Langerhans Cell Histiocytosis (LCH) is an uncommon neoplastic disorder characterized by accumulation of histiocytes in various tissues. The clinical manifestation is highly variable, ranging from an isolated skin rash or a single bony lesion, to fatal multi-organ failure. Due to its rarity and systemic involvement, the epidemiology of LCH is still not fully understood and most studies focus on the paediatric population. The extent of clinical involvement has important prognostic implications. Treatment options may be local or systemic depending on disease extent. Here we describe a rare case of adult onset LCH who presented with recurrent pneumothoraces, diabetes insipidus and papules and plaques over the scalp and forehead.
We conducted a meta-analysis in order to investigate the relationships between PTEN gene mutations and the prognosis in glioma. The following electronic databases were searched for relevant articles without any language restrictions: Web of Science (1945 ~ 2013), the Cochrane Library Database (Issue 12, 2013), PubMed (1966 ~ 2013), EMBASE (1980 ~ 2013), CINAHL (1982 ~ 2013), and the Chinese Biomedical Database (CBM) (1982 ~ 2013). Meta-analyses were conducted using the STATA software (Version 12.0, Stata Corporation, College Station, Texas USA). Hazard ratio (HR) with its corresponding 95 % confidence interval (95%CI) was calculated. Six independent cohort studies with a total of 357 glioma patients met our inclusion criteria. Our meta-analysis results indicated that glioma patients with PTEN gene mutations exhibited a significantly shorter overall survival (OS) than those without PTEN gene mutations (HR = 3.66, 95%CI = 2.02 ~ 5.30, P < 0.001). Ethnicity-stratified subgroup analysis demonstrated that PTEN gene mutations were closely linked to poor prognosis in glioma among Americans (HR = 3.72, 95%CI = 1.72 ~ 5.73, P < 0.001), while similar correlations were not observed among populations in Sweden, Italy, and Malaysia (all P > 0.05). Our meta-analysis provides direct and strong evidences for the speculation of PTEN gene mutations' correlation with poor prognosis of glioma patients.
INTRODUCTION: Paraquat is a quaternary nitrogen herbicide which is highly toxic to human. Death is usually from respiratory failure and may occur within days up to a month after exposure. It is easily available and commonly abused to commit suicide.
METHODOLOGY: This is a retrospective study describing the demographic characteristics, clinical features and outcomes of paraquat poisoning cases admitted to Hospital Taiping from 1st January 2008 to 30th October 2011. Medical records of 79 patients were reviewed.
RESULT: Majority were of the Indian ethnicity (72.2%) followed by Chinese (13.9%) and Malay (10.1%). Majority was male (73.4%) and between 20 to 29 years old (34.2%). The median age of the patients was 30 years old. The mean length of stay was 6.2 days. Most exposures were intentional (69.6%) and presented to the hospital early at less than 6 hours after exposure (72.2%). Patients with positive urine paraquat result had significantly higher mortality rate compared to patients with negative results (47.4% vs 15.2% respectively). We found that neither hemofiltration nor immunosuppressive therapies help to improve survival.
CONCLUSION: The non-survivor characteristics of patients with paraquat poisoning are intentional exposure, delay from exposure to hospital admission, urine paraquat positivity and manifestation of respiratory failure. The demographic characteristics, reasons for exposure and mortality rate are similar to previous reports. Urine paraquat may be used to assess severity of the exposure as well as prognosis. Hemofiltration and immunosuppression therapy do not improve patients' survival and paraquat remains a lethal killer.
BACKGROUND: In Asia, up to 25% of breast cancer patients present with distant metastases at diagnosis. Given the heterogeneous survival probabilities of de novo metastatic breast cancer, individual outcome prediction is challenging. The aim of the study is to identify existing prognostic models for patients with de novo metastatic breast cancer and validate them in Asia.
MATERIALS AND METHODS: We performed a systematic review to identify prediction models for metastatic breast cancer. Models were validated in 642 women with de novo metastatic breast cancer registered between 2000 and 2010 in the Singapore Malaysia Hospital Based Breast Cancer Registry. Survival curves for low, intermediate and high-risk groups according to each prognostic score were compared by log-rank test and discrimination of the models was assessed by concordance statistic (C-statistic).
RESULTS: We identified 16 prediction models, seven of which were for patients with brain metastases only. Performance status, estrogen receptor status, metastatic site(s) and disease-free interval were the most common predictors. We were able to validate nine prediction models. The capacity of the models to discriminate between poor and good survivors varied from poor to fair with C-statistics ranging from 0.50 (95% CI, 0.48-0.53) to 0.63 (95% CI, 0.60-0.66).
