Calciphylaxis without renal failure.
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BACKGROUND: It is generally accepted that the elevated calcium phosphate product in dialysis patients is associated with the development of extraskeletal calcification. There is, however, no clear association between the raised levels of calcium and phosphate ions and the degree of limboconjunctival calcification. This article focuses on the role of tissue devitalisation in the development of ocular calcification. METHODS: Local injury of the outer eye can be visualised by the Lissamine green staining test. We measured the pre- and postdialysis staining score in 38 dialysis patients. The differences between pre- and postdialysis tear fluid secretion and tearfilm stability were also assessed, as was the degree of limboconjunctival calcification. Serum calcium and phosphate levels were measured in all patients. RESULTS: In 14 of the 38 patients, demonstrable tissue injury was present as a result of a recurrent decrease of tear secretion after each dialysis session. There was a statistically significant association between the degree of limboconjunctival calcification and both the difference between the pre- and postdialysis Lissamine green staining score and the total number of haemodialysis sessions. CONCLUSION: Local factors, such as minor tissue injury of the limboconjunctival epithelium, referred to as "local challenger", are more likely to determine the degree of ocular calcification in dialysis patients than are systemic factors. Tissue injury of the outer eye in these patients is the result of a chronic decrease of tear fluid that occurs after each hemodialysis session.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
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Calciphylaxis is a rare syndrome characterized by progressive vascular calcification and ischemic tissue loss in patients with chronic renal failure. We report our five-year experience with five patients who developed foot gangrene due to calciphylaxis. All five patients had characteristic clinical, laboratory, and radiologic findings of the disorder, but no diagnostic variable was uniformly present. All five had progressed to advanced gangrene at the time of surgical consultation. Despite aggressive local attempts to control infection, all five patients died of septic complications. Parathyroidectomy was performed in three patients but did not alter the course in any case. This small experience suggests that the outcome of foot gangrene associated with calciphylaxis is predicated on the degree of tissue loss. Aggressive local wound care does not appear to be adequate to control infection in patients who have already developed gangrene. Although parathyroidectomy may have important long-term advantages in patients with calciphylaxis, it does not appear to affect outcome in these advanced cases.
We report seven cases (out of 100 dialysis patients) at the University of Kentucky who were diagnosed with calciphylaxis syndrome between 1993 and 1998. Of note is the fact that five of these patients had bone biopsy-proven adynamic renal bone disease, rather low intact PTH, and relatively low calcium-phosphorus product. This is in contrast to the previous view that calciphylaxis is usually seen in patients with excessive parathyroid activity. The reemergence of calciphylaxis with renal bone disease is an intriguing finding and does not correspond to earlier reports associating calciphylaxis with hyperparathyroid bone disease. This report reviews the clinical, biochemical, and bone histology findings of these patients, and provides a review of the literature.
The index patient is a 23-year-old female with end-stage renal disease (ESRD) secondary to chemotherapeutic agents. Continuous cycling peritoneal dialysis (CCPD) has been the renal replacement therapy for the past 5 years since a failed cadaveric renal transplant. Past medical history was significant for diabetes mellitus, hypertension, anemia, bilateral subclavian vein thrombosis with superior vena cava syndrome, secondary hyperparathyroidism, leukemia (at age 8), and hyperlipidemia. On presentation, soft tissue nodules were noted in the anterolateral surfaces of the legs. After 3 months of continued low-calcium-dialysate CCPD, calcitriol, and oral phosphate binders, a 2 x 3 cm nodule was noted on the posterior aspect of the thorax at the scapula. The only complaint at this time was shoulder pain at the acromioclavicular joint. Radiological examination revealed a 3 x 4 cm soft tissue opacity in the superior segment of the left lower lobe laterally. Despite a prior subtotal parathyroidectomy, phosphate binders, and calcitriol, the parathyroid hormone levels continued to increase, with development of tumoral calcinosis, worsening renal osteodystrophy, and calciphylaxis. Computed tomography examination revealed extensive soft tissue calcification consistent with tumoral calcinosis. An ulcerative lesion (1 cm) developed on the lateral aspect of the upper thigh owing to warfarin necrosis versus calciphylaxis. At this time, the phosphate binder was changed from calcium acetate to sevelamer hydrochloride. Aggressive wound treatment and aggressive calcium and phosphate control added to the treatment regimen has resulted in healing of the single ulcer and a decrease in the size of the tumoral lesions. In conclusion, early recognition and aggressive treatment of calciphylaxis can result in reduced morbidity and mortality from calciphylaxis in ESRD patients.