BACKGROUND Calciphylaxis is a form of vascular calcification more commonly associated with renal disease. While the exact mechanism of calciphylaxis is poorly understood,most cases are due to end stage kidney disease....BACKGROUND Calciphylaxis is a form of vascular calcification more commonly associated with renal disease. While the exact mechanism of calciphylaxis is poorly understood,most cases are due to end stage kidney disease. However, it can also be found in patients without kidney disease and in such cases is termed non-uremic calciphylaxis for which have multiple proposed etiologies.CASE SUMMARY We describe a case of a thirty-year-old morbidly obese Caucasian female who had a positive history of alcoholic hepatitis and presented with painful calciphylaxis wounds of the abdomen, hips, and thighs. The hypercoagulability panel showed low levels of Protein C and normal Protein S, low Antithrombin Ⅲ and positive lupus anticoagulant and negative anticardiolipin. Wound biopsy confirmed the diagnosis of non-uremic calciphylaxis in the setting of alcoholic liver disease. The calciphylaxis wounds did not improve when Sodium Thiosulfate was used alone. The patient underwent a series of bedside and surgical debridement. Broad spectrum antibiotics were also used for secondary wound bacterial infections. The patient passed away shortly after due to sepsis and multiorgan failure.CONCLUSION Non-uremic Calciphylaxis can occur in the setting of alcoholic liver disease. The treatment of choice is still unknown.展开更多
Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a fatal disease with high mortality that mostly affects patients with end stage kidney disease (ESKD) on dialysis. In recent years the incidence of ...Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a fatal disease with high mortality that mostly affects patients with end stage kidney disease (ESKD) on dialysis. In recent years the incidence of CUA has been increasing among patients on peritoneal dialysis (PD). The exact mechanism and risk factors are not well described.展开更多
Calcific uremic arteriolopathy (CUA) is a rare pathology affecting 5% of dialysis patients but with a poor prognosis. It is characterized by calcification and thrombotic lesions of the microcirculation leading to hype...Calcific uremic arteriolopathy (CUA) is a rare pathology affecting 5% of dialysis patients but with a poor prognosis. It is characterized by calcification and thrombotic lesions of the microcirculation leading to hyperalgesic ischemic skin lesions. Several risk factors have been identified, mainly warfarin treatment, mineral and bone disorders (MBD), inflammation and malnutrition. In the evocative forms, the diagnosis is made based upon the physical examination finding of classic painful ulcerated lesions that are covered by a black eschar. Skin biopsy, due to the risk of aggravation and delayed healing, is only performed in case of doubt diagnosis. The therapeutic attitude due to the lack of solid randomized studies is based on expert consensus and requires a multidisciplinary approach. We report here the case of a patient with CUA revealed in the form of multiple ulcerative-necrotic skin lesions associated with pressure sores and arterial wounds.展开更多
Calciphylaxis is a condition of induced hypersensitivity in which tissues respond to challenge agents with local calcification. This article reports the first known case of diffuse calciphylaxis associated with acute ...Calciphylaxis is a condition of induced hypersensitivity in which tissues respond to challenge agents with local calcification. This article reports the first known case of diffuse calciphylaxis associated with acute myeloid leukemia resulting in death from hypoxic respiratory failure and refractory hypotension.展开更多
Calciphylaxis is a serious disorder that presents itself as ischemia and ne-crosis of the skin which occurs more frequently in patients with an end-stage chronic kidney disease, but not exclusively. The pathogenesis i...Calciphylaxis is a serious disorder that presents itself as ischemia and ne-crosis of the skin which occurs more frequently in patients with an end-stage chronic kidney disease, but not exclusively. The pathogenesis is a result of the reduction of arteriolar blood flow, caused by calcification, fibrosis, and thrombus formation that primarily involve the arterioles of the dermis and hypodermis, with a poor prognosis. Case presentation: A 44-year-old patient with a previous diagnosis of chronic kidney disease receiving hemodialysis secondary to polycystic kidney disease, with a history of parathyroidectomy due to primary hyperparathyroidism in 2011. In 2014 the patient presented skin lesions, for which a diagnostic biopsy of calciphylaxis was performed and began treatment with sodium thiosulfate with a poor progression and evolution. New histology compatible with the diagnosis of pyoderma gangrenosum and findings of calciphylaxis were performed. The patient begins treatment with corticosteroids and cyclosporine, with poor clinical evolution and the patient eventually passes away. The objective of this manuscript is to understand this pathology better, which is infrequent but with a high rate of morbidity and mortality.展开更多
Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis,with high mortality and no proven therapy.Here,we reported a severe uremic calciphylaxis patient with progr...Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis,with high mortality and no proven therapy.Here,we reported a severe uremic calciphylaxis patient with progressive skin ischemia,large areas of painful malodorous ulcers,and mummified legs.Because of the worsening symptoms and signs refractory to conventional therapies,treatment with human amnion-derived mesenchymal stem cells(hAMSCs)was approved.Preclinical release inspections of hAMSCs,efficacy,and safety assessment,including cytokine secretory ability,immunocompetence,tumorigenicity,and genetics analysis in vitro,were introduced.We further performed acute and long-term hAMSC toxicity evaluations in C57BL/6 mice and rats,abnormal immune response tests in C57BL/6 mice,and tumorigenicity tests in neonatal Balbc-nu nude mice.After the preclinical research,the patient was treated with hAMSCs by intravenous and local intramuscular injection and external supernatant application to the ulcers.When followed up to 15 months,the blood-based markers of bone and mineral metabolism improved,with skin soft tissue regeneration and a more favorable profile of peripheral blood mononuclear cells.Skin biopsy after 1-month treatment showed vascular regeneration with mature noncalcified vessels within the dermis,and 20 months later,the re-epithelialization restored the integrity of the damaged site.No infusion or local treatment-related adverse events occurred.Thus,this novel long-term intravenous combined with local treatment with hAMSCs warrants further investigation as a potential regenerative treatment for uremic calciphylaxis due to effects of inhibiting vascular calcification,stimulating angiogenesis and myogenesis,anti-inflammatory and immune modulation,multidifferentiation,re-epithelialization,and restoration of integrity.展开更多
文摘BACKGROUND Calciphylaxis is a form of vascular calcification more commonly associated with renal disease. While the exact mechanism of calciphylaxis is poorly understood,most cases are due to end stage kidney disease. However, it can also be found in patients without kidney disease and in such cases is termed non-uremic calciphylaxis for which have multiple proposed etiologies.CASE SUMMARY We describe a case of a thirty-year-old morbidly obese Caucasian female who had a positive history of alcoholic hepatitis and presented with painful calciphylaxis wounds of the abdomen, hips, and thighs. The hypercoagulability panel showed low levels of Protein C and normal Protein S, low Antithrombin Ⅲ and positive lupus anticoagulant and negative anticardiolipin. Wound biopsy confirmed the diagnosis of non-uremic calciphylaxis in the setting of alcoholic liver disease. The calciphylaxis wounds did not improve when Sodium Thiosulfate was used alone. The patient underwent a series of bedside and surgical debridement. Broad spectrum antibiotics were also used for secondary wound bacterial infections. The patient passed away shortly after due to sepsis and multiorgan failure.CONCLUSION Non-uremic Calciphylaxis can occur in the setting of alcoholic liver disease. The treatment of choice is still unknown.
文摘Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a fatal disease with high mortality that mostly affects patients with end stage kidney disease (ESKD) on dialysis. In recent years the incidence of CUA has been increasing among patients on peritoneal dialysis (PD). The exact mechanism and risk factors are not well described.
文摘Calcific uremic arteriolopathy (CUA) is a rare pathology affecting 5% of dialysis patients but with a poor prognosis. It is characterized by calcification and thrombotic lesions of the microcirculation leading to hyperalgesic ischemic skin lesions. Several risk factors have been identified, mainly warfarin treatment, mineral and bone disorders (MBD), inflammation and malnutrition. In the evocative forms, the diagnosis is made based upon the physical examination finding of classic painful ulcerated lesions that are covered by a black eschar. Skin biopsy, due to the risk of aggravation and delayed healing, is only performed in case of doubt diagnosis. The therapeutic attitude due to the lack of solid randomized studies is based on expert consensus and requires a multidisciplinary approach. We report here the case of a patient with CUA revealed in the form of multiple ulcerative-necrotic skin lesions associated with pressure sores and arterial wounds.
