International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

BACKGROUND: 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. OBJECTIVE: 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. METHODS: 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. RESULTS: 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.

Patient recall and appropriate timing for obtaining informed consent for endoscopic procedures.

OBJECTIVES: Although informed consent is an issue in many medical malpractice claims, there is no standardized time or method to obtain informed consent for endoscopic procedures. The objectives of this study were to determine whether sedation for endoscopic procedures interfered with pre-endoscopic informed consent and to determine the appropriate time to obtain informed consent. METHODS: Patients undergoing a sedated esophagogastroduodenoscopy had informed consent obtained either 48-72 h before the procedure (group 1A, n = 50) or 10-60 min before the procedure (group 1B, n = 50). Patients undergoing an unsedated flexible sigmoidoscopy had informed consent obtained either 48-72 h before (group 2A, n = 47) or 10-60 min before the procedure (group 2B, n = 49). Methods of informed consent consisted of an oral and a written explanation about the procedure. Patients were sedated with midazolam and meperidine. A Trieger test evaluated recovery from sedation. Recall was assessed by asking six questions about the procedure before discharge and again 2-3 days later. RESULTS: Standard t tests and Mann-Whitney U nonparametric rank tests were used to compare the 1) 1-h recall scores, 2) 2-3-day recall scores, and 3) recall difference scores for groups 1A and 1B, 1A and 2A, 2A and 2B, and 1B and 2B. There were no differences in recall for the different groups. CONCLUSIONS: This study shows that sedation for endoscopic procedures does not interfere with pre-endoscopic informed consent. Informed consent for endoscopic procedures can be obtained at any time before sedation with similar recall.

Academic models for practice relief, recruitment, and retention at the University of New Mexico Medical Center and East Carolina University School of Medicine.

Locum tenens programs were independently developed by the University of New Mexico Health Sciences Center and East Carolina University School of Medicine to address health care needs within their states. The programs represent distinct models to provide locum tenens services for practices in rural and medically underserved regions through collaboration with stage government representatives and agencies. Differences between programs include years of operation, days of coverage provided, types of learners and providers involved, sources of funding, costs of the programs, and extent of institutional support. Common beneficial outcomes of the programs include coverage for struggling practices, training in rural medicine for locum tenens providers, recruitment of physicians to rural practice sites, and improved relationships with program partners. Adequate funding and institutional support are essential for success of locum tenens programs.

Prospective evaluation of a teaching model to determine competency in performing flexible sigmoidoscopies.

OBJECTIVE: There are limited objective criteria for determining competency in performing flexible sigmoidoscopies. The authors developed a teaching model using eight objective criteria that measured both cognitive and technical skills in performing flexible sigmoidoscopies. The objective was to determine whether the teaching model was a valid method and then to compare it to a commonly used Overall Competence Score or independent observer method. METHODS: A total of 120 procedures with 10 residents were evaluated using the two methods. Eight objective parameters had been determined before validating the teaching model. Preset criteria determined competency on a single procedure, and competent performance on five procedures in a row determined certification to independently perform procedures. The teaching model was separately tested for validity and then compared with the independent observer method. RESULTS: A total of 120 procedures were graded using the teaching model method: 73 competent and 47 incompetent. Six of the seven objective parameters were associated with competency in the teaching model method. The eighth objective parameter was excluded because of lack of variation. Fifty of the 120 procedures were graded using the independent observer method: 36 competent and 14 incompetent. Two of the seven objective parameters were associated with competency in the independent observer method. Comparison of the two methods revealed seven discrepancies regarding competent and incompetent procedures. The correlation between the two methods was 0.71. CONCLUSION: The teaching model method offers objective criteria to evaluate skill in performing flexible sigmoidoscopies.

Campho-Phenique ingestion: an intentional overdose.

We report a suicide attempt with the camphorated phenol preparation Campho-Phenique. The total dose ingested was 68 mg/kg of camphor and 28.9 mg/kg of phenol. The patient had grand mal seizures minutes after ingestion. Supportive medical care and intubation resulted in full recovery within 12 hours. Although Campho-Phenique has been discussed extensively in the pediatric literature and its accidental ingestion by adults has occasionally been reported, intentional ingestion of the preparation has not been reported. We discuss our unusual case and review the literature.

Antibiotic prophylaxis in percutaneous endoscopic gastrostomy.

OBJECTIVES: The benefit of antibiotic prophylaxis in percutaneous endoscopic gastrostomy is controversial. The aim of this study is to determine whether prophylactic antibiotic treatment with Cefazolin reduces the incidence of peristomal infection after percutaneous endoscopic gastrostomy. METHODS: Of the 131 hospitalized or nursing home patients referred for percutaneous endoscopic gastrostomy, 115 were enrolled in a prospective randomized double-blind placebo controlled trial. Sixty-one (group 1) were randomized in a double-blind fashion and received either Cefazolin or saline pregastrostomy. Fifty-four patients (group 2) were on antibiotics for prior medical indications pregastrostomy. Patients had their peristomal area evaluated on a daily basis for 1 wk after gastrostomy. Erythema and exudate were scored on a scale from 0 to 4; induration was scored on a scale of 0 to 3; a maximum score of 8 or higher or the presence of pus was criteria for infection. RESULTS: Wound infection occurred in 4 of 30 (13%) participants receiving Cefazolin and in 6 of 31 (19%) participants receiving saline (p > 0.5). In the 54 patients on antibiotics for prior indications, wound infection was observed in 2 subjects (3%). This finding was a significant difference when compared with the placebo group (p < 0.02). CONCLUSIONS: A single dose of Cefazolin prophylaxis does not reduce the overall peristomal wound infection in percutaneous endoscopic gastrostomy. Patients receiving prior extended antibiotic therapy have fewer peristomal wound infections.

Colobronchial fistula: an unusual complication of Crohn's disease.

Fistulas secondary to Crohn's disease occur in about 33% of patients. A colobronchial fistula complicating Crohn's disease is very rare, having been reported only twice previously. We present an unusual fistula secondary to Crohn's colitis that originated from the splenic flexure and crossed the diaphragm to involve the bronchial tree.

Mycobacteremia and granulomatous hepatitis following initial intravesical bacillus Calmette-Guerin instillation for bladder carcinoma.

We describe the first case of mycobacteremia and granulomatous hepatitis occurring after the initial intravesical instillation of bacillus Calmette-Guerin (BCG) for bladder cancer. Eight days after BCG instillation, a liver biopsy revealed well-defined granulomas and acid-fast bacilli. A blood culture drawn 8 h after BCG instillation grew Mycobacterium bovis. We summarize the reported complications of BCG intravesical immunotherapy, the associated risk factors, and discuss options for treatment. Although rare, mycobacteremia and granulomatous hepatitis are important systemic side effects of intravesical instillation of BCG for bladder cancer, and should be considered in any patient who presents with persistent fever and abnormal liver function tests after instillation of BCG.

Small cell carcinoma of the esophagus in a patient with longstanding primary achalasia.

Achalasia is believed to be a predisposing factor for the development of esophageal cancer. Small cell carcinoma of the esophagus is a rare neoplasm, with fewer than 150 cases having been reported in the world literature, and it has been described only once previously in a patient with longstanding achalasia. We describe a case of an 85-yr-old woman with long-term primary achalasia who developed primary small cell carcinoma of the esophagus. We hypothesize that this patient's recurrent, worsening dysphagia is related to a paraneoplastic phenomenon. We discuss this association and review the literature.