bluuebunny/crossref_metadata_2025_split huggingface dataset:
Familial iron overload with possible autosomal dominant inheritance
Authors: Eason R. J. , Adams P. C. , Aston C. E. , Searle J. 
Published: March 2008

AbstractA 96 member Melanesian kindred with 31 cases of iron overload is reported. Liver biopsies from 19 of these patients showed features similar to those of genetic haemochromatosis in Caucasians, but in contrast to the previous reported HLA‐linked autosomal recessive pattern of inheritance for haemochromatosis, this family shows a pattern that is most consistent with autosomal dominant inheritance. This is suggested by involvement of three and possibly four consecutive generations, with a high frequency of transmission from parents to children and equal gender distribution. Linkage and segregation analysis supported dominant inheritance, with no demonstrable HLA linkage.
Continuous arteriovenous haemodiafiltration: optimal therapy for acute renal failure in an intensive care setting?
Authors: Bellomo R. , Ernest D. , Love J. , Parkin G. , Boyce N. 
Published: March 2008

AbstractWe report the results of continuous arteriovenous haemodiafiltration (CAVHD) treatment in 12 critically ill intensive care patients with acute renal failure (eight males, four females ‐ mean age 60.9 years ‐ range 47 to 76) (APACHE II score 28.8, range 18–37). All patients were oligo‐anuric or had a rising creatinine ( 100 JμM/L per day). Vascular access was obtained by Scribner shunt or wide‐bore femoral arterial and venous cannulae. At the beginning of CAVHD therapy the mean plasma urea was 38 mM/L (SE 4.5, 95% confidence interval (CI) 25.1 to 75.6 mM/L) and the mean creatinine was 604 μM/L (SE 70, 95% CI 450–756 μM/L). After 72 hours of therapy, despite oligoanuria, urea concentration had fallen to a mean of 15.7 mM/L (SE 2.4, 95% CI 12.5–22.9 mM/L) and the creatinine concentration to 297 μM/L (SE 25, 95% CI 243–351 μM/L), respectively. The mean ultrafiltrate volume was 441 mL/hr (SE 33, 95%, range 50–1050 mL/hr). There were no complications related to the extracorporeal circuit, the filter, anticoagulant therapy, electrolyte status or changes in patients' haemodynamic state. Excellent biochemical control of azotaemia was uniformly achieved during CAVHD therapy. Five patients (41.6%) survived to be discharged from the Intensive Care Unit. CAVHD is a simple, safe and effective continuous renal replacement therapy. CAVHD offers technical advantages over alternative therapy while providing equivalent or better biochemical control of azotaemia and volume status in critically ill patients with acute renal failure.
Burning oral and mid‐facial pain in ventral pontine infarction
Authors: Reutens D. C. 
Published: March 2008

AbstractThe symptom of burning orofacial pain may help to identify the site of ischaemia in otherwise pure motor strokes resulting from infarction of the ventral pons. A patient with hemiplegia due to ventral pontine infarction, in whom burning oral and mid‐facial pain was a prominent initial symptom, is described. Similar pain preceded transient episodes of the ‘locked‐in’ state. Awareness of this herald symptom may permit early recognition and careful monitoring of patients at risk of progressing to the ‘locked‐in’ state.
Effect of eicosapentaenoic acid on restenosis rate, clinical course and blood lipids in patients after percutaneous transluminal coronary angioplasty
Authors: Nye E. R. , Ablett M. B. , Robertson M. C. , Ilsley C. D. J. , Sutherland W. H. F. 
Published: March 2008

Abstract In order to study the effects of eicosapentaenoic acid (Maxepa®), Maxepa® placebo and aspirin/dipyridamole combination on the clinical course and restenosis rate of atherosclerotic lesions after percutaneous transluminal coronary angioplasty 79 men and 29 women were randomly divided into three treatment groups and restudied angiographically within one year of the procedure.Angina recurred less in the Maxepa® group than in the other groups, although not statistically so. Restenosis rate was significantly reduced in the Maxepa® group (11%) compared to the placebo group (30%) but, while less, was not significantly lower than in the aspirin/dipyridamole group (17%).Maxepa® treatment appears to reduce restenosis rate of coronary artery lesions after percutaneous transluminal coronary angioplasty and may be an acceptable and equally effective alternative therapy to aspirin/dipyridamole.
Length of hospital stay after acute myocardial infarction
Authors: Heller R. F. , Dobson A. J. , Steele P. L. , Alexander H. M. , Al‐Roomi K. , Malcolm J. A. , Gibberd R. W. 
Published: March 2008

