Pseudohypoparathyroidism, an often delayed diagnosis: a case series

Pseudohypoparathyroidism refers to a heterogeneous group of disorders characterized by parathyroid hormone (PTH) resistance. Pseudohypoparathyroidism is an uncommon sporadic or inherited genetic disorder subdivided into several distinct entities (type Ia, Ib, Ic, type II). We report cases of four children (aged 8 to 13 years) in the winter season 2007-'08. The present work highlights the variable mode of presentation of pseudohypoparathyroidism and the difficulty of an early diagnosis. We stress the importance of a complete biochemical investigation of the calcium-phosphate metabolism to recognize typical biochemical alterations associated with this condition (hypocalcaemia, hyperphosphataemia with increased phosphate tubular reabsorption and elevated PTH levels) in spite of a phenotypic aspect that often lacks the presence of all the peculiar clinical features of Albright hereditary osteodistrophy

Comparison of Surgical Outcomes between Canaloplasty and Schlemm’s Canal Scaffold at 24 Months’ Follow-Up

The results of canaloplasty (CP) and Hydrus Microstent (HM) implantation were retrospectively compared at 24 months' follow-up in a cohort of subjects referred to our Institution for uncontrolled IOP in primary or secondary (e.g., pseudoexfoliative and pigmentary) open-angle glaucoma. The outcome was labelled as "complete" success, "qualified" success, or "failure" if, two years after surgery, the eyes operated on needed "no" hypotensive medications, "some" hypotensive medications, or further glaucoma surgery to attain the target IOP, respectively. Both CP and HM implant allowed significant IOP reductions, with comparable rate of clinical success and safety profile. A slightly (albeit not significant) better trend for a "complete" clinical success was observed in the CP group

Manufacturing reshoring and sustainable development goals: A home versus host country perspective

After decades of offshoring their manufacturing activities, an increasing number of companies are revising their location strategies and implementing reshoring decisions, including backshoring (relocation in the home country) and nearshoring (relocation in the home region) alternatives. It has been recognized that reshoring strategies are consistent with the sustainable production approach, since they allow companies to produce goods in a manner that is socially beneficial, economically viable, and environmentally less harmful over the whole life cycle of those goods. Additionally, there are early indications that reshoring can also promote and support sustainable consumption approaches. Consequently, reshoring has a wide range of impacts in both the home and the host countries, also in terms of the Sustainable Development Goals (SDGs). However, this topic has received little attention in the extant reshoring literature. This paper seeks to contribute to the discussion by adopting a two-step approach. Initially, we analyze the very few contributions available on this topic; then, we identify and discuss which of the 17 SDGs are impacted by reshoring decisions at both the home and the host country level. It emerges that reshoring decisions have several and differentiated impacts in terms of SDGs. In general, these impacts are positive for home countries and negative for host ones. For this reason, a trade-off emerges when a single relocation decision is taken and implemented. Based on this evidence, implications for scholars, managers and policy makers are presented and discussed

Improvement of spatial contrast sensitivity threshold after surgical reduction of intraocular pressure in unilateral high-tension glaucoma.

PUKPOSE. To measure the effect of a surgical reduction of IOP on the spatial contrast sensitivity threshold in eyes showing a considerably increased IOP but no glaucomatous visual field defect, on white-on-white computer-assisted static perimetry. METHODS. Prospective clinical trial, lasting 36 months; 10 consecutive subjects with untreated IOP ≥ 30 mm Hg in one eye and <18 mm Hg in the fellow eye, no evidence of field damage in both eyes, best corrected visual acuity ≥ 20/20 in both eyes, and scheduled for a primary trabeculectomy in the eye showing a high IOP. The spatial contrast sensitivity threshold was measured before surgery and at each follow-up visit. RESULTS. Preoperative spatial contrast sensitivity was worse in those eyes bearing a high IOP relative to the normal fellow eyes (paired samples t-test, P < 0.0005). An improvement of contrast sensitivity threshold, exceeding the 95% confidence limits of the preoperative test-retest variability, was observed at 3, 6, and 12 cyc/deg in each surgical eye at the end of follow-up. No change was observed in the fellow untreated normal eyes. The improvement correlated directly with the amount of decrease in pressure obtained by surgery. CONCLUSIONS. Eyes with no field defects on white-on-white computer-assisted static perimetry, but bearing a IOP ≥ 30 mm Hg, show a decreased spatial contrast sensitivity. A surgically obtained reduction of IOP is paralleled by an improvement of spatial contrast sensitivity

