Cystinosis: practical tools for diagnosis and treatment

Cystinosis is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. In older patients cystinosis can mimic idiopathic nephrotic syndrome due to focal and segmental glomerulosclerosis. Measuring elevated white blood cell cystine content is the corner stone for the diagnosis. The diagnosis is confirmed by molecular analysis of the cystinosin gene. Corneal cystine crystals are invariably present in all patients with cystinosis after the age of 1 year. Treatment with the cystine depleting drug cysteamine should be initiated as soon as possible and continued lifelong to prolong renal function survival and protect extra-renal organs. This educational feature provides practical tools for the diagnosis and treatment of cystinosis

The IAHS Science for Solutions decade, with Hydrology Engaging Local People IN one Global world (HELPING)

The new scientific decade (2023-2032) of the International Association of Hydrological Sciences (IAHS) aims at searching for sustainable solutions to undesired water conditions – whether it be too little, too much or too polluted. Many of the current issues originate from global change, while solutions to problems must embrace local understanding and context. The decade will explore the current water crises by searching for actionable knowledge within three themes: global and local interactions, sustainable solutions and innovative cross-cutting methods. We capitalise on previous IAHS Scientific Decades shaping a trilogy; from Hydrological Predictions (PUB) to Change and Interdisciplinarity (Panta Rhei) to Solutions (HELPING). The vision is to solve fundamental water-related environmental and societal problems by engaging with other disciplines and local stakeholders. The decade endorses mutual learning and co-creation to progress towards UN sustainable development goals. Hence, HELPING is a vehicle for putting science in action, driven by scientists working on local hydrology in coordination with local, regional, and global processes

Creating Community for Early-Career Geoscientists

The American Geophysical Union (AGU) and the European Geosciences Union (EGU) play central roles in nurturing the next generation of geoscientists. Students and young scientists make up about one quarter of the unions’ active memberships [American Geophysical Union, 2013; European Geosciences Union, 2014], creating a major opportunity to include a new generation of geoscientists as more active contributors to the organizations’ activities, rather than merely as consumers. Both organizations are now explicitly expanding their bottom-up organizational structures to include early-career members (ECMs) by appointing student (AGU) and early-career scientist (EGU) representatives for their scientific divisions. (We refer to “early-career members” because AGU and EGU define student and postdoc members differently). Because this expansion is a recent development, it is still unclear what roles these representatives will play and how these roles will evolve over the coming years. We are ECMs in the hydrological sciences. Here we show how the Young Hydrological Society (YHS) used bottom-up initiatives, aligned closely with the newly appointed AGU and EGU representatives, to help improve the professional development of student and postdoc members by providing opportunities to increase their contributions to the geoscience unions. We call for a conversation on how ECMs can make the best use of these new opportunities to engage proactively with the unions.Water ManagementCivil Engineering and Geoscience

Cross-cultural consistency and relativity in the enjoyment of thinking versus doing

© 2018 American Psychological Association. Which is more enjoyable: trying to think enjoyable thoughts or doing everyday solitary activities? Wilson et al. (2014) found that American participants much preferred solitary everyday activities, such as reading or watching TV, to thinking for pleasure. To see whether this preference generalized outside of the United States, we replicated the study with 2,557 participants from 12 sites in 11 countries. The results were consistent in every country: Participants randomly assigned to do something reported significantly greater enjoyment than did participants randomly assigned to think for pleasure. Although we found systematic differences by country in how much participants enjoyed thinking for pleasure, we used a series of nested structural equation models to show that these differences were fully accounted for by country-level variation in 5 individual differences, 4 of which were positively correlated with thinking for pleasure (need for cognition, openness to experience, meditation experience, and initial positive affect) and 1 of which was negatively correlated (reported phone usage).status: publishe