Mortality and Malnutrition Among Populations Living in South Darfur, Sudan: Results of 3 Surveys, September 2004.

CONTEXT: Mass violence against civilians in the west of Sudan has resulted in the displacement of more than 1.5 million people (25% of the population of the Darfur region). Most of these people are camped in 142 settlements. There has been increasing international concern about the health status of the displaced population. OBJECTIVE: To perform rapid epidemiological assessments of mortality and nutritional status at 3 sites in South Darfur for relief efforts. DESIGN, SETTING, AND PARTICIPANTS: In August and September 2004, mortality surveys were conducted among 137,000 internally displaced persons (IDPs) in 3 sites in South Darfur (Kass [n = 900 households], Kalma [n = 893 households], and Muhajiria [n = 900 households]). A nutritional survey was performed concomitantly among children aged 6 to 59 months using weight for height as an index of acute malnutrition (Kass [n = 894], Kalma [n = 888], and Muhajiria [n = 896]). A questionnaire detailing access to food and basic services was administered to a subset of households (n = 210 in each site). MAIN OUTCOME MEASURES: Crude and under 5-year mortality rates and nutritional status of IDPs in Kass, Kalma, and Muhajiria, South Darfur. RESULTS: Crude mortality rates, expressed as deaths per 10,000 per day, were 3.2 (95% confidence interval [CI], 2.2-4.1) in Kass, 2.0 (95% CI, 1.3-2.7) in Kalma, and 2.3 (95% CI, 1.2-3.4) in Muhajiria. Under 5-year mortality rates were 5.9 (95% CI, 3.8-8.0) in Kass, 3.5 (95% CI, 1.5-5.7) in Kalma, and 1.0 (95% CI, 0.03-1.9) in Muhajiria. During the period of displacement covered by our survey in Muhajiria, violence was reported to be responsible for 72% of deaths, mainly among young men. Diarrheal disease was reported to cause between 25% and 47% of deaths in camp residents and mainly affected the youngest and oldest age groups. Acute malnutrition was common, affecting 14.1% of the target population in Kass, 23.6% in Kalma, and 10.7% in Muhajiria. CONCLUSION: This study provides epidemiological evidence of the high rates of mortality and malnutrition among the displaced population in South Darfur and reinforces the need to mount appropriate and timely humanitarian responses

Male Accessory Gland Protein Reduces Egg Laying in a Simultaneous Hermaphrodite

Seminal fluid is an important part of the ejaculate of internally fertilizing animals. This fluid contains substances that nourish and activate sperm for successful fertilization. Additionally, it contains components that influence female physiology to further enhance fertilization success of the sperm donor, possibly beyond the recipient's optimum. Although evidence for such substances abounds, few studies have unraveled their identities, and focus has been exclusively on separate-sex species. We present the first detailed study into the seminal fluid composition of a hermaphrodite (Lymnaea stagnalis). Eight novel peptides and proteins were identified from the seminal-fluid-producing prostate gland and tested for effects on oviposition, hatching and consumption. The gene for the protein found to suppress egg mass production, Ovipostatin, was sequenced, thereby providing the first fully-characterized seminal fluid substance in a simultaneous hermaphrodite. Thus, seminal fluid peptides and proteins have evolved and can play a crucial role in sexual selection even when the sexes are combined

A clinical-radiological framework of the right temporal variant of frontotemporal dementia

The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy has previously been described as memory loss, prosopagnosia, getting lost and behavioural changes. Accurate detection is challenging, as the clinical syndrome might be confused with either behavioural variant FTD (bvFTD) or Alzheimer’s disease. Furthermore, based on neuroimaging features, the syndrome has been considered a right-sided variant of semantic variant primary progressive aphasia (svPPA). Therefore, we aimed to demarcate the clinical and neuropsychological characteristics of rtvFTD versus svPPA, bvFTD and Alzheimer’s disease. Moreover, we aimed to compare its neuroimaging profile against svPPA, which is associated with predominant left anterior temporal atrophy. Of 619 subjects with a clinical diagnosis of frontotemporal dementia or primary progressive aphasia, we included 70 subjects with a negative amyloid status in whom predominant right temporal lobar atrophy was identified based on blinded visual assessment of their initial brain MRI scans. Clinical symptoms were assessed retrospectively and compared with age- and sex-matched patients with svPPA (n = 70), bvFTD (n = 70) and Alzheimer’s disease (n = 70). Prosopagnosia, episodic memory impairment and behavioural changes such as disinhibition, apathy, compulsiveness and loss of empathy were the most common initial symptoms, whereas during the disease course, patients developed language problems such as word-finding difficulties and anomia. Distinctive symptoms of rtvFTD compared to the other groups included depression, somatic complaints, and motor/mental slowness. Aside from right temporal atrophy, the imaging pattern showed volume loss of the right ventral frontal area and the left temporal lobe, which represented a close mirror image of svPPA. Atrophy of the bilateral temporal poles and the fusiform gyrus were associated with prosopagnosia in rtvFTD. Our results highlight that rtvFTD has a unique clinical presentation. Since current diagnostic criteria do not cover specific symptoms of the rtvFTD, we propose a diagnostic tree to be used to define diagnostic criteria and call for an international validation