Abstract A number of trials show that long stay in hospital after an acute myocardial infarction (AMI) is not necessary for many patients and that stays of three‐ten days may be adequate. All patients aged under 70 years with a diagnosis of AMI admitted to the seven public hospitals in the Lower Hunter Region of New South Wales are monitored as part of the WHO MONICA Study. Between August 1984 and December 1985 of 438 hospitalised patients with a ‘definite’ AMI according to MONICA criteria and a clinical discharge diagnosis of AMI, 386 (88%) patients were discharged alive from hospital. Four patients had lengths of stay between 46 and 77 days and have been omitted from further analysis. The mean length of hospital stay was 13.6 days (95% confidence intervals 12.9 to 14.3 days); 74% of all patients stayed in hospital for more than ten days. The mean length of stay in the Coronary Care Unit (CCU) was 4.5 days (95% confidence intervals 4.2 to 4.8 days) with 60% staying longer than three days. Mean hospital stay varied from 10.5 to 17.4 days among the seven hospitals, although most of this variation was accounted for by three hospitals with few patients. Restricting analysis to the four hospitals with 90% of all the patients, multiple regression analysis showed that the CK enzyme levels, the evolution of Q waves on ECG, the presence of an anterior AMI and the use of nitrates and digoxin during hospitalisation were all associated with increased length of stay in hospital. Nevertheless the proportion of the variance in length of stay explained by all the statistically significant variables together was only 17%.We conclude that, at the time of this study, length of stay in hospital following AMI was longer than necessary. Patient characteristics and severity of infarction explain little of the variability. This suggests that length of hospital stay could be reduced without adverse effects for the patients.
Permanent junctional reciprocating tachycardia misdiagnosed as ‘cardiomyopathy’
Authors: McGuire M. A. , Knight P. , Lau Kai‐chiu , Uther J. B. , Ross D. L. , Davis L. M. 
Published: March 2008

AbstractIn a nine‐year‐old female, cardiac failure was erroneously attributed to idiopathic cardiomyopathy and cardiac transplantation was planned. The actual cause of cardiac failure was permanent junctional reciprocating tachycardia (PJRT), a rare form of supraventricular tachycardia. The diagnostic error was discovered before transplantation was performed and the arrhythmia was treated surgically. This resulted in return of near normal cardiac function. (Aust NZ J Med 1991; 21: 239–241.)
Acute pancreatitis and rhabdomyolysis: a new association
Authors: Nankivell B. J. , Gillies A. H. B. 
Published: March 2008

Abstract Fourteen cases of acute severe pancreatitis complicated by non‐traumatic rhabdomyolysis are described and compared to case controls. Pancreatitis of various aetiologies was confirmed by surgical diagnosis, laparotomy, abdominal paracentesis, CAT scan and post mortem. Pancreatitis was severe with a high Ranson prognostic score (7.4 ± 0.5 vs controls 1.9 ± 0.4, p< 0.001), longer ICU admission and a mortality of 79%. Rhabdomyolysis occurred two to 19 days after the onset of pancreatitis (with a median CPK peak at 6.5 days) and was accompanied by multiple organ failure in 93% of cases. Severe rhabdomyolysis and myoglobinuric renal failure occurred in three patients out of 12 with acute renal failure. Hypocalcaemia was common (93%), severe (with a mean minimum value of 1.79 ± 0.07 vs 2.34 ± 0.04mmol/L, p<0.01) and prolonged (remaining abnormal for 5.2 ± 0.8 vs 0.07 ± 0.07 days, p < 0.001). Intravenous calcium supplements were required in 50% of patients. Plasma phosphate, potassium, urate and anion gap were elevated (all p<0.05) and accompanying clinical features included fever, ascites, leuco‐cytosis, hypoalbuminaemia and abnormal liver function tests.Rhabdomyolysis is associated with acute severe pancreatitis, appearing as a late phenomenon in the context of severe prolonged hypocalcaemia, multiple organ failure and a poor outcome. (Aust NZ J Med 1991; 21: 414–417.)
GM‐CSF after allogeneic bone marrow transplantation: accelerated recovery of neutrophils, monocytes and lymphocytes*
Authors: Atkinson K. , Bradstock K. , Biggs J. C. , Lowenthal R.M. , Downs K. , Dale B. , Juttner C. , Szer J. 
Published: March 2008

