South Eastern Sydney and Illawarra Area Health Service
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Research Activity 2004/05

 
Research project Institution on whose behalf the research is being undertaken Researcher Description

Allied Health

Dietetics

Evaluation of a family-based weight management program in a paediatric outpatient setting. Sydney Children’s Hospital Foundation. Katz T,
Aitken M,
Jackson R,
Neville K.
This study aims to determine whether group education of parents of overweight children
by a dietician and a clinical psychologist, is a successful management strategy when compared with traditional one-on-one consultations by a dietician alone.

Occupational Therapy

Accessible toilet facilities for children and adolescents: enhancing the evidence base. Sydney Children’s Hospital Foundation. Kingsley A, Croudace S. Study to determine whether toilets built for use by those with a physical disability are accessible to the majority of children and adolescents with physical disabilities aged 4 to 18 years.

Physiotherapy

Inter-rater reliability of physiotherapists using the Pirani assessment of congenital talipes equinovarus. Sydney Children's Hospital (SCH). Harvey N,
Sims S,
Mudge A.
Study to ascertain the reliability of a club foot assessment tool when used between physiotherapists, and / or by one physiotherapist on more than one occasion.

Psychology

Social skills groups for children with traumatic brain injury. Motor Accidents Authority of NSW. Brookes N,
Epps A.
Development and evaluation of a school-based social skills group intervention for children with social difficulties following a traumatic brain injury.
Frequency of emotional and behavioural symptomatology in children with acquired brain
injury at a specialist paediatric unit.
University of Sydney;
SCH.
Soo C,
Tate R,
Brookes N.
Examination of the frequency and nature of behavioural and emotional problems following acquired brain injury in an Australian paediatric population
Predictors of sibling adjustment following a child traumatic brain injury. University of Sydney; SCH. Lah S,
Sambuco M, Brookes N,
Hunt C.
Study identifying core factors influencing sibling adjustment in families of children with traumatic brain injury.
The development of a sleep disturbances scale for children aged 2 to 5 years. Charles Sturt University; University of NSW School of Psychology; SCH. Dryer R,
Kiernan M,
Betts G, Teng A, MacGregor F,  Williamson B.
This study aims to develop a psychometrically valid questionnaire to detect sleep disturbances in young children, and to examine the relationship between parent behaviours used to settle children to sleep and sleep disturbance.
The development of a scale to assess the impact of chronic paediatric pain on school functioning SCH. Piira T, Betts G, McInerney M, Chan E,
Crawford M, Workman T, Champion D.
This study aims to develop the first psychometrically reliable and valid school function questionnaire for chronic pain patients.
The cognitive effects of early onset versus late onset juvenile insulin dependent diabetes mellitus. SCH. Betts G, Verge C,
Lynch R,
Bennett N, Frilingos M.
This study compares children with early versus late onset diabetes on cognitive function and follows them longitudinally for three years. It also examines the potentially cumulative and interactive effects of early versus late onset dependent diabetes and blood glucose control (high versus low levels) over time.

