Wolman disease: morphological, clinical and genetic studies on the first Scandinavian cases

On the Aland Islands, a 1-month-old girl was diagnosed as having Wolman disease. The diagnosis was confirmed neurochemically; a decreased activity of acid lipase was noted in the proband and her parents had typical carrier values. This is the first Scandinavian case reported. The skin biopsy revealed cytoplasmic accumulations identical to those noted in two sibs who highly probably had Wolman disease during the 1950s. Both these sibs died at the age of about 3 months and presented a heavy accumulation of lipid material in lymph nodes, spleen, adrenal glands, liver, gut, and also some pathological alterations in other organs. Electron microscopic findings from deparaffinized samples showed cytoplasmic accumulation of lipid material similar to that noted in Wolman disease. Genealogical analyses revealed that the index families had ancestors from the same restricted area and also common ancestors during the 17th century. The parents of the two affected sibs were born on a small island and were related in many different ways. On the basis of genealogical studies and other genetic investigations performed, the importance of founder and drift effect for manifestations of rare hereditary disorder in isolates is stressed.     

Multiple sclerosis in the Shetland Islands: an update

Annual incidence rates of multiple sclerosis (MS) in the Shetland Islands have been updated from 1938 to 1986. These reveal a significant decline in MS incidence beginning between 1951 and 1968. No significant change in prevalence, age-specific prevalence, duration of MS, or mean age of the MS population in Shetland has been found since the last survey in 1974, although a trend towards an older MS population was noted.     

我国西沙群岛吸血昆虫的种群动态和鼠形动物病原感染检测

目的 研究我国西沙群岛吸血昆虫的种群动态以及鼠形动物病原感染情况,为当地传染病防控提供资料.方法 2014年1月至7月在西沙群岛的永兴岛和石岛以灯诱法诱捕昆虫,每个月2次.结合形态和分子特征对昆虫进行种类鉴定并统计种群数量.用胶体金试剂条和PCR检测鼠形动物的常见病原感染情况,依据形态学初步鉴定鼠形动物体外寄生虫.结果 西沙群岛的吸血昆虫中,库蚊、阿蚊和库蠓为优势种群,蠓在4月密度最高(55.55％,6 984/12 573),并在西沙群岛采集到贝氏司蛉(n=11).鼠形动物血清中A型金黄色葡萄球菌肠毒素和A型肉毒毒素的阳性检出率分别为3.45％(1/29)和14.00％(7/50),鼠疫和立克次体病抗体均为阴性.巢氏PCR扩增70只鼠形动物的立克次体Sta58基因片段,阳性率为11.43％(8/70).获得鼠形动物体外寄生虫共248个个体,其中革螨占93.55％(232/248).结论 吸血昆虫在西沙群岛未见明显的种群动态变化,本研究首次记述了西沙群岛的白蛉;鼠形动物中恙虫病立克次体和2种细菌的毒素携带率较高.     

Study of HLA genes in Russia Bering Island Aleuts

HLA-A, -B, -C and -DRB1 alleles and haplotypes have been studied in a group of Aleuts from Bering Island (Commander Islands, Russia). Many of their ancestors were original from other Aleutian Islands, like Attu and Atka Islands (USA) and may have had a low degree of admixture with Russians. HLA haplotypes are found to be specific and quite different from other First North America Inhabitants (including Amerindians, Na-Dene and Eskimo), as it was previously shown in a less numerous Aleut population. HLA-A*24:02 is found in a very high frequency; this character is shared by Pacific and Amerindian populations. In conclusion, HLA, other genetic markers, anthropological and linguistic traits make Aleuts to be different from First America Inhabitants and closer to Europeans and Asians: specifically Aleut relatedness has been found with Scandinavian Saami (Lapps) and Finns and Baikal Lake area Buryats, where all of them may have initialing being originated.     

Trade and the Nutrition Transition: Strengthening Policy for Health in the Pacific

This article describes pathways through which trade policy change in two Pacific Island countries has contributed to changes in the food supply, and thereby to the nutrition transition. The effect of various trade policies from 1960 to 2005 on trends in food imports and availability is described, and case studies are presented for four foods associated with the nutrition transition and chronic disease in the Pacific. Trade policies (including liberalization, export promotion, protection of the domestic meat industry and support for foreign direct investment) have contributed to a reduced availability of traditional staples, and increased availability of foods associated with the nutrition transition, including refined cereals (particularly polished rice and white flour), meat, fats and oils, and processed food products. This study suggests that promoting healthier imports and increasing production of healthier traditional foods, in both of which trade policy has an important effect, has the potential to improve diets and health, in conjunction with other public health intervention.     

Balearic adults have low intakes of fruits and vegetables compared with the dietary guidelines for adults in Spain

Nutrition is recognized as one of the major health determinants, and so a healthy diet may contribute to the delay or prevention of an important number of chronic diseases. The aim of this study was to assess the current food consumption habits of the Balearic Islands adult population. A cross-sectional nutritional survey was carried out in the Balearic Islands, Spain (2009-2010). A random sample (n = 1388) of the adult population (16-65 years) was interviewed. Dietary habits were assessed by means of a semiquantitative food frequency questionnaire, and consumption results were compared with dietary guidelines for the Spanish population. According to the dietary guidelines for the Spanish population, only consumption levels of milk and dairy products, nuts, potatoes, cereals, bread, and water were adequate. Intakes of fruit, vegetables, olive oil, eggs, and pulses were below the recommendation levels. These findings indicate that, to fulfill the dietary guidelines for the Spanish population, the Balearic Islands adult population should decrease their consumption of meat, sugar and cakes, buns, butter/margarine, and soft drinks and increase their consumption of olive oil, fruits, vegetables, fish, and pulses.     

