Department of Endocrine Surgery, Johns Hopkins University, Baltimore, Maryland 21287, USA.
Endocr Pract. 2011 Mar-Apr;17 Suppl 1:54-6. doi: 10.4158/EP10349.RA.
To discuss when and how to workup calciphylaxis for early diagnosis and to delineate medical vs surgical management of the disease.
Review of evidence-based medical literature on calciphylaxis due to primary, secondary, and tertiary hyperparathyroidism.
Calciphylaxis is usually associated with secondary and tertiary hyperparathyroidism. However, calciphylaxis can also be seen in the absence of chronic renal failure and has been reported in patients with primary hyperparathyroidism due to a parathyroid adenoma or carcinoma. Calciphylaxis occurs when the levels of calcium and phosphate in the blood exceed their solubility level, leading to calcium-phosphate deposits in arteries that compromise the vasculature. These ischemic changes result in plaque-like lesions that progress to painful nodules. Calciphylaxis is diagnosed on the basis of physical examination, laboratory, and histopathologic findings. When medical therapy has failed in the setting of secondary/tertiary hyperparathyroidism with calciphylaxis, parathyroidectomy is the preferred treatment. In primary hyperparathyroidism, early recognition and aggressive wound care with debridement are important in managing this condition. However, resection of the offending parathyroid gland should be strongly considered.
When calciphylaxis is present, early detection is critical to the successful management of this condition. Although medical therapy can be effective, surgical resection of the diseased parathyroid glands can be curative and potentially life saving. A multidisciplinary approach involving early diagnosis, aggressive medical management, operative debridement, and parathyroidectomy has the best chance of improving survival in calciphylaxis.
讨论如何以及何时对钙化防御进行检查以进行早期诊断,并阐明疾病的医学与手术治疗方法。
回顾原发性、继发性和三发性甲状旁腺功能亢进引起的钙化防御的循证医学文献。
钙化防御通常与继发性和三发性甲状旁腺功能亢进有关。然而,在没有慢性肾衰竭的情况下也可以看到钙化防御,并且在由于甲状旁腺瘤或癌而患有原发性甲状旁腺功能亢进的患者中也有报道。当血液中的钙和磷水平超过其溶解度水平时,就会发生钙化防御,导致钙磷沉积在动脉中,从而损害脉管系统。这些缺血性变化导致斑块样病变进展为疼痛性结节。根据体格检查、实验室和组织病理学发现诊断钙化防御。在继发性/三发性甲状旁腺功能亢进伴钙化防御的情况下,如果药物治疗失败,则甲状旁腺切除术是首选治疗方法。在原发性甲状旁腺功能亢进中,早期识别和积极的清创术对于管理这种情况非常重要。但是,强烈建议切除致病的甲状旁腺。
当存在钙化防御时,早期发现对于成功管理这种情况至关重要。虽然药物治疗可能有效,但切除患病的甲状旁腺腺体可能具有治愈作用,并有潜在的挽救生命的作用。涉及早期诊断、积极的药物治疗、手术清创和甲状旁腺切除术的多学科方法最有可能提高钙化防御患者的生存率。