bluuebunny/crossref_metadata_2025 huggingface dataset:
Lipoprotein(a) levels in chronic renal disease states, dialysis and transplantation
Authors: Irish A. B. , Simons L. A. , Savdie E. , Hayes J. M. , Simons J.
Published: March 2008

Lipoprotein(a) is an independent risk factor for cardiovascular disease. Lipoprotein(a) levels were measured in 196 patients (103 Male [M]: 93 Female [F]) with chronic renal diseases and in 116 controls. Median levels of Lipoprotein(a) [Lp(a)] were found to be significantly elevated in patients with untreated chronic renal disease (285, 285 mg/L; M, F; range 30–1675 mg/L) and in those treated with continuous ambulatory peritoneal dialysis (320, 603; M, F; range 50–1450) compared with controls (70, 51; M, F; range 1–750; p<0.01 Males, p<0.001 Females). Lp(a) levels in patients treated by haemodialysis (133, 35; M, F; range 5–685) and renal transplantation (100, 95; MJF; range 10–1700) were not significantly different from controls. Lipoprotein(a) levels correlated inversely with serum albumin in the combined dialysis group (r= ‐034, p < 0.001), and with urinary protein loss in the combined transplant and chronic renal diseases groups (r = 0.29, p <0.01). This correlation of Lp(a) with protein metabolism suggests a similarity with changes in other apolipoprotein‐B containing lipoproteins in nephrosis. These findings may be relevant to the increased risk of atherosclerosis in patients with chronic renal disease and to their optimum mode of renal replacement therapy. (Aust NZ J Med 1992; 22:243–248.)
Co‐existence of coeliac disease and insulin‐dependent diabetes mellitus in children: screening sera using an ELISA test for gliadin antibody
Authors: Gadd S. , Kamath K. Ramanand , Silink M. , Skerritt J. H.
Published: March 2008

The prevalence of coeliac disease in children with insulin‐dependent diabetes mellitus was investigated using a screening test of serum for antigliadin antibody by ELISA. One hundred and eighty (180) unselected diabetic children were screened for IgA and IgG class antigliadin antibodies (AGA); children with either grossly elevated or slightly elevated AGA had small bowel biopsies. The four children with the highest IgA AGA had total villous atrophy. These four children were considered to have unsuspected coeliac disease. The prevalence of coeliac disease in this group of children was one in 45. Anri‐gliadin IgA and IgG tests are suitable for screening children at high risk of having coeliac disease.
Fungal Pseudoallescheria boydii lung infiltrates unresponsive to amphotericin B in leukaemic patients
Authors: Walsh M. , White L. , Atkinson K. , Euno A.
Published: March 2008

The management of patients with acute leukaemia is often complicated by serious fungal infections, especially of the lungs. The outcome of therapy has historically depended on the early use, efficacy and tozicity of amphotericin B. Pseudoalksdttria boydii is an uncommon cause of such infections but as it is more often resistant to amphotericin B. early identification may enable the prompt use of alternative and newer antifungal agents. Here we report our experience and review the literature in three cases of P. boydii infection in patients with kukeamia, showing unique features such as childhood and central nervous system disease, positive blood cultures and response to itraconazole.
Recently characterised autoantibodies and their clinical significance
Authors: Sturgess A.
Published: March 2008

Multisystem autoimmune diseases such as systemic lupus uythcmatosus (SLE), primary Sjögrcn's syndrome (SS), sclcroderma and polymositis are characterised by the presence of antinuclear antibodies (ANAs). Immunoblotting and cDNA cloning studies reveal that the autoantigens of the multisystem autoimmune dixsucs are important proteins involved in nucleic acid metabolism, including tRNA charging, intron splicing, DNA uncoiling, and RNA polymuase co‐factors.

Each spaific syndrome associates with a restricted variety of ANAs e.g. anti‐La with primary SS, anti‐Sm with SLE, and‐synthetase enzymes with myositis, and‐topoisomerase 1 (Scl 70) with scleroderma, and anti‐centromere with CREST. Precise characterisation of an ANA provides valuable diagnostic and prognostic information, and should be performed when an ANA is detected.
Lipoprotein(a) concentration in diabetes: relationship to proteinuria and diabetes control
Authors: Irish A. B. , Simons L. A. , Simons J.
Published: March 2008

Diabetic patients are at increased risk of cardiovascular disease, particularly when proteinuria is present. Lipoprotein(a)[Lp(a)] levels were assessed in 37 patients with insulin dependent (IDDM) and in 75 patients with non‐insulin dependent (NIDDM) diabetes who showed varying degrees of proteinuria and glycaemic control. Median Lp(a) in 112 diabetic patients was significantly greater than in 116 healthy controls (113 vs 48 mg/L; p <0.01). 86 of the patients had first morning urine albumin concentration < 30 mg/L (normoalbuminuria = NA), 16 patients 30–200 mg/L (microalbuminuria = MA) and ten patients < 200 mg/L (albuminuria = ALB). There was no significant difference in median Lp(a) concentration between the three groups (NA = 108, MA = 163, ALB = 98 mg/L; p > 0.5). No significant difference in median Lp(a) concentration was found between patients with IDDM, NIDDM treated with insulin, or NIDDM treated with oral agents and/or diet (120, 98, 115 mg/L respectively; p > 0.7). When the 86 NA patients were divided on the basis of median fructosamine concentration (357 umol/L), no significant difference was found in median Lp(a) levels between those grouped below or above this median (98 mg/L vs 118 mg/L; p < 0.5). Across all diabetics studied there was no significant correlation present between Lp(a) and urinary protein or glycaemic control. These cross‐sectional results suggest that median Lp(a) concentration is increased in both IDDM and NIDDM patients, but this increase is not related to the degree of proteinuria or short‐term glycaemic control.
The value of complement measurements in the assessment of lupus activity
Authors: Milis L. , Timmermans V. , Morris C. A. , Pussell B. A. , Charlesworth J. A.
Published: March 2008

