Lymphangiectasia
Lymphangiectasia
is a condition in which the
lymphatics are dilated and is commonly associated with
lymphedema.. When
the lymph channels,vessels become dilated, the lymph valves cease to
work
correctly and the lymph fluid flows backward. Eventually, the fluid
leaks into
the interstitium causing swelling.
The condition can be either
primary (present from birth or
congenital) or secondary (acquired through trauma or injury to the
lymphatics).
Specific causes of secondary lymphangiectasia include radical
mastectomy with or
without radiation therapy; irradiation alone for various malignancies;
metastatic lymph node obstruction; and various scarring processes, such
as
infections, keloids, scleroderma, and scrofuloderma. It has also been
described
in the penis and the scrotum after removal of a sacrococcygeal tumor;
they may
also arise on the vulva and the inner thigh after surgery for cervical
cancer.
Other
complications associated with
lymphangiectasia are determined by the area of the body that is
effected. For a
complete detail of each type, please see the internal Lymphedema People
links at
the bottom of the page.
Historically,
intestinal lymphangiectasia (IL)
was thought to be the most common type. However, there is
more and more
documentation in the medical literature of the fact the lymphedema
patients have
secondary cutaneous (skin) lymphangiectasia as a result of the
streatching and
swelling involved with that condition. Other areas of
recorded
lymphangiectasia include the lungs, kidney (Renal), vulva,
penis,scrotum and
even conjunctiva.
Also, should
the lymphedema move into the
abdominal and/or chest cavities, intestinal and/or pulmonary
lymphangiectasia
can result.
-----------------------------------------------------
Lymphangiectasia
Is
a condition resulting in dilation of the lymphatic vessels
that is caused
by lymphatic damage, which leads to the blockage of local lymphatic
drainage.
This can be either a congenital or an acquired disorder of the
lymphatic
system. The most common form of lymphangiectasia is the
intestinal
form. (IL) The condition may however, be in other parts of
the body.
Other forms include pulmonary (lungs), renal (kidneys), skin,
or
retroperitoneal.
On microscopic examination,
lymphangiectases are
characterized by large, dilated lymph vessels lined by a single layer
of
endothelial cells. They are usually found in the papillary dermis and
the
reticular dermis. Involvement in the deeper dermis is rare. The
overlying
epidermis is usually associated with acanthosis and hyperkeratosis but
is less
marked than in angiokeratomas. At times, acquired lymphangiectases grow
above
the level of adjacent skin.
Lymphangiectases lack the
subcutaneous muscle-coated
cisternae characteristic of lymphangioma circumscriptum. Although no
specific
histologic criteria exist with which to differentiate lymphangioma
circumscriptum from lymphangiectasia, some believe that the absence of
subcutaneous cisternae in lymphangiectases is a true histologic
difference and
that this finding reflects the difference in the pathogenesis of both
disorders.
Risk
Factors
- congenital (primary) - most
common cause is a congenital malformation of the lymphatics.
- acquired (secondary)
-
- congestive heart failure
- constrictive
pericarditis
- cirrhosis
- any disorder causing
inflammation of the mesentery (i.e., Crohns Disease)
- severe abdominal
swelling or edema (although IL also exacerbates existing
swelling)
- lymphedema
Signs and Symptoms
- Low Lymphocyte Count
Patients with lymphangiectasia
have lymphocytes rolling out their lacteals and into their
intestine. The low blood lymphocyte count is so consistent
with lymphangiectasia that it is difficult to make this diagnosis if
this finding is not present.
- Low Cholesterol
Cholesterol is part of the lymph fluid being
lost.
- Low Albumin Level
Low blood albumin level is the most
consistent finding in lymphangiectasia, though it is possible to have
lymphangiectasia in a small portion of the intestine only and still
maintain a normal albumin level. There are only four ways a
patient can develop a low albumin level.
- Diarrhea is
a symptom of intestinal lymphangiectasia
...............................................
AN IMPORTANT CAUSE
OF INTESTINAL PROTEIN LOSS
WHAT IS A PROTEIN-LOSING
ENTEROPATHY?
“Protein-losing
Enteropathy” is a fancy way of saying
there is something wrong with the intestine such that protein is being
lost from
the body through the intestine. This is a serious problem as the body’s
proteins are not easily replaced and the only way to replace them
involves the
absorption of protein constituents (the amino acids that make up
proteins) from
the intestine. If the intestine is actually leaking nutrients out
instead of
absorbing them in, the result is a nutritional disaster.
