Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

DESCRIPTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.

Surgeon clinical practice variation and patient preferences during the informed consent discussion: a mixed-methods analysis in lumbar spine surgery.

Background: Patients with lumbar disc herniation may greatly benefit from microdiscectomy. Although spine surgeons performing microdiscectomy routinely obtain informed consent, the potential adverse events they disclose often vary. Moreover, little is known about what disclosures are deemed most valuable by patients. The aim of this mixed-methods study was to determine practice variations among spine surgeons in regard to the disclosure of potential adverse events during informed consent discussions for lumbar microdiscectomy and to determine which topics patients perceived to be valuable in the consent discussion. Methods: A survey evaluating the frequency with which spine surgeons disclose 15 potential adverse events related to lumbar microdiscectomy during informed consent discussions was distributed among Canadian Spine Society members. Additionally, semistructured interviews were conducted with preoperative patients, postoperative patients, attending spine surgeons, spine fellows and orthopedic residents. Interview transcripts were analyzed using thematic analysis with open coding. Results: Fifty-one Canadian Spine Society members completed the survey. The number of potential adverse events not routinely discussed was greater among orthopedic surgeons than among neurosurgeons (relative risk 1.83; 95% confidence interval 1.22-2.73; p = 0.003). Three preoperative patients, 7 postoperative patients, 6 attending spine surgeons, 3 spine fellows and 5 orthopedic residents participated in the semistructured interviews. The interviews identified gaps in information provided to patients, particularly on topics relating to postoperative care such as expected recovery time, activity restrictions and need for a caregiver. Conclusion: There is variation in the disclosure of potential adverse events during informed consent discussions for lumbar microdiscectomy among Canadian spine surgeons. Patients desire more information regarding their postoperative care. Further research should focus on developing guidelines to reduce practice variation and optimize the effectiveness of consent discussions.

Contexte: Les patients atteints d'une hernie discale lombaire pourraient profiter grandement d'une microdiscectomie. Bien que les chirurgiens spécialistes de la colonne vertébrale réalisant des microdiscectomies obtiennent toujours le consentement éclairé du patient, les événements indésirables potentiels présentés varient souvent. De plus, on en connaît peu sur les informations les plus importantes du point de vue des patients. L'objectif de cette étude à méthodes mixtes était de déterminer les différentes pratiques des chirurgiens en ce qui a trait à la présentation des événements indésirables potentiels pendant les discussions sur le consentement éclairé pour les microdiscectomies lombaires et de déterminer les sujets les plus importants pour les patients pendant ces discussions. Méthodes: Un sondage sur la fréquence à laquelle les chirurgiens présentent 15 événements indésirables potentiels associés à la microdiscectomie lombaire pendant les discussions sur le consentement éclairé a été distribué aux membres de la Société canadienne du rachis. De plus, des entretiens semi-dirigés ont été réalisés auprès de patients en période préopératoire, de patients en période postopératoire, de chirurgiens spécialistes de la colonne vertébrale, de fellows en chirurgie spinale et de résidents en chirurgie orthopédique. Des analyses thématiques utilisant un code ouvert ont été réalisées sur les transcriptions des entretiens. Résultats: Cinquante-et-un membres de la Société canadienne du rachis ont répondu au sondage. Le nombre d'événements indésirables potentiels non systématiquement mentionnés était plus élevé chez les chirurgiens orthopédiques que chez les neurochirurgiens (risque relatif 1,83; intervalle de confiance de 95 % 1,22­2,73; p = 0,003). Dans les entretiens semi-dirigés, on a recueilli les commentaires de 3 patients en période préopératoire, de 7 patients en période postopératoire, de 6 chirurgiens spécialistes de la colonne vertébrale, de 3 fellows en chirurgie spinale et de 5 résidents en chirurgie orthopédique. Les entretiens ont révélé des lacunes dans l'information transmise aux patients, particulièrement sur les soins postopératoires, comme le temps de récupération attendu, les restrictions quant aux activités et la nécessité d'un soignant. Conclusion: On a trouvé une variation dans la présentation des événements indésirables potentiels pendant les discussions sur le consentement éclairé pour les microdiscectomies lombaires chez les chirurgiens spécialistes de la colonne vertébrale au Canada. Les patients veulent en savoir plus sur les soins postopératoires. Des lignes directrices devraient être établies pour réduire les différences entre les pratiques et optimiser l'efficacité des discussions sur le consentement.

