Non-uremic calciphylaxis associated with alcoholic hepatitis: A case report

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.

Calciphylaxis是误称,“钙化防御”是误译

本综述解释了calciphylaxis一词起源于1961年皮肌炎的动物实验模型,后来被借用过来以解释肾病患者的钙化现象。由于两者发病机制和病理的不同,显然把calciphylaxis用在肾病患者就是误称(misnomer)。于是就引入了钙化性尿毒症性细动脉病(calcific uremic arteriolopathy,CUA)以描述肾病患者的这一综合征。但是它也可以发生于肾功能正常的人,故CUA也不大妥。所以,最近我们提出钙化性细动脉病(calcific arteriolopathy)一词,以更准确地描述这一临床综合征。同时介绍实际工作中诊断calciphylaxis的体会。还简要提及一个在许多国家不大提到的Martorell HYTILU这一诊断,及其与calciphylaxis的关系。重点指出,calciphylaxis是由calcium(钙)和anaphylaxis(严重过敏反应)这两个词组合而来。而“钙化防御”(“calcification defense”)显然是来源于calcium(钙)和prophylaxis(预防)的组合。也就是既往的译者把calciphylaxis所含的anaphylaxis误解为prophylaxis。因此,把calciphylaxis改成calcific arteriolopathy并译成钙化性细动脉病,可能是更好的选择。

A Case Report: Calciphylaxis Presenting as Digital Ischemia in Patient with End Stage Kidney Disease on Peritoneal Dialysis

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.

Calcific Uremic Arteriolopathy or Calciphylaxis in a Hemodialysis Patient: Case Study and Review of the Literature

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.

A case of calciphylaxis and acute myeloid leukemia: A previously unreported association

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.

Calciphylaxis in Patients with Chronic Kidney Disease Case Presentation and Review

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.

A novel long-term intravenous combined with local treatment with human amnion-derived mesenchymal stem cells for a multidisciplinary rescued uremic calciphylaxis patient and the underlying mechanism

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.