Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery?

Primary hyperparathyroidism is a common endocrinological disorder. In rare circumstances, it is associated with familial syndromes, such as multiple endocrine neoplasia type 1. This syndrome is caused by a germline mutation in the multiple endocrine neoplasia type 1 gene encoding the tumor-suppressor protein menin. Usually, primary hyperparathyroidism is the initial clinical expression in carriers of multiple endocrine neoplasia type 1 mutations, occurring in more than 90% of patients and appearing at a young age (20-25 years). Multiple endocrine neoplasia type 1/primary hyperparathyroidism is generally accompanied by multiglandular disease, clinically manifesting with hypercalcemia, although it can remain asymptomatic for a long time and consequently not always be recognized early. Surgery is the recommended treatment. The goal of this short review is to discuss the timing of surgery in patients when primary hyperparathyroidism is associated with multiple endocrine neoplasia type 1

Effect of Pore Size, Lubricant Viscosity, and Distribution on the Slippery Properties of Infused Cement Surfaces

The fabrication of slippery liquid-infused porous surfaces (SLIPS) usually requires the use of structured substrates, with specifically designed micro- and nanoroughness and complementary surface chemistry, ideally suited to trap lubricants. It is not yet established whether a random roughness, with a range of pores with a variable size reaching deep into the bulk of the material, is suitable for successful infusion. In this study, a highly porous material with random and complex roughness, obtained by using portland cement (the most common type of cementitious material), was tested for its potential to act as a SLIP surface. Atomic force microscopy meniscus measurements were used to investigate the distribution of lubricants on the surface upon subsequent stages of depletion because of the capillary absorption of the lubricant within the porous structure. Factors such as curing time of the cement paste, time since infusion, and lubricant viscosity were varied to identify the conditions under which infusion could be considered successful. A sensitive method to evaluate the penetration of liquid (low-temperature differential scanning calorimetry) was used, which could be applicable to many porous materials. The optimized infusion of cement surfaces ultimately resulted in the desired hallmarks of SLIPS, that is, high water repellence and slipperiness, effective for several weeks, reduced water permeability, and icephobicity

When Parathyroidectomy Should Be Indicated or Postponed in Adolescents With MEN1-Related Primary Hyperparathyroidism

Multiple Endocrine Neoplasia Type 1 (MEN1) is a rare inherited endocrine tumor syndrome principally affecting parathyroid glands, neuroendocrine tissues of the gastro-entero-pancreatic and thoracic tracts, and anterior pituitary, caused by germline inactivating mutations of the MEN1 tumor suppressor gene. Primary hyperparathyroidism (PHPT) is usually the first clinical manifestation of the syndrome, normally manifesting during the third decade of life. Cases of affected children and adolescents have been described by the age of 5. Clinical characteristics and therapeutic management of MEN1 in adolescents have been described mainly by case reports. Only two studies on MEN1 patient series under the age of 22 years have recently been published. Given the scarcity of data and the lack of a consistent number of targeted studies, there are currently no specific guidelines available for children and adolescents with MEN1; diagnostic and therapeutic management is, thus, usually the same as for adult patients. Here, we report our experience with 19 adolescent MEN1 patients, developing MEN1-associated PHPT before the age of 20. Fourteen of them, manifesting hypercalcemic PHPT before the age of 20 underwent parathyroidectomy before the age of 25 to control calcemia. Parathyroid surgery restored normal calcemia in all the operated patients. No post-surgical nephrolithiasis has been reported after a mean of 12.0 ± 5.8 years of follow-up. Comparison between pre-surgical and post-surgical values of bone mineral density (BMD) in 2 patients evidenced an improvement of bone mass after parathyroid adenoma ablation. Two patients (14.28%) developed permanent post-surgical hypoparathyroidism

Total and Subtotal Parathyroidectomy in Young Patients With Multiple Endocrine Neoplasia Type 1-Related Primary Hyperparathyroidism: Potential Post-surgical Benefits and Complications

