In the treatment of refractory epilepsy,especially in Dravet syndrome and Lennox-Gastaut syndrome,cannabidiol,an anticonvulsant component of marijuana,has shown significant efficacy and safety.However,its mechanism of action and interaction with other drugs are still unclear.For refractory patients who have been treated with a variety of antiepileptic drugs,cannabidiol makes their treatment more promising but also more complicated.This review focuses on the mechanism of cannabidiol and its clinical research progress in the treatment of refractory epilepsy.

Cholesterol gallstone formation represents a failure of biliary cholesterol homeostasis in which the physical-chemical balance of cholesterol solubility in bile is disturbed.Lithogenic bile is mainly caused by persistent hepatic hypersecretion of biliary cholesterol and sustained cholesterol-supersaturated bile is an essential prerequisite for the precipitation of solid cholesterol monohydrate crystals and the forma-tion of cholesterol gallstones.The metabolic determinants of the supply of hepatic cholesterol molecules that are recruited for biliary secretion are dependent upon the input-output balance of cholesterol and its catabolism in the liver.The sources of cholesterol for hepatic secretion into bile have been extensively investigated;however,to what extent each cholesterol source contributes to hepatic secretion is still unclear both under normal physiological conditions and in the lithogenic state.Although it has been long known that biliary lithogenicity is initiated by hepatic cholesterol hypersecretion,the genetic mecha-nisms that cause supersaturated bile have not been defined yet.Identification of the Lith genes that determine hepatic cholesterol hypersecretion should provide novel insights into the primary genetic and pathophysiological defects for gallstone formation.In this review article,we focus mainly on the path-ogenesis of the formation of supersaturated bile and gallstones from the viewpoint of genetics and pathophysiology.A better understanding of the molecular genetics and pathophysiology of the formation of cholesterol-supersaturated bile will undoubtedly facilitate the development of novel,effective,and noninvasive therapies for patients with gallstones,which would reduce the morbidity,mortality,and costs of health care associated with gallstones,a very prevalent liver disease worldwide.

Eye globe abnormalities can be readily detected on dedicated and non-dedicated CT and MR studies. A primary understanding of the globe anatomy is key to characterising both traumatic and non-traumatic globe abnormalities. The globe consists of three primary layers: the sclera (outer), uvea (middle), and retina (inner layer). The various pathological processes involving these layers are highlighted using case examples with fundoscopic correlation where appropriate. In the emergent setting, trauma can result in hemorrhage, retinal/choroidal detachment and globe rupture. Neoplasms and inflammatory/infective processes predominantly occur in the vascular middle layer. The radiologist has an important role in primary diagnosis contributing to appropriate ophthalmology referral, thereby preventing devastating consequences such as vision loss.
Adult* ; Diagnosis ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Ophthalmology ; Pathologic Processes ; Referral and Consultation ; Retina ; Rupture ; Sclera ; Uvea

Pediatric-onset multiple sclerosis is underreported because of difficulty in diagnosis and assessment. In Western series, pediatric-onset disease showed significant differences from adult-onset disease with higher female preponderance, polysymptomatic in onset, frequent systemic manifestation in relapses, higher relapse rate, but less disability, and fewer lesions in brain magnetic resonance imaging. Multiple sclerosis manifests differently in Asians, yet there was no large series of pediatric-onset multiple sclerosis reported. We found that pediatric-onset disease in Asians showed greater similarity with adult-onset disease without the reported differences in female preponderance, relapse rate, and magnetic resonance imaging findings. There were also similar proportion and clinical features in optico-spinal form, and long spinal cord lesions were common in both groups. The significant difference was less disability among the pediatric-onset group. Thus, although multiple sclerosis in Asia is different from Western countries, there is greater similarity between the pediatric-onset and adult-onset group in Asia.
Multiple Sclerosis ; Adult ; Asia ; Pediatric ; Cancer Relapse

Objective: Employment is important for patients with chronic illness, and to remain employed is a robust support to them. This study aimed to examine the employment rate and to identify factors associated with employment among multiple sclerosis (MS) in Hong Kong. Methods: A cross-sectional study was performed from 2010 to 2011 at five major public hospitals. Fifty-nine clinical definite MS patients with no evidence of dementia (Mini-Mental State Examination ≥ 22) were recruited. Demographic data and neuropsychological test results including memory, visual perception, psychological well-being, motor, executive domain and processing speed were collected. Principal component analysis and logistic regression with multiple imputation were used in data analyses. Results: The employment rate among MS patients was 56%. Patients with better cognitive functions were more likely to be employed (p=0.002). No significant association was found between employment status and age, gender, level of education, types of MS, disease duration, frequency of relapse or use of interferon. Conclusion: MS patients had high unemployment rate (44%) which was 11.5 times higher than the general population in Hong Kong. MS patients with better cognitive functions had higher employment rates.
Multiple Sclerosis

