Multiple extremity necrosis in fatal calciphylaxis: Case report
ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Nefrologia
2020-07-01
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| Series: | Brazilian Journal of Nephrology |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002021000200274&tlng=pt |
| _version_ | 1818972507426783232 |
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| author | Diego Ennes Gonzalez Renato Demarchi Foresto Ana Luiza Santos Maldonado Wallace Stwart Carvalho Padilha Fernanda Badiani Roberto Maria Eduarda Vilanova da Costa Pereira Marcelino de Souza Durão Junior Aluizio Barbosa Carvalho |
| author_facet | Diego Ennes Gonzalez Renato Demarchi Foresto Ana Luiza Santos Maldonado Wallace Stwart Carvalho Padilha Fernanda Badiani Roberto Maria Eduarda Vilanova da Costa Pereira Marcelino de Souza Durão Junior Aluizio Barbosa Carvalho |
| author_sort | Diego Ennes Gonzalez |
| collection | DOAJ |
| description | ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness. |
| first_indexed | 2024-12-20T15:09:22Z |
| format | Article |
| id | doaj.art-e97e53a00dfe44f68c1eac09e5cab929 |
| institution | Directory Open Access Journal |
| issn | 2175-8239 |
| language | English |
| last_indexed | 2024-12-20T15:09:22Z |
| publishDate | 2020-07-01 |
| publisher | Sociedade Brasileira de Nefrologia |
| record_format | Article |
| series | Brazilian Journal of Nephrology |
| spelling | doaj.art-e97e53a00dfe44f68c1eac09e5cab9292022-12-21T19:36:22ZengSociedade Brasileira de NefrologiaBrazilian Journal of Nephrology2175-82392020-07-0143227427810.1590/2175-8239-jbn-2020-0025Multiple extremity necrosis in fatal calciphylaxis: Case reportDiego Ennes Gonzalezhttps://orcid.org/0000-0003-1455-9158Renato Demarchi Forestohttps://orcid.org/0000-0001-9370-0265Ana Luiza Santos MaldonadoWallace Stwart Carvalho Padilhahttps://orcid.org/0000-0003-3716-6058Fernanda Badiani Robertohttps://orcid.org/0000-0003-2870-7131Maria Eduarda Vilanova da Costa PereiraMarcelino de Souza Durão Juniorhttps://orcid.org/0000-0003-1341-5697Aluizio Barbosa Carvalhohttps://orcid.org/0000-0001-5907-7764ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002021000200274&tlng=ptVascular CalcificationRenal Insufficiency, ChronicCalciphylaxisMonckeberg Medial Calcific Sclerosis |
| spellingShingle | Diego Ennes Gonzalez Renato Demarchi Foresto Ana Luiza Santos Maldonado Wallace Stwart Carvalho Padilha Fernanda Badiani Roberto Maria Eduarda Vilanova da Costa Pereira Marcelino de Souza Durão Junior Aluizio Barbosa Carvalho Multiple extremity necrosis in fatal calciphylaxis: Case report Brazilian Journal of Nephrology Vascular Calcification Renal Insufficiency, Chronic Calciphylaxis Monckeberg Medial Calcific Sclerosis |
| title | Multiple extremity necrosis in fatal calciphylaxis: Case report |
| title_full | Multiple extremity necrosis in fatal calciphylaxis: Case report |
| title_fullStr | Multiple extremity necrosis in fatal calciphylaxis: Case report |
| title_full_unstemmed | Multiple extremity necrosis in fatal calciphylaxis: Case report |
| title_short | Multiple extremity necrosis in fatal calciphylaxis: Case report |
| title_sort | multiple extremity necrosis in fatal calciphylaxis case report |
| topic | Vascular Calcification Renal Insufficiency, Chronic Calciphylaxis Monckeberg Medial Calcific Sclerosis |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002021000200274&tlng=pt |
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