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Adrenal xanthomatosis

MedGen UID:
1841796
•Concept ID:
C5826533
•
Disease or Syndrome
Synonym: Adrenal xanthomas
 
HPO: HP:0034622

Definition

Lipid deposits localized within the adrenal gland. Xanthomas are benign but are often an important visible sign of systemic conditions such as familial hypercholesterolemia. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenal xanthomatosis

Professional guidelines

PubMed

Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Sitosterolemia: diagnosis, investigation, and management.
Escolà-Gil JC, Quesada H, Julve J, Martín-Campos JM, Cedó L, Blanco-Vaca F
Curr Atheroscler Rep 2014 Jul;16(7):424. doi: 10.1007/s11883-014-0424-2. PMID: 24821603

Recent clinical studies

Etiology

Adult Orbital and Adnexal Xanthogranulomatous Disease.
Davies MJ, Whitehead K, Quagliotto G, Wood D, Patheja RS, Sullivan TJ
Asia Pac J Ophthalmol (Phila) 2017 Sep-Oct;6(5):435-443. Epub 2017 Aug 22 doi: 10.22608/APO.2017246. PMID: 28829109
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Necrobiotic xanthogranuloma as an unusual cause of refractive chronic bilateral leg ulceration.
Ghiasi N, Alavi A, Coutts PM, Ghazarian D, Sibbald RG
Int J Low Extrem Wounds 2012 Dec;11(4):293-5. doi: 10.1177/1534734612465434. PMID: 23222162
Prenatal monitoring for Wolman's disease in a pregnancy at risk: first case in the Federal Republic of Germany.
Christomanou H, Cáp C
Hum Genet 1981;57(4):440-1. doi: 10.1007/BF00281702. PMID: 7286988

Diagnosis

Xanthogranulomatous adrenalitis masquerading as a functioning adrenocortical malignancy: a case report.
Jayanth KS, Balamurugan S, Sureshkumar S, Anandhi A, Sree Rekha J, Abdulbasith KM, Rajesh BS, Tajudeen M
Tumori 2020 Dec;106(6):NP84-NP88. Epub 2020 Aug 24 doi: 10.1177/0300891620949656. PMID: 32830993
Inborn Errors of Bile Acid Metabolism.
Heubi JE, Setchell KDR, Bove KE
Clin Liver Dis 2018 Nov;22(4):671-687. Epub 2018 Aug 22 doi: 10.1016/j.cld.2018.06.006. PMID: 30266156
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Sitosterolemia: diagnosis, investigation, and management.
Escolà-Gil JC, Quesada H, Julve J, Martín-Campos JM, Cedó L, Blanco-Vaca F
Curr Atheroscler Rep 2014 Jul;16(7):424. doi: 10.1007/s11883-014-0424-2. PMID: 24821603
Primary hypophysitis: clinical-pathological correlations.
Gutenberg A, Hans V, Puchner MJ, Kreutzer J, Brück W, Caturegli P, Buchfelder M
Eur J Endocrinol 2006 Jul;155(1):101-7. doi: 10.1530/eje.1.02183. PMID: 16793955

Therapy

Inborn Errors of Bile Acid Metabolism.
Heubi JE, Setchell KDR, Bove KE
Clin Liver Dis 2018 Nov;22(4):671-687. Epub 2018 Aug 22 doi: 10.1016/j.cld.2018.06.006. PMID: 30266156
Adult Orbital and Adnexal Xanthogranulomatous Disease.
Davies MJ, Whitehead K, Quagliotto G, Wood D, Patheja RS, Sullivan TJ
Asia Pac J Ophthalmol (Phila) 2017 Sep-Oct;6(5):435-443. Epub 2017 Aug 22 doi: 10.22608/APO.2017246. PMID: 28829109
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Sitosterolemia: diagnosis, investigation, and management.
Escolà-Gil JC, Quesada H, Julve J, Martín-Campos JM, Cedó L, Blanco-Vaca F
Curr Atheroscler Rep 2014 Jul;16(7):424. doi: 10.1007/s11883-014-0424-2. PMID: 24821603
Primary hypophysitis: clinical-pathological correlations.
Gutenberg A, Hans V, Puchner MJ, Kreutzer J, Brück W, Caturegli P, Buchfelder M
Eur J Endocrinol 2006 Jul;155(1):101-7. doi: 10.1530/eje.1.02183. PMID: 16793955

Prognosis

Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Necrobiotic xanthogranuloma as an unusual cause of refractive chronic bilateral leg ulceration.
Ghiasi N, Alavi A, Coutts PM, Ghazarian D, Sibbald RG
Int J Low Extrem Wounds 2012 Dec;11(4):293-5. doi: 10.1177/1534734612465434. PMID: 23222162
61-year-old woman presenting with deep coma during treatment for adrenal insufficiency.
Iwaki T, Sawa H, Tateishi J
Neuropathology 2002 Dec;22(4):353-5. doi: 10.1046/j.1440-1789.2002.00465.x. PMID: 12564777
Letterer-Siwe disease. A survey and a review of the course in two subchronic cases.
Nyholm K, Reed G, Sjolin KE
Acta Pathol Microbiol Scand 1967;70(4):481-500. PMID: 5582374

Clinical prediction guides

The molecular basis of autosomal recessive diseases among the Arabs and Druze in Israel.
Zlotogora J
Hum Genet 2010 Nov;128(5):473-9. Epub 2010 Sep 18 doi: 10.1007/s00439-010-0890-8. PMID: 20852892
Wolman's disease. Roentgen observations in 3 siblings.
Queloz JM, Capitanio MA, Kirkpatrick JA
Radiology 1972 Aug;104(2):357-9. doi: 10.1148/104.2.357. PMID: 5044990

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