Abstract Ten patients given HLA‐identical sibling marrow transplants for lymphoid malignancy received recombinant human granulocyte macrophage‐colony stimulating factor (GM‐CSF) from day 7 to day 13 inclusive post transplant. Patients were prepared for transplantation with busulphan 16 mg/kg and cyclophosphamide 120 mg/kg.Immunosuppression to minimise the risk of graft‐versus‐host disease (GVHD) was cyclosporin/short methotrexate. Results were compared with a historical control group of patients (n = 16) given matched sibling transplants for acute leukaemia and receiving the same immune suppressive regime but not given GM‐CSF. Recovery of total white cells, neutrophils, monocytes and lymphocytes was more rapid in the GM‐CSF recipients (p< 0.02). There was a suggestion of a decrease in non‐viral infections in the first 30 days in the GM‐CSF recipients (p = 0.09). There was, however, no significant difference in the severity of oropharyngeal mucositis nor in the duration of the transplant hospitalisation. Surprisingly, the severity of acute GVHD was higher in the GM‐CSF recipients with six of eight evaluable patients having grade II‐IV acute GVHD (p = 0.003). Two GM‐CSF recipients developed a fluid retention/capillary leak syndrome. These findings indicate a need for caution in the use of GM‐CSF after allogeneic marrow transplantation.
A comparison of standard Madopar and controlled release Madopar in Parkinson's disease
Authors: Graham J. S. , Henderson J. M. , Morris J. G. L. , Yiannikas C. 
Published: March 2008

Abstract:In a randomised, double‐blind cross‐over study, objective measures were used to compare the effect of a single oral dose of a standard preparation of levodopa‐benserazide (Madopar M) with a sustained‐release preparation (Madopar HBS) in 9 Parkinsonian patients with ‘end of dose deterioration’. The response of patients to an optimised regimen of each preparation was also assessed using a patient diary.In all patients the onset of effect of Madopar HBS following a single dose was delayed compared with Madopar M. The duration of effect of a single dose of Madopar HBS was substantially (38‐120%) longer than Madopar M in five patients and the same or shorter in four patients. According to the patients diaries, six patients noted an increase in ‘on’ hours while on Madopar HBS. The duration and severity of dyskinesia was similar for the two preparations.Madopar HBS is likely to be useful in some Parkinsonian patients with ‘end of dose deterioration’. As the effect of each dose is delayed it is probably best given in combination with standard Madopar.
Ventricular dysfunction following direct‐current shock atrioventricular junction ablation
Authors: Warren R. J. , Vohra J. K. , Chan W. , Lichtenstein M. , Mond H. G. , Hunt D. 
Published: March 2008

Abstract:Catheter‐induced His bundle ablation for refractory supraventricular arrhythmias is most commonly performed with direct‐current shock energy of 200‐300 joules. The high energy pulse delivered by direct‐current shock produces a lesion in the atrioventricular node by fulguration, with the residual energy being dissipated as a pressure wave.The effect of direct‐current shock His bundle ablation on global and regional ventricular function was assessed in 14 consecutive patients by radionuclide ventriculography performed before and after ablation and again three months later. All studies were performed with ventricular pacing at 110 bpm.Global left ventricular ejection fraction was found to be significantly reduced at the three month study (0.43 ± 0.03 vs 0.50 ± 0.03, pre ablation, p= 0.02). A significant reduction in wall‐motion score was also seen in six of the seven patients who had normal wall motion in pacing rhythm prior to ablation. Deterioration was mainly seen at the left and right ventricular apices.The observed reduction in ventricular function that follows direct‐current shock His bundle ablation may result from myocardial damage from electro‐coagulation or from barotrauma and supports continued investigation into alternative, less traumatic energy sources for the procedure.
Diarrhoea—an unrecognised hazard of coeliac plexus block
Authors: Dean A. P. , Reed W. D. 
Published: March 2008

Abstract:Coeliac plexus block is a safe procedure with relatively few side‐effects. We report two cases of persistent severe diarrhoea following coeliac plexus block and explore the possible reasons for this previously unrecognised side‐effect. We postulate that somatostatin analogue may be useful as treatment for diarrhoea following neurolytic coeliac plexus block that is unresponsive to conventional anti‐diarrhoeal agents.
The musculo‐skeletal examination
Authors: Ahern M. J. , Soden M. , Schultz D. , Clark M. 
Published: March 2008

