Familial hypercholesterolaemia commonly presents with Achilles tenosynovitis.

BACKGROUND: Patients with heterozygous familial hypercholesterolaemia (HeFH) develop tendon xanthomata (TX), most commonly in their Achilles tendons. Even before tendons are chronically enlarged, tenosynovitis may occur and medical advice be sought. Untreated HeFH carries a high risk of premature coronary heart disease, which can be ameliorated by early diagnosis. OBJECTIVE: To determine the prevalence of episodes of Achilles tendon pain in HeFH before its diagnosis. METHODS: Patients with definite HeFH (Simon Broome criteria) attending a lipid clinic were identified. They completed a questionnaire asking about symptoms relating to their Achilles tendons. Unaffected spouses or cohabiting partners served as controls. RESULTS: 133 patients (47% men) and 87 controls (51% men) participated. TX had been recognised by the referring physicians in <5% of cases. However, 62 (46.6% (95% confidence interval (CI) 38.1 to 55.1)) patients had experienced one or more episodes of pain in one or both Achilles tendons lasting >3 days, whereas only 6 (6.9% (1.6 to 12.2)) controls had done so (difference p<0.001; likelihood ratio 6.75). Typically, in the patients with HeFH the pain lasted 4 days (median). It was described as severe or very severe in 24/62 (38.7% (30.4 to 47.0)) patients with HeFH, but never more than moderate in controls. 35 (26.3% (18.8 to 33.8)) patients with HeFH had consulted a doctor about Achilles tendon pain, but in no case had this led to a diagnosis of HeFH. None of the controls had consulted a doctor. CONCLUSIONS: Measurement of serum cholesterol in patients presenting with painful Achilles tendon could lead to early diagnosis of HeFH.

Discrepancies between clinical and autopsy diagnosis and the value of post mortem histology; a meta-analysis and review.

The autopsy is in decline, despite the fact that accurate mortality statistics remain essential for public health and health service planning. The falling autopsy rate combined with the Coroners Review and Human Tissue Act have contributed to this decline, and to a falling use of autopsy histology, with potential impact on clinical audit and mortality statistics. At a time when the need for reform and improvement in the death certification process is so prominent, we felt it important to assess the value of the autopsy and autopsy histology. We carried out a meta-analysis of discrepancies between clinical and autopsy diagnoses and the contribution of autopsy histology. There has been little improvement in the overall rate of discrepancies between the 1960s and the present. At least a third of death certificates are likely to be incorrect and 50% of autopsies produce findings unsuspected before death. In addition, the cases which give rise to discrepancies cannot be identified prior to autopsy. Over 20% of clinically unexpected autopsy findings, including 5% of major findings, can be correctly diagnosed only by histological examination. Although the autopsy and particularly autopsy histology are being undermined, they are still the most accurate method of determining the cause of death and auditing accuracy of clinical diagnosis, diagnostic tests and death certification.

Pyoderma gangrenosum in a thoracotomy wound associated with a pulmonary cavitating lesion.

We present a patient with both pyoderma gangrenosum in a thoracotomy wound and a pulmonary cavitating lesion with the histological features of Wegener's granulomatosis. An ulcer with blue undermined edges developed in our patient's thoracotomy scar after a lobectomy was carried out for the cavitating lung lesion and c Antineutrophil Cytoplasmic Antibodies (cANCA), which is highly specific for active Wegener's granulomatosis but was negative at the time of her skin lesion. This suggests that our patient had cutaneous pyoderma gangrenosum rather than cutaneous Wegener's granulomatosis, although the two conditions may occasionally have similar clinical and histological features.

Accuracy of magnetic resonance imaging in determining cause of sudden death in adults: comparison with conventional autopsy.

