肿瘤样钙质沉着症

报告1例肿瘤样钙质沉着症.患儿男,8岁.全身结节、肿块1年余.皮肤科检杳:颈部、躯干、四肢、臀部及手足可见约百个大小不一的结节和肿块,部分融合,边界清晰,少数包块顶端破溃,流出石灰状物质.皮损组织病理检查示真皮及皮下组织大量颗粒状灶性钙沉积.诊断:肿瘤样钙质沉着症.     

Idiopathic Calcinosis of the Scrotum: Scanning Electron Microscopic Study with X-Ray Microanalysis

In a 31-year-old man 12 nodules up to 1 cm in diameter were observed in the scrotum; these had developed over 3 years. Energy-dispersive x-ray analysis of the dermal foci showed a high content of calcium and phosphorus even in nodules smaller than 1 mm in diameter. No increased mineral deposition was observed in the surrounding connective tissue, however. Scanning electron microscopy revealed finely granulated crystals, and cellular remnants giving rise to development of calcified nodules were disclosed. The results support the hypothesis of a degenerative origin of scrotal calcinosis.     

Fine needle aspiration cytology in tumoral calcinosis cases ＆ review of literature

Objective:To investigate them by the non-invasive technique of fine needle aspiration cytology(FNAC).Methods: In this study cases were described in which FNAC was indicative of tumoral calcinosis.Total numbers of cases studied were 18.Male to female ratio was 1∶8.11 cases(61.11%) were less than 20 years of age.3 cases had history of trauma in the past(16.67%).8 cases had lesions located in the hip region(44.44%).Results:The size of lesion varied from 2.5 to 4 cm.In none of the case diagnosis of tumoral calcinosis was considered clinically.All other investigations were normal and no significant family or medical history was present.Cytology in all cases showed only abundant acellular calcium.The patients on follow up were clinically well with no changes.Conclusion:The cases are interesting,since the cytohistological findings in the aspirate sample are strongly indicative of tumoral calcinosis.     

脂蛋白a水平及心脏超声心动图钙化评分与冠心病的相关性

目的 评价血清脂蛋白a[Lp（a）]水平及心脏超声心动图钙化评分（eCS）与冠心病（CHD）的相关性。方法 纳入229例CHD患者,根据病变累及范围分为单支亚组（n=87）、双支亚组（n=47）及三支亚组（n=95）;以同期113名经冠状动脉CT血管造影排除CHD的志愿者作为对照组。比较组间及亚组间基本资料、实验室指标及eCS差异,结合eCS及Lp（a）水平进行风险（低、中、高）分层;采用Logistic多因素回归分析评价各指标与CHD的相关性。结果 CHD组与对照组之间,性别、年龄、吸烟史、糖尿病史、高血压病史、空腹血糖、Lp（a）、高密度脂蛋白、载脂蛋白A及肌酐水平差异均具有统计学意义（P均<0.05）。CHD组eCS ≥ 1分者占比（176/229,76.86%）明显高于对照组（24/113,21.24%）,且CHD组各亚组eCS与对照组差异均有统计学意义（P均<0.05）。CHD组各亚组Lp（a）水平均明显高于对照组（P均<0.05）。CHD组与对照组风险分层差异有统计学意义（P<0.05）。Logistic回归分析显示糖尿病史、eCS和/或Lp（a）水平是CHD的独立危险因素。结论 eCS及Lp（a）水平是CHD的独立危险因素,可作为筛查CHD的良好指标。     

钙的异常骨内沉积与甲状旁腺激素

目的探讨甲状旁腺功能减退钙的异常骨内沉积的临床和影像学表现。方法报告甲状旁腺功能减退钙的异常骨内沉积11例,对其临床表现和骨关节CT、X线检查进行分析。结果11例患者有不同程度的骨内钙质沉积。临床主要症状为手足抽搐、惊厥、癫痫等。生化检查表现为血清甲状旁腺激素浓度降低、低钙血症和高磷血症。CT和放射学表现为广泛性或局限性骨质增生硬化、弥漫性骨肥厚、骨皮质和骨小梁的骨矿物质密度增高、颅骨增厚、长骨干骺端骨密度增高、骶髂关节和髋关节骨硬化、儿童骺软骨和骺板的早期骨化、生长障碍线再现、与椎骨终板平行的“骨中骨”致密线、关节韧带和肌腱的骨化及软组织钙化等。结论骨关节CT和X线检查对甲状旁腺功能减退骨内钙质沉积的诊断与鉴别诊断很有价值。     