The main
protein which one cannot afford to lose is called “Albumin.”
This protein normally is produced by one’s liver and circulates in the
bloodstream acting as a carrier for biochemicals that require transport
but
cannot actually dissolve in blood. Albumin can be considered sort of a
mass
transit system in the bloodstream, a bus or subway, if you will,
carrying
important biochemicals from one place to another.
Albumin, by
being the most prevalent blood protein, also is
responsible for actually keeping water in one’s bloodstream.
When water
cannot be held within the vasculature, it leaks out causing fluid
accumulation
in tissue (i.e. edema) or in within the chest or abdomen (i.e.
effusion).
Of course, in
a protein-losing enteropathy, other proteins are
lost, too. Antibodies, proteins of blood clotting, enzymes, etc. all
leak out
the intestine and are forever lost in the feces that exits the body.
The body tries
hard to maintain its albumin level by
extracting protein from other sources (like muscle), and having the
liver make
albumin from the components of these other proteins. This may
help
maintain a workable amount of albumin in the bloodstream but it comes
at the
expense of muscle tissue and other protein.
There are four
chief cases of protein-losing enteropathy:
The first
three conditions are reviewed elsewhere and will not
be discussed here.
WHAT IS INTESTINAL
LYMPHANGIECTASIA?
Lymph is a
fluid that circulates through the body similar to
the way blood does; though blood is pumped actively through the body by
the
heart while lymph is pumped passively via the normal muscle activity of
the
body. Lymph consists of cells called “lymphocytes,” which are
cells of
the immune system. Lymph also consists of fluid which collects from the
tissues
and shunts into actual vessels similar to veins.
The word
“lymphangiectasia” means “dilated lymph
vessels.” In the intestinal tract, lymphangiectasia is usually caused
by some
kind of inflammation which puts back pressure on the lymph vessels
leading them
to dilate. Lymph flow may be blocked by the inflammatory events in the
intestine
or local structures.
Lacteals are
special lymph vessels in the intestinal tract
designed to absorb nutritional fats. When there is high pressure within
the
lymph vessels, the tender lacteals burst and instead of absorbing fats,
the
lymph inside, its cells, fats, and precious proteins are
lost. The
intestine may be able to reabsorb some of these valuable substances at
other
sites but if the inflammatory intestinal disease that started the
problem in the
first place is widespread, the balance may have shifted to nutritional
loss
rather than gain.
WHAT DOES ONE SEE AT HOME?
Weight loss is
the most consistent sign along with chronic
diarrhea, vomiting, and fluid accumulation in the abdomen creating a
bloated
appearance.
HOW DO WE MAKE A DIAGNOSIS?
In most cases,
an obviously sick dog is brought to the
veterinarian. Sometimes the above classical signs are present but
sometimes
there is no specific hint of this condition until blood test results
are in.
- Low Lymphocyte Count
Animals with lymphangiectasia have
lymphocytes rolling out their lacteals and into their
intestine. The low blood lymphocyte count is so consistent
with lymphangiectasia that it is difficult to make this diagnosis if
this finding is not present.
- Low Cholesterol
Cholesterol is part of the lymph fluid being
lost.
- Low Albumin Level
Low blood albumin level is the most
consistent finding in lymphangiectasia, though it is possible to have
lymphangiectasia in a small portion of the intestine only and still
maintain a normal albumin level. There are only four ways a
patient can develop a low albumin level.
- A
protein-losing enteropathy
- Glomerular
disease (such as glomerulonephritis) in which albumin is lost through
diseased kidneys
- Reduced
albumin production in the liver due to liver failure
- Serum
leakage through extreme skin damage (such as third degree burns)
These
conditions can be easily ruled out one by one. It is
obvious if there are third degree burns present. If there are none, a
routine
urinalysis will indicate if there is significant protein loss in the
urine and
if glomerular disease should be pursued. A liver function test such as
a bile
acids test will indicate whether or not there is liver failure latently
present.
If none of these three conditions are present, then there must be a
protein-losing enteropathy.
BIOPSY
As mentioned,
there are four likely causes of protein-losing
enteropathy. To distinguish them and initial the correct treatment, an
intestinal biopsy is essential. This can be done surgically or via
endoscopy but
rational treatment is not possible without a tissue sample.
TREATMENT AND WHAT TO EXPECT
The first step
in treatment is to address the underlying
cause. In most cases of lymphangiectasia, the underlying cause involves
inflammation and most treatment of lymphangiectasia involves
suppression of
inflammation.
