Hafner J, Keusch G, Wahl C, Burg G
Department of Dermatology, University Hospital, Zurich, Switzerland.
Vasa. 1998 Aug;27(3):137-43.
Calciphylaxis is a rare condition of rapidly extending ischemic skin necrosis or acral gangrene of fingers, toes or penis in patients with chronic renal failure. It may be accompanied by extensive metastatic calcification of soft tissues. Histology of infarcted tissues shows prominent medial calcification and intimal hyperplasia of subcutaneous arteries and/or digital arteries, respectively. The pathogenesis of calciphylaxis is only poorly understood. Most patients have hyperparathyroidism and an elevated calcium-phosphate-product, which is thought to be a major pathogenetic factor of calciphylaxis.
All published cases of calciphylaxis including nine of own (155 patients in total) from 1936 through 1996 were reviewed and subjected to statistical meta-analysis (Fisher's exact test).
Proximal locations of necrosis (thighs, buttocks, trunk) carried an unfavourable prognosis (63% mortality) compared to distal locations (calves, forearms, fingers, toes, penis) with 23% mortality (p < 0.0001). Parathyroidectomy was associated with a favourable outcome (p < 0.004). Diabetics with chronic renal failure had acral gangrene in 61% compared to 34% of the non-diabetic calciphylaxis-patients (p < 0.007).
The present analysis of all published cases of calciphylaxis is limited by patient selection and publication bias. The unfavourable prognosis of patients with proximal necrosis is impressive and might justify an early and aggressive treatment in such cases. However, the general benefit of parathyroidectomy remains debatable. Hyperparathyroidism should be managed primarily by conservative means. Parathyroidectomy should be reserved for patients with very high parathyroid hormone level and calcium-phosphate-product or with a rapidly progressive disease.
钙化防御是一种罕见的疾病，发生于慢性肾衰竭患者，表现为手指、脚趾或阴茎迅速扩展的缺血性皮肤坏死或肢端坏疽。它可能伴有软组织广泛的转移性钙化。梗死组织的组织学检查分别显示皮下动脉和/或指动脉的显著中层钙化和内膜增生。钙化防御的发病机制目前了解甚少。大多数患者患有甲状旁腺功能亢进和钙磷乘积升高，这被认为是钙化防御的主要致病因素。
回顾了1936年至1996年期间所有已发表的钙化防御病例，包括作者自己的9例（共155例患者），并进行了统计荟萃分析（Fisher精确检验）。
与远端部位（小腿、前臂、手指、脚趾、阴茎）相比，坏死近端部位（大腿、臀部、躯干）的预后较差（死亡率63%），远端部位死亡率为23%（p < 0.0001）。甲状旁腺切除术与良好预后相关（p < 0.004）。慢性肾衰竭的糖尿病患者发生肢端坏疽的比例为61%，而非糖尿病钙化防御患者为34%（p < 0.007）。
目前对所有已发表的钙化防御病例的分析受到患者选择和发表偏倚的限制。近端坏死患者的不良预后令人印象深刻，可能证明对此类病例应尽早进行积极治疗。然而，甲状旁腺切除术的总体益处仍有争议。甲状旁腺功能亢进应主要通过保守方法治疗。甲状旁腺切除术应保留给甲状旁腺激素水平和钙磷乘积非常高或疾病进展迅速的患者。
