Tumoral calcinosis in infancy

The most frequent form of idiopathic calcinosis is tumoral calcinosis (TC) which rarely occurs at young ages. We describe here a TC case of a young boy with its light microscopy completed with electron microscopic examinations. X-ray microanalysis revealed in the intracellular crystals CaCl₂ besides the previously described hydroxyapatite. The significance of this finding is unknown at the moment.     

Progressive pulmonary calcification in a child after orthotopic liver transplantation

We present a case of progressive pulmonary calcification associated with prolonged respiratory insufficiency in a 2-year-old boy with a history of orthotopic liver transplantation. This case demonstrates the potentially progressive nature of pulmonary calcification and that it can present with respiratory insufficiency at a later period after transplantation than previously thought. We describe radiological findings and discuss established as well as plausible pathological mechanisms contributing to the development of calcifications in these patients.     

罗格列酮抑制糖基化终产物诱导大鼠血管平滑肌细胞钙化机制

目的探讨罗格列酮抑制糖基化终产物(AGEs)诱导大鼠血管平滑肌细胞(VSMCs)钙化的可能机制。方法在含10 mmol·L~(-1)β-甘油磷酸培养液中加入200 mg·L~(-1)的AGEs及不同浓度(10~(-7)～10~(-5)mol·L~(-1))的罗格列酮体外培养VSMCs,采用甲-酚酞络合酮方法测定细胞钙含量,real time-PCR检测肿瘤坏死因子α(Tnf-α)、白细胞介素6(Il-6)及糖基化终产物受体(Rage)mRNA表达,Western blotting检测Rage蛋白表达,ELISA法检测细胞上清Tnf-α、Il-6水平。结果 AGEs可使VSMCs钙含量增加,Tnf-α、Il-6及Rage mRNA和蛋白表达增加(P<0.01);罗格列酮组均可降低VSMCs细胞层钙含量,抑制上述影响(P<0.05)。结论罗格列酮可能通过抑制VSMCs Rage表达,降低Tnf-α、Il-6表达和分泌,减轻AGEs诱导的VSMCs钙化。     

Child‐onset systemic sclerosis positive for anticentromere antibodies: Two Japanese cases

Systemic sclerosis (SSc) is an uncommon connective tissue disease of childhood. Moreover, pediatric SSc positive for anticentromere antibodies (ACA) is extremely rare. We describe two cases of ACA‐positive SSc in Japanese girls with clinical findings. Case 1 is a 15‐year‐old female. From disease onset at 7 years, she developed limited cutaneous (no internal involvement) SSc positive for ACA and anti‐U1RNP antibodies, but negative for antitopoisomerase I antibodies (ATA). She also showed calcinosis, sclerodactyly, and telangiectasia, but not Raynaud's phenomenon. Case 2 is also a 15‐year‐old female with onset at the same age. Her diagnosis was limited cutaneous SSc positive for ACA, anti‐SSA/Ro antibodies, and anti‐thyroid antibodies, but not ATA. She showed sclerodactyly, telangiectasia, and Raynaud phenomenon, as well as complicated Sjögren's syndrome and chronic thyroiditis with euthyroidism. We reported that two Japanese girls with SSc were positive for ACA and other antibodies with the exception of ATA.     

Calcinosis cutis following contact with calcium chloride solution

Calcinosis cutis is the deposition of insoluble calcium in the cutaneous tissue. Calcinosis cutis can be classified as metastatic, dystrophic, idiopathic or exogenous. We report a 48‐year‐old white man who was dismantling a portable ice skating rink when calcium chloride solution from the pipes spilt onto his clothing. Several days later, he started to develop mildly pruritic erythematous papules, some studded with white deposits and some with umbilication over the exposed areas corresponding to the spillage of the calcium chloride solution. Histological features revealed interstitial fibrohistiocytic reaction with calcium‐encrusted degenerated collagen bundles in the dermis which was further confirmed by von Kossa stain. He was commenced on topical corticosteroid cream twice daily and the lesions cleared completely between 6 to 10 weeks.     

