Queensland Paediatric Cardiac Service: Lessons from evolution to a unified service in a tertiary children's hospital.

We describe a complex change process for the paediatric cardiac service in Queensland that involved transitioning the service out of an essentially adult hospital into one of two children's hospitals in Brisbane. This initial step was complex as the governance was changed from Queensland Health to Mater Health, an independent faith-based organisation who became the new employer. Six years later, the service was again transitioned; this time to the newly constructed Queensland Children's Hospital, with a Hospital and Health Services Board as the employer under the aegis of Queensland Health. This was a complex journey. As with all change processes there was resistance to change on the part of some individuals. Five years on from the second major change, the service is settled, has an excellent workplace culture, has excellent clinical outcomes and has become research intensive.

Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles.

Introduction: We describe the case of a 16-year old male with cystic fiborosis (CF) who presented with an osteosarcoma of his right distal tibia. Case Report: Treatment consisted of neoadjuvant chemotherapy of cisplatin, doxorubicin and high dose methotrexate followed by distal tibial resection and free fibula flap reconstruction and consolidation chemotherapy. Treatment was complicated by a pulmonary exacerbation, where Pseudomonas aeruginosa (PsA) and Staphylococcus aureus were grown on sputum culture which was treated with a 2-week course of intravenous piptazobactam and tobramycin. Mycobacterium intracellulare and Mycobacterium abscessus were also cultured following commencement of chemotherapy and successfully treated with a 6-month course of oral azithromycin, ethambutol, and moxifloxacin along with a 1-month course of inhaled amikacin. Pulmonary function improved during his treatment from baseline FEV1 of 3.8 l (93.9%) to 4.15 l (102.3% predicted) whilst nutritional status remained stable. Discussion: The combination of CF and osteosarcoma is rare with only one previous case reported (1). Our case is instructive as the patient faced the challenge of chronic PsA and the first reported culturing and successful treatment of non-tuberculous mycobacterium (NTM) during chemotherapy. Fatal outcomes have been reported previously for CF patients during immunosuppression (2). In concordance with our findings, a recent report noted an improvement in respiratory function in a child treated for leukemia (3). The anti-inflammatory nature of some chemotherapy agents could be responsible for the observed clinical improvement in CF with low dose methotrexate having been shown to increase FEV1 in adolescents with advanced CF (4). Whilst doxorubicin could improve pulmonary outcomes through increased total cellular CFTR protein expression and CFTR associated chloride secretion (5). It is hypothesized that the improved pulmonary function in patients with CF who require chemotherapy could be due to increased production of Multi-Drug Resistance Proteins (MDR) and Multi-Drug Resistant Associated Proteins (MRP) that may complement the depleted CFTR protein (6). Concluding Remarks: We report the well-tolerated management of osteosarcoma in a patient with CF including the first reported identification and eradication of NTM during chemotherapy. The observed positive pulmonary outcome following chemotherapy highlights several potential cellular mechanisms that deserve to be explored.

Necrotizing pneumonia: an emerging problem in children?

BACKGROUND: In children, necrotizing pneumonia (NP) is an uncommon, severe complication of pneumonia. It is characterized by destruction of the underlying lung parenchyma resulting in multiple small, thin-walled cavities and is often accompanied by empyema and bronchopleural fistulae. REVIEW: NP in children was first reported in children in 1994, and since then there has been a gradual increase in cases, which is partially explained by greater physician awareness and use of contrast computed tomography (CT) scans, and by temporal changes in circulating respiratory pathogens and antibiotic prescribing. The most common pathogens detected in children with NP are pneumococci and Staphylococcus aureus. The underlying disease mechanisms are poorly understood, but likely relate to multiple host susceptibility and bacterial virulence factors, with viral-bacterial interactions also possibly having a role. Most cases are in previously healthy young children who, despite adequate antibiotic therapy for bacterial pneumonia, remain febrile and unwell. Many also have evidence of pleural effusion, empyema, or pyopneumothorax, which has undergone drainage or surgical intervention without clinical improvement. The diagnosis is generally made by chest imaging, with CT scans being the most sensitive, showing loss of normal pulmonary architecture, decreased parenchymal enhancement and multiple thin-walled cavities. Blood culture and culture and molecular testing of pleural fluid provide a microbiologic diagnosis in as many as 50% of cases. Prolonged antibiotics, draining pleural fluid and gas that causes mass effects, and maintaining ventilation, circulation, nutrition, fluid, and electrolyte balance are critical components of therapy. Despite its serious nature, death is uncommon, with good clinical, radiographic and functional recovery achieved in the 5-6 months following diagnosis. Increased knowledge of NP's pathogenesis will assist more rapid diagnosis and improve treatment and, ultimately, prevention. CONCLUSION: It is important to consider that our understanding of NP is limited to individual case reports or small case series, and treatment data from randomized-controlled trials are lacking. Furthermore, case series are retrospective and usually confined to single centers. Consequently, these studies may not be representative of patients in other locations, especially when allowing for temporal changes in pathogen behaviour and differences in immunization schedules and antibiotic prescribing practices.

