Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): A complication of chronic renal failure and benefit from parathyroidectomy

Hafner, Jürg; Keusch, G; Wahl, Christoph; Sauter, Bernhard; Hürlimann, Andreas; Weizsäcker, Fritz von; Krayenbühl, Michèle; Biedermann, K; Brünner, U; Helfenstein, Ulrich; Burg, Günter

doi:10.1016/0190-9622(95)90286-4

Cited by 244 publications

(153 citation statements)

References 42 publications

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“…The ulcerative lesions become infected, causing septicemia and death. In patients with renal disease, the lesions can be proximal (involving abdomen, thighs, and buttocks) or distal (primarily involving the calves), with the latter having a better prognosis (11). To avoid the introduction of nonhealing wounds, not all cases of calciphylaxis undergo biopsy.…”

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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“…The effectiveness of parathyroidectomy is controversial according to the literature. Some of the studies and case reports support the beneficial effects but others do not (14,21,25,31,32). The significant regression of the calcification in the six months after the operation indicates that hyperparathyroidism could have played a major role in systemic CUA and valvular calcification in this case.…”

Section: Treatment Strategies Of Cuamentioning

confidence: 59%

“…iron therapy, vitamine D analogs, and systemic glucocorticoids, and hypoalbuminemia (14,(20)(21)(22)(23). The dialysis exposure and longer duration on dialysis treatment are important facilitators for CUA although CUA can develop in non-CKD patients (9).…”

Section: Türk Nefroloji Diyaliz Ve Transplantasyon Dergisi Turkish Nementioning

confidence: 99%

“…However there are some described strategies in literature such as aggressive wound care (if necessary debridement may be performed), avoidance of subcutaneous and muscular injections, oxygen therapy (10-15 L, two hours/day or 2.5 atm for 90 minutes/day), maintaining calcium, phosphorus, CaxP (<55 mg 2 /dL 2 ) and PTH levels (>300 pg/dL, cinacalcet or surgery) in the target range, intravenous sodium thiosulfate (25 gr, diluted in 100 mL of physiological saline solution and administered over 30 to 60 minutes, three times a week until complete wounds healing is achieved), increased HD dose using low dialysate calcium (1-1.5 mmol/L) and stopping or switching the drugs that affect the calcium-phosphorus metabolism (14,21,(25)(26)(27)(28)(29)(30). Vitamin K, denosumab, vitamin D receptor agonists, teriparatide, avoiding zinc deficiency and bisphophonates may be other potential alternatives although more relevant data are required for routine use (7).…”

Section: Treatment Strategies Of Cuamentioning

confidence: 99%

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Endocarditis and Calcific Uremic Arteriolopathy: Is it a Coincidence or an Association? Case Report

Kurultak¹,

Kılıç²,

Sezer³

et al. 2016

Turk Neph Dial Transpl

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The uncommon complications regarding CKD tend to increase in clinical setting because of the chronic kidney disease (CKD) population has been expanding every passing day. Perhaps the most important one of these is calcific uremic arteriolopathy (CUA, calciphylaxis). Calcific uremic arteriolopathy is a clinical syndrome which includes systemic calcification of the small vessels, causes to ischemia, subcutaneous necrosis and it has high mortality and morbidity rates (1,2). There is a large body of data in literature about CUA although very little for its cardiac involvement. Herein we presented a case of calcific endocarditis with CUA and the review of the literature. According to our observation, parathyroidectomy was effective to provide clinical and radiological improvement in the presented case. The clinician should consider adding parathyroidectomy to conventional strategies immediately if the risk of such uncontrolled hyperparathyroidism is present.

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“…Clinically, CUA is characterized by very painful placques or subcutaneous nodules, and violaceous, mottled skin lesions that may progress to nonhealing ulcers, tissue necrosis, and gangrene. The clinical course may be complicated by surgical resections and amputations with a 1-year mortality rate .50%, with most deaths due to sepsis (4,5). Its pathology is significant for small vessel involvement and distal calcifications with intimal proliferation often accompanied by microthrombi.…”

Section: Discussionmentioning

confidence: 99%

Painful Skin Ulcers in a Hemodialysis Patient

Sprague

2014

Clinical Journal of the American Society of Nephrology

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SummaryCalciphylaxis, also referred to as calcific uremic arteriolopathy, is a relatively rare but well described syndrome that occurs most commonly in patients with late stage CKD. It is characterized by very painful placques or subcutaneous nodules and violaceous, mottled skin lesions that may progress to nonhealing ulcers, tissue necrosis, and gangrene with a 1-year mortality rate >50%. The pathogenesis of calciphylaxis is poorly understood. Risk factors include female sex, obesity, hyperphosphatemia, hypercalcemia, hyperparathyroidism, longer dialysis vintage, hypercoagulable states, and use of calcium-containing phosphate binders and warfarin. Treatment strategies for calciphylaxis are limited by inadequate understanding of its pathophysiology. Therapy is generally focused on correcting disturbances of calcium, phosphorus, and parathyroid hormone metabolism. Additional therapy focuses on decreasing inflammation and on dissolution of tissue calcium deposits with sodium thiosulfate and/or bisphosphonates. Successful treatment generally results in improvement of pain and healing of the lesions within 2-4 weeks, but the disorder generally takes many months to completely resolve.Clin J Am Soc Nephrol 9: 166-173, 2014. doi: 10.2215/CJN.00320113Case Description Abhijit Naik, MD (Renal Fellow). A 54-year-old white man was referred from an outside dialysis clinic for evaluation of necrotic skin lesions. He had a 10-year history of diabetes and hypertension, with ESRD secondary to diabetes. His history was also significant for coronary artery disease, with two prior myocardial infarctions and atrial fibrillation. He had been undergoing thrice-weekly hemodialysis for 2 years with a Kt/V between 1.3-1.4. Three months before presentation he noticed several small firm and very painful nodules on both anterior thighs. He stated that after several weeks the lesions became much larger, black, and spread to the lateral thighs and buttocks. He was treated with mupirocin ointment and a vascular evaluation revealed normal blood flow in his legs. Aside from these painful lesions, he stated that he generally felt "ok." Dialysis had been proceeding without problems. He denied chest pain, dyspnea, abdominal pain, or any gastrointestinal complaints. Review of systems was otherwise unremarkable. He never smoked and drank alcohol very infrequently. Medications included aspirin, amiodarone, simvastatin, famotidine, glipizide, clopidogrel, sevelamer carbonate, hydrocodone, and gabapentin. He had no known allergies.On examination his vital signs were as follows: temperature, 98.8°F; heart rate, 80 beats per minute; BP, 94/60 mmHg, with no orthostatic changes; and respiratory rate, 20 breaths per minute. His BP was generally low, with systolic BP averaging between 90 and 100 mmHg. He was an ill appearing male in mild distress from extremity pain. His lungs were clear and a cardiovascular examination revealed a regular rate and rhythm with a 2/6 holosystolic murmur. His abdomen had normal bowel sounds and was mildly distended with some asc...

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Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): A complication of chronic renal failure and benefit from parathyroidectomy

Cited by 244 publications

References 42 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

Endocarditis and Calcific Uremic Arteriolopathy: Is it a Coincidence or an Association? Case Report

Painful Skin Ulcers in a Hemodialysis Patient

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