Centre for Children's Cancer & Blood Disorders

Biology study for Neuroblastoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Neuroblastoma is a tumour of nerve cells usually found in the abdomen, chest or neck of young children. The research of this study is to define the characteristics of this disease and to predict the best therapy for each child.
A study using drugs and stem cell transplantation in children with juvenile myelomonocytic leukemia (JMML). Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
JMML is a rare form of leukemia usually found
in children no older than 5 years. The purpose
of this study is to find out what effects a new treatment program has on JMML.
Treatment of newly diagnosed lymphoblastic lymphoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Lymphoblastic lymphoma is a cancer of the lymph system. This study compares four chemotherapy treatments.
Protocol for treatment of children with hepatoblastoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study uses a drug which could help protect normal cells from chemotherapy and stop some of the side effects that often occur following the chemotherapy treatment of hepatoblastoma.
Chemotherapy, surgery and radiation therapy for children with non-metastatic brain tumour. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Non-metastatic brain tumour is a type of cancer in the central nervous system (brain and spinal cord). This research investigates if this treatment will help improve the cure rate for young children with brain tumour that has not spread beyond the primary site.
Therapy for children and adolescents with newly diagnosed intermediate risk Hodgkin Disease. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study uses drugs (chemotherapy) and high-dose x-rays (radiation therapy) and examines the negative effects from the treatment later in life.
Treatment for children with refractory meningeal malignancies. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
The purpose of this study is to find out what effects an anti-cancer drug has when given directly into the cerebrospinal fluid in children and adolescents with neoplastic meningitis (cancer that has spread to the lining of the brain and spinal cord).
Treatment of advanced-stage anaplastic large cell lymphoma (ALCL). Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study is designed for patients less than 21 years old with ALCL, a cancer of the lymphatic tissue.
Therapy in the treatment of children with low and intermediate risk germ cell tumours. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Germ cell tumours are a type of cancer found
in the ovaries or testes. This tumour may also
be found in other areas of the body such as the brain, chest or abdomen.
Chemotherapy in children with recurrent/refractory
B-cell non-Hodgkin lymphoma and B-cell acute lymphoblastic leukemia.
Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study involves a new drug in an attempt to improve the survival of patients with these types of cancer that have not responded well to standard treatment.
Study for children with refractory/relapsed Hodgkin Disease. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
The major goal of this study is to look at how patients with Hodgkin Disease that has come back and/or become resistant to standard treatment, respond to a new treatment.
National Wilms tumour late effects study. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
The purpose of this research is to learn more about the causes of Wilms tumour and the effects of treatment for Wilms tumour. Children are evaluated on a long-term basis to monitor long-term effects from their treatment.
Study involving children with chronic myelogenous leukemia. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Chronic myelogenous leukemia is a specific kind of leukemia (blood cancer) that is associated with the presence of an abnormal chromosome. This study tests the safety and effectiveness of
a new drug for this cancer.
A study for collecting and/or banking research specimens. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Cancer samples are collected and kept in a tissue bank with the aim of learning more about cancer and how to prevent, stop it from spreading to other parts of the body, and cure it.
Surgery for low-risk neuroblastoma. Children’s Oncology Group USA.  Marshall G,
Cohn R,
Russell S,
O’Brien T.
A study to determine if low risk patients can be treated with surgery only, avoiding the side effects of chemotherapy, as recent experience indicates that chemotherapy may not be needed.
Treatment for infants and children with intermediate risk neuroblastoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study involves anti-cancer medicines (chemotherapy) with the purpose of investigating if less anti-cancer treatment can be used and still cure most children with this cancer.
Chemotherapy for astrocytoma in children less than ten years old. Children’s Oncology Group USA.  Marshall G,
Cohn R,
Russell S,
O’Brien T.
This brain tumour study investigates whether chemotherapy can substitute radiation in children in whom the risk of side effects from radiation is higher.
Study for patients with intermediate risk rhabdomyosarcoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Rhabdomyosarcoma is a type of soft tissue tumour. This study determines if substituting a new drug in place of a currently used drug will improve the cure rate.
Study for children with recurrent or progressive brain tumours. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study tests an experimental drug to find
out its effect in patients with recurrent or progressive (the tumour has come back or
has become worse after standard therapy) childhood brain tumours.
Trial for children with Ewings sarcoma and related tumours. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Ewings sarcoma is a type of cancerous tumour that forms in the bone or soft connective tissues. These tumours spread quickly to other areas of the body including the lungs, other bones, bone marrow and the brain. This research is being undertaken to determine if a new method can cure more patients.
