bluuebunny/crossref_metadata_2025 huggingface dataset:
DIALYSIS AND TRANSPLANTATION IN PATIENTS WITH RENAL FAILURE DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS. THE AUSTRALIAN AND NEW ZEALAND EXPERIENCE
Authors: POLLOCK C. A. , IBELS L. S.
Published: March 2008

Abstract Between 1977 and 1985, 5726 patients in Australia and New Zealand entered end stage renal failure programmes. Of these, 63 patients had renal failure due to systemic lupus erythematosus (a prevalence of 1.1% of patients entering renal replacement programmes). When compared with patients with other forms of glomerulonephritis, there was a female preponderance and a younger age distribution in patients with renal failure due to lupus nephritis. Integrated patient, dialysis, and transplant survival data showed that results in patients with renal failure due to lupus nephritis were comparable with those in patients with other forms of glomerulonephritis or in patients with renal failure due to any cause. Age at entry significantly affected survival, with significant differences being found in those patients under as opposed to over 50 years of age. Causes of death in patients with lupus nephritis were similar to those in patients with renal failure due to other causes. It is concluded that dialysis and transplantation are acceptable forms of treatment for patients with end stage renal failure due to systemic lupus erythematosus.
LOCALISING VALUE OF PRIMARY POSITION UPBEATING NYSTAGMUS
Authors: HANKEY G. J. , SILBERT P. L. , EDIS R.H.
Published: March 2008

Abstract Primary position upbeating nystagmus has been associated clinically with intra‐axial brainstem and cerebellar lesions but evidence for more precise localisation to the ponto‐mesencephalic and ponto‐medullary junctions is accumulating.

We report the occurrence of primary position upbeating nystagmus in three patients who had clinical signs of pontine lesions at the ponto‐mesencephalic and ponto‐medullary junctions. Radiological confirmation was possible in two cases.
PROLONGED SURVIVAL WITH CONVENTIONAL CISPLATINUM/VINBLASTINE/BLEOMYCIN THERAPY ALONE FOR MEDIASTINAL ENDODERMAL SINUS TUMOUR
Authors: MACKINTOSH J. F. , HAWSON G. A. T.
Published: March 2008

Abstract Combination chemotherapy with cisplatin, vinblastine, and bleomycin (PVB) is considered to be the best treatment for disseminated non‐seminomatous germ cell tumours, with a 70% long term disease‐free survival rate. For one subgroup of patients, those with primary mediastinal endodermal sinus turnours, results with PVB have been disappointing and more intensive regimens with major toxicity have recently been proposed. We report a patient who, following standard PVB chemotherapy, is one of the longest reported survivors of mediastinal endodermal sinus tumours. Optimal chemotherapy for endodermal sinus tumours is yet to be determined.
MINIMAL CHANGE GLOMERULONEPHRITIS ASSOCIATED WITH SCHISTOSOMA HEMATOBIUM INFECTION ‐ RESOLUTION WITH PRAZIQUANTEL TREATMENT
Authors: TURNER I. , HARRISON A. , IBELS L. S. , MOIR D. , ALEXANDER J. H.
Published: March 2008

Abstract In a patient presenting with hematuria and proteinuria (albuminuria), bladder biopsy demonstrated Schistosoma hematobium infection and renal biopsy showed a minimal change glomerulonephritis. The hematuria and proteinuria resolved following praziquantel therapy. The pathogenesis of glomerulonephritis in association with Schistosoma infection is discussed.
CHLORAMBUCIL ALLERGY—A SERIES OF THREE CASES
Authors: HITCHINS R. N. , HOCKER G. A. , THOMSON D. B.
Published: March 2008

Abstract Three cases of chlorambucil‐related skin eruption in patients with low‐grade non‐Hodgkin's lymphoma are described. Cases were confirmed by exclusion of other drugs, rechallenge, skin testing and histology. One patient died due to florid erythema multiforme. Only two other adequately documented cases are described.
FACIAL NERVE TUMOURS OR ALL THAT PALSIES IS NOT BELL'S
Authors: MA K. H. , FAGAN P. A.
Published: March 2008

:Most cases of facial nerve palsy are idopathic, that is to say, Bell's palsy. A few cases are due to tumours on the facial nerve. Early diagnosis will avoid extensive tissue destruction with loss of hearing and lead to satisfactory facial reinnervation.

Five cases are presented. In three, there was a significant delay in diagnosis. A facial nerve tumour generally presents as a paralysis of slow onset and the degree of paralysis often fluctuates. Diagnosis can be made in most cases by high resolution CT. (AustNZ J Med 1988; 18: 613–616).
THE ROLE OF PERMANENT PACING AFTER ANTERIOR MYOCARDIAL INFARCTION COMPLICATED BY TRANSIENT COMPLETE ATRIOVENTRICULAR BLOCK
Authors: GRIGG L. , KERTES P. , HUNT D. , GOBLE A. , PITT A. , BOXALL J. , HALE G.
Published: March 2008

A randomised study was performed to assess the influence of prophylactic permanent pacing on the incidence of late sudden death in patients surviving transient complete atrioventricular block with acute anterior myocardial infarction. Fourteen patients were studied, of whom ten died within two years, confirming the high overall late mortality in this group. The deaths included eight of the nine paced patients, of whom seven died suddenly. We conclude that prophylactic permanent pacing is not warranted in patients surviving transient complete atrioventricular block with acute anterior myocardial infarction.
DEATHS FROM SUDDEN INFANT DEATH SYNDROME ON PUBLIC HOLIDAYS AND WEEKENDS
Authors: MITCHELL E. A. , STEWART A. W.
Published: March 2008

