Building a pulmonary vascular service: the 12-year experience and outcomes of the Auckland pulmonary arterial hypertension clinic.

BACKGROUND AND AIMS: Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality. In New Zealand, access to PAH-specific pharmacotherapy has been restricted for economic reasons. Since 2009, access to medication has been provided by a Special Authority scheme that ensures equity of access for all patients. Management of patients with PAH in this environment poses unique challenges. This study describes outcomes of patients managed in the Auckland PAH clinic over a 12-year period. METHODS: The clinic database was interrogated. Patients were eligible for this study if they had PAH (World Health Organization (WHO) Class 1, 1' and 4) and had been managed in the clinic from the year 2000. RESULTS: One hundred and twenty-six patients (75% female, mean age at diagnosis 50) were included. Most had idiopathic PAH (37%) or PAH because of connective tissue disease (29%). At diagnosis, patients had advanced disease (median WHO Class III, 6-min walk distance 367 m and pulmonary vascular resistance of 10.6 Wood Units). Initial therapy was sildenafil in most cases. PAH-specific therapy was associated with improved WHO class and longer 6-min walk distance (P < 0.01 for both). Thirty per cent of patients were escalated to combination therapy. Survival was 91% at 1 year and 67% at 5 years. CONCLUSIONS: Despite historic difficulties with access to PAH-specific therapy, these data confirm benefits of therapy (primarily sildenafil as first-line treatment) for patients with PAH managed within the Auckland PAH clinic. Survival data are comparable with other reported cohorts.

Factors influencing the implementation of best practice in burn care in Western Australia.

AIMS AND OBJECTIVES: The study aimed to determine the factors which influence clinician behaviour and adherence to best practice when clinicians provide the initial care for paediatric burn patients admitted to a burns unit. BACKGROUND: Optimal initial care of burn patients influences morbidity and mortality. Non-burn specialist clinician adherence to best practice is influenced by previously unexplored factors. DESIGN: General inductive qualitative methods were used to explore factors which influenced clinicians providing acute pre-admission burn care for children in Western Australia. METHODS: Interviews of nineteen clinicians using standardised open-ended questions based on the Gilbert Behaviour Engineering Model were used to collect data. RESULTS: The main influencing factors identified were the telehealth service which supported practice, whilst IT issues provided challenges to clinicians. CONCLUSION: Telehealth services support clinicians when providing burn care, however IT issues are an major barrier to both best practice and accessing the telehealth service and should be optimised to support clinical care IMPACT STATEMENT: What does this paper contribute to the wider global community? It provides burn clinicians with an insight into the factors which facilitate optimal care for patients prior to transfer to burn units, as well as the barriers faced by non-burn specialist clinicians when patients initially present for care. Models of care which acknowledge these factors can help facilitate optimal patient care.

A locked knee in extension: a complication of a degenerate knee with patella alta.

We present a case of superior dislocation of the patella trapped by interlocked osteophytes. Unlike previous reports, in which the mechanism resulted from a blow to the inferior pole, it is postulated that increased load on the extensor mechanism, combined with patella alta and patellofemoral osteophytes, caused locking of the knee in extension.

An unusual complication of total knee replacement: a case report.

After total knee replacement a 57-year-old woman developed increasing pain in her left calf on exercise. This was due to erosion of the popliteal artery by a spur of cement. Removal of the spur with resection and Dacron grafting of the damaged section of the vessel cured her symptoms.

Importance of diabetic foot admissions at Middlemore Hospital.

AIMS: To investigate the costs and mortality associated with admission for diabetic foot problems. METHODS: Identification of patients by codes for diabetes and peripheral vascular disease from routinely collected hospital discharge data. Information was collected retrospectively from charts. Post discharge outcome was assessed by discussion with general practitioners. RESULTS: 357 patients accounted for 503 admissions. 11-15% of patients (n = 49) were admitted for diabetic foot problems. Patients with type 1 diabetes had shorter lengths of stay than those with type 2 diabetes, were more likely to be admitted with chronic renal failure and less likely to be admitted with ischaemic heart disease. Maori and Pacific Island patients with type 2 diabetes were admitted at a younger age than Europeans (53 (SD12) vs 56 (11) vs 69 (13) years respectively, p < 0.001). Admissions for diabetic foot problems resulted in the longest hospital stay in comparison with other causes (19 (1-184) days vs 8 (1-116)) days. Average cost per diabetic foot admission was $12,500 with a total annual cost of over $600,000. CONCLUSION: The diabetic foot is expensive and yet these costs are largely avoidable with improvement in patient education, motivation, monitoring and earlier intervention. It would be better to have in place improved community and hospital care before the predicted growth in the diabetic population associated with ageing.