文摘Calciphylaxis is a condition of induced hypersensitivity in which tissues respond to challenge agents with local calcification. This article reports the first known case of diffuse calciphylaxis associated with acute myeloid leukemia resulting in death from hypoxic respiratory failure and refractory hypotension.
文摘Calciphylaxis is a serious disorder that presents itself as ischemia and ne-crosis of the skin which occurs more frequently in patients with an end-stage chronic kidney disease, but not exclusively. The pathogenesis is a result of the reduction of arteriolar blood flow, caused by calcification, fibrosis, and thrombus formation that primarily involve the arterioles of the dermis and hypodermis, with a poor prognosis. Case presentation: A 44-year-old patient with a previous diagnosis of chronic kidney disease receiving hemodialysis secondary to polycystic kidney disease, with a history of parathyroidectomy due to primary hyperparathyroidism in 2011. In 2014 the patient presented skin lesions, for which a diagnostic biopsy of calciphylaxis was performed and began treatment with sodium thiosulfate with a poor progression and evolution. New histology compatible with the diagnosis of pyoderma gangrenosum and findings of calciphylaxis were performed. The patient begins treatment with corticosteroids and cyclosporine, with poor clinical evolution and the patient eventually passes away. The objective of this manuscript is to understand this pathology better, which is infrequent but with a high rate of morbidity and mortality.
基金funded by the National Natural Science Foundation of China(81270408,81570666,81730041,and 81671447)the International Society of Nephrology(ISN)Clinical Research Program(18-01-0247)+7 种基金Construction Program of Jiangsu Provincial Clinical Research Center Support System(BL2014084)Jiangsu Province Key Medical Personnel Project(ZDRCA2016002)CKD Anemia Research Foundation from China International Medical Foundation(Z-2017-24-2037)Outstanding Young and Middle-Aged Talents Support Program of The First Affiliated Hospital of Nanjing Medical University(Jiangsu Province Hospital)the National Key Research and Development Program of China(2017YFC1001303)the Program of Jiangsu Province Clinical Medical Center(YXZXB2016001,BL2012009)the State Key Laboratory of Reproductive Medicine Program(SKLRM-GC201803)the Program of Jiangsu Commission of Health(H201605).
文摘Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis,with high mortality and no proven therapy.Here,we reported a severe uremic calciphylaxis patient with progressive skin ischemia,large areas of painful malodorous ulcers,and mummified legs.Because of the worsening symptoms and signs refractory to conventional therapies,treatment with human amnion-derived mesenchymal stem cells(hAMSCs)was approved.Preclinical release inspections of hAMSCs,efficacy,and safety assessment,including cytokine secretory ability,immunocompetence,tumorigenicity,and genetics analysis in vitro,were introduced.We further performed acute and long-term hAMSC toxicity evaluations in C57BL/6 mice and rats,abnormal immune response tests in C57BL/6 mice,and tumorigenicity tests in neonatal Balbc-nu nude mice.After the preclinical research,the patient was treated with hAMSCs by intravenous and local intramuscular injection and external supernatant application to the ulcers.When followed up to 15 months,the blood-based markers of bone and mineral metabolism improved,with skin soft tissue regeneration and a more favorable profile of peripheral blood mononuclear cells.Skin biopsy after 1-month treatment showed vascular regeneration with mature noncalcified vessels within the dermis,and 20 months later,the re-epithelialization restored the integrity of the damaged site.No infusion or local treatment-related adverse events occurred.Thus,this novel long-term intravenous combined with local treatment with hAMSCs warrants further investigation as a potential regenerative treatment for uremic calciphylaxis due to effects of inhibiting vascular calcification,stimulating angiogenesis and myogenesis,anti-inflammatory and immune modulation,multidifferentiation,re-epithelialization,and restoration of integrity.