Severe factor VIII and factor IX deficiency in females.

A survey of 11 hemophilia centers produced data concerning 28 females with extremely low levels of factor VIII or IX coagulant activity. Ten of the 28 have hemophilia A, six have hemophilia B, and 12 have severe von Willebrand's disease. The 16 females who have severe factor VIII or factor IX deficiency as an isolated defect exemplify several of the possible genetic explanations for the occurrence of hemophilia in females. All 16 bruise excessively, and several have had recurrent hemarthroses. Three of these girls, ages five, 10 and 23 years, have evidence of chronic hemophilic arthropathy. The 12 females with severe von Willebrand's disease are either homozygous for von Willebrand's disease or severely affected heterozygotes. All 12 have mucous membrane bleeding. In addition, five of the 12 have recurrent hemarthroses and three have evidence of chronic joint disease. However, the major problem in the adult females with von Willebrand's disease has been extreme menorrhagia. One of the seven adults underwent irradiation sterilization and another had a hysterectomy because of menorrhagia. The others have been managed with anovulatory drugs or plasma infusions and EACA. Despite menorrhagia, five pregnancies and deliveries have been uneventful in three of these women.     

Women and heart failure

Almost half of the patients affected with congestive heart failure (CHF) in the United States are women. However, past studies have included predominantly men and generalized results to women. Many women with CHF are older, have hypertension, and have higher ejection fractions. Survival differences have been reported previously with conflicting results. Although treatment for left ventricular dysfunction is somewhat standardized, treatment for diastolic dysfunction is less defined. Clinical trials for this group of patients, many of whom are women, have not been performed.In comparison with men, women have several cardiovascular differences as well as differences in electrical properties. In addition, response to medical (pharmacologic) therapy may differ in men and women.Finally, functional status has been shown to be compromised in both men and women with CHF; however, some studies have shown women to experience more exercise intolerance. This may be because more women than men have diastolic dysfunction. Few women have been included in exercise trials. Future trials must address women with CHF, many of whom are older and have normal (or near normal) left ventricular function or diastolic dysfunction.     

结核性胸腔积液三种不同治疗方式近期疗效观察

目的　探讨结核性渗出性胸腔积液治疗方式。方法 123例中等量以上积液的结核性胸膜炎病人作为研究对象。患者按入院先后顺序随机分为3组。A组42例,胸腔置管引流并胸腔内注射微卡(母牛分枝杆菌菌苗);B组45例,单纯置管引流;C组36例,常规胸腔穿刺抽液每周1～2次。3组患者的化疗方案均为2HRZE(S)/4HR。C组口服强的松30mg/d。治疗1个月检查。结果 1个月A组胸液控制率95.3%,显效36例(85.7%),有效4例(9.6%),无效2例(4.7%),6个月总有效率100%;B组胸液控制率91.1%,显效36例(80%),有效5例(11.1%),无效4例(8.9%),6个月总有效率100%;C组胸液控制率66.6%,显效3例(8.3%),有效21例(58.3%),无效12例(33.3%),6个月总有效率83.3%。结论 深静脉导管引流胸腔积液,注药与不注药无显著性差异,但明显优于常规胸穿抽液。     

Bioengineered Vascular Grafts: Can We Make Them Off-the-Shelf?

Surgical treatments for vascular disease have progressed during the past century from autologous bypass conduits to synthetic materials, animal-derived tissues, cryopreserved grafts, and, finally, bioengineered conduits. In all cases, alternative vascular grafting materials have been developed with the goal of treating patients who have severe vascular disease requiring bypass but who have no suitable autologous conduit. Synthetic vascular grafts, animal-derived tissues, and cryopreserved grafts all have drawbacks in terms of availability and functionality that have limited their routine clinical adoption. Although bioengineered vascular graft technologies remain early and highly investigational, they have the potential to revolutionize the way in which severe vascular disease is treated. However, before they can have a clinical impact, bioengineered grafts must be available immediately and "off-the-shelf."     

