Salt Wasting Research Articles

Experimental and numerical study on pyrolysis characteristics of organic impurities in waste salt

The efficient removal of organic impurities in waste salts is significant for their recycling and environmental protection. The pyrolysis process of organic impurities in waste salt was investigated by thermogravimetric experiment to analyze the reaction kinetics characteristics. The results indicate that the pyrolysis reaction temperature range for the present waste salt is between 641.15 K and 726.22 K. The activation energy is 177.59 kJ mol−1, and the pre-exponential factor is 2.98 × 1011 s−1. Based on this, a numerical model was established to investigate the pyrolysis performance of organic impurities in practical tubular reactor. The effects of configuration and operating parameters on reaction performance and energy consumption were revealed. It was discovered that increasing the heating rate and wall temperature can accelerate pyrolysis rate but results in higher energy consumption. To balance reaction time and energy consumption, the heating rate of 15 K/min and heating temperature of 773.15 K are recommended. The content of organic impurities and bed porosity have minimal impact on the reaction rate and energy consumption, indicating that the tubular reactor has wide applicability in various working conditions. Additionally, reducing bed porosity can improve the processing rate and daily processing capacity. This paper provides a method to analyze the pyrolysis performance of organic impurities in waste salt, which is significant for designing and selecting working conditions of practical reactor for waste salt treatment.

Salt Wasting Syndrome: Serial Cases

Hyponatremia is one of the electrolyte disturbances that can be found in patients with central nervous system disorders, the most cases of hyponatremia are the Syndrome of Inappropriate Antidiuretic Hormone/SIADH compared to hyponatremia in Cerebral Salt Wasting Syndrome/CSWS and it is not uncommon to also occur in patients undergoing chemotherapy treatment without accompanying head abnormalities are referred to as Renal Salt Wasting Syndrome (RSWS). There is difficulty differentiating these two conditions due to almost the same clinical symptoms. Evaluation of volume status and monitoring of urate excretion fraction can help differentiate SIADH from RSWS and CSWS. Management requiring prompt diagnosis is essential to prevent severe hyponatremia from substantially causing cerebral edema and thereby reducing the risk of seizures. Fluid resuscitation therapy and sodium restoration are important. This case series report injured 2 CSWS patients after head trauma with different treatments and hyponatremia due to cisplatin induced RSWS.

Sustainable production and preparative purification of thermostable alkaline α-amylase by Bacillus simplex (ON754233) employing natural deep eutectic solvent-based extractive fermentation

Using PEG-based deep eutectic solvents (PDES), the current study proposes extractive fermentation as a sustainable process integration for the production and purification of α-amylase from Bacillus simplex (ON754233). Glucose: PEG 400 outperformed five PDES in terms of tie lie length (58) and slope value (1.23) against sodium sulphatt. Apple cider pomace was used as a low-cost, sustainable carbon source to produce-amylase, with a maximum enzyme production of 2200.13 U/mL. PDES concentration (20% w/v), salt (12.75 w/v), and apple waste (2.75 g/mL) were all optimized using response surface methodology. When scaled upto 3 L benchtop bioreactor, extractive fermentation was proved to be better technology with maximum recovery of 92.4% with highest partition coefficient (3.59). The partially purified enzyme was further purified using a Sephadex G 100 followed by DEAE-Sephadex anion exchange chromatography with a purity fold of 33. The enzyme was found to be thermostable at the temperature (60 °C), remains alkaline (pH 8), and the activity was stimulated in the presence of Mg2+ ions. With SDS PAGE electrophoresis, the molecular weight was found to be around 140 kDa. Finally, the enzyme kinetics parameters were evaluated with observed Km (0.00396 mM) and Vmax (37.87 U/mL). Thus scaling up extractive fermentation entails increasing production capacity with improved extraction efficiency using green solvents.

Aqueous sodium tosylate: a sustainable medium for alkylations

A simple, efficient alkylation strategy in a recyclable aqueous medium is presented, repurposing the generated waste as a reaction medium.

Combination of central diabetes insipidus and salt wasting syndrome in a patient with severe traumatic brain injury

Combination of central diabetes insipidus and salt wasting syndrome in a patient with severe traumatic brain injury

Cerebral Salt Wasting Syndrome in an Elderly Patient with Cerebral Small Vessel Disease

Background: Cerebral salt wasting syndrome is reported in neurosurgical literature from traumatic and non-traumatic brain injuries. No cases have been reported in the medical literature secondary to cerebral small vessel disease. Introduction: Hyponatremia is a common electrolyte disturbance seen among the acute geriatric admissions with two common diagnostic entities; the secretion of inappropriate antidiuretic hormone (SIADH) and cerebral salt wasting syndrome (CSWS) that have different clinical and biochemical presentations, different pathogenesis and therapeutic approaches. Hyponatremia caused by CSWS in patients with cerebral small vessel disease (cSVD)- a prevalent condition among the elderly, can be masked in geriatric patients with concomitant fluctuating neurological deficits. Correct diagnosis is crucial to appropriate management. In this case report we describe an association between hyponatremia caused by CSWS in a patient with cSVD.