Deaths from sudden infant death syndrome (SIDS) between 1979 and 1984 were tabulated by month for weekdays, weekends and public holidays. Examination of deaths by day of the week showed the weekend had more deaths than the weekdays (χ2= 26.3, df = 6, p= 0.0002) and that this pattern occurred in both the under three months and three‐11 months age group. There were 40 deaths on the 78 public holidays in the six years under study. Modelling the data showed that there was no difference between the number of deaths on holidays compared to weekends.
BRUCELLOSIS COMPLICATED BY AORTIC VALVE ENDOCARDITIS
Authors: SKILLINGTON PETER D. , McGIFFIN DAVID C. , KEMP RICHARD , BETT J. H. N. , HOLT GEOFFREY , FORGAN‐SMITH ROSS
Published: March 2008

A 30 year old veterinary surgeon developed a febrile illness with serological evidence of Brucellosis. He was known to have aortic valve disease and during the course of the illness, the clinical features of endocarditis became evident, with a vegetation visible echocardiographically on the aortic valve. Because of persisting fever despite appropriate antibiotic therapy, aortic valve replacement with a viable cryopreserved allograft aortic valve was undertaken. Organisms consistent with Brucella species were demonstrated in the excised vegetation. The patient received a six week course of antibiotics and his post‐operative course was uneventful.
LEAD INTOXICATION IN SYDNEY HARBOUR BRIDGE WORKERS*
Authors: POLLOCK C. A. , IBELS L. S.
Published: March 2008

Thirty‐eight Sydney Harbour Bridge workers were assessed for possible lead intoxication. Forty‐seven per cent were found to have significant lead intoxication as assessed by calcium disodium edetate chelation (Ca EDTA) testing and were subsequently effectively and safely treated with Ca EDTA. The prevalence of neurological, constitutional, gastrointestinal and musculoskeletal symptoms was significantly greater in those with, than those without, lead toxicity. Although blood lead levels and, to a lesser extent, hematological parameters were of some use in diagnosis, they were not sufficiently sensitive and thus should not be used in screening workers at risk of lead intoxication. Ca EDTA testing remains the diagnostic method of choice. Patients exposed to lead dust and fumes, in whom symptoms of lead intoxication are present, should undergo such testing and if a positive result is obtained, then EDTA chelation therapy should be instituted. (Aust NZ J Med 1988; 18: 46–52).
MITOCHONDRIAL MYOPATHY, ENCEPHALOPATHY, LACTIC ACIDOSIS AND STROKE‐LIKE EPISODES (MELAS): ADOLESCENT ONSET WITH SEVERE CEREBRAL EDEMA
Authors: DANKS R. A. , DOREVITCH M. , CUMMINS J. T. , BYRNE E.
Published: March 2008

A 19‐year‐old woman with long‐standing sensorineural deafness, bilateral cataracts and mild clumsiness, presented with acute focal edema in the left temperoparieto‐occipital area which required surgical decompression as a life‐saving measure. Investigation revealed a persistent lactic acidemia and evidence of many ragged red fibres in a skeletal muscle biopsy specimen, suggesting a diagnosis of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke‐like episodes (MELAS) syndrome. The patient developed two further stroke‐like episodes over a short period. One sibling died at the age of 14 years with a progressive neurological illness characterised by seizures, bilateral optic atrophy, ataxia, myoclonus and progressive dementia.

The diagnosis of MELAS syndrome should be considered in young people presenting with stroke‐like episodes that fail to conform to a given vascular territory, particularly if they have longstanding minor neurological abnormalities or a family history of obscure early onset neurological disease.

The different clinical pictures in the two affected siblings in this family suggest that MELAS syndrome is part of a spectrum of inherited mitochondrial cytopathies rather than a discrete disease entity.
PAROXYSMAL CEREBELLAR ATAXIA
Authors: FEENEY G. F. X. , BOYLE R. S.
Published: March 2008

This report describes the clinical features of 13 cases of periodic, paroxysmal cerebellar ataxia. A family history suggestive of an autosomal dominant disorder was present in 10 cases, the first two reported Australian families involved with this disorder. Variation in the age of onset, frequency of attacks and the presence of distinct symptom complexes within one kindred confirm the heterogeneous nature of this disorder. CT head scans were performed on nine cases and were normal in eight. EEG findings were abnormal in six of nine performed. Treatment with acetazolamide resulted in abolition of paroxysms in nine of the 10 treated cases. In addition, two children had dramatic improvement in previously impaired gross and fine motor skills.
PERICARDITIS ASSOCIATED WITH TICK‐BORNE Q FEVER
Authors: BEAMAN M. H. , HUNG J.
Published: March 2008

A case of pericarditis associated with Q fever is described. Transmission was probably via an arthropod vector, which was most likely the kangaroo tick Amblyomma triguttatum. Complete recovery occurred in association with the use of non‐steroidal anti‐inflammatory agents only. This is a rare presentation of Q fever implicating transmission by a novel vector.
COMMUNITY‐ACQUIRED ACINETOBACTER PNEUMONIA
Authors: GOTTLIEB T. , BARNES D. J.
Published: March 2008

We describe the first case report of community‐acquired Acinetobacter pneumonia in Australia. Well recognised risk factors for this entity (alcoholism, diabetes mellitus and chronic lung disease) were present in our patient. His pneumonia ran a fulminant course, with death occurring within 24 hours of presentation to hospital. Whilst rare, this infection is being described with increasing frequency, particularly in developing countries including Papua New Guinea.
THE KETOCONAZOLE‐CYCLOSPORIN INTERACTION IN AN ELDERLY RENAL TRANSPLANT PATIENT
Authors: CHARLES B. G. , RAVENSCROFT P. J. , RIGBY R. J.
Published: March 2008