Therapy for newly diagnosed low-risk patients with embryonal/botryoid rhabdomyosarcoma. Children’s Oncology Group USA.  Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study determines the most effective and least toxic chemotherapy for patients with a
low risk of the disease recurring.
Therapy for selected children and adolescents with newly diagnosed rhabdomyosarcoma. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study involves metastatic tumours, that is a tumour that has already invaded other tissues in the body.
A study for the treatment of children with refractory or relapsed solid tumours. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study examines the effects that the study drug has on certain tumours that occur in children and young adults.
Study in children with refractory leukemias. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study tests the use of a new drug for the treatment of children with refractory leukemia - leukemia that does not respond to standard treatment.
Trial for children with solid tumours. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study involves a type of cancer which has continued to grow despite treatment with standard therapy.
Treatment of patients with brain tumours. St Jude Hospital, Memphis, USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study uses new molecular tests to analyse tumours in the hope of coming up with tumour “profiles” that can predict how tumours might respond to therapy. Using this information, physicians may be able to tailor future therapies for individual tumour types, which may increase the possibility of treatment success
Australian study of causes of acute lymphoblastic leukemia in children (AUS ALL). Telethon Institute of Childhood Health Research, Western Australia. Marshall G,
Cohn R,
Russell S,
O’Brien T.
The aim of the study is to investigate genetic, dietary and environmental causes of acute lymphoblastic leukemia.
Dual combined umbilical cord blood transplantation in life threatening diseases. SCH. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study investigates the effectiveness of combining two cord blood units for use in transplant for children with life-threatening diseases of the blood and bone marrow.
Study of acute lymphoblastic leukemia in children (ALL STUDY 8 PILOT). Australian & New Zealand Children’s Haematology/Oncology Group. Marshall G,
Cohn R,
Russell S,
O’Brien T.
The purpose of this study is to identify children who have a lower chance of cure with standard acute lymphoblastic leukemia treatments and use a new intensified treatment (radiotherapy and chemotherapy) to attempt to increase their chances of long term cure. The study also uses laboratory tests to detect very small amounts of leukaemia .
Treatment of lymphoma/leukemia. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
This study examines if a new treatment is better than treatments used in the past and to find out the side effects of treatment. Follow-up only.
National Wilms tumour study 5: therapeutic trial and biology study. Children’s Oncology Group USA. Marshall G,
Cohn R,
Russell S,
O’Brien T.
Wilms tumour is a rare tumour that occurs in children and is treated with a combination of surgery, chemotherapy and in some cases radiotherapy. Follow-up only.
Evaluation of the therapeutic efficacy and mechanisms of a novel treatment for childhood neuro-blastoma combining Histone Deacetylase Inhibitors, 13-cis retinoic acid and interferon-alpha. Sydney Children’s Hospital Foundation. Marshall G, Nguyen T. A laboratory-based study looking at a potential new therapy for this form of childhood developmental tumour.
Complementary and alternative therapies in paediatric oncology: identifying psychological correlates of use. Sydney Children’s Hospital Foundation. Goodenough B. Exploring predictors of parent's use and interest in non-conventional treatments for children with cancer.
Children’s understanding of pain, fatigue and nausea: a priority for symptom measurement
and management.
University of NSW.  Goodenough B. Investigating children’s understanding of the causes of their symptoms and relationship to intensity ratings.
Long-term adjustment in survivors of childhood cancer and their parents and siblings. University of NSW; SCH. Suneson B, Goodenough B, Cohn R,
Johnston K.
Documenting prevalence of stress and thriving outcomes after childhood cancer cure in family members.
KidCom: linking home and hospital. Baxter Medical Trust. Goodenough B, Cohn R. Field trial of webcam-based communication for reducing psychosocial distress in families separated by hospitalisation.
Hoping and coping with cancer: cognitive behavioural therapy for children and adolescents with leukaemia. University of Sydney; NHMRC. Pritchard M, Butow P,
Stevens M, Goodenough B, Cohn R.
Investigating the effectiveness of an individual cognitive behavioural program for improving children’s illness perceptions, coping and treatment adherence in acute lymphoblastic leukaemia.
Paediatric oncology end-of-life context: the basis and impact of the decision to die at home. SCH. Drew D, Goodenough B. Retrospective investigation of decision-making and palliative care experienced in parents who have lost a child to cancer.
Telephone support group for bereaved parents. SCH. Nair K, Goodenough B. Evaluating the efficacy and acceptability of post-bereavement support by teleconferencing to isolated families.
Measuring nausea in children 4-7 years of age. SCH. Senner A,
Crisp J.
Eliciting descriptions of nausea from children, their parents and health care professionals as the first stage of developing a measurement tool for children in this age group undergoing treatment for cancer.
A comparison of the survival rates of all adolescents and young adults diagnosed with cancer in NSW between 1993 and 2001, in relation to whether they were treated in an adult or paediatric facility. Cancer Council NSW; SCH. Senner A, Marshall G, O’Connell D, Carroll S,
O’Brien T.
Comparing the survival statistics of adolescents with cancer by whether they were treated in an adult or paediatric facility.