The complement system was studied prospectively in 29 patients, predominantly renal (25), with systemic lupus erythematosus (SLE) to examine the value of complement assays in the distinction between active and inactive disease. Disease activity was evaluated primarily by clinical, biochemical and histological parameters which were obtained at the time of assessment. Fourteen patients had active disease, as assessed by clinical and laboratory criteria. Clq, C4, C4a, C2, C3, C3a, C5, total haemolytic activity (CH50) and complement inhibitors were measured in each patient. The ratios of C4a:C4 and C3a:C3 were also calculated.

Values for all components except C5 were different between control subjects and active patients while only CH50 was different between inactive patients and controls. All parameters except C4a:C4 and C5 were different between active and inactive patients. There was a highly significant difference in the number of active patients with reduced levels of C2, C3 and C3a:C3 compared to inactive patients (i.e. p < 0.001) whereas lesser or no difference was observed for other parameters. The concentration of complement inhibitors was elevated in both groups.

We conclude that, among readily available complement parameters, C2 and C3 provide the best assessment of disease activity in patients with SLE.
Symptomatic choroidal metastases in breast cancer
Authors: Doig R. G. , Olver I. N. , Jeal P. N. , Bishop J. F.
Published: March 2008

A series of 21 consecutive patients with carcinoma of the breast and symptomatic choroidal metastases was reviewed. All patients were symptomatic of choroidal metastases at presentation. Most patients had disease progression at other sites at the time of diagnosis. The incidence of cerebral metastases was not increased in this group compared to that in patients with metastatic breast cancer at other sites. Radiotherapy using a direct anterior field proved effective palliation for this disease site. Of the 32 eyes treated, serial visual acuity was documented in 23. Seventeen showed objective improvement and six showed stabilisation. The median duration of improvement was 11.5 months.
Prevalence of antibodies to hepatitis C virus in patients receiving renal replacement therapy, and in the staff caring for them
Authors: Blackmore T.K. , Stace N.H. , Maddocks P. , Hatfield P.
Published: March 2008

Two hundred and forty‐three patients receiving renal replacement therapy (RRT) and 20 renal unit staff were tested for antibodies to hepatitis C (HCV). Three patients (1.2%) were positive by the first generation test kit, the lowest rate in patients receiving RRT reported in the literature to date. These three, and eight other patients tested positive by the second generation kit, a prevalence rate of 4.5%. Anti‐HCV antibody positivity was associated with higher mean serum alanine aminotransferase (p= 0.0003) and aspartate aminotransferase (p= 0.018) levels. However, only one of the 11 anti‐HCV positive patients had liver transaminase levels more than twice the upper limit of the laboratory reference range. Anti‐HCV positivity was associated with a higher mean number of units of blood transfused (p= 0.035). None of 20 staff were anti‐HCV positive. Twenty‐five of 212 (11.7%) patients reported a history of liver disease; none of these were anti‐HCV positive. Hepatitis B surface antigen was detected in eight of 215 (3.7%) patients, of which three were e antigen positive. There was evidence of past hepatitis B infection in 53 of 215 (24.7%) patients, more frequently in Maoris (p= 0.001). Overall, significantly raised liver transaminases were present in three of 198 (1.5%) patients and in no staff. This unit has a remark‐ably low prevalence of antibodies to HCV, an observation supported by the low rate of abnormal serum liver enzymes.
Heparin‐induced thrombocytopenia
Authors: Chong B. H.
Published: March 2008

Thrombocytopenia is a common adverse effect of heparin therapy. Two types of heparinlinduced thrombocytopenia (HIT) are observed clinically ‐ an early onset mild thrombocytopenia (Type I) in which the patients remain asymptomatic and a delayed onset severe thrombocytopenia (Type II). Patients with Type II HIT have an increased risk of thrombotic complications which frequently cause crippling disability e.g. limb amputation or even death. Type I HIT, the commoner of the two types, is believed to be due to the platelet proaggregating effect of heparin itself but Type II HIT is generally agreed to be caused by an immune mechanism, in which heparin‐antibody complexes bind to platelets resulting in platelet activation, reduced platelet survival, thrombocytopenia and, in some cases, thrombosis. The diagnosis of HIT is made mainly on a clinical basis but in patients with suspected Type II HIT, laboratory test for the heparin‐dependent antibody using platelet aggregometry or the two‐point14C‐serotonin release method, allows confirmation of the diagnosis. In most Type I and all Type II patients, heparin should be stopped and warfarin commenced if there is a recent or new thrombosis requiring continuing anticoagulation. An alternative antithrombotic drug such as low molecular weight heparinoid (Org 10172) or dextran should be given at the same time until warfarin becomes therapeutic. The use of low molecular weight heparins (e.g. Fragmin) should be avoided unless it can be demonstrated that the HIT antibody does not cross‐react with these drugs. (Aust NZ J Med 1992; 22: 145–152.)
Immediate and long‐term results of percutaneous balloon aortic valvuloplasty: a report of 33 procedures
Authors: Sathe S. , Warren R. , Wong J. , Hunt D.
Published: March 2008

