NEW shared & interconnected ASL resources: SignStream® 3 Software; DAI 2 for web access to linguistically annotated video corpora; and a sign bank

2017 marked the release of a new version of SignStream® software, designed to facilitate linguistic analysis of ASL video. SignStream® provides an intuitive interface for labeling and time-aligning manual and non-manual components of the signing. Version 3 has many new features. For example, it enables representation of morpho-phonological information, including display of handshapes. An expanding ASL video corpus, annotated through use of SignStream®, is shared publicly on the Web. This corpus (video plus annotations) is Web-accessible—browsable, searchable, and downloadable—thanks to a new, improved version of our Data Access Interface: DAI 2. DAI 2 also offers Web access to a brand new Sign Bank, containing about 10,000 examples of about 3,000 distinct signs, as produced by up to 9 different ASL signers. This Sign Bank is also directly accessible from within SignStream®, thereby boosting the efficiency and consistency of annotation; new items can also be added to the Sign Bank. Soon to be integrated into SignStream® 3 and DAI 2 are visualizations of computer-generated analyses of the video: graphical display of eyebrow height, eye aperture, an

Severe paraneoplastic hypoglycemia secondary to a gastrointestinal stromal tumour masquerading as a stroke

We report the case of a 70-year-old previously healthy female who presented acutely to the Accident and Emergency department with left-sided vasomotor symptoms including reduced muscle tone, weakness upon walking and slurred speech. Physical examination confirmed hemiparesis with VIIth nerve palsy and profound hepatomegaly. A random glucose was low at 1.7 mmol/l, which upon correction resolved her symptoms. In hindsight, the patient recalled having had similar episodes periodically over the past 3 months to which she did not give much attention. While hospitalized, she continued having episodes of symptomatic hypoglycaemia during most nights, requiring treatment with i.v. dextrose and/or glucagon. Blood tests including insulin and C-peptide were invariably suppressed, in correlation with low glucose. A Synacthen stimulation test was normal (Cort (0′) 390 nmol/l, Cort (30′) 773 nmol/l). A computed tomography scan showed multiple lobulated masses in the abdomen, liver and pelvis. An ultrasound guided biopsy of one of the pelvic masses was performed. Immunohistochemistry supported the diagnosis of a gastrointestinal stromal tumour (GIST) positive for CD34 and CD117. A diagnosis of a non islet cell tumour hypoglycaemia (NICTH) secondary to an IGF2 secreting GIST was confirmed with further biochemical investigations (IGF2=96.5 nmol/l; IGF2:IGF1 ratio 18.9, ULN <10). Treatment with growth hormone resolved the patient's hypoglycaemic symptoms and subsequent targeted therapy with Imatinib was successful in controlling disease progression over an 8-year observation period

Metabolic phenotype of male obesity-related secondary hypogonadism pre-replacementand post-replacement therapy with intra-muscular testosterone undecanoate therapy

Aim: To explore the metabolic phenotype of obesity-related Secondary Hypogonadism (SH) in men pre- and post-replacement therapy with long-acting intramuscular (IM) testosterone undecanoate (TU). Methods: A prospective observational pilot study on metabolic effects of TU IM in male obesity-related SH (Hypogonadal [HG] group, n=13), including baseline comparisons with controls (Eugonadal [EG] group, n=15). Half the subjects (n=7 in each group) had Type 2 Diabetes Mellitus (T2D). Baseline metabolic assessment on Human Metabolism Research Unit: fasting blood samples; BodPod (body composition), and; whole-body indirect calorimetry. The HG group was treated with TU IM therapy for 6-29 months (mean 14.8-months [SD 8.7]), and assessment at the Human Metabolism Research Unit repeated. T-test comparisons were performed between baseline and follow-up data (HG group), and between baseline data (HG and EG groups). Data reported as mean (SD). Results: Overall, TU IM therapy resulted in a statistically significant improvement in HbA1C (9mmol/mol, P=0.03), with 52% improvement in HOMA%B. Improvement in glycaemic control was driven by the HG subgroup with T2D, with 18mmol/mol [P=0.02] improvement in HbA1C. Following TU IM therapy, there was a statistically significant reduction in fat mass (3.5Kg, P=0.03) and increase in lean body mass (2.9Kg, P=0.03). Lipid profiles and energy expenditure were unchanged following TU IM therapy. Comparisons between baseline data for HG and EG groups were equivalent apart from differences in testosterone, SHBG and BMR. Conclusion: In men with obesity-related SH (including a subgroup with T2D), TU IM therapy improved glycaemic control, beta cell function and body composition

