Difficult decisions in the intensive care unit: an illustrative case.

Difficult clinical decision-making is a common experience in intensive care units. There is often considerable pressure on time and decisions may have to be made in a stressful environment. Patients in the intensive care unit not infrequently present with extreme or rare manifestations of a disease process. Clinical evidence to guide management of such patients may be incomplete, non-existent, or its relevance to the problem at hand may be questionable. In this context, formal decision-making analytical tools are often impractical. Unconscious cognitive biases have been shown to play an important role in medical decision-making, particularly in these settings. While mostly these contribute to doctors making appropriate and timely decisions, occasionally they lead to errors. Despite 30 years of research into models of clinical reasoning, most doctors are unaware of how biases affect their thinking and are unfamiliar with techniques of detecting and neutralising bias in clinical practice. We present the case of a patient with Wegener's granulomatosis, which highlights many of the difficulties outlined above. We review the clinical evidence for our decisions at each stage and explain the rationale for our choices, highlighting the many situations for which high quality evidence was lacking. Examples of cognitive bias are identified and techniques of metacognition (thinking about thinking) that can be useful in limiting the effects of bias on complex decision-making are reviewed. The intensivist's evaluation of management alternatives has an important role in steering medical management towards optimal patient outcomes.

Synovial osteochondromatosis.

A 75-year-old man of Fijian-Indian extraction complained of a 3-year history of progressive right knee pain and stiffness which were limiting his mobility. On examination, multiple hard nodules were palpable in the popliteal fossa and along the path of the quadriceps tendon. The joint line was not tender, knee flexion was limited to 60 degrees and there was a fixed flexion deformity of 5 degrees. The knee ligaments were intact. Examination of other joints did not reveal nodules. His past medical history included: (i) polyarticular gout, (ii) osteoarthritis of the left knee requiring total knee joint replacement 7 years previously, (iii) ischaemic cardiomyopathy, (iv) chronic atrial fibrillation, (v) chronic renal impairment, (vi) recent bacteraemic melioidosis without a primary focus, (vii) chronic bilateral rotator cuff tears, (viii) low-grade multiple myeloma and (ix) idiopathic pulmonary fibrosis.

Emotional distress induced rhabdomyolysis in an individual with carnitine palmitoly-transferase deficiency.

A 48-year-old male patient with underlying CPT II enzyme deficiency is described. Emotional stress appeared to precipitate recurrent myalgias, rhabdomyolysis and reversible renal impairment over a 40-year period. Our search of the English literature indicates this to be the first time that the emotional stress has been documented to precipitate the CPT II syndrome. Although the pathogenesis of this syndrome has yet to be established, existing knowledge is briefly reviewed and the likely metabolic and neuroendocrine mechanisms which link emotional stress to muscle metabolism are examined. These mechanisms influence the extent of lipolysis or glycolysis that occurs during the process of muscle ATP generation. It is suggested that neuroendocrine and other stress related changes which favour lipolysis over glycolysis adversely affect muscle energy metabolism in patients whose mitochondria are deficient in CPT II enzyme. Possible treatment strategies are those that favour glycolysis over fatty acid metabolism and include a variety of ways of modulating sympathetic and parasympathetic tone. The use of carbohydrate supplementation beta-blockers and anxiolytic agents is discussed.

Relationship between rheumatoid arthritis and periodontitis.

BACKGROUND: Because of several similar features in the pathobiology of periodontitis and rheumatoid arthritis, in a previous study we proposed a possible relationship between the two diseases. Therefore, the aims of this study were to study a population of rheumatoid arthritis patients and determine the extent of their periodontal disease and correlate this with various indicators of rheumatoid arthritis. METHODS: Sixty-five consecutive patients attending a rheumatology clinic were examined for their levels of periodontitis and rheumatoid arthritis. A control group consisted of age- and gender-matched individuals without rheumatoid arthritis. Specific measures for periodontitis included probing depths, attachment loss, bleeding scores, plaque scores, and radiographic bone loss scores. Measures of rheumatoid arthritis included tender joint analysis, swollen joint analysis, pain index, physician's global assessment on a visual analogue scale, health assessment questionnaire, levels of C-reactive protein, and erythrocyte sedimentation rate. The relationship between periodontal bone loss and rheumatological findings as well as the relationship between bone loss in the rheumatoid arthritis and control groups were analyzed. RESULTS: No differences were noted for the plaque and bleeding indices between the control and rheumatoid arthritis groups. The rheumatoid arthritis group did, however, have more missing teeth than the control group and a higher percentage of these subjects had deeper pocketing. When the percentage of bone loss was compared with various indicators of rheumatoid arthritis disease activity, it was found that swollen joints, health assessment questionnaire scores, levels of C-reactive protein, and erythrocyte sedimentation rate were the principal parameters which could be associated with periodontal bone loss. CONCLUSIONS: The results of this study provide further evidence of a significant association between periodontitis and rheumatoid arthritis. This association may be a reflection of a common underlying disregulation of the inflammatory response in these individuals.

