Hepatic Function and Fibrinolysis in Patients with Hereditary Angioedema Undergoing Long-Term Treatment with Tranexamic Acid

Prophylactic treatment with antifibrinolytic agents, epsilon-aminocapriod and tranexamic acid, reduces the incidence and severits of attacks in patients with hereditary angioedema. Long-term ellectivenessor risk of antifibrinolytic agents has not been established. Sixteen patients needing continuous prophylaxis because of frequency and severity of attacks were treated with tranexamic acid. In four patients this treatment was ineffective and the drug was withdrawn after 2 months. A remission or reduction in the frequency or serverity of attacks was observed in 12 patients treated for a period ranging from 8 to 34 months. Hepatic tests and blood fibrinolytic activity were not influenced by long term oral treatment with tranexamic acid.     

Treatment of hereditary angioedema

Summary The purpose of this study was to report the results of different treatments in 20 patients with hereditary angioedema. Effectiveness of tranexamic acid in preventing swellings was evaluated in 15 patients: in all but 3 subjects tranexamic acid was effective without serious side effects. 15 severe attacks of edema were managed with intravenous infusions of either kallikrein inhibitor (8 cases) or concentrate of C 1 esterase inhibitor (7 cases). In only 1 case was the kallikrein inhibitor unsuccessful. C 1 esterase inhibitor concentrate proved highly effective in the treatment of acute attacks (the result was lacking in one patient because of too low dosage of the drug). No side effects were observed with both treatments, but improvement was more rapidly achieved with infusion of C 1 esterase inhibitor. The serum levels of C 4 and C 1 esterase inhibitor and the activity of C 1 esterase inhibitor before and after long-term prophylaxis and acute attacks treatment were investigated.     

Cimetidine and tranexamic acid in the treatment of acute upper-gastrointestinal-tract bleeding

We studied the effects of tranexamic acid (an antifibrinolytic agent) and cimetidine on acute upper-gastrointestinal-tract bleeding in a double-blind randomized placebo-controlled trial in 775 patients with hematemesis or melena or both. Mortality was significantly reduced in patients receiving either tranexamic acid (mortality, 6.3 per cent) or cimetidine (7.7 per cent), as compared with patients receiving placebo (13.5 per cent) (P = 0.0092 for tranexamic acid vs. placebo, P = 0.045 for cimetidine vs. placebo). Ninety-nine patients were withdrawn before the code was broken, mainly because their primary illness was considered not to be due to acute upper-gastrointestinal-tract bleeding. Mortality among those withdrawn was high (22 per cent), and their exclusion reduced death rates to 4 per cent in those given tranexamic acid, 8 per cent in those given cimetidine, and 11 per cent in those given placebo (P = 0.0072 for tranexamic acid vs. placebo, P greater than 0.50 for cimetidine vs. placebo). The reduced mortality associated with tranexamic acid was detectable at both participating hospitals and in most of the main subgroups of patients classified according to site of bleeding. However, treatment with this agent was not associated with any decrease in the rate of rebleeding or the need for operation.     