Abstract:One hundred and sixty‐six patients admitted to general medical wards of a teaching hospital were examined on the day of discharge to determine whether they had been assessed for musculoskeletal disorders during their admission.Of these patients, 54.8% had musculo‐skeletal symptoms with 17.5% having a significant rheumatological disorder which had been ignored. A history of musculo‐skeletal symptoms was recorded in 40.4% of all patients and the examination in only 14.5%. This contrasted with the documentation of the cardiovascular (99.4%), respiratory (100%), gastrointestinal (97.6%) and central nervous (53%) systems' examination. Eighty per cent of symptomatic patients received either no treatment for their rheumatic disorder, or treatment that we regarded as suboptimal or inappropriate.Musculo‐skeletal symptoms are common in patients admitted to medical wards, but are being inadequately assessed or at worst ignored. The omission of the musculo‐skeletal system examination, in contrast to the almost universal inclusion of other systems' examination, demands correction. Undergraduate and postgraduate training programmes require re‐evaluation. The implications of these findings are discussed.
T cell receptor and immunoglobulin gene rearrangement analysis as a laboratory aid in the diagnosis of human malignant lymphoproliferative diseases
Authors: Dietzsch E. , Hong J. , Leslie D. E. , Martin L. , Rolland J. , Benson E. , Toh B. Hock , McCluskey J. 
Published: March 2008

Abstract:The identification of clonal rearrangements of the immunoglobulin heavy chain and T cell receptor beta chain gene loci by Southern blot analysis has led to advances in the diagnosis and classification of lymphoproliferative disorders. This paper reviews our experience with this technique over a three and a half year period. Specimens from 99 patients with suspected haemato‐logical malignancy were tested for leucocyte immunophenotype and immunoglobulin or T cell receptor gene rearrangement. Genotyping provided evidence of clonality in malignancies from 28 patients and demonstrated malignant cell lineage in eight patients not formally deduced from immunophenotyping alone. Our findings suggest that this technique can be employed in conjunction with immunophenotyping to aid in the determination of malignant cell lineage derivation and identification of malignant cell clonality, as well as potentially estimating the extent of disease, detecting relapse, and monitoring disease progression.
Spontaneous haemarthrosis in amyloid arthropathy of the shoulder
Authors: Vecchio P. , Hazelton R. A. 
Published: March 2008

Abstract:A patient is reported who has had episodes of spontaneous haemarthroses of the shoulder. On aspiration of the acute haemarthrosis, amyloid was found after appropriate staining of the centrifuged sediment. This may be a predisposing factor for haemarthrosis in haemodialysed patients.
Chorioretinitis as a possible very early manifestation of subacute sclerosing panencephalitis
Authors: Zagami A. S. , Lethlean A. K. 
Published: March 2008

Abstract:Ocular changes occur in up to 50% of cases of subacute sclerosing panencephalitis (SSPE) usually at the same time as other neurological signs. Occasionally, visual symptoms precede other manifestations by a few weeks or months. Fundal changes (most commonly a macular chorioretinitis) are the most frequent ocular manifestation of SSPE. Inclusion bodies occur in the retina of patients with chorioretinitis and SSPE and it is believed that the lesions are due to actual viral invasion. We report a case of SSPE in which an atypical chorioretinitis preceded the onset of the typical clinical features of the disease by almost two years. Our case is unusual because of the long interval between the visual symptoms and the onset of other features of SSPE. We wish to emphasise the importance of thinking of SSPE in cases of chorioretinitis which are atypical or occur in young patients. Although treatment of SSPE thus far has been mostly disappointing, earlier diagnosis may offer the hope of better results.
Transoesophageal echocardiography in heart disease—old technologies, new tricks
Authors: Lee Lincoln C. L. , Black Ian W. , Hopkins Andrew , Walsh Warren F. 
Published: March 2008

