Transcatheter embolization of a congenital intrahepatic arterioportal venous malformation: A case report

Congenital intrahepatic arterioportal venous malformations (APVM) are uncommon lesions. A congenital intrahepatic APVM found incidentally in a 51-year-old man during pre-operative aortography for an abdominal aortic aneurysm is reported here. This was successfully treated by transcatheter embolization of the involved hepatic artery prior to surgical repair of the aortic aneurysm.     

Role of Social,Cultural and Economic Capitals in Perceived Quality of Life Among Old Age People in Kerala,India

Objective:

To find out the relationship of collective social, economic, and cultural properties of a population on the perceived quality of life (QOL) among old age people.

Materials and Methods:

In a community-based cross-sectional study, we analyzed information on a representative sample of 900 old age (aged > 60 years) from 28 villages in Kollam district of Kerala. WHO-Quality of Life - BREF questionnaire was used. Ethical clearance from Institutional Ethics Committee was obtained. The mean scores for perceived QOL for domains such as physical health, psychological health, social relations, and control of environments were calculated. The three scales (social capital, cultural capital, and economic capital) were standardized using z-score transformation to make them comparable. Using multiple linear regression, we calculated the independent effect of economic capital, social capital, and cultural capital on perceived QOL among old people adjusted for age, sex, and the presence of chronic disease.

Results:

For overall QOL, only cultural capital contributed significantly. An increase of one unit z-score cultural capital led to three units increase in overall QOL score (β = 3.362; 95% CI: 2.645-4.078). Social capital and cultural capital contributed significantly to the physical health domain of QOL. With one z-score increase in social capital and cultural capital, QOL score of physical health domain increased by 0.2 units (β = 0. 227; 95% CI: 0.020-0.434), and 0.5 (β = 0. 596; 95% CI: 0.384-0.808) units, respectively. Psychological health domain and environmental domain were affected by all three capitals significantly. But, the social relations domain was significantly affected only by cultural capital (β = 0. 576; 95% CI: 0.373-0.779).

Conclusion:

Hence, the policies for old people should envision retaining our cultural and social norms along with the economic interventions for a better palliative care.     

Muir–Torre syndrome-associated pleomorphic liposarcoma arising in a previous radiation field

Muir–Torre syndrome is a variant of Lynch syndrome, characterised by sebaceous neoplasia and/or keratoacanthomas associated with visceral malignancies. Muir–Torre syndrome is caused by germline mutations of one of the mismatch repair genes, frequently MSH2 and less frequently MLH1 and MSH6. Visceral malignancies associated with Muir–Torre syndrome and Lynch syndrome include colorectal, endometrial and other gastrointestinal, urological and gynaecological malignancies. Small numbers of Lynch syndrome-associated soft tissue sarcomas have been reported, but there are no reported cases of soft tissue sarcomas in Muir–Torre syndrome. In this study, we report a 74-year-old man with known Muir–Torre syndrome with confirmed MSH2 germline mutation, diagnosed with pleomorphic liposarcoma of the right buttock in a previous radiation field. The tumour showed loss of expression of MSH2 and MSH6 on immunohistochemistry. Immunohistochemistry on another pleomorphic liposarcoma in a different patient with no previous history of Muir–Torre syndrome or Lynch syndrome showed no loss of expression of mismatch repair proteins. This is the first report of Muir–Torre syndrome-associated sarcoma and the first case of post-radiation sarcoma in Lynch syndrome.     

呼吸中枢化学反应性、发作性睡病与HLA-DQB1*0602基因

呼吸中枢低氧反应性的高低受遗传的调控,尽管动物实验发现了多个候选基因,但目前尚未见人体相关基因的报告.发作性睡病(narcolepsy)是一种少见睡眠疾病,以难以控制的嗜睡、发作性猝倒、睡瘫、入睡幻觉及夜间睡眠紊乱为主要临床特点,超过95%的典型患者存在人类白细胞抗原(HLA)DQB1*0602基因阳性、脑脊液下丘脑分泌素降低或消失等表现.患者常合并睡眠呼吸暂停,动物实验结果显示下丘脑分泌素缺乏可导致清醒状态下的呼吸中枢高CO2反应性降低,提示发作性睡病患者存在呼吸调节功能缺陷.