静脉输注氨甲环酸对全膝关节置换后隐性失血的影响

背景：人工全膝关节置换过程中,止血带的使用、手术创伤等因素会导致纤溶系统的异常激活,是术后失血的主要原因。氨甲环酸是一类抗纤溶药物,在人工全膝关节置换中使用,可减少置换后显性失血、总失血量,并降低异体血输血率。然而,氨甲环酸对人工全膝关节置换后隐性失血的影响尚未明确。 目的：观察静脉输注氨甲环酸对初次全膝关节置换后隐性失血的影响。 方法：回顾性分析2013年6至12月北京大学第三医院行初次单侧全膝关节置换54例患者的临床资料,按照是否使用氨甲环酸分为两组。氨甲环酸组22例在术中经静脉给予总量2 g的氨甲环酸,对照组32例使用等量生理盐水。两组患者置换后均口服利伐沙班抗凝。记录患者置换前及置换后连续5 d的血红蛋白、血红细胞压积,采用Gross方程计算总失血量和隐性失血量,比较两组间失血量的差异性。置换后1周行下肢静脉超声检查,判断有无下肢深静脉血栓形成。 结果与结论：两组患者一般资料、围术期情况等比较差异均无显著性意义(P > 0.05)。氨甲环酸组患者置换后引流量、显性失血量、总失血量、自体血回输量、异体血输注量均明显少于对照组,差异有显著性意义(P < 0.05)。根据Gross方程计算隐性失血量,氨甲环酸组为(302.9±189.9) mL,对照组为(596.8±271.4) mL,差异有显著性意义(P < 0.05)。置换后两组各发生1例下肢肌间静脉血栓。提示静脉输注氨甲环酸能显著减少单侧全膝关节置换后隐性失血量,降低异体血输血量,同时不增加下肢静脉血栓的发生率。中国组织工程研究杂志出版内容重点：人工关节;骨植入物;脊柱;骨折;内固定;数字化骨科;组织工程     

Efficacy of an air-cleaning device equipped with a high efficiency particulate air filter in house dust mite respiratory allergy

The efficacy of an air-cleaning device equipped with a high efficiency particulate air (HEPA) filter (without further avoidance measures) was studied in patients allergic to house dust mite. The effects of the air-cleaner on indoor Dermatophagoides sp. levels, symptom score and bronchial hyperresponsiveness in nine mite-allergic patients were assessed using a cross-over controlled study. No significant effect was demonstrated on indoor Dermatophagoides sp. levels when comparing the period of air-cleaner activity (2 months) with the control period (2 months). The Dermatophagoides sp. levels in the houses studied were lower than the risk level for asthmatic attacks, making it difficult to assess any effect on asthma; however, neither bronchial hyperresponsiveness nor rhinitis symptom score were changed by air-cleaner activity. During the trial period, however the mean level of Dermatophagoides sp. allergen in the houses changed spontaneously from 4.4 micrograms/g (mean level in the first 2 trial months) to 1.75 micrograms/g of dust (second 2 months) (P less than 0.05). Owing to this change, the mean rhinitis symptom score also decreased (P less than 0.05), even if no significant correlation was demonstrated (r = 0.4 P = 0.089). HEPA filter air-cleaners appear insufficient as substitutes for standard avoidance measures in mite allergic patients.     

Tranexamic acid: Preoperative prophylactic therapy for patients with hereditary angioneurotic edema

Short-term therapy of 96-hr duration with tranexamic acid was prophylactically effective as defined by the absence of attacks of angioedema in 14 patients with hereditary angioedema undergoing 10 dental and 4 general surgical procedures. Eight of the 14 patients had previously undergone dental extractions without prophylactic therapy with antifibrinolytic agents and each had experienced one or more attacks of angioedema. Seven of these 8 patients had a cumulative experience of 13 episodes of laryngeal edema after dental extractions and the eighth had a bout of cutaneous angioedema. Although the number of dental extractions conducted without prophylactic antifibrinolytic therapy cannot be accurately defined in retrospect, the prominence of laryngeal edema in this circumstance is striking when compared with the absence of attacks in the presence of prophylaxis with tranexamic acid. Methyltestosterone and impeded androgens are now known to be effective prophylaxis for spontaneous and, presumably, postoperative attacks when employed chronically because their administration is associated with correction of the biochemical defect of hereditary angioneurotic edema, but their chronic administration to children and women of childbearing age requires further definition because of their potential pituitary suppressive action. Tranexamic acid prophylaxis makes it possible to offer to untreated patients with hereditary angioneurotic edema dental work and other operative procedures that in the past were withheld or conducted with considerable risk.     