AIM: To determine the accuracy and define the limitations of post mortem magnetic resonance imaging (MRI) in determining the cause of sudden death in adults. METHODS AND RESULTS: Sudden unexpected adult deaths in the community, reported to the Coroner (n = 10), excluding suspicious, violent or potentially drug-related deaths, were submitted to whole body MRI, followed by full invasive autopsy. The MRI scans were reported independently by four radiologists, blinded to the autopsy findings; two had previous experience of post mortem MRI. An abnormality that related to the cause of death as identified at autopsy, was identified by at least one radiologist in eight cases. These were pulmonary consolidation (autopsy finding pneumonia) (n = 1), pneumoperitoneum (autopsy finding perforated peptic ulcer) (n = 2), left ventricular failure (autopsy finding ischaemic heart disease) (n = 4), and disseminated bronchial carcinoma (n = 1). However, in only one case were all radiologists able to provide a confident cause of death (disseminated bronchial carcinoma). In two cases, in which death occurred 2-6 days and 3-6 days before MRI, early decomposition prevented interpretation of the images. Severe coronary artery atheroma was detected at autopsy in 7/10, but these lesions were not detected by MRI. Previous experience in reporting post mortem MRI, without autopsy comparison, did not result in more accurate interpretation of the images. CONCLUSIONS: This pilot study suggests that post mortem MRI can identify some abnormalities relating to the common causes of sudden death in adults, but there is a need for greater experience in correlating MRI with autopsy findings before a reliable cause of death can be made by MRI alone. Inability to image coronary artery lesions, differentiating thrombus from clot and pulmonary oedema from pneumonic exudates, are specific problems that may be corrected with greater experience and higher resolution scans.

The autopsy: complete or not complete?

Less invasive or non-invasive alternatives to the complete autopsy have been sought for some time, and a range of methods, ranging from needle sampling to endoscopy to magnetic resonance imaging, have been considered. Evaluations of these methods are few and far between, but generally confirm the predictable conclusion that incomplete autopsies provide incomplete information. It is not difficult to envisage a situation whereby pressure for non-invasive autopsies will allow them to become prevalent, whether properly evaluated or not. However, used appropriately, non-invasive or less invasive autopsies may be valuable tools capable of answering specific questions in situations where it is not possible to perform a complete autopsy.

"Bones of Contention". The donation of temporal bones for dissection after the organ-retention scandals.

It is essential for the Otologist in training to become familiar with the anatomy of the temporal bone. Generations of trainees have acquired this knowledge, together with basic surgical skills and techniques by dissection of cadaver temporal bones. In recent years, however, the supply of temporal bones for dissection has become a contentious issue in many British hospitals. Media and public attention has recently focused on issues surrounding the retention of human tissues following post-mortem examinations. In response, the Chief Medical Officer, the Royal College of Pathologists in the UK, the Faculty of Pathology of the Royal College of Physicians in Ireland, the British Medical Association, and other bodies have issued new guidelines that are of relevance to the Otolaryngologist. This paper gives details of some of the UK guidelines and where they can be accessed. A copy of the Royal College of Pathologists preferred post-mortem consent form is presented. The authors' experience in Manchester is discussed and details of an information leaflet on the subject of temporal bone donation are given. It is concluded that, with knowledge of the existing guidelines and good communication with other hospital colleagues, as well as families of the deceased, it is still possible legally to obtain temporal bones for dissection.

Attitudes of nursing staff to the autopsy.

We assessed the views of hospital nurses on the autopsy, using a questionnaire which was distributed by hand to all qualified nurses working at Manchester Royal Infirmary during a 48-h period. We found that many respondents (43%) had had occasion to discuss autopsy with relatives, and the majority (90%) would like to learn more about counselling the bereaved. Few (15%) clearly understood the legal distinction between coroners' and hospital autopsies. Almost all (97%) thought the main reason for autopsy was determination of the cause of death, and only 12% supported a role in clinical audit. More respondents saw benefits for doctors rather than for nurses, and only 56% perceived a benefit for relatives. In general, nursing staff have a more positive view of the autopsy than we anticipated, and we would advocate making more information about the benefits of autopsy available to nurses.

What is a natural cause of death? A survey of how coroners in England and Wales approach borderline cases.

