Multi-organ failure as first clinical sign of macrophage activation syndrome in childhood Still's disease

Anales de pediatría : publicación oficial de la Asociación Española de Pediatría (A.E.P.)
M López-SánchezE Peiro-Callizo

Abstract

Macrophage activation syndrome is a form of secondary haemophagocytic lymphohistiocytosis seen in the context of rheumatic diseases. It is seen most frequently in association with systemic onset juvenile arthritis or childhood Still's disease. Hemophagocytosis is part of a sepsis-like clinical syndrome caused by hypercytokinemia due to a highly stimulated but ineffective immune response. Coagulopathy and hemorrhages, decreased white cell count, elevated levels of aspartate aminotransferase, fever, rash, hepatosplenomegaly and central nervous system dysfunction are some of diagnostic criteria of macrophage activation syndrome, but it is very difficult to diagnose due to the lack of specific clinical signs. We report a 8-year-old child who was admitted to the ICU with lethargy, fever, acute respiratory failure, coagulopathy, metabolic acidosis and multiorgan failure. Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU.

References

Oct 5, 2001·Expert Opinion on Pharmacotherapy·S ImashukuS Hibi
Oct 23, 2001·Archives of Disease in Childhood·S SawhneyK J Murray
Jan 23, 2003·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Debra L PalazziSheldon L Kaplan
Jun 19, 2003·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·E NahumT Schonfeld
Nov 8, 2006·Current Opinion in Allergy and Clinical Immunology·Alexandra H Filipovich
Jun 26, 2007·Blood Reviews·Gritta E Janka
Sep 1, 2007·Current Opinion in Rheumatology·Alison Kelly, Athimalaipet V Ramanan
Mar 28, 2009·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Leticia Castillo, Joseph Carcillo
Oct 31, 2009·Arthritis and Rheumatism·Alessandro ParodiUNKNOWN Lupus Working Group of the Paediatric Rheumatology European Society

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.