Large iliac arteries: a high-risk group for endovascular aortic aneurysm repair.

PURPOSE: To examine if the presence of large iliac arteries is a potential risk factor for the development of a type Ib endoleak (iliac sealing zone) or need for iliac artery-related secondary intervention in patients undergoing endovascular abdominal aortic aneurysm repair. METHODS: The medical notes and all preoperative and postoperative plain abdominal radiographs and computer tomographic scans were reviewed for a consecutive series of 100 patients (89 men; mean age 75 years, range 56-91) with large iliac arteries (mean 19.7 mm, range 16-22) who had Zenith endovascular stent-grafts inserted for management of aortoiliac aneurysmal disease from January 1999 until September 2002. Endpoints were all-cause mortality, aneurysm-related death, endoleak, secondary intervention, secondary interventions, and stent-graft migration. RESULTS: Mean follow-up was 30.1+/-8.3 months; at the last follow-up, 30% of patients were dead, 3% were aneurysm-related. Seven (7%) patients developed a type Ib endoleak, with the remainder being type II (29%), type Ia (2%), type III (1%), and type V (endotension, 1%). Eight (27.5%) type II endoleaks persisted, with the remainder closing spontaneously with sac shrinkage. The iliac artery-related secondary intervention rate was 10%, and the overall secondary intervention rate was 16%. CONCLUSION: Iliac arteries between 16 and 22 mm in diameter may be treated with a cuff to the iliac limb with an expectation of 90% efficacy. Surveillance is required, with a high index of suspicion for type 1b endoleaks. Early secondary iliac intervention with extension to the external iliac artery is recommended if there is an increase in sac size after 6 months.     

Hemorrhagic complications in patients treated with anticoagulant doses of a low molecular weight heparin (enoxaparin) in routine hospital practice.

Low molecular weight heparins (LMWHs) are a rapidly growing class of anticoagulant drug. Their efficacy has been demonstrated in several clinical settings where they are rapidly becoming the anticoagulant of choice. Controlled clinical studies in patients with deep vein thrombosis, pulmonary embolism, and unstable angina have documented that the frequency of major hemorrhage is 0.5-4%. The purpose of the study was to determine the frequency of minor and major hemorrhage occurring in patients receiving anticoagulant doses of an LMWH (enoxaparin) during routine clinical practice. A prospective, observational study of consecutive patients receiving enoxaparin 1 mg/kg twice daily for at least 24 hours in five internal medicine wards of a university teaching hospital was performed. Five hundred forty-nine patients were studied. The mean age was 67.5+/-15.5 years and the mean duration of enoxaparin therapy was 3.8+/-1.5 days. Hemorrhage was documented in a total of 94 patients (17.3%). Major hemorrhage occurred in 14 patients (2.6%), injection-site hemorrhage occurred in 55 patients (10%), and minor hemorrhage (noninjection site) was documented in 25 patients (4.7%). There were two deaths attributed to hemorrhage. Patients with major hemorrhage were older than patients with minor or no hemorrhage (75.5+/-10.4 versus 66.8+/-15.2 years; p=0.03) and occurred in patients receiving enoxaparin for a longer period (5.14+/-3.8 days) than those with minor (4+/-2.5 days) or no hemorrhage (2.9+/-2.1 days). Major hemorrhage was significantly associated with impaired renal function, chronic liver disease, and concomitant treatment with warfarin or a proton pump inhibitor. Enoxaparin used in anticoagulant doses in unselected medical patients is not associated with more major hemorrhagic complications than observed in controlled clinical trials. Major hemorrhage may be more likely in older patients, in patients with chronic liver disease and impaired renal function, in patients receiving prolonged enoxaparin therapy, and in patients receiving warfarin or proton pump inhibitors.     

A common founder mutation in FANCA underlies the world's highest prevalence of Fanconi anemia in Gypsy families from Spain

Fanconi anemia (FA) is a genetic disease characterized by bone marrow failure and cancer predisposition. Here we have identified Spanish Gypsies as the ethnic group with the world's highest prevalence of FA (carrier frequency of 1/64-1/70). DNA sequencing of the FANCA gene in 8 unrelated Spanish Gypsy FA families after retroviral subtyping revealed a homozygous FANCA mutation (295C>T) leading to FANCA truncation and FA pathway disruption. This mutation appeared specific for Spanish Gypsies as it is not found in other Gypsy patients with FA from Hungary, Germany, Slovakia, and Ireland. Haplotype analysis showed that Spanish Gypsy patients all share the same haplotype. Our data thus suggest that the high incidence of FA among Spanish Gypsies is due to an ancestral founder mutation in FANCA that originated in Spain less than 600 years ago. The high carrier frequency makes the Spanish Gypsies a population model to study FA heterozygote mutations in cancer.     