Administration of ketoconazole to an elderly renal transplant patient who was taking cyclosporin resulted in markedly increased whole blood cyclosporin concentrations and evidence of both renal and hepatic dysfunction.
CORONARY ARTERY VASCULITIS AND MYOCARDIAL INFARCTION ASSOCIATED WITH ANTIPHOSPHOLIPID ANTIBODIES IN A PREGNANT WOMAN
Authors: RALLINGS P. , EXNER T. , ABRAHAM R.
Published: March 2008

A 28‐year‐old, 16 week primigravida presented with an acute anteroseptal myocardial infarction and a past history of recurrent venous thromboembolism and primary infertility. Although she lacked other clinical features of systemic lupus erythematosus, she had a circulating lupus' anticoagulant, anticardiolipin antibodies, a weakly positive anti‐nuclear antibody and thrombocytopenia. She died suddenly despite corticosteroid therapy and autopsy revealed coronary arteritis and thrombosis.
ALLERGIC FUNGAL SINUSITIS PRESENTING AS A PARANASAL SINUS TUMOUR
Authors: ROBSON J. M. B. , HOGAN P. G. , BENN R. A. V. , GATENBY P. A.
Published: March 2008

Allergic fungal rhinosinusitis is a rare complication of atopic upper airways disease which may present initially as an expansive tumour of the paranasal sinuses. This reported case was caused by the rare fungal pathogen Bipolaris hawiiensis and illustrates typical clinical and laboratory features of this disorder. Although the optimum management of allergic fungal sinusitis is controversial, combined therapy with surgical clearance, antifungal agents and corticosteroids produced a favourable outcome.
HYPERCALCEMIA AND SQUAMOUS CELL CARCINOMA COMPLICATING CHRONIC OSTEOMYELITIS
Authors: FENTON A. J. , KING A. R. , REID I. R.
Published: March 2008

A case of chronic osteomyelitis complicated by the development of squamous cell carcinoma in the sinus tract is described. This complication was itself associated with the development of humoral hypercalcemia of malignancy, a phenomenon not previously described in this context. Common misconceptions relating to the interpretation of serum parathyroid hormone levels led to the misdiagnosis of hyperparathyroidism and parathyroid exploration. Forced saline diuresis, mithramycin and oral phosphate supplements were not able to provide long‐term control of the hypercalcemia but the patient was subsequently managed successfully with intravenous (3‐amino‐1‐hydroxypropylidene)‐1, 1‐bisphosphonate (APD) and resection of the causative tumour.
CLINICAL MANIFESTATIONS OF THE THROMBOCYTOPENIA AND ABSENT RADII (TAR) SYNDROME
Authors: GOUNDER D. S. , PULLON H. W. , OCKELFORD P. A. , NICOL R. O.
Published: March 2008

Six patients with the classical features of the TAR syndrome were diagnosed at birth. In one case an older sibling was also affected. The characteristic features of foreshortened forearms and radially deviated hands were noted in all cases at presentation and confirmed radiologically. With one exception skeletal abnormalities of the lower limbs were also present. Varying degrees of thrombocytopenia were present at birth with three of the five patients having platelet counts below 50 ± 109/L. Bone marrow examination was performed in two patients and revealed an absence of normal megakaryocytes. Two patients with severe thrombocytopenia had bleeding complications during infancy requiring transfusion support. Severe gastroenteritis occurred in two patients, in one of whom it was attributed to cow's milk intolerance. In all patients the platelet count has risen progressively since birth. Orthopedic surgical procedures have been performed without hemorrhagic complications.
CEREBRAL DYSFUNCTION WITH EVIDENCE OF CEREBRAL HIV INFECTION AMONGST ASYMPTOMATIC HIV SEROPOSITIVE SUBJECTS
Authors: FIELD M. , KUNZE H. , TATE J. , FRAZER I.H.
Published: March 2008

Twelve asymptomatic HIV seropositive subjects ages 21 to 40 years were examined for serologic evidence of cerebral HIV infection, for cerebral structural abnormalities, and for neuropsychologic evidence of cerebral dysfunction using standard methods. Eleven of the 12 had antibody to HIV in the cerebrospinal fluid (CSF). Nine subjects had oligoclonal immunoglobulins in the CSF, of whom five had some for which there were no corresponding serum oligoclonal immunoglobulins (‘unique’ oligoclonal immunoglobulins). Intracerebral synthesis of HIV specific antibodies was demonstrated for four subjects. Significant deficits of memory and frontal lobe function were found in five of the 12 subjects. Subjects who had oligoclonal immunoglobulins unique to the CSF all had significant neuropsychological abnormalities. No structural cerebral abnormalities were demonstrated using CT scanning for any subject tested. These results support other evidence that HIV is neurotropic and capable of directly inducing brain damage even in immunologically normal subjects. Tests of memory and frontal lobe function are frequently abnormal in patients with early HIV infection, and identify as abnormal a similar group of patients to immunological or biochemical tests which might indicate cerebral HIV infection.
ERYTHROCYTE SODIUM‐POTASSIUM PUMP IN THYROTOXIC PERIODIC PARALYSIS
Authors: LAM K. S. L. , YEUNG R. T. T. , BENSON E. A. , WANG C.
Published: March 2008

