Hemichorea associated with polycythaemia vera

Abstract Chorea is a rare complication of polycythaemia vera. Polycythaemic chorea occurs predominantly in females and usually in generalised form. We present a 66-year-old woman with acute onset hemichorea-ballism with no vascular pathology in the basal ganglia region. A clear relationship was observed between the onset of chorea and worsening of haematological parameters in the patient. After repeated phlebotomies the patient's clinical status was improved. Polycythaemic chorea must be considered, especially in the elderly, as early diagnosis leads to effective treatment and prevention of complications.     

A phase II study of sulofenur,a novel sulfonylurea,in recurrent epithelial ovarian cancer

Summary A total of 16 patients with recurrent epithelial ovarian cancer were treated with sulofenur (LY 186641), a novel oral sulfonylurea. All subjects had received previous chemotherapy. Anaemia occurred in all 16 patients, 14 of whom required a blood transfusion, and 2/16 patients received methylene blue for breathlessness due to methaemaglobinaemia. Treatment was discontinued in 2/16 cases due to rising liver enzyme values, which reverted to normal on cessation of the drug. There was no nausea or alopecia. Only two minor responses were seen. Plasma drug levels were insufficient to result in antitumour activity as extrapolated from animal data. Further studies that attempt to increase the bioavailability and improve the therapeutic index are warranted.     

COVID-19 Treatment: Drug Safety Prior to Conception and During Pregnancy and Breastfeeding

In December 2019, a new viral respiratory infection known as coronavirus disease 2019 (COVID-19) was first diagnosed in the city of Wuhan, China. COVID-19 quickly spread across the world, leading the World Health Organization to declare it a pandemic on March 11, 2020. The disease is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a similar virus to those involved in other epidemics such as severe acute respiratory syndrome coronavirus (SARS-CoV) and Middle East respiratory syndrome coronavirus (MERS-CoV). Epidemiological studies have shown that COVID-19 frequently affects young adults of reproductive age and that the elderly and patients with chronic disease have high mortality rates. Little is known about the impact of COVID-19 on pregnancy and breastfeeding. Most COVID-19 cases present with mild flu-like symptoms and only require treatment with symptomatic relief medications, whereas other cases with COVID-19 require treatment in an intensive care unit. There is currently no specific effective treatment for COVID-19. A large number of drugs are being used to fight infection by SARS-CoV-2. Experience with this therapeutic arsenal has been gained over the years in the treatment of other viral, autoimmune, parasitic, and bacterial diseases. Importantly, the search for an effective treatment for COVID-19 cannot expose pregnant women infected with SARS-CoV-2 to the potential teratogenic risks of these drugs. Therefore, it is necessary to determine and understand the safety of anti-COVID-19 therapies prior to conception and during pregnancy and breastfeeding.Key words: COVID-19, SARS-CoV-2, antiviral, pregnancy, breastfeeding     

Myoblast transplantation can repair heart damage

Cell and gene based therapies hold tremendous po- tential to treat cardiovascular diseases. However, signifi- cant obstacles need to be overcome before any realization of potential benefits. There is no doubt that intracoronary or direct injection of either cells or genes into small animal models (mouse, rat, hamster or rabbit) for heart disease pro- duce improvements in cardiac function.1-4 The challenges, however, to treat human heart disease are far greater than in experimental animal syste…     

Increased circulating CSF-1 (M-CSF) in myeloproliferative disease: association with myeloid metaplasia and peripheral bone marrow extension

Myeloproliferative disease (MPD) is heterogeneous in phenotypic expression and may display features consistent with expansion and activation of the monocyte/macrophage population during its course. The role of colony-stimulating factor-1 (CSF-1) in the pathophysiology of MPD was investigated by measuring circulating CSF-1 levels and examining their relationship to disease phenotype. Serum CSF-1 concentrations, measured by radioimmunoassay, were elevated in all MPD phenotypes. CSF-1 levels differed significantly between groups of patients with essential thrombocythemia, polycythemia vera, and postpolycythemic or agnogenic myeloid metaplasia (in ascending order). CSF-1 serum levels were positively correlated with spleen size and the degree of peripheral bone marrow extension, determined by scintigraphy using a macrophage-seeking isotope. There was no correlation between CSF-1 concentration and circulating levels of erythrocytes, neutrophils or platelets, or the presence of bone marrow fibrosis. Elevated serum CSF-1 levels appear to be associated with an expanded monocyte/macrophage population in MPD. In view of the known cooperativity between CSF-1 and other growth factors in regulating hematopoiesis, the finding of increased serum CSF-1 concentrations and its association with myeloid metaplasia and bone marrow extension may indicate a pathophysiologic role for CSF-1 in determining the phenotypic expression of MPD.     