Medications
such as prednisone,
and/or azathioprine
are commonly used, especially if inflammatory
bowel disease is present.
The second
step in treatment is dietary though success has
been mixed. Traditionally, rather nasty tasting Medium Chain
Triglycerides have
been used in lymphangiectasia treatment. Triglycerides (a fancy word
for
“fats”) are very long molecules. Some are longer than others. The more
usual
dietary fats are called “Long Chain Triglycerides” and, when absorbed
into
one’s body, must be repackaged into fat globules called “chylomicra”
and
are normally absorbed into the lymph vessels. In lymphangiectasia, we
want to
reduce the pressure in the lymph vessels. We want less lymph. The idea
was that
if the patient ate shorter fat chains, the fats could be absorbed right
into
bloodstream directly and bypass the lymph system altogether. Whether or
not this
actually happens is still a matter of controversy but the addtion of
Medium
Chain Triglycerides (or “MCT’s”) in conjunction with a low fat diet are
common recommendations in the therapy of lymphangiectasia.
Other
treatments include the use of diuretics (such as furosemide)
to help increase urination and ultimately reduce fluid accumulation in
the chest
or abdomen. Actual tapping of the body cavity and suctioning the fluid
affected
may be needed periodically.
If the
underlying condition is treatable then prognosis for
lymphangiectasia is good. It should be understood that
lymphangiectasia is
unlikely to be cured and at best can be managed.
http://www.marvistavet.com/html/intestinal_lymphangiectasia.html
..............................................
Pulmonary
lymphangiectasia
Primary
pulmonary lymphangiectasia in infancy and childhood
P.M.
Barker1, C.R. Esther,
Jr1,
L.A. Fordham2, S.J.
Maygarden3
and W.K. Funkhouser3
Depts of 1
Paediatrics (Division of Paediatric
Pulmonology), 2 Radiology, and 3
Pathology, University of
North Carolina at Chapel Hill, Chapel Hill, NC, USA
Correspondence:
P.M. Barker, Division of Paediatric
Pulmonology, Dept of Paediatrics, 200 Mason Farm Road, CB 7220,
University of
North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA. Fax: 1
9199666179.
E-mail: Pierre_Barker@med.unc.edu
Keywords: Growth,
pathology, radiology, spirometry
Received:
February 4, 2004
Accepted May 20, 2004
C.R. Esther Jr
is supported by a Parker B. Francis Pulmonary
Fellowship.
Primary
pulmonary lymphangiectasia (PPL) is a rare disorder of
unknown aetiology characterised by dilatation of the pulmonary
lymphatics.
PPL is widely reported to have a poor prognosis in
the neonatal
period and little is known about the clinical features
of patients
who survive the newborn period.
The current
authors report the outcome in nine patients diagnosed in
infancy with PPL over a 15-yr period at a single university-based
hospital
clinic and followed for a median of 6 yrs.
Although all
of the patients initially experienced respiratory distress,
respiratory symptoms improved in most patients after infancy
and were
notably better by the age of 6 yrs. Many patients
had poor
weight gain in the first years of life, which eventually
improved.
Radiological scans showed progressive resolution of
neonatal
infiltrates, but were characterised by hyperinflation and
increased
interstitial markings in older children. Most patients
had evidence
of bronchitis and grew pathogenic organisms from
quantitative
bronchoalveolar lavage culture. Pulmonary function
tests showed
predominantly obstructive disease that did not
deteriorate over time.
In conclusion,
these results suggest that primary pulmonary lymphangiectasia
does not have as dismal a prognosis as previously described
and
symptoms and clinical findings improve after the first
year of life.
http://erj.ersjournals.com/cgi/content/abstract/24/3/413
...............................................
Congenital
dilatation of the pulmonary lymphatics
SN
Javett, I Webster and JL Braudo
904 Medical Centre Jeppe Street, Johannesburg, South Africa
The disease
variously referred to as congenital dilatation of the pulmonary,
lymphatics congenital pulmonary cystic lymphangiectasis, and
congenital
lymphangiomatosis of the lung is a rarity known almost exclusively
to
pediatric pathologists. Up to 1959, 21 cases had appeared in
world
literature, with only two reports in clinical journals. Congenital
dilatation
of the pulmonary lymphatics has been observed as a chance finding
in
fetuses dying in utero, stillborn infants, and neonates, without
any
mention being made as to its possible significance. The great majority
of
those born alive have suffered in the immediate neonatal period from an
acute
respiratory disease exhibiting dyspnea and cyanosis, death following
within
a few hours. As a consequence, the opportunity for clinical observation
and investigation has been strictly limited. Confirmation has
been
possible only by autopsy. With virtually no clinical information to
work
on, the pathologists have centered interest almost entirely on the
identification
of lymphatics, the histopathology, and theories of causation
without
being in a position to offer correlation between histologic findings
and symptoms. In the absence of any clinical build-up, too, it is
not
surprising that the disease has escaped the attention of most
clinicians.
