?hwid=nord943
HARVARD
“Joint Program in Nuclear Medicine - Erdheim-Chester Disease”
http://www.med.harvard.edu/JPNM/TF00_01/Oct3/WriteUp.html
“Diagnosis: Erdheim-Chester Disease - Brief history:”
http://gamma.wustl.edu/bs115te146.html
NATIONAL HEART LUNG AND BLOOD INSTITUTE
“Erdheim Chester Disease Research Study - Diagnosis and Followup
Protocol”
http://patientrecruitment.nhlbi.nih.gov/ErdheimChester.aspx
For further information please contact our research coordinator (tel.
301-496-1500, E-mail: LipidStudy@nhlbi.nih.gov
THE DOCTOR’S DOCTOR
http://www.thedoctorsdoctor.com/diseases/erdheim_chester.htm
(A great deal of information is here)
VIVISIMO CLUSTERING ENGINE
http://vivisimo.com/search?query=Erdheim-Chester+&v%3Asources=AltaVista%2CMSN%2CNetscape%2CLycos%2CLooksmart%2CBBC%2CGigaBlast%2CVizzavi%2CLII%2CCNN%2CNYTimes%2CUSAToday%2CReuters%2CWashingtonPost%2CBBCNews%2CYahooNews%2CWorldNews%2CWired&form=Settings&overRequest=2&num=200&language=all&showURL=1&showSummary=1&showOpener=1&showSe=1&showPreview=1&target=self&timeout=5000&filter=none&enablejs=
HELP AND INFORMATION RESOURCES
Histiocytosis Association of America
302 North Broadway
Pitman, NJ 08071
Tel: (856)548-2758
Fax: (856)589-6614
Tel: (800)548-2758
Email: histiocyte@aol.com
Internet: http://www.histio.org
NIH/National Arthritis and Musculoskeletal and Skin Diseases
Information Clearinghouse
One AMS Circle
Bethesda, MD 20892-3675
Tel: (301)495-4484
Fax: (301)587-4352
TDD: (301)565-2966
NIH/National Institute of Neurological Disorders and Stroke
"Brain Resources and Information Network" (BRAIN)
PO Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Internet: http://www.ninds.nih.gov/
SEARCH STRATEGY
SEARCH ENGINE USED:
Google ://www.google.com
SEARCH TERMS USED:
CHESTER ERDHEIM DISEASE
“CHESTER-ERDHEIM”
“ERDHEIM-CHESTER”
“ERDHEIM-CHESTER” PROGNOSIS
ECD
“LIPOGRANULOMATOSUS ERDHEIM-CHESTER”
LIPOGRANULOMATOSUS
HISTIOCYTES
“JAKOB ERDHEIM”
“WILLIAM CHESTER” |
Request for Answer Clarification by
conflating-ga
on
02 Mar 2003 20:37 PST
I'm just not sure you're the best person to have answered this
question. The links you provide offer me technical descriptions
without any 'layman's terms' understanding of the symptoms or
diagnoses described. While I think you've technically (and quite
minimally, frankly) answered my question, I find this to be a pretty
thin synopsis of the disease itself and the various points I asked for
help with. In fact, the majority of your answer was cribbed straight
off one of the pages you linked me to. What would be useful as
clarification would be to actually explain the symptoms and causes,
and without resorting to jargon. For instance, in the first five
minutes of reviewing your links I read that the disease usually
presents itself in late adulthood, with 53 as the average age. You
neglected to mention this seemingly useful point. In addition, the
links you found state clear symptoms, and while these admittedly do
vary from case to case, I would have thought you would at least list
them (and explain them in non-technical language) rather than simply
state that "symptoms and treatment vary from one
patient to another depending on the area(s) affected and the extent of
involvement but some patients exhibit no symptoms at all", which is as
bland and uninformative a sentence as I have ever read. You don't
even explain why the accumulation of histiocytes is detrimental and
how this causes any of the listed symptoms. I think you are probably
not someone with medical training, and what you've done here is
essentially pull all the non-jargon sentences out of the links you've
found and provided them in your answer. Obviously I could do this
myself. The point of my question was for someone with some medical
expertise to actually clarify and explain what's on the web. I would
appreciate it if as clarification to your answer you could do this.