The purpose of this study was to evaluate the immediate and long‐term clinical and haemodynamic effects of Percutaneous Balloon Aortic Valvuloplasty (PBAV). Thirty‐three procedures were performed in 27 patients, 11 males and 16 females with a mean age of 72 years. The peak to peak systolic gradient across the aortic valve decreased by 52%, from 67 ± 24 mmHg to 31 ± 15 mmHg (p< 0.0001) and the aortic valve area increased by 35%, from 0.48 ±0.16 to 0.67 ± 0.21 cm2 (p <0.001) after PBAV. There were no procedural deaths. Minor complications occurred in seven patients. Eighty‐one per cent of patients (27 procedures) showed immediate symptomatic improvement, five remained symptomatic and one died in hospital before discharge. In a mean follow‐up of 17 ± 12 months (range six to 38 months), eight patients died due to congestive cardiac failure, six underwent repeat PBAV, three died due to noncardiac causes and three had recurrent symptoms controlled on medical therapy. Event free survival at 15 months was 43%.

In conclusion PBAV produces a satisfactory immediate clinical and haemodynamic result, however, considering the high rate of symptom recurrence, this procedure has a beneficial role only in short‐term palliation of severely symptomatic patients who are unable to undergo aortic valve replacement. (Aust NZ J Med 1992; 22: 647ndash;651.)
Autoantibodies to neutrophil cytoplasmic (ANCA) and endothelial cell surface antigens (AECA) in chronic inflammatory bowel disease
Authors: Romas E. , D'Apice A. J. F. , Paspaliaris B. , Elliott P. R.
Published: March 2008

Sera from 103 patients with chronic inflammatory bowel disease (IBD) were tested prospectively for antibodies against neutrophil cytoplasmic antigens (anti‐neutrophil cytoplasm antibodies, ANCA) and endothelial cell surface antigens (anti‐endothelial cell antibodies, AECA) by indirect immunofluorescence (IIF) and assays based on whole fixed neutrophils, purified neutrophil enzyme substrates and human umbilical vein endothelial cells.

Using IIF, ANCA were found in 26 IBD sera (25%) and in none of 51 controls. Twenty‐two positive sera (85%) were from patients with ulcerative colitis (UC). The pattern of distribution of immunofluorescence was always perinuclear (P‐ANCA). A majority of UC patients positive for these autoantibodies (68%) had active colitis, but none had evidence of vasculitis. Using a whole neutrophil ELISA, binding was demonstrable in 73% of UC sera compared to 27% of Crohn's (CD) sera and only 4% of controls. Unlike vasculitis sera, UC sera with P‐ANCA did not bind strongly to myeloperoxidase (MPO). Forty‐five per cent of IBD sera tested positive for IgG AECA in an endothelial cell ELISA, compared to seven of 51 (14%) controls. Binding correlated with both active and extensive colitis.

A type of P‐ANCA, in most cases distinct from MPO‐specific P‐ANCA observed in vasculitis, is detected in a significant proportion of patients with UC, but rarely Crohn's colitis and therefore may be of differential diagnostic value. IgG AECA are also frequent in CIBD sera but are less disease specific than ANCA. (Aust NZ J Med 1992; 22: 652ndash;659.)
A comparison of peripheral blood stem cell mobilisation after chemotherapy with cyclophosphamide as a single agent in doses of 4 g/m2 or 7 g/m2 in patients with advanced cancer
Authors: Rowlings P. A. , Bayly J. L. , Rawling C. M. , Juttner C. A. , To L. B.
Published: March 2008

We used cyclophosphamide at a dose of 7 g/m2 in patients with advanced cancer and compared the efficacy of this treatment to generate peripheral blood stem cells (PBSC) with the previously reported regimen of cyclophosphamide 4 g/m2 in a similar group of patients. None of these patients received haemopoietic growth factors. Twenty‐two patients received 7 g/m2 and 37 received 4 g/m2. PBSC were collected by apheresis after the leukocyte count recovered to 1.0times109/L.

The yield of colony forming unit‐granulocyte macrophage (CFU‐GM) was higher for the 7 g/m2 group with a median of 35 times 104/kg versus 15 times 104/kg body weight (BW) (p<l0.05) and higher mononuclear cell yield with medians of 4.2 times 108/kg compared with 3.1 times 10s/kg BW (p <0.001). The percentage of patients achieving the minimum safe level of > 15 times 104 CFU‐GM/kg BW was higher in the 7 g/m2 cyclophosphamide group (82%) than the 4 g/m2 cyclophosphamide group (51%). The duration of significant neutropaenia was a median of 11 compared with nine days (p <0.004) and all patients receiving 7 g/m2 required admission to hospital and intravenous antibiotic therapy compared with 44% in the 4 g/m2 group. There was one death during the period of neutropaenia after cyclophosphamide in each group. Nineteen per cent of patients required platelet transfusions after cyclophosphamide 7 g/m2 compared with 18% after 4 g/m2.

