HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the ...brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults.
The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease.
The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches.
The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.
The prevalence of type 2 diabetes in children and adolescents is increasing, especially in populations with a very high overall prevalence of the disease.1 There is some evidence that diabetes in ...pregnancy might have a role in this increased prevalence. In a study of type 2 diabetes among Pima Indian children, the prevalence rose significantly between 1967 and 1996.18 This increase was felt to be due to an increase in weight seen in the children, as well as an increased frequency of exposure to diabetes in utero. In-utero exposure to diabetes is thought to lead to fetal hyperinsulinaemia, which causes an increase in fetal fat cells, leading to obesity and insulin resistance in childhood. These symptoms in turn lead to impaired glucose tolerance and diabetes in adulthood. Therefore, a cycle is set in motion of in-utero exposure to diabetes, leading to childhood obesity and glucose intolerance, and subsequently to diabetes in pregnancy. This sequence of events has not only be noted in Pima Indian children, but also in a more heterogeneous population in Chicago, USA, where children of mothers with diabetes were found to be more obese and have higher rates of impaired glucose tolerance than children of non-diabetic mothers.19
To summarize available evidence on preoperative cardiac risk stratification so that the internist may 1) use clinical and electrocardiographic findings to stratify a patient's perioperative risk for ...myocardial infarction and death; 2) decide which tests provide useful additional risk-related information; and 3) understand the benefits, risks, and evidence surrounding the decision to undertake coronary revascularization before elective noncardiac surgery.
A MEDLINE search and review of the reference lists of identified articles. Sensitivities, specificities, and likelihood ratios for diagnostic tests were calculated, and a quality rating for study methods was applied.
Myocardial infarction and mortality were the major outcomes considered, and a quality rating for study methods was applied.
Clinical and electrocardiographic findings, organized by multivariate prediction indices, accurately identify patients as having low, intermediate, or high risk for myocardial infarction or death. Pharmacologic stress imaging with thallium or echocardiography probably improves risk stratification for intermediate-risk patients having vascular surgery. These tests have not been shown to be effective prognostic indicators for patients having nonvascular surgery. No studies of angiography for risk prediction have been reported. Decision analyses and retrospective series suggest that the risks incurred by doing coronary angiography and revascularization before elective surgery outweigh the benefits. Prospective, controlled studies of coronary revascularization are lacking. Evidence from a randomized, controlled trial has shown a survival benefit with the perioperative use of beta-blockers in patients at risk for coronary artery disease.
Evaluation of all surgical patients by use of clinical indices is recommended. Low-risk patients need no further evaluation before surgery. High-risk patients need optimal management of their high-risk problems, including (if appropriate) beta-blocker use, and may need to have their elective procedures canceled. Intermediate-risk patients probably benefit from further noninvasive stress testing, especially if they are having vascular surgery. Further clinical trials are needed for most areas of concern.
In this document, the Canadian Task Force on Preventive Health Care (CTFPHC) updates its earlier breast-feeding recommendations1 by presenting evidence on interventions that improve the initiation or ...duration of breast-feeding (or both). Breast-feeding has been shown in both developing and developed countries to improve the health of infants and their mothers, making it the optimal method of infant nutrition.2,3 Although the prevalence of breast-feeding in Canada has risen, with over three-quarters of mothers now initiating breast-feeding, the duration of this practice remains short of the recommended World Health Organization (WHO) targets of exclusive breast-feeding for 6 months and partial breast-feeding for up to 2 years.4,5 Recent Canadian data indicate that 22% of recent mothers aged 15-49 years breast-feed for less than 3 months, and 35% do so for at least 3 months.6 This premature discontinuation is more a result of difficulty with breast-feeding, including lack of information and support, than of women's choice.7 In fact, the number of Canadian hospitals that would qualify as "baby-friendly" according to WHO-UNICEF criteria8 was 5 of 523 hospitals responding to a 1993 survey,9 and according to UNICEF only a single hospital had that designation in 2002.10 Interventions consisting of antepartum structured breast-feeding education are effective at improving both initiation and continuation of breast-feeding during the first 2 months postpartum, compared with usual care.14-28 These interventions, consisting of individual or group instruction about breast-feeding knowledge, practical skills and problem-solving techniques, were effective when provided by lactation specialists or nurses, and both single sessions and multiple sessions were effective. Postpartum telephone or in-person support by lactation specialists, nurses or peer counsellors enhanced the effectiveness of these interventions. In addition, the use of peer counsellors improved breast-feeding rates and duration, and these types of programs may represent a cost-effective alternative to professionally delivered services, especially in locations or settings where professional services are scarce or not available.13,20,29-32 The CTFPHC recommends against the use of written materials (which have not been shown to be effective when used alone,16,19,22,26,33-36 although no harm was demonstrated) and commercial discharge packages (which have been shown to decrease breast-feeding rates).37 Unfortunately, advice from a woman's primary clinician (such as family physician, obstetrician or midwife) has not been sufficiently evaluated, and a research gap remains in this area.
