Disorders of menstruation and their effect on the quality of life in women with congenital factor VII deficiency

AIMS AND OBJECTIVES: To review menstrual problems in women with congenital FVII deficiency and to study their effect on the quality of life during menstruation in women with congenital FVII deficiency. METHOD: 14 women with congenital factor VII deficiency registered with the Haemophilia Centre at the Royal Free Hospital were interviewed and their case notes reviewed. All women completed Pictorial Blood assessment Chart (PBAC) for assessment of menstrual blood loss and a quality of life questionnaire during menstruation. Similar questionnaire and PBAC was completed by an age matched healthy control group of 23 women. RESULTS: Median age of study group was 35 yrs and of control group 34 yrs. Median FVII level of the study group was 31.5 IU/dL Two women had severe FVII deficiency (FVII level < 10 IU/dL) and 12 women had mild-moderate FVII deficiency. 57% women (8/14) from study group had menorrhagia (PBAC score > 100) compared with 17% (4/23) women from the control group. Six women (43%) from the study group were diagnosed with anaemia due to heavy periods, compared to two (9%) in the control group. The quality of life scores during menstruation were significantly worse in the women with FVII deficiency, compared to controls. CONCLUSION: Women with factor VII deficiency exhibit a spectrum of bleeding symptoms, menorrhagia being one of the commonest symptoms. This has adverse effect on their quality of life.     

A Systematic Review: The use of desmopressin for treatment and prophylaxis of bleeding disorders in pregnancy

Summary. Desmopressin (DDAVP) is commonly used for treatment and prevention of bleeding complications in patients with bleeding disorders including haemophilia A, von Willebrand’s disease (VWD) and other less common disorders. This article reviews the current evidence for the use of DDAVP in pregnancy to clarify its efficacy and safety with regard to maternal and foetal outcome. A search of the literature found 30 studies that reported DDAVP use in pregnancy for prophylaxis or treatment of bleeding complications with 216 pregnancies reported in total. The most common indication was prophylaxis for prevention of bleeding during pregnancy and postpartum haemorrhage. DDAVP was used successfully in the first and early second trimester for bleeding prophylaxis in 50 pregnancies. No postpartum bleeding complications were reported in 167 out of 172 pregnancies when DDAVP was used for peripartum haemostatic cover. Twenty‐nine studies reported no significant adverse events as a result of treatment with DDAVP. One case of water intoxication seizure and one case of premature labour following the use of DDAVP was reported in a single study. Other maternal side effects included facial flushing and headache and were reported by one study. These side effects were generally well tolerated by patients. There were no other significant adverse events reported in any of the studies as a result of DDAVP use. Foetal outcome was recorded in ten studies with no adverse foetal outcomes. In conclusion, this review shows that DDAVP in selected cases is effective in reducing bleeding complications associated with pregnancy and childbirth with a good safety record. Further research is needed to confirm these findings as they are based on the currently available evidence from small studies and case series only.     

Bradycardiomyopathy: The Case for a Causative Relationship Between Severe Sinus Bradycardia and Heart Failure

Bradycardiomyopathy . A 28‐year‐old man presented with progressive fatigue. Physical examination and ECG revealed severe sinus bradycardia. Echocardiography showed features of noncompaction cardiomyopathy and moderate aortic valve regurgitation. We hypothesized that the chronic volume overload exaggerated by prolonged diastole due to the bradycardia resulted in heart failure and noncompaction cardiomyopathy look‐alike features. After implantation of an AAI pacemaker, his symptoms and signs of cardiomyopathy were fully recovered. (J Cardiovasc Electrophysiol, Vol. pp. 822‐824, July 2010)     

Evaluation of the role of varicocelectomy including external spermatic vein ligation in patients with scrotal pain