CONCLUSION: The discriminatory performance of existing prediction models for de novo metastatic breast cancer in Asia is modest. Development of an Asian-specific prediction model is needed to improve prognostication and guide decision making.
Involvement of a multidisciplinary team in cancer care may have added benefits over the existing system of patient management. A paradigm shift in the current patient management would allow more focus on nutritional support, in addition to clinical care. Malnutrition, a common problem in cancer patients, needs special attention from the early days of cancer care to improve quality of life and treatment outcomes. Patient management teams with trained oncology dietitians may provide quality personalized nutritional care to cancer patients.
The Gross Motor Function Classification System (GMFCS) was developed to establish uniform communication between healthcare providers, patients, and the patients' families. It is also used to prognosticate the outcome of motor function. Based on previous reports, prognostication of ambulation status in cerebral palsy is based on the motor development curve, which shows a plateau at a certain known age.
The modified Rodnan skin score is widely accepted as a validated tool to assess skin involvement in systemic sclerosis, which is a hallmark of this heterogeneous disease. Ultrasonography is increasingly being utilized in the study of other rheumatic diseases. The utility of ultrasonography to measure skin thickness in systemic sclerosis has been explored since three decades ago. The aim of this review was to examine the validity of ultrasonography as an outcome measure of skin involvement in systemic sclerosis. Original articles in English, published before December 2010, pertaining to the use of B mode ultrasound assessing skin involvement in systemic sclerosis were reviewed. Data were extracted with a focus on criterion and construct validity, reproducibility and responsiveness to change. Seventeen papers were analyzed. Skin thickness was most commonly studied, although skin echogenicity has also been examined. There was heterogeneity with regards to subjects, definitions used and sites imaged. Although there was limited information regarding reliability, when reported, the results showed excellent reproducibility. There was also a lack of construct and criterion validity and evidence for sensitivity to change. Ultrasound has potential as an outcome measure in systemic sclerosis. However, more work needs to be done in order to prove that it is a feasible outcome measure with proven validity.
The incidence and mortality of breast cancer continues to rise rapidly in Asian countries. However, most of our current knowledge on breast cancer has been generated in Western populations. As the socio-economic profile, life style and culture of Asian and Western women are substantially different, and genetic backgrounds vary to some extent, we need to answer the question on whether to 'adopt' or 'adapt' Western knowledge before applying it in the Asian setting. It is generally accepted that breast cancer risk factors, which have mainly been studied in Western populations are similar worldwide. However, the presence of gene-environment or gene-gene interactions may alter their importance as causal factors across populations. Diagnostic and prognostic study findings, including breast cancer prediction rules, are increasingly shown to be 'setting specific' and must therefore be validated in Asian women before implementing them in clinical care in Asia. Interventional research findings from Caucasian patients may not be applicable in patients in Asia due to differences in tumour biology/profiles, metabolism of drugs and also health beliefs which can influence treatment acceptance and adherence. While breast cancer research in Asia is warranted in all domains of medical research, it is felt that for Asian breast cancer patients, needs are highest for diagnostic and prognostic studies. International clinical trials meanwhile need to include breast cancer patients from various Asian settings to provide an insight into the effectiveness of new treatment modalities in this part of the world.
The incidence of oral cancer is high for those of Indian ethnic origin in Malaysia. Various clinical and pathological data are usually used in oral cancer prognosis. However, due to time, cost and tissue limitations, the number of prognosis variables need to be reduced. In this research, we demonstrated the use of feature selection methods to select a subset of variables that is highly predictive of oral cancer prognosis. The objective is to reduce the number of input variables, thus to identify the key clinicopathologic (input) variables of oral cancer prognosis based on the data collected in the Malaysian scenario. Two feature selection methods, genetic algorithm (wrapper approach) and Pearson's correlation coefficient (filter approach) were implemented and compared with single-input models and a full-input model. The results showed that the reduced models with feature selection method are able to produce more accurate prognosis results than the full-input model and single-input model, with the Pearson's correlation coefficient achieving the most promising results.
Breast cancer is the leading cause of cancer mortality among women in Malaysia. Delayed diagnosis is preventable and has major effects on patients' prognosis and survival. The objectives of our study were to identify the magnitude of delayed diagnosis and its associated factors in women with breast cancer in Malaysia.
