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Failure of cisplatin-based chemotherapy in advanced germ cell tumour (GCT) is associated with a poor outcome. High-dose chemotherapy and auto-SCT is one therapeutic option, although the long-term outcome after this procedure is unclear. We conducted a multicentre cohort study of consecutive patients undergoing a single auto-SCT for GCT between January 1986 and December 2004. Of 71 subjects, median follow-up is 10.1 years. OS at 5 years is 44.7% (95% confidence interval (CI) 32.9-56.5%) and EFS is 43.5% (95% CI 31.4-55.1%). There were seven (10%) treatment-related deaths within 100 days of auto-SCT. Three (4.2%) patients developed secondary malignancies. Of 33 relapses, 31 occurred within 2 years of auto-SCT. Two very late relapses were noted 13 and 11 years after auto-SCT. In multivariate analysis, favourable outcome was associated with IGCCC (International Germ Cell Consensus Classification) good prognosis disease at diagnosis, primary gonadal disease and response to salvage chemotherapy. We conclude that auto-SCT results in successful outcome for a relatively large subgroup of patients with high-risk GCT. Late relapses may occur, a finding not previously reported. [ABSTRACT FROM AUTHOR]

The Joint United Nations Programme on HIV/AIDS (UNAIDS) estimates that in 2004, there were 39.4 million people living with HIV/AIDS worldwide (UNAIDS/WHO Report on the global HIV/AIDS epidemic, 2004). Children less than 15 years of age comprise 2.2 million of these individuals. As more children globally gain access to highly active antiretroviral therapy (HAART), more children are growing to the age when disclosure of their HIV status is inevitable. This information may affect a child's disease trajectory, and in the context of HAART, may have wide-ranging impact in the management of paediatric HIV infection. This study is an investigation of the effect of disclosure of a child's own HIV infection status on death and CD4 decline in a cohort of 325 HIV-infected Romanian children receiving highly active antiretroviral therapy (HAART). A retrospective database analysis was conducted. Data from a nearly three-year period were examined. Children who were aware of their HIV diagnosis were compared with those who were not aware. We found significant associations between not knowing the HIV diagnosis and death, and not knowing the HIV diagnosis and disease progression defined as either death or CD4 decline. Our results imply that in the context of HAART, knowledge of one's own HIV infection status is associated with delayed HIV disease progression. [ABSTRACT FROM AUTHOR]

Objective: To assess the efficacy of oral acyclovir in treating adults with varicella and to describe the natural history of adult varicella.Design: Double-blind, placebo-controlled randomized trial.Setting: A naval hospital.Patients: One hundred forty-eight of 206 consecutive adult active duty Navy and Marine Corps personnel who were hospitalized for isolation and inpatient therapy of varicella and who could be treated within 72 hours of rash onset completed the study. The diagnosis of varicella was confirmed by acute and convalescent serology in 143 of 144 patients with available paired sera.Intervention: Patients were randomly assigned to receive either acyclovir, 800 mg orally five times per day for 7 days, or an identical placebo. Separate randomization codes were used for patients presenting within 24 hours of rash onset and for those presenting 25 to 72 hours after rash onset.Measurements: Daily lesion counts, symptom scores, temperature measurements, and laboratory tests were used to monitor the course of the illness.Results: Early treatment (initiated within 24 hours of rash onset) reduced the total time to (100%) crusting from 7.4 to 5.6 days (P = 0.001) and reduced the maximum number of lesions by 46% (P = 0.04). Duration of fever and severity of symptoms were also reduced by early therapy. Late therapy (25 to 72 hours after rash onset) had no effect on the course of illness. Only four patients had pneumonia, and no encephalitis or mortality was noted.Conclusions: Early therapy with oral acyclovir decreases the time to cutaneous healing of adult varicella, decreases the duration of fever, and lessens symptoms. Initiation of therapy after the first day of illness is of no value in uncomplicated cases of adult varicella. The low frequency of serious complications of varicella (pneumonia, encephalitis, or death) precluded any evaluation of the possible effect of acyclovir on these outcomes. [ABSTRACT FROM AUTHOR]

A 35 year old woman with clinical features of hyperkalaemic periodic paralysis confirmed on provocative exercise testing was investigated. DNA sequence analysis of the gene for the alpha-subunit of the skeletal muscle voltage gated sodium channel (SCN4A) on chromosome 17q23 identified a point mutation at nucleotide position 2188. This results in a threonine to methionine substitution at amino acid position 704. The patient was intolerant of diuretic medication but showed a striking clinical and electrophysiological improvement with salbutamol therapy. Treatment with beta-adrenergic agents should be considered in patients with hyperkalaemic periodic paralysis who are intolerant of, or resistant to, diuretic medications. [ABSTRACT FROM AUTHOR]

Bleeding from duodenal varices is an unusual event. We report the case of a 50-yr-old man with portal hypertension due to alcoholic cirrhosis who presented with upper gastrointestinal bleeding and encephalopathy. Emergent endoscopy revealed an actively bleeding duodenal varix. The bleeding was treated successfully with injection sclerotherapy. Only four cases of injection sclerotherapy of bleeding duodenal varices have been reported previously. We review and compare reported cases of sclerotherapy of duodenal varices and also review the other therapeutic options. Endoscopic injection sclerotherapy of bleeding duodenal varices appears to be a useful first-time therapy. [ABSTRACT FROM AUTHOR]

Objective To investigate user satisfaction of the paediatric CF homecare service (Nursing and Physiotherapy) and identify areas for improvement. Method 307 paediatric CF families were sent an anonymous semi-structured postal questionnaire, irrespective of whether they had accessed the homecare service. Results 35% response rate (108/307), Age range 4 months to 16 years. Overall satisfaction: 72% scored the service 8–10/10 (59% 10/10), 21% had not received homecare input. The service excelled at communication, school/nursery education and timing of visits (frequency – mainly as required – and length – averaging 64 minutes per visit). Improvements are required to raise the profile of the service – 10% were unaware of the homecare service. Geographical location of patients’ homes restricted visits from the nursing service. Other CF team members requested to visit at home: 10% Psychology and 9% Dietitians. Improvements: Raising service profile: information leaflets were produced for outpatients and the trust website. Geographical boundaries for nursing-led visits were removed. A dedicated dietetic paediatric CF homecare post was created and psychology input into the homecare environment was increased. The physiotherapy team expanded ensuring all newly diagnosed families are reviewed at home within 1 month and the number of families reviewed annually increased from 37% to 55%. Conclusion The RBH CF homecare team deliver a service that is well valued and achieves a high satisfaction rating – highlighting the importance families’ place on a homecare service for communication, expert knowledge, support and understanding in an environment that minimises disruption to daily life. [ABSTRACT FROM AUTHOR]

Presents a case study on effect of drugs on diseases in Europe. Development of acute renal failure following recombinant interferon treatment; Observation of nicoumalone related skin disorders; Association of skin lesion with intravenous streptokinase administration.