EMQN best practice guidelines for the molecular genetic testing and reporting of fragile X syndrome and other fragile X-associated disorders

Different mutations occurring in the unstable CGG repeat in 5'' untranslated region of FMR1 gene are responsible for three fragile X-associated disorders. An expansion of over ∼200 CGG repeats when associated with abnormal methylation and inactivation of the promoter is the mutation termed ‘full mutation'' and is responsible for fragile X syndrome (FXS), a neurodevelopmental disorder described as the most common cause of inherited intellectual impairment. The term ‘abnormal methylation'' is used here to distinguish the DNA methylation induced by the expanded repeat from the ‘normal methylation'' occurring on the inactive X chromosomes in females with normal, premutation, and full mutation alleles. All male and roughly half of the female full mutation carriers have FXS. Another anomaly termed ‘premutation'' is characterized by the presence of 55 to ∼200 CGGs without abnormal methylation, and is the cause of two other diseases with incomplete penetrance. One is fragile X-associated primary ovarian insufficiency (FXPOI), which is characterized by a large spectrum of ovarian dysfunction phenotypes and possible early menopause as the end stage. The other is fragile X-associated tremor/ataxia syndrome (FXTAS), which is a late onset neurodegenerative disorder affecting males and females. Because of the particular pattern and transmission of the CGG repeat, appropriate molecular testing and reporting is very important for the optimal genetic counselling in the three fragile X-associated disorders. Here, we describe best practice guidelines for genetic analysis and reporting in FXS, FXPOI, and FXTAS, including carrier and prenatal testing.     

Comprehensive health status assessment of centenarians: results from the 1999 large health survey of veteran enrollees

BACKGROUND: Information on the health status of centenarians provides a means for understanding the health care needs of this growing population. Therefore, we examined the health status of a national cohort of centenarian veteran enrollees. METHODS: Ninety-three centenarian veteran enrollees returned a complete health history questionnaire, which included questions about sociodemographic information, age-associated conditions, health behaviors, health-related quality of life as measured by the Veterans SF-36, and change in health status. RESULTS: Centenarian veteran enrollees are a group with major impairment across multiple dimensions of health-related quality of life despite having a relatively low prevalence of diseases. They had considerable physical limitations as reflected by their physical health summary scores (26.2 +/- 8.3). However, their mental health was comparatively good (mental health summary score 44.1 +/- 12.5). Compared to younger elderly veterans (ages 85-99), centenarians had a lower prevalence of hypertension, angina or myocardial infarction, diabetes, and chronic low back pain (p <.05). Centenarians had significantly worse physical functioning, role physical, vitality, and social functioning scores than did younger elderly veterans. The two groups did not differ in their general health, bodily pain, role emotional, and mental health scores. Centenarians did not perceive much decline in their physical or mental health during the preceding year. CONCLUSIONS: Centenarian veteran enrollees are a group with a low number of age-associated diseases and good mental health despite substantial physical limitations. These results support future studies of services directed toward improvement of function as opposed to those focused solely on the treatment of diseases.     

Experience of providing cultural safety in mental health to Aboriginal patients: A grounded theory study

The need for mental health clinicians to practice cultural safety is vital in ensuring meaningful care and in moving towards improving the mental health outcomes for Aboriginal people. The concept of cultural safety is particularly relevant to mental health professionals as it seeks to promote cultural integrity and the promotion of social justice, equity and respect. A substantive theory that explained the experience of providing cultural safety in mental health care to Aboriginal patients was developed using grounded theory methodology. Mental health professionals engaged in a social psychological process, called seeking solutions by navigating the labyrinth to overcome the experience of being unprepared. During this process participants moved from a state of being unprepared to one where they began to navigate the pathway of cultural safety. The findings of this research suggest health professionals have a limited understanding of the concept of cultural safety. The experience of providing cultural safety has not been adequately addressed by organizations, health services, governments, educational providers and policy makers. Health services, organizations and government agencies must work with Aboriginal people to progress strategies that inform and empower staff to practice cultural safety.     

