Calciphylaxis in a haemodialysis patient: functional protein S deficiency?

Perez-Mijares, R.; Guzman-Zamudio, J. L.; Payan-Lopez, J.; Rodríguez-Fernández, Ana; Gómez-Fernández, Pablo; Almaraz-Jimenez, M.

doi:10.1093/ndt/11.9.1856

Cited by 22 publications

(12 citation statements)

References 9 publications

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“…This results in clinical pictures similar to calciphylaxis in patients with or without renal failure [5,20,21,22,23,24,25,26,27,28]. In one report on five patients, protein C activity was noted to be significantly reduced in patients with calciphylaxis, reflecting the circulation of dysfunctional or activated protein C [28].…”

Section: Discussionmentioning

confidence: 99%

Calciphylaxis in pediatric end-stage renal disease

et al. 2005

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Calciphylaxis is a rare, but life-threatening complication of end-stage renal disease (ESRD) that has been reported mostly in adult patients. The exact etiology is unknown, but the disease is commonly associated with a high calcium-phosphorus product and elevated levels of parathyroid hormone (PTH). We herein review the published reports on calciphylaxis in ESRD patients less than 18 years old and report the case of a patient with severe calciphylaxis who presented with lower extremity pain, muscle tenderness and difficulty in walking. The serum PTH was low, and the calcium-phosphorus product was normal. The diagnosis of calciphylaxis was confirmed by a muscle biopsy. Treatment with low calcium peritoneal dialysate and substitution of calcium-based phosphorus binders with sevelamer (Renagel) was unsuccessful. The patient's clinical condition progressed to extensive soft tissue calcification and ulcerating skin lesions. Nine months after the onset of symptoms, the patient died of cardiopulmonary arrest.

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Section: Discussionmentioning

confidence: 99%

Calciphylaxis in pediatric end-stage renal disease

et al. 2005

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“…In these 5 patients, the levels of functional protein C were significantly reduced as compared with the healthy subjects or the patients with renal disease undergoing dialysis who did not develop calciphylaxis. A literature review revealed 39 calciphylaxis cases (for patients with or without ESRD) in which protein C levels were reported 2,5,8,12,13,[21][22][23][24][25][26][27][28][29][30][31][32][33][34] ; 14 (36%) of these patients had decreased protein C levels. Protein S levels were noted in 22 cases, with 11 patients (50%) having decreased levels.…”

Section: Discussionmentioning

confidence: 99%

“…2,5,8,13,[22][23][24][27][28][29][30][31][32][33][34] It has been hypothesized that low protein S levels in patients undergoing dialysis may result from loss of protein S (in peritoneal dialysis) or decreased free (active) protein S as a result of increased levels of binding proteins (in hemodialysis). 32 Most of the patients with calciphylaxis and 35,36 Calciphylaxis has been reported in at least 9 patients with primary hyperparathyroidism who did not have ESRD. [6][7][8][9][10] However, 6 of the 9 patients had some degree of impaired renal function because of nephrocalcinosis related to long-standing hyperparathyroidism, diabetic nephropathy, or pyelonephritis.…”

Section: Discussionmentioning

confidence: 99%

Calciphylaxis associated with acute, reversible renal failure in the setting of alcoholic cirrhosis

Chavel

Taraszka

Schaffer

et al. 2004

Journal of the American Academy of Dermatology

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“…The leading cause of death is sepsis from infected skin lesions, but death due to internal organ involvement has also been reported. Associated entities include end‐stage renal disease (ESRD), diabetes mellitus, elevated calcium–phosphate product, hyperphosphataemia, hypercalcaemia and hyperparathyroidism . Therapies are often directed at these abnormalities, and include phosphate binders, sodium thiosulfate and bisphosphonates.…”

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confidence: 99%

Calciphylaxis with evidence of hypercoagulability successfully treated with unfractionated heparin: a multidisciplinary approach

et al. 2015

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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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Calciphylaxis in a haemodialysis patient: functional protein S deficiency?

Cited by 22 publications

References 9 publications

Calciphylaxis in pediatric end-stage renal disease

Calciphylaxis in pediatric end-stage renal disease

Calciphylaxis associated with acute, reversible renal failure in the setting of alcoholic cirrhosis

Calciphylaxis with evidence of hypercoagulability successfully treated with unfractionated heparin: a multidisciplinary approach

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