We conclude that the 7 g/m2 cyclophosphamide gives a higher yield of haemopoietic progenitor cells than the 4 g/m2 but at increased clinical toxicity. (Aust NZ J Med 1992; 22: 660ndash;664.)
The structured interview as a tool for predicting premature withdrawal from medical school
Authors: Powis D. A. , Bristow T. , Waring T. C. , O'Connell D. L.
Published: March 2008

A 1:1 matched case‐control study was carried out to assess whether comments written about a candidate during a semi‐structured interview can identify students likely to withdraw from a medical course better than global numerical scores. Fifty‐nine students who withdrew from the undergraduate medical course at the University of Newcastle prior to completion were matched for sex, year and mode of entry into the course, prior academic qualifications and age at entry, with 59 students who had not discontinued or been excluded from the course.

No significant differences were found between the numerical scores allocated for personal attributes to students who later withdrew from the course and those who continued. However, students who withdrew had statistically significantly more negative comments made about them at interview with respect to their supportive and encouraging behaviour (p= 0.04) and their motivation to become doctors (p= 0.05). Although no other comparisons reached statistical significance, students who withdrew consistently received more negative comments and fewer positive comments than their continuing peers. A stepwise logistic regression procedure identified the number of negative comments made by interviewers about a candidate on their supportive and encouraging behaviour as the only statistically significant predictor of later withdrawal (odds ratio 1.65 95% confidence interval 1.01–2.70). (Aust NZ J Med 1992; 22: 692ndash;698.)
The Australian and New Zealand Cardiothoracic Organ Transplant Registry: first report 1984ndash;1992
Authors: Keogh A. M. , Kaan A.
Published: March 2008

This initial report of the Australian and New Zealand Cardiothoracic Organ Transplant Registry summarises the results of all cardiothoracic transplants performed between February 1984 and April 1992. A total of 549 first cardiothoracic transplant procedures and six cardiac retransplant operations were performed in five transplant units throughout Australia and New Zealand. There were 466 orthotopic cardiac transplants and one heterotopic transplant with overall survival 86% at one year and 80% at five years. Two of six patients who underwent cardiac retransplan‐tation are alive. Fifty‐three heart‐lung transplants were performed with 72% one year and 42% five year survival. Twenty‐nine single lung transplant procedures were undertaken, with actuarial survival 72% at 12 months. Factors influencing waiting period and post‐transplant survival for each type of procedure are detailed. The relative lack of donors compared with recipient demand has produced increased waiting times for every type of cardiothoracic organ transplant. (Aust NZ J Med 1992; 22: 712ndash;717.)
Yttrium synovectomy: a meta‐analysis of the literature
Authors: Jones G.
Published: March 2008

Background:
Yttrium synovectomy for chronic synovitis of the knee enjoys widespread usage in Australia with approximately 400 patients receiving yttrium‐90 in 1991. Despite abundant anecdotal evidence of its efficacy there is a paucity of controlled trials and those that have been done have produced conflicting results and have been of insufficient sample size.

Aims:
To critically and quantitatively evaluate the published English literature on comparative trials of yttrium‐90 therapy for chronic synovitis of the knee.

Methods:
The technique of meta‐analysis was utilised. The literature search was carried out using the MeSH terms of synovectomy and knee; and yttrium. This was augmented by referring to reviews, current textbooks and back‐references. Outcome measures varied between trials but could be grouped as treatment success. The Peto modification of Mantel and Haenszl was used for statistical pooling of data yielding a pooled odds ratio (OR).

Results:
The literature search revealed ten controlled trials of which two were excluded from further analysis. Yttrium was superior to placebo (OR 2.42, 95% CI 1.02–5.73) but this result should be interpreted with caution due to possible publication bias. Yttrium was not superior to triamcinolone (OR 1.89, 95% CI 0.81‐10.55) or other active modalities (OR 1.04, 95% CI 0.72‐1.52). Further research comparing yttrium with other modalities is necessary to properly determine its place in rheumatological practice. (Aust NZ J Med 1993; 23: 272–275.)
Clinical analysis of 125 patients with the lupus anticoagulant
Authors: Morgan M. , Chesterman C. N. , Downs K. , Biggs J. C.
Published: March 2008

:A retrospective analysis of 125 consecutive lupus anticoagulant (LA) positive patients and 125 age, sex matched lupus anticoagulant negative controls is reported with the aims of defining further the clinical spectrum of disease, determining at‐risk subgroups and management strategies. There was no significant difference in the incidence or pattern of complications in those with systemic lupus erythematosus (SLE) and related disorders, and those without SLE. Venous thromboembolism, immune thrombocytopenia, foetal loss, depression and hypertension were statistically more common in the LA group than in the control group. In contrast to previous reports, children aged ten years or less with the LA developed significantly more complications than controls. Patients with the LA secondary to drugs also developed complications, a finding which is also at variance with previous reports. There was a significant difference in the outcome of arterial disease (p < 0.04) and venous thromboembolism (p< 0.001) when long term anticoagulation was part of the treatment regimen. (Aust NZ J Med 1993; 23: 151–156.)
Wegener's granulomatosis: clinical features and prognosis in 37 patients
Authors: Romas E. , Kennedy J. T. , Murphy B. F. , Niall J. F. , 'Apice A. J. F.
Published: March 2008