动态监测血清相关指标对重型颅脑损伤术后肺部感染的预测价值

目的分析动态监测重型颅脑损伤术后患者血清降钙素原(PCT)、可溶性髓样细胞表达的激发受体-1(sTREM-1)及C反应蛋白与白蛋白比值(CRP/ALB)的变化对肺部感染的早期预测价值。方法选取手术治疗的196例重型颅脑损伤患者,监测并记录术后1、3、5 d的血清PCT、CRP、ALB、sTREM-1及CRP/ALB水平,记录患者术后肺部感染情况。分析重型颅脑损伤术后肺部感染的危险因素,通过绘制受试者工作特征(ROC)曲线分析相关指标对肺部感染的早期预测价值。结果重型颅脑损伤术后发生肺部感染者76例(38.78%),感染发生时间为术后6~13 d,中位数为7 d。无肺部感染组术后3、5 d血清PCT、sTREM-1及CRP/ALB低于肺部感染组,差异有统计学意义(P0.05);肺部感染组术后5 d血清ALB低于无肺部感染组,差异有统计学意义(P0.05)。机械通气时间、术后格拉斯哥昏迷量表(GCS)评分、术后急性生理与慢性健康评分Ⅱ(APACHEⅡ)以及术后5 d血清PCT、sTREM-1及CRP/ALB是术后肺部感染的独立危险因素(P0.05)。ROC曲线显示,单独检测时,术后5 d血清CRP/ALB预测肺部感染的曲线下面积(AUC)值、约登指数、特异度最高,sTREM-1预测的敏感度最高;联合检测的AUC、约登指数、灵敏度、特异度均高于单独检测。肺部感染诊断时临床肺部感染评分(CPIS)、全身炎症反应综合征修正(ASS)评分均与术后5 d PCT、sTREM-1、CRP/ALB水平呈显著正相关(P0.05)。结论术后5 d血清PCT、sTREM-1及CRP/ALB水平均可作为重型颅脑损伤术后肺部感染早期预测的有效依据,而联合检测的预测价值更高,更有利于早期对肺部感染程度及病情进展的评估。     

22例皮质醇增多症的临床研究

目的：探讨皮质醇增多症的临床表现和内分泌检查等辅助检查的意义.方法：从年龄、性别、病程及实验室检查等方面,观察22例不同原因所致皮质醇增多症患者不同的临床表现和测定实验室检查指标.结果：22例中诊断库欣病（增生型）14例[63.6％,其中13例（92.9％）得到MRI检查证实],肾上腺腺瘤6例[27.3％,均得到MRI检查证实（100％）],另有肾上腺结节样增生1例（4.5％）,异位ACTH综合征1例（4.5％）.临床表现：按出现的频率前4位依次为,库欣病：高血压（100％）、满月脸（92.9％）、向心性肥胖（85.7％）、多血质（85.7％）,肾上腺腺瘤：高血压（100％）、满月脸（100％）、向心性肥胖（100％）、多血质（83.3％）.实验室检查：小剂量地塞米松不能抑制：库欣病与肾上腺腺瘤均为100％.结论：根据高血压、满月脸、向心性肥胖,小剂量地塞米松抑制试验和MRI检查可诊断绝大多数皮质醇增多症.     

Hypoplastic right-heart syndrome presenting as multiple miscarriages

Reversible causes of miscarriage are many, but they affect only 1% of women who are trying to conceive. Herein, we describe the case of a 23-year-old woman who presented for evaluation of repeated miscarriages and was found to have hypoxemia and erythrocytosis. Further evaluation revealed hypoplastic right-heart syndrome with an intracardiac shunt. She underwent hybrid repair with pulmonary valve balloon valvuloplasty, followed by surgery to perform atrial septal defect closure and a Glenn anastomosis. The erythrocytosis and hypoxemia resolved, and she was able to conceive and deliver a healthy baby at term 2 years later. This is a unique case of a rare congenital heart defect that went unnoticed until adulthood, when attempts at pregnancy failed because of the associated hypoxemia. Timely and appropriate treatment led to a successful pregnancy after repeated miscarriages. This case exemplifies the need for a comprehensive medical evaluation of every woman with a history of multiple miscarriages to determine whether a reversible cause exists.