Penile calciphylaxis: analysis of risk factors and mortality

Penile calciphylaxis: analysis of risk factors and mortality
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DOI:
10.1097/01.ju.0000064334.85656.a1
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发表时间:
2003-06-01
期刊:
影响因子:
6.6
通讯作者:
Kurzrock, EA
Kurzrock, EA
中科院分区:
医学1区
文献类型:
--
作者:
Karpman, E;Das, S;Kurzrock, EA

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目的:阴茎钙化耐受症是一种罕见的导致感染和坏疽的疾病。大多数病例与全身性钙过敏症有关。阴茎钙过敏症的病理生理学、诊断和治疗作为一个独特的实体很少受到关注。材料与方法:回顾性分析1例阴茎钙化过敏症的临床资料,并结合文献进行分析。患者的特点,介绍,血清化学研究,管理和outcomes reported.Results:在文献中,包括我们的病人,共确定了34例阴茎钙化。患者平均年龄为58岁。所有患者均患有终末期肾病,76%的患者合并糖尿病。在三分之二的患者中发现了生殖器以外的坏疽区域。平均磷酸钙产品为78.5 mg。(2)/dl。(2)(范围20.6至52.5),平均甲状旁腺素水平为553 pg/ ml. (10至65)。8例行甲状旁腺切除术。所有患者均接受局部清创/伤口护理或部分/全阴茎切除术治疗。接受甲状旁腺切除术的患者(75%)的生存率高于仅接受局部清创术或阴茎切除术的患者(28%)。与这种疾病相关的总死亡率为64%,平均死亡时间为2.5个月。结论:阴茎钙化耐受是由于中膜钙化和血管纤维化。与这种疾病相关的并发症和死亡率极高。继发性甲状旁腺功能亢进和磷酸钙增加是特征性的,需要积极的药物治疗。阴茎病变和甲状旁腺激素的手术治疗是有争议的。我们的综述表明,甲状旁腺切除术可能会提高生存率,而生存率与阴茎病变的局部治疗类型无关。
Purpose: Penile calciphylaxis is a rare condition resulting in infection and gangrene. Most cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as a distinct entity have received little attention. We reviewed the literature to increase understanding of this disease.Materials and Methods: A retrospective review of the literature was performed after treating a case of penile calciphylaxis. Patient characteristics, presentation, serum chemistry studies, management and outcomes are reported.Results: A total of 34 cases of penile calciphylaxis were identified in the literature including our patient. Average patient age was 58 years. All patients had end stage renal disease, and diabetes mellitus was a co-morbidity in 76%. Additional areas of gangrene beyond the genitalia were found in two-thirds of patients. Average calcium phosphate product was 78.5 mg.(2)/dl.(2) (range 20.6 to 52.5) and mean parathormone level was 553 pg./ml. (10 to 65). Parathyroidectomy was performed in 8 patients. All patients were treated with either local debridement/wound care or partial/total penectomy. Survival was better in patients who underwent parathyroidectomy (75%) than in those treated with local debridement or penectomy alone (28%). The overall mortality associated with this disease was 64% with a mean time to death of 2.5 months.Conclusions: Penile calciphylaxis is a result of medial calcification and fibrosis of blood vessels. The co-morbidity and mortality associated with this disease are extremely high. Secondary hyperparathyroidism and an increased calcium phosphate are characteristic and require aggressive medical management. Surgical management of penile lesions and parathormone is controversial. Our review suggests that parathyroidectomy may improve survival and that survival is independent of the type of local treatment for the penile lesions.