To search for a genetic marker in patients with thyrotoxic periodic paralysis (TPP), we studied the erythrocyte sodium‐potassium pump activity in 13 patients with TPP; 30 thyrotoxic patients with no history of paralysis (T) and 69 euthyroid controls. In thyrotoxic patients (TPP and T), erythrocyte ouabain sensitive sodium efflux rate constant were decreased while erythrocyte sodium content as increased. All these changes reverted to normal when the patients became euthyroid. Maximal ouabain binding capacity correlated positively with ouabain sensitive sodium efflux rate constant (r = 0.542; p < 0.001; n = 155) and negatively with serum thyroxine concentration (r = ‐0.571; p < 0.001; n = 60) and erythrocyte sodium content (r = ‐0.521; p < 0.001; n = 155). In the thyrotoxic state, maximal ouabian binding capacity was just significantly higher in TPP when compared with T (0.268 ± 0.014 and 0.234 ± 0.009 pmol/109 cells respectively; p < 0.05). This difference could not be demonstrated when the patients became euthyroid. Our findings suggest that patients with TPP respond to thyrotoxicosis with a smaller decrement in erythrocyte sodium‐potassium ATPase activity than patients without a history of paralysis. However, the difference is too small to represent a useful genetic marker for this disease entity. (Aust NZ J Med 1989; 19: 6–10.)
FAMILIAL HYPOPARATHYROIDISM DUE TO AN ABNORMAL PARATHYROID HORMONE MOLECULE
Authors: McELDUFF A. , WILKINSON M. , LACKMANN M. , GRANT S. , WEISSBERGER A. , ROBINSON B. , WILLIAMSON P. , WILMSHURST E.
Published: March 2008

The case of a 53‐year‐old man with familial hypoparathyroidism in the presence of circulating immunoreactive PTH is discussed. The patient responded to exogenous PTH by an increase in urinary cAMP excretion and by several post cyclase parameters including an increase in serum calcium and 1,25‐dihydroxyvitamin D, an increase in urinary phosphate excretion and a decrease in urinary calcium. Immunoreactive PTH was detected in this patient's serum by three separate anti‐PTH antisera. This immunoreactive PTH behaved aberrantly with these antisera. Nonparallelism to the standard curve was seen in two radioimmunoassays and the material was detected by an antiserum which preferentially binds bovine PTH. No circulating PTH binding activity was detectable. Family studies confirmed the genetic nature of the abnormality. HPLC studies revealed the presence of an abnormally hydrophobic fraction containing immunoreactive PTH. We believe these findings constitute strong evidence for the presence of an abnormal PTH molecule with reduced biological activity resulting in hypoparathyroidism. (Aust NZ J Med 1989; 19: 22–30.)
MALIGNANT PARAGANGLIOMA CAUSING BILATERAL PLEURAL EFFUSIONS
Authors: CLARNETTE ROGER M. , BAYLISS E. J. , MATZ L.
Published: March 2008

A male patient presented with dyspnea due to a large left pleural effusion, and pleural biopsy revealed a malignant paraganglioma. Raised urinary catecholamine levels confirmed a functioning tumour. Aggressive local spread occurred which did not respond to cytotoxic chemotherapy. The primary site of the tumour was most likely the aorticosympathetic chain. (Aust NZ J Med 1989; 19: 61–63).
Wegener's granulomatosis—use of cyclosporin‐A: a case report
Authors: Harley N. , Ihle B.
Published: March 2008

Wegener's granulomatosis is a chronic relapsing condition requiring long term immunosuppressive treatment. Periodic exacerbations may require increased dosage of steroids and/or cyclophosphamide. We present a case of a patient with end‐stage renal failure who had repeated pulmonary cavitating lesions but was unable to tolerate high dose standard therapy. After only a few months with cyclosporin A there was almost complete resolution of his lung lesions.
Plasma concentrations of salbutamol in acute severe asthmatics
Authors: Lewis L.D. , McLaren M. , Essex E. , Cochrane G.M.
Published: March 2008

We studied prospectively 11 asthmatic patients presenting to the Accident and Emergency department with acute asthma. (Four patients reported historically that they were taking some form of oral salbutamol as part of their maintenance therapy.) Standard cardiovascular and respiratory parameters and plasma salbutamol concentrations were measured before and one hour after treatment with five milligrams of nebulised salbutamol.

The median plasma salbutamol concentration before treatment was below the level of detection of the assay (less than 3 ug.l‐1) with a range from less than 3 ug.l‐1 to 34.6 ug.l‐1. One hour post treatment the median plasma salbutamol concentration was 7.4 ug.l‐1 (range<3.0 ug.1‐1 to 56.0 ug.1‐1) p <0.05. (Wilcoxons test).

Correlations were investigated between the measured pretreatment physiological variables and pre‐treatment plasma salbutamol concentrations. None were found to be significant. Similar analysis of the measured post‐treatment physiological variables and post‐treatment plasma salbutamol concentrations again revealed no significant correlations. However, a significant negative correlation was noted between the change in plasma salbutamol concentration with treatment and the change in respiratory rate (Rs=–0.56, p= 0.04).

If asthmatics do indeed use high doses of inhaled beta‐2‐agonists (salbutamol) in an attempt to abort an acute attack (as many clinicians suspect) little, if any, of the drug appears to reach the systemic circulation. In this study the administration of five milligrams of nebulised salbutamol to acute asthmatics did not produce excessive increases in plasma salbutamol concentration, even in those patients taking oral salbutamol as part of their maintenance therapy.
Intestinal lymphangiectasia with protein losing enteropathy, toxic copper accumulation and hypoparathyroidism
Authors: O'Donnell D. , Myers A. M.
Published: March 2008

Abstract A 13‐year‐old girl presented with malabsorption which was ascribed to intestinal lymphangiectasia. Three years later a generalised seizure resulted from hypocalcaemia that was shown to be due to hypoparathyroidism during investigation of which toxic copper accumulation was recognised. The chance occurrence of three rare conditions is extremely remote making intestinal lymphangiectasia likely as the primary pathology. It is suggested that chronic intestinal loss of the copper‐carrying caeruloplasmin resulted in toxic parathyroid deposition of copper leading to hypoparathyroidism with consequent hypocalcaemic seizure.
Creutzfeldt‐Jakob disease in a recipient of human pituitary‐derived gonadotrophin
Authors: Cochius J. I. , Burns R. J. , Blumbergs P. C. , Mack K. , Alderman C. P.
Published: March 2008

Abstract A forty‐year‐old female presented with an unsteady gait 13 years after receiving an eight‐month course of human pituitary‐derived gonadotrophin injections as treatment for infertility. Over the next ten months the patient subsequently developed generalised myoclonic jerks and dementia and finally died. Neuropathological examination revealed changes in the brain consistent with Creutzfeldt‐Jakob disease.

