ABSTRACT
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, mucocutaneous telangiectasia with systemic manifestations due to visceral telangiectasia and arterio-venous malformations (AVMs). We describe unusual complications of HHT in a 68-year-old male who developed high-output cardiac failure with pulmonary hypertension in combination with hepatic encephalopathy due to hepatic AVMs. This case shows the importance of a multi-system approach to HHT and considers the treatment of its hepatic complications.
Subject(s)
Hepatic Encephalopathy/etiology , Hypertension, Pulmonary/etiology , Telangiectasia, Hereditary Hemorrhagic/complications , Aged , Fatal Outcome , Hepatic Encephalopathy/diagnostic imaging , Humans , Hypertension, Pulmonary/diagnostic imaging , Male , UltrasonographyABSTRACT
BACKGROUND: Fertility is a priority for many young women with breast cancer. Women need to be informed about interventions to retain fertility before chemotherapy so as to make good quality decisions. This study aimed to prospectively evaluate the efficacy of a fertility-related decision aid (DA). METHODS: A total of 120 newly diagnosed early-stage breast cancer patients from 19 Australian oncology clinics, aged 18-40 years and desired future fertility, were assessed on decisional conflict, knowledge, decision regret, and satisfaction about fertility-related treatment decisions. These were measured at baseline, 1 and 12 months, and were examined using linear mixed effects models. RESULTS: Compared with usual care, women who received the DA had reduced decisional conflict (ß=-1.51; 95%CI: -2.54 to 0.48; P=0.004) and improved knowledge (ß=0.09; 95%CI: 0.01-0.16; P=0.02), after adjusting for education, desire for children and baseline uncertainty. The DA was associated with reduced decisional regret at 1 year (ß=-3.73; 95%CI: -7.12 to -0.35; P=0.031), after adjusting for education. Women who received the DA were more satisfied with the information received on the impact of cancer treatment on fertility (P<0.001), fertility options (P=0.005), and rated it more helpful (P=0.002), than those who received standard care. CONCLUSION: These findings support widespread use of this DA shortly after diagnosis (before chemotherapy) among younger breast cancer patients who have not completed their families.
Subject(s)
Breast Neoplasms/pathology , Decision Support Techniques , Fertility Preservation , Adult , Anxiety , Breast Neoplasms/psychology , Conflict, Psychological , Decision Making , Depression , Emotions , Female , Humans , Neoplasm Staging , Patient Education as Topic , Patient Satisfaction , Prospective Studies , Referral and Consultation , Surveys and QuestionnairesABSTRACT
The Ministry of Health (MOH) has published clinical practice guidelines on Management of Gambling Disorders to provide doctors and patients in Singapore with evidence-based guidance on the management of gambling disorders. This article reproduces the introduction and executive summary (with recommendations from the guidelines) from the MOH clinical practice guidelines on Management of Gambling Disorders for the information of readers of the Singapore Medical Journal. Chapters and page numbers mentioned in the reproduced extract refer to the full text of the guidelines, which are available from the Ministry of Health website (http://www.moh.gov.sg/mohcorp/publications.aspx?id=26136). The recommendations should be used with reference to the full text of the guidelines. Following this article are multiple choice questions based on the full text of the guidelines.
Subject(s)
Behavior, Addictive , Gambling , Mental Health Services , Humans , Behavior, Addictive/diagnosis , Behavior, Addictive/psychology , Behavior, Addictive/therapy , Evidence-Based Medicine , Gambling/diagnosis , Gambling/psychology , Gambling/therapy , Mass Screening , Mental Health Services/organization & administration , SingaporeABSTRACT
Amyotrophic lateral sclerosis (ALS) is a universally fatal neurodegenerative disease of the human motor system. Aetiological mechanisms implicated in the development of ALS have been linked to the glutamatergic neurotransmitter system, with destruction of motor neurons triggered through excessive activation of glutamate receptors at the synaptic cleft. This 'excitotoxicity' theory of ALS gave rise to the development of therapeutic approaches and ultimately clinical trials involving riluzole, initially thought to act solely as an inhibitor of glutamate release. Subsequent effects of riluzole have been postulated to include indirect antagonism of glutamate receptors, in addition to inactivation of neuronal voltage-gated Na+ channels. Riluzole remains the only disease-modifying therapy available to patients with ALS. Despite having been clinically available since the mid-1990 s, the in vivo pharmacological targets of riluzole have been poorly defined. An improved understanding concerning the potential neuroprotective mechanisms of riluzole may unearth pathophysiological processes that mediate neurodegeneration in ALS. The present review summarises the known chemical and pharmacological properties of riluzole. The failure of other putative neuroprotective therapies to demonstrate positive treatment outcomes in this intractable disease will be reviewed. Finally, the hypothesis that Na+ conductances may be involved in the processes of neuronal and axonal degeneration in ALS will be explored.