Cystic fibrosis diagnosed in adult patients.

AIM: To review the presentation, diagnosis and long-term, clinical follow-up of cystic fibrosis in adult patients diagnosed in adulthood at Green Lane Hospital. METHODS: A retrospective review of the case notes of patients with cystic fibrosis diagnosed in adulthood at Green Lane Hospital or referred there for management. Information was collected on diagnostic tests, including sweat tests and genotyping. Relevant family history was documented as were spirometry results and microbial colonisation. RESULTS: Six patients conclusively fulfilled the diagnostic criteria for cystic fibrosis. There was a wide range of ages at diagnosis (18-68) and half of the patients had a positive family history. A single mutation was identified in all, but in only one of the cases was the second mutation identified. All patients had evidence of bronchopulmonary suppuration and all had retained pancreatic function. Colonisation with P aeruginosa was associated with marked impairment in lung function. CONCLUSION: The patients at Green Lane Hospital represent part of the broad-spectrum disease in adult patients diagnosed with cystic fibrosis and highlight the differences between this group and those patients diagnosed in childhood with the more classical phenotype. Patients generally have less severe lung disease and retain pancreatic function. Sweat testing is useful diagnostically but gene testing is of limited value in making the diagnosis.

Relationship between academic achievement and COMLEX-USA Level 1 performance: a multisite study.

The purpose of this study was to examine the relationship of performance on the Comprehensive Osteopathic Medical Licensing Examination (COMLEX-USA) Level 1 with academic performance at colleges accredited by the American Osteopathic Association (AOA). Eighteen (95%) of 19 AOA-accredited colleges and 2146 students (91% of those taking the June 1999 examination) met criteria and participated. Students were classified by school representatives on the basis of academic performance in the first 2 years of the curriculum. The relationships of Level 1 performance with assigned classifications and grade point averages (GPAs) were studied. Of students classified in the highest 20% academically, the Level 1 pass rate was 100%, with a mean score of 599. Of students classified in the lowest 5%, the pass rate was 63.5%, with a mean of 416.3. For 16 schools that provided GPAs, the within-school correlations between Level 1 scores and GPAs ranged from r = 0.76 to r = 0.85, with a mean correlation of r = 0.79. School representatives were also asked to indicate, for each student, whether they expected the student to pass the examination. Pass rate for students in the "sure pass" category was 98.9%; "borderline," 82.5%; and "concerns," 61.5%. Academic performance in the first 2 years of osteopathic medical school was strongly associated with performance on COMPLEX-USA Level 1. The national pass rate for this examination was similar to those in previous years, and it remains unclear why school representatives overpredicted the number of failures. Further research is needed.

Hot ash burns in the children of Western Australia: how and why they happen.

INTRODUCTION: Burns from hot ash are common in the paediatric population in Western Australia. Fifty children were admitted to the paediatric burn centre with hot ash contact burns to the feet in 2011 and 2012. It is important to examine the extent of the problem, seasonal variations, and identify those at risk to determine strategies for prevention campaigns. METHOD: Retrospective review of medical notes for all admissions to the paediatric burns unit was undertaken for 2011 and 2012. Data were collected for patient demographics, time, circumstance of injury, burn severity and treatment. RESULTS: Hot ash burns accounted for 8.6% of admissions but 16.1% of burns sustained in non-metro areas. Median age was just under 3 years, male or female. Median burn TBSA was 2%, and 44% of children required surgery. The burns were less common in summer, more common on non-school days and in children who were on camping trips away from home. DISCUSSION: Previous work has shown the value of targeted campaigns. The group for targeted prevention campaigns are the carers of very young children who go camping. Information distributed at camping shows and stores about the principles of campfire safety would reach the people at risk.

A prospective randomised clinical pilot study to compare the effectiveness of Biobrane® synthetic wound dressing, with or without autologous cell suspension, to the local standard treatment regimen in paediatric scald injuries.

