I’m happy to report that Dr. Kirk Druey and his team from the U.S. National Institutes of Health (NIH), together with physicians in Australia, have had their paper "SCLS in Children" just now published in _Pediatrics_, the official journal of the American Academy of Pediatrics.
This article will surely disseminate state-of-the-art knowledge about SCLS among pediatricians around the world, so they may be able to diagnose and treat the syndrome in children as quickly and as best as possible.
I am grateful to the parents of SCLS children from this Community who authorized their physicians to collaborate with Dr. Druey and team in gathering the medical histories and blood samples necessary for this research effort to go forth and bear fruit.
The article was posted on February 23rd as an "eFirst" contribution, published online in advance of appearing in a standard, paper issue of the journal. It is available online for journal subscribers, or others with access (e.g., through a university or public library subscription), to the journal’s restricted website, http://pediatrics.aappublications.org/content/early/recent.
Those Community members who lack access and wish to receive this pre-publication version of the article may request it from me at _aporzeca@american.edu_ Please include your RareShare username in the email to me.
The article's abstract reads as follows:
"Adult subjects with SCLS present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema.
We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from the United States, Australia, Canada, and Italy. Serum cytokines from SCLS subjects and a group of 10 healthy children were analyzed.
Children with SCLS (aged 5-11 years old) presented with at least 1 acute, severe episode of hypotension, hypoalbuminemia, and hemoconcentration in the absence of underlying causes for these abnormalities.
In contrast to what is observed in adult SCLS, identifiable infectious triggers precipitated most episodes in these children, and none of them had a monoclonal gammopathy (MGUS).
We found elevated levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α in baseline SCLS sera compared with the control group.
All patients are alive and well on prophylactic therapy, with 4 patients receiving intravenous or subcutaneous immunoglobulins at regular intervals.
The clinical manifestations of pediatric and adult SCLS are similar, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy.
Prophylactic medication, including high-dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children."