Surgical Management of Calciphylaxis Associated with Primary Hyperparathyroidism: A Case Report and Review of the Literature

Bishop, Jennifer; Brown, Eric; Podesta, Augusto; Troy, Cathrine; Dong, Xiang Eric

doi:10.1155/2010/823210

Cited by 24 publications

(11 citation statements)

References 20 publications

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“…However, syndromes resembling calciphylaxis have been reported in individuals with pre-end-stage renal disease-staged CKD and even more rarely in those without any evidence of renal dysfunction but with inflammatory bowel disease, malignancy, trauma, and primary hyperparathyroidism. [4][5][6][7] This disease entity is characterized by calcification, intimal hypertrophy, and thrombosis of small vessels that slowly lead to tissue ischemia followed by infarction. Predominantly affecting subcutaneous tissue, this microvasculopathic process leads to the formation of painful nodules or plaques, which are often described as erythematous or violaceous foci that later erupt as necrotizing, nonhealing ulcers.…”

Section: Discussionmentioning

confidence: 99%

“…3,8 Several strategies aimed at treating and preventing this affliction in the nondialysis population have been reported in the literature. These include early parathyroidectomy, 5,7 sodium thiosulfate, 8,9 vitamin D analogs, 9 bisphosphonates, 10 cinacalcet, 10,11 and hyperbaric oxygen. 12 Unfortunately, none of these maneuvers have been shown to be universally beneficial and the outcome for most patients afflicted with calciphylaxis remains quite poor.…”

Section: Discussionmentioning

confidence: 99%

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Calciphylaxis presenting in early chronic kidney disease with mixed hyperparathyroidism

Brucculeri¹,

Haydon²

2011

IJNRD

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Calciphylaxis is a disabling and life-threatening complication that primarily affects patients who are dialysis dependent. Reports have grown in the literature of cases occurring in those who have advanced chronic kidney disease (pre-end-stage renal disease) or in the setting of transplantation. There are also a few reports of cases occurring in those without any form of chronic kidney disease but with primary hyperparathyroidism. This disease entity is characterized by calcification, intimal hypertrophy, and thrombosis of small vessels that result in necrotizing, nonhealing ulcers -many of which are life threatening. Although several strategies aimed at treating and preventing this affliction have been reported in the literature, the outcome for most patients with calciphylaxis remains quite poor. We describe a patient with comparatively early stage-3 chronic kidney disease who developed calciphylaxis in the setting of both primary and secondary hyperparathyroidism. Predictably, after subtotal parathyroidectomy, her wounds did not completely heal and her biochemical markers of hyperparathyroidism did not completely normalize until her underlying secondary hyperparathyroidism was treated medically. It was only after initiating cinacalcet that the patient experienced complete wound healing and resolution of her calciphylaxis. It also supports other authors' findings that cinacalcet may be an important adjunct in the treatment of calciphylaxis. Keywords: calciphylaxis, chronic kidney disease, cinacalcet, parathyroidectomy Case reportA 66-year-old Caucasian woman with a medical history significant for chronic kidney disease (CKD) stage 3, hypertension, and morbid obesity was followed regularly at our nephrology and hypertension clinic. She was being treated aggressively for her hypertension, proteinuria, hyperuricemia, and secondary hyperparathyroidism (SHPT). Her baseline creatinine fluctuated between 1.5 mg/dL and 2.0 mg/dL, and eGFR (6 variable Modification of Diet in Renal Disease) between 24 and 34 mL/min/1.73 m 2 . Her home medications included paricalcitol, gemfibrozil, febuxostat, pantoprazole, furosemide, lisinopril, latanoprost ophthalmic, and ferrous sulfate. Our patient had never taken warfarin, vitamin D, or any oral phosphate binders. In 2009, her intact parathyroid hormone (PTH) levels were noted to be rising despite her receiving oral paricalcitol 1 mcg daily. The dose was eventually adjusted up to 2 mcg daily without controlling her PTH which climbed as high as 216 pg/mL. Her calcium levels climbed from 9.0 to 10.4 mg/dL but never higher. Her serum phosphorous levels ranged from 2.9 to 3.7 mg/dL before, during, and after subsequent treatment. Most importantly, the Ca 2+ × PO 4 2-solubility product was never greater than 33.7. In August 2010, she presented with painful eruptions and ulcerations along the medial aspects of her thighs Dovepress submit your manuscript | www.dovepress.com

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Calciphylaxis presenting in early chronic kidney disease with mixed hyperparathyroidism

Brucculeri¹,

Haydon²

2011

IJNRD

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“…Although there are no studies demonstrating the optimal duration of intensified dialysis, I strongly recommend a course of dialysis 5-7 days per week for the first couple weeks of treatment, with the duration of this intensified dialysis therapy depending on clinical response. The relationship of calciphylaxis with hyperparathyroidism is best seen in nonuremic patients (4,14,21,42). There are reports that parathyroidectomy may benefit wound healing (43)(44)(45) and increase survival (5,39,45), whereas other studies demonstrated no benefit to parathyroidectomy (4,46).…”

Section: Managementmentioning

confidence: 99%

“…In fact, Budisavljevic and colleagues reviewed 47 patients with CUA, 31of whom underwent parathyroidectomy; 50% of these patients died within 9 weeks of surgery (47). Thus, the role of parathyroidectomy remains controversial (5,8,42,(48)(49)(50). Parathyroidectomy is unlikely to be useful when PTH concentrations are not markedly elevated (1,5,41).…”