Creating the right evidence for system change.

Most evaluative research is focused on assessing new technologies at the patient level. Comparatively little is focused on assessing how system changes could improve the delivery of healthcare. In this article, the authors describe an opportunity to conduct evaluative trials of system changes affordably and efficiently by using a cluster randomized design and mandatory reporting data, using the prevention of Clostridium difficile infection as an example. They then describe what must be done to make similar trials a regular tool of healthcare policy.

Does this patient with diabetes have osteomyelitis of the lower extremity?

CONTEXT: Osteomyelitis of the lower extremity is a commonly encountered problem in patients with diabetes and is an important cause of amputation and admission to the hospital. The diagnosis of lower limb osteomyelitis in patients with diabetes remains a challenge. OBJECTIVE: To determine the accuracy of historical features, physical examination, and laboratory and basic radiographic testing. We searched for systematic reviews of magnetic resonance imaging (MRI) in the diagnosis of lower extremity osteomyelitis in patients with diabetes to compare its performance with the reference standard. DATA SOURCES: MEDLINE search of English-language articles published between 1966 and March 2007 related to osteomyelitis in patients with diabetes. Additional articles were identified through a hand search of references from retrieved articles, previous reviews, and polling experts. STUDY SELECTION: Original studies were selected if they (1) described historical features, physical examination, laboratory investigations, or plain radiograph in the diagnosis of lower extremity osteomyelitis in patients with diabetes mellitus, (2) data could be extracted to construct 2 x 2 tables or had reported operating characteristics of the diagnostic measure, and (3) the diagnostic test was compared with a reference standard. Of 279 articles retrieved, 21 form the basis of this review. Data from a single high-quality meta-analysis were used to summarize the diagnostic characteristics of MRI in osteomyelitis. DATA EXTRACTION: Two authors independently assigned each study a quality grade using previously published criteria and abstracted operating characteristic data using a standardized instrument. DATA SYNTHESIS: The gold standard for diagnosis is bone biopsy. No studies were identified that addressed the utility of the history in the diagnosis of osteomyelitis. An ulcer area larger than 2 cm2 (positive likelihood ratio [LR], 7.2; 95% confidence interval [CI], 1.1-49; negative LR, 0.48; 95% CI, 0.31-0.76) and a positive "probe-to-bone" test result (summary positive LR, 6.4; 95% CI, 3.6-11; negative LR, 0.39; 95% CI, 0.20-0.76) were the best clinical findings. A erythrocyte sedimentation rate of more than 70 mm/h increases the probability of a diagnosis of osteomyelitis (summary LR, 11; 95% CI, 1.6-79). An abnormal plain radiograph doubles the odds of osteomyelitis (summary LR, 2.3; 95% CI, 1.6-3.3). A positive MRI result increases the likelihood of osteomyelitis (summary LR, 3.8; 95% CI, 2.5-5.8). However, a normal MRI result makes osteomyelitis much less likely (summary LR, 0.14; 95% CI, 0.08-0.26). The overall accuracy (ie, the weighted average of the sensitivity and specificity) of the MRI is 89% (95% CI, 83.0%-94.5%). CONCLUSIONS: An ulcer area larger than 2 cm2, a positive probe-to-bone test result, an erythrocyte sedimentation rate of more than 70 mm/h, and an abnormal plain radiograph result are helpful in diagnosing the presence of lower extremity osteomyelitis in patients with diabetes. A negative MRI result makes the diagnosis much less likely when all of these findings are absent. No single historical feature or physical examination reliably excludes osteomyelitis. The diagnostic utility of a combination of findings is unknown.

A systematic review of contact dermatitis treatment and prevention.

