Background
Gaucher disease (GD) manifests heterogeneously and other conditions are often misdiagnosed in its place, leading to diagnostic delays. The Gaucher Earlier Diagnosis Consensus (GED‐C) ...initiative proposed a point‐scoring system (PSS) based on the signs and covariables that are most indicative of GD to help clinicians identify which individuals to test for GD.
Aims
To validate the PSS retrospectively in a test population including patients with GD and other conditions with overlapping manifestations.
Methods
Four cohorts of adults with GD, liver disease, haematological malignancy or immune thrombocytopenia were identified from hospital records. Clinical data were audited for GED‐C factors identified as potentially indicative of GD and aggregate scores calculated (sum of scores/number of factors) based on published PSS weightings. Threshold discriminatory PSS scores, sensitivity and specificity were determined by receiver‐operating characteristic analysis.
Results
Among 100 patients (GD, n = 25; non‐GD, n = 75), analyses based on 11 possible factors estimated group mean (standard deviation) PSS scores of: GD (n = 14), 1.08 (0.25); non‐GD (n = 38), 0.58 (0.31). Mean between‐group difference (95% confidence interval) was −0.49 (−0.68, −0.31) and area under the receiver‐operating characteristic analysis curve (95% confidence interval) was 0.88 (0.78, 0.97). A threshold PSS score of 0.82 identified all 14 patients with GD in the analysis set (100% sensitivity) and 27 of 38 patients in the non‐GD group (71% specificity). Patients with liver disease and haematological malignancy were most likely to have manifestations overlapping GD.
Conclusions
Preliminary validation of the GED‐C PSS discriminated effectively between patients with GD and those with overlapping signs.
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
The 2018 conservation status assessment of freshwater invertebrate taxa was the most comprehensive to date. Of the Threatened and At Risk taxa in 2018 (177 taxa), 48 were listed as Nationally ...Critical, 14 as Nationally Endangered, 16 as Nationally Vulnerable, 10 as Declining, and 89 as Naturally Uncommon. Most taxa were listed as either Not Threatened (315 taxa) or required more information for assessment (Data Deficient; 178 taxa). Analyses of temporal changes in conservation status since 2002 revealed that the majority of changes were due to greater understanding of taxa distributions, rather than gains or losses in population sizes or area of occupancy. Insufficient data on taxa distributions, along with a lack of autecological information, are two of the major knowledge gaps facing freshwater invertebrate conservation in New Zealand. Further impediments to conservation include (i) a lack of systematics research, (ii) a large number of species remain undiscovered and undescribed, and (iii) the status and trend of populations is unknown for most taxa. We also provide an analysis of the habitats that harbour many of the rarer taxa. Finally, we conclude by recommending a number of actions that should, collectively, help fill these knowledge gaps and address these issues.
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BFBNIB, GIS, IJS, IZUM, KILJ, KISLJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Riccia (Ricciaceae) is a widespread, cosmopolitan genus of thalloid liverworts. Here we document the presence of R. cavernosa Hoffm. in New Zealand for the first time. A full description based on New ...Zealand specimens is provided. Riccia cavernosa was discovered in New Zealand in February 2016 growing on the margins of a series of artificial wetlands constructed to aid in the recovery of a threatened endemic wading bird (Himantopus novaezelandiae). Following a review of the status of R. cavernosa in New Zealand we conclude that the species is most likely a recent natural, long distance trans-Tasman wind-dispersed arrival from Australia where the species is locally common. The discovery increases the number of Riccia reported from New Zealand from 10 to 11. Prior to the discovery of R. cavernosa, only four of the 10 Riccia recorded from New Zealand were considered indigenous. The conservation status of R. cavernosa is briefly discussed and a provisional assessment of 'Threatened/Nationally Critical' assigned to the species using the New Zealand Threat Classification System.
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BFBNIB, GIS, IJS, KISLJ, NUK, PNG, UL, UM, UPUK
A new species of the New Zealand endemic grass
,
is described. The new species is segregated from and compared with
and
.
occurs mostly in lightly shaded areas of seasonally dry alluvial forest. A ...distribution map and an assessment of the conservation status of the new species are presented. Genetic variation in the genus was examined, building on previously published work but including additional sampling. Analysis of nrDNA ITS and ETS and plastid
L intron and
L-F intergenic spacer sequences show
to be more closely related to
than to
. NeighborNet analyses of AFLP profiles for the three species of
show each to consist of distinct clusters of genotypes well separated from each other.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Source: National Library of New Zealand Te Puna Matauranga o Aotearoa, licensed by the Department of Internal Affairs for re-use under the Creative Commons Attribution 3.0 New Zealand Licence.
A new species of the New Zealand endemic grass
Simplicia
,
Simplicia
felix
is described. The new species is segregated from and compared with
Simplicia
buchananii
and
Simplicia
laxa
.