We have had the opportunity of observing and investigating a
patient
with congenital dilatation of the pulmonary lymphatics, still
alive,
albeit not well, at the age of 8 months. It is the purpose of this
paper
to describe as a clinical entity a condition hitherto regarded as a
pathological
curiosity.
http://pediatrics.aappublications.org/cgi/content/abstract/31/3/416
...............................................
Lymphangiectasia,
retroperitoneal
|
Lymphangioma, retroperitoneal,
|
|
 |
|
a developmental malformation and not a true
neoplasm, though it presents as a retroperitoneal mass.
Lymphangiectasia is the dilatation of abnormal lymphatic channels,
which have failed to establish normal communication with the rest of
the lymphatic system. The dilated lymphatic channels conglomerate and
form a unilocular or multilocular cystic mass, known as a lymphangioma.
The
diagnosis of retroperitoneal lymphangioma is usually made in infancy.
It occurs with equal frequency in males and females. Common clinical
findings include pain, abdominal distension, fever, fatigue, weight
loss and haematuria.
A
retroperitoneal lymphangioma can be imaged by ultrasound, CT or MRI. It
may be elongated in contour and characteristically spans more than one
retroperitoneal compartment. Uncomplicated fluid is seen within the
mass or locules, which are separated by thin septa. The fluid appears
complicated in the presence of haemorrhage or infection. Rarely,
calcification and chyle are present
|
http://www.amershamhealth.com/medcyclopaedia/medical/volume%20iv%202/lymphangioma%20retroperitoneal.asp
...............................................
Renal Lymphangiectasia
Lorenz T.
Ramseyer, MD
1
From Bass Baptist Memorial Hospital, 600 S
Monroe, Enid, OK 73701. Received May 28, 1999; revision requested July
20;
revision received August 26; accepted August 30. Address
correspondence to
the author (e-mail: lramseyer@peakonline.com)
Index terms:
Diagnosis Please
• Kidney, CT, 81.12112 • Lymphatic system, abnormalities, 994.829 •
Lymphatic system, CT, 994.12912 • Urography, 81.11
HISTORY
A 28-year-old
obese man had microscopic hematuria. Physical examination
results were normal. His medical history was unremarkable, and
there
was no pertinent family history. Excretory urography (Fig
1) and computed tomography (CT) of the abdomen
were performed after oral and intravenous administration of
contrast material.
IMAGING FINDINGS
The excretory
urogram demonstrated bilateral nephromegaly with distortion
of the pelvocaliceal systems (Fig 1).
The CT scan of
the abdomen showed fluid collections in the perinephric space
bilaterally,
surrounding the renal cortex. In addition, there
were peripelvic
fluid collections bilaterally, with distortion of
the pelvocaliceal
systems. There were fluid collections in the
retroperitoneum that
crossed the midline at the level of the renal
hila, adjacent to the
abdominal aorta and the inferior vena cava.
DISCUSSION
Several
differential diagnostic possibilities are to be considered with
bilateral nephromegaly with pelvocaliceal splaying and distortion.
Adult polycystic kidney disease, lymphoma, nephroblastomatosis,
and
other causes of multiple renal masses, such as von Hippel-Lindau
disease
and tuberous sclerosis, are considerations. Adult polycystic
kidney
disease has characteristic findings of numerous bilateral renal
cysts, with or without hepatic or pancreatic cysts. The cysts
typically vary in size and are scattered throughout the parenchyma.
Lymphoma and other malignancies can demonstrate soft-tissue
masses
involving the kidneys, pelvocaliceal systems, or
retroperitoneum. The
fluid attenuation of the perinephric collections
in the test case, as
evidenced by attenuation measurements of 0–10
HU in exclude lymphoma and other soft-tissue masses.
Perinephric
and retroperitoneal soft-tissue masses can be
seen in retroperitoneal
fibrosis but are again excluded by the fluid
attenuation in the test
case. Nephroblastomatosis is a cause of
nephromegaly in children and
is characterized by multiple subcapsular and
parenchymal soft-tissue
nodules composed of metanephric blastema (1).
