Cutaneous necrosis from calcific uremic arteriolopathy

Coates, Toby; Kirkland, GS; Rb, Dymock; Murphy, BF; Brealey, JK; Th, Mathew; Ap, Disney

doi:10.1053/ajkd.1998.v32.pm9740153

Cited by 304 publications

(292 citation statements)

References 27 publications

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“…Because the mineral that is deposited in these lesions is composed of calcium phosphate (15), attention has been given to disturbances of calcium and phosphorus in the development of CUA. A review of case-control series in adult patients demonstrated that hyperphosphatemia but not hypercalcemia or hyperparathyroidism was a risk factor in the development of CUA (16).…”

Section: Discussionmentioning

confidence: 99%

Sodium Thiosulfate Treatment for Calcific Uremic Arteriolopathy in Children and Young Adults

Araya¹,

Fennell²,

Neiberger³

et al. 2006

Clinical Journal of the American Society of Nephrology

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In adult patients with ESRD, calcific uremic arteriolopathy (CUA) is an uncommon but life-threatening complication. No effective therapy exists, although anecdotal case reports highlight the use of sodium thiosulfate (STS), a calcium-chelating agent with antioxidant properties. CUA is rare in children, and STS use has not been reported. The objective of this study was to determine the influence of STS treatment on three patients with CUA in a pediatric chronic dialysis unit. The patients were between 12 and 21 yr of age; two were male; and primary diagnoses were obstructive uropathy, renal dysplasia, and calcineurin nephrotoxicity. Time from ESRD to CUA diagnosis was 1, 9, and 20 yr. Diagnosis was made by tissue biopsy and three-phase bone scan. Pain was the presenting symptom. Initial treatment included discontinuation of calcitriol and use of non-calciumbased phosphate binders and low-calcium dialysate concentration. STS dosage was 25 g/1.73 m 2 per dose intravenously after each hemodialysis session. For optimization of removal of calcium deposits, patient three received a combination of STS and continuous venovenous hemofiltration for the first 10 d. All patients demonstrated rapid pain relief. Within weeks, skin induration and joint mobility of the extremities improved. Radiographic evidence of reduction in the calcium deposits occurred within 3 mo of initiation of STS. The only complication was prolonged QT interval in one patient as a result of hypocalcemia, who was resolved by use of a higher dialysate calcium concentration. STS seems well tolerated in children and young adults with CUA and has mild adverse effects. For determination of its efficacy, optimum dosage, duration of therapy, and dialysis modality, controlled trials are needed.

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Section: Discussionmentioning

confidence: 99%

Sodium Thiosulfate Treatment for Calcific Uremic Arteriolopathy in Children and Young Adults

Araya¹,

Fennell²,

Neiberger³

et al. 2006

Clinical Journal of the American Society of Nephrology

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“…1,2 Incidence has been reported to be increasing recently. 3 With a high mortality between 60 and 80%, most patients die from complications of sepsis secondary to infection of the calciphylaxis wounds, which results from impaired integrity of the epidermis and dermis. 4 The exact pathogenesis is unclear but involves vascular calcification with calcium deposition in the media of the dermal and subcutaneous arterioles and cutaneous necrosis.…”

Section: Introductionmentioning

confidence: 99%

Patient Outcomes and Factors Associated with Healing in Calciphylaxis Patients Undergoing Adjunctive Hyperbaric Oxygen Therapy

McCulloch

Wojcik

Heyboer

2015

Journal of the American College of Clinical Wound Specialists

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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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“…The original concept of calciphylaxis has markedly changed over the past few years as more cases have been reported (13). Recently, Coates et al (14) reported that the classic divalent ion abnormalities and high PTHassociated with calciphylaxis are not common.Wedescribe here a patient with end-stage renal disease secondary to SLE whoreceived administration of corticosteroids, and in whomacral gangrene and mesenteric ischemia developed without noticeable secondary hyperparathyroidism (HPT) or elevation of serum calcium-phosphorus product.…”

Section: Introductionmentioning

confidence: 99%

Calciphylaxis in a Patient with End-Stage Renal Disease Secondary to Systemic Lupus Erythematosus Associated with Acral Gangrene and Mesenteric Ischemia.

et al. 2001

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Apatient with end stage renal disease secondary to systemic lupus erythematosus (SLE) ultimately required amputation of the four extremities and developed mesenteric ischemia. The patient presented with widespread medial calcification involving various small to mediumsized arteries, although no noticeable secondary hyperparathyroidism was observed. We speculated that SLEassociated with systemic vasculitis and uremicmilieu over a numberof years may represent the perfect preexisting condition for calcific arteriolopathy to occur following which several factors including chronic administration of corticosteroids, photosensitivity in lupus, and significant weight loss may have contributed to acral gangrene and mesenteric ischemia.

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Cutaneous necrosis from calcific uremic arteriolopathy

Cited by 304 publications

References 27 publications

Sodium Thiosulfate Treatment for Calcific Uremic Arteriolopathy in Children and Young Adults

Sodium Thiosulfate Treatment for Calcific Uremic Arteriolopathy in Children and Young Adults

Patient Outcomes and Factors Associated with Healing in Calciphylaxis Patients Undergoing Adjunctive Hyperbaric Oxygen Therapy

Calciphylaxis in a Patient with End-Stage Renal Disease Secondary to Systemic Lupus Erythematosus Associated with Acral Gangrene and Mesenteric Ischemia.

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