:Thirty‐seven patients (21 female, 16 male) with Wegener's granulomatosis (WG)were reviewed. Patients were followed for a mean six years after diagnosis; 14 were followed for more than seven years. The clinical features were similar to those in previous studies. In this series, only 13 patients (35%) had renal disease at presentation and the cumulative incidence of renal involvement was 51%. Thirty‐one patients received treatment which included cyclophosphamide (CP). The case fatality rate of the six patients not treated with CP was 83% (five deaths). By contrast, all CP treated patients improved and 21 (68%) had complete remissions. Nine (29%) were in complete remission for a mean 4.9 years after discontinuing all treatment. Two were disease free for over ten years. The actuarial probability of survival for these patients was 97% at one year and 71% at ten years. Only three CP treated patients (10%) progressed to end‐stage renal disease. The case fatality rate was 26% (eight patients) and sepsis was the cause of death in five. Fourteen patients (45%) treated with CP had at least one relapse of vasculitis and seven (23%) had multiple (two or more) relapses. These data indicate that CP is effective in inducing remissions and prolonging survival in patients with WG; however, relapses are frequent. (Aust NZ J Med 1993; 23: 168–175.)
Kennedy's disease: genetic diagnosis of an inherited form of motor neuron disease
Authors: Choi W‐T. , MacLean H. E. , Chu S. , Warne G. L. , Zajac J. D.
Published: March 2008

:Kennedy's disease (X‐linked spinal and bulbar muscular atrophy) is an inherited form of motor neuron disease that may be diagnosed genetically using the polymerase chain reaction (PCR). This form of motor neuron disease principally affects the proximal limb girdle muscles as well as those involved with deglutition and phonation. Onset is usually late, in the fourth to fifth decades of life, and progression is slow. Moderate gynecomastia and testicular atrophy are usually present, suggesting a defect in androgen receptor function. Being inherited in an X‐linked recessive manner, only males are affected, with females as the unaffected carriers.

The genetic abnormality that causes Kennedy's disease is an enlargement of the androgen receptor (AR) gene, which is located on the proximal long arm of the X chromosome. In patients with this disease, a region in the gene containing repeated CAG triplet nucleotides is approximately twice the size of that found in normal people. Using PCR to amplify this region of the AR gene, this study confirms this genetic mutation in 12 males from eight different families. All these families live on the east coast of Australia. This mutation was not found in five patients with other forms of motor neuron disease. Twelve heterozygote females, the daughters of affected males and carrier females, have also been identified. In addition, there are 14 asymptomatic and as yet untested sons of carriers, ranging in age from less than one year to over 40 years of age. Each has a 50% chance of inheriting the abnormal gene from his mother and thus developing Kennedy's disease.

This study shows that Kennedy's disease may be diagnosed genetically using whole blood, and discusses the ethics of prenatal and presymptomatic testing, particularly in males under 16. (Aust NZ J Med 1993; 23: 187–192.)
Transoesophageal echocardiography in the investigation of stroke: experience in 135 patients with cerebral ischaemic events
Authors: Jones E. F. , Calafiore P. , Donnan G. A. , Tonkin A. M.
Published: March 2008

Background: The importance of cardiogenic embolism as a cause of cerebral ischaemic events may be underestimated if potential cardiac sources of embolism remain undetected. Transoesophageal echocardiography (TOE) is more sensitive in detecting such abnormalities than earlier methods and may result in more frequent recognition of cardiogenic embolism.

Aims: To establish in an unselected stroke population the prevalence of potential cardiac sources of embolism detectable on TOE and their relationship to atrial fibrillation and carotid stenosis.

Methods: One hundred and thirty‐five consecutive patients with cerebral ischaemic events were studied using transthoracic and monoplane transoesophageal contrast echocardiography and carotid Duplex imaging or cerebral angiography.

Results: Thirty patients (22%) had atrial fibrillation. In 43 patients (32%), no cardiac source of embolism was identified; in eight echocardiography was normal. At least one potential cardiac source of embolism was identified in 92 patients (68%) with multiple findings in 41; these patients were older (70 ± 9 years vs 61 ± 14 years;P= 0.0001), more frequently in atrial fibrillation (28/92 vs 2/43; p= 0.0017) and more frequently had cortical or large subcortical stroke (71/92 vs 23/43; p = 0.005). Carotid stenoses were detected in 55% of patients, not significantly different in those with and without cardiac abnormalities.

Conclusions: When bias in selection of patients is avoided, TOE detects a potential cardiac source of embolism in most patients with cerebral ischaemia, particularly those older and in atrial fibrillation. Multiple cardiac abnormalities and coexistent carotid disease are common. The implication of these findings for prevention of stroke awaits age‐matched controlled studies. (Aust NZ J Med 1993; 23: 477–483.)
Immunoreactive calmodulin in cystic fibrosis kidneys
Authors: Couper R. , Kilbourn J. P. , Bentur L. , Wolf P.
Published: March 2008

Aims: To determine whether CF kidneys obtained at autopsy contain normal amounts of calmodulin and whether the presence of immunoreactive calmodulin correlates with nephrocalcinosis.

Methods: An immunoperoxidase method was used to stain kidneys obtained from six CF individuals aged three to 27.5 years (mean 15.75 ± 10.2 years) and five individuals who died of chronic debilitating disease aged eight to 17 years (mean 13.6 ± 3.9 years). Calcium staining was undertaken using the von Kossa (silver precipitation) and Alizarin red methods.

Results: No quantifiable difference in immunoreactive calmodulin staining was demonstrated in autopsy renal specimens between six CF patients and five chronically diseased controls. Three of six CF patients aged 22.8 ± four years and two of five chronically diseased controls aged eight to 16 years respectively had detectable immunoreactive calmodulin staining. Within the CF group the presence of microscopic nephrocalcinosis did not correlate with immunoreactive calmodulin staining.

