肿瘤样脱髓鞘病3例临床病理分析

目的探讨肿瘤样脱髓鞘病临床和病理学特点。方法对3例肿瘤样脱髓鞘病患者的临床特征进行了分析,并对活检脑组织进行了组织病理学和免疫组化研究。结果3例患者均呈亚急性起病,临床表现各不相同,有复发缓解过程。头部MRI显示3例患者分别在额、顶叶及小脑半球单发大面积病变。组织病理学观察均显示中心区组织高度水肿,呈空泡样变,有囊性坏死区。白质髓鞘高度崩解,轴索相对保留完好。免疫组化染色显示大量炎性细胞主要是淋巴细胞及少量单核吞噬细胞围绕血管呈袖套样浸润。结论肿瘤样脱髓鞘病临床表现各异,当影像学表现为孤立、单发、巨大病灶时,应想到脱髓鞘疾病的可能,病理学检查对确定诊断有重要价值。     

Acute Ischemic Stroke Therapy in Infective Endocarditis: Case Series and Systematic Review

Objectives: To evaluate the safety of acute ischemic stroke (AIS) therapy in patients with infective endocarditis (IE) with intravenous thrombolysis (IVT) or endovascular therapy (EVT) such as mechanical thrombectomy. Methods: We conducted a retrospective study of patients who underwent AIS therapy with IVT or EVT at a tertiary referral center from 2013 to 2017, that were later diagnosed with acute IE as the causative mechanism. We then performed a systematic review of reports of acute ischemic reperfusion therapy in IE since 1995 for their success rates in terms of neurological outcome, and mortality, and their risk of hemorrhagic complication. Results: In the retrospective portion, 8 participants met criteria, of whom 4 received IVT and 4 received EVT. Through systematic review, 24 publications of 32 participants met criteria. Combined, a total of 40 participants were analyzed: 18 received IVT alone, 1 received combined IVT plus EVT, and 21 received EVT alone. IVT compared to EVT were similar in rates of good neurologic outcomes (58% versus 76%, P= .22) and mortality (21% versus 19%, P= .87), but had higher post-therapy intracranial hemorrhage (63% versus 18% [P= .006]). Conclusion: IV thrombolysis has a higher rate of post-therapy intracranial hemorrhage compared to EVT. EVT should be considered as first-line AIS therapy for patients with known, or suspected, IE who present with a large vessel occlusion.     

Alpha 1-antitrypsin deficiency associated with PZ and MP phenotypes. Clinical and laboratory correlations

In a large kindred we have identified two siblings with the hitherto unreported PZ phenotype and eight other subjects with the MP phenotype. In subjects with the MP phenotype serum alpha₁-antitrypsin levels are near the lower limits of normal. In contrast, subjects with the PZ phenotype have severely depressed alpha₁-antitrypsin levels. One subject with the PZ phenotype at age 34 already shows evidence of obstructive lung disease. We found no convincing evidence of obstructive lung disease in family members with the MP phenotype. After purification of alpha₁-antitrypsin from the serum, isoelectric focusing and acrylamide gel electrophoresis can be used to distinguish normal protein from the products of the Pi^P and Pi^Z alleles. Subjects with the PZ phenotype have more Pi^P than Pi^Z product.     

Cardioadrenergic factor in essential hypertension

Determination of STI in 54 untreated essential hypertensive subjects and 17 normal subjects revealed marked differences among three groups of patients. Those with borderline hypertension (29) had a short PEP and IVC periods (93 ± 2.1 and 28 ± 0.7 msec., respectively, p < 0.001) (mean ± S.E.) reduced $\text{PEP}\text{LVET}$ (0.323 ± 0.009, p < 0.05) and increased $\text{DP}\text{IVC}$ (3,484 ± 257 mm. Hg per second, p < 0.001). Among those with established hypertension, two groups of equal age and diastolic pressure were identified: nine with marked variations in blood pressure and a hyperkinetic heart clinically and 16 with fixed hypertension; none had cardiac or renal decompensation. Those with a hyperkinetic circulation had normal PEP, IVC, and $\text{PEP}\text{LVET}$ despite a high diastolic pressure (122 ± 7.1 mm. Hg); $\text{DP}\text{IVC}$ was elevated (3,651 ± 497 mm. Hg per second, p < 0.001) as in those with borderline hypertension. In contrast, the patients with fixed hypertension had longer PEP and IVC (p < 0.001), higher $\text{PEP}\text{LVET}$ (p < 0.001), and normal $\text{DP}\text{IVC}$. Propranolol (10 mg. intravenously) slowed heart rate and prolonged PEP and IVC more in patients with a hyperkinetic circulation and in those with borderline hypertension than in those with fixed hypertension.These results suggest the presence of an increased cardioadrenergic drive not only in borderline hypertension, but also in a subgroup of patients with established hypertension. Left ventricular hypertrophy (ECG) was found in 1 out of 9 patients with hyperkinetic heart but in 6 out of 16 with fixed hypertension; cardiac index was high normal in the first group but reduced in the latter (3.32 vs 2.38 L./min./M.², p < 0.001). This factor as determined by the systolic time interval might, therefore, be important in determining cardiac prognosis or planning therapy.     