This is the first reported case of Creutzfeldt‐Jakob disease in a recipient of human derived gonadotrophin.
Monoclonal immunoglobulin bands in the cerebrospinal fluid
Authors: McCombe P. A. , Barr A. E. , Brown N. N. , Parkin L.
Published: March 2008

Isoelectric focusing and immunofixation allow the detection of abnormal immunoglobulin bands in the cerebrospinal fluid (CSF). In normal subjects, the immunoglobulins in the CSF are derived from serum. In inflammatory disorders of the nervous system, there may be intrathecal immunoglobulin synthesis usually seen as oligoclonal bands confined to the CSF. Monoclonal immunoglobulin bands in the CSF are not common. We surveyed 1490 CSF samples, and found that a total of nine had a monoclonal immunoglobulin band and that in three this was not present in the serum. Of these three, one patient had chronic inflammatory demyelinating polyneuropathy and the antibody may have been secreted by lymphocytes which had infiltrated the nervous system. The other patients had either lymphoma or lymphomatoid granulomatosis within or adjacent to the nervous system and the monoclonal immunoglobulin was probably secreted by B lymphocytes within those lesions. (Aust NZ J Med 1991; 21: 227–229.)
A reliable screening test for coeliac disease: enzyme‐linked immunosorbent assay to detect anti‐gliadin antibodies in serum
Authors: Gonczi J. , Skerritt J. H. , Mitchell J. D.
Published: March 2008

Abstract A simple, rapid, highly reproducible enzyme‐linked immunosorbent assay detecting anti‐gliadin antibodies in serum to screen for coeliac disease (CD) is described. By combining the results of anti‐gliadin IgA and IgG determinations the overall sensitivity of the assay was found to be 100% and the specificity 96% for children and 99% for adults. Significantly elevated anti‐gliadin IgA and IgG antibodies were detected in all 20 children and all 25 adults with untreated CD. False positive results were found in 1/79 histologically normal control and 5/86 disease control children, while for adults false positive rates were 0/74 and 1/34 for the healthy and disease control groups, respectively. Anti‐gliadin IgA and IgG was measured in serum samples from 52 coeliac patients (11 children and 41 adults) treated with a gluten‐free diet (GFD). Each of the children and 28 of the adults who followed a strict GFD had significantly lower IgA and IgG levels than untreated CD patients. The serum anti‐gliadin IgA and IgG levels of the 13 adults not complying with a GFD were similar to those found for untreated CD patients. This assay is recommended as a screening test for CD as well as a tool for follow‐up of treated patients.
vasive aspergillosis in AIDS
Authors: Fairley K. , Mijch A. , Kent J. , Hoy J. , Street
Published: March 2008

Abstract Invasive aspergillosis (IA) is a rare infection in patients with the Acquired Immune Deficiency Syndrome (AIDS). We report the first Australian cases of histologically and microbiologically proven IA diagnosed antemortem in AIDS patients. We also describe the first case of laryngeal involvement and the unusual case of a pneumothorax due to IA. These three cases illustrate the varied clinical and pathological features of IA in AIDS and highlight some of the difficulties in diagnosis and treatment. The infections occurred in the setting of advanced immunodeficiency and multiple opportunistic infections and responded poorly to treatment.
Biochemical and molecular investigation of mitochondrial disease: an illustrative case showing the value of a multifaceted approach
Authors: Byrne E. , Jean‐Francois B. , Thyagarajan D. , Collins S. , Dennett X. , Marzuki S.
Published: March 2008

Abstract :Detailed biochemical and molecular investigations in a patient with Kearns‐Sayre syndrome are presented. Polarographic studies in isolated mitochondria revealed a global impairment in respiratory capacity consistent with an admixture of functional and non‐functional mitochondria. Cytochrome difference spectra revealed a selective deficiency in cytochrome aa3. Western immunoblot studies revealed normal subunit content of Complexes 1, III and IV. Southern blot studies of mtDNA showed a deletion of approximately 5 Kb coexisting with wild type DNA. PCR analysis confirmed that this deletion lies between the ATPase8 and NAD coenzyme Q oxidoreductase subunit 5 (ND5) genes. Breakpoint sequencing revealed a 13 nucleotide direct repeat flanking sequence (ACCTCCCTCACCA) consistent with slippage in mtDNA during replication as the mechanism of deletion. Histochemical studies of skeletal muscle revealed many cytochrome oxidase negative fibres and immunocytochemical studies showed cytochrome oxidase negative areas with abundant respiratory complex protein suggesting upregulation. The value of a multifaceted approach in unravelling the pathophysiology of mitochondrial diseases is emphasised. (Aust NZ J Med 1991; 21: 837–843.)
Infradiaphragmatic Hodgkin's disease, long term follow‐up of a rare presentation
Authors: Liew K. H. , Ding J. C. , Cruickshank D. , Quong G. G. , Wolf M. M. , Cooper I. A.
Published: March 2008

Hodgkin's disease limited to the infradiaphragmatic region was seen in 30 of 306 (9.8%) of all Stage I and II patients referred to the Peter MacCallum Cancer Institute between 1968 and 1980. The male:female ratio was 2.3:1 with median age of 43.5 years at presentation.