Renal
lymphangiectasia is a rare disorder. Patient symptoms described
in the literature (2–4) include
hematuria, flank pain, and abdominal pain. The
condition has been
found in children and in adults (2,4–7).
The origin of this disorder is speculative.
There is a familial
association in some cases, which argues for a
congenital cause (6).
There was no known family history of renal
lymphangiectasia in the
test case. Others argue for an acquired cause,
which suggests that
the lymphatic vessels may become blocked owing
to inflammation or
other obstruction and so cause lymphatic
ectasia (8).
Others have suggested that these lesions may be
a true neoplasm (9).
The nomenclature of this disorder is confusing
and has evolved in
recent years. Other names have included "renal
lymphangiomatosis"
(3,6),
"renal lymphangioma"
(5), "peripelvic
lymphangiectasia" (2),
and "renal peripelvic multicystic
lymphangiectasia" (8).
"Renal lymphangiectasia" is the preferred name,
given that
the disorder is characterized by ectatic
perirenal, peripelvic, and
intrarenal lymphatic vessels (10,11).
Imaging
findings of renal lymphangiectasia include peripelvic cysts
and perirenal fluid collections. The finding of retroperitoneal
fluid
collections, presumably dilated lymphatic vessels, is a
more variable
finding but has been noted in multiple cases in
the literature (2–4,6).
Renal
lymphangiectasia has been found associated with renal venous
thrombosis and hypertension (4,6,12).
Ascites and large perinephric fluid collections
have been found and
are exacerbated by pregnancy (6).
The natural
history of this disorder is not completely
understood. In a neonatal
case, partial regression was reported (7).
The diagnosis
of renal lymphangiectasia can be confirmed with needle
aspiration of chylous fluid from the perinephric fluid collections
(4).
However, the ultrasonographic and CT findings are
characteristic for
this disease and allow the diagnosis to be made
confidently (2,6).
Treatment is not usually necessary. Complicated
cases may be treated
with nephrectomy, percutaneous drainage, or
marsupialization (4).
In the case
presented here, the diagnosis of renal lymphangiectasia was
based on the characteristic CT findings. The perirenal, peripelvic,
and retroperitoneal collections had attenuation measurements
consistent with fluid (0–10 HU) rather than with
the soft-tissue
attenuation seen with lymphoma or other causes
of bilateral renal
soft-tissue masses . No invasive procedures to
confirm diagnosis were deemed
appropriate for this patient.
Our
congratulations to the 26 individuals who submitted the most
likely diagnosis (renal lymphangiectasia) for Diagnosis Please,
Case
34. The names and locations of the individuals, as
submitted, are as
follows
- Marc P.
Banner, Philadelphia, Pa; Lawrence R. Bigongiari,
MD, Hope, Ark; Marc G. de Baets, MD, Lugano,
Switzerland
- Kemal
Demir, MD, Ataköy, Istanbul, Turkey; Giovanna
Demurtas, MD, Cagliari, Italy; Luis
E. Fajre, MD, Tucuman, Argentina; Sandra K.
Fernbach, Chicago, Ill; Milton R. Fuentealba, MD,
General Roca, Rio Negro, Argentina;
- Celso
Ichihara, Brazil; Kartik Jhaveri, MD, Mumbai,
India; Douglas S. Katz, MD, Mineola, NY;
Glenn Krinsky, MD, New York, NY;
Prof. Dr. Luis Mendez Uriburu, Tucuman,
Argentina; Sergio J. Moguillansky, MD, Cipolletti,
Rio Negro, Argentina; Adalberto
Montanhini Júnior, Brazil; Steven
Perlmutter, MD, Mineola, NY; Timothy J.
Phalen, MD, Cincinnati, Ohio;
Dr. Arturo Ramos-Pablos, Cd. Obregón, Son.,
México; Luiz Antonio Rossi, São Paulo,
Brazil; Anthony J. Scuderi, MD, Johnstown,
Pa; Matt Shapiro, MD, Lowell,
Mass; Paolo Siotto, MD, Cagliari,
Italy; Douglas L. Teich,
MD, Brookline, Mass; Christopher Vittore, MD, Rockford,
Ill; Keith Wittenberg, MD, Rochester,
Minn; Joe Yut, Olathe, Kan
FOOTNOTES
Part 1 of this
case appeared 4 months previously and may contain larger
images.
REFERENCES
- Amis
ES, Newhouse JH. The kidney: tumors. Essentials of uroradiology Boston,
Mass: Little, Brown, 1991; 136.