Apoptosis is an ordered and orchestrated cellular process that occurs in physiological and pathological conditions. It is also one of the most studied topics among cell biologists. An understanding of the underlying mechanism of apoptosis is important as it plays a pivotal role in the pathogenesis of many diseases. In some, the problem is due to too much apoptosis, such as in the case of degenerative diseases while in others, too little apoptosis is the culprit. Cancer is one of the scenarios where too little apoptosis occurs, resulting in malignant cells that will not die. The mechanism of apoptosis is complex and involves many pathways. Defects can occur at any point along these pathways, leading to malignant transformation of the affected cells, tumour metastasis and resistance to anticancer drugs. Despite being the cause of problem, apoptosis plays an important role in the treatment of cancer as it is a popular target of many treatment strategies. The abundance of literature suggests that targeting apoptosis in cancer is feasible. However, many troubling questions arise with the use of new drugs or treatment strategies that are designed to enhance apoptosis and critical tests must be passed before they can be used safely in human subjects.
This study describes weight changes experienced by Malaysian women with breast cancer. Women with breast cancer (n=368) were recruited from eight hospitals and four breast cancer support groups in Peninsular Malaysia. Current weight was measured and weight at the time of diagnosis and a year preceding diagnosis were based on self-reports. Change in weight was determined from the year preceding breast cancer diagnosis to study entry (time 1), at the time of diagnosis to study entry (time 2) and from a year preceding breast cancer diagnosis to the time of diagnosis (time 3). Current body mass index, at a year preceding diagnosis and at the time of diagnosis were determined. Waist circumference was also measured. The sample comprised 57% Malay, 34% Chinese and 9.8% Indian women. The mean age of the women was 54 ∓ 9.04 years and over 80% were post-menopausal. Majority of the women were in stage I and stage II breast cancer at the time of diagnosis. The most common treatments received by these women were chemotherapy followed by radiotherapy and mastectomy. Overweight and obesity were prevalent in over 40% of the survivors at all three periods. Significant weight changes were observed during time 1 (-0.74 ∓ 4.78kg, p< 0.001), time 2 (2.73 ∓ 8.06kg, p< 0.001) and time 3 (3.47 ∓ 7.53kg, p< 0.001). At time 1, almost 50% showed no changes in their weight. At time 2, nearly two-thirds had gained weight and at time 3, 69% had gained weight, abdominal obesity was observed in nearly two-thirds of the women at study entry. A significant difference in weight change among age groups was observed in time 2 and time 3. All ethnic groups had significant weight change in time 1 and time 2. Significant weight gain was observed in relation to body mass index prior to diagnosis, at diagnosis and at study entry. However, no significant difference in weight change by educational level, family history of cancer and cancer stages were observed in all 3 periods. In conclusion, significant weight gain was evident in this sample of women after diagnosis of breast cancer and treatment. Women with breast cancer should be encouraged to maintain normal body mass index and waist circumference through appropriate diet and regular physical activity which may help to reduce their risk of recurrence, secondary cancer and metastasis.
This is a prospective study to determine the severity of disability and prognosis of acute stroke patients related to their Body Mass index (BMI). A total of 79 consecutive CT-scan-proven acute stroke patients who were admitted to Hospital Tuanku Ja'afar, Seremban between November 2006 and April 2007 were recruited (male:female 49:30; mean age 62.7 years; ischemic stroke 70, intracerebral bleed 9). The patients were divided according to BMI less than 25 (Group A) and equal or greater than 25 (Group B). Severity of disability was measured between 24-48 hours by modified Rankin's score. Patients were followed up after one month. Thirty-seven patients had severe disability (Rankin Score 5). Twenty-nine patients had adverse outcomes including 11 deaths and 18 rehospitalizations or prolonged hospital/nursing home stay. 34.3% of Group B had severe disability compared to 56.8% of Group A (chi2 P = 0.046). Conversely 42.9% of Group B had adverse events at one month compared to 31.8% of Group A (chi2 P = 0.312). There were no statistical differences between high- and low-BMI groups for gender ratio, smoking, hypertension, diabetes, prior cardiovascular disease, mean age, mean lipid profile and blood pressure. When comparing patients with Rankin Score 1-4 versus 5, age and BMI were statistically significant between the two groups. By multivariate analysis only age is independent predictor for severe disability (P < 0.05). The results of this pilot study should be confirmed in larger prospective multicentre trial.
A five-month-old Indian girl, product of consanguineous marriage, presented with diarrhoea with an onset within two days after birth, severe malnutrition and metabolic acidosis. The diarrhoea persisted even with lactose-free formula, amino acid-based formula and glucose-containing oral rehydration solution, but stopped when fasted. She required prolonged parenteral nutrition. Fructose and glucose tolerance tests were performed, confirming the child was able to absorb and metabolize fructose but not glucose, indicating a diagnosis of glucose-galactose malabsorption. This case illustrate how simple and pertinent clinical observations and laboratory investigations is sufficient to allow a firm diagnosis to be made.
