Despite international waters covering over 60% of the world's oceans, understanding of how fisheries in these regions shape ecosystem processes is surprisingly poor. Seabirds forage at fishing ...vessels, which has potentially deleterious effects for their population, but the extent of overlap and behavior in relation to ships is poorly known. Using novel biologging devices, which detect radar emissions and record the position of boats and seabirds, we measured the true extent of the overlap between seabirds and fishing vessels and generated estimates of the intensity of fishing and distribution of vessels in international waters. During breeding, wandering albatrosses (Diomedea exulans) from the Crozet Islands patrolled an area of over 10 million km² at distances up to 2500 km from the colony. Up to 79.5% of loggers attached to birds detected vessels. The extent of overlap between albatrosses and fisheries has widespread implications for bycatch risk in seabirds and reveals the areas of intense fishing throughout the ocean. We suggest that seabirds equipped with radar detectors are excellent monitors of the presence of vessels in the Southern Ocean and offer a new way to monitor the presence of illegal fisheries and to better understand the impact of fisheries on seabirds. A pesar de que las aguas internacionales cubren más del 60% de los océanos, el conocimiento sobre cómo las pesquerías en estas regiones le dan forma a los procesos del ecosistema es sorprendentemente pobre. Las aves marinas buscan alimento en los navíos de pesca, lo que presenta efectos potencialmente dañinos para su población. Aun así, la extensión de la coincidencia en el tiempo y el comportamiento en relación con los barcos no se conoce ampliamente. Con el uso de aparatos novedosos de bioregistro, los cuales detectan emisiones de radar y registran la posición de los barcos y las aves marinas, medimos la verdadera extensión de la coincidencia en el tiempo entre las aves marinas y los navíos de pesca, y generamos estimaciones de la intensidad de pesca y distribución de los navíos en aguas internacionales. Durante la época de reproducción, los albatros errantes (Diomedea exulans) de las Islas Crozet patrullaron un área de más de 10 millones de km² a distancias de hasta 2500 km desde la colonia. Hasta el 79.5% de los registradores de datos adjuntos a las aves detectaron navios. La extensión de la coincidencia en el tiempo entre los albatros y las pesquerías tiene implicaciones amplias para el riesgo de captura accesoria de aves marinas y revela las áreas de pesca intensa en el océano. Sugerimos que las aves marinas equipadas con detectores de radar son monitores excelentes de la presencia de navíos en el océano del sur y ofrecen una nueva manera de monitorear la presencia de pesca ilegal y de mejorar el entendimiento del impacto de la pesca sobre las aves marinas.
Pompe disease is a rare metabolic disorder, due to mutations in the gene encoding acid alpha-glucosidase (GAA), of which infantile and late-onset forms may occur. Aim of the work was to analyze ...clinical and laboratory data of a cohort of late-onset Pompe disease (LOPD) patients, collected during the last 15 years and to point out unusual phenotypic/genotypic features as well as enzyme replacement therapy (ERT) responses. We diagnosed 30 LOPD patients; at follow-up, they underwent motor, respiratory, cardiac and muscle MRI evaluations. Motor performances were tested by Walton Gardner-Medwin, GSGC and 6MWT tests. Respiratory function was assessed as FVC % in upright/supine position. LOPD presentations were represented by presymptomatic hyperCKemia (37 %), proximal/axial muscle weakness (53 %) and respiratory impairment (10 %). Median diagnostic delay was 8.6 years (±8.8). Atypical features were observed in 4 patients: marked distal muscle weakness and severe hearing loss at onset, as well as leukoencephalopathy and mesial temporal sclerosis during the disease course. By
GAA
sequence analysis, two causing mutations were detected in 22/30 patients, only one in the remaining 8 subjects. Overall, 29/30 patients harbored the common c.−32−13T>G mutation (2 were homozygous). Two new DNA variations were discovered (c.2395C>G, c.1771C>T). 14 patients received ERT for up to 60 months. Our study confirms LOPD clinical and genetic heterogeneity: atypical features may contribute to expand the clinical phenotype highlighting its multi-systemic nature. A timely diagnosis could allow early ERT start. An accurate follow-up is recommended to evaluate treatment responses.
