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    Anterior Ischemic Optic Neuropathy Due to Calciphylaxis.
    J Neuroophthalmol 2017 Oct 6. Epub 2017 Oct 6.
    Departments of Ophthalmology (MSS, ØKJ), Pathology (EHS) and Dermatology (KMAE), Oslo University Hospital, Oslo, Norway.
    A 72-year-old woman experienced anterior ischemic optic neuropathy in her left eye. The funduscopic and fluorescein angiographic findings were strongly suggestive of giant cell arteritis. Temporal artery biopsy revealed extensive calcification in the vessel wall consistent with calciphylaxis. Read More

    Parathyroidectomy in Hyperparathyroidism-Associated Calciphylaxis in End-Stage Renal Disease Should be Prompt and Radical - a Case Report with Two Original Therapeutic Modifications and Successful Outcome.
    Acta Medica (Hradec Kralove) 2017 ;60(2):85-88
    Institute of Clinical Biochemistry and Diagnostics, University Hospital Hradec Králové, Czech Republic.
    We present a case of severe calciphylaxis in both thighs and calves in a patient with end-stage renal disease and advanced secondary hyperparathyroidism with successful outcome after modified therapeutic approach. The cause of calciphylaxis is multifactorial. In our case, not only severe hyperparathyroidism and mediocalcinosis, but also medication (warfarin, calcium and active vitamin D) was involved. Read More

    Extraosseous Calcification of the Esophagus: Clinicopathologic Correlates of Esophageal Mucosal Calcinosis.
    ACG Case Rep J 2017 13;4:e108. Epub 2017 Sep 13.
    Department of Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH.
    Esophageal mucosal calcinosis (EMC) is a rare cause of dysphagia with high morbidity. We present a patient who experienced melena and 3 months of solid and liquid dysphagia along with bilateral lower extremity pain, erythema, and edema later determined to be calcific uremic arteriolopathy (CUA), or calciphylaxis. An esophagogastroduodenoscopy revealed nodularity and linear ulcerations in the upper third of the esophagus. Read More

    Seeing the difference-Painless progressive vision loss in a vasculopath.
    Surv Ophthalmol 2017 Sep 18. Epub 2017 Sep 18.
    Save Sight Institute, University of Sydney, Sydney, NSW, Australia.
    A 78-year-old Caucasian man with significant vascular disease reported sudden onset of worsened vision during a routine wound-care appointment for nonhealing necrotic leg ulcers. He described painless blurring of vision with grey scotomas in his right eye, his only well-seeing eye, after trauma to the left eye as a child. He presented with retinal ischemia, a cotton wool spot, and optic nerve swelling. Read More

    Evaluation of osteopontin expression in chronic wounds: a potential prognostic and therapeutic biomarker.
    J Wound Care 2017 Sep;26(Sup9):S4-S8
    Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida, US.
    Objective: Osteopontin (OPN) is abundantly expressed during tissue repair, acting as a powerful chemokine that recruits inflammatory cells such as neutrophils, macrophages, and Langerhans cells. The role of OPN in chronic wounds has not been explored. In this study, we assess the expression levels of OPN in chronic wounds to assess its potential contribution to the exacerbated inflammation seen in chronic ulcers, which is thought to contribute to poor healing. Read More

    Sodium Thiosulfate in the Treatment of Lung and Breast Calciphylaxis: CT and Bone Scintigraphy Findings.
    Clin Nucl Med 2017 Nov;42(11):893-895
    From the Department of Nuclear Medicine, West China Hospital of Sichuan University, Chengdu, Sichuan, People's Republic of China.
    A 19-year-old woman, with normal kidney function, was diagnosed as having visceral calciphylaxis, especially diffuse breast and lung calcification. The calcification findings were clearly shown on CT, and bone scintigraphy revealed diffuse uptake in the thoracic area. Although there was no standard therapeutic approach for this clinical setting, the patient received empiric therapy with sodium thiosulfate. Read More

