腹膜透析患者血管钙化的相关因素分析

[目的]观察腹膜透析患者血管钙化的发生情况并探讨导致血管钙化的相关因素。[方法]利用彩超对腹膜透析患者颈内动脉斑块及内膜厚度进行测量,根据颈内动脉斑块钙化程度分为斑块组和无斑块组,并检测比较两组患者骨保护素（OPG）及血管钙化相关的临床指标。[结果]40例腹膜透析患者,有60％患者有颈内动脉内膜增厚钙化,其中斑块组和无斑块的病人相比,糖尿病比例更高,年龄较大;斑块组OPG、C反应蛋白（CRP）、血磷明显高于非斑块组,且两组相比差异有显著性（均P〈0．01）。颈内动脉厚度与OPG呈正相关（R=0．332）。[结论]腹膜透析患者普遍存在血管钙化,其与透析患者微炎症状态、高血磷有关,OPG水平可较好地反映血管钙化程度。     

Acquired perforating calcific collagenosis in a drug addict with rhabdomyolysis and transient hypercalcemia

Acquired perforating calcific collagenosis (APCC), which is characterized by the calcification of dermal collagen fibers with subsequent transepidermal elimination and perforation, is an extremely rare entity. Thus far, it has only been reported in a patient with direct contact exposure to calcium chloride. Here, we report a unique case of APCC occurring in a drug addict admitted for rhabdomyolysis. The present case is a 20‐year‐old male patient hospitalized for drug‐related rhabdomyolysis and multiple organ damage. During hospitalization, he gradually developed unusual skin rashes. There were multiple confluent umbilicated and keratotic erythematous to brownish papules and plaques with scratch‐like linear plaques on his lower abdomen, inguinal areas and gluteal sulci. Also, multiple well‐demarcated flesh‐colored rough, hard and thin plaques with a “crepe paper”‐like texture were found on the bilateral popliteal fossae, olecranon fossae and axillae. The histopathology of two biopsied lesions demonstrated acquired perforating calcific collagenosis. The lesions appeared during the rhabdomyolysis‐related hypercalcemia phase and resolved spontaneously after the calcium level returned to normal. This is the first reported case of disseminated APCC occurring during transient hypercalcemia due to rhabdomyolysis.     

Demographic and clinical characteristics of spinal calcinosis in systemic sclerosis: Possible association with peripheral angiopathy

The objective was to evaluate the demographic and clinical characteristics of systemic sclerosis (SSc) patients with spinal calcinosis. Paraspinal and intraspinal calcinosis was assessed blindly by orthopedic surgeons specializing in spinal diseases using chest high‐resolution computed tomography (CT) that was performed for the screening and prospecting of interstitial lung disease in 159 Japanese SSc patients. Among these patients, we identified 27 (17%) with spinal calcinosis, and the most common site was cervical level at 77.8% (21/27). The frequency of spinal calcinosis in the late stage was higher than in the early stage (44.4% vs 29.6%). Multiple calcinosis was identified in 18.5% (5/27). The frequency of paraspinal calcinosis only was 59.3%, intraspinal calcinosis only 18.5%, and both intraspinal and paraspinal calcinosis 22.2%. Among SSc patients, 4.4% (7/159) had CT‐based evidence of spinal cord compression. Among cases with spinal cord compression, only one had neurological symptoms, and surgical removal improved the symptoms. The other six SSc patients with spinal calcinosis (3.8% of 159) had no symptoms. Male sex (29.6%) and severe peripheral vasculopathy such as digital ulcers (55.6%) and acro‐osteolysis (33.3%) were significantly more frequent in the SSc patients with spinal calcinosis than in the SSc patients without spinal calcinosis (10.6%, 32.6% and 14.4%, respectively). Our results suggest that severe peripheral vasculopathy may be associated with the development of spinal calcinosis. Because SSc patients are prone to spinal calcinosis, when SSc patients claim symptoms such as pain, numbness and movement disorder of the extremities, spinal calcinosis is a complication that should be taken into consideration.     

Ochronotic Involvement of the Aortic and Mitral Valves in a 72-Year-Old Man

Ochronosis, an autosomal recessive metabolic disorder, causes an excess of homogentisic acid that results in adverse pigmentation, calcification, and inflammation of cartilaginous and other tissues. Cardiovascular abnormalities are less frequently reported than are other manifestations. In rare cases, ochronosis can cause valvular heart disease. We report the case of a 72-year-old man with aortic stenosis and mitral insufficiency who was diagnosed with ochronosis while undergoing surgical aortic and mitral valve replacement. We discuss the history and surgical management of alkaptonuric ochronosis.     

Miscellaneous non-inflammatory musculoskeletal conditions. Hyperphosphatemic familial tumoral calcinosis (FGF23, GALNT3 and αKlotho)

Familial tumoral calcinosis (TC) is a rare disorder distinguished by the development of ectopic and vascular calcified masses that occur in settings of hyperphosphatemia (hFTC) and normophosphatemia (nFTC). Serum phosphorus concentrations are relatively tightly controlled by interconnected endocrine activity at the level of the intestine, kidney, and skeleton. Discovering the molecular causes for heritable forms of hFTC has shed new light on the regulation of serum phosphate balance. This review will focus upon the genetic basis and clinical approaches for hFTC, due to genes that are related to the phosphaturic hormone fibroblast growth factor-23 (FGF23). These include FGF23 itself, an FGF23-glycosylating enzyme (GALNT3), and the FGF23 co-receptor α-Klotho (αKL). Our understanding of the molecular basis of hFTC will, in the short term, aid in understanding normal phosphate balance, and in the future, provide potential insight into the design of novel therapeutic strategies for both rare and common disorders of phosphate metabolism.     

Clinical pearls and therapeutic challenges in connective tissue disease

ABSTRACT: The management of autoimmune skin disease is extremely challenging. This article provides the clinician with clinical pearls and highlights novel/anecdotal treatments for the management of recalcitrant discoid lupus erythematosus, lupus profundus, calcinosis cutis associated with dermatomyositis and scleroderma, and intractable pruritus in autoimmune disease. The disfigurement that occurs as a result of autoimmune skin diseases may be great. This article includes a discussion of the cosmetic and psychosocial concerns of patients. Lastly, wounds and wound care in autoimmune diseases are addressed along with practical management of challenging cases such as digital ulcers in Raynaud's disease, pyoderma gangrenosum, and ulcerating striae and leg ulcers in scleroderma and dermatomyositis.     

冠状动脉旋磨术治疗严重钙化病变的护理体会

中重度钙化病变是导致经皮冠状动脉腔内成形术(PTCA)手术失败和血管急性闭塞的主要危险因素.在一些严重狭窄尤其是伴严重钙化的病变,球囊可能无法通过或病变难以扩张开.严重钙化的狭窄病变行介入治疗时,冠状动脉旋磨术已成为首选.冠状动脉旋磨术系采用呈橄榄形带有钻石颗粒旋磨头,根据"差异切割"或"选择性切割"的理论选择性去除纤维化或钙化的动脉硬化斑块,而具有弹性的血管组织在高速旋转的旋磨头通过时会自动弹开,故旋磨术不对有弹性的组织起作用.旋磨后往往可获得光滑的血管内腔,特别是对于严重钙化病变,内膜撕裂的发生率明显低于单纯球囊扩张.我院2003年11月～2006年2月成功施行冠状动脉旋磨术26例,现将护理体会报告如下.