Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal
Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. Its pathogenesis is multifactorial and is the consequence of many factors causing ectopic calcifications in patients with chronic kidney disease,...
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| Format: | Article |
| Language: | English |
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Elsevier
2018-11-01
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| Series: | Nefrología (English Edition) |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2013251418301068 |
| _version_ | 1818425012150861824 |
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| author | David Cucchiari Jose-Vicente Torregrosa |
| author_facet | David Cucchiari Jose-Vicente Torregrosa |
| author_sort | David Cucchiari |
| collection | DOAJ |
| description | Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. Its pathogenesis is multifactorial and is the consequence of many factors causing ectopic calcifications in patients with chronic kidney disease, such as calcium-phosphate metabolism disorders, hyper- or hypo-parathyroidism, diabetes, obesity, systemic inflammation and the use of vitamin K antagonists, among others. From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. Treatment is primarily based on managing the wounds, eliminating all the possible precipitating factors of ectopic calcification and administering agents which are capable of inhibiting the process of calcification. Resumen: La calcifilaxis, también denominada arteriolopatía urémica calcificante, es un síndrome raro que causa típicamente necrosis cutánea y que afecta principalmente a los pacientes en diálisis. La patogénesis es multifactorial y depende de la suma de todos los factores que producen calcificaciones ectópicas en el paciente con enfermedad renal crónica, como las alteraciones del metabolismo calcio-fósforo, el hiper o el hipoparatiroidismo, la diabetes, la obesidad, la inflamación sistémica y el uso de inhibidores de vitamina K, entre otros. Desde un punto de vista clínico, la calcifilaxis puede evolucionar desde una púrpura dolorosa hasta extensas áreas de necrosis cutánea que pueden sobreinfectarse y llegar a causar el fallecimiento del paciente por sepsis. El tratamiento se basa fundamentalmente en el manejo de las heridas, la eliminación de todos los elementos que puedan precipitar la calcificación ectópica y el uso de agentes inhibidores del proceso de calcificación. Keywords: Calciphylaxis, Vascular calcifications, Hyperparathyroidism, Bisphosphonates, Palabras clave: Calcifilaxis, Calcificaciones vasculares, Hiperparatiroidismo, Bifosfonatos |
| first_indexed | 2024-12-14T14:07:10Z |
| format | Article |
| id | doaj.art-82f33a1abca54a509fac856cf504b068 |
| institution | Directory Open Access Journal |
| issn | 2013-2514 |
| language | English |
| last_indexed | 2024-12-14T14:07:10Z |
| publishDate | 2018-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Nefrología (English Edition) |
| spelling | doaj.art-82f33a1abca54a509fac856cf504b0682022-12-21T22:58:26ZengElsevierNefrología (English Edition)2013-25142018-11-01386579586Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatalDavid Cucchiari0Jose-Vicente Torregrosa1Servicio de Nefrología y Trasplante Renal, Hospital Clínic, Barcelona, Spain; Nephrology and Dialysis Unit, Humanitas Clinical Research Center, Rozzano, ItalyServicio de Nefrología y Trasplante Renal, Hospital Clínic, Barcelona, Spain; Corresponding author.Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. Its pathogenesis is multifactorial and is the consequence of many factors causing ectopic calcifications in patients with chronic kidney disease, such as calcium-phosphate metabolism disorders, hyper- or hypo-parathyroidism, diabetes, obesity, systemic inflammation and the use of vitamin K antagonists, among others. From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. Treatment is primarily based on managing the wounds, eliminating all the possible precipitating factors of ectopic calcification and administering agents which are capable of inhibiting the process of calcification. Resumen: La calcifilaxis, también denominada arteriolopatía urémica calcificante, es un síndrome raro que causa típicamente necrosis cutánea y que afecta principalmente a los pacientes en diálisis. La patogénesis es multifactorial y depende de la suma de todos los factores que producen calcificaciones ectópicas en el paciente con enfermedad renal crónica, como las alteraciones del metabolismo calcio-fósforo, el hiper o el hipoparatiroidismo, la diabetes, la obesidad, la inflamación sistémica y el uso de inhibidores de vitamina K, entre otros. Desde un punto de vista clínico, la calcifilaxis puede evolucionar desde una púrpura dolorosa hasta extensas áreas de necrosis cutánea que pueden sobreinfectarse y llegar a causar el fallecimiento del paciente por sepsis. El tratamiento se basa fundamentalmente en el manejo de las heridas, la eliminación de todos los elementos que puedan precipitar la calcificación ectópica y el uso de agentes inhibidores del proceso de calcificación. Keywords: Calciphylaxis, Vascular calcifications, Hyperparathyroidism, Bisphosphonates, Palabras clave: Calcifilaxis, Calcificaciones vasculares, Hiperparatiroidismo, Bifosfonatoshttp://www.sciencedirect.com/science/article/pii/S2013251418301068 |
| spellingShingle | David Cucchiari Jose-Vicente Torregrosa Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal Nefrología (English Edition) |
| title | Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal |
| title_full | Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal |
| title_fullStr | Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal |
| title_full_unstemmed | Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal |
| title_short | Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal |
| title_sort | calciphylaxis in patients with chronic kidney disease a disease which is still bewildering and potentially fatal |
| url | http://www.sciencedirect.com/science/article/pii/S2013251418301068 |
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