(SCTp/HANE/Keloids)

From: Terrence.Jones@ncal.kaiperm.org
Tue Dec 9 16:16:34 1997


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Dr. Bui, heard some discussion took place at British Society for Immunology -------------------------------------------------------------------------- Annual Congress (Brighton) this past week on stem cell transplantation!? The -------------------------------------------------------------------------- suggestion was that critical timing (12 wks gestation) have resulted in "promising results". Any comments on what Dr. (Rhodri) Jones was 'promising'?

Dr. Lovsin, have no experience with HANE in preg. - (Type 1 (quantitative deficit) or Type 2 (qualitative deficit?). C1 inhibtor can be obtained as C1 INATTIVATORE UMANO from Immuno (Italy) - think this is a bit closer to You than Behring (Germany/Switz). ("Don' know much about Geography"!) The product (from Human plasma) is 'vapor heated' to inactivate Hep. and HIV viruses. By plasma rxn I'm guessing you might be referring to TRALI (transfusion- related acute lung injury) secondary to leukoagglutinating antibodies in donor plasma (Br J Haem (6/96), 93:707-13)? Might check with Mats Bergstrom for availability of TXA, as He has commented on its recommended use in the setting of abruption by Dr. Birgir Astedt (note the little circle over the A) from Univ of Lund. See Mats letter in the archives, from 1/17/96, and note his admonishion re: thrombosis, as APC resistance HAS been reported with HANE. Malcolm's post would suggest there will be little trouble obtaining it, if I were guessing the closest availability, I'd pick "Tranex" (Malesci) or Amcacid (Glaxo) both, also, from Italy. Another nearby resource might be Exacyl, from Bournonville, Belgium. Tho somewhat less potent in its effect, an alternative, if there's difficulty obtaining TXA, is EACA (epsilon-amino- caproic acid) --Mayo Clin Proc (12/96), 11:75-8). Watch for SLE-like activity as a consequence of diminished C4. And consider eval of newborn (cord blood) to protect against any unusually early manifestations (Pediatr Res (2/94), 35:184-7). As Malcolm mentioned, Danazol has been reported for maternal prophylaxis at delivery (His ref -- Boulos Br J O/G 12/94).

Picked up this request from molecular genetics list. For those with affected Pts. this may be a step in defining the condition, and later, treatment. tj. -------------------------------------------------------------------------- Reply-To: Ernst Reichenberger <ernstrei@warren.med.harvard.edu> -------------------------------------------------------------------------- Subject: KELOIDS --------------------------------------------------------------------------

Dear Colleagues,

We are currently working on the positional cloning of genes responsible for heritable keloid formation. For our linkage analysis studies we are looking for additional families affected with true keloids. I would greatly appreciate your collaboration if yo u can contribute such a family or if you have patients with familial keloids or know of somebody who has. This study has IRB approval.

Ernst Reichenberger, Ph.D. Harvard Medical School Department of Cell Biology 240 Longwood Ave. Boston MA 02115

Tel. (617) 432-1762 FAX (617) 432-0638

ernstrei@warren.med.harvard.edu

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