肿瘤样钙质沉着症的临床和影像学分析

目的:探讨肿瘤样钙质沉着症的临床及影像学表现,提高本病的影像诊断水平.方法:回顾性分析6例经手术病理证实的肿瘤样钙质沉着症的临床及影像学资料.6例均行X线平片检查,2例行MRI检查.结果:6例患者主要临床表现为关节附近无痛性肿块,多发肿块2例,单发肿块4例.主要X线表现为关节周围团块状软组织钙化.MRI表现:T1WI上呈结节状不均匀低信号,T2WI上呈不均匀高信号.X线和MRI均显示邻近关节结构正常.结论:肿瘤样钙质沉着症的典型影像学表现为不侵及关节的软组织内多发钙化性肿块,平片和MRI有助于本病的诊断和鉴别诊断.     

稳定型与不稳定型心绞痛患者电子束CT 冠状动脉钙化检测的比较

【目的】比较稳定型心绞痛（SAP）与不稳定型心绞痛（UAP）患者冠状动脉(冠脉)钙化（CAC）的不同模式。【方法】67例SAP与94例UAP患者,行选择性冠脉造影和电子束CT（EBCT）检查,分析两组CAC积分（CS）的差异及其与冠脉狭窄程度之间的关系。【结果】①SAP组CAC检测阳性率（100%）显著高于UAP组（87.23%）（P<0.05）。②SAP组CAC积分显著高于UAP组,其自然对数转换值［ln（CS+1）］的均数按心脏整体计算为（5.24±1.72）对（3.13±1.96）（P<0.001）,按冠脉分支计算为（3.28±2.08）对（1.68±1.62）（P<0.001）。③在每一相同的年龄段内比较,SAP组的CAC程度均明显高于UAP组。④ln(CS+1)与冠脉狭窄程度在两组均呈正相关,但SAP组相关程度相对较好;SAP组重度狭窄的冠脉多发生重度或中度钙化,而UAP组重度狭窄的冠脉则多数无钙化或仅有轻度钙化。【结论】稳定型与不稳定型心绞痛患者冠脉钙化存在不同的模式,前者冠脉钙化大多较为严重,钙化积分与冠脉狭窄程度的相关性较好,而后者钙化程度多数较轻,钙化积分与冠脉狭窄程度的相关性很差。     

Bisacylphosphonates Inhibit Hydroxyapatite Formation and Dissolution in Vitro and Dystrophic Calcification in Vivo

Some geminal bisphosphonates are used clinically for a number of important bone/calcium related diseases; however, side effects and lack of selectivity impede their wide use. This work reports the synthesis and evaluation of bisacylphosphonates (e.g., adipoyl- and suberoylbisphosphonate). These compounds were found to inhibit significantly hydroxyapatite formation and dissolution in vitro and the calcification of bioprosthetic tissue implanted subdermally in rats. These are the first instances of nongeminal bisphosphonates [P–(C)n– P, n 2] that have been reported to be active in calcium-related disorders. The reported bisacylphosphonates possess apparent lower toxicity, and their calcium complexes/salts have improved solubility properties. Therefore, they are of potential importance for clinical applications.     

皮肌炎伴钙质沉着一例并国内外105例回顾分析

报道1例皮肌炎伴钙质沉着并总结相关文献。共分析总结105例皮肌炎伴钙质沉着患者,其中女59例,男46例,1~12岁患者最多,21%的患者皮损局限,以臀部、四肢为主。除典型结节、斑块外,3.8%的患者可形成钙性液体或钙化性囊性病灶,14.3%患者伴严重关节挛缩、畸形。皮肌炎伴钙质沉着多有危险因素,如持续活动性炎症反应、局部隐性损伤、抗NXP-2抗体阳性等。患者多采用多种药物联合,或者药物与外科手术联合治疗。TNF-α抑制剂等生物制剂正逐步应用于临床个案治疗。