BACKGROUND: Scald is the most common cause of burn in children in Australia. The time taken by the burn wound to heal impacts on scar outcome. Commonly scald injuries are treated conservatively; in our unit the practice is that if healing does not occur within 10 days, surgery is used to aid healing with the aim of improving scar outcome. This randomised controlled pilot study compares early treatment regimens to facilitate tissue salvage and reduce the incidence of definitive surgery at 10 days following scald injury. METHODS: All paediatric patients with partial thickness scald injury were clinically assessed between July 1, 2009 and June 30, 2010. A burn of 2% TBSAB or more and deemed not to heal within 10 days, were considered for the trial. These patients were randomised to one of three treatment arms: the local standard treatment (Intrasite™, Acticoat™ and Duoderm(®) dressings every 2-3 days) with surgery at 10 days, Biobrane(®) only or Biobrane(®) and autologous cell suspension using the ReCell(®) kit. The primary outcome was surgery performed after 10 days; secondary outcomes were rates of healing, pain experienced, and scar outcomes. RESULTS: 15% of scald presentations in the 12 month period met the eligibility criteria. 13 patients were recruited into the pilot study; early intervention was associated with a decreased time to healing with fewer dressing changes, less pain and better scar outcomes. CONCLUSION: Investment of surgical resources in the acute stages within 4 days of injury saved on nursing time, dressing, analgesic and scar management costs.

The timing of femoral component insertion in cemented hip arthroplasty.

The time to insertion of the femoral component into a cemented medullary cavity during total hip arthroplasty was reviewed. A survey of current practice among a group of surgeons was undertaken, and reproducible model of femoral cement penetration was developed. Under standard conditions, this showed maximum penetration of Vacu-Mix Plus CMW1 gentamicin cement (DePuy, Leeds, UK) at 3 minutes.

Induced sputum and bronchoscopy in the diagnosis of pulmonary tuberculosis.

BACKGROUND: Previous studies suggest that bronchoscopy and a single induced sputum sample are equally effective for diagnosing pulmonary tuberculosis. METHODS: In a prospective study of subjects with possibly active pulmonary tuberculosis, the diagnostic yield of three induced sputum tests was compared with that of bronchoscopy. Subjects either produced no sputum or (acid fast) smear negative sputum. Bronchoscopy was only performed if at least two induced sputum samples were smear negative. RESULTS: Of 129 subjects who completed all tests, 27 (21%) had smear negative and culture positive specimens, 14 (52%) on bronchoscopy and 26 (96%) on induced sputum (p<0.005). One patient was culture positive on bronchoscopy alone compared with 13 on induced sputum alone; 13 were culture positive on both tests. Induced sputum positivity was strikingly more prevalent when chest radiographic appearances showed any features of active tuberculosis (20/63, 32%) than when appearances suggested inactivity (1/44, 2%; p<0.005). Induced sputum costs were about one third those of bronchoscopy, and the ratio of costs of the two tests per case of tuberculosis diagnosed could be as much as 1:6. CONCLUSIONS: In subjects investigated for possibly active or inactive tuberculosis who produce no sputum or have smear negative sputum, the most cost effective strategy is to perform three induced sputum tests without bronchoscopy. Induced sputum testing carries a high risk of nosocomial tuberculosis unless performed in respiratory isolation conditions. The cost benefits shown could be lost if risk management measures are not observed.

Poliomyelitis in intraspinally inoculated poliovirus receptor transgenic mice.

Mice transgenic with the human poliovirus receptor gene develop clinical signs and neuropathology similar to those of human poliomyelitis when neurovirulent polioviruses are inoculated into the central nervous system (CNS). Factors contributing to disease severity and the frequencies of paralysis and mortality include the poliovirus strain, dose, and gender of the mouse inoculated. The more neurovirulent the virus, as defined by monkey challenge results, the higher the rate of paralysis, mortality, and severity of disease. Also, the time to disease onset is shorter for more neurovirulent viruses. Male mice are more susceptible to polioviruses than females. TGM-PRG-3 mice have a 10-fold higher transgene copy number and produce 3-fold more receptor RNA and protein levels in the CNS than TGM-PRG-1 mice. CNS inoculations with type III polioviruses differing in relative neurovirulence show that these mouse lines are similar in disease frequency and severity, demonstrating that differences in receptor gene dosage and concomitant receptor abundance do not affect susceptibility to infection. However, there is a difference in the rate of accumulation of clinical signs. The time to onset of disease is shorter for TGM-PRG-3 than TGM-PRG-1 mice. Thus, receptor dosage affects the rate of appearance of poliomyelitis in these mice.

Growth in the presence of salicylate increases fluoroquinolone resistance in Staphylococcus aureus.

Salicylate and acetylsalicylate slightly increased fluoroquinolone resistance in ciprofloxacin-susceptible and -resistant Staphylococcus aureus. Salicylate allowed a greater number of cells from ciprofloxacin-susceptible and -resistant strains to survive on high fluoroquinolone concentrations. Salicylate also increased the frequency with which a susceptible strain mutated to become more resistant to ciprofloxacin.