The case for improving road safety in Pacific Islands: a population‐based study from Fiji (TRIP 6)

Objective : To estimate the incidence and demographic characteristics associated with road traffic injuries (RTIs) resulting in deaths or hospital admission for 12 hours or more in Viti Levu, Fiji. Methods : Analysis of the prospective population‐based Fiji Injury Surveillance in Hospitals database (October 2005 – September 2006). Results: Of the 374 RTI cases identified (17% of all injuries), 72% were males and one third were aged 15–29 years. RTI fatalities (10.3 per 100,000 per year) were higher among Indians compared to Fijians. Two‐thirds of deaths (largely ascribed to head, chest and abdominal trauma) occurred before hospital admission. Conclusion and implications: While the RTI fatality rate was comparable to the global average for high‐income countries, the level of motorisation in Fiji is considerably lower. To avert rising RTI rates with increasing motorisation, Fiji requires a robust road safety strategy alongside effective trauma‐care services and a reliable population‐based RTI surveillance system.     

南海官兵所驻岛礁甲、乙、丙和戊型肝炎病毒调查研究

目的了解我国南海官兵所生活岛礁的甲、乙、丙和戊型肝炎病毒分布情况。评估感染风险度,制定预防措施。方法 (1)上礁前,采集158名将上岛守礁官兵血清样本,进行常规肝炎病毒检测。(2)无菌采集上述158名上岛礁3个月后官兵的血液样本和礁上物体表面生理盐水样本390份,共548份。收集后保存于-20℃冰柜中,立即随补给船送回实验室进行乙型肝炎病毒DNA和甲、丙、戊型肝炎病毒RNA检测。结果 (1)158名守礁官兵上礁前检出血清甲肝抗体(lgG)阳性5人(3.16%);乙肝病毒表面抗原、丙肝和戊型肝炎抗体检测均为阴性。(2)上礁3个月以后,158份血清样本检出甲肝抗体阳性7份(4.43%),其中lgG阳性5份,lgM阳性2份(甲肝RNA为阳性);乙肝DNA检测有2份阳性(1.27%)。丙肝和戊型肝炎病毒RNA均为阴性。390份礁上的物体表面样本:检出甲、丙、戊肝病毒RNA阳性分别为24份(6.15%)、3份(0.77%)、2份(0.51%);乙肝病毒DNA阳性5份(1.28%)。结论守礁官兵所驻岛礁生活环境中存在甲、乙、丙、戊肝传染源,卫生部门要提高认识,有效地控制传染源,培养官兵良好的生活习惯,切断传染途径,预防守礁官兵病毒性肝炎传播和流行,并制定相应的病毒性肝炎流行治疗预案,配备治疗药品。     

Alcohol, tobacco, marijuana and hallucinogen use in Samoan adolescents

Introduction and Aims. Adolescent substance use has increased with globalisation, and yet few data exist from lower‐ and middle‐income countries and the Pacific Islands. This study examines the prevalence of three aspects of substance use over the past 30 days in Samoan adolescents: (i) use of alcohol, tobacco and other drugs; (ii) polysubstance use; and (iii) possible substance use problems. Design and Methods. A survey was administered to secondary school children (n = 879) between 12 and 19 years of age at a single school in Apia, the capital of Samoa, in August 2008. Results. There were important gender differences in substance use with boys reporting significantly higher rates of any use of each substance and polysubstance use. Boys were also three times more likely to report behaviours indicative of substance use problems. There were no significant differences in regular use of any substance with the exception of marijuana. Although the use of hallucinogens is prominent for boys and girls in the younger age group (12–15), consumption decreases with age. Boys showed substantial increases in any use of alcohol and marijuana and daily use of tobacco by age. There was also a significant increase in the number of boys reporting behaviours indicative of disordered use by age to 21% of 16‐ to 19‐year‐old boys. Discussion and Conclusions. One of few studies on substance use in Samoa, the data provide a basis for setting priorities to address health risks posed by adolescent use and understanding the influence of rapid change.[Odden HL. Alcohol, tobacco, marijuana and hallucinogen use in Samoan adolescents. Drug Alcohol Rev 2012;31:47–55]     

Alcohol, cannabis and amphetamine-type stimulants use among young Pacific Islanders

Introduction and Aims. There are many factors that impact substance use in young people in the Pacific Island Countries and Territories (PICTs). However, the extent and nature of substance use by young people in the PICTs is not clear because of a lack of data on this group. Design and Methods. A desk‐based review (including both white and grey literature) was conducted to explore substance use among young people in the Western Pacific region. This paper presents findings from the PICTs. Results. Prevalence of alcohol, cannabis and amphetamine‐type stimulants use by young people is reported from various sources—primarily based on data derived from the Youth Risk Behaviour and the Second Generation Behaviour Surveillance Surveys. Discussion and Conclusion. There appear to be evidence of risky alcohol consumption and higher levels of cannabis and amphetamine‐type stimulants use in some PICTs compared with Australia and New Zealand. However, data are generally unavailable to establish any trends. Regular, reliable and routine monitoring of patterns and trends in substance use among young people in the PICTs can assist in identifying concerns and developing evidence‐informed interventions to prevent, contain and treat current and any emerging issues.[Howard J, Ali H, Robins L. Alcohol, cannabis and amphetamine‐type stimulants use among young Pacific Islanders. Drug Alcohol Rev 2011;30;104–110]