Thanks to everyone for participating in the Washington Post article. It really turned out great. The article went live this evening on the Post's website. Here's the direct link: "Sharing the Pain":http://www.washingtonpost.com/wp-dyn/content/article/2009/02/06/AR2009020603102.html

I read the Washington Post article and learned about this disease that I had no idea existed. I am a scientist, and the descritiptions I found in that article triggered my memory. I worked on an artificial blood development project at Walter Reed Army Institute of Research and did a lot of experiments on blood loss and related hemorrhagic shock. The symptoms I observed there and the symptoms of SCLS share eary similarity. We published a couple of papers and succesfully completed the research phase of the blood subsitute we worked on, and described the importance of the intravascular activation of the complement system in the onset of the disease. I did one additional experiment to clarify why does the take hour(s) for the irreversible phase of the hemorrhagy shock to occure. Why is it that the massive and often fatal capillary leakage happens only after that irreversible phase occurs. The tentative (and unpublished) answer I found was that there is an important pro-enzyme in the blood that is activated together with the complement system and blood clothing during the very early phase of shock, but its half life is only about 5 minutes. The entire pool of this enzyme is depleted in couple of hours, or even as quickly as one hour. After that there is nothing to stop the full development of (hemorrhagic) shock, activation of granulocytes and multiple organ failure leading to and including capillary leakage. The data I have are prety strong. We were able to stop this process by inhibiting the complement system or blocking the C5a anaphylatoxin receptor. I hypothesize that replacing the enzyme would be helpful as well. My question to the group is whether you know a research group that would be interested in working with me to decide whether this theory of mine has any merit? If the SCLS patient's blood has low level or has a mutated form of that enzyme, it could be a perfect explanation of the symtpoms and could open the flodgate for finding therapy. Regards Lajos Baranyi, PhD; lajos.baranyi@lentigen.com Lentigen

Dr. Baranyi This is an excelent insight - that there may be an enzyme deficiency that causes SCLS- A close parallel to SCLS is a disorder called Acquired C1esterase Inhibitor Deficiency. A monoclonal protein causes the deficiency by reacting with the inhibitor of C1 esterase. The complement system is turned on and patients get attacks characterized by swelling of the face and upper airway(angioedema) and abdominal pain associated with bowel edema presumably. Like SCLS it is episodic and the attacks are stereotyped. As in SCLS the monoclonal protein is often present and measurable in the serum. I saw a new patient with this syndrome yesterday - No one had made the diagnosis 8 years after the initial symptoms despite attacks every 6-8 weeks. Studies being done at NIH may point the way to a similar scenario. Phil Greipp

We have published a couple of papers on similar topic, one in Nature Medicine, describing the complement inhibitory effects of human intravenous immunoglobulins. Since it is an FDA approved drug, it may be useful in treating bouts of SCLS. Anecdotal, but a friend of mine saved his father with it when he suffered from rupture of arterosclerotic plaque and had semicrystalline cholesteriemia that caused massive intravasal complement activtion. IvIg treatment stipped the tissue decay and he recovered. IF a systmic complement activation (and depetion of that proenzyme) is involved in SCLS, IvIg could be a life saver. Could you tell me who is workig on the C1 esterase inhibitor deficiency at NIH? Or anyone on SCLS? There are only 27 articles in Medline, and most of those are a decade old. The rest of the papers are describing patients. I am looking for somebody to collaborate with. We at Lentigen are equipped to develope a treatment but before that have to verify that the mechanism I propose is real. regards: Lajos

Lajos Please send an email address - It is great that a company is interested in such a rare disease. And you are right - knowing he mechanism is key. Phil Greipp greipp@mayo.edu

Thank you very much for your suggestions, Dr. Baranyi! I've passed them on to the NIH scientists looking into this matter. Arturo

My email address is: lajos.baranyi@lentigen.com regards: Lajos

Wow, once again good show Arturo. I'm sorry this is a little bit late to repond the the article. I've been dealing with a bypass surgery. I'm amazed at how this forum can foster new insights and cross pollinate ideas. Dee you and your husband have my sympathy. My first attack sounds much like your husbands second one. I've had mostly less serious attacks since. It seems to me that physical or emotional stress is a big trigger for me. This fourm is full of people who are willing to share experience. At least for me it was a wonderfull feeling to know that I'm no tthe only one who has this thing.

Dear Dee: I'm very sorry to hear about your husband's life-threatening second episode. Please insist that his doctors get in touch with Dr. Greipp at the Mayo Clinic ASAP in order to learn how to manage his condition better from now on. Contact me directly at aporzeca@american.edu so I may send you additional life-saving information. Arturo