氨甲环酸静脉或局部及联合应用初次单侧全髋关节置换：围置换期出血及输血率的比较

BACKGROUND: The tranexamic acid has been widely used in hip arthroplasty. Studies have confirmed that the safety and efficacy of tranexamic acid when it is used in topical or intravenous application, but the effects are not clear when it is used in both topical and intravenous application. OBJECTIVE: To compare perioperative blood loss and transfusion rate in primary unilateral total hip arthroplasty with topical, intravenous and both injection of tranexamic acid.  METHODS: 136 patients undergoing primary unilateral total hip arthroplasty in the First Affiliated Hospital, Xinjiang Medical University between July 2014 and June 2015 were divided into three groups. Topical group: a total of 1 g tranexamic acid was given in the articular cavity of 44 cases. Intravenous group: 1 g tranexamic acid was immersed in 250 mL physiological saline, and given 10 minutes before operation in 44 cases. Combined group: 1 g tranexamic acid was intravenously given before operation, and then given in the articular cavity in 48 cases. Total blood loss, transfusion rate, the maximum hemoglobin decrease, the maximum hematocrit decrease, and incidence of deep vein thrombosis were compared among the three groups.  RESULTS AND CONCLUSION: Total blood loss, the maximum hemoglobin decrease and the maximum hematocrit decrease were significantly lower in the combined group than in the topical group and intravenous group (P < 0.01). No significant difference in total blood loss, the maximum hemoglobin decrease and the maximum hematocrit decrease was detected between the topical group and intravenous group (P > 0.05). Doppler ultrasound examination of lower limb blood vessel at 5 days and 1 month after surgery in 136 patients did not demonstrate deep vein thrombosis. None of them suffered from pulmonary embolism. These findings confirm that intravenous and topical application of tranexamic acid could obviously reduce blood loss in patients with total hip arthroplasty. Curative effect and safety were superior to intravenous or topical application alone. No significant difference in transfusion rate was detected among the three methods.      

氨甲环酸联合不同抗凝药对全膝关节置换术围手术期失血量的影响

目的 探讨氨甲环酸与不同抗凝药联用对全膝关节置换术围手术期失血量的影响。 方法 纳入2014年10月至2019年10月初次行全膝关节置换术并符合标准的158例膝骨性关节炎患者,按氨甲环酸注射方法及术后使用抗凝药分为4组：A组,术中静脉注射氨甲环酸＋术后利伐沙班抗凝;B组,术中静脉注射氨甲环酸＋术后依诺肝素抗凝;C组,术中静脉＋局部注射氨甲环酸＋术后利伐沙班抗凝;D组,术中静脉＋局部注射氨甲环酸＋术后依诺肝素抗凝。各组患者一般情况、术前准备、手术方式及术后处理一致,比较其失血量、凝血功能、输血及围手术期不良事件的发生率。 结果 联合使用氨甲环酸的患者能有效控制总出血量、显性出血量、输血率及血浆D二聚体;术后使用利伐沙班或依诺肝素抗凝,出血量、输血率及围手术期不良事件发生率相当。 结论 TKA术中应用氨甲环酸静脉＋局部注射能有效减少围手术期的失血量、输血率及血浆D二聚体;术后使用利伐沙班或依诺肝素进行抗凝,两者对失血量及围手术期不良事件发生率的影响无明显差异。     

Outcome in patients with asymptomatic neck bruits

Five hundred asymptomatic patients with cervical bruits were followed prospectively by clinical and Doppler examination for up to four years (mean, 23.2 months) to identify the variables predicting outcome. Thirty-six patients had strokes or transient ischemic attacks, 51 had cardiac ischemic events, and 45 died. At one year the incidence of cerebral ischemic events (transient ischemic attacks and strokes) was 6 percent, that of cardiac ischemic events was 7 percent, and that of death was 4 percent. The overall incidence of stroke at one year was 1.7 percent (1 percent in patients without previous transient ischemic attacks), but the incidence was 5.5 percent in patients with severe carotid-artery stenosis (greater than 75 percent). Cerebral ischemic events were most frequent in patients with severe carotid-artery stenosis (P less than 0.0001), progressing carotid-artery stenosis (P less than 0.0005), or heart disease (P less than 0.0005) and in men (P less than 0.025). The degree of carotid-artery stenosis on initial presentation was a powerful predictor of neurologic sequelae. Patients with asymptomatic cervical bruits have a higher risk of a cardiac ischemic event than of a stroke. Although the risk of cerebral ischemic events is highest in patients with severe carotid-artery stenosis, in most instances even these patients do not have strokes without some warning.     