Endocrinology

Pathophysiology and prevention of post-operative hyponatraemia in children. Sydney Children’s Hospital Foundation. Walker J,
Neville K, Sandeman N.
Hyponatraemia, or low blood salt levels, are common in children and adults in hospital, particularly after surgery. This prospective randomised study is examining the hormonal and biochemical changes that may predispose some children to developing this problem.

Emergency

Screening for urinary tract infection in children. SCH. Wilson L,
O’Meara M.
Identification and implementation of the most timely and cost-effective method for screening urine samples for possible infection.
Child protection assessment for all infants presenting with fractures. SCH. Pennington T,
Zwi K, Tzioumi D, Zordan R, O’Meara M,
Moran K.
Assessment of a strategy to improve the rate
of detection of possible non-accidental injury
in infants presenting to the Emergency Department.

Executive Unit

Cultural diversity, parenting practices and health care. University of Western Sydney; SESIAHS Multicultural Health Unit. Chalmers S, White L. A study of cultural mismatches between parents/carers and health professionals.
Culturally specific expectations for acute health services in South East Health Multicultural Health Unit. SESIAHS. Lissing J, Chalmers S. Attempt to broaden current knowledge on how choices made by consumers of child health services are influenced by ethnicity.
Negotiating the challenge of cultural diversity in children’s health care: the Australian context. Australian Research Council. Chalmers S,
Ang I, Mason J,
Choucair S,
White L, Lord B,
Eastwood J.
This project is mapping the range of cultural practices in health service provision, providing culturally appropriate and relevant data to inform policy, and the planning and resourcing
of health services for parents/carers in NSW.
Anchoring preventive health care to positive learning: an exploration of local methods of organising and improving clinical practices. Australian Research Council. Iedema R, Braithwaite J, White L,
Williams K,
Sorensen R.
This research explores interaction of clinical-professional teams to assess whether these display practical vigilance, mutual attentiveness and innovative flexibility.

Gastroenterology

Utility of stool markers in diagnosis of organic disease in children with gastrointestinal symptoms. Sydney Children’s Hospital Foundation. Day A,
Sidler M,
Leach S.
A prospective study focusing on non-invasive markers of gut inflammation in children presenting with gut symptoms.
Randomised, double-blind, placebo-controlled, cross-over trial of VSL#3 in children with Crohn’s disease. Sydney Children’s Hospital Foundation.  Day A, Mitchell H,
Lemberg A.
 
Study to assess the role of probiotics in active Crohn’s disease.
Long-term trial of VSL#3 in Crohn’s disease in children in remission. SCH. Day A,
Lemberg A,
Mitchell H.
Study to assess the role of probiotics in the maintenance of remission in quiescent Crohn’s disease.
Evaluation of inflammatory markers in children with gastrointestinal symptoms. SCH. Day A. Examination of novel markers of inflammation
in children with inflammatory bowel disease.
Do enteral feeds alter intestinal flora in children with Crohn’s disease? SCH. Day A. Prospective study to determine the effects of enteral feeds upon the intestinal flora in children with Crohn’s disease.
Natalizumab in adolescents with Crohn’s disease. SCH. Day A,
Bohane T.
International multi-centre study evaluating
the safety and efficacy of Natalizumab in adolescents.
Nationwide assessment of disease-related knowledge in children with Crohn’s disease and
their parents.
The Australian Crohn’s and Colitis Association; SCH. Day A. Surveying children and their families to determine the level of knowledge about the illness.
The role of mucous-associated bacteria in the gut. SCH. Day A,
Mitchell J,
Zheng L.
A detailed examination of the bacteria living in the mucous layer of the gut, and the potential relevance of these organisms to gut inflammation.
Validation of S100 A12 as a faecal marker of gut inflammation. SCH. Day A,
Leach S,
deJong N.
A study establishing and validating a novel faecal test of gut inflammation in children.
S100 proteins in family members of children with inflammatory bowel disease. SCH. Day A,
Leach S,
Shalloo N.
A prospective study evaluating levels of key faecal proteins in siblings and parents of children with known inflammatory bowel disease.

General medicine

Australian Paediatric Surveillance Unit Study: Haemoglobinopathies (excluding carrier states). SCH. Emder P,
Argent E,
Mowat D, Sachdev R, Russell S, Monagle, Stone C,
Zeigler D, Peterson T.
A study designed to determine the incidence of these diagnoses in the Australian community.
A comparative study of blood sample collection from peripheral intravenous cannula device versus standard venipuncture. Sydney Children’s Hospital Foundation. Brydon M,
Neville K,
Katf H,
Cameron P.
This study is to assess the reliability of basic clinical chemistry and haematology tests taken from indwelling intravenous lines, in order to potentially save paediatric patients the need for a separate blood test.

