儿童指突状树突细胞肉瘤合并骨髓增生异常综合征一例报告并文献复习

指突状树突细胞肉瘤(interdigitating dendritic cell sarcoma,IDCS)是一种罕见的树突状细胞肿瘤,目前全球仅百余例报道,常以无痛性淋巴结肿大起病,侵袭性较强、预后较差[1-2]。骨髓增生异常综合征(myelodysplastic syndromes,MDS)为起源于造血干、祖细胞的恶性克隆性疾病,以单系或多系病态造血、易向白血病转化为特征,目前被认为是一种老年性疾病[3]。本研究报道1例同患IDCS和MDS的患儿,为国内外首次报道2种肿瘤同时发生,旨在探讨2种肿瘤的诊治要点,避免漏诊、误诊。     

Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome: A case report

BACKGROUNDAdrenal incidentaloma (AI) has been frequently encountered in the clinical setting. It has been shown that primary aldosteronism (PA) or subclinical Cushing’s syndrome (SCS) are the representative causative diseases of AI. However, the coexistence of PA and SCS has been reportedly observed. Recently, we encountered a case of AI, in which PA and SCS coexisted, confirmed by histopathological examinations after a laparoscopic adrenalectomy. We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARYA 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg. A subsequent computed tomography scan revealed right ureterolithiasis, which was the cause of right abdominal pain, and right AI measuring 22 mm × 25 mm. After the disappearance of right abdominal pain, subsequent endocrinological examinations were performed. Aldosterone-related evaluations, including adrenal venous sampling, revealed the presence of bilateral PA. In addition, several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma. A laparoscopic right adrenalectomy was then performed. The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma, while CYP11B2 immunoreactivity was absent in this adenoma. However, in the adjacent non-neoplastic adrenal, multiple CYP11B2-positive adrenocortical micronodules were detected, showing the presence of aldosterone-producing adrenocortical micronodules. CONCLUSIONCareful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.     

约束隐结构研究冠心病痰湿证的定量化辨证规则＊

目前在中医界已发布的冠心病痰湿证辨证标准是以主症、次症形式定性地给出，存在主观性较强的问题。本文引入约束隐结构分析，该方法将主症、次症的语义作为约束条件加入隐结构分析过程，得到含有主症、次症语义约束的定量化中医证候辨证规则。使用该方法对冠心病痰湿证患者556条无标签数据的分析，得到其约束隐结构模型，最后建立定量化痰湿证辨证规则，舌胖边有齿痕（3.16）、苔腻（3.12）、苔白滑（4.72）、胸闷（1.73）、脉濡或滑（6.04）；次症：肢体困重（0.48）、口黏（0.63）、体胖（0.49）、大便粘滞（1.38）、脘腹痞满（0.97）、面色晦浊（0.79）、嗜睡（1.18）、纳差（1.07）。与经典隐结构模型得到规则和中医界已发布的定性化辨证规则相比，约束隐结构得到的规则客观性强，具有可重复性。在证候类大小、规则的量化合理度上较好地反映了主症、次症的特点，得到的规则切合中医实际，为冠心病痰湿证辨证标准的定量化研究提供帮助和参考。     

内囊预警综合征的临床特征和预后

目的 分析内囊预警综合征（capsular warning syndrome，CWS）的临床特点、影像学特征及治疗预后，
以提高对此病的认识。
方法 回顾性分析战略支援部队特色医学中心神经内科2013年1月-2018年12月收住院治疗的CWS的
临床资料、影像学特征及治疗预后情况。
结果 共12例患者，男性8例（66.67%），中位年龄为60岁。高脂血症9例（75.0%），高血压8例
（66.67%），糖尿病4例（33.33%），肿瘤病史3例（25.0%）。纯运动性卒中4例（33.33%），运动伴有构
音障碍者4例（33.33%），运动及感觉性障碍3例（25.00%），纯感觉障碍1例（8.33%）。影像学示豆纹
动脉供血区新发脑梗死6例（50%）。所有患者给予口服双联抗血小板治疗，其中3例症状复发患者又
给予静脉溶栓和静脉抗血小板治疗。3个月随访，所有患者均未再出现症状发作，10例mRS评分为0
分，2例mRS评分分别为4分及3分。
结论 CWS主要表现为运动障碍，发作刻板，常见原因为动脉粥样硬化性。在本组病例中发现双重
抗血小板治疗有效，静脉溶栓效果不肯定。     