The Canadian Medical Association has long been a champion of enhancing the quality of clinical practice guidelines.2 The objective of the recently published Canadian Medical Association Handbook on ...Clinical Practice Guidelines3 (Box 1, Table 1) is to gather up-to-date, evidence-based, experiencedriven guidance on how to use guidelines most effectively to improve patient care. This new handbook combines and updates the 1994 and 1997 documents "Guidelines for Canadian Clinical Practice Guidelines"4 and "Implementing Clinical Practice Guidelines: a Handbook for Practitioners."5 The new handbook places the role of guidelines in health care into perspective, outlining where they are most useful. It helps the reader to decide if an existing guideline can be adapted or if a new guideline should be created, providing resources for both scenarios. The handbook also reviews evidence to guide those responsible for implementing recommendations through the bewildering array of available implementation strategies. In recognition of increasing demands for accountability and increasing emphasis on quality of care, the final chapter reviews the process of evaluating the effectiveness of guidelines.3 As for any systematic review, it is vital that a systematic search strategy be used in the development of clinical practice guidelines. Relying on experts' recollection of the literature, as was common with consensus guidelines, is no longer sufficient. For example, Gilbert and associates8 compared historical recommendations with a systematic review of observational studies of the effect of infant sleeping position on sudden infant death syndrome. They found that by 1970, the literature demonstrated a statistically significantly increased risk of sudden infant death for sleeping on the front relative to sleeping on the back (pooled odds ratio 2.93, 95% confidence interval 1.15-7.47); however, guidelines did not consistently recommend the back-sleeping position until 1992. These authors concluded that use of systematic review techniques could have led to earlier recognition of the risks of sleeping on the front and might have prevented more than 10 000 infant deaths in the United Kingdom and at least 50 000 in Europe, the United States and Australasia.8 The recommendations in any clinical practice guideline should consider the results of the totality of the literature, giving greater weight to better-designed studies. One recent study9 found that applying 2 different quality-evaluation methods (Cochrane or best-evidence synthesis) led to different recommendations. Listing a level of evidence for each recommendation forces the guideline developer to identify the strength of the evidence supporting the statement. In the end, even well-designed guidelines in the same area will occasionally differ in their recommendations. Developers should therefore consider, during the development phase of any guideline, its ease of implementation (implementability). The recent GLIA (GuideLine Implementability Appraisal) instrument takes the developer through a series of validated questions that ask about factors known to predict the relative ease of implementation of guideline recommendations.17 The currently recommended approach to implementation is summarized in Box 2. Implementation strategies may be most effective when they are targeted to locally identified facilitators and barriers to implementation. Barriers may be effectively identified through a process as simple as structured reflection by the implementation group.18 Many implementation strategies have shown modest benefit, and multiple strategies often work better than single ones.19 Although the sheer number of possible implementation strategies precludes their description here, the interested reader is directed to the handbook, 3 which reviews the major implementation strategies that have been assessed in the literature.
To qualitatively explore the process of the provision of futile care in Canadian intensive care units (ICUs).
A mailed, semistructured survey was sent to medical and nursing unit directors of all ...Canadian ICUs, asking them to estimate the frequency of provision of futile care, when care becomes “futile,” the reasons such care is provided, and the resources that are available to help make end-of-life decisions. Nurse/physician agreement was assessed by
χ
2 analysis or Fisher exact test. Content analysis to identify common themes was carried out by 4 raters using a Delphi process.