Immunology and infectious diseases

Severe combined immunodeficiency: a national surveillance study. Australian Perinatal Surveillance Unit, NSW Health. Yee A, Ting S,
Elliott E, Ziegler J.
Surveillance of the incidence of severe combined immunodeficiency in Australia
The Australian candidaemia study: a population-based laboratory surveillance. The Australian Candidemia Study Group. Palasanthiran P. This study will describe the epidemiology
(incidence, distribution, control and prevention) of candidaemia in adult and paediatric patients throughout Australia.
Surveillance of severe food allergy in Australian children. CSL Limited; Australian Perinatal Surveillance Unit, NSW Health. Dosen A,
Ziegler J,
Kemp A, Ruhno J, Gold M, Loh R, Peake J.
Study of the epidemiology (incidence, distribution, control and prevention) of allergic reactions to food requiring hospital treatment, the causative foods, risk factors, and immediate and long-term management.
Congenital cytomegalovirus surveillance study. Australian Perinatal Surveillance Unit , NSW Health. Rawlinson W, Palasanthiran P, et al. Surveillance study of the incidence of congenital cytomegalovirus Australia-wide.
Surveillance study of non-tuberculous mycobacterial (NTM) infection in children. Australian Perinatal Surveillance Unit, NSW Health. Palasanthiran P, Best E, Blyth C, et al. Surveillance study of the incidence of non-tuberculous mycobacterial (NTM) infections in children Australia-wide.
Study to investigate the cause of an inherited disorder characterised by liver disease and immune deficiency. Children’s Hospital at Westmead; SCH. Lindeman R, Roscioli T,
Kirk E, Buckley M, Ziegler J.
Identification of the gene which causes vaso-occlusive disease with immunodificiency (VODI).
Genetics of Kawasaki Disease. University of Western Australia; SCH. Burgner D, Palasanthiran P, Ziegler J. Study of genetic host factors predisposing to Kawasaki’s Disease.
Immune responses of pneumococcal vaccination. Sydney Children’s Hospital Foundation; Canberra Hospital. Ziegler J, Palasanthiran P.  Study investigating pneumococcal vaccine immune responses in children previously infected with pneumococcus.
Decline of transplacentally acquired maternal hepatitis C (HCV) antibodies. Sydney Children’s Hospital Foundation. Palasanthiran P, Ziegler J, Robertson P. This study investigates the rate and pattern of decline of maternally acquired HCV antibodies in infants born to HCV infected mothers.
Role of microtransfusion in the perinatal transmission of HIV. Internal;
Australian Postgraduate Award research scholarship to J Warning.
Warning J, Ffrench R,
Ziegler J.
Use of cellular and genetic methods to identify maternal cells in infant blood, seeking also evidence of immune responses to HIV in exposed, uninfected infants.
Immune responses to hepatitis C in exposed, uninfected individuals. Internal; Australian Postgraduate Award  research scholarship to L Elliott. Elliott L,
Ffrench R,
Ziegler J,
Lloyd A.
A study involving serological testing and evidence of T cell immunity in various cohorts.
Transplacental transmission of viruses and pathogenesis of stillbirths, pre-term births and congenital anomalies. SCH. Rawlinson W, Jeffery H,
Jones C, Craig M, Palasanthiran P.
This prospective study is based on the hypothesis that undiagnosed viral infections are a cause of a range of pregnancy outcomes.
Cost-effectiveness of antenatal testing for HIV. SCH. Graves N,
Ziegler JB.
Analysis of service delivery and laboratory costs of testing, health service savings made and life years gained in various scenarios.
Chronic fatigue in adolescents. SCH. Gill A, Dosen A, Ziegler J. Study to determine the long-term outcome of adolescent chronic fatigue and assess the impact of early therapy.
Diagnosis of peanut allergy in peanut sensitive children. Internal; Sydney Children’s Hospital Foundation scholarship to B Wainstein. Wainstein B, Ziegler J. This project plans to assess the utility of a combination of clinical history, skin prick testing, immediate patch testing and measurement of circulating IgE antibody in the diagnosis of peanut allergy.
Hepatitis in mothers and infants study (HIMI). SCH. Palasanthiran P, Elliot, Ziegler J,
Best E, et al.
Study of hepatitis C virus (HCV) vertical transmission and HCV antibody decline.

Intensive Care

Acute respiratory distress syndrome in Australia. SCH. Numa A. This is a 12-month observational study of Acute Respiratory Distress Syndrome in children admitted to ICU in Australia and New Zealand, looking at incidence, outcomes and causes.
Hypothermia in traumatic brain injury in children (HITBIC). SCH. Numa A. This is a multi-centre study of the use of hypothermia as a treatment for severe head injury.