AIM: Many deaths fall in the "grey" area between those that are clearly natural and those that are unnatural. There are no guidelines to help doctors in dealing with such cases and death certification is often arbitrary and inconsistent. In an attempt to initiate debate on these difficult areas, and with the ultimate aim of achieving national consensus, the views of coroners in England and Wales were sought. METHODS: Sixteen clinical scenarios, with causes of death, were circulated to all coroners in England and Wales. For each case they were asked to provide a verdict, with explanation. The deaths fell into three groups: (1) postoperative, (2) a combination of trauma and natural disease, and (3) infectious disease. RESULTS: Sixty four questionnaires were returned. There was near consensus (> 80% concordance) in only two of the 16 cases. In five, there was no significant agreement between coroners in the verdicts returned ("natural causes" versus "misadventure/accidental"). These included all three cases in which death resulted from a combination of trauma and natural disease (a fall after a grand mal fit; falls resulting in fractures of bones affected by metastatic carcinoma and osteoporosis), bronchopneumonia after hip replacement for osteoarthritis, and new variant Creutzfeldt-Jakob disease. The comments made for each case indicate that the variation between coroners in whether or not to hold an inquest, and the verdict arrived at, reflect the lack of a definition for natural causes, together with differences in the personal attitudes of each coroner. CONCLUSIONS: There is considerable variation in the way in which coroners approach these borderline cases, many of which are common in clinical practice. This study indicates a need for discussion, working towards a national consensus on such issues. It highlights the importance of good communication between coroners and medical staff at a local level.

Aspergillus fumigatus catalases: cloning of an Aspergillus nidulans catalase B homologue and evidence for at least three catalases.

The presence of catalases in the water soluble fractions of three Aspergillus fumigatus strains was investigated using non-denaturing and denaturing polyacrylamide gel electrophoresis and Western analysis. Using non-denaturing polyacrylamide gel electrophoresis and staining for catalase activity, three separate catalases were identified. An A. fumigatus catalase gene (catB) was cloned from genomic DNA using the Aspergillus niger catR gene as a probe. Polyclonal antibodies were raised to a glutathione S-transferase-CatB fusion product expressed in Escherichia coli. Western analysis indicated that, under denaturing conditions, the polyclonal antibody recognised a 90-kDa band and under non-denaturing conditions, two separate bands were identified. These results indicate that A. fumigatus in addition to CatB, produces at least two other catalases, one of which is similar in size to CatB. The polyclonal antibody was also used to observe catalase expression in mice, experimentally infected with A. fumigatus. Staining was observed heterogeneously throughout the fungal hyphae. This result indicates that catalase is produced by A. fumigatus during invasive aspergillosis.

Pathology and the OSCE: insights from pilot study.

Undergraduate medical education in Manchester in undergoing wholesale revision, with the introduction of problem-based learning (PBL) in each successive year of the curriculum, as the cohort of students who joined the faculty in 1994 advances through the course. This cohort has now entered year 3, which primarily hospital-based. In preparation for this, we have explored the development of an OSCE, not only to assess core interpersonal skills such as history taking, clinical examination, and the ability to explain things to patients, but also to integrate the examination of important skills relating to investigational sciences. These include the correct choice of laboratory tests, accurate interpretation of data, and appropriate selection of clinical responses to test results.

Conversion of a traditional image archive into an image resource on compact disc.

The conversion of a traditional archive of pathology images was organised on 35 mm slides into a database of images stored on compact disc (CD-ROM), and textual descriptions were added to each image record. Students on a didactic pathology course found this resource useful as an aid to revision, despite relative computer illiteracy, and it is anticipated that students on a new problem based learning course, which incorporates experience with information technology, will benefit even more readily when they use the database as an educational resource. A text and image database on CD-ROM can be updated repeatedly, and the content manipulated to reflect the content and style of the courses it supports.

A survey of general practitioners' views on autopsy reports.

AIMS: To study the views of general practitioners on the quality and utility of autopsy reports, and on autopsies in general. METHODS: For a period of six months, a questionnaire was enclosed with each autopsy report sent to a general practitioner from the mortuary at Manchester Royal Infirmary. RESULTS: Most (93.3%) general practitioners found the autopsy report useful, and many (66.7%) thought the bereaved relatives would do so too. However, only a minority (25.2%) would discuss the report with the relatives. A considerable proportion (20.0%) found the cause of death surprising, and a significant number (10.4%) felt the report would modify their future clinical practice. There was approval of autopsies in general, with most (88.6%) agreeing that autopsies reveal lesions not detected in life, and many (74.4%) indicating that loss of the autopsy would impair severely the monitoring of clinical standards. CONCLUSIONS: General practitioners appreciate autopsy reports, which may have a significant impact on clinical practice. Autopsy reports provide both case audit and information for relatives.