The response rate was 72%. The majority reported futile therapy had been provided in their ICU over the last year (nurses, “N” = 95%, physicians, “P” = 87%,
P = .02). The most commonly stated reasons for providing futile care were family request (N = 91%, P = 91%,
P = NS) and attending physician request (N = 91%, P = 87%
P = NS). Physicians were cited to provide futile care because of prognostic uncertainty (N = 73%, P = 84%,
P = .047) and legal pressures (N = 84%, P = 75%,
P = NS). Comment review revealed 8 main reasons why futile care was provided, the most common of which were that “death was perceived as treatment failure,” and poor provider-family communication. Few providers were aware of societal (N = 26%, P = 51%) or local (22%, all) guidelines relating to the provision of futile care, but of those who were aware, the majority found these useful (range, 73%-74%). Twenty-seven percent expressed the need for someone to discuss difficult ethical issues, such an individual with ethics training specifically assigned to the ICU.
Caregivers voice the opinion that provision of futile care occurs, for multiple reasons, not the least of which is provider-driven. Nurses and physicians of Canadian ICUs perceive the need for increased availability of more ICU-directed and ethically trained resources to help them in providing end-of-life care.
Screening is best accomplished with a fasting plasma glucose test. Diabetes is diagnosed if the fasting plasma glucose level is 7.0 mmol/L or greater, or if the plasma glucose level is 11.1 mmol/L or ...greater in a 2-hour oral glucose tolerance test (OGTT).2 Either test should be done on 2 occasions before a diagnosis can be made. Impaired fasting glucose is diagnosed if the fasting glucose level is 6.1-6.9 mmol/L, and impaired glucose tolerance is diagnosed if the plasma glucose level is 7.8-11.0 mmol/L in a 2-hour OGTT. Although there are studies suggesting a benefit of treating people who have impaired glucose tolerance to reduce the incidence of progression of diabetes and possibly cardiovascular disease, the evidence is still inadequate to recommend screening for impaired fasting glucose or impaired glucose tolerance. However, people with the latter condition may nonetheless be identified in the course of their health care. These patients should be treated with lifestyle interventions aimed at lowering weight and increasing exercise, because such interventions may lower the incidence of diabetes (level I evidence).31-33 Acarbose treatment can also be considered for these patients, because it has been shown to reduce the incidence of cardiovascular outcomes and hypertension (level I evidence).34 Although the use of metformin33 and acarbose35 in patients with impaired glucose tolerance has been shown to reduce the incidence of diabetes over 3 years, the rate of diabetes dropped when metformin was discontinued.36 Of note, the prevention trials were all of 3 to 6 years' duration, and it is unclear whether the effects of lifestyle or pharmacologie intervention persist beyond that period. Furthermore, it is still uncertain whether diabetes can truly be prevented or whether these strategies simply delay its onset. The impact of delaying diabetes for a few years on preventing microvascular complications would likely be small, since the risk of complications is low in the first 15 years after diabetes diagnosis. The beneficial effects of lifestyle modification on cardiovascular events in people with impaired glucose tolerance also remain to be demonstrated. Finally, the cost-effectiveness of screening for impaired glucose tolerance and offering lifestyle interventions only to those with a positive test result and not to all people with diabetes risk factors has not been examined.
Along with evidence, clinical policies must take patients' values into account. Particularly where evidence is limited and where assumptions of utility-maximizing behavior may not be valid, new ...methods such as trade-off techniques (TOTs), which allow elicitation of patients' treatment alternatives, might be useful in policy formulation. We used TOTs to assess breast cancer patients' attitudes toward two clinical policies designed to ration adjuvant postlumpectomy breast radiation therapy.
Cross-sectional interviews were performed in a tertiary cancer center. A total of 102 patients were presented with information about the side effects and benefits associated with two hypothetical decisions: (1) willingness to receive treatment elsewhere to shorten the wait for radiation therapy, and (2) foregoing radiation therapy in the face of small marginal benefits. For each scenario, a TOT was used to identify the maximal acceptable wait time (MAWT) for therapy and the benefit threshold at which the patient would forego therapy. Associations of clinical and demographic factors with these decisions were determined by regression analysis.
Patients would be willing to wait, on average, 7 weeks before wanting to leave their city for radiation therapy, less than the 13-week delay our patients actually faced. Older patients were less willing to wait (P = .013); 46% of patients would not give up radiation therapy, even in the face of no stated benefit. Willingness to give up radiation therapy was predicted by willingness to accept delay (odds ratio OR, 1.84; 95% confidence interval CI, 1.05 to 3.37) and being employed (OR, 2.61; 95% CI, 1.08 to 6.54). Patients with larger tumors were less willing to give up radiation therapy (OR, 0.57; 95% CI, 0.31 to 0.97).