Medical Genetics

An investigation of the cognitive and behavioural phenotype of Mowat-Wilson Syndrome. Sydney Children’s Hospital Foundation. Mowat D,
Einfeld S,
Wilson M,
Evans E.
A study of the clinical features and cognitive and behavioural characteristics (genetic and environmental) of this newly recognised genetic syndrome.
A study of the behavioural profile of Mowat-Wilson syndrome. UNSW Faculty Research Grant. Einfeld S,
Mowat D,
Wilson M,
Evans D.
A questionnaire and direct assessment of the clinical features, level of intellectual disability and behaviour in this syndrome.
Microarray screening for DNA deletions causing intellectual disability. UNSW Faculty Research Grant. Mowat D,
Einfeld S,
Turner G.
Utilising another method to identify submicroscopic chromosomal deletions and/or duplications.
The Australian Child to Adult Development Study. NH&MRC. Einfeld S,
Tonge B, Parmenter T, Turner G,
Mowat D.
This is a longitudinal study of behavioural and emotional problems in young people with intellectual disability, investigating the cause of intellectual disability in a community sample of young people.
Implementation of screening for Dystrophin point mutations in NSW families with Duchenne Muscular Dystrophy. Muscular Dystrophy Association of NSW. Buckley M,
Mowat D.
Improving mutation detection in Duchenne/Becker muscular dystrophy.
A clinical approach to Duchenne/Becker Muscular Dystrophy.  Muscular Dystrophy Association of NSW. Buckley M,
Mowat D.
Improving mutation detection in Duchenne/Becker muscular dystrophy.
Outcome for children with de novo balanced chromosomal translocations detected on prenatal testing. Sydney Children’s Hospital Foundation. Kirk E. This study aims to discover the outcome for children born following the diagnosis during prenatal testing of a particular type of chromosome problem known as balanced reciprocal translocation.

Multicultural Health Service

Accounting for culture within children’s health services in Sydney: a survey of parents and carers. Westmead Children’s Hospital; SCH. Chalmers S, Choucair S,
White L, Lord B,
Eastwood J, Rosso-Buckton A, Crawford J.
Investigation how parents and carers of ill children from culturally and linguistically diverse (CALD) backgrounds fare during their child’s hospitalisation.

Nephrology

HMG CoA reductase inhibitors (statins) improve endothelial dysfunction in children with chronic renal failure. Sydney Children’s Hospital Foundation.  Mackie F, Rosenberg A, Kainer G, Miller O, Celermajer D. This trial examines the effect of statins (hormones produced by the hypothalamus
which stimulate the release of hormones in the pituitary gland) on the function of cells lining blood and lymphatic vessels in children with chronic renal failure.
Detection and characterisation of smell and taste dysfunction in children with chronic renal failure using psychophysical and anatomical methods. Sydney Children’s Hospital Foundation. Laing D,
Kainer G.
This project will characterise smell and taste changes in patients with chronic renal failure so that strategies can be developed for managing their diet and nutrition.
Agreement on reporting of ambulatory blood pressure monitoring in children. Sydney Children’s Hospital Foundation. Kennedy S, Mackie F, Rosenberg A, Craig E, Kainer G. A study of the interpretation of ambulatory
blood pressure monitoring in children by three paediatric nephrologists.
Urinary tract infections in children: does 10 years make a difference? SCH. Doyle K,
Argent E,
Kainer G.
An audit to determine whether the organisms that cause urinary tract infection, and their antibiotic sensitivities, in children have changed in the last 10 years.
Waiting time and outcome of kidney transplantation in adolescence. SCH. Kennedy S, Mackie F, Rosenberg A, McDonald S. To determine if adolescent kidney transplant recipients have poorer graft survival rates than children of other ages.

Neurology & Neuroscience

The neuropsychological and language profile of children with benign rolandic epilepsy. Australian Brain Foundation; Financial Markets Foundation for Children. Bye A,
Connolly A, Northcott E, McIntyre J, Christie J,
Lawson J,
Berroya A, Bleasel A.
A study to evaluate the quality of life, cognitive and language profiles in children with benign rolandic epilepsy.
The application of statistical parametric mapping to the MRI of children with epilepsy and malformations cortical dysplasia. Sydney Children’s Hospital Foundation. Lawson J, Bye A. The technique was evaluated for detection of brain tumours and brain malformations in children with epilepsy.
Evaluation of a problem-based learning and teaching approach in paediatric neurology. Sydney Children’s Hospital Foundation.
 
Bye A, Looker P,
Netherton C,
Evaluation of the teaching about epilepsy through the use of video, by a clinician.
Immune-mediated inflammation in dorsal root ganglia after peripheral nerve injury and in sensory neuropathies. NH&MRC.
 
McLachlan E, Cunningham A.
 
