Familial hematuria

Hematuria is a common presenting complaint in pediatric nephrology clinics and often has a familial basis. This teaching article provides an overview of causes, diagnosis, and management of the major forms of familial hematuria, Alport syndrome, and thin basement membrane nephropathy

The Impact of Small and Large Impoundments on Freshwater Mussel Distribution in the Hawkesbury-Nepean River, Southeastern Australia

The influence of weirs on the distribution of freshwater mussels was investigated in the Hawkesbury-Nepean River, Australia. Distribution of species and densities of size classes were strongly correlated with catchment level factors (e.g. location around a major impoundment, stream order). At catchment scale, weir height, presence of a fish barrier, fish ladder type and position above or below small weirs did not influence the presence/absence of mussel populations. Lower mussel densities in the upper catchment may therefore reflect inhibition of host fish migration. Where present, weir height and geomorphic reach type were linked to differences in densities among species. Geomorphic reach-based differences were reflected by the Hyridella species, but not Velesunio ambiguus. When population structure was described by size class distribution, there were significant differences between densities of small and medium mussels from weirs above, compared to weirs below, a major impoundment, but not for large mussels. Upstream populations may therefore be functionally extinct. Distribution of mussel size classes differed among geomorphic reach types with highest densities for each class found in the least human-impacted reaches. Small mussels were almost invariably found below the major impoundment, most frequently below weirs. Distribution patterns were inconsistent across species, suggesting habitat preference. V. ambiguus and Hyridella australis were most abundant in shale reaches, where assemblages were influenced by fish ladder type. Hyridella depressa and H. australis dominated in sandstone gorges and straights with assemblage density related to weir height. In upper catchment sandstone reaches, mussel assemblages comprising predominantly V. ambiguus were influenced by fish ladder type and weir height. While multiple factors defined localized distribution, large impoundments were linked with reduced population densities. The probable mechanism is the restriction of host fish movement and resulting lack of recruitment. In the Hawkesbury-Nepean River, smaller weirs also seriously impacted recruitment

Interpreting the real-time dynamic ‘sliding sign’ and predicting pouch of Douglas obliteration: an interobserver, intraobserver, diagnostic-accuracy and learning-curve study

Objective: To determine inter- and intraobserver agreement, diagnostic accuracy and the learning curve required for interpreting the ‘sliding sign’ and predicting pouch of Douglas (POD) obliteration. Methods: This was an inter-/intraobserver, diagnostic-accuracy and learning-curve study involving six observers with different medical backgrounds, clinical skill sets and prior gynecological ultrasound experience: five non-specialist observers who had performed 0–750 previous gynecological scans and an expert sonologist who had performed > 15 000. Following a formal theoretical and practical training session, they each viewed 64 offline transvaginal ultrasound (TVS) ‘sliding-sign’ videos from two anatomical locations (retrocervix and posterior uterine fundus (PUF)) in 32 women presenting with chronic pelvic pain, interpreting the videos as positive or negative for sliding sign and predicting, on that basis, the POD status. For intraobserver agreement analysis they re-analyzed the same video sets, in a different order, at least 7 days later. The expert sonologist was the reference standard for interpreting the sliding sign and the gold standard, laparoscopy, was used for the POD analysis. Learning-curve cumulative summation (LC-CUSUM) tests were conducted to assess if observer performance reached acceptable levels, using LC-CUSUM score < −2.45 as a cut-off. Results: With respect to interpretation of the sliding sign, the overall multiple-rater agreement was moderate (Fleiss' kappa, K = 0.499). Observers were more consistent in their interpretation of the second compared with the first observation set (K = 0.547 vs 0.453) and for the retrocervical compared with the PUF region (K = 0.556 vs 0.346). Regarding prediction of POD status, the accuracy, sensitivity, specificity and positive and negative predictive values for individual observers ranged from 65.4 to 96.2%, 80.0 to 100%, 64.7 to 100%, 50.0 to 100% and 94.7 to 100%, respectively. Using LC-CUSUM score < −2.45, the observer with experience of 200 previous gynecological scans reached acceptable levels for predicting POD obliteration and interpreting the sliding sign at each region (retrocervix and PUF) at 39, 54 and 28 videos and the observer with experience of 750 scans at 56, 53 and 53 videos. Conclusions: Performance of a minimum number of gynecological ultrasound examinations is necessary for interpreting offline videos of the real-time dynamic sliding sign and predicting POD obliteration. Non-specialist observers with prior experience of 200 or more gynecological scans were more consistent in interpreting the sliding sign at the retrocervix vs PUF

Ponds and the importance of their history: an audit of pond numbers, turnover and the relationship between the origins of ponds and their contemporary plant communities in south-east Northumberland, UK

An increasing focus of interest in ponds over the last two decades arose largely because of concerns at the loss of ponds in intensively developed landscapes. In the UK, pond numbers declined from approximately 800,000 in the nineteenth century to 200,000 by the 1980s. Since then pond numbers have started to increase. The focus on overall pond numbers overlooks the importance of the history and origins of different pond types. This study combines a detailed map based audit of pond numbers in south-east Northumberland, UK, recorded at seven time intervals since the mid nineteenth century with a survey of contemporary plant communities in ponds with known and distinct histories to examine changes to numbers of ponds and communities associated with ponds with different origins. 222 ponds were recorded in the study area in the midnineteenth century, 257 in 2005/08. However, only 23 of the original ponds had survived with substantial losses and gains at all the map survey dates linked to changed land use from agriculture to coal mining then development of nature reserves and golf courses. Contemporary ponds on nature reserves, golf courses and subsidence ponds supported rather different plant communities to each other, with non-native invasives in golf and nature reserve sites, whilst individual reserves differed from one another perhaps due to intentional planting. Surviving old farm ponds were usually degraded. The results show that the history of ponds in a region can create an important cultural biodiversity which pond conservation strategies should incorporate

Bivalves in a bottleneck: taxonomy, phylogeography and conservation of freshwater mussels (Bivalvia: Unionoida) in Australasia

The conservation biology of Australasian freshwater mussels is hindered by lack of a taxonomic framework that employs molecular data as a complement to shell characters, larval forms and internal anatomy. The fauna includes more than 32 known species (30+ Hyriidae, 2 Unionidae), but has not been revised for 55 years, despite minor amendments. The hyriids are relics of Gondwana, represented in Australia and New Guinea by the ancestral Velesunioninae and in Australia and New Zealand by the Hyriinae (Tribe Hyridellini). Many taxonomic and phylogeographic issues await resolution, including the relationships between Australasian and South American species, and between Australian and New Zealand species, and the status of species in New Guinea (including uncertain reports of Unionidae) and the Solomon Islands. Once these are clarified, it will be easier to identify threatened species and evaluate the conservation status of the fauna. At present, only seven taxa are named in the IUCN Red List or under national/state legislation, and these are not representative. Threatening processes include altered flow regimes, catchment disturbances, salinisation, pollution and invasive species. While the need for a taxonomic revision is paramount, progress in conservation may depend also upon involving the wider community