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Visceral calciphylaxis

MedGen UID:
1664968
Concept ID:
C4749793
Disease or Syndrome
Synonym: visceral calciphylaxis
SNOMED CT: Visceral calciphylaxis (771236000)
 
Monarch Initiative: MONDO:0017217
Orphanet: ORPHA280068

Definition

A rare life-threatening non-inflammatory vasculopathy disorder characterized by diffuse precipitation of calcium in viscera (mainly in the heart or lungs, but also in the stomach or kidneys) leading to fibrosis and thrombosis, which eventually cause necrotic ulcerations of the tissue. Patients may present with dyspnea, cough and respiratory failure or acute heart block and subsequent sudden cardiac death, depending on the affected organ. The disease mainly affects patients on dialysis or patients having undergone renal transplantation. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVisceral calciphylaxis

Professional guidelines

PubMed

Chiriac A, Grosu OM, Terinte C, Perţea M
J Dermatolog Treat 2020 Aug;31(5):545-548. Epub 2019 May 31 doi: 10.1080/09546634.2019.1618435. PMID: 31075991

Recent clinical studies

Etiology

Beyzaei A, Francis J, Knight H, Simon DB, Finkelstein FO
Adv Perit Dial 2007;23:112-7. PMID: 17886615

Diagnosis

Chiriac A, Grosu OM, Terinte C, Perţea M
J Dermatolog Treat 2020 Aug;31(5):545-548. Epub 2019 May 31 doi: 10.1080/09546634.2019.1618435. PMID: 31075991
Shen G, Huang R, Liu B, Kuang A
Clin Nucl Med 2017 Nov;42(11):893-895. doi: 10.1097/RLU.0000000000001837. PMID: 28872557
Shapiro C, Coco M
Clin Nephrol 2007 Feb;67(2):119-25. doi: 10.5414/cnp67119. PMID: 17338433

Therapy

Chiriac A, Grosu OM, Terinte C, Perţea M
J Dermatolog Treat 2020 Aug;31(5):545-548. Epub 2019 May 31 doi: 10.1080/09546634.2019.1618435. PMID: 31075991
Shen G, Huang R, Liu B, Kuang A
Clin Nucl Med 2017 Nov;42(11):893-895. doi: 10.1097/RLU.0000000000001837. PMID: 28872557

Supplemental Content

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