title: Acute adrenal failure as the presenting feature of primary antiphospholipid syndrome in a child.
abstract: BACKGROUND
  Antiphospholipid syndrome (APS) is characterized by recurrent arterial and venous thrombosis and detection of antiphospholipid antibodies (aPLs). This syndrome may be associated with connective tissue disorders, or with malignancies, but it may also appear in isolated form (primary APS). We report on a pediatric patient presenting with acute adrenal failure as the first manifestation of primary APS.
  
  
  METHODS
  A previously healthy 11-year-old boy developed fever, abdominal pain, and vomiting. An abdominal computed tomography scan showed nodular lesions in the adrenal glands. He was referred to our Department and a diagnosis of APS and acute adrenal failure was considered, based on positive aPLs (IgG and IgM), elevated ACTH levels and low cortisol levels. Other features were anemia, thrombocytopenia, elevated inflammatory parameters, hypergammaglobulinemia, prolonged partial thromboplastin time, positive antinuclear, anticardiolipin, anti-platelet antibodies, with negative double-stranded DNA antibodies. Lupus anticoagulant and Coomb's tests were positive. MRI revealed a bilateral adrenal hemorrhage. A treatment with intravenous metylprednisolone, followed by oral prednisone and anticoagulant, was started, resulting in a progressive improvement. After 2 months he also showed hyponatremia and elevated renine levels, indicating a mineralcocorticoid deficiency, requiring fludrocortisones therapy.
  
  
  CONCLUSIONS
  The development of acute adrenal failure from bilateral adrenal haemorrhage in the context of APS is a rare but life-threatening event that should be promptly recognized and treated. Moreover, this case emphasizes the importance of the assessment of aPLs in patients with acute adrenal failure in the context of an autoreaction.
authors.affiliation: Department of Pediatrics, Federico II University, Naples, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Improda
authors.forename: Maria
authors.initials: M
authors.lastname: Alessio
authors.forename: Donatella
authors.initials: D
authors.lastname: Capalbo
authors.forename: Giustina
authors.initials: G
authors.lastname: Russo
authors.forename: Ida
authors.initials: I
authors.lastname: D'Acunzo
authors.forename: Loredana
authors.initials: L
authors.lastname: Palamaro
authors.forename: Claudio
authors.initials: C
authors.lastname: Pignata
authors.forename: Mariacarolina
authors.initials: M
authors.lastname: Salerno
mesh_terms: D000208:Acute Disease; D000309:Adrenal Insufficiency / Q000175:diagnosis* / Q000188:drug therapy; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male
chemical_list: D000925:Anticoagulants; D005938:Glucocorticoids; D007166:Immunosuppressive Agents