AbstractCardiac ultrasound and upper gastrointestinal endoscopy are relatively old technologies. With the introduction of new ultrasound probes and by incorporating ultrasound technology into conventional endoscopes, ‘new tricks’ in cardiac imaging were discovered. Posterior structures of the heart are now able to be imaged clearly by the ultrasound probe from the oesophagus. Consequently, better resolution of cardiac anatomy allows more accurate diagnosis of cardiac pathologies which is not possible using conventional transthoracic (TT) approach.Over a period of two years, 1200 cases of transoesophageal echocardiography (TOE) were undertaken in our institution. The major indications were diseases of the aorta (10%), source of cardioembolism (28%), assessment of native and prosthetic valve function (20%), suspected endocarditis and its complication (17%), pre and post percutaneous transluminal mitral valvotomy (PTMV [13%], congenital heart disease (2%) and others (10%).The greatest impact with TOE is in the diagnosis of aortic dissection and transection, TOE is superior to conventional TT approach in detecting potential source of embolism, valvular vegetations and its complication, native and prosthetic valve dysfunction and LA thrombus prior to PTMV.Observations by TOE such as spontaneous echo contrast (SEC) in the left atrium open new challenges for further research in its role in the pathogenesis of LA thrombus and its association with cardioembolic event. Other areas of interest include; reclassification of distal aortic dissection and the use of TOE in intra‐operative work.
Mitral valve billowing and prolapse—an overview
Authors: Barlow John B. 
Published: March 2008

AbstractThree decades after it was demonstrated that nonejection systolic clicks and late systolic murmurs have a mitral valve origin and that a specific syndrome is associated with the primary degenerative mitral lesion, numerous questions remain unanswered. A principal cause of confusion is the use of the term ‘prolapse’, which essentially implies a pathological state, in many patients with minimal evidence of a mitral valve anomaly. It should be recognised that no specific feature, whether evaluated by high standard echocardiography or indeed by careful morphological and histological examination, can be defined which distinguishes a normal variant from a pathological valve. There is a grandation from the normal billowing doring ventricular systole of mitral leaflet bodies to marked billowing. With advanced billowing or floppy leaflets, failure of leaflet edge apposition supervenes (true prolapse). This is functionary abnormal and allows mitral regurgitation. Prolapse in turn may progress to a flail twfltT and hence gross regurgitation.Relatively rare complications of this degenerative mitral valve anomaly include systemic emboli, infective endocarditis, arrhythmias and, arguably, autonomic nervous system abnormalities. An attempt is made to ctarify the management of some symptoms and other aspects of mitral prolapse ‐ including rheumatic anterior leaflet prolapse (without billowing) which remains prevalent in South Africa and Third World countries.
Prevention of sudden cardiac death: the ICD, or an electrical end‐point with preceding opportunities for intervention?
Authors: Tonkin Andrew M. 
Published: March 2008

AbstractSudden cardiac death (SCD) is usually due to monomorphic ventricular tachycardia and/or ventricular fibrillation. However, in the vast majority of patients these arrhythmias are associated with advanced structural disease. In our society, this is usually due to coronary artery disease (CAD).The implantable cardioverter – defibrillator is the logical approach to management in survivors of SCD. Its rational use must be guided by electrophysiology study. However, a realistic and cost‐effective approach to the prevention of a first cardiac arrest must be multifaceted and take cognisance of other aspects including primary prevention. Limitation of the size of myocardial infarction (MI) is vital. Trials already suggests that effective thrombolysis may impinge long‐term on arrhythmic end‐points. Following infarction, ventricular arrhythmias and sudden death may also be decreased by aspirin, beta‐blockers, and possibly angiotensin converting enzyme inhibitors and amiodarone.Many post‐infarction studies employ a combined end‐point of death and clinical arrhythmias. However, death is usually confined to those with an ejection fraction <35%. In them, treatment of associated heart failure is often a consideration and if the ejection fraction < 15–20%, depending on donor availability, transplantation may even be the preferred therapeutic option to the cardioverter‐defibrillator.
Reduced synthesis of tissue plasminogen activator by vascular endothelium during acute myocardial infarction
Authors: Norris R. M. , Ockelford P. A. , Cross D. B. , Rivers J. T. , Smith J. M. , Takayama M. , White H. D. 
Published: March 2008

Abstract:We management levels of tissue plasminogen activator (t‐PA) antigen in 100 patients within six hours of the onset of acute myocardial infarction, in 34 patients with chronic angina but no recent infarction, and in 36 normal subjects. We also assayed von Willebrand factor in the acute patients and in the normal subjects. Measurements were repeated in 40 acute patients at three weeks after myocardial infarction. Although resting levels of t‐PA antigen were not significantly different from normal during myocardial infarction, the capacity of the vascular endothelium to release t‐PA after five minutes of venous occlusion was impaired (P<0.01). The acute phase vessel wall release of von Willebrand factor was increased during acute iafarction (p<0.01). We conclude that impairment of t‐PA production is associated with acute coronary thrombosis although it is not possible to differentiate between a causative role or a secondary response due to exhaustion of the t‐PA producing mechanism.