This project explores basic mechanisms underlying the inflammatory response to injury and disease in the nervous system, with relevance to nerve injury and post-traumatic pain syndromes.
The biology of olfactory neural stem cells and studies of cell lineage and differentiation in the olfactory system using an in vitro neurosphere model and time-lapse videomicroscopy. The Garnett Passe and Rodney Williams Memorial Foundation; SCH. Cunningham A, Kaplinovsky T, Marlicz W, Meedeniya A. A multifaceted cell biological project aiming to identify and characterise the unique neural stem cell present in the olfactory (smell) nervous system.
New therapies for neurological injuries and diseases based on neural stem cells. Australian Brain Foundation. Cunningham A. This project aims to further define the potential of adult nerve stem cells for cell replacement therapies in nervous system disease and traumatic injury.
Adult neural stem cells and their potential therapeutic role in perinatal cerebral injury.  Lesley Stevens Fund for Newborn Care. Ward M, Cunningham A. This project examines the responses of endogenous stem cells in the premature and term brain, to various insults including lack of oxygen and blood flow, mirroring the insults that can occur at the time of birth. This has direct relevance to the underlying causes of newborn brain damage and resultant conditions such as cerebral palsy.
Expression of the GDNF family of growth factors in the olfactory system. The Garnett Passe & Rodney Williams Memorial Foundation; SCH. Maroldt H, Kaplinovsky T, Cunningham A. The olfactory (smell) system is unique in its support of robust nerve generation and communication throughout adult life. The regulation of these processes and the growth factors involved are as yet poorly defined. This study involves characterisation of the GDNF family of trophic factors in the olfactory nervous system, aiming to define their mechanisms of action on these unique stem cells and nerve cells.
Neurturin and its receptor components in the olfactory system. Sydney Children’s Hospital Foundation. Meedeniya A, Maroldt H, Cunningham A. Neurturin is a member of the GDNF family of growth factors thought to be important in nervous system development and sensory systems. This project explores the expression of the protein and its receptor complex in the olfactory nervous system.
Differential expression of two isoforms of the RET receptor tyrosine kinase in the olfactory system, substantia nigra and other central nervous system regions. The Garnett Passe and Rodney Williams Memorial Foundation; SCH. Kaplinovsky T, Cunningham A. GDNF is one of the most potent growth factors in the human brain and has been used in trials for Parkinson's disease, a condition of basal ganglia degeneration. This project is defining the detailed localisation of two distinctive isoforms of the GDNF receptor complex, which are known to be capable of stimulating different downstream signalling pathways, in brain regions relevant to Parkinson's disease, memory and learning, and smell.
Proliferation, differentiation and phenotypic outcome of endogenous neural stem cells in an animal model of post-traumatic syringomyelia. Dalcross Private Hospital; SCH. Liao J,
Stoodley M,
Tu J,
Cunningham A.
A project using an animal neurosurgical model of syringomyelia, a cystic degenerative condition of spinal cord that occurs commonly after spinal cord injury. These studies trace the response of stem cells in the damaged spinal cord and determines the type of nerve cells they become at various times post-injury.
Studies of the up-regulation of expression of immature intermediate filament proteins in the intestine of children with inflammatory bowel disease. SCH. Leach S,
Day A, Cunningham A.
The diseased bowel wall becomes inflamed and appears to up-regulate immature cells, perhaps in an attempt to repair the process. This project is studying the expression of various immature markers in biopsies from patients with inflammatory bowel disease, in an attempt to better understand these pathological processes.
The usefulness of the sleep disturbance scale for children (Bruni) as a predictor of physiological problems during sleep in Duchenne muscular dystrophy. SCH. Moline A, Williamson B, Teng A, Betts G,
Pedersen R, Johnston H.
A study to determine if a questionnaire can detect breathing problems during sleep in boys with Duchenne muscular to help to identify who needs specialised sleep monitoring.

Neurosurgery

Vascular targeting enhancement of radiosurgery
for arteriovenous malformations.
SCH. Stoodley M, Storer K,
Smee R,
Tu J.
Congenital malformations of brain blood vessels are a leading cause of stroke in children and young adults. Prevention of bleeding can be achieved only by surgical removal or by obliteration with high doses of radiation, which causes slow occlusion of vessels. Some lesions are too risky to remove and too large to be treated with radiation. The aim of this project is to enhance vascular occlusion after radiation.