PURPOSE: Obesity is a significant risk factor for pelvic organ prolapse (POP), but the effects of obesity on outcomes of surgery for POP are poorly understood. The aim of this study was to assess the relationship between POP symptomatology, subjective outcomes of surgery and body mass index (BMI) in women undergoing vaginal hysterectomy for POP. METHODS: Pre- and postoperative data from a validated pelvic floor questionnaire (electronic Personal Assessment Questionnaire-Pelvic Floor) were collected prospectively from 60 women undergoing vaginal hysterectomy for POP. Of these, 20 were normal weight (BMI 18.5–24.9 kg/m²), 20 were overweight (BMI 25–29.9 kg/m²), and 20 were women with obesity (BMI 30–34.9 kg/m²). The relationship between BMI and symptom scores for prolapse, impact on vaginal symptoms on quality of life (VS-QoL) and ‘overall change in condition’ was assessed. Pre- and postoperative symptom scores were compared using repeated mixed analysis of variance test for BMI as a categorical variable (normal, overweight, and obese). Spearman rank order correlation test was carried out to evaluate BMI as a continuous variable. All women underwent vaginal hysterectomy using a standardized technique. RESULTS: Overall, 93% of women reported improvement in their condition. The main finding was that ‘overall change in condition’ was negatively correlated with increasing BMI (r(s)=-0.324, P=0.028). Irrespective of BMI, significant improvements were observed in symptoms of prolapse and VS-QoL at 3-month postoperation. CONCLUSIONS: With increasing BMI, women are likely to report lower levels of satisfaction following prolapse surgery, despite reporting equivalent improvements in symptoms. BMI is known to affect how individuals perceive their general health and well-being with obese individuals reporting poorer levels of subjective health status. Women with obesity may perceive change in their condition after prolapse surgery differently to women of normal weight. Reduction of weight prior to prolapse surgery could be considered in obese women to improve subjective outcomes of surgery.
Body Mass Index ; Diagnostic Self Evaluation ; Female ; Humans ; Hysterectomy ; Hysterectomy, Vaginal ; Obesity ; Overweight ; Patient Outcome Assessment ; Pelvic Floor ; Pelvic Organ Prolapse ; Prolapse ; Prospective Studies ; Quality of Life ; Risk Factors

The immunological role of megakaryocytes is not well known. This project studies the involvement of megakaryocytes on immuno-inflammatory processes and the possible mechanism via the adhesion molecule CD36 and the synthesis of relevant cytokines. The expression of adhesion protein CD36 on human platelets, megakaryocytes and megakaryocytic cell lines (Meg-01, Dami, CHRF-288-11 and M-07e) was analyzed by using flow cytometry, ELISA and immunocytochemical methods. The expression of interleukin-1 (IL-1) to interleukin-10 (IL-10), TNF-alpha, TNF-gamma and IFN-gamma on the four megakaryocytic cell lines was also determined by RT-PCR. The effect of IL-1beta, IL-3, IL-6 and TPO on murine megakaryocyte colony formation (CFU-MK) was studied by using a plasma clot culture system. The CFU-MK was confirmed by acetylcholine esterase staining. The results showed that: (1) CD36 was expressed on platelets, megakaryocytes and the four megakaryocytic cell lines, the relative expression level is as follows: platelets > megakaryocytes > Meg-01 > Dami > CHRF-288-11 > M-07e, suggesting that the level of CD36 expression correlates with the degree of maturity of megakaryocytic differentiation; (2) inflammatory cytokines TNF-alpha, IL-1beta, IL-3 and IL-6 were detected in all the four megakaryocytic cell lines, suggesting that different stages of megakaryocytes can be as a source of inflammatory cytokines; and (3) IL-1beta, IL-3 and IL-6, as well as TPO, play a stimulating effect on CFU-MK formation, suggesting that there is an "autocrine" effect on megakaryocytopoiesis. The data obtained suggest that megakaryocytes may involve in immuno-inflammatory processes via the synthesis of platelet adhesion molecules and inflammatory cytokines.

