Re: Article - EDS and Osteoporosis

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Jill,

I would like a copy of the newer article...please and thank you, dear

list leader! You're the BEST!

Love Lana

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Me too Jill,

I'd like the newer article too! How ya feeling?

Sincerely, S.

Article - EDS and Osteoporosis

> I have a more current article then this for anyone who wants it....but

this

> is still useful. If you want the more current one, shout!

>

>

> EHLERS-DANLOS SYNDROME AND OSTEOPOROSIS

>

> Dr. Atul A Deodhar. MD. MRCP

> Division of Arthritis and Rheumatic Diseases (L329A)

> The Oregon Health Sciences University

>

>

> Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue

> that shows extreme variations in its clinical presentations. The most

common

> symptoms are loose skin and hypermobility of the joints. The serious

> manifestation of dilatation of the blood vessels is fortunately rare. Bone

> is an important member of the connective tissue group, but very little is

> known about bone involvement in EDS. Osteoporosis is the most common

disease

> that affects bones. This article argues that people with EDS are likely to

> be genetically susceptible to osteoporosis and hence need to learn more

> about this very important health issue. It also suggests preventive

> strategies by modifying the important environmental factors that are known

> to accelerate bone loss and development of osteoporosis.

>

> Osteoporosis can be defined as a reduction in bone mass that leads to

> increased risk of fractures. It is an enormous public health problem for

> most societies in terms of disability, pain and socioeconomic costs. With

> the aging of these communities these costs can only increase. We have only

> recently become aware of the magnitude of the problem. In the United

States

> for a white woman above the age of 50, the lifetime risk of any hip,

> vertebral or distal forearm fracture is 40 percent and for a man it is13

> percent. In England and Wales, approximately 50,000 hip fractures occur

> every year. As a result, half of these patients cannot walk independently

> and 30 percent of them become totally dependent. A fifth of 70 year old

> women have a vertebral fracture causing considerable disability, pain and

> loss of quality of life. The problem of forearm fractures is

underestimated

> even though it results in prolonged pain and impaired function. The

overall

> hospital cost of osteoporotic fractures to the National Health Service in

UK

> is estimated to be £615 million per year and is rising.

>

> Bone is a living tissue, continually remodeling and renewing itself

> throughout life, to respond to physical stresses. Bone grows from birth

> through puberty to reach its maximum around the age of thirty-five. This

> phase in life when we have the best possible bone strength is termed the

> peak bone mass. Bone mass then begins to decline due to the imbalance of

> formation and resorption. This loss is particularly rapid in women in the

> first 10 years after menopause because of the lack of female sex hormone,

> estrogen. Osteoporosis therefore is the end result of an inadequate

> attainment of peak bone mass, or a subsequent rapid loss, or a combination

> of both. Evidence suggests that there is a strong genetic influence on

peak

> bone mass. The level of peak bone mass is also modified by environmental

> factors like hormones, nutrition and exercise.

>

> The environmental influences on bone are well known. For instance, a life

> long high intake of calcium is associated with greater bone mass; there is

a

> positive effect of physical activity on bone observed in athletic

> individuals and there is a loss of bone mass following prolonged

immobility.

> The use of certain medications like heparin and corticosteroids are also

> known to cause rapid bone loss. The other very important factors are the

sex

> hormones; estrogen and testosterone. Lack of these sex hormones for

instance

> in delayed puberty, prolonged absence of menstrual periods or early

> menopause can influence bone mass adversely.

>

> Let us now consider the genetic influence on bone strength. A number of

> family and twin studies have demonstrated that genetic factors play a very

> important role in the development of osteoporosis by influencing peak bone

> mass. Identical twins, who share 100% of their genes, have remarkably

> similar bone density. In family studies, daughters of women with prior

> vertebral fractures have been shown to have low bone density. Collagen is

> the main protein constituent of bone. Genetic defects in the formation of

> collagen can cause osteoporosis. Osteogenesis imperfecta is a good example

> of this.

>

> Osteogenesis imperfecta (01) is a genetic connective tissue disease,

> characterized by increased fragility of bones. Affected persons may have

> multiple fractures with minimal trauma. Some patients with Ol have a very

> severe form of osteoporosis while mild cases with Ol can present late in

> life, and can be mistaken for 'post-menopausal osteoporosis'. Ol has been

> associated with defects in the structure or synthesis of type I collagen.

> Type I collagen not only plays a crucial scaffolding role in bone, it is

> also an important protein in the skin. In some types of EDS, the defects

at

> the molecular level have been identified and include similar abnormalities

> in the synthesis and processing of types I and 3 collagens. EDS and Ol are

> therefore closely related and are known to co-exist. Theoretically we can

> postulate that some forms of EDS with type 1 collagen defects could have

> osteoporosis.