Insulin,Insulin Resistance,Obesity, and Cancer

Epidemiologic studies have proposed a link between obesity, type 2 diabetes, and cancer. The pathophysiologic mechanisms involved in the development of type 2 diabetes, namely hyperinsulinemia and insulin resistance, have also been implicated in cancer development. Patients with type 2 diabetes are reported to have a worse response to cancer chemotherapy, have more complications, and have a poorer prognosis than patients with cancer without diabetes. Studies also have reported that insulin, insulin secretagogues, and metformin may have effects on tumor growth. Given the escalating worldwide prevalence of obesity and type 2 diabetes, their relationship to cancer has generated great interest and research across many fields of medicine.     

A Microsurgical Methodology for Human Cells in Vitro: Evolution and Applications

A methodology for micromanipulating human cells of normal and malignant origin, in vitro, has evolved from the study of about 2000 HeLa, ERK (a subline of HeLa cells), and human embryonic lung cells during interphase and mitosis. It is now possible to microinject interphase cells with aqueous and nonaqueous fluids intracytoplasmically. Chromosomes from human embryonic lung metaphase cells have been transplanted. Chromosomes have been manipulated within mitotic human embryonic lung, ERK, and HeLa cells. Clones have been obtained from HeLa cells subjected to such manipulation. Predictable derangements of mitotic cells and their progeny have been obtained. Intranuclear injections of silicone oil, DNA, and sodium chloride solutions have been made with survival of the cells. HeLa cells have been cloned from such injected cells. Subcellular fractions have been introduced into the nuclei and cytoplasms of HeLa and human embryonic lung cells. The lung cells have been subjected to nuclear micropuncture in groups and a clone has been obtained. Virus suspensions have been introduced into the nuclei of HeLa cells without killing the cells. Applications of this methodology are discussed.     

Hematopoiesis from pluripotent stem cell lines

Embryonic stem cells (ESCs) can differentiate into various types of hematopoietic cells (HPCs) when placed in an appropriate environment. Various methods for the differentiation of ESCs into specific HPC lineages have been developed using mouse ESCs. These ESC-differentiation methods have been utilized also as an in vitro model to investigate hematopoiesis in embryos and they provided critical perceptions into it. These methods have been adapted for use with human ESCs, which have the possibility of being employed in regenerative medicine; further improvement of these methods may lead to the efficient production of HPCs for use in transfusions. The generation of transplantable hematopoietic stem cells is a medical goal that is still difficult to achieve. Recently, induced pluripotent stem (iPS) cells have been established from differentiated cells. Thereby, iPS cells have expanded further possibilities of the use of pluripotent stem cell lines in clinical application. Indeed, iPS cells have been established from cells with disease genes and those which have undergone reprogramming and targeting have generated phenotypically normal HPCs. Here, we mainly summarize the recent progress in research on hematopoiesis conducted with ESCs and iPS cells.     

HIV and liver diseases: recent clinical advances

Because the life expectancy of patients infected with HIV has been prolonged, liver diseases have assumed far greater importance as a cause of morbidity and mortality in these patients. Given the shared risks of transmission, patients who have HIV often are coinfected with hepatotrophic viruses such as hepatitis C and hepatitis B. Further, antiretroviral therapy (ART) used by patients who have HIV is often hepatotoxic, contributing to liver damage. With increasing immunosuppression caused by AIDS, patients who have HIV have to deal with these issues and the increased risk of infection with opportunistic viral, fungal, bacterial, and protozoal pathogens. In addition, steatosis and lipodystrophy now are recognized more commonly in patients who have HIV, particularly in the setting of ART. Thus, understanding of liver diseases in the setting of HIV infection becomes an important focus in caring these individuals. There have been numerous advances in the treatment of liver disease in patients who have HIV, particularly in treating viral hepatitis C and B. This article reviews various liver manifestations in patients who have HIV and the recent advances in diagnostic and therapeutic options.     

Antiatherogenic actions of etidronate on atherosclerosis

Bisphosphonates including etidronate have been suggested to have antiatherogenic actions in experimental animals or in vitro. However, there has been no report indicating that bisphosphonates have antiatherogenic actions in humans. We have recently reported that etidronate, one of the bisphosphonates, decreased the intima-media thickness in subjects with type 2 diabetes, indicating a possibility that etidronate may have antiatherogenic action in humans.     