The ascidian Ciona intestinalis is a model organism of developmental and evolutionary biology and may provide crucial clues concerning two fundamental matters, namely, how chordates originated from the putative deuterostome ancestor and how advanced chordates originated from the simplest chordates. In this paper, a whole-life-span culture of C. intestinalis was conducted. Fed with the diet combination of dry Spirulina, egg yolk, Dicrateria sp., edible yeast and weaning diet for shrimp, C. intestinalis grew up to average 59 mm and matured after 60 d cultivation. This culture process could be repeated using the artificially cultured mature ascidians as material. When the fertilized eggs were maintained under 10, 15, 20, 25 degrees C, they hatched within 30 h, 22 h, 16 h and 12 h 50 min respectively experiencing cleavage, blastulation, gastrulation, neurulation, tailbud stage and tadpole stage. The tadpole larvae were characterized as typical but simplified chordates because of their dorsal nerve cord, notochord and primordial brain. After 8 - 24 h freely swimming, the tadpole larvae settled on the substrates and metamorphosized within 1- 2 d into filter feeding sessile juvenile ascidians. In addition, unfertilized eggs were successfully dechorionated in filtered seawater containing 1% Tripsin, 0.25% EDTA at pH of 10.5 within 40 min. After fertilization, the dechorionated eggs developed well and hatched at normal hatching rate. In conclusion, this paper presented feasible methodology for rearing the tadpole larvae of C. intestinalis into sexual maturity under controlled conditions and detailed observations on the embryogenesis of the laboratory cultured ascidians, which will facilitate developmental and genetic research using this model system.
Animals ; Ciona intestinalis ; growth & development ; Embryonic Development ; physiology ; Female ; Male ; Metamorphosis, Biological ; physiology ; Zygote ; growth & development

BACKGROUNDChildhood cancer survivors were at risk of development of second malignant neoplasms. The aim of this study is to evaluate the incidence, risk factors and outcome of second malignant neoplasms in childhood cancer survivors in a tertiary paediatric oncology centre in Hong Kong, China.

METHODSWe performed a retrospective review of patients with childhood cancer treated in Children's Cancer Centre in Prince of Wales Hospital, Hong Kong, China between May 1984 and June 2009. Case records of patients who developed second malignant neoplasms were reviewed.

RESULTSTotally 1374 new cases aged less than 21-year old were treated in our centre in this 25-year study period. Twelve cases developed second malignant neoplasms with 10-year and 20-year cumulative incidence of 1.3% (95% confidence interval 0.3% - 2.3%) and 2.9% (95% confidence interval 1.1% - 4.7%) respectively. Another 4 cases were referred to us from other centres for the management of second malignant neoplasms. In this cohort of 16 children with second malignant neoplasms, the most frequent second malignant neoplasms were acute leukemia or myelodysplastic syndrome (n = 6) and central nervous system tumor (n = 4). Median interval between diagnosis of primary and second malignant neoplasms was 7.4 years (range 2.1 - 13.3 years). Eight patients developed second solid tumor within the previous irradiated field. Radiotherapy significantly increased the risk of development of second solid tumor in patients with acute lymphoblastic leukemia (P = 0.027). Seven out of 16 patients who developed second malignant neoplasms had a family history of cancer among the first or second-degree relatives. Nine patients died of progression of second malignant neoplasms, mainly resulted from second central nervous system tumor and osteosarcoma.

CONCLUSIONSCumulative incidence of second cancer in our centre was comparable to western countries. Radiotherapy was associated with second solid tumour among patients with acute lymphoblastic leukemia. Patients who developed second brain tumor and osteosarcoma had a poor outcome.

Adolescent ; Adult ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Hong Kong ; epidemiology ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasms ; epidemiology ; Neoplasms, Second Primary ; epidemiology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Survivors ; statistics & numerical data ; Young Adult

BACKGROUND: Recent predictions of catastrophic epidemic surge of HIV infection in East Asia concern experts and governmental organizations. As in many other areas, countries in East Asia show diversities in their HIV epidemics, both geographically and temporally. However, they have similar regional, cultural and racial characteristics which allow them to have certain common factors. Having a clear picture of the current extent and feature of HIV/AIDS in this region is a very difficult task largely due to the fast pacing of expending epidemic and difficulty in data-sharing among countries in the region. Hence, we decided to study the epidemiologic feature of HIV/AIDS in East Asia through East Asia Network on HIV (EAN-HIV). MATERIALS AND METHODS: The epidemiological patterns of HIV/AIDS in East Asian countries were investigated by collecting data through EAN-HIV. RESULTS: The HIV/AIDS epidemic in East Asia started relatively late at mid 1980s. Since then, the number of newly infected HIV/AIDS cases has been steadily increasing with stiffer escalation in recent years. In China and Taiwan, IDU plays an important part in the swiftly growing HIV epidemics; however, in other regions like Korea, Japan, and Hong Kong, MSM (men who have sex with men) seems to be more of a problem. The major subtypes of HIV in East Asia are subtype B, C, and CRF01_AE, and rapidly evolving circulating recombinant forms (CRF) between subtypes such as CRF07_BC give dynamic change to the current status. CONCLUSION: The incidence of HIV/AIDS is rapidly increasing in East Asia. The epidemic pattern has undergone dynamic changes over time. China seems to be the leading source of HIV/AIDS epidemic in East Asia due to its large population and rapidly growing epidemics.
Asian Continental Ancestry Group ; China ; Epidemiology* ; Far East* ; HIV ; HIV Infections ; Hong Kong ; Humans ; Incidence ; Japan ; Korea ; Taiwan