    Pseudoxanthoma elasticum-like changes in nonuremic calciphylaxis: Case series and brief review of a helpful diagnostic clue.
    J Cutan Pathol 2017 Sep 4. Epub 2017 Sep 4.
    Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida.
    Calciphylaxis is a rare syndrome of vascular calcification with thrombosis that occurs most often in patients with end-stage renal disease, and it frequently portends a guarded prognosis. Rarely, nonuremic calciphylaxis (NUC) may occur; in this context, a strongly supportive histology is crucial in establishing the diagnosis. Herein, we describe 2 cases of NUC associated with pseudoxanthoma elasticum-like changes, identified in both initial nondiagnostic and subsequent diagnostic biopsy specimens. Read More

    Direct oral anticoagulant medications in calciphylaxis.
    Int J Dermatol 2017 Oct 30;56(10):1065-1070. Epub 2017 Aug 30.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Background: Recent studies suggest that calciphylaxis is a thrombotic condition in which arteriolar thrombosis leads to painful skin infarcts and consequent morbidity and mortality. Paradoxically, warfarin is implicated as a risk factor for calciphylaxis. Our objective is to report the use of oral direct thrombin and factor Xa inhibitors (termed direct oral anticoagulants [DOACs]) in patients with calciphylaxis. Read More

    Calciphylaxis of the Postmenopausal Female Breast: An Uncommonly Encountered Mimic of Carcinoma.
    Case Rep Pathol 2017 9;2017:4541620. Epub 2017 Aug 9.
    Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
    Calciphylaxis is a serious medical condition that is typically associated with end-stage renal disease and presents as the sequelae of calcifications in arterioles with subsequent ischemia of affected tissues. Classically, calciphylaxis produces ulcerated and necrotic skin lesions. These may be biopsied to aid in confirmation of the diagnosis. Read More

    Vasculopathies, cutaneous necrosis and emergency in dermatology.
    G Ital Dermatol Venereol 2017 Jul 28. Epub 2017 Jul 28.
    Dermatology Department, Hospital Universitario de La Princesa, Madrid, Spain.
    The most of the emergency in dermatology comprise a variety of entities that most are benign in course. However the vasculopathies and vasculitis are not common,but they could represent the 1,9% and 4,4% according to some studies of emergency dermatology department. They become an important diseases which have to identified early to established an appropiate managment and treatment. Read More

    Medicina (B Aires) 2017 ;77(4):331-333
    Departamento de Ortopedia y Traumatología, Hospital Universitario, Fundación Favaloro, Buenos Aires, Argentina.
    Calciphylaxis is vasculopathy characterized by ischemia and painful skin necrosis due to calcification and intimal fibroplasia of thrombosis of the panicular arterioles. It most frequently compromises patients with terminal chronic renal failure and has a high mortality rate. Biopsy of skin lesions is used as a diagnostic method. Read More

    Calciphylaxis with extensive arterial calcification.
    Clin Case Rep 2017 Aug 3;5(8):1418-1419. Epub 2017 Jul 3.
    Division of Nephrology, Hypertension and Renal TransplantationUniversity of FloridaGainesvilleFlorida.
    Calciphylaxis is a clinical syndrome of arteriolar calcification with resultant tissue necrosis, most commonly seen in patients with end-stage renal disease (ESRD) on dialysis. This condition carries a high mortality rate mainly due to infection. Helpful interventions include adequate dialytic therapy, medical management of hyperparathyroidism, hyperphosphatemia, and intravenous sodium thiosulfate therapy. Read More

    Efficacy of intralesional sodium thiosulfate injections for disabling tumoral calcinosis: Two cases.
    Semin Arthritis Rheum 2017 Jun 17. Epub 2017 Jun 17.
    Service de pédiatrie, Hopital de la Mère et de l'Enfant, Limoges, France. Electronic address:
    Introduction: Tumoral calcinosis (TC) is a difficult-to-treat complication that can occur during several diseases such as dermatomyositis or genetic hyperphosphatemia. It is a painful and disabling condition that can lead to local complications including joint mobility reduction, cutaneous ulceration and superinfection. For the largest lesions, the treatment relies essentially on surgery. Read More

