Herz- und Herz-Lungen-Transplantation

Orthotopic heart transplantation (HTX) is nowadays the worldwide accepted gold standard for the treatment of terminal heart failure. The main indications for HTX are non-ischemic dilatative (54%) and ischemic (37%) heart failure. In the acute phase after HTX the survival rate is approximately 90%. Good short and long-term results with survival rates ranging from 81% after 1 year to more than 50% after 11 years demonstrate that there is currently no real treatment alternative to HTX for treatment of end-stage heart failure. In the case of irreversible pulmonary hypertension in combination with end-stage heart failure or complex congenital heart syndromes, a combined heart and lung transplantation (HLTX) is necessary. Compared with HTX the short-term survival of HLTX is reduced, mostly for technical reasons. Improved long-term results after HTX and HLTX are a result of highly specialized transplantation units and effective immunosuppression. However, a major problem is the shortage of organ donors in Germany and the resulting long waiting times for patients with frequently occurring blood groups of up to 10 months for transplantation. The consequence of the latter is the ever increasing number of implanted cardiac assist devices in patients not only as a bridge to transplant but also as destination therapy.     

Product type and the risk of inhibitor development in nonsevere haemophilia A patients: a case‒control study

Inhibitor development is a major complication of treatment with factor VIII concentrates in nonsevere haemophilia A. It has been suggested that plasma-derived factor VIII (FVIII) concentrates elicit fewer inhibitors than recombinant FVIII concentrates, but studies in severe haemophilia A patients have shown conflicting results. We designed a case‒control study to investigate the clinical and genetic risk factors for inhibitor development in nonsevere haemophilia A patients. We investigated whether the type of FVIII concentrate was associated with inhibitor development in nonsevere haemophilia A patients. This nested case‒control study includes 75 inhibitor patients and 223 controls, from a source population of the INSIGHT study, including all nonsevere haemophilia A patients (FVIII:C 2–40%) that were treated with FVIII concentrates in 33 European and one Australian centre. Cases and controls were matched for date of birth and cumulative number of exposure days (CED) to FVIII concentrate. A conditional logistic regression model was used to calculate unadjusted and adjusted odds ratios. No increased risk for inhibitor development was found for any type of FVIII concentrate; either when comparing recombinant FVIII concentrates to plasma-derived FVIII concentrates (adjusted odds ratio 0·96, 95% confidence interval (CI) 0·36–2·52) or for specific types of FVIII concentrates.     

Eight novel F13A1 gene missense mutations in patients with mild FXIII deficiency: in silico analysis suggests changes in FXIII-A subunit structure/function

Mild FXIII deficiency is an under-diagnosed disorder because the carriers of this deficiency are often asymptomatic and reveal a phenotype only under special circumstances like surgery or induced trauma. Mutational reports from this type of deficiency have been rare. In this study, we present the phenotypic and genotypic data of nine patients showing mild FXIII-A deficiency caused by eight novel heterozygous missense mutations (Pro166Leu, Arg171Gln, His342Tyr, Gln415Arg, Leu529Pro, Gln601Lys, Arg703Gln and Arg715Gly) in the F13A1 gene. None of these variants were seen in 200 healthy controls. In silico structural analysis of the local wild-type protein structures (activated and non-activated) from X-ray crystallographic models downloaded from the protein databank identified potential structural/functional effects for the identified mutations. The missense mutations in the core domain are suggested to be directly influencing the catalytic triad. Mutations on other domains might influence other critical factors such as activation peptide cleavage or the barrel domain integrity. In vitro expression and subsequent biochemical studies in the future will be able to confirm the pathophysiological mechanisms proposed for the mutations in this article.     

HIV Prevention Needs Among Street-Based Male Sex Workers in Providence,Rhode Island

