Wilson's disease in children: 37-year experience and revised King's score for liver transplantation.

Wilson's disease (WD) is a rare liver-based disorder of copper metabolism. Prognostic criteria described by our group in 1986 to predict death without transplantation have not been universally validated. The clinical features of 88 children were reviewed, retrospectively in 74 and prospectively in 14. Data from the retrospectively recruited patients that died or survived on long-term chelation were used to evaluate the validity of our old scoring system and to devise a new prognostic index, then assessed in the 14 prospectively recruited patients. Using the old scoring system, 5 children scoring > or = 7, the cutoff value for death without transplantation, survived, whereas 4 scoring < or = 7 died (sensitivity 87% and specificity 90%). A new index based on serum bilirubin, international normalized ratio, aspartate aminotransferase (AST), and white cell count (WCC) at presentation identified a cutoff score of 11 for death and proved to be 93% sensitive and 98% specific, with a positive predictive value of 88%. When the new index was evaluated prospectively in 14 patients, it predicted the need for transplantation in only the 4 who required it, although 1 child with a score of 11 survived on medical treatment. In conclusion, the new Wilson Index is more sensitive and specific in predicting mortality without transplantation than the old scoring system, but needs to be validated in a larger number of patients.     

Residents can be trained to detect abdominal aortic aneurysms using personal ultrasound imagers: a pilot study.

Our objective was to test the hypothesis that internal medicine residents can be trained to screen for abdominal aortic aneurysm (AAA) using personal ultrasound imagers. We trained 5 randomly chosen internal medicine residents to image the abdominal aorta for patients with risk factors for AAAs using personal ultrasound imagers. Residents were trained in 3 or 4 one-on-one sessions with an instructor. To be eligible, patients had to be older than 65 years and have hypertension. After training, each of the 5 residents studied 3 patients independently. In 12 of the residents' 15 unsupervised studies, their abdominal aorta measurements were within 5 mm of the instructor's measurements with standard echocardiography (mean difference 3 mm, range 0-6 mm). Residents detected 3 previously unknown AAAs measuring 5.2, 4.2, and 3.9 cm in diameter. We conclude residents can be trained to image the abdominal aorta with personal ultrasound imagers and to identify AAAs in patients at risk.     

WONCA Online电子病例介绍——球孢子菌病

·病例简介:患者,男,22岁。因鼻部(见图1)、左侧肩部(见图2)、右侧背部及左侧大腿部皮疹就诊。既往身体健康,近期有到加利福尼亚中央谷旅游史,否认异地长期居住史,近期无服药史。患者无发热,无夜间盗汗,无寒战。既往诊断为耐甲氧西林金黄色葡萄球菌(MRSA)感染,接受甲氧苄氨嘧啶     

Botulinum Toxin A in the Treatment of Chromhidrosis

Background. Chromhidrosis is an uncommon disorder characterized by secretion of colored sweat by apocrine glands, typically localized to the face or axilla. The current treatments available for chromhidrosis are time consuming and frequently ineffective.
Objective. Our purpose is to demonstrate a novel approach to the treatment of apocrine chromhidrosis.
Methods. We report a case of apocrine chromhidrosis successfully treated with botulinum toxin A (BTX-A; Botox).
Results. BTX-A therapy successfully controlled facial chromhidrosis, and the effects were visible at 19 weeks post-treatment. The therapeutic benefits may be attributed to its inhibitory effects on cholinergic stimulation, adrenergic stimulation, and substance P release, although further studies are necessary to elucidate the precise mechanism of action.
Conclusion. This report demonstrates a new therapeutic approach to patients suffering from chromhidrosis.     

Delivering syringe exchange services through “satellite exchangers”: the Sacramento Area Needle Exchange, USA

An important operational aspect of Syringe Exchange Programmes (SEPs) is the venue of service delivery. This report describes the programmatic features of the Sacramento Area Needle Exchange (SANE), an illegal SEP operating in California, USA. SANE utilises “satellite exchangers” to distribute the bulk of its syringes and HIV risk reduction supplies. Advantages of relying primarily on Designated Exchangers (DE) for delivery of SEP services are that it: (1) allows for coverage of a large geographical area; (2) keeps operational cost low; (3) provides syringes to clients who may not want to or cannot use fixed site programmes; (4) limits the possibility of detection of programme personnel and clients by law enforcement. Limitations are that: (1) it is not as conducive as fixed sites to providing a wide range of ancillary services; (2) it may not be optimal for drug users who do not want to be reliant on other people for access to syringes; (3) those who receive services from a satellite exchanger may not derive as much counselling and referral services as direct exchangers. The lack of legal status, political support and adequate funding threatens the programme’s existence.