Of the seven patients with clinical stage (CS) IA‐IIA disease who had staging laparotomy and splenectomy only one CSIIA patient had splenic involvement. The patients were staged as pathological stage (PS) IA 2, PSIIA 5, CSIA 4, CSIIA 10, CSIIB 9.

Primary treatment was by radiation in 24 patients, combination chemotherapy in five and surgical excision in one. Twenty‐five patients achieved complete response. Relapse free survival (RFS) at five and ten years was 59% and the five and ten‐year survival was 75% and 67% respectively.

On univariate analysis the significant prognostic factors for RFS and survival were stage, constitutional symptoms and presence of bulky disease.

Using Cox regression analysis the only significant variable for RFS and survival was bulky disease (p= 0.01, 0.02).

A treatment policy for patients with infradiaphragmatic Hodgkin's disease is recommended.
Kohlmeier‐Degos disease: a multisystem vasculopathy with progressive cerebral infarction
Authors: Burrow J. N. , Blumbergs P. C. , Iyer P. V. , Hallpike J. F.
Published: March 2008

Kohlmeier‐Degos disease, or malignant atrophie papulosis, is a rare and clinically distinctive vasculopathy characterised by cutaneous features with frequent gastrointestinal and neurological involvement and, almost invariably, with fatal outcome. The case is reported of a 22‐year‐old male who died of progressive cerebral infarction nine months from the time of development of pathognomonic skin lesions. Treatments with anti‐platelet agents and plasma exchange were ineffectual in altering the progressive course of the disease. At autopsy, there was an extensive small vessel occlusive vasculopathy with similar features in skin, brain and bowel wall. The aetiology of Kohlmeier‐Degos disease remains unknown. Diagnosis is on clinico‐pathological grounds. Awareness of the disease is important, particularly in the differential diagnosis of cerebrovascular‐arteritic‐syndromes presenting in young adults.
Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever
Authors: Abernethy M. , Bass N. , Sharpe N. , Grant C. , Neutze J. , Clarkson P. , Greaves S. , Lennon D. , Snow S. , Whalley G.
Published: March 2008

Background: The incidence of acute rheumatic fever in New Zealand remains relatively high. Reliable early diagnosis of carditis is difficult and important in management.

Aim: To determine if Doppler echocardiography contributed to the early diagnosis of carditis in acute rheumatic fever.

Methods: Forty‐seven patients admitted to hospital with suspected acute rheumatic fever and 19 control patients, with a febrile illness due to a documented non‐cardiac bacterial infection, were assessed two days and two weeks following admission. Presence or absence of clinical carditis was determined by a cardiologist unaware of the suspected diagnosis, from clinical examination, chest radiograph, electrocardiogram (ECG) and two dimensional echocardiogram. Doppler echocardiography was then performed and interpreted by a second cardiologist unaware of the diagnosis. After completion of the study the Jones criteria were applied, to categorise the patients with suspected acute rheumatic fever into four groups for the final diagnosis: no acute rheumatic fever, possible acute rheumatic fever, definite acute rheumatic fever without carditis, and definite acute rheumatic fever with carditis.

Results: In 19 patients with a final diagnosis of acute rheumatic fever and carditis at the baseline assessment carditis was detected by clinical assessment in 15 patients, compared with 19 patients with evidence of significant valve regurgitation by Doppler echocardiography. Following the two week assessment, all 19 patients had both clinical and Doppler evidence of carditis. Five patients with a final clinical diagnosis of possible acute rheumatic fever or definite acute rheumatic fever without carditis, had a Doppler abnormality detected. There was no clinical or Doppler abnormality in the febrile controls.

Conclusions: Doppler echocardiography is more sensitive than clinical assessment in the detection of carditis in acute rheumatic fever, and can contribute to earlier diagnosis. (Aust NZ J Med 1994; 24: 530–535.)
Selection of patients for randomised trials: a study based on the MACOP‐B vs CHOP in NHL study
Authors: Stone J. M. , Laidlaw C. R. , Page F. J. , Cooper I.
Published: March 2008

Background: Selection of patients for a clinical trial is affected by awareness of the existence of the trial, interest in the study question and clinical practices and views of the clinicians.

Aims: To investigate the selectivity that may have occurred at Peter MacCallum Cancer Institute (PMCI) during the ANZ Lymphoma Group trial of MACOP‐B vs CHOP in non‐Hodgkin's lymphoma (NHL).

Methods: NHL patients at PMCI in the study period were assessed against the trial's eligibility criteria. Comparisons were made between eligible (except for consent) non‐trial patients and all patients actually randomised into the trial.

Results: Of 497 patients presenting during the trial period, 320 (64%) did not meet the specified eligibility criteria, 102 (21%) were unsuitable on other grounds (age and medical) and 75 (15%) were eligible. Of those eligible, 43 (57%) were entered into the trial and 32 (43%) were not. Four non‐trial patients had inappropriate application of eligibility criteria and 13 unknown reason. Eligible non‐trial patients were similar to trial patients in most patient and tumour characteristics and overall survival. Significantly more non‐trial patients had higher stage disease (p = 0.02). More non‐trial patients had lower grade histology, but this was not significant.