>

> Clinical evidence to suggest that patients with EDS also have osteoporosis

> is limited. The scientific literature contains only two small bone

density

> studies in patients with EDS. Coelho and colleagues from Portugal assessed

> bone density in 4 patients with type I EDS. They were 3 males and 1 female

> with ages ranging from 16 to 25 years. None of these patients had any

other

> significant risk factors for low bone mass and none had a clinical or

> radiological evidence of fractures. All patients had low bone density in

the

> lumbar spine but not in the hip. We measured bone density in five female

and

> two male patients with EDS in Cornwall. Six had type 2 and one patient had

> type 3 EDS. They were between the ages of 16 to 70 and were referred by

> their family physicians for assessment of bone density. They did not have

> any predisposing factor for osteoporosis. We found that four out of seven

> patients had significantly reduced bone mass both in the hip and in the

> lumbar spine, two had significantly reduced bone mass n the hip only and

the

> 16 year old had moderately reduced bone density in the spine. They were

thus

> at increased risk of developing fractures. We hypothesize that the

> abnormality of the collagen framework in EDS may lead to faulty deposition

> of calcium in the bones', with resultant reduced bone mass.

>

> The presence of osteoporosis can be suggested by history and physical

> examination but the only way to diagnose osteoporosis with certainty is by

a

> bone density measurement. This test usually takes 10 to 15 minutes to

> perform, is painless and uses very small quantities of x-rays. Generally a

> repeat measurement is carried out a year or so later to check for the rate

> of loss of bone mass. If you are worried about the possibility of

> osteoporosis, speak to your family physician and ask for a bone density

> measurement test. If you are found to be osteoporotic. What can be done?

>

> As we can not change our genetic background, management of osteoporosis in

> the setting of Ehlers-Danlos syndrome will depend on awareness of the

> problem and modifying the environmental factors. As a general measure,

every

> person with EDS should have adequate calcium intake (I to 1.5 grams per

day)

> in their diet. A pint of skimmed milk or equivalent amount of dairy

products

> per day will provide one gram of calcium. If for some reason, the person

is

> unable to tolerate dairy products the diet should be supplemented with

> tablets of calcium. For those above the age of 65, it may be worth

> considering vitamin D supplements (800 international units per day) too.

> Increased physical activity in the form of regular weight bearing

exercises

> like brisk walking, have beneficial effects on bone mass. The majority of

> osteoporotic fractures are associated with falls. The risk factors for

> falling are numerous, but are mainly related to advanced age or ill

health.

> A general improvement in fitness has the greatest effect in reducing the

> risk of falling.

>

> There are no studies in patients with EDS looking at the benefits of

> different medication in treatment of osteoporosis. However, till such

> studies become available, there is no reason to deny patients with EDS and

> osteoporosis the benefits of currently available therapies. Women with EDS

> at menopause should consider the use of hormone replacement therapy (H RT)

> for at least ten years unless there is an absolute contraindication. HRT

> has been shown to prevent loss of bone and halve the risk of myocardial

> infarction and stroke. For these reasons, it may be worth considering HRT

in

> every woman with EDS at menopause. In the last few years, many other

> treatments have become available to treat pre-existing osteoporosis.

> Intra-nasal calcitonin, oral slow release fluoride and newer

bisphosphonates

> such as alendronate have been shown to be effective in halting the bone

loss

> and in some cases reducing fracture risks. These treatments can be very

> potent and need to be used under careful monitoring by a specialist. Wit

the

> present resurgence of interest in osteoporosis; new hormonal and

> non-hormonal agents are undergoing clinical trials. The next decade

promises

> to be very exciting in the development of novel therapies to treat

> osteoporosis.

>

> SUGGESTED READING:

>

> Ehlers Danlos syndrome and osteoporosis (Letter) A.A. Deodhar, A. D.

Woolf.

> ls of the Rheumatic Diseases. 53(12):841-2. I994 Dec.

>

> Osteoporosis and Ehlers Danlos Syndrome (Letter) P. C. CoeIho, R. A.

Santos,

> J. A. Gomes ls of the Rheumatic Diseases. 53(3):212-3. I994 Mar.

>

> Collagens and their abnormalities in a wide spectrum of diseases. (Review)

> I. Kivirikko

> ls of Medicine. 25(2):l 13-26, 1993 Apr.

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> To learn more about EDS, visit our website: http://www.ceda.ca

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