INTRODUCTION: ADAMTS13 (a disintegrin-like and metalloproteinase with a thrombospondin Type 1 motif, member 13) plays a fundamental role in the regulation of haemostasis and thrombosis. Its deficiency leads to an accumulation of ultra-large von Willebrand multimers, inducing spontaneous platelet aggregation, thrombosis in the microvasculature, and thrombotic thrombocytopenic purpura (TTP), a condition with 90% mortality when left untreated. Prompt quantification of ADAMTS13 antigen, activity and autoantibody plays a crucial role in the diagnosis and management of TTP and can help differentiate it from other thrombotic microangiopathies (TMAs). Reference ranges for ADAMTS13 are generally derived from Caucasian patients. Given that polymorphism in the ADAMTS13 gene can be associated with variable ADAMTS13 levels, we aimed to establish the first reference range in Singapore and provide a crucial laboratory test for institutions here and elsewhere. METHODS: 150 healthy voluntary donors (75 men, 75 women) aged 21-60 years, with an ethnic mix mirroring Singapore's population profile, were recruited. ADAMTS13 antigen, activity and autoantibody levels were measured using the fluorescence resonance energy transfer-vWF73 and enzyme-linked immunosorbent assay methodologies. RESULTS: Levels (activity 0.65-1.79 IU/mL, antigen 0.36-1.17 IU/mL, autoantibody 1.4-12.5 U/mL) were not statistically different between the genders and various age groups. CONCLUSION TTP and TMAs are encountered in a wide range of specialties. The availability of new assays in Singapore will aid clinicians in the timely management of these conditions. Standardising reference ranges established for Singapore against World Health Organization standards allows harmonisation of measurements between laboratories and for future research collaborations.
ADAMTS13 Protein/analysis* ; Adult ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Male ; Purpura, Thrombotic Thrombocytopenic/diagnosis* ; Reference Values ; Singapore

Bacterial Infections ; Humans ; Lymphoma, Large B-Cell, Diffuse/diagnosis* ; Mucormycosis/diagnosis* ; Respiratory Sounds ; Thyroid Gland

Aged ; Aged, 80 and over ; Betacoronavirus ; genetics ; Coronavirus Infections ; epidemiology ; therapy ; DNA, Viral ; analysis ; Disease Management ; Female ; Humans ; Male ; Pandemics ; Pneumonia, Viral ; epidemiology ; therapy ; Singapore

INTRODUCTIONIntrathecal baclofen (ITB) therapy is a proven, effective treatment for disabling cortical spasticity. We describe the first local series of five patients with acquired brain injury (ABI) who received ITB and were followed up for 63.8 months.

METHODSA retrospective review of medical and rehabilitation records of patients who received ITB therapy was carried out. Data studied included baseline demographic and injury variables, implantation data, spasticity and function, ITB dosage over time and complications.

RESULTSFrom 2006 to 2010, a total of five patients received ITB therapy via implanted pumps about 39.4 months after ABI. Four out of five patients experienced significant reductions in their lower limb spasticity scores and improvements in global function and dependency. One patient had minor adverse events associated with baclofen-related sedation. The mean ITB dose at one year was 182.7 ± 65.6 mcg/day.

CONCLUSIONOur preliminary study showed encouraging long-term outcomes and safety for ITB therapy after ABI-related intractable spasticity. Individual ITB responses over time were variable, with gender differences. The outcomes experienced by our centre were comparable to those in the general ABI population, supporting the efficacy of ITB therapy for chronic disabling spasticity.

Baclofen ; administration & dosage ; Brain Injuries ; complications ; drug therapy ; Dose-Response Relationship, Drug ; Female ; Follow-Up Studies ; Humans ; Infusion Pumps, Implantable ; Injections, Spinal ; Male ; Muscle Relaxants, Central ; administration & dosage ; Muscle Spasticity ; diagnosis ; drug therapy ; etiology ; Retrospective Studies ; Severity of Illness Index ; Singapore ; epidemiology ; Tertiary Care Centers ; Treatment Outcome

Primary malignant B-cell-type dural lymphoma is a rare subtype of primary central nervous system lymphoma (PCNSL). We herein report an unusual case of diffuse B-cell lymphoma that presents as a chronic subdural haematoma without extracranial involvement. The notable aspects of this case include the patient's immunocompetence, a short clinical history of symptom onset, rapid neurological deterioration and a fi nal diagnosis of high-grade PCNSL. This case highlights the challenges neurosurgeons face, especially in the emergency setting, when the disease manifests in varied presentations.
Brain Neoplasms ; diagnosis ; surgery ; Hematoma, Subdural ; diagnosis ; surgery ; Humans ; Lymphoma, B-Cell ; diagnosis ; surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multimodal Imaging ; Tomography, X-Ray Computed

