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yago:WikicatVascular-relatedCutaneousConditions owl:Thing yago:Illness114061805 umbel-rc:AilmentCondition yago:PathologicalState114051917 yago:Disease114070360 yago:Abstraction100002137 wikidata:Q12136 yago:PhysicalCondition114034177 yago:Condition113920835 dbo:Disease yago:State100024720 yago:IllHealth114052046 yago:Attribute100024264
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Calcifilaxia Calciphylaxis Calcifilassi التأق التكلسي Calciphylaxie
rdfs:comment
La calcifilaxia es una enfermedad de los vasos sanguíneos (vasculopatía) rara, aunque grave, con una elevada tasa de morbimortalidad.​ Se caracteriza por isquemia y necrosis cutánea secundaria a la calcificación, fibrodisplasia de la íntima y trombosis de pequeñas arteriolas dermoepidérmicas​​​​​​ Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome characterized by painful skin lesions. The pathogenesis of calciphylaxis is unclear but believed to involve calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and eventual death of skin cells due to lack of blood flow. It is seen mostly in people with end-stage kidney disease but can occur in the earlier stages of chronic kidney disease and rarely in people with normally functioning kidneys. Calciphylaxis is a rare but serious disease, believed to affect 1-4% of all dialysis patients. It results in chronic non-healing wounds and indicates poor prognosis, with typical life expectancy of less than one year. La calcifilassi è il risultato di una reazione di ipersensibilità in presenza di paratormone, vitamina D e ipercalcemia. La calcifilassi, nota anche come arteriolopatia uremica calcifica (CUA) o "scala di grigi", è una rara sindrome dolorosa di calcificazione dei piccoli vasi sanguigni situati all'interno del tessuto adiposo e degli strati più profondi della pelle, coaguli di sangue e morte delle cellule della pelle a causa del flusso sanguigno troppo basso. Si osserva principalmente nelle persone con malattia renale allo stadio terminale, ma può verificarsi nelle prime fasi della malattia renale cronica e raramente nelle persone con reni normalmente funzionanti. Si traduce in ferite croniche non cicatrizzanti ed è generalmente fatale. La calcifilassi è una malattia rara ma grave, che si r التأق التكلسي (المعروفة أيضًا باسم اعتلال الشرايين اليوريمي الكلسي أو التدرج الرمادي)، هي متلازمة مؤلمة نادرة لتكلس الأوعية الدموية الصغيرة الموجودة داخل الأنسجة الدهنية، والطبقات العميقة من الجلد والجلطات الدموية وموت خلايا الجلد بسبب نقص في تدفق الدم. يظهر هذا المرض غالبًا عند الأشخاص المصابين بالمرحلة النهائية من أمراض الكلى، ولكن يمكن أن يحدث في المراحل المبكرة من مرض الكلى المزمن، ونادراً ما يُصاب به الأشخاص الذين لا يعانون من أمراض الكلى. يملك المصاب بالمرض العديد من الجروح المزمنة الغير قابلة للشفاء وعادة ما تكون قاتلة. يُعتبر التأق التكلسي مرض نادر ولكنه خطير، ويُعتقد أنه يؤثر على 1-4 ٪ من جميع مرضى غسيل الكلى. Die Calciphylaxie oder Kalziphylaxie (syn.: urämisch-kalzifizierende Arteriolopathie, abgekürzt UCA; metastatische Kalzifizierung) ist eine besonders schwere und schmerzhafte Verlaufsform des Knochenabbaus durch Nierenerkrankungen (renale Osteodystrophie). Sie wird durch krankhafte Einlagerungen von Calcium- und Phosphat-Salzen in die Blutgefäßwände und in das Unterhautfettgewebe charakterisiert. Diese Einlagerungen führen zur schweren Schädigung und Entzündung der betroffenen Blutgefäße (Vaskulitis) und des Unterhautfettgewebes (Pannikulitis) und können ein Absterben (Nekrose) der betroffenen Gewebe und Gefäße nach sich ziehen.
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Calciphylaxis
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Calciphylaxis
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Clinical, skin biopsy may aid diagnosis
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Painful necrotic skin lesions
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Dialysis, analgesics, surgical wound debridement, parathyroidectomy
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Calciphylaxis on the abdomen of a patient with end stage kidney disease. Markings are in cm.
