10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report

In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

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“…SCLS has also been reported in fourteen children during the past six years. 5, 17, 21, 25, 28, 35, 50, 52 …”

Section: Incidence Of Idiopathic Sclsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

132

167

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“…Acute treatment depends on aggressive fluid replacement and crystalloid solutions [ 16 , 17 ]. Corticosteroid therapy against cytokine-mediated endothelial damage along with plasmapheresis and intravenous immunoglobulin has proved to be successful in the acute phase [ 5 , 18 ]. Infliximab (antitumor necrosis factor) and bevacizumab (anti-VEGF) had been used in the treatment of ISCLS attacks; however, their effects are not clear yet [ 19 ].…”

Section: Discussionmentioning

confidence: 99%

A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature

Ata

Özen

Gökşen

et al. 2020

Case Reports in Immunology

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Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeability followed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy was admitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, and oral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchy edema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L, hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourth day, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immunoglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased on the fifth day, and the patient was deemed clinically well. There was no compartment syndrome, rhabdomyolysis, or pulmonary edema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) and enterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serum glucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive. However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit, 22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis, there was no ISCLS attack. Conclusion. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare in childhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of the disease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to the literature since there have been no pediatric diabetic patients with ISCLS reported.

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“…Although immune and inflammatory mediators are hypothesized to play a key role in SCLS although the exact mechanism of the capillary leak syndrome is still not elucidated, cytokines, and inflammatory mediators and oxidation injury-induced apoptosis are plausible postulated or likely mechanism for endothelial injury. [ 12 13 14 ]…”

Section: Discussionmentioning

confidence: 99%