- Murray
KK, McLellan GL. Renal peripelvic lymphangiectasia: appearance at CT.
Radiology 1991; 180:455-456.
[Abstract]
- Varela
JR, Bargiela A, Requejo I, Fernandez R, Darriba M, Pombo F. Bilateral
renal lymphangiomatosis: US and CT findings. Eur Radiol 1998; 8:230-231.
[Medline]
- Riehl
J, Schmitt H, Schafer L, Schneider B, Sieberth HG. Retroperitoneal
lymphangiectasia associated with bilateral renal vein thrombosis.
Nephrol Dial Transplant 1997; 12:1701-1703.
[Abstract]
- Blumhagen
JD, Wood BJ, Rosenbaum DM. Sonographic evaluation of abdominal
lymphangiomas in children. J Ultrasound Med 1987; 6:487-495.
[Abstract]
- Meredith
WT, Levine E, Ahlstom NG, Grantham JJ. Exacerbation of familial renal
lymphangiomatosis during pregnancy. AJR Am J Roentgenol 1988;
151:965-966. [Medline]
- Pickering
SP, Fletcher BD, Bryan PJ, Abramowsky CR. Renal lymphangioma: a cause
of neonatal nephromegaly. Pediatr Radiol 1984; 14:445-448.
[Medline]
- Kutcher
R, Mahadevia P, Nussbaum MK, Rosenblatt R, Freed S. Renal peripelvic
multicystic lymphangiectasia. Urology 1987; 30:177-179.
[Medline]
- Leonidas
JC, Brill PW, Bhan I, et al. Cystic retroperitoneal lymphangioma in
infants and children. Radiology 1978; 127:203-208.
[Abstract]
- Levine
E. Renal lymphangiectasia (letter). Radiology 1992; 182:582.
[Medline]
- Levine
E. The kidney. In: Haaga JR, Lanzieri CF, Sartoris DJ, Zerhouni EA,
eds. Computed tomography and magnetic resonance imaging of the whole
body. 3rd ed. St Louis, Mo: Mosby, 1994; 1199.
- Lindsey
JR. Lymphangiectasia simulating polycystic disease. J Urol 1970;
104:658-662. [Medline]
http://radiology.rsnajnls.org/cgi/content/full/219/2/442
...............................................
A
Model for Mechanics of Primary Lymphatic Valves
Journal
of Biomechanical Engineering -- June 2003 -- Volume 125, Issue 3, pp.
407-414
Ernesto
Mendoza and Geert
W. Schmid-Schönbein
Department of Bioengineering, The Whitaker
Institute for Biomedical Engineering, University of California San
Diego, La Jolla, CA 92093-0412
(Received Sep. 2002; revised Dec. 2002)
Recent
experimental evidence indicates that lymphatics have two
valve systems, a set of primary valves in the
wall of the endothelial cells of initial
lymphatics and a secondary valve system in the
lumen of the lymphatics. While the intralymphatic secondary valves
are well described, no analysis of the primary valves is
available. We propose a model for primary lymphatics valves
at the junctions between lymphatic endothelial
cells. The model consists of two overlapping
endothelial extensions at a cell junction in the initial
lymphatics. One cell extension is firmly attached to the adjacent
connective tissue while the other cell extension is not attached
to the interstitial collagen. It is free to bend into
the lumen of the lymphatic when the lymphatic pressure falls
below the adjacent interstitial fluid pressure. Thereby the cell
junction opens a gap permitting entry of interstitial fluid
into the lymphatic lumen. When the lymphatic
fluid pressure rises above the adjacent
interstitial fluid pressure, the endothelial extensions contact each
other and the junction is closed preventing fluid reflow
into the interstitial space. The model
illustrates the mechanics of valve action and
provides the first time a rational analysis of the
mechanisms underlying fluid collection in the initial lymphatics and
lymph transport in the microcirculation.
===============
Other resources, organizations
- Genetic
Alliance
4301 Connecticut Avenue NW
Washington DC 20008-2304
Phone #: 202-966-5557
800 #: 800-336-4363
e-mail: info@geneticalliance.org
Home page: http://www.geneticalliance.org
- March
of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains NY 10605
Phone #: 914-428-7100
800 #: 888-663-4637
e-mail: Askus@marchofdimes.com
Home page: http://www.marchofdimes.com
- NIH/National
Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda MD 20892-3570
Phone #: 301-654-3810
800 #: 800-891-5389
e-mail: nddic@info.niddk.nih.gov
Home page: http://www.niddk.nih.gov
- National
Lymphatic and Venous Diseases Foundation, Inc.
