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Calciphylaxis or calciphic uremic arteriolopathy (CUA) is a rare syndrome characterized by the deposition of calcium within the walls of small and medium size vessels in the dermis and in the subcutaneous tissue. The disease mainly affects patients with end-stage renal disease. We report here our experience with 4 cases of calciphylaxis in dialysis patients. The main predisposing factor observed in our 4 patients was warfarin use (2 patients, 50%), while local traumas and diabetes were respectively present in only one patient. None of our patients was obese. Lower legs were the most frequently involved site of CUA (3/4 patients, 75%). In our experience biopsy was crucial to achieve a correct diagnosis and did not cause aggravation of the ulcers. Therapeutic approach was multimodal: mainly hyperbaric oxygen therapy, cinacalcet and sodium thiosulphate. Although many recent case reports have shown exceptional results and healing with the use of sodium thiosulphate, we did not experience any change in the poor prognosis of our patients with the use of this drug, at a dosage of 5 g thrice weekly endovenously.
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PMID:Calciphylaxis in dialysis patients, a severe disease poorly responding to therapies: report of 4 cases. 2400 47

Calciphylaxis causes calcification, thrombosis, cutaneous ischemia, and necrosis in the skin and subcutaneous tissue. It is unclear to what extent it involves other organs. To identify whether other organs are affected we reviewed pathology reports of patients with calciphylaxis who underwent autopsy at Mayo Clinic, Rochester, Minnesota, between January 1, 1970, and December 31, 2011. Three patients were identified: two patients had a diagnosis of end-stage renal disease secondary to diabetes mellitus before the diagnosis of calciphylaxis; the third patient had calciphylaxis associated with metastatic cholangiocarcinoma without end-stage renal disease. Autopsy reports showed that despite evidence of vessel calcification elsewhere, there was no evidence of calciphylaxis in other organs. All patients had histopathologic evidence of cardiovascular calcification, and atherosclerosis of coronary arteries and aorta. Calcification of pancreatic vessels and renal vessels was also noted. In this study population, calciphylaxis was a cutaneous process alone.
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PMID:Calciphylaxis is a cutaneous process without involvement of internal organs in a retrospective study of postmortem findings in three patients. 2409 24

Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.
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PMID:Temporal Artery Calciphylaxis Presenting as Temporal Arteritis in a Case of Rhinoorbitocerebral Mucormycosis. 2485 18

Calciphylaxis is characterized by calcification and thrombosis of arteries resulting in ischemic necrosis of predominantly skin and subcutaneous tissue. Primarily affecting patients with end-stage renal disease, calciphylaxis is diagnosed rarely in the absence of renal replacement therapy. We report an elderly obese woman presented with leg pain and ulceration. She had chronic kidney disease, diabetes mellitus, hypertension, and peripheral vascular disease. Angiography revealed occlusion of the left superficial femoral, popliteal, and distal tibial arteries. Amputation was performed. Histological examination demonstrated medial calcification, intimal hyperplasia, and thrombosis of small- and medium-sized arteries in the subcutaneous tissue. This case features calciphylaxis in a patient with chronic kidney disease before the onset of uremia. Calciphylaxis and atherosclerotic peripheral vascular disease have several risk factors in common. This report calls attention to a disorder that can be masqueraded as leg ulceration due to peripheral vascular disease in the absence of renal replacement therapy.
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PMID:Pre-uremic calciphylaxis. 2487 53

Calciphylaxis or calcific arteriolopathy is a rare, life-threatening obstructive pathology of the small cutaneous and subcutaneous vessels. It mainly affects patients with chronic renal failure but it also has been described in patients with normal renal function. The principal risks factors apart from renal failure and phosphocalcic metabolism imbalance are: the female sex, obesity, peripheral vascular disease, diabetes and oral anti-coagulation. We present a very rare case of abdominal, mammarian and upper thighs calciphylaxis in a patient with normal renal function. She presented a severe obesity with a recent important loss of weight and had been treated by oral anticoagulants for a long time. She benefited of a multidisciplinary approach with dermatologists, plastic surgeons and anesthesists permitting a recovery in fourteen weeks. Multidisciplinary approach is necessary but the place of the surgery is not well defined. We report a case in which early and wide surgical approach permitted to obtain a favourable evolution of the pathology. Then, we propose a therapeutic strategy after review of the literature.
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PMID:[Massive panniculectomy and bilateral subtotal mastectomy in a case of calciphylaxis: A case report and up date]. 2579 28

Calciphylaxis is characterized by abnormal calcification of vessels and skin; however, its aetiology and pathogenesis remain unclear. Entities frequently associated with calciphylaxis are end-stage renal disease, diabetes mellitus, hypercalcaemia, hyperphosphataemia, elevated calcium-phosphate product, hyperparathyroidism and possible hypercoagulable states. Skin lesions may remain quiescent or may develop suddenly and progress rapidly. They are more common on the legs. Treatment of calciphylaxis is very challenging and requires interdisciplinary management. We present a case that highlights the difficulty of treating calciphylaxis. A multidisciplinary approach was vital for the proper treatment of our patient. This case also demonstrates the importance of searching for underlying hypercoagulable states, especially in recalcitrant cases. In cases of calciphylaxis with vessel occlusion from microthrombi, heparin therapy would be a logical next step. The effect of anticoagulation may be rapid and impressive.
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PMID:Calciphylaxis with evidence of hypercoagulability successfully treated with unfractionated heparin: a multidisciplinary approach. 2629 Jan 27

