Angiokeratoma is a dark violaceous keratotic papule which can be solitary or multiple. Generalised systemic angiokeratoma constitute angiokeratoma corporis diffusum, a rare X-linked recessive inborn error of glycosphingolipid metabolism due to deficiency of alpha galactosidase A. Patients with this disease have premature death due to the vascular complications. A case of possible Fabry's disease is presented.
Gestational trophoblastic disease is a spectrum of pregnancy disorder arising from the placental trophoblastic tissues. It characterised by the secretion of a distinct tumour marker, the beta-HCG. This condition is highly curable even in the presence of metastasis. The incidence of this disease is higher in the Asian population. The major well-established risk factors for gestational trophoblastic disease are advanced maternal age and a past history of gestational trophoblastic disease. Common clinical presentations include vaginal bleeding in early trimester, uterus larger than gestational age, absence of fetal parts after 20 weeks of gestation. Ultrasonography is a reliable non-invasive tool for diagnosis of gestational trophoblastic disease in the clinical setting. All placental tissue following miscarriage or curettage should have histopathological evaluation to exclude gestational trophoblastic disease. Since this group of disorders is one of the highly curable neoplasms, early diagnosis and prompt treatment is necessary.
New knowledge on the pathogenesis of the acute coronary syndromes provides the clinician with a better understanding of these important, often life-threatening, events and opens up new ways of managing them. The identification of the vulnerable plaque whilst important and possible pathologically still requires elaborate and often invasive methods. The central role of inflammation and the platelets in these syndromes has already been translated into practical therapeutics. Identifying and predicting which plaque would rupture and thereby facilitating the formation of an acute obstructive thrombus continue as major challenges to the clinician, but, in the meantime, regressing and stabilising these plaques are achievable goals.
Alzheimer's disease (AD) is a neurodegenerative disease with high prevalence in the rapidly growing elderly population in the developing world. The currently FDA approved drugs for the management of symptomatology of AD are marketed mainly as conventional oral medications. Due to their gastrointestinal side effects and lack of brain targeting, these drugs and dosage regiments hinder patient compliance and lead to treatment discontinuation. Nanotechnology-based drug delivery systems (NTDDS) administered by different routes can be considered as promising tools to improve patient compliance and achieve better therapeutic outcomes. Despite extensive research, literature screening revealed that clinical activities involving NTDDS application in research for AD are lagging compared to NTDDS for other diseases such as cancers. The industrial perspectives, processability, and cost/benefit ratio of using NTDDS for AD treatment are usually overlooked. Moreover, active and passive immunization against AD are by far the mostly studied alternative AD therapies because conventional oral drug therapy is not yielding satisfactorily results. NTDDS of approved drugs appear promising to transform this research from 'paper to clinic' and raise hope for AD sufferers and their caretakers. This review summarizes the recent studies conducted on NTDDS for AD treatment, with a primary focus on the industrial perspectives and processability. Additionally, it highlights the ongoing clinical trials for AD management.
Malaysia conducted the first BMT in the country in 1987. Since then, there have been 1155 transplantations performed in a total of eight transplant centres. A majority of the transplantations were allogeneic, including myeloablative and nonmyeloablative. A vast majority of donors are HLA identical siblings. The mean age of transplanted patients was 26 years. The major reason for transplantation was hematological malignancies. The overall survival was 60% for allogeneic transplantation and 52% for autologous transplantation. The most common cause of death in transplanted patients was the underlying disease followed by infection-related complications. Currently, the government is expanding the existing public cord blood bank as well as the local donor registry.
Matched MeSH terms: Graft vs Host Disease/etiology
A 47 year old man with a long history of chronic loud snoring and daytime sleepiness presented with hypercapnic respiratory failure and right ventricular failure. The diagnosis of obstructive sleep apnoea (OSA) leading to the 'obesity-hypoventilation syndrome', was supported by the findings of an overnight cardio-respiratory monitoring during sleep. His symptoms and arterial blood gases improved following treatment with nocturnal nasal continuous positive airway pressure (CPAP).
Prior to 1993, bone marrow transplantation for adult patients was not available in Malaysia. Adult allogeneic bone marrow transplantation commenced in Malaysia when the first transplant was conducted at the University Hospital, Kuala Lumpur on 2 November 1993. Up till July 1995, 10 adult bone marrow transplants had been conducted at the University Hospital. Five patients had acute myeloid leukaemia in first remission, 4 had chronic myeloid leukaemia and 1 had acute lymphoblastic leukaemia in first partial remission. The age range of patients at the time of transplant is 16-40 years (mean 25.5 years). All patients engrafted successfully and the survival for the first 100 days post-transplant is 90%. One patient demonstrated haematological relapse post-transplant but achieved remission with donor buffy-coat infusion. The mean drug cost incurred was RM28,269 for the first 100 days. Locally available adult allogeneic bone marrow transplantation is safe, affordable and has comparable results with reputable overseas transplant centres.
Matched MeSH terms: Graft vs Host Disease/etiology
A 3-month-old Malay male infant presented with multiple infections (candidiasis, Pseudomonas aeruginosa, Cytomegalovirus), persistent pneumonia, intractable diarrhoea and failure to thrive. There was lymphopaenia affecting both T and B subsets. He developed Graft versus Host disease weeks following transfusion with non irradiated blood. In spite of aggressive microbicidal and supportive therapy including regular immunoglobulin infusions, the child succumbed to infection before a bone marrow transplant could be instituted.
Matched MeSH terms: Graft vs Host Disease/etiology
Between June 1980 and April 1986 796 cases of female sterilization were performed in a private clinic in Malaysia using Mark IVa and Mark VI Filshie clips. There was one failure. Technical failure, surgical difficulties and complications were minimal.
There is growing interest in treating inflammatory conditions with helminth infection. Recently, Loukas and colleagues have reported promising results from using experimental hookworm infection to reduce gluten sensitivity in celiac disease patients. Analysis of microbiota samples from the trial is contributing to our understanding of the complexity underlying helminth–microbiota–host relationships.
Kawasaki disease is primarily a condition that affects young children and it is associated with cardiac morbidity and mortality. This disease has been known to cause coronary artery aneurysms which occurs as a sequelae of vasculitis. The progression of triple vessel disease in adult which results from cardiac complications from Kawasaki disease is rare. We report a case of a young man with history of Kawasaki disease at infancy presenting with triple vessel disease requiring cardiac bypass surgery at the age of 20 years old.