Updated

Updated · abcnews.com · Jul 23

Colorado Father Warns After 5-Year-Old Dies From Rare Syndrome With Fewer Than 500 Reported Cases

Updated

Updated · abcnews.com · Jul 23

Colorado Father Warns After 5-Year-Old Dies From Rare Syndrome With Fewer Than 500 Reported Cases

Q: What is the unknown trigger that causes a healthy body’s blood vessels to suddenly leak?

There is no known single trigger. In idiopathic systemic capillary leak syndrome, or Clarkson disease, doctors still do not know what makes a previously healthy person’s capillaries suddenly become abnormally “leaky.” What is known is the mechanism, not the cause. During an episode, the lining of the small blood vessels becomes hyperpermeable, allowing protein-rich plasma to escape into surrounding tissues. That can cause a sharp drop in blood pressure, swelling, thickened blood, shock, and organ failure. Researchers believe immune-related injury to the endothelium and inflammatory signaling are involved, but the exact event that sets it off remains unclear. In some patients, episodes appear to follow a viral-like illness or inflammation, which is one reason the condition is often mistaken for infection. But in idiopathic cases like the one described in Justin Vu’s death, no definitive trigger can be identified. That is what “idiopathic” means: the cause is unknown. Doctors do distinguish this from secondary capillary leak syndrome, where leakage can occur in connection with sepsis, certain infections such as Hantavirus, autoimmune disease, or some cancer treatments. But for primary or idiopathic SCLS, the central medical answer remains that the trigger is still unknown.

1 articles · Updated · abcnews.com · Jul 23

Justin Vu, 5, died on July 8, five days after he was first hospitalized during a family vacation in Oregon, prompting his father to speak publicly about systemic capillary leak syndrome.
Doctors initially suspected appendicitis or an infection after Justin developed a stomachache and vomiting on the trip’s fourth night, then transferred him to a Portland children’s hospital and treated him with four antimicrobial drugs.
Systemic capillary leak syndrome, also called Clarkson’s disease, causes fluid to leak from capillaries and can trigger a rapid drop in blood pressure; its early symptoms can resemble routine illness.
Fewer than 500 cases have been reported worldwide since the 1960s, according to the National Organization for Rare Disorders, and the condition is especially rare in children.

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abcnews.com2h ago

Father Viet Vu Warns After 5-Year-Old Son Justin Dies from Rare Systemic Capillary Leak Syndrome on Vacation

What is the unknown trigger that causes a healthy body’s blood vessels to suddenly leak?

How can emergency rooms detect a one-in-a-million disease that mimics a common stomach bug?

Could AI-powered diagnostics help doctors spot ultra-rare diseases before they turn fatal?

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1 total

abcnews.com2h ago

Father Viet Vu Warns After 5-Year-Old Son Justin Dies from Rare Systemic Capillary Leak Syndrome on Vacation

Colorado Father Warns After 5-Year-Old Dies From Rare Syndrome With Fewer Than 500 Reported Cases

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