Giant Gastric Bezoar Complicating Congenital Esophageal Atresia Repaired by Gastric Interposition-A Case Report.

We describe a giant gastric phytobezoar in a child with repaired congenital esophageal atresia. At age two, a gastric interposition (pull-up) procedure was performed for severe and recurrent esophageal strictures. For 12 months post-gastric interposition, he experienced frequent respiratory illnesses requiring hospital admissions but it was not initially appreciated that these episodes were likely secondary to recurrent aspiration from a gastric bezoar with "spill-over" aspiration.

A pioneer of Australian paediatrics: Dr Henry Edward Brown (1858-1931).

The emergence of paediatrics as a specialty in Australian medicine dates from the last two decades of the 19th century. Among the pioneers of pre-Federation paediatrics, we include Dr Henry Edward Brown (1858-1931), an Irish-born physician and surgeon who became the first paediatrician to practise in the northern half of the Australian continent. In 1885, he was appointed as the medical superintendent of the Rockhampton Children's Hospital, itself a pioneer institution in the care of sick and injured children. Dr H.E. Brown also served as medical officer of health concurrently in three Queensland shires. He was a leader in the literary and sporting life of the busy port town of Rockhampton and a scholar and significant philanthropist in the domain of French literature. His life was and remains an exemplar of a class of pre-Federation paediatricians who established the ethos of clinical and societal service, which remains as a core feature of the speciality discipline of paediatrics in the 21st century.

Barium aspiration in an infant: a case report and review of management.

We describe a case of bilateral inhalation of barium in an infant following a barium swallow for investigation of dusky spells associated with feeds. A bronchoscopy subsequently revealed the presence of a mid-tracheal tracheo-esophageal cleft. To date, little has been reported on barium aspiration in children and there is no consensus for management. We review the literature on barium aspiration, its consequences, and make recommendations for management.

A case for early bronchoscopic airway assessment after disc battery ingestion.

Disc battery ingestion in children is becoming increasingly common with the proliferation of small battery-powered electronic devices. In the case of esophageal impaction, the likelihood and severity of complications are proportionate to the time between ingestion and removal. Tracheo-esophageal fistulae (TOF) are a recognized complication and can be life-threatening. We describe an interesting case of disc battery ingestion with delayed recognition of a TOF. We document the tracheal mucosal healing process of a large airway defect and describe the role of bronchoscopy in guiding the timing of surgical intervention. This case highlights the important role of early bronchoscopic assessment in management of these patients.

Cough and exhaled nitric oxide levels: what happens with exercise?

Cough associated with exertion is often used as a surrogate marker of asthma. However, to date there are no studies that have objectively measured cough in association with exercise in children. Our primary aim was to examine whether children with a pre-existing cough have an increase in cough frequency during and post-exercise. We hypothesized that children with any coughing illness will have an increase in cough frequency post-exercise regardless of the presence of exercise-induced broncho-constriction (EIB) or atopy. In addition, we hypothesized that Fractional exhaled nitric oxide (FeNO) levels decreases post-exercise regardless of the presence of EIB or atopy. Children with chronic cough and a control group without cough undertook an exercise challenge, FeNO measurements and a skin prick test, and wore a 24-h voice recorder to objectively measure cough frequency. The association between recorded cough frequency, exercise, atopy, and presence of EIB was tested. We also determined if the change in FeNO post exercise related to atopy or EIB. Of the 50 children recruited (35 with cough, 15 control), 7 had EIB. Children with cough had a significant increase in cough counts (median 7.0, inter-quartile ranges, 0.5, 24.5) compared to controls (2.0, IQR 0, 5.0, p = 0.028) post-exercise. Presence of atopy or EIB did not influence cough frequency. FeNO level was significantly lower post-exercise in both groups but the change was not influenced by atopy or EIB. Cough post-exertion is likely a generic response in children with a current cough. FeNO level decreases post-exercise irrespective of the presence of atopy or EIB. A larger study is necessary confirm or refute our findings.

Understood consent versus informed consent: a new paradigm for obtaining consent for pediatric research studies.

All too often the informed consent process is viewed by members of research teams as a challenge of getting a parent or young person's signature on a form. Informed consent is, however, much more than a signed form. Rather, it is a process, often iterative, in which the parent or young person is given sufficient information about a study in order that they can make a truly informed decision about participation. Substantial effort is required in producing appropriately formatted and readable documents using plain language at about Grade 6 or 12-year old reading level. Achieving truly understood consent involves the researcher spending significant one-on-one time with the parent or young person explaining in simple language what is proposed and then using so-called repeat-back techniques to test the understanding of the participants. This is critically important if the research involves randomization to different treatments or use of a placebo arm and, in particular if the research involves more than minimal risk.