70 Heritage Ave
Portsmouth NH 03801
Phone #: 603-334-8600
800 #: 800-301-2103
e-mail: N/A
Home page: N/A
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Waldmann%20Disease
...............................................
All
About Lymphangiectasia Yahoo Support Group
Support group for parents, patients, children who suffer from all forms
of
lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can
affect the intestinal tract, lungs and other critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
...............................................
External
Links:
..............
Support for Children with
Lymphangiectasia
Little Leakers
http://littleleakers.com/
.........
Caring Bridge
http://www2.caringbridge.org/ca/ironkidmike/
.........
Sick Kids Foundation
http://www.sickkidsfoundation.com/
.........
Little Leaks
http://www.littleleaks.org/
Hypoproteinemia in intestinal
lymphangiectasia;
Contribution of albumin "trapping" in the lymphedematous extremity
T Herskovic, SJ Winawer, R Goldsmith, R Klein and N Zamcheck
Department of Medicine, Yale University School of Medicine, New Haven,
Connecticut
http://pediatrics.aappublications.org/cgi/content/abstract/40/3/345
.........
Intestinal
Lymphangiectasia
http://www.merck.com/mrkshared/mmanual_home2/sec09/ch125/ch125f.jsp
.........
Congenital Pulmonary
Lymphangiectasia
New England Journal of Medicine
http://content.nejm.org/cgi/reprint/349/22/e21.pdf
..............................................
Primary
intestinal and thoracic lymphangiectasia: a response to antiplasmin
therapy
http://pediatrics.aappublications.org/cgi/content/full/109/6/1177
..............................................
Acquired
cutaneous lymphangiectasia with
mesothelial cells reflux in a patient with cirrhotic ascites
Lippincott,
Williams & Wilkins
..............................................
Primary
intestinal lymphangiectasia (Waldmann's
disease).
Orphanet
..............................................
Limb
lymphedema as a first manifestation of
primary intestinal lymphangiectasia (Waldmann's disease)
Masson.fr
..............................................
Videocapsule
endoscopy as a useful tool to
diagnose primary intestinal lymphangiectasia
Elsevier
..............................................
A
primary intestinal lymphangiectasia patient
diagnosed by capsule endoscopy and confirmed at surgery: a case report
World
Journal of Gastroenterology
..............................................
Chronic renal
insufficiency in a boy with cystic renal
lymphangiectasia: morphological findings and long-term follow-up
PubMed
..............................................
Immunohistochemical
studies in a hydroptic fetus with
pulmonary lymphangiectasia and trisomy 21.
PubMed
..............................................
Osteomalacia in a
patient with primary intestinal
lymphangiectasis (Waldmann's disease).
PubMed
..............................................
Renal
Lymphangiectasia
British
Journal of Radiology
----------------------------------
Internal
Lymphedema People Links
Intestinal
Lymphangiectasia
http://www.lymphedemapeople.com/thesite/lymphedema_intestinal_lymphangiectasia.htm
Pulmonary Lymphangiectasia
http://www.lymphedemapeople.com/thesite/lymphedema_pulmonary_lymphangiectasia.htm
Lympangiectasia
http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiectasia.htm
Hennekam
Syndrome
http://www.lymphedemapeople.com/thesite/hennekam_syndrome.htm
Hennekam
Lymphangiectasia Syndrome
http://www.lymphedemapeople.com/thesite/lymphedema_hennekam_lymphangiect.htm
Hennekam
Syndrome
http://www.lymphedemapeople.com/thesite/hennekam_syndrome.htm
Pulmonary
Cystic Lymphangiectasis
http://www.lymphedemapeople.com/thesitepulmonary_cystic_lymphangiectasis
Lymphedema
People Lymphangiectasia Forum
http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=19
Lymphangiectasia
Online Yahoo Support Group
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Lymphangiectasia
Xanga
http://www.xanga.com/lymphangiectasia
Lymphangiectasia
Blog Spot
http://lymphangiectasia.blogspot.com/
=======================
Join
us as we work for lymphedema patients everywehere:
Advocates
for Lymphedema
Dedicated to
be an advocacy group for lymphedema
patients. Working towards education, legal reform, changing insurance
practices,
promoting research, reaching for a cure.