Separation and purification of nylon 54 salts from fermentation broth by an integrated process involving microfiltration, ultrafiltration, and ion exchange.

Nylon 54 is a novel, biodegradable polyamide with excellent thermal resistance and water absorption properties. It can be polymerized using bio-based cadaverine and succinic acid as monomers. Traditional separation methods isolate individual monomers from the fermentation broth through acidification or alkalization, resulting in significant amounts of waste salts; however, synchronous separation of dibasic acids and diamines has not been reported. This study investigated an integrated process for the separation and extraction of nylon 54 salts from a co-fermentation broth without acidification or alkalization. We meticulously optimized the operational parameters of the integrated process to achieve maximum separation efficiency. Following microfiltration, ultrafiltration, and decolorization, the bacterial eliminating rate was ≥99.83%, and the protein concentration was ≤40mg/L. The absorbance of the decolorized solution was ≤0.021 at 430nm, and the recovery rate of nylon 54 salt reached 97%. Then, the pretreated solution was passed through sequential chromatographic columns, which effectively removed organic acid by-products (such as acetic acid and lactic acid), SO4 2-, and NH4 + from the fermentation broth, resulting in a cadaverine yield of 98.01% and a succinic acid yield of 89.35%. Finally, by concentrating and crystallizing the eluent, the simulated fermentation broth yielded nylon 54 salt with a purity of 99.16% and a recovery rate of 58%, and the real fermentation broth yielded nylon 54 salt with a purity of 98.10% and a recovery rate of 56.21%. This integrated process offers a sustainable and environmentally friendly pathway for the complete biosynthesis of nylon 54 salt and has the potential to be extended to the preparation of other nylon salts.

Separation of fission products from high-level waste salt systems by partial crystallization: CsCl-NaCl-LiCl-KCl study

Pyroprocessing refers to a series of high-temperature molten-salt processes used to manage and recycle nuclear materials to reduce the waste volume and hazardous environmental impact of the final waste products. Fission products with high halide affinity, including cesium (Cs-137) with a half-life of 30 years, are dissolved into a molten-salt bath. The separation and recovery of cesium can benefit waste-stream minimization because Cs-137—a strong gamma-ray emitter—is a potential source of environmental contamination. This work proposes the recovery of Cs+ from a chloride-salt matrix using a thermally controlled solid-liquid separation. Thermodynamic calculations for the CsCl-NaCl-LiCl-KCl system were performed to predict the partitioning of Cs+ into liquid and solid phases as functions of salt composition and temperature. The selected composition simulated the formulation of high-level salt waste from used fuel treatment. These calculations showed that once the solidus temperature was reached, Cs+ partitions to the liquid phase. According to the thermodynamic calculations, a thermally controlled liquid-solid separation holds the potential to recover up to 92.8 % of the initial cesium chloride. The phase transition temperatures of the sample, including the solidus and liquidus temperatures, were determined using differential scanning calorimetry. The phase composition of the system was studied at various temperatures using high-temperature X-ray diffraction. A laboratory-scale crystallization apparatus was used to melt the sample partially, allowing the collection of the liquid fraction for elemental analysis. All results demonstrated that Cs+ in this system concentrates in the liquid phase and confirmed the viability of recovering CsCl through thermally controlled solid-liquid separation.

Synthesis and characterization of super occluded LiCl-KCl in zeolite-4A as a chloride salt waste form intermediate

This paper reports the hygroscopic properties of eutectic LiCl-KCl after absorption into zeolite-4A, up to salt loadings of 75 wt%. Samples of the salt occluded zeolite were hydrated in a humidity chamber at constant temperature and relative humidity for up to 100 h. At up to 45 wt% salt loading, the un-occluded phase of salt consisted primarily of NaCl, which forms when the Na+ ions present in the zeolite framework exchange with Li+ and K+ ions from the eutectic LiCl-KCl. This results in minimal water absorption and corrosion of contacted stainless steel. At greater than 45 wt% salt loading, water absorption and corrosion progressively worsened. The mixture has a significant amount of excess LiCl-KCl, making it highly hygroscopic. This study reveals an option for the intermediate treatment of waste salt from spent nuclear fuel electrorefiners that could facilitate it to be stored in a non-inert atmosphere for extended periods of time before final conversion into a permanent waste form.