Background: The choice of surgical treatment for patients with Multiple Endocrine Neoplasia type 1 (MEN1)-related primary hyperparathyroidism (PHPT) remains controversial and it has not been specifically addressed in young patients.Methods: This is a retrospective case series study. The study includes the surgical data and the follow-up of 38 patients younger than 30 years of age, all diagnosed with MEN1, collected and followed-up between 1991 and 2017 at the Regional Referral Center for Inherited Endocrine Tumors of the Tuscany Region, and operated by parathyroidectomy. Genetic and/or clinical MEN1 diagnosis was made before surgery in all patients. Subtotal (9/38 patients) or total parathyroidectomy with auto-transplantation (28/38 patients) were performed in all patients but one, in whom a single mediastinal adenoma was excised from the aorto-pulmonary window. All patients but one, who was operated in 2017, had a post-operatory follow-up of at least 12 months.Results: Total parathyroidectomy (TPTX), with auto-transplantation, was the most frequently adopted operation both as primary (20/38 patients) and secondary (8/38 patients) surgery, followed by subtotal parathyroidectomy (SPTX; 9/38 patients) and limited parathyroidectomy (1/38 patient). At follow-up, lasting a mean of 11.8 ± 6.6 years (range 0–23 years), no persistent PHPT was observed. PHPT recurred in 4/28 TPTX (14%) and in 2/9 SPTX (22%). Permanent hypoparathyroidism showed no statistically significant difference between the procedures (2/9 in SPTX and 5/28 in TPTX).Conclusions: Data from this retrospective study showed the efficacy of TPTX for the treatment of MEN1-PHPT, also in adolescent and young patients, showing, in our series, no risk of PHPT permanence and a longer disease-free period and, subsequently, the possibility to postpone re-intervention with respect to both limited PTX and SPTX. The risk of permanent hypoparathyroidism in TPTX was comparable to STPX, and could be mitigated over the years

Digital twin for civil engineering systems: an exploratory review for distributed sensing updating

We live in an environment of ever-growing demand for transport networks, which also have ageing infrastructure. However, it is not feasible to replace all the infrastructural assets that have surpassed their service lives. The commonly established alternative is increasing their durability by means of Structural Health Monitoring (SHM)-based maintenance and serviceability. Amongst the multitude of approaches to SHM, the Digital Twin model is gaining increasing attention. This model is a digital reconstruction (the Digital Twin) of a real-life asset (the Physical Twin) that, in contrast to other digital models, is frequently and automatically updated using data sampled by a sensor network deployed on the latter. This tool can provide infrastructure managers with functionalities to monitor and optimize their asset stock and to make informed and data-based decisions, in the context of day-to-day operative conditions and after extreme events. These data not only include sensor data, but also include regularly revalidated structural reliability indices formulated on the grounds of the frequently updated Digital Twin model. The technology can be even pushed as far as performing structural behavioral predictions and automatically compensating for them. The present exploratory review covers the key Digital Twin aspects—its usefulness, modus operandi, application, etc.—and proves the suitability of Distributed Sensing as its network sensor component.This research was funded by Fondazione CARITRO Cassa di Risparmio di Trento e Rovereto, grant number 2021.0224.Peer ReviewedPostprint (published version

Ozone-elicited secondary metabolites in shoot cultures of Melissa officinalis L.

The effects of ozone treatment (200 ppb, 3 h) on the accumulation of main secondary metabolites have been investigated in Melissa officinalis (lemon balm) aseptic shoot cultures in order to evaluate the biotechnological application of this gas for improving the yield of secondary metabolites of medicinal plants. During the treatment, we found (i) an activation of enzymes involved in phenolic metabolism [as confirmed by the increase of shikimate dehydrogenase, phenylalanine ammonia-lyase and cinnamyl alcohol dehydrogenase activities (about twofold higher than controls)], (ii) a development of cellular barriers with a higher degree of polymerization of monolignols [as indicated by the increase of lignin (+23% compared to controls)], (iii) an accumulation of phenolic compounds, in particular rosmarinic acid (about fourfold compared to control plants cultivated in filtered air) and (iv) an increase of antioxidant capacity [as documented by the improved 1,1-diphenyl-2-picrylhydrazyl radical (DPPH) scavenging activity]. The effect of ozone as elicitor of the production of secondary metabolites in lemon balm shoot cultures was dependent on the specific regime, the time of exposure and the concentration of the stressor. After the end of the treatment, we found cell death and hydrogen peroxide (H2O2) deposition concomitant with a prolonged superoxide anion-generation suggesting that a transient oxidative burst had occurre