We examined data derived from a needs assessment of the personal and social characteristics and HIV risk behavior of street-based male sex workers, in Providence, Rhode Island, who engage in transactional sexual intercourse with other men. Substance use, injected drugs, needle sharing, and psychosocial distress were highly prevalent among the sample. History of physical, sexual, and emotional abuse was associated with increased risk of condomless anal sexual intercourse with paying male clients.In the United States, studies show that male sex workers (MSWs) engage in sexual and drug-using risk behaviors that put themselves and their partners at risk for HIV acquisition. Male sex workers have a significantly higher HIV prevalence (7.3% vs 1.1%) and incidence (4.7% vs 0.9%) than do men who have sex with men but who do not engage in transactional sexual intercourse.1–6 Street-based MSWs have been shown to have the highest rates of HIV risk behavior both with clients and nontransactional partners.5,7 Several psychosocial factors, including sexual abuse, homelessness, and substance use (particularly injecting), are known to heighten sexual risk behavior among street-based MSWs.8–13Given the clandestine nature of sex work, there are limited surveillance data on MSWs in the United States. Project Weber (http://www.projectweber.org), a nonprofit health service organization serving MSWs in Providence, Rhode Island, estimates that there are between 300 and 500 street-based MSWs in the city. Collected by Project Weber as part of a needs assessment of street-based MSWs in Providence, the data reported here detail the personal and social characteristics and HIV risk behavior among this group.     

Associations between Dietary Patterns and Cardiometabolic Risk Factors—A Longitudinal Analysis among High-Risk Individuals for Diabetes in Kerala,India

The association between dietary patterns and cardiometabolic risk factors is not well understood among adults in India, particularly among those at high risk for diabetes. For this study, we analyzed the data of 1007 participants (age 30–60 years) from baseline and year one and two follow-ups from the Kerala Diabetes Prevention Program using multi-level mixed effects modelling. Dietary intake was measured using a quantitative food frequency questionnaire, and dietary patterns were identified using principal component analysis. Two dietary patterns were identified: a “snack-fruit” pattern (highly loaded with fats and oils, snacks, and fruits) and a “rice-meat-refined wheat” pattern (highly loaded with meat, rice, and refined wheat). The “snack-fruit” pattern was associated with increased triglycerides (mg/dL) (β = 6.76, 95% CI 2.63–10.89), while the “rice-meat-refined wheat” pattern was associated with elevated Hb1Ac (percentage) (β = 0.04, 95% CI 0.01, 0.07) and central obesity (OR 1.16, 95% CI 1.01, 1.34). These findings may help inform designing dietary interventions for the prevention of diabetes and improving cardiometabolic risk factors in high-diabetes-risk individuals in the Indian setting.     

青年人群腰椎骨密度及标准差对骨质疏松检出率的影响：多中心、大样本分析

背景: 不同地区骨峰值和标准差不同，对骨质疏松诊断率有较大影响。探讨建立一完整数据库为中国人骨质疏松诊断准确性提供依据。 目的：探讨青年人腰椎骨密度和标准差正常参考值影响骨质疏松症检出率的程度。 设计、时间及地点：调查分析，于1997-01/1999-12分别在北京、上海、广州、南京、嘉兴和成都市完成。 对象：采用前瞻性及回顾性方法对全国6个中心骨密度参考数据库中11 418人进行调查统计分析；男3 666人，女7 752人；年龄20岁~90岁；分别来自北京(2 385人)、广州(1 178人)、上海(1 404人)、南京(2 938人)、成都(1 425人)、嘉兴(2 088人),受试者来源于社区调查、健康体检和健康志愿者。 方法：用GE-Lunar公司的DXA仪测量骨密度，调查全国6个中心11 418人L2~L4腰椎后前位和髋部骨密度，建立了骨密度参考数据库。6个中心的仪器内部精度0.3%~0.7%，仪器间的精度1.1%。 主要观察指标：①6个中心不同年龄组腰椎骨密度分布。②青年人群骨密度及其标准差值对骨质疏松症检出率的影响。 结果：中国汉族女性以腰椎进行骨质疏松症诊断的青年人群的骨密度和标准差值，6个中心，最大差值分别为0.098 g/cm2和0.027 g/cm2。用6个中心及总体各自的青年人平均骨密度和标准差值为参考标准，对同一人群计算T-score和获得的骨质疏松症检出率不相同；发现青年人平均骨密度每变化0.01 g/cm2，则骨质疏松症检出率变化1.6%(呈正相关)，其标准差值每变化 0.01 g/cm2，则骨质疏松症检出率变化4%(呈负相关)。 结论：青年人平均骨密度和标准差值不同引起骨质疏松症检出率也不相同。为了让不同中心的骨质疏松症检出率有可比性,建议同一个类型的骨密度仪,同一个种族,同一个地区用一个设计较完善大样本的参考数据库,以其青年人正常参考值计算T-score。