Simplicia
felix
...occurs mostly in lightly shaded areas of seasonally dry alluvial forest. A distribution map and an assessment of the conservation status of the new species are presented. Genetic variation in the genus was examined, building on previously published work but including additional sampling. Analysis of
nrDNA
ITS and ETS and plastid
trn
L intron and
trn
L–F intergenic spacer sequences show
Simplicia
felix
to be more closely related to
Simplicia
laxa
than to
Simplicia
buchananii
. NeighborNet analyses of AFLP profiles for the three species of
Simplicia
show each to consist of distinct clusters of genotypes well separated from each other.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
The 2018 conservation status assessment of freshwater invertebrate taxa was the most comprehensive to date. Of the Threatened and At Risk taxa in 2018 (177 taxa), 48 were listed as Nationally ...Critical, 14 as Nationally Endangered, 16 as Nationally Vulnerable, 10 as Declining, and 89 as Naturally Uncommon. Most taxa were listed as either Not Threatened (315 taxa) or required more information for assessment (Data Deficient; 178 taxa). Analyses of temporal changes in conservation status since 2002 revealed that the majority of changes were due to greater understanding of taxa distributions, rather than gains or losses in population sizes or area of occupancy. Insufficient data on taxa distributions, along with a lack of autecological information, are two of the major knowledge gaps facing freshwater invertebrate conservation in New Zealand. Further impediments to conservation include (i) a lack of systematics research, (ii) a large number of species remain undiscovered and undescribed, and (iii) the status and trend of populations is unknown for most taxa. We also provide an analysis of the habitats that harbour many of the rarer taxa. Finally, we conclude by recommending a number of actions that should, collectively, help fill these knowledge gaps and address these issues.
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BFBNIB, GIS, IJS, IZUM, KILJ, KISLJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Cerebral small vessel disease is a common cause of vascular cognitive impairment in older people, with no licensed treatment. Cerebral blood flow is reduced in small vessel disease. Tadalafil is a ...widely prescribed phosphodiesterase-5 inhibitor that increases blood flow in other vascular territories. The aim of this trial is to test the hypothesis that tadalafil increases cerebral blood flow in older people with small vessel disease.
Perfusion by Arterial Spin labelling following Single dose Tadalafil In Small vessel disease (PASTIS) is a phase II randomised double-blind crossover trial. In two visits, 7-30 days apart, participants undergo arterial spin labelling to measure cerebral blood flow and a battery of cognitive tests, pre- and post-dosing with oral tadalafil (20 mg) or placebo.
54 participants are required to detect a 15% increase in cerebral blood flow in subcortical white matter (p < 0.05, 90% power). Primary outcomes are cerebral blood flow in subcortical white matter and deep grey nuclei. Secondary outcomes are cortical grey matter cerebral blood flow and performance on cognitive tests (reaction time, information processing speed, digit span forwards and backwards, semantic fluency).
Recruitment started on 4th September 2015 and 36 participants have completed to date (19th April 2017). No serious adverse events have occurred. All participants have been recruited from one centre, St George's University Hospitals NHS Foundation Trust.
European Union Clinical Trials Register: EudraCT number 2015-001235-20 . Registered on 13 May 2015.
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A revision of the New Zealand endemic Lepidium oleraceum and allied species is presented. Sixteen species are recognised, 10 of these are new. The new species are segregated on the basis of ...morphological characters supported by molecular data obtained from three DNA markers (two rDNA and one cpDNA). One species, Lepidium castellanum sp. nov., is endemic to the Kermadec Islands where it is sympatric with Lepidium oleraceum. The North Island of New Zealand supports four species, with two of them, Lepidium amissum sp. nov. and Lepidium obtusatum, now extinct. The South Island supports six species, that, aside from Lepidium banksii, Lepidium flexicaule and Lepidium oleraceum, are all confined to the south-eastern half of the island (Lepidium aegrum sp. nov., Lepidium crassum sp. nov. and Lepidium juvencum sp. nov.). One of these, Lepidium juvencum sp. nov., extends to Stewart Island. The Chatham Islands support six species (Lepidium flexicaule, Lepidium oblitum sp. nov., Lepidium oleraceum, Lepidium oligodontum sp. nov., Lepidium panniforme sp. nov., and Lepidium rekohuense sp. nov.), one of which, Lepidium oligodontum sp. nov., extends to the Antipodes Islands group. The remote, subantarctic Bounty Islands group supports one endemic, Lepidium seditiosum sp. nov., which is the only vascular plant to be recorded from there. Lepidium limenophylax sp. nov. is known from islands off the south-western side of Stewart Island/Rakiura, The Snares and Auckland islands. Lepidium naufragorum, although not related to Lepidium oleraceum and its allies, is also treated because populations with entire leaves are now known. Typification is undertaken for Lepidium banksii, Lepidium oleraceum, Lepidium oleraceum var. acutidentatum, var. frondosum and var. serrulatum.
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