Glyoxalase 1 copy number variation in patients with well differentiated gastroentero-pancreatic neuroendocrine tumours (GEP-NET)

Background: The glyoxalase-1 gene (GLO1) is a hotspot for copy-number variation (CNV) in human genomes. Increased GLO1 copy-number is associated with multidrug resistance in tumour chemotherapy, but prevalence of GLO1 CNV in gastro-entero-pancreatic neuroendocrine tumours (GEP-NET) is unknown. Methods: GLO1 copy-number variation was measured in 39 patients with GEP-NET (midgut NET, n = 25; pancreatic NET, n = 14) after curative or debulking surgical treatment. Primary tumour tissue, surrounding healthy tissue and, where applicable, additional metastatic tumour tissue were analysed, using real time qPCR. Progression and survival following surgical treatment were monitored over 4.2 ± 0.5 years. Results: In the pooled GEP-NET cohort, GLO1 copy-number in healthy tissue was 2.0 in all samples but significantly increased in primary tumour tissue in 43% of patients with pancreatic NET and in 72% of patients with midgut NET, mainly driven by significantly higher GLO1 copy-number in midgut NET. In tissue from additional metastases resection (18 midgut NET and one pancreatic NET), GLO1 copy number was also increased, compared with healthy tissue; but was not significantly different compared with primary tumour tissue. During mean 3 - 5 years follow-up, 8 patients died and 16 patients showed radiological progression. In midgut NET, a high GLO1 copy-number was associated with earlier progression. In NETs with increased GLO1 copy number, there was increased Glo1 protein expression compared to non-malignant tissue. Conclusions: GLO1 copy-number was increased in a large percentage of patients with GEP-NET and correlated positively with increased Glo1 protein in tumour tissue. Analysis of GLO1 copy-number variation particularly in patients with midgut NET could be a novel prognostic marker for tumour progression

Pinna nobilis in the Greek seas (NE Mediterranean): on the brink of extinction?

The Mediterranean endemic fan mussel Pinna nobilis is suffering an ongoing basin-scale mass mortality event (MME) since 2016. As most Mediterranean populations have collapsed, the species has been declared as Critically Endangered in the IUCN Red List of threatened species. In an effort to track the progress of the MME and provide updated information on the status of the species in the Greek seas, data collected through dedicated surveys and opportunistic assessments during 2019 and 2020 have been compiled. During surveys conducted at 258 sites, a total of 14,589 fan mussels were recorded, of which 81.1% were dead. Of the remaining 2,762 live individuals, 256 were juveniles. Two marine areas that still sustain living populations were identified, namely Kalloni Gulf (Lesvos Island), and Laganas Bay (Zakynthos Island). The inner part of Kalloni Gulf appears to maintain the largest surviving population of the species in the eastern Mediterranean, with an abundance estimate of 684,000 individuals (95% confidence interval: 322,000-1,453,000). Solitary, potentially resistant, scattered individuals were recorded at several sites. Other previously abundant populations that had been assessed in the past, specifically those of Lake Vouliagmeni (Korinthiakos Gulf), Souda Bay (Crete) and Gera Gulf (Lesvos Island) with a total of ~350,000 individuals, have now been wiped out. Our results document the collapse of most P. nobilis populations throughout the Greek seas. The MME has progressed substantially between early 2019 and mid-2020, as indicated by the increase in mortality at sites consecutively monitored multiple times. This work highlights the urgent need for continuous monitoring of surviving populations and calls for immediate implementation of an effective protection and management strategy that will ensure the persistence of surviving individuals and the production of resistant offspring

