Tamayo-Isla Ramón Alberto, Cuba de la Cruz Mauro
Internal Medicine Department, Pietersburg Provincial Hospital, Polokwane Kidney and Dialysis Centre, Limpopo Province, South Africa.
Int J Nephrol Renovasc Dis. 2015 Feb 13;8:13-8. doi: 10.2147/IJNRD.S78310. eCollection 2015.
Calciphylaxis is a rare medical condition that is usually diagnosed in patients suffering from end-stage renal disease who are already receiving renal replacement therapy and in those post-transplantation. The pathogenesis still remains to be fully elucidated; hence, the treatment is not uniform. The prognosis is generally poor. The ulcerative stage exhibits a worse prognosis than the nonulcerative one. Calciphylaxis presenting in terminal kidney disease prior to dialytic treatment has only rarely been reported.
A 32-year-old female Caucasian clerk sought medical attention for increasing tiredness and lower limb skin ulcers. Polycystic kidney disease was diagnosed in her late father and two of her siblings. At the first nephrology consultation, obesity, pallor, bilateral flank masses with ballottement, and two ulcers with a dark necrotic center on the distal left leg were noted. In addition, another indurated light bluish lesion of 5 cm just above the right knee with intact skin was observed. All lesions were very tender and warm on touch. Laboratory results yielded hypercalcemia, hyperphosphatemia, anemia, and parathyroid hormone levels that were more than ten times the normal values in the patient, and with a glomerular filtration rate of 4 mL/minute. Skin biopsy confirmed the suspicion of calciphylaxis. The patient was placed on peritoneal dialysis with low Ca concentration baths, cinacalcet, and aluminum hydroxide. The results included correction of hypercalcemia, improvement of phosphate levels, and the product of both Ca and phosphate, but only a transitory decrease in serum parathyroid hormone levels. The ulcerations were completely healed after 2 months of treatment. Cinacalcet was discontinued after 18 months, but multiple large-size, nonulcerative indurated areas appeared 3 months later in the lower limbs after discontinuation of the drug. A parathyroidectomy performed 17 months later revealed a four-gland hyperplastic disease. The patient experienced relief of skin symptoms soon after the procedure and remains in a very satisfactory condition.
Calciphylaxis is a very complex clinical entity. Calciphylaxis presenting prior to dialytic treatment in end-stage renal disease is rare in the absence of a trigger. Cinacalcet and parathyroidectomy should be considered in selected patients.
钙过敏是一种罕见的病症，通常在接受肾脏替代治疗的终末期肾病患者以及移植后患者中被诊断出来。其发病机制仍有待充分阐明；因此，治疗方法并不统一。总体预后较差。溃疡期的预后比非溃疡期更差。在透析治疗前的终末期肾病中出现钙过敏的情况鲜有报道。
一名32岁的白人女性职员因日益疲劳和下肢皮肤溃疡就医。她的父亲和两个兄弟姐妹在晚年被诊断出患有多囊肾病。在首次肾病会诊时，发现患者肥胖、面色苍白、双侧腰部有肿块且有浮沉感，左腿远端有两个溃疡，溃疡中心呈暗黑色坏死，此外，右膝上方还有一个5厘米大小、质地坚硬、淡蓝色、皮肤完整的病变。所有病变触诊时都非常疼痛且发热。实验室检查结果显示患者血钙过高、血磷过高、贫血，甲状旁腺激素水平超过正常值十倍以上，肾小球滤过率为4毫升/分钟。皮肤活检证实了钙过敏的怀疑。患者接受了低钙浓度浴的腹膜透析、西那卡塞和氢氧化铝治疗。结果包括高钙血症得到纠正、血磷水平及钙磷乘积有所改善，但血清甲状旁腺激素水平仅短暂下降。治疗2个月后溃疡完全愈合。18个月后停用西那卡塞，但停药3个月后下肢出现多个大尺寸、非溃疡的硬结区域。17个月后进行的甲状旁腺切除术显示为四腺增生性疾病。手术后患者的皮肤症状很快得到缓解，目前状况非常良好。
钙过敏是一种非常复杂的临床病症。在终末期肾病中，透析治疗前无诱因出现钙过敏的情况很罕见。对于特定患者应考虑使用西那卡塞和进行甲状旁腺切除术。