Transient cerebral ischaemic attacks and calcium-magnesium imbalance: clinical and paraclinical findings in 106 patients under 50 years of age

One hundred and six selected patients (71 women, 35 men) suffering from transient cerebral ischaemic attacks (TIA) when aged less than 50 years received a comprehensive diagnostic assessment. Patients were classified into three subgroups, into which 9% could be assigned in approximately equal proportions. These were group A; TIA patients with tetanic syndrome (median age of first TIA 32 years, mainly female, 3.2 attacks per year and changing vascular area); group B; TIA patients with migraine (median age of first TIA 26 years, no predominance of male or female sex, 2.0 attacks per year and mostly in the same vascular area); group C: TIA patients suffering from premature arteriosclerosis (median age of first TIA 40 years, no predominance of male or female sex. 0.8 attacks per year and mostly in the same vascular area). Magnesium (P less than 0.001) and calcium (P less than 0.05) in plasma were reduced in group A, and magnesium (P less than 0.05) in group B, versus group C. The increased propensity to vasoconstriction appears to be an important pathogenic factor, particularly in group A, but also in group B.     

Treatment for hereditary angioedema with normal C1‐INH and specific mutations in the F12 gene (HAE‐FXII)

Hereditary angioedema with normal C1 esterase inhibitor and mutations in the F12 gene (HAE‐FXII) is associated with skin swellings, abdominal pain attacks, and the risk of asphyxiation due to upper airway obstruction. It occurs nearly exclusively in women. We report our experience treating HAE‐FXII with discontinuation of potential trigger factors and drug therapies. The study included 72 patients with HAE‐FXII. Potential triggers included estrogen‐containing oral contraceptives (eOC), hormonal replacement therapy, or angiotensin‐converting enzyme inhibitors. Drug treatment comprised plasma‐derived C1 inhibitor (pdC1‐INH) for acute swelling attacks and progestins, tranexamic acid, and danazol for the prevention of attacks. Discontinuation of eOC was effective in 25 (89.3%) of 28 women and led to a reduction in the number of attacks (about 90%). After ending hormonal replacement therapy, three of eight women became symptom‐free. Three women with exacerbation of HAE‐FXII during intake of quinapril or enalapril had no further HAE‐FXII attacks after discontinuation of those drugs. Eleven women were treated with pdC1‐INH for 143 facial attacks. The duration of the treated facial attacks (mean: 26.6 h; SD: 10.1 h) was significantly shorter than that of the previous 88 untreated facial attacks in the same women (mean: 64.1 h; SD: 28.0 h; P < 0.01). The mean reduction in attack frequency was 99.8% under progestins after discontinuing eOC (16 women), 93.8% under tranexamic acid (four women), and 100% under danazol (three women). For patients with HAE‐FXII, various treatment options are available which completely or at least partially reduce the number or duration of attacks.     