Osteoarticular brucellosis with long latent period.

We describe the case of a young male who manifested Brucella suis osteomyelitis after a 5-year period of relapsing monoarthritis. We discuss some of the difficulties regarding the diagnosis of osteoarticular brucellosis and review presentation patterns of the disease.

Is there a relationship between rheumatoid arthritis and periodontal disease?

AIM: The aim of this study was to determine whether there is a relationship between disease experience of rheumatoid arthritis and periodontal disease. METHODS: 1,412 individuals attending the University of Queensland's School of Dentistry were assessed for the prevalence of periodontal disease and rheumatoid arthritis. Analysis of data obtained from a self-reported health questionnaire and dental records was carried out and included: number of individuals referred for advanced periodontal care (test group); number of individuals attending for routine dentistry; determination of rheumatoid arthritis, cardiovascular disease and diabetes mellitus through self-reporting and assessment of prescription medications; assessment of periodontal disease through assessment of existing oral radiographs. RESULTS: In patients referred for periodontal treatment, the prevalence of self-reported rheumatoid arthritis was 3.95% which is significantly higher than that seen in patients not referred for periodontal treatment (0.66%) and also that reported in the general population (1%). Of those referred patients with rheumatoid arthritis, 62.5% had advanced forms of periodontal disease. These results were mirrored in the results of the self-reported prevalence of cardiovascular disease and diabetes mellitus which was consistent with the published higher prevalence in periodontal patients. CONCLUSIONS: Based on data derived from self-reported health conditions, and not withstanding the limitations of such a study, we conclude that there is good evidence to suggest that individuals with moderate to severe periodontal disease are at higher risk of suffering from rheumatoid arthritis and vice versa.

Perceptual variation in categorizing individuals according to American College of Rheumatology classification criteria for hand, knee, and hip osteoarthritis (OA): observations based on an Australian Twin Registry study of OA.

OBJECTIVE: The American College of Rheumatology (ACR) classification criteria for osteoarthritis (OA) permit the categorization of individuals for hand, knee, and hip OA and are of defined sensitivity and specificity. They depend on both clinical and radiographic aspects of OA. The clinical diagnosis of OA in the peripheral skeleton is dependent on the skilled examination of several clinical features characteristic of the condition, while the interpretation of radiographs is a perceptual skill based on appreciating specific structural features on plain radiographs. We investigated the interrater reliability of the ACR classification criteria for OA when applied in a community based sample. METHODS: The study was part of a multifaceted diagnostics protocol, evaluating methodologic issues, in the conduct of genetic research in OA. From a cohort of 118 pairs of twins registered with the Australian Twins Registry (ATR), standard clinical examinations of hands, knees, and hips were performed on 74 complete and 11 incomplete pairs of twins over age 50 years. The pairs were selected to represent both twin pairs who had previously self-reported a diagnosis of OA, as well as those who had not. Rheumatologists who performed the assessments were blind to the original self-report. All subjects were examined independently by one of 2 pairs (NB/AK or NB/KM) of consultant rheumatologists, blind to one another's assessments. Each rheumatologist separately assessed the hands, knees, and hips, rating them clinically by ACR criteria for OA. The observations were made without reference to any radiographic or serologic information. RESULTS: Interrater agreement was different for the 3 different anatomic areas and was different for the 2 pairs of rheumatologists. The actual (observed) interrater agreements based on ACR clinical criteria were as follows: hand OA NB/AK = 0.92, NB/KM = 1.00; knee OA NB/AK = 0.94, NB/KM = 0.92; hip OA NB/AK = 0.98, NB/KM = 0.97. Interrater agreement based on ACR clinical criteria, as assessed by the adjusted kappa statistic, was as follows: hand OA NB/AK = 0.84, NB/KM = 1.00; knee OA NB/AK = 0.87, NB/KM = 0.84; hip OA NB/AK = 0.95, NB/KM = 0.93. CONCLUSION: Since clinical agreement was extremely high in all 3 anatomic sites, and for both pairs of assessors, we conclude that for genetic epidemiology purposes, subjects can be examined by a single experienced rheumatologist using the ACR classification criteria.