Conclusions: Physician selectivity occurred with respect to patient entry, but trial and non‐trial patients were similar in most characteristics. Eligibility criteria should specify that patients can withstand all trial drugs and patient availability for treatment and follow‐up. PMCI trial accural could have been up to 33% greater. These results suggest the trial accrual period could have been 25% shorter. Patient entry into this trial by PMCI clinicians compared favourably with other centres. (Aust NZ J Med 1994; 24: 536–540.)
Volumetric and visual assessment of the mesial temporal structures in Alzheimer's disease
Authors: Desmond P. M. , O'Brien J. T. , Tress B. M. , Ames D. J. , Clement J. G. , Clement P. , Schweitzer I. , Tuckwell V. , Robinson G. S.
Published: March 2008

Background: Alzheimer's disease is the commonest cause of dementia. Clinical diagnosis of Alzheimer's disease may be difficult. Magnetic resonance imaging has a role to play in diagnosis.

Aim: To assess whether volumetric and/or visual assessment of the mesial temporal structures is useful in separating patients with Alzheimer's disease from age matched controls.

Methods: Twenty‐four patients with Alzheimer's disease diagnosed by NINCDS/ADRDA criteria and 15 age matched controls were studied with magnetic resonance imaging (MRI) and volumetric techniques. Segmented volumes of the mesial temporal structures were assessed visually and volumetrically.

Results: Volumetric analysis demonstrated significant (p<.001) differences between the two groups, but showed overlap in individual cases. Discriminant function analysis predicted correct group membership (patient or control) in 85% of cases. Visual assessment alone demonstrated a sensitivity of 92% and a specificity of 93% in distinguishing the Alzheimer patients from controls.

Conclusion: Volumetric and visual assessment of the mesial temporal structures is useful in separating Alzheimer patients from controls. Overlap is present in individual cases. Visual assessment was as useful in separating the two groups as the volumetric analysis. (Aust NZ J Med 1994; 24: 547–553.)
Low serum cholesterol is not associated with depression in the elderly: data from an Australian community study
Authors: McCallum J. , Simons J. , Simons L. , Friedlander Y.
Published: March 2008

Background: Low serum cholesterol may contribute to depressive symptoms in the elderly.

Aims: To test the relationship between depressive symptoms and low serum cholesterol in an elderly cohort.

Methods: This was an examination of cross‐sectional data in a community study of 1237 men and 1568 women aged 60 + years in Dubbo, NSW. Quintiles of serum cholesterol were defined for men and women. The Center for Epidemiological Studies Depression Scale was used as a continuous, dependent variable in multiple regression analyses.

Results: Low serum cholesterol was not associated with depressive symptoms in older men or women. Health status, measured by poorer self‐ratings, recent hospitalisation, higher disability levels and higher consumption of prescribed and self‐prescribed drugs, predicted depressive symptoms. As well, the significance in the statistical model of financial difficulties, low self esteem, low feelings of self efficacy, the adequacy of practical help and emotional support, and recent widowhood, confirmed the importance of social origins of depressive symptoms. (Aust NZ J Med 1994; 24: 561–564.)
Comparative hospitalisation and mortality rates of Aboriginal and non‐Aboriginal Western Australians in their sixth and seventh decades
Authors: Gracey M. , Veroni M.
Published: March 2008

Background: Aboriginals have higher hospitalisation and mortality rates and die, on average, about 15 years earlier than non‐Aboriginals in Western Australia (WA).

Aims: To investigate Aboriginal morbidity and mortality rates in WA in comparison with the rest of the population, with particular reference to the ages of 50 to 65 years.

Methods: Mortality rates from 1983–1989 inclusive for Aboriginals and non‐Aboriginals in WA were compared. Major causes of Aboriginal mortality in males and females were matched to the ages at which similar rates from the same causes occurred in non‐Aboriginals. Rate ratios (Aboriginahnon‐Aboriginal) for causes of death at ages 60–64 years were determined. Hospitalisation rates for Aboriginal and non‐Aboriginal people aged 50–64 years in WA in 1988 were used to estimate hospitalisation rate ratios.

Results: Hospitalisation rates in WA were much higher among 50 to 64 year old Aboriginals than non‐Aboriginals for most diseases, particularly for infectious and parasitic diseases, and injury and poisoning. Admissions for circulatory diseases were double to four times as frequent among Aboriginals. The main causes of deaths in Aboriginal males were circulatory diseases, injury and poisoning, respiratory diseases, neoplasms, and digestive diseases; in Aboriginal females the main causes of deaths were circulatory diseases, neoplasms, diabetes, respiratory diseases, and injury and poisoning. Except for neoplasms, deaths from these causes occurred among 50–54 year old Aboriginals at rates that were experienced by non‐Aboriginal people ten to 30 years later in life. These results underline special needs of the Aboriginal population that have not been adequately met by appropriate services.
A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy
Authors: Chandran GEETHA , Ahern M. J. , Smith M. , Roberts‐Thomson P. J.
Published: March 2008

Background: Scleroderma is a systemic rheumatic disorder seen in a wide range of clinical specialties.

Aims: To establish the prevalence and mortality rates of scleroderma in South Australia (SA), to determine the relative frequency and characteristics of the three principal subsets (diffuse, limited and overlap), and to examine the role of nailfold capillaroscopy in subset identification and implied prognosis.

Methods: Outpatient and discharge diagnostic indexes from five major teaching hospitals in SA were reviewed between February 1987 and November 1993. A total of 215 patients with scleroderma were identified. Case notes of 115 of these patients were reviewed in order to validate scleroderma diagnosis, and subset characteristics such as sex, mean age at diagnosis, extent of skin involvement, internal organ involvement and serology were analysed. Fifty‐two of these patients were then examined prospectively to confirm positive discharge diagnosis, and nailfold capillaroscopy was performed on these patients.