BACKGROUND Calciphylaxis is associated with a high mortality that approaches 80%. The diagnosis is usually made when obvious skin lesions (painful violaceous mottling of the skin) are present. However, visceral involvement is rare. We present a case of calciphylaxis leading to lower gastrointestinal (GI) bleeding and rectal ulceration of the GI mucosa. CASE REPORT A 66-year-old woman with past medical history of diabetes mellitus, hypertension, end-stage renal disease (ESRD), recently diagnosed ovarian cancer, and on hemodialysis (HD) presented with painful black necrotic eschar on both legs. The radiograph of the legs demonstrated extensive calcification of the lower extremity arteries. The hospital course was complicated with lower GI bleeding. A CT scan of the abdomen revealed severe circumferential calcification of the abdominal aorta, celiac artery, and superior and inferior mesenteric arteries and their branches. Colonoscopy revealed severe rectal necrosis. She was deemed to be a poor surgical candidate due to comorbidities and presence of extensive vascular calcifications. Recurrent episodes of profuse GI bleeding were managed conservatively with blood transfusion as needed. Following her diagnosis of calciphylaxis, supplementation with vitamin D and calcium containing phosphate binders was stopped. She was started on daily hemodialysis with low calcium dialysate bath as well as intravenous sodium thiosulphate. The clinical condition of the patient deteriorated. The patient died secondary to multiorgan failure. CONCLUSIONS Calciphylaxis leading to intestinal ischemia/perforation should be considered in the differential diagnosis in ESRD on HD presenting with abdominal pain or GI bleeding.
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PMID:Gastrointestinal Bleeding Secondary to Calciphylaxis. 2657 38

Calciphylaxis is characterized by calcification in the medium and small vessel arterioles and can be a life-threatening complication often associated with chronic kidney disease (CKD). A review of the literature was conducted to explore existing evidence about the relationship between obesity and calciphylaxis. A total of 54 publications (published between 1962 and 2015) were identified. Most studies noted a variety of risk factors for calciphylaxis, including CKD, female gender, Caucasian race, liver disease, and lower serum albumin. Obesity was identified as a risk factor in 6 of the 8 studies reviewed. In one study, obesity was found to increase the risk of calciphylaxis 4-fold. The majority of calciphylaxis lesions in obese persons were proximal in distribution; all studies report proximal lesions are associated with a higher mortality rate than distal lesions. The mortality rate of persons with CKD and calciphylaxis is 8 times higher than that of persons with CKD without calciphylaxis. There is no definitive evidence to support the belief current epidemic rates of obesity, diabetes, (diabesity), and chronic renal disease will predispose more patients to the development of calciphylaxis. However, until more information from the calciphylaxis registries and other studies is available, clinicians should maintain a high index of suspicion when a patient presents with indurated, painful nodules or necrotic ulcers, especially if the patient also has CKD.
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PMID:The Relationship Between Obesity and Calciphylaxis: A Review of the Literature. 2677

Calcific uremic arteriolopathy, also known as calciphylaxis, is a rare syndrome of small vessel calcification of unknown etiology causing painful, violaceous skin lesions that progress to form chronic non-healing ulcers and gangrene. Hyperbaric oxygen therapy (HBOT) can be used as adjunctive therapy in the treatment of these ulcers. However, due to paucity of cases, there is limited data on the clinical benefit of HBOT and identifying factors associated with healing. The purpose of this study was to determine patient outcomes and factors associated with healing in patients with calciphylaxis undergoing HBOT. A retrospective chart review was completed on patients who were diagnosed with calciphylaxis and had hyperbaric medicine consultation between May 2012 and January 2016. Clinical outcomes, demographics, risk factors, laboratory values, wound distribution, and HBOT profiles were collected and analyzed. We identified 8 patients. Out of 8 patients consulted for calciphylaxis, five were consented and underwent HBOT (2 males and 3 females). All had coexisting ESRD and Diabetes. All males were able to tolerate being in the chamber and received therapeutic treatments (at least 20 HBOT) with complete resolution of ulcers. HBOT was discontinued in one female due to an inconsistent biopsy report and two others due to death secondary to septic shock or respiratory arrest and severe uremia. Calciphylaxis is a devastating disease with a high mortality rate. Our results demonstrated a positive response to HBOT especially when receiving at least 20 treatments. A majority of calciphylaxis cases are females and indeed female gender has been cited as a risk factor for this disease. However, current literature has not conferred a relationship between gender nor the number of HBOT received and outcomes. Our results showed that males had a more favorable outcome provided they received at least twenty HBOT. Further prospective studies are needed to elucidate these outcomes.
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PMID:Patient Outcomes and Factors Associated with Healing in Calciphylaxis Patients Undergoing Adjunctive Hyperbaric Oxygen Therapy. 2805 62

Calciphylaxis is a major cause of morbidity and mortality in end-stage renal disease (ESRD). Intravenous sodium thiosulfate (STS) is the mainstay of therapy for calciphylaxis. In peritoneal dialysis (PD) patients with calciphylaxis, intravenous STS poses logistic and financial challenges. Even though pharmacokinetic studies show poor bioavailability of oral STS, we report successful use of oral STS in 2 PD patients with calciphylaxis.A 55-year-old Latina American woman with diabetes was initiated on PD after access failure and chronic hypotension. She developed painful ischemic lesions in the left middle finger and left big toe 4 months later. The ischemia in the left hand progressed, requiring amputation of two fingers. She later developed extensive painful calcific areas in the abdominal wall. She was initially started on oral STS 1500 mg twice daily that was subsequently increased to 3750 mg daily, which resulted in substantial pain relief and a decrease in the size of the calcific plaques.Another diabetic patient with ESRD who was on PD presented with a painful ischemic finger for 2 years. He was treated with oral STS 1500 mg twice daily, resulting in prompt pain relief.Oral STS can be an effective treatment for calciphylaxis.
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PMID:Treatment of Calciphylaxis: A Case for Oral Sodium Thiosulfate. 2898 90


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