The risk of seabird–fishery interactions in the Western and Central Pacific Ocean (WCPO) was examined by analysing the overlap of seabird distributions with tuna and swordfish pelagic longline ...fisheries managed by the Western and Central Pacific Fisheries Commission (WCPFC) and its constituent members. The study used spatially-explicit Productivity–Susceptibility Analysis (PSA). Key data inputs were species productivity, fishing effort, likelihood of capture and species density by region. The outputs tailored results to the needs of fisheries- and wildlife-managers, indicating areas of greatest risk of species interactions, species of greatest concern for population impacts, and the flags or fisheries most likely to contribute to the risk. Large albatross species were found to be most likely to suffer population effects when exposed to longline fishing activity, followed by the larger petrels from the genuses Procellaria, Macronectes and Pterodroma. A mixture of coastal states with nesting seabird populations in their Exclusive Economic Zones (New Zealand, Australia and United States of America), distant water fishing nations (Japan, Taiwan) and flags of convenience (Vanuatu) contributed 90% of the risk to seabird populations. Recommendations include enhancing the level of fisheries observer monitoring in areas indicated as high to medium risk for seabird interactions, and consideration of spatial management tools, such as more intensive or more stringent seabird bycatch mitigation requirements in high- to medium-risk areas. The methods used, and similar studies conducted in the Atlantic Ocean could lead to improved targeting of monitoring resources, and greater specificity in the needs for seabird-mitigation measures. This will assist in reducing seabird mortality in longline fishing operations and with more effective use of resources for fishery managers in both domestic fisheries and RFMOs.
► Methods were developed to assess risk of non-target species-fishery interactions. ► Among seabirds studied, albatrosses were most vulnerable to longline fisheries effects. ► Risk was concentrated in temperate areas and varied seasonally by area. ► Six distant water fishing nations and coastal states contributed 90% of the risk. ► Targetting of risk reduction to specific areas and seasons is now possible.
Pompe disease is an autosomal recessive disorder in which deficiency of the lysosomal enzyme acid alpha-glucosidase results in the accumulation of glycogen mostly in muscle tissues. Several reports ...suggest a higher incidence of intracranial vascular abnormalities (IVAs) in this condition, as well as brain microbleeds and cerebral vasculopathy. The aim of our study was to evaluate through neuroimaging studies the incidence of these anomalies in our cohort of late-onset Pompe disease (LOPD) patients asymptomatic for cerebrovascular disease, looking for correlations with clinical and genetic data. We studied 18 LOPD patients with brain magnetic resonance angiography (MRA), or contrast-enhanced computed tomography (CECT). Diameters of individual arteries were measured and compared with average values as proposed in the literature. We found IVAs in 13 of the 18 patients, mostly dilatative arteriopathy affecting the vertebrobasilar system. The anterior circle was involved in seven of the 18 patients. The diameter of the basilar artery at 1 cm was found to correlate both with age (spearman rho,
p
= 0.037) and disease duration (
p
= 0.004), but no other statistically significant correlation was documented. The incidence of intracranial dilatative arteriopathy in LOPD was higher than in the general population, confirming the literature data. However, we did not find intracranial aneurysms microbleeds or significant cerebrovascular disease. Abnormalities in the anterior and the posterior circle of Willis correlated with age and disease duration, but not with the severity of muscle/respiratory involvement or with genetic data. Further studies in larger cohorts of patients are needed to confirm these findings.
A multicentre observational study was aimed to assess the prevalence of late-onset Pompe disease (LOPD) in a large high-risk population, using the dried blood spot (DBS) as a main screening tool.