Multi-frequency, multi-sonar mapping of shallow habitats – Efficacy and management implications in the National Marine Park of Zakynthos, Greece

In this work, multibeam echosounder (MBES) and dual frequency sidescan sonar (SSS) data are combined to map the shallow (5&#8315;100 m) benthic habitats of the National Marine Park of Zakynthos (NMPZ), Greece, a Marine Protected Area (MPA). NMPZ hosts extensive prairies of the protected Mediterranean phanerogams Posidonia oceanica and Cymodocea nodosa, as well as reefs and sandbanks. Seafloor characterization is achieved using the multi-frequency acoustic backscatter of: (a) the two simultaneous frequencies of the SSS (100 and 400 kHz) and (b) the MBES (180 kHz), as well as the MBES bathymetry. Overall, these high-resolution datasets cover an area of 84 km2 with ground coverage varying from 50% to 100%. Image texture, terrain and backscatter angular response analyses are applied to the above, to extract a range of statistical features. Those have different spatial densities and so they are combined through an object-based approach based on the full-coverage 100-kHz SSS mosaic. Supervised classification is applied to data models composed of operationally meaningful combinations between the above features, reflecting single-sonar or multi-sonar mapping scenarios. Classification results are validated against a detailed expert interpretation habitat map making use of extensive ground-truth data. The relative gain of one system or one feature extraction method or another are thoroughly examined. The frequency-dependent separation of benthic habitats showcases the potentials of multi-frequency backscatter and bathymetry from different sonars, improving evidence-based interpretations of shallow benthic habitats

Split tendon transfers for the correction of spastic varus foot deformity: a case series study

<p>Abstract</p> <p>Background</p> <p>Overactivity of anterior and/or posterior tibial tendon may be a causative factor of spastic varus foot deformity. The prevalence of their dysfunction has been reported with not well defined results. Although gait analysis and dynamic electromyography provide useful information for the assessment of the patients, they are not available in every hospital. The purpose of the current study is to identify the causative muscle producing the deformity and apply the most suitable technique for its correction.</p> <p>Methods</p> <p>We retrospectively evaluated 48 consecutive ambulant patients (52 feet) with spastic paralysis due to cerebral palsy. The average age at the time of the operation was 12,4 yrs (9-18) and the mean follow-up 7,8 yrs (4-14). Eigtheen feet presented equinus hind foot deformity due to gastrocnemius and soleus shortening. According to the deformity, the feet were divided in two groups (Group I with forefoot and midfoot inversion and Group II with hindfoot varus). The deformities were flexible in all cases in both groups. Split anterior tibial tendon transfer (SPLATT) was performed in Group I (11 feet), while split posterior tibial tendon transfer (SPOTT) was performed in Group II (38 feet). In 3 feet both procedures were performed. Achilles tendon sliding lengthening (Hoke procedure) was done in 18 feet either preoperatively or concomitantly with the index procedure.</p> <p>Results</p> <p>The results in Group I, were rated according to Hoffer's clinical criteria as excellent in 8 feet and satisfactory in 3, while in Group II according to Kling's clinical criteria were rated as excellent in 20 feet, good in 14 and poor in 4. The feet with poor results presented residual varus deformity due to intraoperative technical errors.</p> <p>Conclusion</p> <p>Overactivity of the anterior tibial tendon produces inversion most prominent in the forefoot and midfoot and similarly overactivity of the posterior tibial tendon produces hindfoot varus. The deformity can be clinically unidentifiable in some cases when Achilles shortening co-exists producing foot equinus. By identifying the muscle causing the deformity and performing the appropriate technique, very satisfying results were achieved in the majority of our cases. In three feet both muscles contributed to a combined deformity and simultaneous SPLATT and SPOTT were considered necessary. For complex foot deformities where the component of cavus co-exists, supplementary procedures are required along with the index operation to obtain the best result.</p