High prevalence of renal salt wasting induced by haptoglobin-related protein without signal peptide is linked to new syndrome of salt wasting in Alzheimer disease.

The subject of hyponatremia is undergoing significant changes after developing a more pathophysiologic approach that is superior to the ineffective volume approach and can more effectively identify the different causes of hyponatremia. This new approach identified cerebral salt wasting (CSW) in 24 (38%) of 62 hyponatremic patients from the medical wards of the hospital with 21 showing no evidence of cerebral disease to support our proposal to change CSW to renal salt wasting (RSW). RSW had to be differentiated from the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) because of diametrically opposite therapeutic goals of water-restricting water-logged patients with SIADH or administering salt water to volume-depleted patients with RSW. Both syndromes present with identical clinical parameters that require a difficult protocol to make such a differentiation possible. We describe rat clearance studies demonstrating natriuretic activity in the plasma of patients with neurosurgical and Alzheimer diseases (AD) and eventually identify the protein as haptoglobin-related protein without signal peptide, which can serve as a biomarker to simplify diagnosis of RSW and delivery of the proper management to improve clinical outcomes. We also discuss the introduction of a new syndrome of RSW in AD and its implications. The high prevalence of RSW and identification of the natriuretic factor have created debates over the existence of RSW with none questioning or addressing the pathophysiologic data that identified patients with RSW. We also discuss the potentially large group of patients with RSW who are normonatremic.

Renal/Cerebral Salt Wasting in a Patient with COVID-19

Renal/Cerebral Salt Wasting in a Patient with COVID-19

Coexistence of Neonatal Bartter Syndrome and Congenital Cutis Laxa, in which a new mutation in SLC12A1 was identified

Introduction: Neonatal Bartter Syndrome (NBS) is an extremely rare congenital chronic renal tubular transport disorder characterized by preterm birth, polyhydramnios, polyuria, salt wasting, and severe dehydration. Congenital Cutis Laxa (CCL) is a rare disease characterized by loss of skin flexibility and laxity, and atypical facial appearance.
 Case Presentation: A 1290-gram female baby at 30+6 weeks of gestation was hospitalized due to premature birth and respiratory distress. During physical examination, an atypical facial appearance, wrinkles on the forehead, skin laxity, and philtrum were visible as well as a small chin and arachnodactyly in the fingers and toes. The patient displayed metabolic alkalosis in her blood gases. The patient, with an atypical face and current findings submitted her exome for genetic diagnosis. Genetic analysis revealed the presence of homozygous NM_001184832.2:c.2485+5G>A (rs749269268) in SLC12A1, and NBS Type 1 was diagnosed. A novel heterozygous NM_000501.4:c.352G>T, p.Val118Phe (rs781922544) variant was detected in ELN, and CCL syndrome with autosomal dominant inheritance was diagnosed.
 Conclusions: The rare co-occurrence of NBS and CCL syndrome in our patient was seen as a mere coincidence. Since this is the first reported case in the literature, it was deemed appropriate to present this case.

Renal Salt Wasting: Earliest Manifestation of Rhabdomyolysis, Supreet/Mehandru Syndrome

Acute kidney injury is a feared and common complication of rhabdomyolysis, occurring in 10-40% of hospitalized patients with mortality rate as high as 59% in critically ill patients. However, there is a lack of information in the literature on renal salt wasting in rhabdomyolysis. We report 3 cases of rhabdomyolysis who developed renal salt wasting in presence of normal glomerular filtration rate. To the best of our knowledge, this is the first case report in humans displaying this phenomenon. Authors noted that all the three patients had urine pH of over 7.0. Literature reveals, patients with rhabdomyolysis and urine pH<6.5 develop acute kidney injury whereas urine pH>7.0 experienced renal salt wasting in presence of normal glomerular filtration rate. All 3 cases, previously not known to have hypertension, were noted to have elevated blood pressure during acute phase of rhabdomyolysis. Animal studies have shown that increase in blood pressure causes increase in renal interstitial pressures that results in naturesis and diuresis. All 3 cases had increased levels of plasma renin activity and aldosterone. There is a direct proportionality between increased blood pressure and naturesis. Clinicians should be aware of salt wasting as an early renal manifestation in patients with rhabdomyolysis

THU188 Clinical Conundrum: Salt Wasting Congenital Adrenal Hyperplasia With Paradoxical High Cortisol