Catheter ablation has come to play an important therapeutic role in the management of some patients with ventricular arrhythmias. An important advance in catheter ablation of ventricular tachycardia (VT) has been the development of three-dimensional catheter-based mapping systems, which permit identification of the tachycardia circuit and facilitate a strategy for catheter ablation. As a result, patients who suffer from "focal" forms of VT have been identified. This can have implications with respect to underlying arrhythmia mechanism, patient prognosis, and therapeutic strategies. The article reviews some insights learned from catheter ablation of focal forms of VT.
There are few data on paediatric melioidosis in endemic areas outside rural north-eastern Thailand and northern Australia. This study reports 16 culture-confirmed cases of melioidosis in children aged < or = 15 years seen between 1976 and 2005 at an urban teaching hospital in Kuala Lumpur, Malaysia. Seven (43.8%) patients had septicaemic melioidosis (with three known deaths) and nine (56.2%) had localised disease (one death). Eleven (68.8%) patients had underlying diseases, including five with haematological malignancies. Skin, soft tissue and lymph nodes were most commonly affected. There were no cases of parotitis or pharyngocervical disease (seen in Thailand), or encephalomyelitis (seen in Australia). The differences in disease seen in this study compared with the mostly rural patients described in previous studies might be owing to a different patient population in an urban environment. Septicaemic melioidosis has a high mortality, but localised disease has a good prognosis, and selected cases may be cured without the full recommended treatment regimen.
Pre-eclampsia or pregnancy induced hypertension (PIH) affects 6-8% of all pregnancies. Although the underlying mechanism of PIH is still unknown, it is widely believed that the placenta plays an important role. It was thought that an ischemic placenta due to poor perfusion can precipitate the signs and symptoms of PIH. This study aims to investigate the possible role of Type 1(AT1) and Type 2 (AT2) angiotensin II receptor subtypes in the mechanism of PIH. AT1 receptor stimulation causes vasoconstriction and AT2 receptor stimulation causes vasodilatation. Investigating the interactions of these two receptors in the placenta provides an insight as to the balance that may exist between AT1 and AT2 receptors in normal pregnancy. Any disruption to the balance might cause a disruption of the blood flow in the placenta, leading to PIH. Placentas were collected from 11 PIH patients and 11 normal patients. Immunohistochemistry techniques were performed on the placental tissue to determine the distribution of AT1 and AT2 receptors in the placental tissue qualitatively and quantitatively. It was observed that in normal patients, the balance between AT1 and AT2 receptors is that the level of AT2 receptors is higher than the level of AT1 receptors. However in the PIH patient, it was observed that the normal balance was disrupted. In PIH patients the level of AT1 receptors was observed to be higher than the level of AT2 receptors. This study suggests that disruption of the balance between AT1 and AT2 receptors observed in PIH placentas might cause a decrease in blood flow to the placenta, causing it to be poorly perfused. This may cause placental ischemia which may lead to PIH.
Haemophagocytic syndrome (HPS) should be included in the differential diagnosis of pyrexia of unknown origin (PUO). The hallmark of HPS is the accumulation of activated macrophages that engulf haematopoietic cells in the reticuloendothelial system. We describe a patient with unexplained fever in which a final diagnosis of HPS was established in a bone marrow study.
Extrinsic allergic alveolitis (also known as hypersensitivity pneumonitis) is caused by repeated inhalation of mainly organic antigens by sensitized subjects. This induces a hypersensitivity response in the distal bronchioles and alveoli and subjects may present clinically with a variety of symptoms. The aims of this review are to describe the current concepts of the immunological response, the diverse clinical presentation of this disease, the relevant investigations and management, and areas for future studies.
Although Asian people have the lowest incidence and mortality rates of prostate cancer in the world, these rates have risen rapidly in the past two decades in most Asian countries. Prostate cancer has become one of the leading male cancers in some Asian countries. In 2000, the age-adjusted incidence was over 10 per 100000 men in Japan, Taiwan, Singapore, Malaysia, the Philippines and Israel. Although some of the increases may result from enhanced detection, much of the increased incidence may be associated with westernization of the lifestyle, with increasing obesity and increased consumption of fat. The differences in incidences between native Americans and Asian immigrants are getting smaller, reflecting a possible improvement of diagnostic efforts and changes of environmental risk factors in Asian immigrants. Nevertheless, the huge variations in incidences among ethnic groups imply that there are important genetic risk factors. The stage distributions of prostate cancer in Asian populations are still unfavorable compared to those of Western developed countries. However, a trend towards diagnosing cancer with more favorable prognosis is seen in most Asian countries. Both genetic and environmental risk factors responsible for elevated risks in Asian people are being identified, which may help to reduce prostate cancer incidence in a chemopreventive setting.