Tilt testing for syncope: a reappraisal

PURPOSE OF REVIEW: Tilt-table tests have provided a diagnostic window on the most common cause of syncope, the common faint. The purpose of this review is to summarize the major contributions of tilt tests and provide a critical assessment of the true validity and usefulness of this diagnostic test. RECENT FINDINGS: Tilt-table tests have provided mixed benefits in the field of neurally mediated syncope. They have greatly improved informed care of syncope patients and have led to a revived interest in the field. They have provided study populations having at least one objective finding in common for diagnostic studies, long-term observational studies, and randomized clinical trials. Tilt tests have been used as platforms for physiologic studies and pilot treatment studies. However, more specific benefits have proven illusive. The main problem is that the neurally mediated syncope syndrome is defined by the test, rather than by evidence-based and widely accepted clinical criteria. Tilt tests have a complex mix of significant methodological variables, have not been validated against gold standard populations, are only moderately reproducible, do not provide prognostic predictive power, and have not been shown useful in selecting efficacious therapies. It may be difficult to achieve important advances in the field until a clinical reflex syncope syndrome is defined by evidence-based diagnostic criteria. SUMMARY: Tilt tests have made neurally mediated syncope amenable to clinical study, but their true usefulness will only be known when an evidence-based, widely accepted definition of this syndrome is developed.     

Longitudinal esophageal bands associated with esophageal aperistalsis: speculations on pathogenesis

A 6-year-old girl with ataxia telangiectasia syndrome was found to have multiple bands longitudinally traversing her esophagus, and esophageal aperistalsis. In the past, the patient was also known to have had esophageal candidiasis. The bands may have been congenital or may have been produced when linear ulcers caused by fungal esophagitis undermined mucosal strips and then healed, leaving tissue bands attached only at their extremities to the esophageal wall. The esophageal motor dysfunction may have been attributable to esophageal candidiasis, or may have been a hitherto unrecognized component of the ataxia telangiectasia syndrome.     

The Role of Antihistamines in Asthma Management

Histamine is an important mediator in airway inflammation. It is elevated in the airways of asthmatic patients and is responsible for many of the pathophysiological features in asthma. Antihistamines block the actions of histamine and also have effects on inflammation which is independent of histamine-H(1)-receptor antagonism. Antihistamines have been shown to have bronchodilatory effects, effects on allergen-, exercise-, and adenosine-monophosphate-challenge testing, and also to prevent allergen-induced nonspecific airways hyperresponsiveness. Clinical studies have shown mixed results, and some studies have reported beneficial effects of azelastine, cetirizine, desloratadine, and fexofenadine on asthma symptoms or physiological measures in patients with asthma. The combination of an antihistamine and a leukotriene receptor antagonist has been shown to have additive effects in certain studies. Antihistamines have also been shown to delay or prevent the development of asthma in a subgroup of atopic children. These data suggest that antihistamines may have beneficial effects in the management of asthma.     

Unraveling the mysteries of classical preconditioning

Nearly two decades have passed since the first report about ischemic preconditioning. Although we do not yet know unequivocally what the final effector is, we have learned a lot about the signal transduction pathways that result in protection, and have some good prospects for the final step that results in survival or necrosis of the ischemic myocardium. Many investigators have contributed to our current knowledge. We were heartened to learn that four of our JMCC publications are included in the 20 top-cited papers in the journal's history. It is gratifying that our prior publications have generated some interest and stimulated important debate as documented by the high number of citations by scientists in the bibliographies of their own papers. In this document, we have been asked to reflect on those four papers and comment on where they have led us.     

Management of mycobacteriosis in general hospital without isolation ward for tuberculosis patients. 3. Current status of tuberculosis patient care in university hospitals and general hospitals in Japan

