Princess Margaret Hospital for Children

About: Princess Margaret Hospital for Children is a healthcare organization based out in Perth, Western Australia, Australia. It is known for research contribution in the topics: Population & Randomized controlled trial. The organization has 1501 authors who have published 2068 publications receiving 75773 citations. The organization is also known as: Perth Children's Hospital & PMH.

Clinical and endoscopic predictors of histological oesophagitis in infants.

Abstract: Objective: To define the earliest age at which histological changes can be used to diagnose oesophagitis and to determine the relationships between clinical, endoscopic and histological features of oesophagitis in infants. Methodology: The case records and biopsies of 113 infants aged 2–18 months with clinically significant gastro-oesophageal reflux (GOR), undergoing oesophagoscopy between 1978 and 1994 were retrospectively reviewed. The biopsies were independently evaluated and graded by two pathologists. Results: Forty-five cases (40%) had histological oesophagitis but only 16 (14%) had abnormal endoscopic findings (excluding erythema). Endoscopy was found to be highly specific (93%) for histological oesophagitis but lacked sensitivity (25%). Irritability was inversely related to the presence of endoscopic abnormalities, and there was poor correlation between symptoms and histological changes with only haematemesis showing a statistically significant association with histological abnormalities (P= 0.033). Intraepithelial lymphocytes were the earliest of the histological features noted and were present before 4 months of age. The numbers of intraepithelial eosinophils and lymphocytes and the presence of papillary elongation all increased with age. Conclusions: The presence of oesophagitis is difficult to predict on the basis of symptoms. The presence of intraepithelial lymphocytes is the earliest histological change to be seen in infants with GOR, and can develop before 4 months of age. Oesophagoscopy without biopsy is unreliable in the diagnosis of oesophagitis in infants.

The natural history of scoliosis in females with Rett syndrome

Abstract: Study Design. Population-based longitudinal observational study. Objective. To describe the prevalence of scoliosis in Rett syndrome, structural characteristics and progression, taking into account the influences of age, genotype, and ambulatory status. Summary of Background Data. Scoliosis is the most common orthopedic comorbidity in Rett syndrome yet very little is known about its natural history and influencing factors such as age, genotype, and ambulatory status. Methods. The infrastructure of the Australian Rett Syndrome Database was used to identify all cases with confirmed Rett syndrome in Australia and collect data on genotype and walking status. We identified radiological records and described the Cobb angle of each curve. Time to event analysis was used to estimate the median age of onset of scoliosis and the log-rank test to compare by mutation type. Latent class group analysis was used to identify groups for the trajectory of walking status over time and a multilevel linear model used to assess trajectories of scoliosis development by mutation type and walking status. We used a logistic regression model to estimate the probability of developing a scoliosis with a Cobb angle >60 degrees at 16 years in relation to Cobb angle and walking status at 10 years of age. Results. The median age of scoliosis onset was 11 years with earliest onset in those with a p.Arg255* mutation or large deletion. Scoliosis was progressive for all mutation types except for those with the p.Arg306Cys mutation. Scoliosis progression was reduced when there was capacity to walk independently or with assistance. Cobb angle and walking ability at age 10 can be reliably used to identify those who will develop a very severe scoliosis by age 16. Conclusion. These data on prognosis of scoliosis inform clinical decision making about the likelihood of progression to very severe scoliosis and the need for surgical management.

Factors influencing the provision of fertility counseling and impact on quality of life in adolescents and young adults with cancer

Abstract: Purpose This study investigated the impact of fertility-related discussions on Adolescent and Young Adult (AYA) cancer patients' quality of life (QoL) and the factors influencing provision of these discussions. Methods Recruitment was conducted through population-based state cancer registries. Eligible AYAs were 15-24 years at diagnosis, 3-24 months postdiagnosis, with any cancer (except early stage melanoma). As part of a larger survey, AYAs were asked about their experiences of fertility-related discussions and QoL (FACT-G). Results Of the 207 AYAs returning surveys (29% response rate) 88% reported a discussion about infertility risks, 75% reported a discussion about preservation options and 59% were offered a referral to a fertility specialist. Patients attending health services with an AYA focus were more likely than those attending other types of centers to report discussions of fertility preservation (FP) options (85% versus 67%) and referrals (75% versus 49%). Social well-being was positively related to discussions about preservation options and being provided fertility risk information in a sensitive, supportive manner. Conclusions Providing a sensitive and proactive discussion about fertility-related risks may benefit AYAs' well-being. Services with an AYA focus are fulfilling their mandate of ensuring optimal fertility-related care for AYA cancer patients.