    Gastric Pneumatosis from Isolated Gastric Calciphylaxis.
    ACG Case Rep J 2017 19;4:e91. Epub 2017 Jul 19.
    Department of Gastroenterology, University of Cincinnati Medical Center, Cincinnati, OH.
    A 58-year-old man with end-stage renal disease presented with hypotension and emesis, pale conjunctivae, and a distended abdomen. Labs revealed hypercalcemia and leukocytosis. Abdominal imaging showed gastric pneumatosis. Read More

    A novel pharmacodynamic assay to evaluate the effects of crystallization inhibitors on calcium phosphate crystallization in human plasma.
    Sci Rep 2017 Jul 31;7(1):6858. Epub 2017 Jul 31.
    Laboratoris Sanifit SL, 07121, Palma, Spain.
    Cardiovascular calcification (CVC) is a progressive complication of chronic kidney disease and a predictor of CV events and mortality. The use of biomarkers to predict CV risk and activities of potential or current treatment drugs in these patients could have a crucial impact on therapeutic approaches. Our aim was to develop a novel assay for measurement of the rate of calcium phosphate crystallization in human plasma and provide a tool to evaluate the effects of crystallization inhibitors. Read More

    Iron-based phosphate binders: a paradigm shift in the treatment of hyperphosphatemic anemic CKD patients?
    J Nephrol 2017 Jul 17. Epub 2017 Jul 17.
    Department of Nephrology and Dialysis, Alessandro Manzoni Hospital, ASST Lecco, Via dell'Eremo 9/11, 23900, Lecco, Italy.
    The partial correction of anemia and the normalization of phosphate and blood pressure are the mainstay of treatment of patients with chronic kidney disease (CKD). Available anti-hypertensive drugs, erythropoiesis stimulating agents (ESAs) and iron supplements have resolved quite satisfactorily the goal of controlling hypertension and partially correcting anemia. Unfortunately, the treatment of hyperphosphatemia is still far from resolved. Read More

    Management of a Complex Peristomal Calciphylaxis: A Case Study.
    J Wound Ostomy Continence Nurs 2017 Jul/Aug;44(4):380-383
    Mary Famorca, MAN, RN, WCC, COCN, Mayo Clinic Arizona, Phoenix, Arizona. Debra Beauchaine, MN, RN, AGPCNP, CWOCN-AP, Mayo Clinic Arizona, Phoenix, Arizona. Nancy Angulo, BS, RN, CWOCN, Cancer Treatment Center of America, Goodyear, Arizona.
    Background: Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare and serious syndrome of small blood vessels with a high mortality rate. Calciphylaxis lesions require intensive wound management with medical interventions for the patient to survive the sequelae of sepsis and present unique challenges when found in the peristomal skin.

    Case: A 33-year-old man presented with multiple malodorous stage 4 pressure injuries of his sacrococcygeal and gluteal area. Read More

    [Chronic leg ulcers].
    Hautarzt 2017 Aug;68(8):614-620
    Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Essen, Hufelandstr. 55, 45122, Essen, Deutschland.
    Chronic wounds often manifest as ulcers on the lower legs. The main causes of chronic leg ulcers are chronic venous insufficiency and/or peripheral arterial occlusive disease in approximately 80% of all patients. However, there are also numerous other potentially relevant factors which can cause or almost impede the healing of chronic leg ulcers. Read More

    A systematic review of sodium thiosulfate in treating calciphylaxis in chronic kidney disease patients.
    Nephrology (Carlton) 2017 Jun 11. Epub 2017 Jun 11.
    Renal Division and Institute of Nephrology, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu.
    Background: Calciphylaxis is a severe complication of advanced chronic kidney disease (CKD). Sodium thiosulphate (STS), an antioxidant and calcium chelating agent, has been used for the treatment of calciphylaxis. However, its efficacy and safety have not been systematically analysed and evaluated. Read More