氨甲环酸用于全膝关节置换降低失血量的有效性及安全性

背景：氨甲环酸是一种人工合成的抗纤维蛋白溶解的药物,可以经静脉有效的控制全膝关节置换后失血。 目的：观察氨甲环酸对初次行单侧全膝关节置换患者置换后失血的有效性及安全性。 方法：选取2014至2015年在四川省骨科医院下肢科行单侧全膝关节置换的100例患者。以使用氨甲环酸与否分为试验组和对照组,各50例。试验组患者在全膝关节置换前10 min静脉注射氨甲环酸1 g,而对照组不给予氨甲环酸。 结果与结论：试验组患者置换后围手术期总失血量、置换后24 h引流量、输血量明显少于对照组(P < 0.05);试验组与对照组术中出血量、输血人数差异无显著性意义。两组患者患者置换前血红蛋白水平差异无显著性意义,而置换后血红蛋白水平均下降,且试验组患者置换后血红蛋白水平均明显高于对照组。置换后24 h,两组患者凝血功能差异无显著性意义。置换后6 d,2组患者双下肢静脉彩色多普勒超声检查均未见深静脉血栓。提示初次单侧全膝关节置换前静脉给予氨甲环酸能明显减少围手术期失血量及输血量,降低输血风险及输血费用,利于术后功能恢复,且不增加下肢深静脉血栓的风险。中国组织工程研究杂志出版内容重点：人工关节;骨植入物;脊柱;骨折;内固定;数字化骨科;组织工程     

口服与静脉注射氨甲环酸治疗全膝及全髋关节置换后出血的Meta分析

文题释义：氨甲环酸：是一种抑制纤溶酶原激活的抗纤溶药物,它可以可逆性的阻断纤溶酶和纤溶酶原上赖氨酸结合点从而发挥抗纤维蛋白溶解作用,与赖氨酸结合点从而发挥抗纤维蛋白溶解作用。 Meta分析：是循证医学重要的研究方法和最佳证据来源之一,是临床科研工作者必备的研究方法和技术。应用统计学概念及方法收集整理与分析针对于某个概念,找出相关变量的关系进行比较计算,弥补一般文献综述的研究不足。 背景：接受全膝关节或全髋关节置换的患者可用氨甲环酸减少出血量,氨甲环酸有多种给药方式,目前对于氨甲环酸的最佳使用途径仍然存在较大争议。 目的：对比评价口服与静脉注射氨甲环酸治疗全膝关节及全髋关节置换后出血的疗效及安全性。 方法：全面检索PubMed、Cochrane Library、Embase、Web of Science等数据库,检索时限为各数据库建库时间至2019年5月,收集以口服及静脉应用氨甲环酸减少全膝或全髋关节置换术后出血的临床随机对照试验,提取资料并进行方法学质量评估。采用Revman 5.3对纳入结果进行Meta分析。 结果与结论：①9篇文献纳入研究,共1 080例患者;②Meta分析结果显示,口服与静脉注射氨甲环酸术后总失血量差异无显著性意义[MD=1.43,95%CI(-40.02-42.88),P=0.95],术后血红蛋白减少量差异无显著性意义[MD=-0.03,95%CI(-0.11-0.05),P=0.45];③口服与静脉注射氨甲环酸术后输血率差异无显著性意义[RR=0.91,95%CI(0.55-1.49),P=0.70],术后深静脉血栓发生率差异无显著性意义[RR=0.43,95%CI(0.11-1.64),P =0.22],术后住院时间差异无显著性意义[MD=0.00,95%CI(-0.03-0.03),P=0.93];④结果表明,口服与静脉注射氨甲环酸有着类似的功效和临床价值。但是由于纳入文献量较少且质量有限,尚需方法科学的大样本、多中心、高质量临床随机对照试验来进行进一步验证。 ORCID: 0000-0003-3987-4904(林杰彬) 中国组织工程研究杂志出版内容重点：人工关节;骨植入物;脊柱;骨折;内固定;数字化骨科;组织工程     

Management of pregnancy in hereditary angioedema in a resource constrained setting: Our experience at Chandigarh,North India

Hereditary angioedema (HAE) is a rare genetic disorder distinguished clinically by recurrent episodes of non-pruritic swelling. Although pregnancy has been considered a trigger, it may have variable effect on frequency of attacks of HAE. C1-inhibitor (C1-INH) is the treatment of choice for management of HAE during pregnancy. However, because of non-availability of C1-INH therapy in developing countries, fresh-frozen plasma (FFP) and tranexamic acid remain the drugs of choice in pregnancy for treatment of acute attacks and for prophylaxis respectively. There is paucity of data on outcome of pregnancy with patients with HAE from developing countries such as India where all the first line medications are not available.A retrospective review was done including four HAE patients who conceived (with a total of 9 pregnancies). Our results suggest that frequency of attacks may increase during pregnancy especially during second trimester and post-delivery (during breastfeeding). However, HAE attacks are rare at the time of delivery.In resource limited settings, treatment with FFP/tranexamic acid needs to be individualised.     