Nursing Practice Development Unit

An investigation of multidisciplinary teamwork from the perspective of clinicians working within patient care teams. Sydney Children’s Hospital Foundation. Crisp J, Aston J, Binns V, Wade C,
Dunlop S, Kepreotes H, Marshall L,
Moran P, White L,
Murdoch J,
Prior A, Shi E, Tighe J, Wales S,
Visocnik F.
First stage in a series of studies aimed at building teamwork capacity within the patient care teams. This study examined clinicians’ perceptions of their own and their team’s efficacy, effort and skill levels.
Evaluation of a child and family held care management folder. SCH. Hardy J, Crisp J,
Macmillan C, Moran P,
O’Brien I.
Evaluation of a patient-held folder in improving continuity of care, communication, satisfaction and confidence among children with cancer, their families and health care providers.
Validation of an instrument to measure adherence of adolescents with chronic disease. SCH. Crisp J, Kyngas H, Poole D, Nadew K, Thomsen A, Wales S. Reliability and validity assessment of a tool measuring adherence levels in adolescents with asthma or diabetes.

Pain Medicine Unit

School functioning of children and adolescents with pain disorders: the development and validation of the School Functioning Scale. Sydney Children’s Hospital Foundation. Piira T, Betts G, Chan E, McInerney M. This study aims to develop the first reliable and valid school functioning questionnaire for children with chronic pain.
Effect of attentional coping strategies and individual difference factors on children’s cold-pressor pain outcomes. School of Psychology UNSW; SCH.
 
Piira T,
Hayes B, Goodenough B.
An experimental study to learn more about the efficacy of children’s coping strategies for pain and the role of individual difference factors.
Parent verbal behaviour and infant pain at immunisation: a randomised control trial evaluating a parent intervention. School of Psychology UNSW, SCH. Bustos, T, Piira, T, Salmon K Assessment of whether coaching parents to use more coping promoting talk has a positive effect on infant distress and pain responses following an immunisation injection
Don’t break my heart: Descriptors of social and physical pain across ethnolinguistic groups Dalhousie University, Halifax, Canada;
UCLA, Los Angeles, USA; SCH.
Piira T,
Grégoire M-C, Clemente I.
 
A survey to review the social and physical pain descriptors across a wide range of languages.

Respiratory

S100 proteins as markers of lung inflammation in young children with cystic fibrosis. Rebecca Cooper Foundation.
 
Belessis Y,
Day A, Leach S,
Morton J.
Assessment of novel markers of airway inflammation in children with cystic fibrosis.
Urinary desmosine as a marker of lung inflammation in young children with cystic fibrosis. SCH. Belessis Y, Winfield K, Lui K,
Morton J, Numa A.
Evaluating urinary markers of elastin breakdown as indicators of lung inflammation in young children with cystic fibrosis.
The lung clearance index and its relationship to airway infection and inflammation. SCH. Belessis Y, Lui K,
Numa A, Morton J, O’Donovan B, Hawkins G, Xu L.
 
Assessing the value of a new index of lung function in determining early infection and inflammation in cystic fibrosis.

School of Women's and Children's Health


An integrative model for improving medication safety in children.
Australian Council for Safety and Quality in Health Care. Gazarian M, Graudins L, the medication safety improvement project team. Study evaluating an innovative strategy for reducing medication errors and related harm in hospitalised children.
Safe paracetamol use in children: development and evaluation of caregiver medicine information. Australian Council for Safety and Quality in Health Care. Sinha Y,
Graudins LV, Gazarian M.
Development and evaluation of a medicines information pamphlet for caregivers of young children to improve the safe use of paracetamol.
 
Studies of smell and taste function and dysfunction in neonates. Leslie Stevens Fund for Newborn Care; SCH. Laing D, Lui K, Cunningham A,  Hutchinson I, Bolton-Turner M. Developing tests of smell and taste for infants to determine if they have chemosensory disorders.
Clinical aspects of chemosensory function. SCH; Australian Postgraduate Award esearch scholarship to J Armstrong. Armstrong J, Laing D.
 
This project examines aspects of chemosensory function in children, a little studied area. Studies are being undertaken of potential abnormalities of the senses of smell and taste in disease states, with a view to better managing paediatric patients with these conditions. This includes evaluation of taste and smell in Aboriginal children with chronic otitis media and nasal infections. Another aspect is the development of a new test to assess smell and taste function in young infants.
Once daily gentamicin in infants and children: an evaluation of safety and the role of therapeutic drug monitoring in minimising toxicity. Sydney Children’s Hospital Foundation. Gazarian M, Palasanthiran P, Best E, Cohn R,
Wilkinson M.
Study evaluating the safety of once daily administration of gentamicin in children, especially with regard to hearing and renal function, and the role of routine measurement
of drug levels in minimising toxicity.