Monckeberg's calcific sclerosis of the media of the small-sized and medium-sized arteries is a well described and potentially life-threatening condition seen almost exclusively in patients with end-stage renal disease (ESRD) and with hyperparathyroidism. Penile gangrene resulting from this entity is associated with a mortality as high as 64%. A 65-year-old man with ESRD on dialysis for 6 years was referred to Harborview Medical Center with severe penile pain and partial necrosis of his glans penis, which progressed despite medical management. The patient had previously undergone amputations on all four extremities. After intraoperative biopsies of the proximal corpora cavernosa and spongiosum demonstrated viable tissue, he underwent partial penectomy. Pathologic evaluation revealed calciphylaxis within the media of the penile vessels. Two months later the patient had persistent wound-healing issues with intractable pain and thus underwent a complete penectomy with ultimate resolution of his severe pain.
[Show abstract][Hide abstract] ABSTRACT: Autopsy studies have demonstrated the near universal presence of fatty streaks and fibroatheromas in the general population from which patients with chronic kidney disease (CKD) arise. The vast majority of patients with CKD have multiple conventional cardiovascular risk factors. Vascular atherosclerotic calcification develops in most patients as they transition from the general population to significant CKD as part of cholesterol crystallization within atherosclerotic lesions. Once present, however, atherosclerotic medial calcification can become prominent and has been previously identified as Mönckeberg's sclerosis. A unifying concept supported by the preponderance of pathologic evidence contends that Mönckeberg's sclerosis is a manifestation of accelerated atherosclerosis in patients with CKD. The term has also been used in rare cases to describe vascular calcinosis not related to CKD. This clarification is critical to advance the field in terms of pathologic diagnosis and treatment of CKD bone and mineral disorder. Factors that seem to promote the osteoblastic transformation of vascular smooth muscle cells and enhance deposition of calcium hydroxyapatite crystals include phosphorus activation of the Pit-1 receptor, bone morphogenic proteins 2 and 4, leptin, endogenous 1,25 dihydroxyvitamin D, vascular calcification activating factor, and measures of oxidative stress. These entities work to accelerate the atherosclerotic process in patients with CKD and may be future targets for diagnosis and treatment because randomized trials with hydroxymethylglutaryl-CoA reductase inhibitors have failed to attenuate the rate of progressive vascular calcification.
Clinical Journal of the American Society of Nephrology 08/2008; 3(6):1585-98. DOI:10.2215/CJN.01930408 · 4.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Calciphylaxis is a relatively rare disorder associated with calcification of small- and medium-sized blood vessels, progressive
ischemic skin necrosis, and ulcerations. This situation is a potentially life-threatening condition seen in patients with
endstage renal disease (ESRD). A 64-year-old man with ESRD on dialysis for 6 years was admitted to our clinic with severe
pain and partial necrosis of some fingers on his right hand. In addition, the patient had suffered from diabetes for 22 years
and had been on insulin therapy for 17 years. His necrotic fingers were amputated. After two months, necrosis on his glans
penis developed despite medical management. Penile amputation was performed. In this case report, the patient serves to illustrate
the spectrum of complications with which calciphylaxis can be associated.
Central European Journal of Medicine 09/2009; 4(3):391-394. DOI:10.2478/s11536-009-0036-3 · 0.15 Impact Factor
Kumiko Takahashi, Takako Iino, Daisuke Yamazaki, Hiroyuki Watanabe, Hiroshi Ito
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