Literature DB >> 21321028

Pericarditis as the presenting feature of adrenoleukodystrophy.

Alysha J Taxter1, Melena D Bellin, Bryce A Binstadt.   

Abstract

X-linked adrenoleukodystrophy is characterized by elevated levels of very long chain fatty acids in the serum, brain, and adrenal glands that can lead to neurodevelopmental impairment and decreased adrenal function. We report here the case of a pediatric patient with pericarditis who was found to have adrenoleukodystrophy. More common causes of pericarditis (such as infectious, autoimmune, and metabolic) were excluded. On the basis of the examination finding of cutaneous hyperpigmentation, hypocortisolism was discovered. Further evaluation revealed elevated serum levels of very long chain fatty acids and a partial deletion of the ABCD1 gene, consistent with the diagnosis of X-linked adrenoleukodystrophy. Two of the index patient's brothers were subsequently found to have the same disease. Although pericarditis has been reported previously in association with autoimmune diseases that affect the adrenal glands, this is the first reported case (to our knowledge) of pericarditis in association with hypocortisolism from a nonautoimmune cause. Therefore, we suggest that hypocortisolism itself may lead to pericarditis in some patients.

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Year:  2011        PMID: 21321028     DOI: 10.1542/peds.2010-2232

Source DB:  PubMed          Journal:  Pediatrics        ISSN: 0031-4005            Impact factor:   7.124


  3 in total

1.  Prenatal diagnosis of X-linked adrenoleukodystrophy associated with isolated pericardial effusion.

Authors:  Giovanna Traficante; Roberto Biagiotti; Elena Andreucci; Mariarosaria Di Tommaso; Aldesia Provenzano; Ettore Cariati; Sabrina Giglio
Journal:  Clin Case Rep       Date:  2015-06-11

2.  Venoarterial extracorporeal membrane oxygenation as bridge to effective treatment in a 19-year-old woman with acute adrenal crisis: a case report.

Authors:  Enzo Lüsebrink; Kathrin Krieg; Steffen Massberg; Martin Orban
Journal:  Eur Heart J Case Rep       Date:  2021-02-20

3.  Acute Pericarditis as a Presentation of Adrenal Insufficiency.

Authors:  Sukesh Manthri; Sindhura Bandaru; Abdisamad Ibrahim; Chaitanya K Mamillapalli
Journal:  Cureus       Date:  2018-04-13
  3 in total

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