    Calciphylaxis in the Setting of Alcoholic Cirrhosis: Case Report and Literature Review.
    J Investig Med High Impact Case Rep 2017 Apr-Jun;5(2):2324709617710039. Epub 2017 May 24.
    Advocate Christ Medical Center, Oak Lawn, IL, USA.
    Calciphylaxis can be a severe life-threatening dermatologic disease that is a known complication associated with end-stage renal disease. However, multiple non-uremic etiologies that are not yet well studied can cause calciphylaxis. We report a rare care of a 40-year-old female with history of alcoholic cirrhosis without any evidence of renal dysfunction who presents with calciphylaxis. Read More

    Underreported use of palliative care and patient reported outcome measures to address reduced Quality of Life in calciphylaxis patients: A systematic review.
    Br J Dermatol 2017 Jun 5. Epub 2017 Jun 5.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Calciphylaxis is associated with significant morbidity and mortality. Palliative care (PC) is a subspecialty that treats the pain and stress of serious illness. We performed a systematic review to assess whether quality of life (QoL) indices and the role of palliative care have been studied in calciphylaxis patients. Read More

    The Role of Bone Scintigraphy with Single-photon Emission Computed Tomography-computed Tomography in the Diagnosis and Evaluation of Calciphylaxis.
    World J Nucl Med 2017 Apr-Jun;16(2):172-174
    Department of Nuclear Medicine, University of Ottawa, Ottawa, Ontario, Canada.
    Calciphylaxis, also known as calcific uremic arteriolopathy, is an uncommon disease, typically found in patients with the end-stage renal disease. Pathophysiological features include small vessel vasculopathy with mural calcification, fibrosis, and thrombosis. The clinical presentation varies but often consists of the necrosis of skin and subcutaneous tissues, with or without visceral organ involvement due to small vessel vasculopathy. Read More

    Acenocoumarol as a risk factor for calciphylaxis: a feature clinicians should be aware of.
    Neth J Med 2017 May;75(4):161-164
    Department of Internal Medicine, Catharina Hospital Eindhoven, Eindhoven, the Netherlands.
    In contrast with uraemic calciphylaxis in end-stage renal disease, causes of and risk factors for non-uraemic calciphylaxis are relatively unknown to clinicians and have yet to become fully established. This report describes a case of non-uraemic calciphylaxis, in which the use of acenocoumarol might have been a risk factor. It is important to raise awareness about this association among clinicians, as vitamin K antagonists have to be stopped for an optimal treatment of this severe condition. Read More

    Immune thrombocytopenic purpura masking the fatal potential of calciphylaxis in a haemodialysis patient.
    CEN Case Rep 2016 May 20;5(1):70-73. Epub 2015 Aug 20.
    Nephrology Unit, Department of Medicine, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Serdang, Malaysia.
    Calciphylaxis on the background of immune thrombocytopenic purpura (ITP) has never been described. The pathogenesis of calciphylaxis is complex and not fully understood as yet. ITP has a complex pathogenesis that leads to bleeding or thrombotic events. Read More

    Penile Calciphylaxis: The Use of Radiological Investigations in the Management of a Rare and Challenging Condition.
    Urol Case Rep 2017 Jul 11;13:113-116. Epub 2017 May 11.
    The James Buchanan Brady Urological Institute and Department of Urology, The Johns Hopkins School of Medicine, 600 North Wolfe Street, Baltimore, MD 21287, USA.
    Penile calciphylaxis is a rare phenomenon of penile necrosis observed in patients with hemodialysis-dependent end-stage renal failure. Multiple treatments have been proposed including conservative management, surgical debridement and penectomy; yet, the prognosis remains extremely poor. Here, we describe a patient with protracted resolution of dry gangrene of the glans, which failed conservative management of wound care and pain management. Read More