Background: Microscopic nephrocalcinosis has been detected at autopsy in 35 of 38 (92%) cystic fibrosis (CF) patients. A primary defect of calcium metabolism in the kidney has been postulated. Intracellular calcium and calmodulin (a calcium binding protein) have been shown to be increased in CF tissues.

Conclusion: This study does not support the premise that a calmodulin‐dependent defect of intracellular calcium handling contributes to the microscopic nephrocalcinosis seen in CF. (Aust NZ J Med 1993; 23: 484–488.)
The GUSTO Trail: background and baseline characteristics
Authors: Aylward Philip
Published: March 2008

The GUSTO study was designed to test the hypothesis that achieving early sustained patency with a thrombolytic regimen in acute coronary thrombosis would improve mortality. Four regimens were compared. In 41,021 patients randomised, nearly 3000 of these were in Australia and New Zealand. These regimens were streptokinase (SK) with subcutaneous heparin (9841 patients), SK and intravenous heparin (10,410 patients), accelerated tissue plasminogen activator (t‐PA) and intravenous heparin (10,396 patients) and combined SK and t‐PA (10,374 patients). The patients were well matched for baseline characteristics. The mean time to treatment was two hours. The accompanying regimen of investigation and revascularisation was aggressive ‐ 58% receiving angiography (28% in Australia), 15% angioplasty (5% in Australia) and 9% bypass surgery (4% in Australia). The GUSTO trial results should be interpreted in the light of these background observations.
Early thrombolytic treatment in acute coronary thrombosis
Authors: O'Rourke Michael
Published: March 2008

It is now accepted that the earliest event in acute myocardial infarction is coronary occlusion caused by fissuring of an atherosclerotic plaque, with resultant platelet deposition on the mural flaw and build up of thrombus within the lumen. The symptoms generated are caused by myocardial isckaemia, as first described by Herrick and Levine some 80 years ago. If occlusion is not relieved, irreversible damage (infarction) begins in the ischaemic region within 30 minutes, is half completed by 2.5 hours, and is virtually completed by six hours.

Thrombolysis is now accepted therapy for acute evolving myocardial infarction, but in most cases such therapy is not commenced for four hours after symptom onset, when most of the irreparable damage has already been done and when the potential for benefit has fallen substantially. The average time to thrombolysis in GUSTO worldwide, however, was two hours and, in Australia, 2.8 hours. Only patients who could be submitted to thrombolysis within six hours of the onset of pain were included.

The results of thrombolytic therapy in acute thrombosis are likely to be improved if delays are minimised. This requires education of the public in the symptoms of this condition, and on the actions to take if such symptoms arise. It also requires expeditious transfer to hospital (or appropriate treatment outside) and above all it requires expeditious assessment and treatment when patients arrive in hospital. Use of the diagnosis “myocardial infarction‘ for such patients implies that irreversible damage has already occurred and serves as a disincentive for the speedy, aggressive management that is logical and appropriate for dissolution of the offending coronary thrombus. (Aust NZ J Med 1993; 23: 742–744.)
LATE assessment of thrombolytic efficacy with alteplase (rt‐PA) six‐24 hours after onset of acute myocardial infarction
Authors: Bett J. H. Nick
Published: March 2008

Conflicting results on the benefit of thrombolytic therapy administered six to 24 hours after the onset of myocardial infarction (Ml) led to the LA TE trial. Five thousand seven hundred and eleven patients were treated with recombinant tissue plasminogen activator (t‐PA) or placebo, treatment was begun between six and 24 hours after the onset of infarction. For patients treated within 12 hours, there was a relative reduction in 35 day mortality of 25%, but no benefit for those treated at 12 to 24 hours. The benefits were confined to those whose treatment was begun within three hours of admission to hospital.

These results widen the window for effective treatment from six to 12 hours after the onset of infarction, but emphasise the need for expeditious treatment when the diagnosis of MI is suspected. (Aust NZ J Med 1993; 23: 745–748.)
GUSTO II Trail
Authors: Aylward Philip
Published: March 2008

The GUSTO II trial will investigate the role of antithrombin therapy in acute coronary syndromes, comparing standard doses of heparin with the specific antithrombin agent recombinant hirudin. The two drugs will be compared as primary therapy in patients with unstable angina or patients with myocardial infarction (MI) unsuitable for thrombolysis, and as post thrombolysis treatment in patients who fulfil standard criteria for thrombolysis therapy. A total of 12,000patients will be studied in a global, multifactorial trial.
Transjugular intrahepatic portosystemic stent‐shunt (TIPSS) for variceal haemorrhage: initial results in 28 patients
Authors: Duggan A. , Waugh R. C. , Perkins K. W. , Gallagher N. D. , Selby W. S.
Published: March 2008

Background: Endoscopic sclerotherapy is an effective form of treatment of bleeding varices in patients with cirrhosis. However, the mortality in patients who rebleed is high. Recently, trans‐jugular intrahepatic portosystemic stent‐shunt (TIPSS) has been developed as an alternative to surgical shunt formation in patients who have failed sclerotherapy.

Aim: To review the early experience with TIPSS at a teaching hospital.

Methods: Twenty‐eight patients underwent TIPSS on 30 occasions between September 1991 and June 1993 for bleeding oesophageal or gastric varices. The majority had alcoholic liver disease.