Experience with allergic bronchopulmonary aspergillosis: some unusual features

The clinical and immunologic features often patients with allergic bronchopulmonary aspergillosis (ABPA), observed over periods varying from 12 months to 10 years, are reported. Acute attacks of ABPA were characterized by several, or all of: increased cough and sputum, haemoptysis, pleuritic pain, expectoration of sputum plugs, and increasing airways obstruction. Peripheral blood eosinophilia and acutely elevated serum IgE levels were seen in all patients during acute attacks, sputum eosinophilia and recovery of Aspergillus in sputum was less common. However, blood eosinophilia was not present in all attacks of ABPA and sputum eosinophilia varied similarly from one attack to another. Six patients with previously documented multiple precipitin lines have had no demonstrable precipitins to Aspergillus on several occasions between attacks, three of these patients have also been negative during attacks. Five of the six patients have again developed positive precipitin lines. The total number of episodes in these ten patients was fifty two, three patients have had more than nine acute attacks of ABPA. There is no seasonal variation in this group of patients. Since diagnosis, only three patients have had an attack-free interval longer than 12 months. Two patients are steroid-dependent because of severe asthma, and nine have symptoms of bronchiectasis.     

Plasma fibronectin in asthmatic patients and its relation to asthma attack

This study investigated the relation between asthma attacks and levels of plasma fibronectin (FN) and serum eosinophilic cationic protein (ECP) in patients with bronchial asthma in order to clarify the role of FN in the airway inflammation of bronchial asthma. Plasma levels of FN were significantly higher (P < 0.025) in patients with bronchial asthma than in healthy controls. They were also significantly higher (P < 0.05) in non-atopic asthmatics than in atopic asthmatics. Furthermore, plasma FN was lower during the attack than the non-attack stage (P < 0.025), and a significant increase of plasma FN was noted (P < 0.05) in asthmatics who had more severe and more frequent attacks. Serum levels of ECP were significantly higher during the attack than the non-attack stage (P < 0.005). An increase of plasma FN in the non-attack stage after attacks showed a significant correlation (P < 0.05) with a decrease of serum ECP. These observations clearly indicate that the decrease in plasma FN associated with attacks is closely related to aggravation of airway inflammation, and that the increase in plasma FN in the non-attack stage reflects chronic airway inflammation. These results suggest that the fluctuation in plasma levels of FN may be one of the factors affecting allergic inflammation and attacks in bronchial asthma.     

High-dose intramuscular triamcinolone in severe, chronic, life-threatening asthma

BACKGROUND. Despite oral corticosteroid therapy, some patients with asthma have frequent exacerbations requiring emergency room visits, hospitalization, and occasionally, mechanical ventilation. We compared the effects of high-dose intramuscular triamcinolone with oral prednisone in patients with severe chronic asthma. METHODS. In a double-blind, placebo-controlled, cross-over study that spanned all seasons, we treated 12 patients with high-dose intramuscular triamcinolone (360 mg over the first three days of the treatment period) or low-dose oral prednisone (median dose, 12.5 mg per day throughout the period; range 0 to 30). The two three-month treatment periods were separated by a three-month washout period. During all periods the patients were allowed to take additional doses of prednisone for acute exacerbations of asthma. RESULTS. After receiving triamcinolone, the patients had significantly better peak expiratory flow rates than while receiving prednisone (the average [+/- SEM] weekly percent of the predicted value during the triamcinolone period was 91.5 +/- 6.9, as compared with 75.0 +/- 5.9 for the prednisone period; P less than 0.05). During the prednisone period there were 21 emergency room visits and 10 hospitalizations, but there were none during the triamcinolone period (P less than 0.05). There were two episodes of ventilatory failure during the prednisone period. Total steroid doses were significantly smaller during the triamcinolone period than during the prednisone period (P less than 0.04). Steroidal side effects were more pronounced after treatment with triamcinolone than after treatment with prednisone (P less than 0.1). CONCLUSIONS. We conclude that high-dose intramuscular triamcinolone is more effective than low-dose prednisone in patients with severe, chronic, life-threatening asthma, but steroidal side effects are somewhat worse.     