Buffering Syndemic Effects in a Sexual Risk-Reduction Intervention for Male Clients of Female Sex Workers: Results From a Randomized Controlled Trial

Objectives. We sought to test the efficacy of a sexual risk intervention for male clients of female sex workers (FSWs) and examine whether efficacy was moderated by syndemic risk.Methods. From 2010 to 2014, we conducted a 2-arm randomized controlled trial (60-minute, theory-based, safer sex intervention versus a didactic time-equivalent attention control) that included 400 male clients of FSWs on the US–Mexico border with follow-up at 4, 8, and 12 months. We measured 5 syndemic risk factors, including substance use and depression. Primary outcomes were sexually transmitted infections incidence and total unprotected sex with FSWs.Results. Although participants in both groups became safer, there was no significant difference in behavior change between groups. However, baseline syndemic risk moderated intervention efficacy. At baseline, there was a positive association between syndemic risk and unprotected sex. Then at 12 months, longitudinal analyses showed the association depended on intervention participation (B = −0.71; 95% confidence interval [CI] = –1.22, –0.20; P = .007). Among control participants there still existed this modest association (B = 0.36; 95% CI = –0.49, 1.22; P = .09); among intervention participants there was a significant negative association (B = −0.35; 95% CI = –0.63, –0.06; P = .02).Conclusion. A brief intervention might attenuate syndemic risks among clients of FSWs. Other populations experiencing syndemic problems may also benefit from such programs.Male clients of female sex workers (FSWs) represent a “bridge population” that has the potential to transmit HIV and sexually transmitted infections (STIs) between higher-risk groups (FSWs) and lower-risk groups (e.g., their wives).1–3 Globally, male clients of FSWs exhibit high-risk sexual behaviors,4–8 as well as higher STI incidence compared with other men.9 Unfortunately, little intervention work has targeted male clients.In 2007, Lowndes et al. implemented a behavioral intervention focusing on condom promotion among male clients of FSWs in Cotonou, Benin.10 Results suggested that male clients of FSWs in a resource-constrained country can reduce their sexual risk behavior through participation in a targeted behavioral intervention. However, this study lacked randomization and a control condition, precluding the ability to evaluate efficacy to reduce sexual risk behavior. A recent review of randomized trials and quasi-randomized trials of behavioral HIV prevention interventions among sex workers and clients did not identify a single randomized controlled trial targeted for male clients.11In Tijuana, Mexico, a border city along San Diego, California, there are an estimated 6000 to 10 000 FSWs offering sexual services to men from both countries.12 Tijuana has an HIV prevalence that is double the national average, and HIV is highest among high-risk groups including FSWs (approximately 6%).12–14In 2008, we conducted pilot research with 400 male clients of FSWs in Tijuana.13 HIV prevalence among clients was similar to that of FSWs (approximately 5%). Clients reported sex with an FSW an average of 26 times in the past year, once every 2 weeks in the past 4 months, and half reported recent unprotected sex with FSWs. Data were used to develop the first sexual risk-reduction intervention for male clients of FSWs. Herein, we aim to first evaluate the efficacy of our brief intervention, using the rigor of a randomized controlled trial. Whereas evaluating an intervention’s efficacy is a necessary step for HIV prevention, scientists must also examine for whom or under what conditions an intervention is efficacious (i.e., test moderators) to best inform prevention strategies both practically and theoretically.In addition to demonstrating risky sexual behavior, our pilot research with male clients in Tijuana showed that history of drug use was prevalent (88% reporting lifetime drug use) and was associated with unprotected sex with FSWs. These data also showed that the clients were more likely to engage in risky sex if they consumed alcohol at hazardous levels.15 Finally, our qualitative data suggested that vulnerability to HIV was associated with mental health problems like depression or loneliness.16,17 These findings are consistent with previous research on syndemic theory.Research has demonstrated the multiple co-occurrence of psychosocial problems (e.g., substance use, violence) and their additive effect on HIV risk behavior, called “syndemics.”18–21 While the specific factors that are included in the operationalization of a syndemic differ and depend on the specific population and region,21,22 studies have essentially found a dose–response relationship such that individuals who report more psychosocial problems are more likely to engage in higher sexual risk behavior or are more likely to be infected with HIV.23 The second aim of the current research was to examine syndemics as a potential moderator of intervention efficacy. In light of the important role that syndemics play in HIV across high-risk populations, and based on our pilot data, we hypothesized that men who experienced more syndemic problems at baseline would be more likely to benefit from the intervention than men who experienced fewer syndemic problems. Although our sexual risk-reduction intervention was not designed to directly address syndemic problems, it was based on cognitive behavioral therapy, social cognitive theory, and the theory of reasoned action, and we included in the intervention theoretical elements and motivational interviewing techniques designed to promote safer sex behavior by addressing perceived barriers to safer sex.