Results: TIPSS was performed successfully in all patients. Immediate control of bleeding was achieved, but one patient rebled within 24 hours. Complications related to the procedure occurred in 30%, but no patient died from these. Thirty‐day mortality was 11% (three of 28), two patients dying from progressive liver failure and one from sepsis. A further three patients died from six weeks to two months following TIPSS, due to liver failure in one, spontaneous bacterial peritonitis in the second and in the third after a fall. This represents an overall mortality of 21%. Three patients have rebled at mean follow‐up of 11.3 months. One of these had repeat TIPSS while the other two had balloon dilatation of the stent with control of bleeding. Four patients developed mild chronic encephalopathy which was readily controlled with medical therapy.

Conclusions: TIPSS is an effective means for control of bleeding from oesophageal and/or gastric varices not responding to other methods. Further follow‐up is required with regard to rates of rebleeding, encephalopathy and survival.
Transjugular intrahepatic portal‐systemic shunts (TIPS) – initial experience and clinical outcome
Authors: Hebbard G. S. , Fitt G. , Thomson K. R. , Angus P. W. , Jones R. , Sewell R. B. , Gibson P. R. , Hennessy O.
Published: March 2008

Background: The clinical role of the transjugular intrahepatic portal‐systemic shunt (TIPS) has not been fully defined.

Aims: To determine the technical results of TIPS and the clinical outcome of patients undergoing the procedure.

Methods: Retrospective audit of the results of the first 31 procedures performed in Melbourne.

Results: Thirty procedures were performed for variceal haemorrhage, one procedure was for ascites. The aetiology of the liver disease was cirrhosis due to alcohol in 20, cryptogenic in five, chronic viral infection in four, and autoimmune chronic active hepatitis in one. Nodular regenerative hyperplasia was present in one patient. Seventy‐seven per cent of procedures were considered successful based on the angiographic demonstration of shunt patency at the end of the procedure. The in‐hospital mortality in all patients undergoing TIPS was 45% and was 42% in patients undergoing technically successful TIPS. Only age could be identified as predictive of death in hospital. In patients leaving hospital, we found a rebleeding rate of 57% with one patient dying of bleeding, one requiring balloon tamponade and two requiring variceal sclerotherapy. Hepatic trauma was documented in six cases, shunt thrombosis in four cases, stent displacement in two cases and severe hepatic encephalopathy in one case.

Conclusions: TIPS has the potential to decompress the portal venous system, but the procedure is technically complex and should be performed in the knowledge that mortality and morbidity can be relatively high, particularly in patients whose condition is poor.
The influence of clinical problems, age and social support on outcomes for elderly persons referred to regional aged care assessment teams
Authors: Cape R. D. T. , Gibson S. J.
Published: March 2008

Abstract Background: Aged Care Assessment Teams (ACATs) have been established throughout Australia during the past seven years. Early studies of their effect have concentrated on their impact on the rate of institutionalisation of disabled elderly, the clinical characteristics of referred cases and the relationship between disability and recommended care plan.

Aims: The aim of this study was to explore the relationship between age, clinical features and social characteristics of AC AT subjects with outcomes at 12 months after assessment.

Methods: The examination of an arbitrary sample of persons referred to ACATs over a year by one generalist geriatrician with follow‐up of all cases by the three ACATs associated with the study was carried out. All analyses were performed on raw data presented as categorical variables in the form of contingency tables.

Results: The sample included 324 subjects who suffered from 2030 clinical problems with a mean of 6.5 per person aged 75 or over and 5.5 for those under 75. Cardiovascular and neurological disease were the commonest source of problems. Study of accommodation outcome at 12 months, for those subjects who survived this period revealed that, in the older group, over 60% of subjects with neurological disease were resident in nursing homes while the majority of all other groups remained in the community, as did two‐thirds of those aged under 75. Admission to a nursing home was independent of social support for older subjects with neurological disease, but it played a significant role in those with cardiopulmonary or musculoskeletal disease.

Conclusions: The study demonstrates that for one‐year survivors there is an increased likelihood of admission to a nursing home of people aged 75 or over with neurological disease, while those under 75 were more likely to remain at home. The association was independent of whether spouse, family or friends were living with the subject. (Aust NZ J Med 1994; 24: 378–385.)
Peak expiratory flow meters (PEFMs) – who uses them and how and does education affect the pattern of utilisation?
Authors: Garrett J. , Mitchell E. , Fenwick J. Mercer , Rea H. , Taylor G.
Published: March 2008

Background: Asthma control may be assisted by educating patients to use peak expiratory flow meters (PEFMs).

Aims: To find out the sociodemographic and clinical characteristics of asthmatics attending an Emergency Room (ER) who owned PEFMs.

Methods: We undertook a study of 352 asthmatics aged seven to 55 years who attended an ER. The following were analysed: their pattern of peak flow monitoring (PFM), the factors associated with ‘appropriate’ or daily PFM on entry to the study and then prospectively; whether asthma education influenced utilisation and whether there was a reduction in ER use or admissions in those who acquired a PEFM.