Perceptual variation in grading hand, hip and knee radiographs: observations based on an Australian twin registry study of osteoarthritis.

OBJECTIVE: The radiographic diagnosis of osteoarthritis (OA) in the peripheral skeleton is dependent on the skilled examination of several morphological characteristics of the condition as visualised on plain radiographs. However, the process is perceptual and generally enhanced by comparison against photographic standards. This study assessed the intra-rater and inter-rater reliability of radiologists experienced in reporting hand, hip and knee films derived from a community-based sample when using the photographic atlas recently developed by Burnett et al. METHODS: This study was part of a multifaceted diagnostics protocol, evaluating methodological issues, in the conduct of genetic research in osteoarthritis. From a cohort of 118 twin pairs, registered with the Australian Twins Registry (ATR), standard clinical examinations were performed on 74 complete and 11 incomplete pairs of twins over age 50 years, followed by standard AP hand, AP pelvis and AP standing radiographs of the knees. The pairs were selected both to represent twin pairs who had previously self reported a diagnosis of OA, as well as those who had not. Radiologists read the films blind to the original self reported diagnosis and without reference to their pairing. The films were read by comparison against photographic standards and were scored according to specific features. All films were read independently by two consultant radiologists blind to one another's assessments, and selected films were thereafter assigned for rereading. Inter-rater and intra-rater agreement were different for different features, different anatomic areas, and, for the former, were different for the two radiologists. RESULTS: Inter-rater agreement was different for different anatomic areas, different radiographic features, and the two radiologists. Intra-rater agreement for the presence or absence of OA was as follows: actual observed agreement = 0.79 to 0.97 and 0.83 to 0.98; adjusted kappa statistic = 0.58 to 0.94 and 0.67 to 0.96; inter-rater agreement was as follows: actual observed agreement = 0. 77 to 0.97; adjusted kappa statistic = 0.54 to 0.94. Agreement was generally high in most of the principal target joints for OA: DIP, PIP, 1st CMC, hip and knee. CONCLUSIONS: Although assessor agreement was not perfect, it is concluded that for genetic epidemiology purposes, while duplicate assessments may be advantageous, it is possible for radiographs to be examined accurately by a single experienced assessor. However, for less experienced assessors independent examinations should be made by at least two assessors and either a consensus reached on disparate examinations or an algorithm developed to adjudicate any discrepancies.

Perceptual variation in categorizing individual peripheral joints for the presence or absence of osteoarthritis using a standard homunculus: observations based on an australian twin registry study of osteoarthritis.

The clinical diagnosis of osteoarthritis in the peripheral skeleton is dependent on the skilled examination of several features characteristic of the condition. However, we have previously observed that even highly experienced rheumatologists in Australia and Canada may not completely agree on the clinical examination in individuals with osteoarthritis (OA), rheumatoid arthritis, ankylosing spondylitis, fibromyalgia, scleroderma and painful shoulder.

Thrombocytopenia corrected by warfarin in antiphospholipid syndrome.

We describe a case of antiphospholipid associated thrombocytopenia in which warfarin, but not heparin, normalized the platelet count. Systemic lupus erythematosus was present in association with chorea, mitral valve stenosis, and renal thrombotic microangiopathy. No evidence of platelet consumption was detected. Low dose aspirin, pulse methylprednisolone, intravenous gammaglobulin, and cyclophosphamide also partially elevated the platelet count. Platelet activation, measured by P selectin assay, was not present during warfarin or aspirin therapy, suggesting that platelet activation may have been decreased by these treatments. We discuss possible mechanisms for correction of thrombocytopenia in antiphospholipid syndrome by warfarin.

Normal paraspinal muscle electromyographic fatigue characteristics in patients with primary fibromyalgia.