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Nephrology
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1
dbp:synonyms
Calcific Uremic Arteriolopathy
dbo:abstract
La calcifilassi è il risultato di una reazione di ipersensibilità in presenza di paratormone, vitamina D e ipercalcemia. La calcifilassi, nota anche come arteriolopatia uremica calcifica (CUA) o "scala di grigi", è una rara sindrome dolorosa di calcificazione dei piccoli vasi sanguigni situati all'interno del tessuto adiposo e degli strati più profondi della pelle, coaguli di sangue e morte delle cellule della pelle a causa del flusso sanguigno troppo basso. Si osserva principalmente nelle persone con malattia renale allo stadio terminale, ma può verificarsi nelle prime fasi della malattia renale cronica e raramente nelle persone con reni normalmente funzionanti. Si traduce in ferite croniche non cicatrizzanti ed è generalmente fatale. La calcifilassi è una malattia rara ma grave, che si ritiene colpisca l'1-4% di tutti i pazienti in dialisi. La calcifilassi è un tipo di calcificazione extra scheletriche. Alcune calcificazioni extrascheletrichel simili sono osservate in alcune persone con alti livelli di calcio nel sangue, comprese le persone con sindrome da latte-alcali, sarcoidosi, iperparatiroidismo primario e ipervitaminosi D. Alcuni farmaci come il warfarin possono anche provocare calcifilassi in rari casi. La presenza di calcifilassi prevede generalmente una prognosi sfavorevole con un'aspettativa di vita tipica inferiore a un anno. La calcifilassi consiste nella calcificazione elettiva di alcuni tessuti o di alcuni organi. È spesso associato ad uno stato di iperparatiroidismo o di ipervitaminosi D. Nei pazienti affetti da insufficienza renale cronica (particolarmente negli stadi avanzati o in emodialisi) la calcifilassi colpisce i piccoli vasi arteriosi ed è caratterizzata da estese calcificazioni dei vasi piccoli e medi che all'esame obiettivo si presentano come delle lesioni rossastre dolenti di tipo nodulare che possono evolvere verso ulcere necrotiche. Calcifilassi sull'addome di un paziente. La calcifilaxia es una enfermedad de los vasos sanguíneos (vasculopatía) rara, aunque grave, con una elevada tasa de morbimortalidad.​ Se caracteriza por isquemia y necrosis cutánea secundaria a la calcificación, fibrodisplasia de la íntima y trombosis de pequeñas arteriolas dermoepidérmicas​​​​​​ التأق التكلسي (المعروفة أيضًا باسم اعتلال الشرايين اليوريمي الكلسي أو التدرج الرمادي)، هي متلازمة مؤلمة نادرة لتكلس الأوعية الدموية الصغيرة الموجودة داخل الأنسجة الدهنية، والطبقات العميقة من الجلد والجلطات الدموية وموت خلايا الجلد بسبب نقص في تدفق الدم. يظهر هذا المرض غالبًا عند الأشخاص المصابين بالمرحلة النهائية من أمراض الكلى، ولكن يمكن أن يحدث في المراحل المبكرة من مرض الكلى المزمن، ونادراً ما يُصاب به الأشخاص الذين لا يعانون من أمراض الكلى. يملك المصاب بالمرض العديد من الجروح المزمنة الغير قابلة للشفاء وعادة ما تكون قاتلة. يُعتبر التأق التكلسي مرض نادر ولكنه خطير، ويُعتقد أنه يؤثر على 1-4 ٪ من جميع مرضى غسيل الكلى. التأق التكلسي هو نوع واحد من تكلس خارج الهيكل العظمي. لوحظت بعض التكلسات المماثلة خارج الهيكل العظمي لدى بعض الأشخاص الذين يعانون من مستويات عالية من الكالسيوم في الدم، بما في ذلك الأشخاص الذين يعانون من متلازمة الحليب القلوي والساركويد وفرط نشاط الدريقات وفرط إفراز الفيتامين د. يمكن أن تؤثر بعض الأدوية مثل الوارفارين على هذه المتلازمة في حالات نادرة. يتنبأ الإنذار الطبي وجود التأق التكلسي عادةً بشكل ضعيف مع متوسط عمر افتراضي أقل من عام واحد. Die Calciphylaxie oder Kalziphylaxie (syn.: urämisch-kalzifizierende Arteriolopathie, abgekürzt UCA; metastatische Kalzifizierung) ist eine besonders schwere und schmerzhafte Verlaufsform des Knochenabbaus durch Nierenerkrankungen (renale Osteodystrophie). Sie wird durch krankhafte Einlagerungen von Calcium- und Phosphat-Salzen in die Blutgefäßwände und in das Unterhautfettgewebe charakterisiert. Diese Einlagerungen führen zur schweren Schädigung und Entzündung der betroffenen Blutgefäße (Vaskulitis) und des Unterhautfettgewebes (Pannikulitis) und können ein Absterben (Nekrose) der betroffenen Gewebe und Gefäße nach sich ziehen. Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome characterized by painful skin lesions. The pathogenesis of calciphylaxis is unclear but believed to involve calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and eventual death of skin cells due to lack of blood flow. It is seen mostly in people with end-stage kidney disease but can occur in the earlier stages of chronic kidney disease and rarely in people with normally functioning kidneys. Calciphylaxis is a rare but serious disease, believed to affect 1-4% of all dialysis patients. It results in chronic non-healing wounds and indicates poor prognosis, with typical life expectancy of less than one year. Calciphylaxis is one type of extraskeletal calcification. Similar extraskeletal calcifications are observed in some people with high levels of calcium in the blood, including people with milk-alkali syndrome, sarcoidosis, primary hyperparathyroidism, and hypervitaminosis D. In rare cases, certain medications such as warfarin can also result in calciphylaxis.
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Infection, sepsis
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Female sex, obesity, use of Warfarin, protein C or S deficiency, hypoalbuminemia, diabetes mellitus, use of vitamin D derivatives
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