Re: e/ abstracts on bleeding, platelets, DDAVP and EDS

[Guest guest]

Hi Joyce My name is , My daughter Petra was tested for VEDS. Because of

outside symptoms and easy bleeding

through this whole process they discovered that she has prolonged bleeding a

low factor 12 and platelet dysfunction.

Her EDS test came back normal they seem to be stumped and want us to relax

Petra has had two episodes of itp.

Were all the platelets in her blood stream depleted. Two transfusions! I

was reading your mail to e Petra has nose bleeds all the time and its

not just your regular nose bleeds the blood is bright red and its like it

jumps out of her nose,

like it under pressure. Any way if you could get back to me about your

thoughts on this that would be great.

I asked the Hemo if he still thought we could be dealing with EDs and he

said no after hearing the results of her biopsy.

Three other DR's believe that she EDS And bleeding tendencies Iam so tired

of being confused. TAnya

e/ abstracts on bleeding, platelets, DDAVP and EDS

> Hi e:

> I am sorry your son has had to go through this with nosebleeds. Yep

> -that arterial one I had made the bedroom look like a murder scene. It

> is certainly frightening to have these episodes. Thankfully - no bleeds

> for a good two years. I had 3 surgeries and now that the airflow is

> better through my nose, I don't seem to be having the bleeds anymore.

> The DDAVP is available as a nose spray or injection. It comes in

> pill form but this would not be good if someone was actively bleeding as

> it would take too long to work. This for,m is used for bedwetting and

> other things. It causes the blood vessels to release clotting factors

> and will help slow or stop bleeding. I have it before any procedures

> which are expected to cause bleeding.

> Here are some of the abstracts below:

>

> Br J Dermatol 1991 Aug;125(2):155-63

>

> Platelet and coagulation studies in Ehlers-Danlos syndrome.

> Anstey A, Mayne K, Winter M, Van de Pette J, Pope FM.

> Department of Dermatology, Wycombe General Hospital, U.K.

>

> Fifty-one patients with Ehlers-Danlos syndrome were investigated for

> abnormalities of platelets and coagulation. Thirty-eight were examined

> prospectively and 13 retrospectively. A bleeding history was taken from

> all patients; only four (8%) gave no history of a bruising or bleeding

> tendency. Nine patients (18%) had significant haemostatic abnormalities

> of whom four (8%) had a platelet release defect, three (6%) had a factor

> XI deficiency and two (4%) had a factor XIII deficiency. Additionally 16

> patients (31%) had mild abnormalities of uncertain significance of whom

> four (8%) had prolonged bleeding times (three in association with

> platelet aggregation abnormalities), 13 (26%) had platelet aggregation

> abnormalities and two had a positive Hess test. Twenty-four patients

> (47%) had normal tests for haemostasis of whom 20 (39%) had a bleeding

> diathesis and four (8%) had no such tendency. Results were analysed

> according to the type of Ehlers-Danlos syndrome, but there was no

> pattern to the abnormalities. The high frequency of a bleeding tendency

> in Ehlers-Danlos patients with normal tests for haemostasis (83%)

> supports the conventional explanation for this clinical feature, that

> defects in the structural integrity of skin and blood vessels lead to

> easy bruising.

>

>

> JAMA 1980 Jul 11;244(2):144-7 Related Articles, OMIM, Books, LinkOut

>

> A new form of Ehlers-Danlos syndrome. Fibronectin corrects defective

> platelet function.

> Arneson MA, Hammerschmidt DE, Furcht LT, King RA.

>

> In a kindred with a mild, recessively inherited variant of the

> Ehlers-Danlos syndrome (EDS), a platelet aggregation defect segregated

> concordantly with skin and joint abnormalities. This defect was

> partially corrected in vitro by addition of normal plasma or

> cryoprecipitate. The plasma of the patients with EDS failed to support

> the aggregation of normal gel-filtered platelets in response to

> collagen; this defect was completely corrected by the addition of normal

> human fibronectin. Since fibronectin is an important adhesive

> glycoprotein in connective tissue and is required for normal platelet

> interactions with collagen, we propose that both platelet malfunction

> and joint hypermobility in this kindred are likely explained by a

> defective fibronectin.