Design of Virtual Hands for Natural Interaction in the Metaverse

[EN] The emergence of the Metaverse is raising important questions in the field of human-machine interaction that must be addressed for a successful implementation of the new paradigm. Therefore, the exploration and integration of both technology and human interaction within this new framework are needed. This paper describes an innovative and technically viable proposal for virtual shopping in the fashion field. Virtual hands directly scanned from the real world have been integrated, after a retopology process, in a virtual environment created for the Metaverse, and have been integrated with digital nails. Human interaction with the Metaverse has been carried out through the acquisition of the real posture of the user's hands using an infrared-based sensor and mapping it in its virtualized version, achieving natural identification. The technique has been successfully tested in an immersive shopping experience with the Meta Quest 2 headset as a pilot project, where a transactions mechanism based on the blockchain technology (non-fungible tokens, NFTs) has allowed for the development of a feasible solution for massive audiences. The consumers' reactions were extremely positive, with a total of 250 in-person participants and 120 remote accesses to the Metaverse. Very interesting technical guidelines are raised in this project, the resolution of which may be useful for future implementations.This research is part of the R+D project PID2020-118021RB-I00, funded by MICIN/AEI/10.13039/501100011033, and R+D project UJI-B2022-48 funded by University Jaume I.Cerdá Boluda, J.; Mora, MC.; Lloret Romero, MN.; Scarani, S.; Sastre, J. (2024). Design of Virtual Hands for Natural Interaction in the Metaverse. Sensors. 24(3). https://doi.org/10.3390/s2403074124

IgA anti-Actin antibodies in children with celiac disease: comparison of immunofluorescence with Elisa assay in predicting severe intestinal damage

<p>Abstract</p> <p>Background</p> <p>Previous studies have demonstrated that the presence of serum IgA antibodies against actin filaments (AAA) in patients with celiac disease (CD) is strongly associated with mucosal damage and severe degrees of villous atrophy.</p> <p>The aims of the present study were (1) to verify the effectiveness of IgA-AAA in newly diagnosed CD patients in a clinical setting (2) to compare the immunofluorescence assay with ELISA assay; (3) to compare the correlation of our IgA anti-tissue transglutaminase antibodies (tTG-Ab) class with mucosal intestinal lesions.</p> <p>Methods</p> <p>90 patients underwent endoscopy and multiple biopsies for suspected CD on the basis of symptoms, in presence of positive tTG-Ab tests. Twenty biopsied and 25 not-biopsied subjects with negative tTG-Ab were tested as control groups.</p> <p>IgA-AAA assays were performed by indirect immunofluorescence using rat epithelial intestinal cells, and by ELISA with a commercial kit. tTG-Ab assay was a radio-binding assay.</p> <p>Intestinal specimens were collected by upper endoscopy and the histological study was done according to the Marsh's classification modified by Oberhuber (M/O). Auto-antibodies assays and histological evaluation have been performed blindly by skilled operators.</p> <p>Results</p> <p>CD diagnosis was confirmed in 82 patients (type I M/O in 2 patients, IIIA in 18 patients, IIIB in 29 patients and IIIC in 33 patients). Two patients with type 1 lesion in presence of positive tTG-Ab and abdominal complaints, started a gluten free diet.</p> <p>The rate of IgA-AAA positivity (sensitivity) by IFI and ELISA in histologically proven celiac disease patients, were 5.5% and 25% patients in IIIA, 27.5% and 34.4% patients in IIIB, 78.8% and 75% in IIIC patients, respectively.</p> <p>Patients with normal or nearly normal mucosa, regardless of tTG-Ab status, presented negative IgA-AAA IFI assay. On the other hand, 1 patient with normal mucosa but positive tTG-Ab, also presented positive IgA-AAA ELISA. All healthy non biopsied controls had negative IgA-AAA. tTG-Ab serum concentration was significantly correlated with more severe intestinal lesion (IIIB, IIIC M/O).</p> <p>Conclusions</p> <p>IgA-AAA may be undetectable in presence of severe mucosal damage. Histology is still necessary to diagnose celiac disease and IgA-AAA cannot be included in usual screening tests, because it has little to offer if compared to the well-established tTG-Ab.</p> <p>IgA-AAA could be an adjunctive, very useful tool to support the diagnosis of CD in case of suboptimal histology, when the biopsy is to be avoided for clinical reasons, or in case of negative parents' consensus.</p