    Calciphylaxis in hemodialysed patients: Diagnostic value of calcifications in cutaneous biopsy.
    Br J Dermatol 2017 May 12. Epub 2017 May 12.
    Department of Dermatology, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
    Calciphylaxis or calcific uremia arteriopathy (CUA) is a rare and highly morbid condition that predominantly affects dialyzed end-stage renal disease (ESRD) patients(1) . One-year survival rate is estimated to be 40-50%, with sepsis emerging as the leading cause of death (2) . Calciphylaxis presents with painful necrotic cutaneous ulcers, livedo racemosa, hemorrhagic patches, indurated plaques, or hemorrhagic bullae predominantly on the proximal lower extremities and trunk (2) . Read More

    Acute Cutaneous Necrosis: A Guide to Early Diagnosis and Treatment.
    J Cutan Med Surg 2017 Sep/Oct;21(5):425-437. Epub 2017 May 4.
    4 University of Missouri Kansas City, Department of Internal Medicine, Kansas City, MO, USA.
    Acute cutaneous necrosis is characterised by a wide range of aetiologies and is associated with significant morbidity and mortality, warranting complex considerations in management. Early recognition is imperative in diagnosis and management of sudden gangrenous changes in the skin. This review discusses major causes of cutaneous necrosis, examines the need for early assessment, and integrates techniques related to diagnosis and management. Read More

    Early clinical presentations and progression of calciphylaxis.
    Int J Dermatol 2017 Aug 23;56(8):856-861. Epub 2017 Apr 23.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
    Background: Untreated calciphylaxis is a fatal disease of intra- and extravascular calcification, most commonly presenting in end-stage renal disease (ESRD) patients. While early identification is critical for timely treatment, early-stage clinical and histopathological descriptions have not, to our knowledge, been elucidated. As early clinical recognition is essential to prompt definitive histopathological diagnosis, this study describes a range of clinical and histopathological manifestations of early-stage calciphylaxis. Read More

    A case report of severe calciphylaxis - suggested approach for diagnosis and treatment.
    BMC Nephrol 2017 Apr 21;18(1):137. Epub 2017 Apr 21.
    Department of Nephrology and Hypertension, Hannover Medical School, Carl-Neuberg-Straße 1, 30635, Hannover, Germany.
    Background: Calciphylaxis is a serious complication in patients with chronic kidney disease associated mineral and bone disorder. It can occur in conditions with low and high bone turnover. So far, there are no definite diagnostic and therapeutic guidelines which may prevent the devastating outcome in many calciphylaxis patients. Read More

    Calciphylaxis Presenting with Various Symptoms: A Case Report.
    Case Rep Dermatol 2017 Jan-Apr;9(1):25-29. Epub 2017 Feb 8.
    Department of Dermatology, School of Medicine, Iwate Medical University, Morioka, Japan.
    Calciphylaxis causes ischemia in multiple organs and skin ulcers owing to progressive calcification in small and medial arteries. It has a poor prognosis and often occurs in patients with hyperparathyroidism associated with end-stage renal failure and those undergoing hemodialysis. Here, we present a case of calciphylaxis associated with a wide range of symptoms, including lower thigh skin ulcers, a rectovaginal fistula, and femoral neck fracture. Read More

    Calciphylaxis: Beyond CKD-MBD.
    Nefrologia 2017 Sep - Oct;37(5):501-507. Epub 2017 Apr 5.
    Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
    Introduction: Calcific uraemic arteriolopathy (CUA), also called calciphylaxis, is a rare but potentially fatal vascular disorder that almost exclusively affects patients with chronic renal failure. The objective of this study was to analyse various risk factors for developing CUA and its subsequent clinical course according to the treatment received.

    Materials And Methods: A retrospective study that included patients diagnosed with CUA from December 1999 to December 2015. Read More

    Curr Opin Nephrol Hypertens 2017 Jul;26(4):276-281
    Division of Nephrology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA.
    Purpose Of Review: Calciphylaxis remains a poorly understood vascular calcification disorder with predilection for patients with end-stage renal disease (ESRD). Recent data from large patient registries and databases have begun to provide information regarding incidence, risk factors, and outcomes in patients with calciphylaxis.