BACKGROUND: Contact dermatitis (CD) is a common occupational disease. There have been no systematic reviews of CD treatment or prevention. METHODS: Multiple databases were systematically searched. Using independent double review and published quality review criteria, articles were rated as good, fair, or poor. Treatment benefit data were tabulated and conclusions were based on the rated strength of published evidence. RESULTS: In all, 49 studies met inclusion criteria. Barrier creams containing dimethicone or perfluoropolyethers, cotton liners, and softened fabrics prevent irritant CD. Lipid-rich moisturizers both prevent and treat irritant CD. Topical skin protectant and quaternium 18 bentonite (organoclay) prevent rhus dermatitis. Diethylenetriamine pentaacetic acid (chelator) cream prevents nickel, chrome, and copper dermatitis. Potent or moderately potent steroids effectively treat allergic CD. There were no macrolide immunomodulator trials that met inclusion criteria. This review did not include studies of children, animals, or non-English language publications. CONCLUSIONS: A limited number of interventions effectively prevent or treat irritant and allergic CD, but well-controlled, outcome-blinded studies, particularly in the area of allergic CD prevention are needed.

"Futile" care: do we provide it? Why? A semistructured, Canada-wide survey of intensive care unit doctors and nurses.

PURPOSE: To qualitatively explore the process of the provision of futile care in Canadian intensive care units (ICUs). MATERIALS AND METHODS: 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 chi(2) analysis or Fisher exact test. Content analysis to identify common themes was carried out by 4 raters using a Delphi process. RESULTS: 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. CONCLUSIONS: 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.

Barriers to communication regarding end-of-life care: perspectives of care providers.

OBJECTIVE: Communication regarding end-of-life care is frequently perceived as suboptimal, despite the intent of both health care providers and patients. We interviewed health care providers to determine their perspective regarding these barriers to communication. MATERIALS AND METHODS: Eleven focus groups with a total of 10 attending physicians, 24 residents, and 33 nurses were convened to explore barriers to end-of-life discussions on the Internal Medicine service at a 600-bed tertiary care hospital in Toronto, Canada. An interview schedule was designed to elicit information regarding the process of end-of-life discussions, barriers to these discussions, and possible interventions for limiting such barriers. Transcripts were qualitatively analyzed by 6 raters who independently identified "themes." Themes were refined using the Delphi technique and classified under broader "categories." RESULTS: Four main categories of barriers emerged, relating to (1) patients, (2) the health care system, (3) health care providers, and (4) the nature of this dialogue. Attending physicians and residents most frequently identified patient-related factors as barriers to discussions, followed by system, dialogue, and provider barriers (43%, 39%, 10%, and 8%, respectively, for attending physicians; 40%, 34%, 13%, and 13%, respectively, for residents). Nurses similarly identified patient-related and system barriers most frequently, but provider barriers were discussed more often than dialogue barriers (46%, 28%, 22%, and 4%, respectively). CONCLUSIONS: Attending physicians, residents, and nurses perceive the recipients of their care, and the system within which they provide this care, to be the major source of barriers to communication regarding end-of-life care. This finding may impact on the effectiveness of quality-improvement initiatives in end-of-life care.

A comprehensive evidence-based approach to fever of unknown origin.

BACKGROUND: Fever of unknown origin (FUO) is defined as a temperature higher than 38.3 degrees C on several occasions and lasting longer than 3 weeks, with a diagnosis that remains uncertain after 1 week of investigation. METHODS: A systematic review was performed to develop evidence-based recommendations for the diagnostic workup of FUO. MEDLINE database was searched (January 1966 to December 2000) to identify articles related to FUO. Articles were included if the patient population met the criteria for FUO and they addressed the natural history, prognosis, or spectrum of disease or evaluated a diagnostic test in FUO. The quality of retrieved articles was rated as "good," "fair," or "poor," and sensitivity, specificity, and diagnostic yield of tests were calculated. Recommendations were made in accordance with the strength of evidence. RESULTS: The prevalence of FUO in hospitalized patients is reported to be 2.9%. Eleven studies indicate that the spectrum of disease includes "no diagnosis" (19%), infections (28%), inflammatory diseases (21%), and malignancies (17%). Deep vein thrombosis (3%) and temporal arteritis in the elderly (16%-17%) were important considerations. Four good natural history studies indicate that most patients with undiagnosed FUO recover spontaneously (51%-100%). One fair-quality study suggested a high specificity (99%) for the diagnosis of endocarditis in FUO by applying the Duke criteria. One fair-quality study showed that computed tomographic scanning of the abdomen had a diagnostic yield of 19%. Ten studies of nuclear imaging revealed that technetium was the most promising isotope, showing a high specificity (94%), albeit low sensitivity (40%-75%) (2 fair-quality studies). Two fair-quality studies showed liver biopsy to have a high diagnostic yield (14%-17%), but with risk of harm (0.009%-0.12% death). Empiric bone marrow cultures showed a low diagnostic yield of 0% to 2% (2 fair-quality articles). CONCLUSIONS: Diagnosis of FUO may be assisted by the Duke criteria for endocarditis, computed tomographic scan of the abdomen, nuclear scanning with a technetium-based isotope, and liver biopsy (fair to good evidence). Routine bone marrow cultures are not recommended.

