Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant [ncbi.nlm.nih.gov]

• Abdominal Pain

  Symptoms Attacks of systemic capillary leak syndrome are often preceded by one to two days of one or more nonspecific symptoms that may include: Irritability Fatigue Abdominal pain Nausea Muscle aches Increased thirst Sudden increase in body weight As [mayoclinic.org]

  The patient presented with acute symptoms of abdominal pain, vomiting, and syncope in the hour preceding the shock. [ncbi.nlm.nih.gov]

  These reduced the intensity of his crises, with only abdominal pain, moderate hypotension and weight gain. [ndt.oxfordjournals.org]

• Nausea

  Although oxaliplatin is generally well tolerated, certain side effects such as nausea, vomiting, and peripheral neuropathy are common. [ncbi.nlm.nih.gov]

  The episodes were preceded by symptoms such as a runny nose, epigastric discomfort with nausea, vertigo, orthostatism and sometimes light fever. [ntvg.nl]

  Symptoms Attacks of systemic capillary leak syndrome are often preceded by one to two days of one or more nonspecific symptoms that may include: Irritability Fatigue Abdominal pain Nausea Muscle aches Increased thirst Sudden increase in body weight As [mayoclinic.org]

  When a patient presents with nausea, report 780.02. When a patient presents with lightheadedness, report 780.4. Report each of these codes in addition to 448.9. [hcpro.com]

• Hypotension

  The serum of the patient was stored soon after the onset of hypotensive crisis and analyzed to profile possible mediators responsible for the capillary leak. [ncbi.nlm.nih.gov]

  The leak phase follows with oliguria, arterial hypotension and rapid development of edema of the face or the upper limbs but sparing the lungs. Hypotension may lead to hypovolemic shock with preserved consciousness. [orpha.net]

• Suggestibility

  Although the implication of vascular endothelial growth factor, angiopoietin-2, and C-X-C motif chemokine 10 has been suggested, the pathogenesis of systemic capillary leak syndrome remains unclear. [ncbi.nlm.nih.gov]

  Limited ultrastructural studies of skeletal muscle endothelial cells from one patients with SCLS suggested apoptosis without intercellular gap formation. 34 In contrast to these findings and other prior studies suggestive of endothelial apoptosis in SCLS [dx.doi.org]

• Oliguria

  Attacks vary in severity and may be lethal.A 49-year-old previously healthy man was admitted to hospital for hypovolemic shock, anasarca with pleuropericardial effusion, muscle fatigue, and oliguria occurring after a flu-like syndrome. [ncbi.nlm.nih.gov]

  Twelve hours after ED presentation, hypoproteinemia (3.4 g/dL), hypoalbuminemia (1.8 g/dL), and elevated CPK (1,502 IU/L) and Hb (23.4 g/dL) were observed in addition to oliguria. [ceemjournal.org]

  The leak phase follows with oliguria, arterial hypotension and rapid development of edema of the face or the upper limbs but sparing the lungs. Hypotension may lead to hypovolemic shock with preserved consciousness. [orpha.net]

Infectious workup failed to reveal a source for presumed sepsis. [pediatrics.aappublications.org]

Immediate treatment with corticosteroids may be life-saving. [ncbi.nlm.nih.gov]

Treatment Treatment is directed at prevention of attacks using agents aimed at decreasing capillary leakage and aimed at interfering with hormone like cytokines that induce the leakage. [rarediseases.org]

Prognosis Despite progress in diagnosis and management that has prolonged the survival of patients, prognosis remains poor as mortality reaches 30-40% after 10 years. [orpha.net]

Its treatment has remained largely supportive and the prognosis is generally poor. Awareness of SCLS is necessary for improvement of the outcome. [ncbi.nlm.nih.gov]

Its treatment has remained largely supportive and the prognosis is generally poor. Awareness of SCLS is necessary for improvement of the outcome. (Internal Medicine 41: 211-215, 2002) [doi.org]

Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown. [ncbi.nlm.nih.gov]

Etiology SCLS is caused by capillary hyperpermeability of unknown etiology. Several physiopathological hypotheses have been considered such as an inflammation-mediated mechanism or a damage of the capillary endothelium by the monoclonal gammopathy. [orpha.net]

Summary Epidemiology SCLS has been reported in less than 150 cases since its first description in 1960. Clinical description Mean age of onset is 45 and pediatric or geriatric cases are rare. [orpha.net]

( 28238385 ) AlyeA9il C....GA1ney S. 2017 30 Epidemiology, risk factors, and prognosis of capillary leak syndrome in pediatric recipients of stem cell transplants: a retrospective single-center cohort study. ( 27734564 ) Lucchini G....Bader P. 2016 31 [malacards.org]

Epidemiology and Pathophysiology The incidence of ES is very variable depending on the criteria used to define ES. [imedpub.com]

Epidemiology One hundred cases of SCLS were reported in the world literature between 1960 and 2006, according to recent reviews (4–6). [doi.org]

There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis. [ncbi.nlm.nih.gov]

Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy. [ncbi.nlm.nih.gov]

Treatment during episodes aims to stabilize symptoms and prevent severe complications. The use of intravenous fluids must be monitored carefully. Monthly infusions of intravenous immunoglobulin (IVIG) can help prevent future episodes. [mayoclinic.org]