    Recent Findings: The most recent estimate places the incidence of calciphylaxis at 3. Read More

    Benefits of intralesional injections of sodium thiosulfate in the treatment of calciphylaxis.
    Int Wound J 2017 Mar 21. Epub 2017 Mar 21.
    Department of Dermatology, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland.
    Calciphylaxis (CPX) is a rare calcifying thrombotic vasculopathy responsible for painful necrotic ulcers, with a high mortality rate, and its management is often difficult. Recently, intravenous infusions of sodium thiosulfate (STS) have shown efficacy. The aim of this study was to assess the efficacy of intralesional STS (IL STS) in four patients. Read More

    Metaplastic ossification of the temporal artery with osteoclast-like giant cells: a mimicker of giant cell (temporal) arteritis.
    Eur J Ophthalmol 2017 May 11;27(3):e99-e103. Epub 2017 May 11.
    Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY - USA.
    Purpose: To describe a patient presenting with suspected giant cell (temporal) arteritis (GCA) in whom subsequent temporal artery biopsy showed luminal narrowing by medial calcification, metaplastic ossification, and fibrointimal proliferation, consistent with calciphylaxis.

    Methods: A 55-year-old man with end-stage renal disease presented with unilateral loss of vision and elevated erythrocyte sedimentation rate and was initially treated as though he had GCA; however, a subsequent temporal artery biopsy showed marked luminal narrowing by medial calcification, metaplastic ossification, and fibrointimal proliferation, consistent with calciphylaxis. In addition, the tunica media of the affected artery contained multinucleate giant cells, but these represented osteoclasts and foreign body giant cells reacting to calcium, rather than a part of GCA. Read More

    Calciphylaxis: Comparison of radiologic imaging and histopathology.
    J Am Acad Dermatol 2017 Aug 9;77(2):241-246.e3. Epub 2017 Mar 9.
    Department of Department of Hyperbaric Medicine and Wound Healing, Greenwich Hospital, Greenwich, Connecticut.
    Background: The current gold standard for diagnosis of calciphylaxis is a skin biopsy specimen demonstrating calcification of small-caliber arteries or arterioles.

    Objective: The aim of this study is to compare diameters of calcified vessels seen in skin biopsy specimens and radiology images of patients with calciphylaxis.

    Methods: We conducted a retrospective study of patients with known calciphylaxis from 2009 to 2016 at a community hospital who had both skin biopsy specimens and radiology images taken as part of their routine care. Read More

    Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis.
    J Eur Acad Dermatol Venereol 2017 Mar 2. Epub 2017 Mar 2.
    Department of Dermatology, Universitätsklinikum Erlangen, Erlangen, Germany.
    Background: Livedoid vasculopathy and calciphylaxis are rare skin disorders. Large cohorts of patients have been missing so far for detailed analysis.

    Patients And Methods: Data from diagnosis-related groups (DRGs) of hospitalized cases of livedoid vasculopathy (ntotal = 1357) and calciphylaxis (ntotal = 699) were analysed for the years 2008-2013 concerning sex, age and frequency of diagnosis. Read More

    Calciphylaxis on bone scan: correlation between molecular and cross-sectional findings.
    Radiol Case Rep 2017 Mar 24;12(1):175-178. Epub 2016 Dec 24.
    Department of Radiology, MSC 10-5530, University of New Mexico, 1 University of New Mexico, Albuquerque, NM 87131, USA.
    Calciphylaxis is a rare devastating medical condition commonly associated with end-stage renal disease and characterized by extensive microvascular calcifications. We describe a case of calciphylaxis presenting on Tc-99m MDP bone scan imaging with asymmetric radiotracer uptake within the lower extremities corresponding to extensive soft tissue calcifications on Computed tomography. Familiarity with the classic clinical presentation and imaging features of this rare entity may help its early identification and treatment. Read More

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