Probiotics for the prevention of antibiotic-associated diarrhea and Clostridium difficile infection among hospitalized patients: systematic review and meta-analysis.

BACKGROUND: Antibiotic-associated diarrhea (AAD) and Clostridium difficile infection (CDI) are associated with high morbidity, mortality, and health care costs. Probiotics may mitigate the existing disease burden. We performed a systematic review and meta-analysis to evaluate the efficacy of co-administration of probiotics with antibiotics in preventing these adverse outcomes in adult inpatients. METHODS: Systematic searches of MEDLINE (1946 to May 2012), Embase (1980 to May 2012), and the Cochrane Central Register of Controlled Trials were undertaken on May 31, 2012, to identify relevant publications. We searched for randomized controlled trials, published in English, of adult inpatients who were receiving antibiotics and who were randomly assigned to co-administration of probiotics or usual care, with or without the use of placebo. Studies were included if they reported on AAD or CDI (or both) as outcomes. Data for predetermined criteria evaluating study characteristics, methods, and risk of bias were extracted. Trials were given a global rating of good, fair, or poor by at least 2 reviewers. Meta-analyses were performed using a random-effects model, and pooled relative risks (RRs) and 95% confidence intervals (CIs) were calculated. RESULTS: Sixteen trials met the criteria for inclusion in this review. Four studies were of good quality, 5 were of fair quality, and 7 were of poor quality. Pooled analyses revealed significant reductions in the risks of AAD (RR 0.61, 95% CI 0.47 to 0.79) and CDI (RR 0.37, 95% CI 0.22 to 0.61) among patients randomly assigned to co-administration of probiotics. The number needed to treat for benefit was 11 (95% CI 8 to 20) for AAD and 14 (95% CI 9 to 50) for CDI. With subgroup analysis, significant reductions in rates of both AAD and CDI were retained in the subgroups of good-quality trials, the trials assessing a primarily Lactobacillus-based probiotic formulation, and the trials for which the follow-up period was less than 4 weeks. INTERPRETATION: Probiotics used concurrently with antibiotics reduce the risk of AAD and CDI.

How can we improve guideline use? A conceptual framework of implementability.

BACKGROUND: Guidelines continue to be underutilized, and a variety of strategies to improve their use have been suboptimal. Modifying guideline features represents an alternative, but untested way to promote their use. The purpose of this study was to identify and define features that facilitate guideline use, and examine whether and how they are included in current guidelines. METHODS: A guideline implementability framework was developed by reviewing the implementation science literature. We then examined whether guidelines included these, or additional implementability elements. Data were extracted from publicly available high quality guidelines reflecting primary and institutional care, reviewed independently by two individuals, who through discussion resolved conflicts, then by the research team. RESULTS: The final implementability framework included 22 elements organized in the domains of adaptability, usability, validity, applicability, communicability, accommodation, implementation, and evaluation. Data were extracted from 20 guidelines on the management of diabetes, hypertension, leg ulcer, and heart failure. Most contained a large volume of graded, narrative evidence, and tables featuring complementary clinical information. Few contained additional features that could improve guideline use. These included alternate versions for different users and purposes, summaries of evidence and recommendations, information to facilitate interaction with and involvement of patients, details of resource implications, and instructions on how to locally promote and monitor guideline use. There were no consistent trends by guideline topic. CONCLUSIONS: Numerous opportunities were identified by which guidelines could be modified to support various types of decision making by different users. New governance structures may be required to accommodate development of guidelines with these features. Further research is needed to validate the proposed framework of guideline implementability, develop methods for preparing this information, and evaluate how inclusion of this information influences guideline use.