Even in difficult decisions such as rationing postlumpectomy breast cancer radiation therapy, TOTs can inform policy formulation by indicating the distributions of patients' preferences.
Aims Foundry work has been associated with an increased risk of adverse cardiovascular events. The objective of this review was systematically and qualitatively to review the published literature to ...determine whether foundry work is significantly associated with cardiac disease. Methods MEDLINE and Cochrane databases were systematically searched to identify relevant English‐language publications between 1966 and October 2002. Articles were rated as ‘good’, ‘fair’ or ‘poor’, using published quality review criteria. Additionally, variables suggesting causality were extracted. A qualitative summation of the literature was presented for two scenarios: all studies, or using only studies rated ‘fair’ and above. Results Fourteen studies were analysed. Four were found to be of ‘fair’ quality, the remainder ‘poor’. No ‘good’ quality studies were found. Nine studies show increased cardiac mortality among foundry worker groups and four studies also show a decreased risk. When only ‘fair’ quality studies are taken into consideration, two support increased risk of cardiac disease, one supports a protective effect of foundry work on cardiac disease and one revealed both increased and decreased risk for different cardiac outcomes. Conclusions The exploration of foundry workers' risks of cardiac events reveals conflicting findings, which can only be partly attributed to confounders. Further prospective research to establish the independent contribution of foundry work to cardiac disease is needed.
Our objective was to develop a clinical practice guideline, based on systematic reviews of the literature, as interpreted by experts, to assist clinicians in managing procedure-related pain and ...distress among children undergoing vaccine injections. The scope was limited to acute (immediate) pain and distress at the time of vaccine injection in children 0 to 18 years of age. We did not consider the management of delayed-onset pain occurring in the hours or days after the injection. Health care providers and researchers often use the term "distress" to refer to the combination of pain and anxiety or fear experienced by children before and during painful medical procedures. For the purposes of this guideline, we considered distress and pain together, referring to the com - bination as "pain." Topical anesthetics reduce pain associated with needle pro - cedures, including venipuncture and intravenous cannulation. 57 Our systematic review12 included 10 trials that evaluated the effects of topical anesthetics in a total of 1156 infants and children (up to 15 years of age).17,58-66 Of the seven studies that compared topical anesthetics with placebo cream or patch, six showed that these drugs were effective in reducing pain.58-63 In the negative study,64 older children (11-15 years) were enrolled. In addition, there were certain methodologic limitations that might explain the results, including use of an insensitive pain assessment method and rating of pain performed with the help of the physician. In two trials that included a no-treatment (control) group,65,66 topical anesthetics were ineffective, and in another trial, they were effective.17 Again, these results might be explained by some methodo - logic limitations, including lack of blinding and nurses' interactions with the no-treatment (control) group, which equalized responses between the groups; increased anticipatory anxiety because of a one-hour application time; and close proximity of peers (children from the same classroom) influencing self-reported pain ratings. We concluded that topical anesthetics are effective for reducing vaccination pain. We found no evidence of interference with vaccine immunogenicity for measles-mumps-rubella vaccine or the vaccines for diphtheria, tetanus, acellular pertussis, poliovirus, Hemophilus influenzae type B and hepatitis B.59-61 Here, we provide specific guidance regarding the method of pain assessment for children of different ages, according to our consensus interpretation of the vaccination literature, considering the validity and feasibility of currently available pain assessment methods. For preverbal children and infants, adult observers (parents, health care providers or both) are required to assess pain. We recommend that health care providers use one of two observational tools: the Modified Behavioural Pain Scale96-98 (for infants up to 18 months of age) or the Face Legs Activity Crying Consolability scale99 (for infants over 18 months of age). Parents should use a global rating scale (e.g., numerical rating scale or visual analogue scale). Verbal children can be asked to self-report pain100 using age-appropriate techniques: either the Poker Chip tool101 (3 to 6 years of age), the Faces Pain Scale - Revised102 (4 to 16 years of age) or a numerical rating scale103,104 (9 years of age and older). We encourage health care providers to document the strategies used to reduce acute pain at the time of vaccine injection, as well as the child's pain score.
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