Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies
Autor(a) principal: | |
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Data de Publicação: | 2018 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.17/4078 |
Resumo: | Calciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies. Learning points: Calciphylaxis in the absence of end-stage kidney disease is called non-uremic calciphylaxis (NUC).This disease is a diagnostic and therapeutic challenge.As therapeutic strategies for NUC mainly derive from those for uremic calciphylaxis, more efficient therapeutic measures and evidence-based recommendations are needed. |
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Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic StrategiesHSAC MEDLeg UlcerCalciphylaxisSepsisVascular CalcificationCalciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies. Learning points: Calciphylaxis in the absence of end-stage kidney disease is called non-uremic calciphylaxis (NUC).This disease is a diagnostic and therapeutic challenge.As therapeutic strategies for NUC mainly derive from those for uremic calciphylaxis, more efficient therapeutic measures and evidence-based recommendations are needed.Repositório do Centro Hospitalar Universitário de Lisboa Central, EPEGomes, FLa Feria, PCosta, CSantos, R2022-05-13T14:33:58Z20182018-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/4078engEur J Case Rep Intern Med. 2018 Dec 27;5(12):00098610.12890/2018_000986.info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-03-10T09:45:12Zoai:repositorio.chlc.min-saude.pt:10400.17/4078Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:21:23.344277Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
title |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
spellingShingle |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies Gomes, F HSAC MED Leg Ulcer Calciphylaxis Sepsis Vascular Calcification |
title_short |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
title_full |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
title_fullStr |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
title_full_unstemmed |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
title_sort |
Non-Uremic Calciphylaxis: a Rare Diagnosis with Limited Therapeutic Strategies |
author |
Gomes, F |
author_facet |
Gomes, F La Feria, P Costa, C Santos, R |
author_role |
author |
author2 |
La Feria, P Costa, C Santos, R |
author2_role |
author author author |
dc.contributor.none.fl_str_mv |
Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE |
dc.contributor.author.fl_str_mv |
Gomes, F La Feria, P Costa, C Santos, R |
dc.subject.por.fl_str_mv |
HSAC MED Leg Ulcer Calciphylaxis Sepsis Vascular Calcification |
topic |
HSAC MED Leg Ulcer Calciphylaxis Sepsis Vascular Calcification |
description |
Calciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies. Learning points: Calciphylaxis in the absence of end-stage kidney disease is called non-uremic calciphylaxis (NUC).This disease is a diagnostic and therapeutic challenge.As therapeutic strategies for NUC mainly derive from those for uremic calciphylaxis, more efficient therapeutic measures and evidence-based recommendations are needed. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018 2018-01-01T00:00:00Z 2022-05-13T14:33:58Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.17/4078 |
url |
http://hdl.handle.net/10400.17/4078 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Eur J Case Rep Intern Med. 2018 Dec 27;5(12):000986 10.12890/2018_000986. |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
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application/pdf |
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reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799131309382041600 |