乙醇、丁二醇改性对牛颈静脉的抗钙化作用

目的探讨两种醇类改性对牛颈静脉带瓣管道的抗钙化作用。方法将戊二醛固定的牛颈静脉带瓣管道分别用80%乙醇(A组)和100%2,3-丁二醇(B组)进行化学改性,分别将两组管壁片及瓣膜片植入12只刚离乳的SD大鼠背部两侧皮下,90 d后处死大鼠,取出植入的组织片进行组织钙质量分数、总蛋白水平测定与光镜、电镜检查。结果A组的牛颈静脉管壁及瓣膜组织钙质量分数分别为(173±61)μg/m g和(2.13±0.85)μg/m g,B组钙质量分数分别为(181±29)μg/m g和(1.73±1.20)μg/m g,两组比较,均无显著性差异(P>0.05);组织学观察显示两组改性管壁及瓣膜组织炎性细胞浸润较少,胶原纤维结构保持良好。A组管壁及瓣膜片总蛋白水平分别为(337±293)m g/L和(142±82)m g/L,B组管壁及瓣膜片总蛋白水平分别为(269±230)m g/L和(139±111)m g/L,两组比较,均无显著性差异(P>0.05)。结论两种醇类均可作为牛颈静脉带瓣管道的改性剂;生物材料钙化与蛋白吸附相关;丁二醇及乙醇改性减少了生物材料的蛋白吸附,从而增强其抗钙化性能。     

Clericuzio‐type poikiloderma with neutropenia in a patient from India

A 9‐year‐old boy presented for evaluation of variegated skin pigmentation. Palms and soles revealed honeycombed hyperpigmented hyperkeratosis. Irregular, firm, skin coloured nodules suggestive of cutaneous calcification were present on both elbows. Total leucocyte count and absolute neutrophil count were 3720/mm3 and 420/mm3 respectively. The neutropenia was not cyclical. Systematic analysis of the whole exome data revealed a homozygous mutation in USB1 gene; chr16:g.58043892TA>‐[1/1]. A final diagnosis of poikiloderma with neutropenia‐ Clericuzio type (PNC) was made. Naegeli Franceschetti Jadassohn, dermatopathia pigmentosa reticularis, PNC and dyskeratosis congenita, all can present with overlapping cutaneous manifestations. Subtle clinical details like thickened nails, hyperextensible joints, calcinosis cutis, characteristic facies and a preceding erythematopapular rash strongly favor the diagnosis of PNC. The index case highlights two novel findings: obliterated dermatoglyphics and mucin deposition (features not described hitherto in PNC).     

The impact of skin calcinosis on digital ulcers in patients with SSc: clinical and prognostic stratification using the “wound bed score”

Digital ulcers (DUs) represent one of the major burdens for patients with systemic sclerosis (SSc), especially when associated with skin calcinosis (SC). The aim of this work is to evaluate the impact of SC in DUs of patients with SSc for clinical characteristics and prognosis assessed by the wound bed score (WBS). We prospectively enrolled 55 patients with DUs and SSc followed in our dedicated wound care clinic. For all the patients we collected clinical and anthropometric data and characteristics of the DU, and we calculated the WBS for each DU. Ninety‐nine DUs were evaluated (24 with SC). SC was prevalent in limited cutaneous SSc (75%) and in patients with longer disease duration (P = 0.02). SC‐DUs were prevalent at the fingertip (P = 0.04). The healing time was significantly higher in patients with SC (10.4 ± 7.9 weeks) compared with non‐SC (7.0 ± 5.7 weeks) P = 0.03. The WBS negatively correlated with the time to achieve complete healing (r = −0.237 P = 0.023) and the correlation was maintained in the non‐SC (r = −0.46, P = 0.033). DUs in SSc patients with SC are common and difficult to heal. When DUs are treated in dedicated centres, the prognosis is good. The WBS is fast and easy and maybe commonly applied in clinical practice.