Heparin has been widely used for intradialytic anticoagulation since the 1940s. Heparin induced anaphylaxis can be life threatening, mandating early recognition and intervention. However, due to its relative rarity many physicians remain unaware. We report the case of a 70-year-old woman requiring dialysis, who developed recurrent anaphylaxis to intradialytic heparin. We describe a systematic approach to confirm the suspected heparin allergy, which must include an evaluation of predisposing factors, the dialysis equipment and concomitant medications. Further workup for safe alternatives employing skin prick and intradermal tests, as well as provocation tests are discussed.
Aged ; Anaphylaxis ; Causality ; Dialysis ; Drug Hypersensitivity ; Female ; Heparin ; Humans ; Hypersensitivity ; Intradermal Tests ; Skin ; Skin Tests

Growing Up in Singapore Towards healthy Outcomes (GUSTO) is Singapore's largest birth cohort study to date. The main aim of GUSTO is to evaluate the role of developmental factors in the early pathways to metabolic compromise. Detailed data is collected for a range of environmental exposures in the parents and offspring, and allergic disorders are among a number of outcomes assessed in infancy and childhood. Under the Allergy domain of GUSTO, this integrated study will describe the epidemiology of allergic manifestations and different phenotypes in the Asian context and help shed light on the association of metabolic disease to allergy. Epigenetic mechanisms and associations with other childhood disorders will also be explored. The aim of this report is to focus on methodology of GUSTO, and to suggest similar approaches (i.e., integrated cohort studies on pediatric allergy) worldwide. Recruitment commenced in 2009 with a cohort of 1,163 pregnant mothers in their first trimester. The mothers and children were followed throughout pregnancy and follow-up will continue until the child reaches 3 years of age. Preliminary results showed that 39.8% of the mothers had a personal history of having at least one allergic disease, which included asthma, eczema and allergic rhinitis. Further data collection and analyses are still ongoing. Allergy is a complex spectrum of disorders with numerous poorly-understood aspects. The ongoing GUSTO cohort study, with its longitudinal design and multi-disciplinary nature, may provide new insights into developmental influences on allergy. As a Singapore-based study, it will be the first integrated allergy cohort in Southeast Asia, of which recruitment started during pregnancy.
Asia, Southeastern ; Asian Continental Ancestry Group ; Asthma ; Child ; Cohort Studies ; Data Collection ; Eczema ; Environmental Exposure ; Epidemiology ; Epigenomics ; Female ; Follow-Up Studies ; Humans ; Hypersensitivity ; Metabolic Diseases ; Mothers ; Parents ; Parturition ; Phenotype ; Pregnancy ; Pregnancy Trimester, First ; Rhinitis, Allergic ; Singapore

The mortality and morbidity of end-stage renal failure patients undergoing conventional thrice weekly in-centre haemodialysis remain alarmingly high despite continuing advances in haemodialysis technologies and improvements in clinical care. Home haemodialysis continues to be under-utilised in many parts of the world despite the reported benefits. Alternative haemodialysis regimens including longer and/or more frequent dialysis (e.g. nocturnal haemodialysis and short daily haemodialysis), haemodiafiltration and the use of high flux dialysers have become more widespread in recent years as nephrologists struggle to improve the dismal survival figures. Whilst most of the encouraging data have come from observational studies, many randomised controlled trials which will provide more robust data are already underway. This review aims to provide a concise update of the recent and novel trends in haemodialysis.
Humans ; Kidney Failure, Chronic ; therapy ; Renal Dialysis ; trends

INTRODUCTIONThe aims of this article were to review the role of surgical resection in the management of high-grade gliomas and to determine whether there is any survival benefit from surgical resection.

METHODSA literature review of the influence of surgical resection on outcome was carried out. Relevant original and review papers were obtained through a PubMed search using the following keywords: glioma, resection, prognosis and outcome.

RESULTSPresently, there is a lack of evidence to support a survival benefit with aggressive glioma resection, but this should not detract patients from undergoing surgery as there are many other clinical benefits of glioma excision. In addition, limiting surgical morbidity through the use of adjuvant techniques such as intraoperative magnetic resonance imaging (MRI), functional MRI and awake craniotomy is becoming increasingly important.

CONCLUSIONSIdeally, a randomised controlled trial would be the best way to resolve the issue of whether (and to what extent) surgical resection leads to improvements in patient outcome and survival, but this would not be ethical. The second best option would be well-controlled retrospective studies with a multivariate analysis of all potential confounding factors.

Glioma ; classification ; surgery ; Humans ; Singapore ; Survival Analysis