BACKGROUND Calciphylaxis,also called calcific uremic arteriolopathy,is characterized by microvascular calcification and occlusion,which is commonly seen in patients with end-stage renal disease(ESRD).Although several ...BACKGROUND Calciphylaxis,also called calcific uremic arteriolopathy,is characterized by microvascular calcification and occlusion,which is commonly seen in patients with end-stage renal disease(ESRD).Although several studies have demonstrated an association of calciphylaxis with ESRD,reports linking calciphylaxis to LT(LT)are scarce.This report presents a rare case of calciphylaxis in a patient who underwent LT,leading to microvascular occlusion and hyperbilirubinemia.A 34-year-old man presented with a 7-day history of jaundice and severe bilateral leg pain.The patient had undergone LT and was put on hemodialysis for one year due to calcineurin inhibitor-induced ESRD.Physical examination revealed jaundice,leathery skin changes,severe muscle pain in both legs,and penile induration.Laboratory tests identified elevated bilirubin levels,gamma-glutamyl-transferase,and alkaline phosphatase,while alanine aminotransferase and aspartate aminotransferase concentrations were within normal limits.Computed tomography(CT)revealed extensive calcifications in the subcutaneous tissue.Three-dimensional CT reconstruc-tion indicated significantly reduced blood flow in the hepatic artery,primarily in the small to medium-sized branches.Contrast-enhanced ultrasonography confirmed hepatic ischemia,with no enhancement seen in hepatic artery branches.Liver biopsy specimen revealed no signs of rejection.The patient decided to receive conservative treatment and succumbed to the illness after six months.CONCLUSION This case indicates that calciphylaxis should be suspected in patients who have undergone LT with ESRD presenting with hyperbilirubinemia and skin lesions.展开更多
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,progressive disorder characterized by subcutaneous adipose and dermal microvascular calcifications,microthrombi,and endothelial damage.It mainly affects patients with chronic kidney disease(CKD...Calciphylaxis is a rare,progressive disorder characterized by subcutaneous adipose and dermal microvascular calcifications,microthrombi,and endothelial damage.It mainly affects patients with chronic kidney disease(CKD),which is also known as calcific uremic arteriolopathy.Skin biopsy is the gold standard for diagnosis,but it is an invasive procedure.Calciphylaxis frequently results in ischemic and nonhealing ulcerations with a high mortality rate.A multidisciplinary targeted approach is the primary treatment method.Vascular calcification,which is a common complication in patients with CKD,cannot completely explain the rapid progression of calciphylaxis.This article reviews the advances in the epidemiological characteristics,risk factors,and diagnosis,including non-uremic calciphylaxis and visceral calciphylaxis,pathogenesis,associated animal models,and treatment of calciphylaxis.The scarcity of animal models that mimic the clinical presentation of calciphylaxis hampers the understanding of its pathogenesis.The acute effects on progressive vascular injury,including the induction of severe ischemia and inflammatory responses,have been emphasized.Actively listening to the voices of patients and their families and building a multidimensional research system with artificial intelligence technologies based on the specific molecular makeup of calciphylaxis patients will help tailor regenerative treatment strategies.Mesenchymal stem cells(MSCs)may represent a novel therapy for calciphylaxis because of their regenerative effects,inhibition of vascular calcification,anti-infection and immunomodulation properties,and improvement of hypercoagulability.Safe,effective,accessible,and economical MSC strategies guided by biomarkers deserve consideration for the treatment of this devastating disease.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
基金Supported by the Guangzhou Basic and Applied Basic Research Foundation,No.2025A04J4730National Natural Science Foundation of China,No.82470635 and No.82300751+2 种基金Guangdong Basic and Applied Basic Research Foundation,No.2024A1515011723Medical Scientific Research Foundation of Guangdong Province of China,No.A2023033Jiangxi Provincial Natural Science Foundation,No.20244BAB28028.
文摘BACKGROUND Calciphylaxis,also called calcific uremic arteriolopathy,is characterized by microvascular calcification and occlusion,which is commonly seen in patients with end-stage renal disease(ESRD).Although several studies have demonstrated an association of calciphylaxis with ESRD,reports linking calciphylaxis to LT(LT)are scarce.This report presents a rare case of calciphylaxis in a patient who underwent LT,leading to microvascular occlusion and hyperbilirubinemia.A 34-year-old man presented with a 7-day history of jaundice and severe bilateral leg pain.The patient had undergone LT and was put on hemodialysis for one year due to calcineurin inhibitor-induced ESRD.Physical examination revealed jaundice,leathery skin changes,severe muscle pain in both legs,and penile induration.Laboratory tests identified elevated bilirubin levels,gamma-glutamyl-transferase,and alkaline phosphatase,while alanine aminotransferase and aspartate aminotransferase concentrations were within normal limits.Computed tomography(CT)revealed extensive calcifications in the subcutaneous tissue.Three-dimensional CT reconstruc-tion indicated significantly reduced blood flow in the hepatic artery,primarily in the small to medium-sized branches.Contrast-enhanced ultrasonography confirmed hepatic ischemia,with no enhancement seen in hepatic artery branches.Liver biopsy specimen revealed no signs of rejection.The patient decided to receive conservative treatment and succumbed to the illness after six months.CONCLUSION This case indicates that calciphylaxis should be suspected in patients who have undergone LT with ESRD presenting with hyperbilirubinemia and skin lesions.
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supported by the National Natural Science Foundation of China(81570666)the Outstanding Young and Middle-Aged Talents Support Program of the First Affiliated Hospital with Nanjing Medical University(Jiangsu Province Hospital)+4 种基金the Clinical Capacity Enhancement Project(JSPH-MA-2023-7)the Specialized Diseases Clinical Research Fund of Jiangsu Province Hospital(XB202403)supported by Jiangsu Provincial Medical Key Discipline(Laboratory)Cultivation Unit(JSDW202206)the ISN Clinical Research Program(18-01-0247)Key Projects of Medical Scientific Research Funded by the Health Commission of Jiangsu Province(K2024005).
文摘Calciphylaxis is a rare,progressive disorder characterized by subcutaneous adipose and dermal microvascular calcifications,microthrombi,and endothelial damage.It mainly affects patients with chronic kidney disease(CKD),which is also known as calcific uremic arteriolopathy.Skin biopsy is the gold standard for diagnosis,but it is an invasive procedure.Calciphylaxis frequently results in ischemic and nonhealing ulcerations with a high mortality rate.A multidisciplinary targeted approach is the primary treatment method.Vascular calcification,which is a common complication in patients with CKD,cannot completely explain the rapid progression of calciphylaxis.This article reviews the advances in the epidemiological characteristics,risk factors,and diagnosis,including non-uremic calciphylaxis and visceral calciphylaxis,pathogenesis,associated animal models,and treatment of calciphylaxis.The scarcity of animal models that mimic the clinical presentation of calciphylaxis hampers the understanding of its pathogenesis.The acute effects on progressive vascular injury,including the induction of severe ischemia and inflammatory responses,have been emphasized.Actively listening to the voices of patients and their families and building a multidimensional research system with artificial intelligence technologies based on the specific molecular makeup of calciphylaxis patients will help tailor regenerative treatment strategies.Mesenchymal stem cells(MSCs)may represent a novel therapy for calciphylaxis because of their regenerative effects,inhibition of vascular calcification,anti-infection and immunomodulation properties,and improvement of hypercoagulability.Safe,effective,accessible,and economical MSC strategies guided by biomarkers deserve consideration for the treatment of this devastating disease.
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.
