1/35. Intensive tandem cryofiltration apheresis and hemodialysis to treat a patient with severe calciphylaxis, cryoglobulinemia, and end-stage renal disease.This is the first report on tandem cryofiltration apheresis (CFA) and hemodialysis (HD). A 44 year old white man with Type II mixed cryoglobulinemia, hepatitis c virus (HCV), severe skin lesions, and end-stage renal disease (ESRD) on maintenance hemodialysis was air-transferred for CFA, which is only available at our medical center. The patient failed to respond to high dose steroids, immunosuppression, intravenous immunoglobulin (IVIG), and plasma exchange for the treatment of his cryoglobulinemia, and he failed alpha-interferon therapy for his HCV. On arrival, he was also found to have severe calciphylaxis secondary to ESRD with generalized, painful skin ulceration, necrosis, and penile gangrene. To treat both conditions, intensive, tandem CFA/HD was initiated. He received extensive wound care and surgical debridement. To prevent pressure ulcers and worsening of skin lesions, he was placed on the FluidAir (Kinetic Concepts Inc., San Antonio, TX) controlled air bed. The patient received 18 tandem CFA/HD treatments, and four extra HD treatments in one month. sodium citrate was used as an anticoagulant for the CFA procedure. His plasma cryoglobulin (CG) level dropped from 6,157 to 420 microg/ml, and his calciphylaxis also improved. The CFA effectively removed 93% of CG, without significant removal of IgG, IgM, IgA, albumin, and fibrinogen. No albumin or fresh frozen plasma (FFP) was required as replacement fluid for CFA. No citrate toxicity or evidence of complement activation with the cryofilter was observed. The entire CFA procedure time (3(1/2) hours) was considered. Intensive, tandem CFA/HD was performed in a critically ill patient with no apparent adverse consequences.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/35. calciphylaxis associated with metastatic breast carcinoma.calciphylaxis is a rare disorder associated with calcification of small- and medium-sized blood vessels, and progressive skin necrosis usually seen in the setting of end-stage renal disease (ESRD) and secondary hyperparathyroidism. It has also been observed in primary hyperparathyroidism, hypercalcemia of malignancy (extensive bony metastasis of breast cancer), and an isolated case reported with end-stage liver disease. We report an unusual case of calciphylaxis associated with metastatic breast carcinoma in the absence of renal or parathyroid disease. calciphylaxis has generally been associated with end-stage renal disease and hyperparathyroidism. One previous case report described calciphylaxis occurring in a patient with metastatic adenocarcinoma of the breast and hypercalcemia. Our case represents the second reported case of calciphylaxis associated with osteolytic, metastatic breast cancer. Although ESRD with secondary hyperparathyroidism is the most common presentation of calciphylaxis, this case demonstrates that other conditions that alter normal calcium metabolism must be considered in the differential diagnosis.- - - - - - - - - - ranking = 0.66666666666667keywords = calciphylaxis (Clic here for more details about this article) |
3/35. Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis.A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
4/35. calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K.calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin k deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. thrombosis and protein c deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein c deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
5/35. heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein s deficiency.skin ulceration is a well-characterized thrombotic complication of the heparin-induced thrombocytopenia (HIT) syndrome. We present the case of a 73-year-old diabetic woman nearing end-stage renal failure who developed extensive upper thigh, abdominal and buttock ulceration following initiation of subcutaneous heparin for prophylaxis against deep vein thrombosis. A preliminary diagnosis of calciphylaxis was made based on the classical distribution and macroscopic appearance of the ulceration in a patient with end-stage renal failure and secondary hyperparathyroidism. However skin biopsy showed complete absence of calcium deposits in the dermal microvasculature. The presence of extensive microthrombi within dermal vessels prompted serologic testing to detect a prothrombotic state. We identified the combined presence of heparin-dependent platelet activating (HIT) antibodies and functional protein s deficiency. To our knowledge this is the first reported case of a dialysis patient presenting with skin ulceration induced by heparin and protein s deficiency. This case highlights the importance of a skin biopsy and testing for a hypercoaguable state in patients with end-stage renal disease and skin ulceration. We suggest that HIT antibodies should be requested in all dialysis patients presenting with skin ulceration.- - - - - - - - - - ranking = 0.16666666666667keywords = calciphylaxis (Clic here for more details about this article) |