|
Clarification of Answer by
tutuzdad-ga
on
03 Mar 2003 11:09 PST
Dear conflating-ga:
As you can see, I have indeed answered your question (as asked) it
it’s entirety. Here you will find the most “common” symptoms (since
symptoms vary in patients who actually exhibit symptoms at all) for
each of these types of the disease:
Skeletal Manifestations (bones)
Neurologic Manifestations (nerves/brain)
Obital Manifestations (eyes)
Retroperitoneal Manifestations (soft tissues)
Pulmonary Manifestations (heart, lungs, blood related organs)
The prognosis of the disease is clearly established in this section of
the article:
Prognosis and Therapy
HARVARD
http://www.med.harvard.edu/JPNM/TF00_01/Oct3/WriteUp.html
Listing symptoms for a disease that is not well understood and
documents as having a variety of symptoms is not possible. Each
patient tends to exhibit symptoms that may differ from other patients
since the disease affects each person differently. Beyond the common
symptoms (see above) very little is known about the disease. This is
an unfortunate scientific fact.
The other links provided address other specific portions of your
question in like manner.
As for an explanation of the causes of the disease, my research
clearly indicated that the cause is not known; therefore I cannot
speculate as to the reasons why some people get this rare disease and
some do not.
As for the reason why an accumulation of histiocytes is detrimental to
ones health, it goes without saying that the abnormal accumulation of
anything to such an extent that it interferes with the proper function
of an organ is dangerous. Little more can be can be said of this in
laymen’s terms that has not already been said.
Regarding your attack of my explanation and your opinion that my
answer was bland and uninformative, this is the result of offering
data in laymen’s terms. Had the medical terminology been something
familiar and useful to you, I would have used that. I’m certain had
this type of explanation had any meaning to you that you would have
appreciated a more technical and flowery explanation rather than the
more useful explanation in laymen’s terms. After translating the
technical terminology, the bland explaination is basically all that is
left – which is why it is preferable to the average person who have
little or no medical training.
With regard to your assumption that I have no medical training, you
are correct. Most physicians are busy practicing their craft and
rarely found on a question/answer forum. Had you asked for a physician
specifically, I would have passed on your question, which would likely
have languished unanswered until a physician came along to work on it
(provided that one did).
Finally, your statement that you found details about the disease in
the links that I provided, that I failed to list in my answer, let me
say this…Your statement comes as no surprise to me, in fact, this is
intentional. The links I provided were reviewed in their entirety for
pertinent content. They were provided to you BECAUSE THEY LISTED THE
INFORMATION, not in lieu of information. The links then ARE the
information, and as such, the supporting data for my accurate and
complete answer. I cannot (and did not, as per your earlier statement)
plagiarize someone else’s work. What I DID DO was provide you with the
most useful and complete information available and/or links to the
information about this rare and mysterious disease (in limited quotes
where necessary in order to convey some data accurately – careful of
course to credit the source – which does not constitute plagiarism, as
you incorrectly asserted). It is indeed unfortunate that you lost a
loved one to this tragic disease, and my heart sincerely goes out to
you. My sole purpose is to help you find the answers you are seeking,
and I feel that I have done that, based on the way your question was
presented. I cannot “make” you appreciate or understand what has been
given to you, nor can I manufacturer information where none exists. I
think you would agree that this would be a disservice to both of us.
At the same time, I am forced to generalize about the condition.
Neither I, nor a non-attending physician could (or would) attempt to
shed light on your loved one’s particular experiences with the
disease, having no previous knowledge of this specific example of the
illness. To attempt this would be profoundly irresponsible and serve
as indisputable evidence of professional incompetence. I hope that in
some small way you find a useful purpose in what little information is
known by medical science, represented completely and concisely by the
answer I have provided.
Please examine the links carefully and where data, statistics and
information are known, you will probably find much of what you need.
Regards;
Tutuzdad-ga
|
Clarification of Answer by
tutuzdad-ga
on
03 Mar 2003 13:39 PST
Please excuse the typo:
"procitive" should read "productive"
|