Results: Those owning a PEFM at entry to the study (54%) had more asthma morbidity (p= 0.0001), had had asthma for longer (p = 0.0001), had seen their medical practitioners more often in the previous nine months (p = 0.0001), were on more asthma medications (p= 0.0001) and were more likely to have been to an Asthma Clinic (p = 0.0001). Those not owning a PEFM were more likely to be of lower social class (p = 0.016) and of Pacific Island origin (p= 0.0001) suggesting that distribution is not ideal and is influenced by disease severity, amount of health care use and sociodemographics. Patients with a self‐management plan (35% of PEFM owners) and those receiving ‘good care’ or management, were more likely to use PFM ‘appropriately’ and to mention PFM in a scenario evaluating their response to worsening asthma control and argues for PEFMs to be distributed only in conjunction with a self‐management plan, and therefore in close association with the patients' medical practitioners.

Most patients (75%) appeared to prefer making management decisions based on symptoms rather than on their peak expiratory flow (PEF) and few (16%) performed daily PFM at entry to the study and fewer (6%) nine months later. There was an improvement in the pattern of PFM after education, but the acquisition of a PEFM made no difference to the frequency of ER use or admission,

Conclusion: More realistic goals need to be defined in relationship to PFM which may improve patients' acceptance of the strategy, and therefore, hopefully their compliance. Such strategies need to be consistently reinforced over time for them to have an impact on asthma morbidity. (Aust NZ J Med 1994; 24: 521–529.)
The boundaryless hospital
Authors: Braithwaite J. , Vining R. F. , Lazarus L.
Published: March 2008

Everyone in the western world has a clear idea of what a hospital is. People think of a series of large multi‐storeyed buildings filled with highly skilled and professional people (the ‘ologists’) offering sophisticated services amid an impressive array of high technology equipment. Over the past century the development of the hospital has involved cramming more and more services, staff and resources into the one location. Many large hospitals today have a staff of several thousand and are like a small city. But will hospital development continue in this direction? We think not. We believe the hospital has reached an evolutionary branch. The fundamental nature of hospitals is about to change because of the application of information and clinical technology, changing medical practices and economic rationalism. To understand why requires an excursion into a number of disciplines including the history of the hospital, organisation behaviour, medical practice, management and health care policy. And this is no mere epistemological exploration: it is vital for society generally and those who work in health care particularly to understand that the existing structure of the principal organisation which delivers health care is coming to an end. Our principal focus is the teaching hospital, but the analysis applies to a substantial degree to the many kinds of hospitals found throughout the industrialised world.
Direct mutational analysis in a family with hereditary non‐polyposis colorectal cancer
Authors: Van de Water N. S. , Jeevaratnam P. , Browett P. J. , Stewart S. M. , Lane M. R. , Jass J. R.
Published: March 2008

Background: It is now known that a proportion of cases of hereditary non‐polyposis colorectal cancer (HNPCC) is caused by mutations in the human homologue of the yeast DNA mismatch repair gene MSH2. A proline to leucine change due to a C to T transition in codon 622 of hMSH2 has been identified in a large HNPCC family of over 240 individuals.

Aim: To develop an assay to detect the family‐specific mutation and apply the findings to genetic screening.

Methods: The C to T change in codon 622 creates a new Mae I site (CTAG) allowing a simple, non‐radioactive assay to be developed in order to detect this mutation. The assay was applied to affected members of the family and their first degree relatives (siblings and offspring) between the ages of 17 and 77 years, a total of 75 subjects within two generations (IV and V).

Results: 13/13 (100%) subjects with cancer were mutation positive, 7/7 (100%) elderly subjects from generation IV and with no evidence of cancer were mutation negative, 23/57 (40%) subjects from generation V were mutation positive and 0/50 (100%) unrelated subjects were mutation negative. Following the demonstration of perfect segregation of the disease with the mutation, family members were invited to receive the results of the test. Sixty‐three (84%) responded within six weeks of receiving the invitation. Genetic screening and counselling members of HNPCC families was perceived as beneficial overall, allowing non‐carriers of the mutant gene (as well as their descendants) to be removed from a programme of colonoscopic surveillance. (Aust NZ J Med 1994; 24: 682–686.)
Bone presentation of non‐Hodgkin's lymphoma: experience at the Royal North Shore Hospital, Sydney; highlighting primary bone lymphoma
Authors: Shannon J. A. , Bell D. R. , Levi J. A. , Wheeler H. R. , Boyle F. M.
Published: March 2008

Background: Primary lymphoma of bone (PLB) is a rare form of extranodal lymphoma. Between 1975 and 1992 39 patients with lymphoma presenting in bone were seen at the Royal North Shore Hospital (RNSH), Sydney. Of these, 12 (31%) had truly localised disease (Stage IE).

Aims: Patients were studied retrospectively to determine the prognostic significance of bony involvement per se versus involvement of a single bony site, and to determine the impact of treatment modality on outcome.

Methods: The 39 patients were divided into three groups according to extent of disease; single osseous site (Stage IE), multifocal bone, and bone plus visceral and/or nodal disease. Kaplan‐Meier survival curves were constructed, and five year actuarial survival stated. Cox regression analysis was used to determine hazard ratios. Overall survival was used as the end‐point.

Results: A trend for better survival was noted with Stage IE disease. Multifocal and disseminated disease appeared to have a poorer outcome when assessed by hazard ratio, with a value of 3 (95% CI 0.87–10.4; ρ= 0.08), compared to unifocal disease. Radiotherapy alone was as effective as combined modality treatment although patient numbers were too small for statistical confirmation.

Conclusions: The stage of lymphoma, rather than bony involvement per se, seems to have more prognostic importance. Radiotherapy alone offered equivalent results to combined modality treatment in this series. (Aust NZ J Med 1994; 24: 701–704.)