Public participation and transparency in water management

Public participation is considered broadly to have a positive impact on the quality of governance. Transparency is the first step in the public participation ladder since it implies that people have access to the necessary information to make informed contributions to decision-making. This chapter gives an overview of the main challenges for the Spanish water sector in terms of public participation in the water planning process of the Water Framework Directive (WFD), and presents results of an assessment of the information transparency of the Spanish water authorities. Although the WFD has contributed to improving the situation, in Spain the tradition of public accessibility to data and public participation in water management decisions is still rather poor. In addition to making all relevant information publicly available, the most compelling challenge is possibly ensuring its reliability and consistency. Another key issue is making the information accessible to different target audiences by adapting it to their level of interest and technical capacit

Short-term Changes in Retinal and Choroidal Relative Flow Volume after Anti-VEGF Treatment for Neovascular Age-related Macular Degeneration

The effects of anti-vascular endothelial growth factor (anti-VEGF) agents on the native ocular vasculature are poorly understood. This pilot study aimed to assess short-term changes in retinal and choroidal perfusion after anti-VEGF treatment for neovascular exudative age-related macular degeneration (nAMD) using the relative flow volume (RFV) parameter derived from laser speckle flowgraphy. Ten treatment-naïve nAMD patients underwent measurements of mean, maximum, minimum, and differential RFV within a retinal arteriolar segment and a choroidal vessel segment outside the neovascular area. Measurement of retinal RFV (rRFV), choroidal RFV (cRFV), and subfoveal choroidal thickness (SCT) was repeated 9 and 35 days after a single anti-VEGF injection. The treatment caused a statistically significant decrease in the mean rRFV, mean cRFV, and SCT during the follow-up (p &lt; 0.05). At the intermediate visit, the mean cRFV and SCT were −17.6% and −6.4% compared to baseline, respectively. However, at the final measurement, the mean cRFV was not different from the baseline value, which indicated waning of the anti-VEGF effect. In conclusion, a single anti-VEGF injection in treatment-naïve nAMD resulted in a decrease in retinal arteriolar and choroidal perfusion, according to the RFV parameter, which is a promising tool to simultaneously assess retinal and choroidal perfusion changes in response to anti-VEGF therapy

Stable manifolds under very weak hyperbolicity conditions

We present an argument for proving the existence of local stable and unstable manifolds in a general abstract setting and under very weak hyperbolicity conditions.Comment: 20 page

Visual outcome and poor prognostic factors in acute retinal necrosis syndrome

Objective: To evaluate the impact of selected clinical parameters on the mid-/long-term visual outcome of patients with acute retinal necrosis (ARN) Design: A retrospective cohort study Methods: Setting: Two University Hospitals (Parma, Italy; Lausanne, Switzerland). Participants: Thirty-nine non-HIV patients (39 eyes) with ARN, as confirmed by polymerase chain reaction on intraocular samples. The following potential predictors were tested using linear regression models: age, sex, etiology, best-corrected visual acuity (BCVA) on admission, delay between ARN symptom onset and treatment initiation, and surgery (performed or not). Main outcome: BCVA at the final follow up Results: Thirty-nine of 39 non-HIV patients (22 men and 17 women; mean age, 50 years) diagnosed with ARN were enrolled in the study. Etiologies were: varicella-zoster virus in 25 eyes (64%), herpes simplex viruses in the remaining 14 eyes. The average follow-up duration was 19 ± 13 months. All patients had undergone systemic antivirals; surgery was performed in 16 eyes. The mean delay between onset of visual symptoms and antiviral treatment initiation was 15 ± 31 days (range, 1–180 days). The mean BCVA at baseline was 0.83 ± 0.75 logMAR, while the mean final BCVA was 0.75 ± 0.81 logMAR. Both initial BCVA and treatment delay (TD) were significantly correlated with the final BCVA (p &lt; 0.05). Conclusions: Initial BCVA and TD seem to be significant predictors of mid-/long-term visual outcome in non-HIV patients affected by ARN