Diffuse central nervous system protoplasmic astrocytoma.

Protoplasmic astrocytoma is an extremely rare form of grade II low grade glioma which usually presents as a discrete mass lesion. We describe a 3-year-old female with diffuse protoplasmic astrocytoma with parenchymal involvement and leptomeningeal spread. This tumour proved extremely difficult to diagnose and followed a progressive course. Three superficial biopsies did not give the diagnosis and this was only confirmed 8 months from presentation from a larger fourth biopsy taken deeper from the cerebellum. To our knowledge this case represents the distinct presentation of protoplasmic astrocytoma presenting as extensive diffuse meningeal disease.

A survey of public transport use by immunocompromised children in the United Kingdom.

BACKGROUND: The advice given to immunocompromised children and their families on the use of public transport at our oncology unit has been inconsistent, with a lack of written or evidence based guidelines. We therefore carried out two surveys, one national and one local on this issue, to determine what advice is given to families. We also undertook an extensive literature search on this subject. METHODS: The first survey was completed by the Paediatric Oncology Nurses Forum link member at each of the 22 United Kingdom Childrens' Cancer Study Group (UKCCSG) centres. We asked their current practice for use of public transport for hospital visits, school attendance, regular weekly use and holidays. We looked at recommendations for travel on holiday in the UK and abroad with reference to neutrophil and platelet counts. The second survey was completed by parents of 64 children attending our outpatient clinic over a 2-week period to ascertain whether the families comply with our verbal recommendations. RESULTS: There was a 100% response from UKCCSG centres. None of the centres had any written or evidence based guidelines. 55% of centres allowed public transport for hospital visits, 82% for school and 90% for holidays. Sixty-eight percent of centres allowed UK travel with a neutrophil count of 0.5-1 x 109/L and 18% if neutrophils <0.5 x 109/L. Eighteen percent of centres allowed foreign travel with a neutrophil count of 0.5-1 x 109/L but only 9% if <0.5 x 109/L. The second survey showed 2% used public transport to attend hospital, 5% for school, 17% for regular use and 50% travelled by plane for holidays. A Pubmed literature search revealed a lack of studies on immunocompromised patients. CONCLUSION: A certain use of public transport is necessary for patients to engage in as normal a life as possible. There is a need for further studies to produce sensible and consistent advice on this issue.

Chickenpox in varicella IgG positive patients: experience of a regional paediatric oncology centre.

Aciclovir prophylaxis was previously given to all immunocompromised patients treated by our unit, following contact with varicella zoster. In 2003, we changed practice according to National Guidelines, giving prophylaxis only to patients without serum varicella zoster immunoglobulin G antibody (VZ IgG) at diagnosis of their malignancy. Since then we have seen nine patients with acute lymphoblastic leukaemia (ALL) and VZ IgG positivity at diagnosis of their malignancy develop chickenpox. Our observations question current practice for patients with ALL.

PEPtalk: postexposure prophylaxis against varicella in children with cancer.

OBJECTIVES: To describe postexposure prophylaxis (PEP) against varicella zoster virus (VZV) in children being treated for malignancy in the UK and Ireland: the population at risk, frequency of exposure, clinical practice and attitudes among healthcare providers. DESIGN: An observational study in three parts: (1) a retrospective survey of serostatus at diagnosis of malignancy, (2) collation of varicella zoster immune globulin (VZIG) dispensing data over a 3-year period and (3) an online survey of paediatric oncologists' clinical practice and beliefs in relation to VZV disease and its prevention. SETTING: UK and Ireland. PARTICIPANTS: Children diagnosed with malignancy in 2009 (serostatus survey) or receiving VZIG between April 2006 and March 2009 (VZIG dispensing study). Paediatric oncologists and haematologists working in tertiary paediatric oncology centres and related shared care units in the UK and Ireland (physician survey). RESULTS: Of 1500 children diagnosed with malignancy each year, at least 24% are VZV seronegative. Few centres make efforts to prevent household exposure by vaccinating VZV-susceptible family members. Exposures to VZV result in the administration of PEP to approximately 250 children with cancer annually: half receive an intramuscular injection of VZIG while the remainder receive a course of oral aciclovir. The choice of PEP is made by doctors. There is no consensus among paediatric oncologists as to which is the better option, reflecting the lack of a secure evidence base. CONCLUSIONS: A randomised controlled trial to compare the effectiveness and acceptability of VZIG and aciclovir as PEP against varicella is both desirable and feasible.

Development and progression of nephropathy in type 2 diabetes: the United Kingdom Prospective Diabetes Study (UKPDS 64).

BACKGROUND: The progression of nephropathy from diagnosis of type 2 diabetes has not been well described from a single population. This study sought to describe the development and progression through the stages of microalbuminuria, macroalbuminuria, persistently elevated plasma creatinine or renal replacement therapy (RRT), and death. METHODS: Using observed and modeled data from 5097 subjects in the UK Prospective Diabetes Study, we measured the annual probability of transition from stage to stage (incidence), prevalence, cumulative incidence, ten-year survival, median duration per stage, and risk of death from all-causes or cardiovascular disease. RESULTS: From diagnosis of diabetes, progression to microalbuminuria occurred at 2.0% per year, from microalbuminuria to macroalbuminuria at 2.8% per year, and from macroalbuminuria to elevated plasma creatinine (>or=175 micromol/L) or renal replacement therapy at 2.3% per year. Ten years following diagnosis of diabetes, the prevalence of microalbuminuria was 24.9%, of macroalbuminuria was 5.3%, and of elevated plasma creatinine or RRT was 0.8%. Patients with elevated plasma creatinine or RRT had an annual death rate of 19.2% (95% confidence interval, CI, 14.0 to 24.4%). There was a trend for increasing risk of cardiovascular death with increasing nephropathy (P < 0.0001), with an annual rate of 0.7% for subjects in the stage of no nephropathy, 2.0% for those with microalbuminuria, 3.5% for those with macroalbuminuria, and 12.1% with elevated plasma creatinine or RRT. Individuals with macroalbuminuria were more likely to die in any year than to develop renal failure. CONCLUSIONS: The proportion of patients with type 2 diabetes who develop microalbuminuria is substantial with one quarter affected by 10 years from diagnosis. Relatively fewer patients develop macroalbuminuria, but in those who do, the death rate exceeds the rate of progression to worse nephropathy.