Paraspinal muscle fatigue mechanisms were compared in 14 primary fibromyalgia patients and 14 age and sex matched normal subjects using a standardized 60-s isometric endurance test of the paraspinal muscles, during which surface integrated electromyographic (IEMG) activity was recorded. Fatigue-induced IEMG increases were similar for both groups during the initial 40 s (up to 112 +/- 20% and 111 +/- 6% of initial values in patients and normal subjects respectively). Thereafter, IEMG fell significantly in patients (P < 0.05) but only slightly in controls, so that at 58 s IEMG was 102 +/- 13% in patients and 109 +/- 12% in controls. If patients were divided according to body mass index (BMI, range 19-25 in controls) those with a BMI < 26 (n = 5) showed IEMG changes similar to those of control subjects throughout the test, while obese patients with BMI > 26 (n = 9) showed greater IEMG declines after 40 s than either normal subjects or in the fibromyalgia group as a whole. Paraspinal muscle fatigue mechanisms appear normal in primary fibromyalgia patients. Isometric force maintenance in overweight patients, despite IEMG declines, illustrates the action of intrinsic fatigue resistance mechanisms which were presumably utilized to a greater extent in these patients to cope with the extra load.

Sensorimotor peripheral neuropathy in rheumatoid arthritis.

We describe 3 patients with severe sensorimotor neuropathy complicating rheumatoid arthritis. Two patients had evidence of vasculitis and an axonal neuropathy. These patients were unusual in that the neuropathy occurred early in the course of rheumatoid arthritis. The third patient had a demyelinating neuropathy with a high cerebrospinal fluid protein level, and is a probable example of a chronic inflammatory neuropathy occurring in rheumatoid arthritis. All patients improved or were stabilized with corticosteroid therapy.

A case of cerebral systemic lupus erythematosus treated with methylprednisolone pulse therapy.

A case of cerebral systemic lupus erythematosus is presented. This was characterised by a combination of organic, persecutory and atypical hypomanic features. After a very stormy course on oral steroids, treatment with intravenous methylprednisolone pulse therapy was begun. Subsequently the patient experienced a prolonged remission. This outcome supports the value of such therapy in cerebral systemic lupus erythematosus.

A comparison of Epstein-Barr virus-specific T-cell immunity in rheumatoid arthritis and osteoarthritis patients.

The level of Epstein-Barr virus (EBV)-specific T-cell-mediated immunity in 20 rheumatoid arthritis (RA) patients was compared with 16 age- and sex-matched osteoarthritis (OA) patients using the regression of EBV-transformation assay. The results show that the level of EBV-specific T-cell immunity in RA patients is significantly depressed compared with OA patients (P less than .001) or healthy laboratory controls (P less than .001). In contrast, lymphocytes from RA and OA patients showed a similar ability to act as a responder population in the mixed leucocyte reaction. It is unlikely that the difference in EBV-specific immunity is due to a general T-cell defect in RA patients since there was no correlation between EBV-specific T-cell immunity and mixed leucocyte reactivity. There was no correlation between EBV-specific T-cell immunity and any of the indicators of disease activity nor was there any difference in the anti-EBV antibody titre between both groups of patients. These results indicate that RA patients are deficient in the EBV-specific cytotoxic T-cell precursor population and may explain some of the reported observations of the involvement of EBV in this disease.

Treatment of xerostomia: a double-blind trial in 108 patients with Sjögren's syndrome.

The first-ever controlled study of a therapeutic modality for xerostomia is reported. A recently described formulation for saliva substitute (SS) has been tested against a glycerine mouthwash as a control saliva substitute (placebo) in a double-blind clinical trial in 108 patients with varying grades of xerostomia of Sjögren's syndrome. The results indicate that SS offered significant relief of nocturnal oral discomfort (p less than 0.02) and more patients reported "excellent" improvement (p less than 0.01) on a five-point graded response. In all other respects, the SS was not significantly better than the placebo. Significant adverse effects were not reported. It is suggested that any such current and future therapeutic modalities for Sjögren's syndrome be subjected to similar critical appraisal of their worth.

Computerised clinical data base system--Australia.

In search for a practical solution to analyse a large quantity of medical information, rheumatic disease data base system (RDDS) is described. Details of data configuration, data entry and retrieval are discussed in some detail. The system incorporates a range of commonly used statistical tests and provisions for more advanced data analysis are made. Additional facilities include provisions for a variety of printed reports, back up facilities and a word processor. The ease of system operation and flexibility are emphasised. Technical support requirements are minimal even when new applications are being developed. The system is fully operational at present and can be implemented on a number of computer systems.