OBJECTIVES: The aim of the present study was to assess and compare pre- and postoperative scrotal pain in patients with varicocele who underwent varicocelectomy with different approaches. METHODS: The study included 144 consecutive patients with left-sided varicocele who had left scrotal pain for more than 3 months. All patients underwent varicocele ligation using either a subinguinal or inguinal approach with or without external spermatic vein ligation. We asked the patients to complete an 'Assessment Questionnaire for Scrotal Pain' both before and after the surgery. RESULTS: The surgery was successful in 101 (83.4%) of the 121 patients available for follow up. Seventy-four (61.1%) patients reported the complete resolution of pain while 27 patients (22.3%) reported partial resolution. Symptoms worsened in a single case and pain persisted postoperatively in 19 cases (15.7%). There were no statistically significant differences in the characteristics of the pain and grade of varicocele between postoperative groups. A significant difference was observed in postoperative success between patients who had external spermatic vein ligation and those who did not, regardless of the surgical approach (inguinal or subinguinal). All patients who reported complete or partial resolution of pain stated that they would recommend surgery to relatives with the same problem. CONCLUSIONS: Varicocelectomy using either inguinal or subinguinal approaches is an effective and reasonable treatment option in this patient group and should include external spermatic vein ligation for a satisfactory outcome.     

阿米乐努西达日蜜膏中没食子酸的含量测定

摘要目的建立测定维吾尔医药阿米乐努西达日蜜膏没食子酸含量的方法。方法采用高效液相色谱法,色谱柱：phenomenex C18（4.6 mm×250 mm,5 μm）;检测波长：273 nm;流动相：甲醇 0.4%磷酸水溶液（10：90）;流速：1.0 mL&#8226;min－1;进样量：10 μL;柱温：25 ℃。结果没食子酸在0.106～0.530 μg范围内峰面积与进样量具有良好的线性关系（r＝0.999 7）,平均加样回收率为97.9%（n＝9）,RSD＝1.13%。结论该方法操作简便、准确、灵敏度高、重复性好,可为阿米乐努西达日蜜膏质量评价提供参考。     

Topical use of tranexamic acid to control perioperative local bleeding in gynaecology patients with clotting disorders: two cases

Operating on patients with abnormal coagulation is a challenge frequently faced by surgeons. Achieving haemostasis perioperatively can involve bleeding points that would not ordinarily present a problem with intact clotting function. Here we present two women with localised wound bleeding following a gynaecological surgery in the presence of a clotting disorder. Haemostasis was successfully achieved with tropical use of tranexamic acid. These two cases illustrate a novel use for this antifibrinolytic agent. We suggest that there is a role for topical use of tranexamic acid in perioperative haemostasis in patients with clotting disorders.     

异常黑胆质成熟剂水提物和醇提物对HepG2细胞m5dc及8-OH-cG形成的影响

目的：观察异常黑胆质成熟剂水提物和醇提物对人肝癌细胞株（HepG2）细胞5-甲基脱氧胞核嘧啶（5-methyldeoxycytosine,m5dC）及8-羟基-2′-鸟嘌呤脱氧核苷酸（8-hydroxy-2′-deoxyguanosine,8-OH-dG）等表遗传信息的影响,从而探讨异常黑胆质成熟剂水提物和醇提物抗癌作用的分子生物学机制.方法：采用高效液相色谱联用紫外检测技术（HPLC-UV）,分别观察以2.5、5.0及7.5mg/ml浓度的异常黑胆质成熟剂水提物或醇提物作用48h后,对HepG2细胞m5dC及8-OH-dG形成的抑制作用.结果：异常黑胆质成熟剂水提物作用后,HepG2细胞m5dC生成率分别为27.0、24.2及18.6%,而醇提物分别为23.5、20.2及16.8%,与空白对照组（34.0%）比较均具有显著性差别（P＜0.05）;异常黑胆质成熟剂水提物作用后,HepG2细胞8-OH-dG的生成率分别为0.58、0.45及0.32%,醇提物分别为0.46、0.36及0.23%,与空白对照组（0.69%）比较具有明显差异（P＜0.05）.结论：异常黑胆质成熟剂水提物和醇提物对HepG2细胞m5dC及8-OH-dG等表遗传学信息的产生具有浓度依赖性的抑制作用,而醇提物的作用优于水提物,说明抑制癌细胞m5dC及8-OH-dG等表遗传学信息的产生是异常黑胆质成熟剂抗癌作用的重要分子生物学机制之一.