A recent epidemiological survey has revealed that the incidence of Mycobacterium tuberculosis (TB) patients in Japan has just increased again after four decades of decline. In fact, recently there have been numerous reports of TB outbreaks in health-care facilities. Although our medical school hospital does not have TB isolation rooms, we have to take care of more than a few TB patients, most of whom have been transferred from primary care clinics. Although, some of these TB patients have highly infectious (sputum smear positive), most of them have not been diagnosed as having highly infectious TB, and therefore, some of their patients ultimately have to be retransferred to a TB hospital. This indicates that most physicians in primary care clinics have little knowledge about TB. This may be partly because of lack of training regarding TB during their medical student days and residencies. To elucidate current TB patient care status in university hospitals in Japan, a survey of physicians working in such hospitals was conducted from September 1997 to January 1998. The survey (questionnaire) revealed that the majority (76%) of these hospitals do not have TB isolation rooms. However, these hospitals have to take care of TB patients in their outpatient clinics and sometimes on their wards because the patients have serious complications that can not be treated in ordinary TB hospitals. The survey also showed that for this reason and from an educational point of view, the majority of the physicians (90%) working in these hospitals thought that university hospitals should have isolation rooms for such patients. Another questionnaire revealed that few physicians and nurses in university hospitals have sufficient experiences in taking care of TB patients. This situation may have been responsible for producing physicians with little knowledge about TB. Recent scientific advances have made it possible to construct TB isolation rooms in ordinary wards by means of separate ventilation systems. Although combatting TB requires a variety of strategies, appropriate education for both medical students and residents using isolation rooms in university hospitals may be an effective means of preventing spread of TB, and this approach may also increase awareness concerning the prevention of TB outbreaks in hospitals and health-care facilities.     

Ischemic myelopathy.

Ischemic myelopathy and Angiology of the Spinal Cord have recently drawn the attention of both paraplegists and angiologists, and their details are now fairly well known. Ischemic myelopathies increased with the rise of vascular surgery, but the means of prevention have been carefully studied and the incidence is now decreasing. Twenty-five hundred cases of cord injury have been reviewed, and among 92 nontraumatic cases, 16 ischemic myelopathies have been tabulated. The anatomy and physiology of the spinal cord circulation have been described.     

Conservation of 70S Ribosomal RNA Genes in the Chloroplast DNAs of Higher Plants

Chloroplast DNAs of higher plants have been found to contain two chloroplast ribosomal RNA genes. The base sequences of these chloroplast ribosomal RNA genes in chloroplast DNAs have been studied by molecular hybridization. The results have shown that these genes have undergone little divergence in the evolution of either monocotyledonous or dicotyledonous plants.     

Comparison of ventricular arrhythmia frequency in patients with ischemic cardiomyopathy versus nonischemic cardiomyopathy treated with implantable cardioverter defibrillators

Implantable cardioverter defibrillators have been shown to provide similar survival benefits for patients who have left ventricular dysfunction due to ischemic heart disease and for subsets of patients who have nonischemic cardiomyopathy. Findings in this study extend these observations by showing that patients who have ischemic or nonischemic heart disease and receive implantable cardioverter defibrillators not only have comparable mortality rates but also similar tachyarrhythmia frequencies during follow-up; further, mortality and tachyarrhythmia outcomes are independent of initial arrhythmia indication.     

Glutaric aciduria type I: Outcome in the Republic of Ireland

Twenty-one patients have been diagnosed with glutaric aciduria type I over a 16-year period in the Republic of Ireland, 11 following clinical presentation and 10 following a high-risk screen. Nineteen have been managed with diet. Eight patients have died, of whom 7 were diagnosed clinically. Six had dystonic and one spastic cerebral palsy. Of the 11 patients who did not have cerebral palsy, 10 were diagnosed following a high-risk screen. Seven of the 11 have no abnormal neurological signs; 6 of the 7 have abnormal CT or MRI findings; and no case of striatal degeneration has occurred during the past 14 years in the high-risk screened group.     

Recurrent ulcer after vagotomy and pyloroplasty: the X-ray appearances and their value in diagnosis

Barium meal studies have shown evidence of a recurrent ulcer or of stenosis in 12 out of 24 patients with recurrent dyspepsia after vagotomy and pyloroplasty. These 12 patients have been subsequently proved to have had recurrences and all but one are now cured by further surgery. Barium meals in 12 patients show no evidence of recurrence or stenosis, and follow-up clinical studies suggest that they do not have recurrent ulcer.Radiological studies appear to have great value in the interpretation of recurrent dyspeptic symptoms after vagotomy and pyloroplasty.     

Cataract Surgery — A Personal Account or “One Satisfied Patient”

Most cataracts are related to aging. By age 80, more than half of all Americans either have a cataract or have experienced cataract surgery. Although this editorial does not have any direct relationship to cardiovascular medicine, it may have some applicability with your patients, many of whom may be older rather than younger, and have cardiovascular disease in addition to visual problems perhaps due to cataracts. Copyright © 2009 Wiley Periodicals, Inc.