Calciphylaxis in a Chronic Hemodialysis Patient with Protein S Deficiency

Rostaing, Lionel; Feki, Sondès El; Delisle, Marie–Bernadette; Durand-Malgouyres, Claire; Ton-That, Hugues; Bonafé, J. L.; Bories, Pierre; Durand, Dominique; Suc, J. M.

doi:10.1159/000168900

Cited by 49 publications

(24 citation statements)

References 11 publications

(18 reference statements)

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“…However, all five patients had high PTH levels [28]. Other reports relate calciphylaxis to protein S deficiency or heparin therapy in ESRD patients with elevated PTH levels [22,26,27].…”

Section: Discussionmentioning

confidence: 78%

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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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“…However, all five patients had high PTH levels [28]. Other reports relate calciphylaxis to protein S deficiency or heparin therapy in ESRD patients with elevated PTH levels [22,26,27].…”

Section: Discussionmentioning

confidence: 78%

“…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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“…The pathogenesis is uncertain and the aetiology is probably multifactorial. Several factors have been reported to be causal: hyperparathyroidism, persistent or transient hyperphosphataemia, vitamin D intoxication, calcium salt administration, steroid therapy in renal transplant recipients, and protein C and S deficiencies [9]. …”

Section: Discussionmentioning

confidence: 99%

“…Hyperparathyroidism is observed in most cases but not in all [9]. The hypercalcaemia appears to differ among patients with no consistent trend.…”

Section: Discussionmentioning

confidence: 99%

Acute Respiratory Failure due to ‘Pulmonary Calciphylaxis’ in a Maintenance Haemodialysis Patient

Matsuo

Tsukamoto²,

Tamura³

et al. 2001

Nephron

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Calciphylaxis is a rapidly developing, fatal process of vascular calcium deposition with prominent cutaneous manifestation. We treated a long-term haemodialysis patient who developed an analogous disorder limited to the lungs. A 57-year-old man was admitted for initiation of peritoneal dialysis because limited cardiac reserve precluded further haemodialysis. He was treated successfully for pneumonia until hypoxia and progressive hypercalcaemia developed. ^99mTc-methylene disphosphonate scintigraphy showed diffusely increased pulmonary uptake. Death supervened despite aggressive and successful treatment of hypercalcaemia. Autopsy studies included immunohistochemistry and morphometric studies of bone. Alveolar capillary walls showed diffuse calcium deposition. Both gross and microscopical findings differed from those of typical metastatic calcification in dialysis patients. Immunoreactivity for parathyroid hormone-related protein was present in the lesions. Bone histomorphometry indicated mild osteitis fibrosa. Pneumonia is believed to have caused local synthesis of parathyroid hormone-related protein that, along with high calcium × phosphorus product, contributed to calcium deposition. By analogy with the cutaneous process we termed the deposition ‘pulmonary calciphylaxis’.

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“…Histopathological examination reveals a mural calcification of small arteries with intimal proliferation, fibrosis and thrombosis [3, 4]. There is also a set of risk factors for the development of calciphylaxis such as obesity, elevated serum calcium and phosphate levels, hyperparathyroidism, warfarin, protein C and protein S deficiency, female sex as well as diabetes [2,5,6,7,8,9]. …”

Section: Introductionmentioning

confidence: 99%

Sodium Thiosulphate as a Promising Therapeutic Option to Treat Calciphylaxis

et al. 2006

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A 35-year-old haemodialysis-dependent woman with chronic renal failure developed large, very painful necrotic ulcers and necrosis on the thighs, buttocks and the abdomen with signs of fast progression. The skin biopsy specimens showed a broad necrosis of the epidermis and thrombosed dermal vessels with focal calcium deposits within the wall. In addi tion, laboratory findings presented an increased product of serum calcium and phosphate concentrations. Thus, we diagnosed calciphylaxis on the basis of clinical, biochemical and histopathological criteria. We initiated a therapy in which our patient was treated with intravenous sodium thiosulphate 3 times weekly. Already after 2 weeks of treatment, no new lesions were detectable and there was a dramatic pain relief. In the following 4 weeks, a successive decline of the ulcers and the healing of individual tissue defects could be seen. Four months after the start of the therapy, the patient underwent successful renal transplantation. Thus, the intravenous therapy of calciphylaxis with sodium thiosulphate might be a new effective alternative in the treatment of this life-threatening disease.

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Calciphylaxis in a Chronic Hemodialysis Patient with Protein S Deficiency

Cited by 49 publications

References 11 publications

Calciphylaxis in pediatric end-stage renal disease

Calciphylaxis in pediatric end-stage renal disease

Acute Respiratory Failure due to ‘Pulmonary Calciphylaxis’ in a Maintenance Haemodialysis Patient

Sodium Thiosulphate as a Promising Therapeutic Option to Treat Calciphylaxis

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