Results: The point prevalence of scleroderma in SA for 1993 was estimated to be 208/106. This figure is a conservative estimate and is higher than most other reported series. The female to male ratio was 4:1. The majority of patients had limited disease with a ratio of 6:1:1.6 limited vs diffuse vs overlap. Systemic involvement excluding the oesophageal component in limited disease was found predominantly in the diffuse group. Autoimmune serology was positive in 90% of patients, with Scl‐70 being more common in diffuse scleroderma, anti‐centromere antibody (ACA) in the limited form and anti‐ribonucleoprotein (RNP) in the overlap form. Nailfold capillaroscopy was useful in predicting disease‐subtype as capillary dilatation was observed predominantly in limited disease, and capillary dropout in diffuse disease.

Conclusions: Scleroderma is more common in SA than previously recognised. Limited disease is more common than diffuse or overlap disease, carries a better prognosis and is associated with ACA. Nailfold capillaroscopy is a useful tool in disease assessment and may provide useful diagnostic and prognostic information.
The surface glycoconjugates of parasitic protozoa: potential targets for new drugs
Authors: McConville MALCOLM J.
Published: March 2008

Protozoan parasites are the cause of many diseases in humans and their domestic livestock. Glycoconjugates (i.e. glycoproteins, glycolipids) on the cell surface of these extremely diverse and very primative eukaryotes play a crucial role in determining the specificity of the host‐parasite interaction and in protecting the parasites within their respective hosts. These molecules frequently share a common structural feature in that they are attached to the plasma membrane via a glycosylphosphatidylinositol (GPI) glycolipid. While GPI protein‐membrane anchors are ubiquitous among the eukaryotes, they are used with exceptionally high frequency in the protozoa. Some kinetopastidparasites also synthesise very high levels of GPI‐related glycolipids that are not linked to protein. Thus GPI‐anchored molecules or free GPI glycolipids tend to dominate the cell surface molecular architecture of these organisms. The highly elevated levels and specialised nature of GPI metabolism in the kinetoplastid and other parasites suggests that the GPI biosynthetic pathway might be a good target for the development of new chemotherapeutic agents. This article reviews the wide range of functions that GPI protein anchors and GPI‐related glycolipids are thought to perform in these organisms and some aspects of their biosynthesj?
Risk factors for ill‐health in a remote desert‐dwelling Aboriginal community in Western Australia
Authors: Gracey M. , Burke V. , Spargo R. M. , Beilin L. J. , Smith P. , Beilby J. , Smith R. M. , Chin C.
Published: March 2008

Abstract Background: Living in small, isolated groups may promote health for Aborigines if traditional lifestyles are followed, but overall health risks in such communities are inadequately documented.

Aim: To document health status of a remote Aboriginal community with reference to nutrition, cardiovascular risks, renal disease and infections and to identify areas where health might be improved.

Methods: All residents of a small community in the Great Sandy Desert underwent medical examinations, anthropometry and measurement of blood pressure. Investigations included cholesterol, triglycerides, glucose, insulin, creatinine, lipoprotein (a), apolipoprotein E phenotype, angiotensin‐converting enzyme genotype, urinalysis, stool microscopy (children), liver function tests and full blood examination.

Results: Children (n=26) were undernourished while 14% of adults (n=51) were underweight, 22% overweight and 40% of women and 13% of men were obese with central obesity in 90% of women and 48% of men. Fifteen per cent of the group were hypertensive. Insulin levels were increased in 55% of subjects, total cholesterol in 21% and triglycerides in 56%, while HDL was decreased in 78%. Angiotensin‐converting enzyme and apolipoprotein E typing and lipoprotein (a) did not suggest increased cardiovascular risk. Proteinuria was present in 39% of subjects, haematuria in 49% and definite or possible urinary tract infections in 30%. Faecal parasites were prevalent and a history of infections, including sexually transmitted diseases, was common.

Conclusions: Increased cardiovascular risk, nutritional disorders, renal disease and infections are major problems in this community which had relocated several years previously from a mission environment closer to western influences, including alcohol.
Risk factors for sudden unexpected cardiac death in Tasmanian men
Authors: Sexton P. T. , Jamrozik K. , Walsh J. , Parsons R.
Published: March 2008

Abstract Background: Sudden unexpected cardiac death (SUCD) accounts for approximately 25% of deaths from ischaemic heart disease (IHD) but is relatively poorly understood because of the difficulties involved in researching aetiology. Clinical differences between instances of SUCD and those cases of acute chest pain that survive long enough to be proven as myocardial infarction but are eventually fatal might reflect differences in aetiology.

Aims: To determine the risk factors for sudden unexpected cardiac death in Tasmanian men.

Methods: A population‐based case‐control method was used with the study population, an estimated 125,225 men aged 25–74 years living in the island State of Tasmania, Australia. The case group of 102 men who had a SUCD was validated using necropsy reports, hospital records and information provided by the usual general practitioner. Cases were matched with 204 community controls. Spouses or partners of eligible subjects answered a detailed questionnaire. Multivariate odds ratios (ORs) for risk factors were calculated using stepwise analysis.

Results: Risk factors measured included: smoking habit, treated hypertension, hyper‐cholesterolaemia, diabetes mellitus, family history of IHD, alcohol intake and exercise habits. Independent risk factors for SUCD were: history of diabetes mellitus (OR=4.2, 95% CI: 1.39, 12.81), current smoking status (OR=3.5, 95% CI: 1.80, 6.82), and family history of IHD (OR=2.6, 95% CI: 1.34, 4.92).

Conclusions: Some accepted risk factors for acute myocardial infarction (AMI) also predict sudden death in men with no history of coronary disease. Efforts to reduce smoking, the incidence of diabetes mellitus and mean blood pressure must be continued as SUCD is, by definition, untreatable but is potentially avoidable in many instances.