A study of unprotected left main intervention in the ACS population 2013–2018

ObjectivesOur objectives were to evaluate the outcomes of left main percutaneous coronary interventions in Acute Coronary Syndrome population.MethodsThis is a retrospective& observational study. Primary endpoint is a composite of death, stent thrombosis/MI, target lesion revascularization. Secondary endpoints include individual components of the primary events analyzed separately.ResultsSeventy five patients, two year follow – up data was analyzed. The primary event analysis showed that the Elective Double Stent (EDS) group had a higher primary events (36% vs. 14%, p value – 0.008, Hazard ratio – 0.76 (0.51–1.15, 95% CI), in secondary event analysis stent thrombosis (ST)/Myocardial infarction (MI) rates were higher in EDS group (8% Vs 36%, p Value – 0.008, Hazard ratio- 0.63(0.35–1.14, 95%CI), there is no difference in target lesion revascularization (TLR)and death rates in both the groups.ConclusionsThe provisional strategy is better than EDS in treatment of left main bifurcation lesions in the ACS population.     

Thymic epithelial tumor with superior vena cava syndrome diagnosed by percutaneous endovascular tumor biopsy: A report of two cases

Thymic epithelial tumor is a rare, potentially progressive disease that commonly infiltrates mediastinal structures. In rare cases, it may cause superior vena cava syndrome. Pretreatment histopathological diagnosis is essential to determine the most effective treatment strategy. Percutaneous endovascular biopsy is a rarely reported non-surgical diagnostic option for large vessel tumoral involvement. We report two cases of thymic epithelial tumor with superior vena cava syndrome diagnosed by percutaneous endovascular biopsy. No procedural complications occurred, and subsequent systemic treatment was promptly administered. This procedure may have potential as a useful diagnostic method for patients with mediastinal tumors involving large vessels.     

Mortality prediction in chronic obstructive pulmonary disease and obstructive sleep apnea

BackgroundWe aimed to assess mortality in chronic obstructive pulmonary disease (COPD), obstructive sleep apnea (OSA), and overlap syndrome, and evaluate which polysomnographic indices—apnea-hypopnea index (AHI) or hypoxemic load measurements—better predict mortality within 10 years.MethodsAdults with symptoms suggestive of sleep apnea and airway disease who underwent both polysomnography and spirometry plus bronchodilator response tests between 2000 and 2018 were included and divided into four groups according to presence of COPD and moderate-to-severe OSA (AHI ≥15/h). We estimated mortality using a Cox model adjusted for demographic/anthropometric covariates and comorbidities; this was called clinical model. To evaluate prognostic performance, we compared the concordance index (C-index) between clinical model and extended models, which incorporated one of polysomnographic indices—AHI, sleep time spent with SpO₂ < 90% (TS90), and mean and lowest SpO₂.ResultsAmong 355 participants, patients with COPD alone (57/355, 16.1%) and COPD–OSA overlap syndrome (37/355, 10.4%) had increased all-cause mortality than those who had neither disease (152/355, 42.8%) (adjusted HR, 2.98 and 3.19, respectively). The C-indices of extended models with TS90 (%) and mean SpO₂ were significantly higher than that of clinical model (0.765 vs. 0.737 and 0.756 vs. 0.737, respectively; all P < 0.05); however, the C-index of extended model with AHI was not (0.739 vs. 0.737; P = 0.15).ConclusionsIn this cohort with symptoms of sleep apnea and airway disease, patients with overlap syndrome had increased mortality, but not higher than in those with COPD alone. The measurement of hypoxemic load, not AHI, better predicted mortality.