17 ...Italian neuromuscular centres were involved in the late-onset Pompe early diagnosis (LOPED) study. Inclusion criteria were: (1) age ≥5 years, (2) persistent hyperCKaemia and (3) muscle weakness at upper and/or lower limbs (limb-girdle muscle weakness, LGMW). Acid α-glucosidase (GAA) activity was measured separately on DBS by fluorometric as well as tandem mass spectrometry methods. A DBS retest was performed in patients resulted positive at first assay. For the final diagnosis, GAA deficiency was confirmed by a biochemical assay in skeletal muscle, whereas genotype was assessed by GAA molecular analysis.
In a 14-month period, we studied 1051 cases: 30 positive samples (2.9%) were detected by first DBS screening, whereas, after retesting, 21 samples were still positive. Biochemical and molecular genetic studies finally confirmed LOPD diagnosis in 17 cases (1.6%). The median time from the onset of symptoms/signs to diagnosis was 5 years. Among those patients, 35% showed presymptomatic hyperCKaemia and 59% showed hyperCKaemia+LGMW, whereas 6% manifested with LGMW.
LOPED study suggests that GAA activity should be accurately screened by DBS in all patients referring for isolated hyperCKaemia and/or LGMW. A timely diagnosis was performed in five patients with presymptomatic hyperCKaemia, but two had already manifested with relevant changes on muscle morphology and MRI. Consequently, enzyme replacement therapy was started in 14/17 patients, including the 2 patients still clinically presymptomatic but with a laboratory evidence of disease progression.
Although the flesh-footed shearwater Puffinus carneipes is a species with large population sizes (tens of thousands of breeding pairs) and widespread sub-tropical distribution across Australasian ...water masses, it is among the species most threatened by longline fisheries mortality in this region. While bycatch mitigation measures have been very successful in reducing mortality in some species, bycatch of flesh-footed shearwaters is still high, with captures estimated to exceed the sustainable take of 514 birds yr−1 by nearly 200 birds for New Zealand fisheries alone. Management agencies aiming to reduce the impact of fisheries mortality on the populations need to understand which marine areas are being used by flesh-footed shearwaters to better target fishery monitoring and mitigation efforts. Foraging studies of seabirds tell us about their use of resources, i.e. the way species segregate the available habitat and help to identify threats that may affect population viability. Breeding shearwaters were tracked from 2 New Zealand colonies using GPS loggers. Individuals foraged over shelf and deep oceanic waters up to 1200 km from their nesting sites during incubation but were mainly within 370 km during early chick rearing. The intensity of potential interactions increased for trawl and surface longline fishing between the January and February study periods but remained at a similar level for bottom longline fishing. Following the field data collection, changes to fishery monitoring were implemented in the areas where shearwaters foraged.
Background: In adult glycogen storage disease type II (GSDII), a single‐gene mutation causes reduction of the lysosomal enzyme acid alpha‐glucosidse. This produces a chronic proximal myopathy with ...respiratory involvement. Enzyme replacement treatment (ERT) has recently become available and is expected to improve muscle strength. This should result in increased lean body mass. In this study we evaluate body composition and nutritional status in GSDII, and assess whether these parameters changed during treatment.
Methods: Seventeen patients with late‐onset GSDII, aged 52.6 ± 16.8 years, received ERT for >18 months. Dietary habits and metabolic profiles of glucids, lipids, and proteins were assessed. Body composition was calculated using anthropometry and bioelectrical impedence analysis.
Results: On inclusion, we found increased fat mass (FM) in five patients in severe disease stage; all had normal body mass index (BMI). FM correlated inversely, and lean mass (LM) directly, with creatine kinase, prealbumin and albumin levels. After treatment, BMI and FM significantly increased, while LM only showed a trend toward increase. Prealbumin and albumin levels increased as early as after the first months of ERT.
Discussion: Body mass index value may underestimate FM in patients in severe stage of disease, due to altered body composition. In severely affected patients, laboratory parameters revealed a relative protein malnutrition, that was reversed by ERT, this reflecting restoration of normal muscle metabolic pathways. Increased BMI may indicate a reduction in energy consumption during exercise or respiration, along with clinical improvement.
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