6/35. Necrotic skin lesions in a dialysis patient: a multifactorial entity.A female dialysis patient with a consistently high serum calcium phosphate product presented with large necrotic skin lesions with ulcers. The clinical course was highly suggestive of calciphylaxis. parathyroidectomy was followed by the healing of the lesions. New skin lesions appeared following relapse of hyperparathyroidism. Her clinical records included a long past of hypertension, which was the cause of her renal failure. She had a limited walking range and previously had presented bilateral ulcers of vascular origin. This case presents a type of lesion which bears a serious prognosis in dialysis patients. The clinical context and the presentation of the lesions are compatible with multiple etiology: vascular lesions and calciphylaxis. The documented longitudinal follow-up illustrates the importance of treating the different factors known to participate in the appearance of skin lesions in dialysis patients. Particularly, it stresses the benefit of performing parathyroidectomy, even if the parathyroid hormone level is not in the range normally accepted as requiring surgical removal of parathyroid glands.- - - - - - - - - - ranking = 0.33333333333333keywords = calciphylaxis (Clic here for more details about this article) |
7/35. calciphylaxis: a case study with differential diagnosis.calciphylaxis is a relatively rare, but horribly disfiguring, skin condition that is most often associated with end-stage renal disease and long-term dialysis. Unfortunately, calciphylaxis-related morbidity and mortality are significant. The case study presented demonstrates many of the findings associated with the typical calciphylaxis patient; end-stage renal disease and an extensive, painful ulcer. The complexity of the patient s history and medical/surgical interventions, especially medication therapy with coumadin and heparin, complicated initial diagnostic processes. Close scrutiny of multiple physical assessment findings, historical factors, and test results was required for correct diagnosis. Crucial components of differential diagnosis of calciphylaxis versus coumadin-induced skin necrosis or heparin-induced thrombocytopenia necrosis include: patient history and characteristics, clinical presentation, and diagnostic test results.- - - - - - - - - - ranking = 0.5keywords = calciphylaxis (Clic here for more details about this article) |
8/35. Progression to wet gangrene in penile necrosis and calciphylaxis.We report a patient who presented with gangrene of the penis due to renal failure and calciphylaxis. In view of his advanced underlying conditions we pursued an expectant approach. superinfection set in necessitating extensive debridement, demasculinisation and a perineal urethrostomy. Of the 34 reported cases in the literature, 12 (35%) were treated conservatively, 18 (53%) had been treated expectantly with delayed surgery following the development of complications and 4 (12%) underwent early surgery (penectomy). 20 (59%) of the patients subsequently died--8/12 (58%) treated conservatively, 11/18 (61%) treated with expectant surgery and 1/4 (25%) treated with early surgery. Progression to wet gangrene developed in the majority of patients--23/34 patients (68%). Penile gangrene is a symptom of severe systemic disease, where two thirds of affected patients decease within six months of onset of symptoms. Expectant treatment results in superinfection and wet gangrene in the majority of cases who ultimately require extensive surgery and debridement.- - - - - - - - - - ranking = 0.83333333333333keywords = calciphylaxis (Clic here for more details about this article) |
9/35. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.- - - - - - - - - - ranking = 1.3333333333333keywords = calciphylaxis (Clic here for more details about this article) |
10/35. An unusual case of calciphylaxis.BACKGROUND: Cutaneous calciphylaxis is a rare disorder that occurs most frequently in patients with end-stage renal disease (ESRD), those on hemodialysis, and renal transplant recipients. It is frequently associated with hyperparathyroidism and a markedly elevated calcium-phosphate product, and it carries a high mortality rate. The usual clinical presentation is of painful, stellate necrosis of the thighs or buttocks, often in the setting of livedo reticularis. death usually results from septicemia. OBJECTIVE: This report documents an unusual case of recurrent, self-limiting calciphylaxis in the setting of a patient with ESRD and discusses the clinical and pathologic features of this potentially very fatal disorder. methods AND RESULTS: A 52-year-old woman presented with a greater than one-year history of relapsing and remitting, exquisitely painful, necrotic, numular plaques on the abdomen, breast, and arm. This patient had a markedly elevated calcium-phosphate product and parathyroid hormone level. The diagnosis of calciphylaxis was made by wedge biopsy of the most recent plaque, revealing calcification of medium-sized subcutaneous vessels and lobular capillaries with associated epidermal necrosis. CONCLUSIONS: This case demonstrates an unusual clinical variant of calciphylaxis that presented without the characteristic stellate necrosis or livedo reticularis that normally marks this condition and spontaneous resolution without incurring septicemia. Regardless of morphology, calciphylaxis should be considered in the differential diagnosis of painful, necrotic lesions occurring in the setting of ESRD.- - - - - - - - - - ranking = 1.5keywords = calciphylaxis (Clic here for more details about this article) |
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