The transition process for paediatric rheumatology clinic patients at a single tertiary paediatric rheumatology centre in Australia.

AIM: This study aimed to examine the transition process of paediatric rheumatology patients from the Monash Children's Hospital (MCH) in Melbourne in order to identify areas that could be improved. METHODS: Retrospective review of clinical data from the rheumatology database of paediatric rheumatology patients eligible for transition between January 2015 and September 2020. RESULTS: One hundred and sixty-five patients were included; 57 patients were transitioned. Of patients transitioned to an adult service, 38 (88%) were on medication and 14 (33%) had active disease. All patients transitioned to the general practitioner (GP) had inactive disease off medication. Juvenile idiopathic arthritis (JIA) (non-systemic) was the most common diagnosis in patients transitioned. The mean age at which transition was first discussed was 18.0 years; the first referral was made at a mean of 18.3 years. The mean age at the first adult appointment was 18.5 years. Thirty-nine (91%) patients had a referral completed and 8 (19%) had a transfer letter. Thirteen (93%) patients transferred to the GP had a transfer letter. Transfer documents to an adult public rheumatology service rated 4.3 for quality, compared to 5.5 to the GP. Transfer of care was confirmed in 40 (93%) patients transitioned to an adult service; however, correspondence was available for only 3 (7%). CONCLUSION: Although the transition process at MCH was adequate, it could be improved through earlier discussion of the process and improved referrals and documentation. A readiness-to-transfer checklist and a young adult clinic have the potential to improve the process of transition to adult rheumatology care.

Infliximab Reverses Symptoms and May Protect from Developing Chronic Restrictive Ophthalmopathy in Children with Familial Orbital Myositis: A Case Report.

BACKGROUND: Orbital myositis is a rare sporadic eye disease associated with extraocular eye muscle inflammation. To date, there have been two reports of familial orbital myositis (FOM), which demonstrate partially penetrant autosomal dominant inheritance. CASES: We report six new Australian cases of FOM, four of whom extend one of the reported pedigrees, as well as a separate mother and daughter manifesting orbital myositis, which constitutes a third report of familial occurrence. We can confirm that the disease has onset in childhood, appearing to go into remission in adult life, and that the inflammation is corticosteroid-responsive. However, one patient went on to develop permanent diplopia in upgaze. We also report two children suffering chronic pain and diplopia who demonstrated complete resolution of symptoms with the anti-TNF-α monoclonal infliximab. CONCLUSION: Uncontrolled FOM in childhood may result in permanent extraocular eye muscle damage, while TNF-α blockade provides an excellent steroid-sparing effect.

Paediatric Salmonellosis-Differences between Tropical and Sub-Tropical Regions of Queensland, Australia.

Salmonellosis is an important cause of morbidity in tropical regions.This study aims to describe the epidemiology of non-typhoidal Salmonellae (NTS) in children presenting to public hospitals in Queensland, Australia, over the past 20 years, with a focus on differences between tropical and sub-tropical zones in the region. This is a retrospective and descriptive cohort study of 8162 NTS positive samples collected in 0-17-year-olds from the Queensland public hospital pathology database (Auslab) over a 20-year period from 1997 to 2016. There were 2951 (36.2%) positive NTS samples collected in tropical zones and 5211 (63.8%) in the sub-tropical zones of Queensland, with a total of 8162 over the region. The tropical zone contributed a disproportionately higher number of positive NTS samples by population sub-analysis. Of the specimens collected, 7421 (90.92%) were faecal, 505 (6.2%) blood, 161 (1.97%) urine, 13 (0.16%) cerebrospinal fluid (CSF) and 62 of other origin. Other categories of specimen types isolated include swab, fluid, aspirate, lavage, bone, tissue, isolate and pus, and these were not included in sub-analysis. The most commonly identified serovars were Salmonella Typhimurium, Salmonella Virchow and Salmonella Saintpaul. This is the first and largest study that emphasises the high burden of invasive and non-invasive NTS infections resulting in hospital presentations in the paediatric population of tropical north Queensland, compared to the sub-tropics.

Hypoxia-inducible factor-1α and its role in the proliferation of retinoblastoma cells.

In order to better understand the role of HIF-1α in the proliferation of the retinoblastoma cells, a siRNA knockdown of HIF-1α followed by a proliferation assay was performed. Further sequencing was then carried out in order to assess knockdown efficiency and expression of HIF-1α. Upregulation of HIF-1α gene expression in CoCl2-treated retinoblastoma cells was demonstrated via melting curve analysis from PCR tests and was further analyzed using western blot and densitometry analysis. Reduction of HIF-1α expression in retinoblastoma, post HIF-1α knockdown, was observed after siRNA transfection into Y-79 cells. Knockdown of HIF-1α resulted in a significant decrease in proliferation thereby demonstrating that HIF-1α is involved in promoting survival and proliferation in retinoblastoma cells. Stabilization of HIF-1α in retinoblastoma cells using CoCl2 was unsuccessful.