What is Clarkson Disease?

Clarkson Disease
(Capillary leak syndrome, or idiopathic systemic capillary leak syndrome [SCLS])

~100 Cases reviewed (24 published in 1990; 75 in 2007)

Clarkson syndrome is a rare medical condition where the number and size of the pores in the capillaries are increased which leads to a leakage of fluid from the blood to the interstitial fluid, resulting in dangerously low hypotension, edema and multiple organ failure due to limited perfusion. It presents with recurrent episodes of shock due to leakage of the plasma, which is reflected by accompanying hemo-concentration, hypo-albuminemia and edema.

capillariesbmp

Photo from Bodies Exhibit in Tampa, FL courtesy of Lindsey Williams

http://www.flickr.com/photos/lindseywilliams/

Each Clarkson’s attack has two phases, the first lasting for several days. During the resuscitation phase, physicians focus on controlling the capillary leak and maintaining the patient’s blood pressure. The second stage is typically referred to as the recruitment phase, when tissue reabsorbs albumin and fluids. The goal of this phase is to try to maintain the patient’s blood pressure at a level just high enough to ward off permanent organ damage. Diuretics could be required, and many SCLS deaths occur during this phase. Several treatments are available for SCLS, many of which utilize intravenous (IV) boluses and drips, coupled with frequent lab work. Physicians might prescribe phenylephrine or norepinephrine for hypotension. Hydrocortisone is sometimes administered via IV, and consultations are often scheduled with a doctor specializing in orthopedics because of increased limb pressures.

Thanks to Vonda Sines at www.helium.com.

http://vondasines.blogspot.com/

Vascular Physiology (Capillary: Part 1/6)

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~ by marbleroad on April 24, 2009.

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