Section: Managementmentioning

confidence: 99%

Painful Skin Ulcers in a Hemodialysis Patient

Sprague

2014

Clinical Journal of the American Society of Nephrology

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SummaryCalciphylaxis, also referred to as calcific uremic arteriolopathy, is a relatively rare but well described syndrome that occurs most commonly in patients with late stage CKD. It is characterized by very painful placques or subcutaneous nodules and violaceous, mottled skin lesions that may progress to nonhealing ulcers, tissue necrosis, and gangrene with a 1-year mortality rate >50%. The pathogenesis of calciphylaxis is poorly understood. Risk factors include female sex, obesity, hyperphosphatemia, hypercalcemia, hyperparathyroidism, longer dialysis vintage, hypercoagulable states, and use of calcium-containing phosphate binders and warfarin. Treatment strategies for calciphylaxis are limited by inadequate understanding of its pathophysiology. Therapy is generally focused on correcting disturbances of calcium, phosphorus, and parathyroid hormone metabolism. Additional therapy focuses on decreasing inflammation and on dissolution of tissue calcium deposits with sodium thiosulfate and/or bisphosphonates. Successful treatment generally results in improvement of pain and healing of the lesions within 2-4 weeks, but the disorder generally takes many months to completely resolve.Clin J Am Soc Nephrol 9: 166-173, 2014. doi: 10.2215/CJN.00320113Case Description Abhijit Naik, MD (Renal Fellow). A 54-year-old white man was referred from an outside dialysis clinic for evaluation of necrotic skin lesions. He had a 10-year history of diabetes and hypertension, with ESRD secondary to diabetes. His history was also significant for coronary artery disease, with two prior myocardial infarctions and atrial fibrillation. He had been undergoing thrice-weekly hemodialysis for 2 years with a Kt/V between 1.3-1.4. Three months before presentation he noticed several small firm and very painful nodules on both anterior thighs. He stated that after several weeks the lesions became much larger, black, and spread to the lateral thighs and buttocks. He was treated with mupirocin ointment and a vascular evaluation revealed normal blood flow in his legs. Aside from these painful lesions, he stated that he generally felt "ok." Dialysis had been proceeding without problems. He denied chest pain, dyspnea, abdominal pain, or any gastrointestinal complaints. Review of systems was otherwise unremarkable. He never smoked and drank alcohol very infrequently. Medications included aspirin, amiodarone, simvastatin, famotidine, glipizide, clopidogrel, sevelamer carbonate, hydrocodone, and gabapentin. He had no known allergies.On examination his vital signs were as follows: temperature, 98.8°F; heart rate, 80 beats per minute; BP, 94/60 mmHg, with no orthostatic changes; and respiratory rate, 20 breaths per minute. His BP was generally low, with systolic BP averaging between 90 and 100 mmHg. He was an ill appearing male in mild distress from extremity pain. His lungs were clear and a cardiovascular examination revealed a regular rate and rhythm with a 2/6 holosystolic murmur. His abdomen had normal bowel sounds and was mildly distended with some asc...

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“…3 To the best of knowledge, an around 25 case of calciphylaxis due to primary hyperparathyroidism has been reported so far in world literature. [4][5] This case had the most extensive presentation of calciphylaxis reported till date. Hence, report this case for its rarity as well as for highlighting its unusual presentation to contribute in part to the better understanding and awareness of this condition.…”

Section: Introductionmentioning

confidence: 99%

A rare extensive clinical presentation of calciphylaxis due to primary hyperparathyroidism

et al. 2020

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Calciphylaxis is a dreadful condition predominantly seen in chronic kidney disease patients on haemodialysis or following renal transplant. Calciphylaxis occurring in patients with normal kidney function is extremely rare. Here we present 53-year-old women presented with painful, extensive eschar-like skin lesions involving bilateral lower limb of 3 months of duration. She had hypercalcemia, high serum parathyroid hormone (PTH) levels and elevated alkaline phosphatase. Ultrasound neck and sestamibi scan demonstrated left inferior parathyroid adenoma. Lower limb radiographic studies showed subcutaneous calcification. Cutaneous biopsy confirmed calciphylaxis. Cinacalcet to control hypercalcemia, antibiotics and pain control medications were started. Left inferior parathyroidectomy and debridement of gangrenous lesions of lower limbs were performed. Histopathology was consistent with parathyroid adenoma. Lower limb wound started granulating with Vacuum-assisted closure (VAC) dressing and skin graft was planned. Unfortunately, she succumbed 11 weeks from surgery due to proximal myopathy, aspiration pneumonitis, sepsis and multiorgan failure.

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Surgical Management of Calciphylaxis Associated with Primary Hyperparathyroidism: A Case Report and Review of the Literature

Cited by 24 publications

References 20 publications

Calciphylaxis presenting in early chronic kidney disease with mixed hyperparathyroidism

Calciphylaxis presenting in early chronic kidney disease with mixed hyperparathyroidism

Painful Skin Ulcers in a Hemodialysis Patient

A rare extensive clinical presentation of calciphylaxis due to primary hyperparathyroidism

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