We conclude that ICMA is more sensitive and precise than IFMA, permitting differentiation of pubertal and prepubertal stage in boys under basal conditions. However, in girls the overlap of basal values was marked, indicating the need for the GnRH test to establish maturity of the hypothalamus-pituitary-gonadal axis.

Male patients with an extra sex chromosome or autosome are expected to present primary hypogonadism at puberty owing to meiotic germ-cell failure. Scarce information is available on trisomy 21, a frequent autosomal aneuploidy. Our objective was to assess whether trisomy 21 presents with pubertal-onset, germ-cell specific, primary hypogonadism in males, or whether the hypogonadism is established earlier and affects other testicular cell populations. We assessed the functional status of the pituitary-testicular axis, especially Sertoli cell function, in 117 boys with trisomy 21 (ages: 2months-20year). To compare with an adequate control population, we established reference levels for serum anti-Müllerian hormone (AMH) in 421 normal males, from birth to adulthood, using a recently developed ultrasensitive assay. In trisomy 21, AMH was lower than normal, indicating Sertoli cell dysfunction, from early infancy, independently of the existence of cryptorchidism. The overall prevalence rate of AMH below the 3rd percentile was 64.3% in infants with trisomy 21. Follicle-stimulating hormone was elevated in patients <6months and after pubertal onset. Testosterone was within the normal range, but luteinizing hormone was elevated in most patients <6months and after pubertal onset, indicating a mild Leydig cell dysfunction. We conclude that in trisomy 21, primary hypogonadism involves a combined dysfunction of Sertoli and Leydig cells, which can be observed independently of cryptorchidism soon after birth, thus prompting the search for new hypotheses to explain the pathophysiology of gonadal dysfunction in autosomal trisomy.

This document is a U.S. government work and is not subject to copyright in the United States. IndiSeas ("Indicators for the Seas") is a collaborative international working group that was established in 2005 to evaluate the status of exploited marine ecosystems using a suite of indicators in a comparative framework. An initial shortlist of seven ecological indicators was selected to quantify the effects of fishing on the broader ecosystem using several criteria (i.e., ecological meaning, sensitivity to fishing, data availability, management objectives and public awareness). The suite comprised: (i) the inverse coefficient of variation of total biomass of surveyed species, (ii) mean fish length in the surveyed community, (iii) mean maximum life span of surveyed fish species, (iv) proportion of predatory fish in the surveyed community, (v) proportion of under and moderately exploited stocks, (vi) total biomass of surveyed species, and (vii) mean trophic level of the landed catch. In line with the Nagoya Strategic Plan of the Convention on Biological Diversity (2011-2020), we extended this suite to emphasize the broader biodiversity and conservation risks in exploited marine ecosystems. We selected a subset of indicators from a list of empirically based candidate biodiversity indicators initially established based on ecological significance to complement the original IndiSeas indicators. The additional selected indicators were: (viii) mean intrinsic vulnerability index of the fish landed catch, (ix) proportion of non-declining exploited species in the surveyed community, (x) catch-based marine trophic index, and (xi) mean trophic level of the surveyed community. Despite the lack of data in some ecosystems, we also selected (xii) mean trophic level of the modelled community, and (xiii) proportion of discards in the fishery as extra indicators. These additional indicators were examined, along with the initial set of IndiSeas ecological indicators, to evaluate whether adding new biodiversity indicators provided useful additional information to refine our understanding of the status evaluation of 29 exploited marine ecosystems. We used state and trend analyses, and we performed correlation, redundancy and multivariate tests. Existing developments in ecosystembased fisheries management have largely focused on exploited species. Our study, using mostly fisheries independent survey-based indicators, highlights that biodiversity and conservation-based indicators are complementary to ecological indicators of fishing pressure. Thus, they should be used to provide additional information to evaluate the overall impact of fishing on exploited marine ecosystems.

Understanding how external pressures impact ecosystem structure and functioning is essential for ecosystem-based approaches to fisheries management. We quantified the relative effects of fisheries exploitation and environmental conditions on ecological indicators derived from two different data sources, fisheries catch data (catch-based) and fisheries independent survey data (survey-based) for 12 marine ecosystems using a partial least squares path modeling approach (PLS-PM). We linked these ecological indicators to the total biomass of the ecosystem. Although the effects of exploitation and environmental conditions differed across the ecosystems, some general results can be drawn from the comparative approach. Interestingly, the PLS-PM analyses showed that survey-based indicators were less tightly associated with each other than the catch-based ones. The analyses also showed that the effects of environmental conditions on the ecological indicators were predominantly significant, and tended to be negative, suggesting that in the recent period, indicators accounted for changes in environmental conditions and the changes were more likely to be adverse. Total biomass was associated with fisheries exploitation and environmental conditions; however its association with the ecological indicators was weak across the ecosystems. Knowledge of the relative influence of exploitation and environmental pressures on the dynamics within exploited ecosystems will help us to move towards ecosystem-based approaches to fisheries management. PLS-PM proved to be a useful approach to quantify the relative effects of fisheries exploitation and environmental conditions and suggest it could be used more widely in fisheries oceanography. Highlights ► We quantified the effects of fishing and environment on two groups of indicators. ► There were consistencies across 12 ecosystems in the association among indicators. ► We derived commonalities in the links among indicators, fishing and environment.

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