Abstract Disclosure: C. Pillai: None. Background: Salt wasting congenital adrenal hyperplasia (CAH) is a severe life-threatening condition and thus a cannot miss diagnosis. In salt wasting CAH, cortisol is generally very low. An ACTH stimulation test is often used to diagnose adrenal insufficiency and initiate life-saving hydrocortisone therapy. Clinical Case: I present a case of a male infant who was found to have salt-wasting CAH crisis despite a paradoxically high serum cortisol. He had an abnormal newborn screen for CAH. He was sent to the ER for reportedly high 17-hydroxyprogesterone (17-OHP). Endocrine was consulted and a serum 17-OHP, ACTH and cortisol level was requested. He was an incredibly difficult patient to collect blood work and after multiple attempts only a serum cortisol was collected. He was admitted for an ACTH stimulation test to rule out salt wasting CAH. His physical exam was unremarkable with male external genitalia with descended prepubertal testes. His cortisol from the ER resulted at 36.5 mcg/dL without any steroid administration in the morning. Family refused to do any additional blood work in the morning. Even anesthesiology was not able to collect the requested labs. The ACTH stimulation test was canceled, and he was discharged with close follow-up in 1 week. The state newborn screen department called and reported his initial 17-OHP was undetectably high. He was seen the same day and his BMP showed clear signs of salt wasting. His serum sodium was 131 mmol/L and potassium was 7.2 mmol/L. He was admitted for a salt wasting crisis and responded well to hydrocortisone and fludrocortisone. Serum sodium and potassium normalized. Blood draw issues continued and only BMP were collected during the hospitalization. 17-OHP after several days of therapy was 3270 ng/dl. 17-OHP, sodium and potassium have normalized on therapy. Genetic testing is pending. Conclusions: By reporting this case, I draw attention to the possibility that high levels of cortisol do not definitely rule out salt-wasting CAH. Reference Ranges: NA 132 - 145 mmol/L K 3.0 - 6.0 mmol/L Cortisol: 1.7 - 14.0 ug/dL 17 OHP:INTERPRETIVE INFORMATION for 17-Hydroxyprogesterone in premature and normal male infants: Premature (26 to 28 weeks) 124 to 841 ng/dL Premature (29 to 35 weeks) 26 to 568 ng/dL Full term Day 3 7 to 77 ng/dL 4 days to 2 months Less than 200 ng/dL. Presentation: Thursday, June 15, 2023

SAT282 Hypogonadism In Men With Congenital Adrenal Hyperplasia. A Retrospective Longitudinal Analysis With A Special Focus On Testicular Adrenal Rest Tumors And 11-oxygenated Androgens

Abstract Disclosure: M.K. Auer: None. C. Lottspeich: None. M. Bidlingmaier: None. H.F. Nowotny: None. L. Tschaidse: None. R.J. Auchus: None. N. Reisch: None. Background: Hypogonadism is a common issue in men with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD). While this is mostly resulting from extra-gonadal steroid production, testicular function may also be impaired due to the presence of testicular adrenal rest tumors (TART). In addition to their local compressive effects, it has recently been demonstrated that TART are a source of 11-oxygenated androgens that might impair testicular function. Little is known about the long-term course of testicular function in men with 21OHD in general and the role of potential influential factors such as presence of TART and 11-oxygenated androgen formation in particular. Methods: Retrospective single-center study in 30 men with classic 21OHD (N = 11 with TART, N = 16 without TART, N = 3 unknown TART status). Median age at baseline was 31.0 years (IQR 26-38). Median observation period was 12 years (IQR 8-13). The median number of outpatient visits and blood samplings per patient during this time was 8.5 (IQR 6-10). Testosterone (T), 17-hydroxyprogesterone (17-OHP), androstenedione (A4) and 11-oxygenated androgens were measured simultaneously from stored serum samples by LC-MS/MS. Results: On average, 43.2% (No TART) and 54.6% (TART) of all T measurements in each patient were below the reference range (n.s.). In most patients, gonadotropin levels were normal or suppressed. In univariate as well as multivariate mixed model analysis, including age, BMI, type of glucocorticoid (GC), GC-equivalence dosage and phenotype (salt wasting vs simply virilizing), T levels were comparable between men with and without TART. While T levels remained stable during the observation period in men without TART (Baseline 11.37 ± 1.52 nmol/l, last visit 12.1 ± 2.1 nmol/l) they increased in those with TART (Baseline 9.48 ± 1.68 vs. last visit 14.9 ± 2.3 nmol/l (Time*Group F1,7.247 = 7.558; p = 0.006). At baseline, the A4/T-ratio as a marker of adrenal T production was significantly higher in men with TART (1.39 ± 1.63) than in those without (0.27 ± 1.63), and there was a Time*Group interaction, again indicating a decrease in the A4/T-ratio in men with TART (F1,47.843 = 2.008; p = 0.04). This resulted in only a trend for the A/T-ratio being higher in men with TART (0.5 ± 1.6 vs 0.3 ± 1.5; p = 0.057) across the whole observation period. 11-ketotestosterone levels were higher in men with TART (1.8 ± 0.006 pg/ml) than in men without TART (0.68 ± 0.006 nmol/l) but remained unchanged over time in both groups. Conclusion: A normal serum T does not exclude hypogonadotropic hypogonadism in men with 21OHD, which is a common problem that appears to remain stable in the long run. The presence of TART does not have a negative effect on T-levels. In contrast, the detection of TART should prompt further assessment, including A4, gonadotropins, and 11-ketotestosterone, followed by treatment optimization to improve gonadal T production. Presentation: Saturday, June 17, 2023