>

> : J Pediatr Hematol Oncol 1997 Mar-Apr;19(2):156-8

>

> DDAVP therapy controls bleeding in Ehlers-Danlos syndrome.

> Stine KC, Becton DL.

> Department of Pediatrics, University of Arkansas for Medical Sciences,

> Little Rock 72202, USA.

>

> PURPOSE: Patients with Ehlers-Danlos syndrome (EDS), especially types

> IV, VI, and VIII, are at increased risk of bleeding, and most do not

> have specific hemostatic deficiencies that would be amenable to

> replacement therapy. We have investigated the ability of DDAVP

> (desmopressin acetate) to control bleeding in EDS. PATIENTS AND METHODS:

> Two children with EDS, types VIII and VI, presented with hemorrhagic

> symptoms and scheduled surgical procedures. Ivy bleeding times (BTs)

> were measured before and after intravenous (i.v.) DDAVP challenge, and

> i.v. DDAVP was used prophylactically for their procedures. Laboratory

> testing was performed to rule out other hemostatic disorders. RESULTS:

> Both patients had prolonged BTs that corrected following i.v. DDAVP

> therapy; all other laboratory values were normal. Both patients had

> excellent clinical hemostasis with surgery, and one has continued to use

> intranasal DDAVP to control epistaxis and gingival bleeding.

> CONCLUSIONS: The bleeding time in both patients was corrected with

> DDAVP, and the patients did not have any postoperative bleeding. DDAVP

> should be considered in other patients who have EDS with bleeding

> tendencies.

>

>

> Haematologica 1999 Oct;84(10):891-6 Related Articles, Books, LinkOut

>

> In vitro and in vivo effects of desmopressin on platelet function.

> Balduini CL, Noris P, Belletti S, Spedini P, Gamba G.

> Istituto di Medicina Interna e Oncologia Medica, IRCCS San Matteo, p. le

> Golgi, 27100 Pavia, Italy. c.balduini@...

>

> BACKGROUND AND OBJECTIVE: Desmopressin (DDAVP) may shorten bleeding time

> in patients with disorders of platelet function, but its mechanism of

> action in these conditions is still a matter of debate. In particular,

> contrasting results have been obtained concerning the ability of DDAVP

> to interact with platelets and to activate them directly. To gain

> further information on the DDAVP-platelet interaction, we studied the in

> vitro and ex vivo effects of DDAVP on platelet function. DESIGN AND

> METHODS: Platelet responses to DDAVP both as a single agent and in

> conjunction with agonists of platelet activation were investigated. For

> in vitro experiments platelets were obtained from healthy adult

> volunteers, while the ex vivo effects of DDAVP were studied in 12

> patients with a bleeding disorder receiving a test dose of this drug.

> RESULTS: DDAVP in vitro did not induce either platelet aggregation or

> surface expression of the activation-dependent antigens; it did,

> however, greatly inhibit platelet aggregation response to vasopressin

> (AVP) and increased the maximal extent of platelet aggregation induced

> by collagen and ADP. DDAVP infusion did not promote the expression of

> activation antigens, but significantly enhanced ex vivo platelet

> aggregation stimulated by ADP and collagen. This priming effect was

> observed in patients with von Willebrand's disease, hemophilia A,

> May-Hegglin anomaly, gray platelet syndrome and Ehlers-Danlos syndrome.

> In all these patients bleeding time was shortened by DDAVP infusion. In

> contrast, neither platelet aggregation nor bleeding time was modified in

> two subjects with Glanzmann's thrombasthenia. INTERPRETATION AND

> CONCLUSIONS: Our in vitro experiments indicate that DDAVP interacts

> directly with platelets and facilitates their activation via other

> agonists. In vivo results suggest that this effect occurs and is

> clinically relevant in patients with platelet dysfunction responding to

> DDAVP with a shortening of bleeding time.

>

>

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