Wireless telemedicine for the delivery of specialist paediatric services to the bedside.

A mobile interactive online health system was used to conduct virtual ward rounds at a regional hospital which had no specialist paediatrician. The system was wireless, which allowed telepaediatric services to be delivered direct to the bedside. Between December 2004 and May 2005, 43 virtual ward rounds were coordinated between specialists based in Brisbane and local staff at the Gladstone Hospital. Eighty-six consultations were provided for 64 patients. The most common conditions included asthma (27%), chest infections (12%), gastroenteritis (10%) and urinary tract infections (10%). In the majority of cases, there were partial (67%) or complete changes (11%) in the clinical management of patients. Specialist services were offered by a team of 13 clinicians at the Royal Children's Hospital: 10 general paediatricians, two physiotherapists and one registered nurse. Feedback from all consultants involved in the service and local staff in Gladstone was extremely positive. In 43 videoconference calls there were three technical problems, probably due to an intermittent mains power supply at the regional hospital. There appears to be potential for other rural and regional hospitals to adopt this model of service delivery.

Craniopagus: second Brisbane case. Case report.

Craniopagus is a rare and intriguing condition with an incidence of one in 2.5 million births. The chance of a neurosurgeon seeing a case in a working lifetime is unlikely. The chances of two cases from the same community within 12 months are remote in the extreme. The authors present a second case of craniopagus born and separated in Brisbane, Australia, in 2001 and discuss the intricacies of surgical separation and the lessons learned.

The family costs of attending hospital outpatient appointments via videoconference and in person.

We compared the costs incurred by families attending outpatient appointments at the Royal Children's Hospital (RCH) in Brisbane with those incurred by families who had a consultation via videoconference in their regional area. In each category 200 families were interviewed. The median time spent travelling for videoconferences was 30 min compared with 80 min for face-to-face appointments. Families interviewed in the outpatient department had travelled a median distance of 70 km, while those who had a videoconference at the local hospital had travelled only 20 km. It cost these families much more to attend an appointment at the RCH than to attend a videoconference. Ninety-six per cent of families (193) reported at least one of the following types of expense: 150 families had expenses related to parking (median A 10 dollars), 156 had fuel expenses (median A 10 dollars) and 122 reported costs related to meals purchased at the RCH (median A 10 dollars). Only 21 families who had their appointment via local videoconference reported any additional costs. Specialist appointments via videoconference were a more convenient and cheaper option for families living in regional areas of Queensland than the conventional method of attending outpatient appointments at the specialist hospital in Brisbane.

Separation of craniopagus joined at the occiput. Case report.

Siamese or conjoined twins have intrigued both the physician and layperson for centuries. The craniopagus type (joined at the head) is exceedingly rare, with an incidence of one in 2.5 million births. Most clinicians never see a case of craniopagus, and those who do rarely see more than one. The authors present a case of the craniopagus type of conjoined twins born and recently separated in Brisbane, Australia. The prenatal diagnosis, subsequent investigations, separation, and outcome are presented.

A comparison of telepaediatric activity at two regional hospitals in Queensland.

We conducted a 15-month feasibility study of telepaediatrics. A novel service was offered to two hospitals in Queensland (Mackay and Hervey Bay). We used data from all other hospitals throughout the state as the control group. Although both intervention hospitals were provided with the same service, the telepaediatric activity generated and the effect on admissions and outpatient activity were markedly different. There was a significant decrease in the number of patient admissions to Brisbane from the Mackay region. In addition, there was an increase in the number of Mackay patients treated locally (as outpatients). In contrast, little change was observed in Hervey Bay. We assessed whether the observed differences between the two hospitals were due to various factors which influenced the use of the telepaediatric service. These factors included the method of screening patients before transfer to the tertiary centre and the physical distance between each facility and the tertiary centre. We believe that the screening method used for patient referrals was the most important determinant of the use of the telepaediatric service.

A comparison of telepaediatric activity at two regional hospitals in Queensland.

We conducted a 15-month feasibility study of telepaediatrics. A novel service was offered to two hospitals in Queensland (Mackay and Hervey Bay). We used data from all other hospitals throughout the state as the control group. Although both intervention hospitals were provided with the same service, the telepaediatric activity generated and the effect on admissions and outpatient activity were markedly different. There was a significant decrease in the number of patient admissions to Brisbane from the Mackay region. In addition, there was an increase in the number of Mackay patients treated locally (as outpatients). In contrast, little change was observed in Hervey Bay. We assessed whether the observed differences between the two hospitals were due to various factors which influenced the use of the telepaediatric service. These factors included the method of screening patients before transfer to the tertiary centre and the physical distance between each facility and the tertiary centre. We believe that the screening method used for patient referrals was the most important determinant of the use of the telepaediatric service.