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Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition

Calcific uremic arteriolopathy, or calciphylaxis, is a highly morbid, life-threatening syndrome of microvascular calcification leading to progressive skin necrosis. It affects 1-4% of the population with end-stage renal disease (ESRD) and is rarely seen in other conditions. The one-year mortality ra...

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Autores principales: Okan, Tetyana, Ghuman, Kulbir, Chaudhary, Faiza, Doshi, Kaushik, Shoaib, Usman
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10687493/
https://www.ncbi.nlm.nih.gov/pubmed/38034199
http://dx.doi.org/10.7759/cureus.48002
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author Okan, Tetyana
Ghuman, Kulbir
Chaudhary, Faiza
Doshi, Kaushik
Shoaib, Usman
author_facet Okan, Tetyana
Ghuman, Kulbir
Chaudhary, Faiza
Doshi, Kaushik
Shoaib, Usman
author_sort Okan, Tetyana
collection PubMed
description Calcific uremic arteriolopathy, or calciphylaxis, is a highly morbid, life-threatening syndrome of microvascular calcification leading to progressive skin necrosis. It affects 1-4% of the population with end-stage renal disease (ESRD) and is rarely seen in other conditions. The one-year mortality rate is 80% for patients with ulcerations. There is no evidence-based treatment, and the response to therapy is poor; thus, it creates a serious therapeutic challenge for clinicians. Our aim is to discuss a diagnostic approach and management of calciphylaxis and to raise awareness about this condition among the medical community. We are describing a case of calciphylaxis in a 68-year-old female with a past medical history of ESRD on hemodialysis, who presented with altered mental status and painful ulcers on her feet and thighs. The patient was admitted for acute encephalopathy due to sepsis secondary to a urinary tract infection (UTI) versus permacath-related bacteremia versus wound infection of pressure ulcers on the lower extremities. Broad-spectrum antibiotics were started. Computed tomography (CT) of bilateral thighs with contrast showed extensive arterial calcifications, characteristic of calciphylaxis. Nephrology recommended sodium thiosulfate with each hemodialysis session. Vitamin D and iron were discontinued. Despite therapy, the wound significantly progressed within the next eight weeks, leading to mortality due to sepsis. In conclusion, calcific uremic arteriolopathy is a challenging disease with a poor prognosis. Early diagnosis is crucial, so aggressive therapy can be started immediately. A multidisciplinary approach may improve survival in cases of calciphylaxis. More studies are needed to improve the diagnostic approach and medical management of the disease.
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spelling pubmed-106874932023-11-30 Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition Okan, Tetyana Ghuman, Kulbir Chaudhary, Faiza Doshi, Kaushik Shoaib, Usman Cureus Geriatrics Calcific uremic arteriolopathy, or calciphylaxis, is a highly morbid, life-threatening syndrome of microvascular calcification leading to progressive skin necrosis. It affects 1-4% of the population with end-stage renal disease (ESRD) and is rarely seen in other conditions. The one-year mortality rate is 80% for patients with ulcerations. There is no evidence-based treatment, and the response to therapy is poor; thus, it creates a serious therapeutic challenge for clinicians. Our aim is to discuss a diagnostic approach and management of calciphylaxis and to raise awareness about this condition among the medical community. We are describing a case of calciphylaxis in a 68-year-old female with a past medical history of ESRD on hemodialysis, who presented with altered mental status and painful ulcers on her feet and thighs. The patient was admitted for acute encephalopathy due to sepsis secondary to a urinary tract infection (UTI) versus permacath-related bacteremia versus wound infection of pressure ulcers on the lower extremities. Broad-spectrum antibiotics were started. Computed tomography (CT) of bilateral thighs with contrast showed extensive arterial calcifications, characteristic of calciphylaxis. Nephrology recommended sodium thiosulfate with each hemodialysis session. Vitamin D and iron were discontinued. Despite therapy, the wound significantly progressed within the next eight weeks, leading to mortality due to sepsis. In conclusion, calcific uremic arteriolopathy is a challenging disease with a poor prognosis. Early diagnosis is crucial, so aggressive therapy can be started immediately. A multidisciplinary approach may improve survival in cases of calciphylaxis. More studies are needed to improve the diagnostic approach and medical management of the disease. Cureus 2023-10-30 /pmc/articles/PMC10687493/ /pubmed/38034199 http://dx.doi.org/10.7759/cureus.48002 Text en Copyright © 2023, Okan et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Geriatrics
Okan, Tetyana
Ghuman, Kulbir
Chaudhary, Faiza
Doshi, Kaushik
Shoaib, Usman
Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title_full Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title_fullStr Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title_full_unstemmed Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title_short Calcific Uremic Arteriolopathy in End-Stage Renal Disease: A Rare Life-Threatening Condition
title_sort calcific uremic arteriolopathy in end-stage renal disease: a rare life-threatening condition
topic Geriatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10687493/
https://www.ncbi.nlm.nih.gov/pubmed/38034199
http://dx.doi.org/10.7759/cureus.48002
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