Mild mental stress in diabetes: changes in heart rate and subcutaneous blood-flow

A TV-game of tennis of 20 min duration was used to study the influence of mild mental stress on subcutaneous blood-flow (SBF), blood-pressure and heart rate in nine insulin-dependent diabetics and nine healthy subjects. SBF was measured on the thigh by local clearance of xenon-133. Measurements were made before, during and after the period of stress. During stress, SBF increased significantly by 26% in the healthy subjects, while SBF remained unchanged in the diabetics. The difference between the two groups was significant (P less than 0.05). Following stress, SBF returned to pre-stress level in the healthy subjects, while a significant decrease of 33% was observed in the diabetics. The pre-stress heart rate level was higher and the stress-induced increase in heart rate was less in the diabetics compared with the healthy subjects (P less than 0.05). During the stress a slight--but insignificant--increase in blood-pressure was observed in both groups. In conclusion, we found that even mild mental strain influences SBF in both normal subjects and in diabetics. The induced alterations in the two groups are different, probably because of a slight parasympathetic dysfunction in the diabetics.     

Diagnostic and therapeutic problems associated with hereditary deficiency of the C1 esterase inhibitor

Six patients in a family with a history of hereditary angioedema reported swelling of the extremities and recurrent abdominal pain occurring spontaneously or after trauma. Attacks of oedema involving the airways, the greatest danger with this disorder, were present only in one case. This autosomal dominant disease is due to deficient activity of the inhibitor of the first component of complement. Low levels of C4, and absence of C1 esterase inhibitor confirm the diagnosis. Two asymptomatic cases with the appropriate biochemical abnormality are reported in this study. For short term prophylaxis of attacks (before surgery especially), fresh frozen plasma is used, or better still, C1 esterase inhibitor. For long term prophylaxis of attacks antifibrinolytic and hormonal drugs are used: in two cases, the authors obtained good results with methyltestosterone after failure of tranexamic acid.     

Abnormal clearance of soluble aggregates of human immunoglobulin G in patients with systemic lupus erythematosus.

In the present study, we tested mononuclear phagocyte system function in nine healthy controls and 15 SLE patients with complement activating 123I-labelled aggregates of human IgG (AIgG). Clearance half-time of AIgG was 26 +/- 8 min in controls, compared to 58 +/- 27 min in patients (P less than 0.005). Binding of AIgG to erythrocytes was significantly lower in patients, 9.3 +/- 8.1 vs 24 +/- 20% (P less than 0.05). The increase of C3a-levels in plasma was significantly lower in patients than in controls (P less than 0.05 at 3 and 8 min), suggesting less complement activation. Liver and spleen uptake of 123I-AIgG was measured with a gamma camera and expressed as liver/spleen uptake ratios. In patients, the liver/spleen uptake ratios were significantly higher than in controls at 15 min, 3.8 +/- 2.0 vs 2.31 +/- 0.7 (P less than 0.05), due to less splenic uptake of AIgG. Correlations between clearance half-time or liver/spleen uptake ratios and immune complex levels or disease activity were not found. This study indicates that clearance of soluble AIgG is abnormal in patients with SLE, due to decreased splenic uptake of AIgG.