http://health.groups.yahoo.com/group/AdvocatesforLymphedema/
| Subscribe: |
AdvocatesforLymphedema-subscribe@yahoogroups.com |
Pat O'Connor
Lymphedema People / Advocates
for Lymphedema
===========================
For information about
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema\
For Information about
Lymphedema Complications
http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_lymphedema
For Lymphedema Personal
Stories
http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=3
For information about How
to Treat a Lymphedema Wound
http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound
For information about
Lymphedema Treatment
http://www.lymphedemapeople.com/wiki/doku.php?id=treatment
For information about
Exercises for Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema
For information on Infections
Associated with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
For information on Lymphedema
in Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
Lymphedema Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
===========================
Lymphedema People - Support
Groups
-----------------------------------------------
Children
with Lymphedema
The time has come for families, parents, caregivers to have a support
group of
their own. Support group for parents, families and caregivers of
chilren with
lymphedema. Sharing information on coping, diagnosis, treatment and
prognosis.
Sponsored by Lymphedema People.
http://health.groups.yahoo.com/group/childrenwithlymphedema/
Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com
......................
Lipedema
Lipodema Lipoedema
No matter how you spell it, this is another very little understood and
totally
frustrating conditions out there. This will be a support group for
those
suffering with lipedema/lipodema. A place for information, sharing
experiences,
exploring treatment options and coping.
Come join, be a part of the family!
http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515
Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com
......................
MEN
WITH LYMPHEDEMA
If you are a man with
lymphedema; a man with a loved one with lymphedema who you are trying
to help
and understand come join us and discover what it is to be the master
instead of
the sufferer of lymphedema.
http://health.groups.yahoo.com/group/menwithlymphedema/
Subscribe: menwithlymphedema-subscribe@yahoogroups.com
......................
All
About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms
of
lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can
affect the intestinal tract, lungs and other critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
......................
Lymphatic
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is
nothing out
there for other lymphatic disorders. Because we have one of the most
comprehensive information sites on all lymphatic disorders, I thought
perhaps,
it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph
system. Includes lymphangiomas, lymphatic malformations,
telangiectasia,
hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of
information
available through sister site Lymphedema People.
http://health.groups.yahoo.com/group/lymphaticdisorders/
Subscribe: lymphaticdisorders-subscribe@yahoogroups.com
......................
All
About Lymphedema
For our Google fans, we have just created this online support group in
Google
Groups:
Homepage: http://groups-beta.google.com/group/All-About-Lymphedema
Group email: All-About-Lymphedema@googlegroups.com
......................
Lymphedema Friends
http://groups.aol.com/lymphedemafriend
If you an AOL fan and looking for a
support group in AOL
Groups, come and join us there.
===========================
Lymphedema
People New Wiki Pages
Have
you seen our new
“Wiki” pages yet? Listed
below
are just a sample of the more than 140 pages now listed in our Wiki
section. We
are also working on hundred more.
Come
and take a stroll!
Lymphedema
Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema
Arm
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=arm_lymphedema
Leg
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=leg_lymphedema
Acute
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=acute_lymphedema
The
Lymphedema Diet
http://www.lymphedemapeople.com/wiki/doku.php?id=the_lymphedema_diet
Exercises
for Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema
Diuretics
are not for
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema
Lymphedema
People Online
Support Groups
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_people_online_support_groups
Lipedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema
Treatment
http://www.lymphedemapeople.com/wiki/doku.php?id=treatment
Lymphedema
and Pain
Management
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pain_management
Manual
Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)
http://www.lymphedemapeople.com/wiki/doku.php?id=manual_lymphatic_drainage_mld_complex_decongestive_therapy_cdt
Infections
Associated with
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
How
to Treat a Lymphedema
Wound
http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound
Fungal
Infections Associated
with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=fungal_infections_associated_with_lymphedema
Lymphedema
in Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
Lymphoscintigraphy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy
Magnetic
Resonance Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Extraperitoneal
para-aortic lymph node dissection (EPLND)
http://www.lymphedemapeople.com/wiki/doku.php?id=extraperitoneal_para-aortic_lymph_node_dissection_eplnd
Axillary
node biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=axillary_node_biopsy
Sentinel
Node Biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=sentinel_node_biopsy
Small
Needle Biopsy - Fine Needle Aspiration
http://www.lymphedemapeople.com/wiki/doku.php?id=small_needle_biopsy
Magnetic
Resonance Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Lymphedema
Gene FOXC2
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_foxc2
Lymphedema Gene VEGFC
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_vegfc
Lymphedema Gene SOX18
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_sox18
Lymphedema
and
Pregnancy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pregnancy
Home page:
Lymphedema People
http://www.lymphedemapeople.com
Page Updated:
April 18, 2008