Rare Genetic Variant in SORL1 May Increase Penetrance of Alzheimer's Disease in a Family with Several Generations of APOE-ɛ4 Homozygosity

BACKGROUND: The major genetic risk factor for late onset Alzheimer's disease (AD) is the APOE-ɛ4 allele. However, APOE-ɛ4 homozygosity is not fully penetrant, suggesting co-occurrence of additional genetic variants. OBJECTIVE: To identify genetic factors that, next to APOE-ɛ4 homozygosity, contribute to the development of AD. METHODS: We identified a family with nine AD patients spanning four generations, with an inheritance pattern suggestive of autosomal dominant AD, with no variants in PSEN1, PSEN2, or APP. We collected DNA from four affected and seven unaffected family members and performed exome sequencing on DNA from three affected and one unaffected family members. RESULTS: All affected family members were homozygous for the APOE-ɛ4 allele. Statistical analysis revealed that AD onset in this family was significantly earlier than could be expected based on APOE genotype and gender. Next to APOE-ɛ4 homozygosity, we found that all four affected family members carried a rare variant in the VPS10 domain of the SORL1 gene, associated with AβPP processing and AD risk. Furthermore, three of four affected family members carried a rare variant in the TSHZ3 gene, also associated with AβPP processing. Affected family members presented between 61 and 74 years, with variable presence of microbleeds/cerebral amyloid angiopathy and electroencephalographic abnormalities. CONCLUSION: We hypothesize that next to APOE-ɛ4 homozygosity, impaired SORL1 protein function, and possibly impaired TSHZ3 function, further disturbed Aβ processing. The convergence of these genetic factors over several generations might clarify the increased AD penetrance and the autosomal dominant-like inheritance pattern of AD as observed in this family

International Paediatric Mitochondrial Disease Scale

published_or_final_versio

Salience-based selection: attentional capture by distractors less salient than the target

Current accounts of attentional capture predict the most salient stimulus to be invariably selected first. However, existing salience and visual search models assume noise in the map computation or selection process. Consequently, they predict the first selection to be stochastically dependent on salience, implying that attention could even be captured first by the second most salient (instead of the most salient) stimulus in the field. Yet, capture by less salient distractors has not been reported and salience-based selection accounts claim that the distractor has to be more salient in order to capture attention. We tested this prediction using an empirical and modeling approach of the visual search distractor paradigm. For the empirical part, we manipulated salience of target and distractor parametrically and measured reaction time interference when a distractor was present compared to absent. Reaction time interference was strongly correlated with distractor salience relative to the target. Moreover, even distractors less salient than the target captured attention, as measured by reaction time interference and oculomotor capture. In the modeling part, we simulated first selection in the distractor paradigm using behavioral measures of salience and considering the time course of selection including noise. We were able to replicate the result pattern we obtained in the empirical part. We conclude that each salience value follows a specific selection time distribution and attentional capture occurs when the selection time distributions of target and distractor overlap. Hence, selection is stochastic in nature and attentional capture occurs with a certain probability depending on relative salience

Association of Ventricular Arrhythmias With Dementia: The Atherosclerosis Risk in Communities (ARIC) Study

Objective We performed a cross-sectional analysis to determine whether nonsustained ventricular tachycardia (NSVT) and premature ventricular contractions (PVCs) were associated with dementia in a population-based study. Methods We included 2,517 (mean age 79 years, 26% Black) participants who wore a 2-week ambulatory continuous ECG recording device in 2016 to 2017. NSVT was defined as a wide-complex tachycardia ≥4 beats with a rate >100 bpm. We calculated NSVT and PVC burden as the number of episodes per day. Dementia was adjudicated by experts. We used logistic regression to assess the associations of NSVT and PVCs with dementia. Results The mean recording time of the Zio XT Patch was 12.6 ± 2.6 days. There were 768 (31%) participants with NSVT; prevalence was similar in White and Black participants. There were 134 (6.5%) dementia cases (5% in White, 10% in Black participants). After multivariable adjustment, there was no overall association between NSVT and dementia; however, there was a significant race interaction (p < 0.001). In Black participants, NSVT was associated with a 3.67 times higher adjusted odds of dementia (95% confidence interval [CI] 1.92-7.02) compared to those without NSVT, whereas in White participants NSVT was not associated with dementia (odds ratio [95% CI] 0.64 [0.37-1.10]). In Black participants only, a higher burden of PVCs was associated with dementia. Conclusions Presence of NSVT and a higher burden of NSVT and PVCs are associated with dementia in elderly Black people. Further research to confirm this novel finding and to elucidate the underlying mechanisms is warranted