Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Calciphylaxis is a rare condition of induced systemic hypersensitivity in which tissues respond to appropriate challenging agents with a sudden local calcification. It is characterized by acute calcium deposition in the medial layer of small and intermediate dermal vasculature that can lead to epidermal ischemia, ulceration, and necrosis. Calciphylaxis typically occurs in patients with end-stage renal disease who are undergoing dialysis and who have secondary hyperparathyroidism. Even in this population the incidence is less than 1%. The cause of calciphylaxis is unknown. However, it has been suggested that deficiencies of protein C and protein S may play a role in the pathophysiology of this disorder. Our patient is the fourth with cirrhosis to be reported to have developed calciphylaxis and adds further evidence that low levels of these anticoagulant factors may be an important etiologic factor for development of calciphylaxis. This report should alert the clinician that calciphylaxis occurs in patients with cirrhosis and should stimulate further research concerning the possible role of protein C and protein S deficiency in calciphylaxis.
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PMID:Calciphylaxis: a rare association with alcoholic cirrhosis. Are deficiencies in protein C and S the cause? 1610 46

Calciphylaxis is an uncommon disease characterized by calcification of dermal vessels that determines skin necrosis. Calciphylaxis has been almost exclusively reported in association with renal failure and altered phosphor-calcium metabolism. Only a few cases have been described in hyperparathyroidism, malignancies, and, recently, cirrhosis. We report a patient that developed calciphylaxis related to end-stage alcoholic cirrhosis, without any alteration in the phosphocalcic and parathyroid hormone metabolisms. Possible contributing factors were repeated albumin infusions and low levels of protein C and S.
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PMID:Calciphylaxis associated with alcoholic cirrhosis. 1668 92

Calciphylaxis is a rare disorder characterized by painful skin necrosis and calcification of small vessels which is seen mainly in the dialysis population. We describe a case of a patient with alcoholic cirrhosis developing calciphylaxis during an episode of acute kidney injury. We also review the literature in prior cases and postulate that unrecognized renal dysfunction in cirrhotic patients may have played a role in the development of this uncommon disorder. Our patient had complete recovery of the lesions with conservative treatment which coincided with recovery of her renal function. This case serves to highlight how small changes in creatinine levels in patients with cirrhosis may represent larger and clinically relevant changes in actual renal function that predispose these patients to calciphylaxis.
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PMID:Calciphylaxis in a patient with acute kidney injury and alcoholic cirrhosis. 2210 55

Calciphylaxis is a rare vascular disorder characterized by calcification of arterioles which causes tissue inflammation and necrosis. It is associated with the metabolic disturbances seen in end-stage renal disease (ESRD) and has also been described in patients with cirrhosis with preserved kidney function. Characteristic calciphylaxis lesions are black eschars surrounded by retiform purpura, and the gold standard for diagnosis is skin biopsy. Reported 1-year mortality rates range between 45% and 80%. No treatment modality has been evaluated in a prospective randomized trial, and reports of treatment efficacy vary. Kidney transplant has been reported as a successful therapy for calciphylaxis; however, cases exist of the initial onset of calciphylaxis following kidney transplant as well as simultaneous liver-kidney (SLK) transplant. The decision to maintain a patient with end-stage renal and liver disease on the waiting list for SLK transplant following the onset of calciphylaxis must consider the high 1-year mortality associated with this condition. More research is necessary to understand how to allocate donor allografts to manage patients with calciphylaxis and ESRD and/or cirrhosis effectively.
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PMID:Calciphylaxis in end-stage liver and renal disease patients before and after transplant. 2971 30

Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare, life-threatening cutaneous systemic disease that typically occurs in the setting of end-stage renal disease (ESRD). ESRD is the most recognized risk factor for calciphylaxis but it is not the sole risk factor. Calciphylaxis has also been associated with liver cirrhosis with or without concurrent renal disease. The current case describes a patient who developed calciphylaxis in the setting of hemodialysis, liver cirrhosis, and atrial fibrillation managed with warfarin therapy, all risk factors for calciphylaxis. The need for alternatives to warfarin therapy, specifically in patients with atrial fibrillation on hemodialysis for ESRD who are at increased risk for calciphylaxis, is discussed. Specifically, the left atrial appendage occluder device is described and the need for interdisciplinary management of these patients is stressed.
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PMID:Calciphylaxis in the Setting of Hemodialysis, Liver Cirrhosis, and Warfarin Therapy for Atrial Fibrillation: An Argument for Alternative Anti-Embolic Therapy. 3252 55