Human growth hormone release. Comparison of provocative test procedures

Twenty normal adult volunteers were systematically tested with five known provocative agents of human growth hormone (HGH) release in order to ascertain which procedure was the most effective stimulus for pituitary testing purposes. Ninety-five per cent responded normally (5 ng/ml increment) to levodopa (l-dopa) and 90 per cent to insulin-hypoglycemia. Arginine, vasopressin and glucagon were less potent stimuli; however, arginine (80 per cent response rate) was superior to vasopressin and glucagon (60 and 55 per cent, respectively).Nine subjects were retested with the same stimuli. Again, the incidence of normal HGH responses was highest with the l-dopa and insulin tolerance tests (100 and 89 per cent, respectively). Results with arginine, vasopressin and glucagon were significantly less consistent.Because of the important additional features of greater simplicity and safety, and until hypothalamic releasing substances become generally available, the routine use of l-dopa as a pituitary test agent warrants great attention.     

A case series of LMWH use in pregnancy: Should trough anti-Xa levels guide dosing?

Introduction

Pregnancy is a thrombogenic state, increasing the risk for venous thromboembolism (VTE), and the risk of valve thrombosis amongst women with mechanical heart valves (MHV). While low molecular weight heparins (LMWH) are generally dosed based on weight (i.e., enoxaparin 1 mg/kg every 12 hours), data in pregnant women have shown that weight-based dosing does not consistently achieve target anti-Xa levels. In women with MHV, our practice includes titrating LMWH doses to target both trough and peak anti-Xa levels, while for those with VTE peak anti-Xa levels guide dosing.

Materials/Methods

This retrospective case series included pregnant women requiring LMWH treatment doses with at least 3 peak (+/− trough) anti-Xa levels. Our primary objective was to describe the actual LMWH dose required to achieve targeted anti-Xa levels relative to weight-based dosing in patients with MHV. Secondarily, we compared the same for VTE patients; compared actual dosing between those with MHV and VTE; and examined maternal and fetal outcomes.

Results/Conclusion

Women with MHV (N = 4) required greater than weight-based dosing of enoxaparin (1.35 mg/kg Q12H) to achieve targeted anti-Xa levels. Importantly, achieving target peak anti-Xa levels did not always ensure maintenance of minimum trough levels. VTE patients (N = 12) did not require more enoxaparin (0.96 mg/kg Q12H) than weight based dosing. MHV patients received more enoxaparin compared to VTE patients (P < 0.001). No bleeding or clotting complications were associated with LMWH administration. In pregnant women with MHV at high risk of thromboembolism, LMWH dosing guided by trough and peak anti-Xa levels should be considered.     

肥厚性硬脑膜炎的临床、病理和影像学特点 (附1例报告)

目的 研究肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床表现、病理和MRI特征.方法 对1例HCP患者的临床、病理和MRI检查资料进行回顾性分析并总结其特点.结果 HCP多慢性起病,临床以头痛和多颅神经麻痹为主要表现.MRI可见受累硬脑膜T1相呈等或略低信号,T2相呈高信号,增强扫描后增厚的硬脑膜明显均匀强化,硬脑膜病理检查可见纤维组织内炎症细胞浸润.激素治疗有效,MRI复查硬脑膜变薄,累及范围缩小,强化减轻.结论 HCP以头痛、多脑神经受累为主要临床表现.MRI扫描可见特征性的硬脑膜强化改变.病理学检查是确诊依据.     

首选多药联合治疗新诊断婴儿痉挛的临床疗效及随访研究

目的探讨首选多药联合治疗新诊断婴儿痉挛的疗效、对预后的影响及影响因素。方法 (1)收集自2007年9月～2010年11月本院收治的108例新诊断的婴儿痉挛患儿的病例资料,首选多药联合治疗,并进行追踪随访,对其临床资料回顾性研究。(2)治疗方案:采用ACTH、托吡酯、维生素B6、丙种球蛋白多药联合治疗。结果 (1)108例中90例完全控制,8例有效,10例无效,完全控制率83.3%,总有效率90.7%;(2)随访全部患儿5个月～3年,无1例死亡,痉挛发作完全控制者中复发率为7.8%(7/90);随访其中21例发作完全控制者入院时及痉挛发作完全控制后1个月时Gesell量表测定结果,无统计学意义,完全控制6个月时与治疗前Gesell量表测定结果比较,有统计学意义。结论 (1)多药联合治疗新诊断婴儿痉挛发作具有较高的完全控制率及有效率,症状性婴儿痉挛完全控制所需时间长于隐源性婴儿痉挛。(2)采用多药联合治疗新诊断婴儿痉挛可提高患儿智能,有助于改善预后。     

Intracerebral Hemorrhage in a Young Urban Population: Etiologies and Outcomes in Patients 50 and Younger

Goal: There is limited research on intracerebral hemorrhage in young urban populations. There is reduced access to healthcare and a high prevalence of multiple comorbidities in this vulnerable population. We studied the etiologies and outcomes of spontaneous intracerebral hemorrhage in an urban North Philadelphia cohort aged 50 years old and younger. Materials and methods: A retrospective chart review of subjects 50 years old and younger who presented with spontaneous intracerebral hemorrhage at Temple University Hospital was conducted. A novel scoring system was used to classify the cause of each intracerebral hemorrhage. This system was used to assign a degree of likelihood that hypertension, amyloid angiopathy, tumor, oral anticoagulants, vascular malformations, infrequent causes, or cryptogenic etiologies were present. Aneurysmal subarachnoid hemorrhage was excluded. The prevalence of each risk factor and outcomes were analyzed. Findings: Of the 110 patients in the study, the most common etiology was hypertension (82.7%). There was no statistically significant difference in mortality between patients with multiple possible etiologies for their hemorrhage. Vascular malformations and cavernomas were rare (5.5%). Conclusions: Hypertension was the most common cause of intracerebral hemorrhage in a young urban population. The presence of multiple possible etiologies does not correlate with a worse prognosis of mortality. There is a need for further research into hemorrhagic stroke in young populations.