THU186 A Case Of 3beta-hydroxysteroid Dehydrogenase Type2 Deficiency Diagnosed By Whole Genome Sequencing In A Patient With Congenital Adrenal Hyperplasia

Abstract Disclosure: S. Chang: None. C. Kim: None. 3β-hydroxysteroid dehydrogenase type 2 deficiency (3βHSD2D) is a very rare type of congenital adrenal hyperplasia (CAH) caused by HSD3B2 gene mutations. The estimated prevalence is less than 0.5% of all CAH and less than 1/1,000,000 at birth. It is a disorder of impaired steroidogenesis and sex steroid deficiency characterized by variable degrees of salt wasting, hypoglycemia, male incomplete masculinization and female virilization. Some newborns with 3βHSD2D have increased level of 17-hydroxyprogesterone (17OHP) that clinicians misdiagnose them as having classic 21-hydroxylase deficiency. A male newborn was referred to our hospital for generalized hyperpigmentation. Laboratory test showed hyponatremia, hyperkalemia and hypoglycemia. Hormonal study showed high level of adrenocorticotropic hormone, 17OHP, progesterone, renin activity, dehydroepiandrosterone (DHEA), androstenedione and low level of aldosterone. Clinically diagnosing with CAH, we administered glucocorticoid and mineralocorticoid replacement. We performed PCR sequencing for CYP21A2 gene mutation to confirm 21 hydroxylase deficiency but revealed normal result. After follow-up for 17 years with replacement treatment, he happened to have the opportunity to perform whole genome sequencing. Whole genome sequencing allowed to detect two pathogenic mutations in the HSD3B2 gene, described in compound heterozygosity (p.Arg249Ter and c.-149_143-1766del). One mutation (c.-149_143-1766del) is inherited from his father and the mother was not done for gene study. While there have been less than 100 cases of 3βHSD2D reported worldwide, in our best knowledge, this is the first case of genetically confirmed 3βHSD2D in Korea. Presentation: Thursday, June 15, 2023

Genotyping in patients with congenital adrenal hyperplasia by sequencing of newborn bloodspot samples.

Genotype-phenotype correlation in congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency ranges from 45 to 97 %. We performed massively parallel sequencing of CYP21A2 on stored newborn bloodspot samples to catalogue the genotypes present in our patients with CAH and enable genotype-phenotype comparison. Participants≤15 years old with clinically diagnosed CAH were recruited from The Sydney Children's Hospitals Network. Phenotype was classified from clinical and biochemical details in the medical record as salt wasting (SW), simple virilising (SV), non-classic (NC) or an intermediate phenotype (SW/SV; SV/NC). Amplicon-based sequencing for CYP21A2 was performed on stored newborn bloodspot samples by the New South Wales Newborn Bloodspot Screening Laboratory on MiSeq™Dx (Illumina, California). Available genetic test results were also obtained from the medical records. Samples from 67 participants (43 % female, age 0.3-15 years) were sequenced, including 9 sibships. SW phenotype was present in 33/67 participants (49 %), SV in 9 (13 %) and NC in 16 (24 %). Intermediate phenotypes included SW/SV in seven participants (10 %) and SV/NC in two (3 %). Variants were identified in 90/116 alleles (78 %). A complete genotype was available in 47/67 participants (70 %). The most common genotype was homozygous c.293-13A/C>G (I2G) in 7/47 participants (15 %). Genotype correlated with the mostcommonly reported phenotype in 36/44 cases (82 %). Correlation was higher in SW and NC phenotypes. This study uses genetic testing of newborn bloodspots to identify and characterise the genotypes present in an ethnically diverse Australian population with CAH. It further strengthens our knowledge of genotype-phenotype correlations in CAH.

The removal of organic contaminants from industrial waste salts by pyrolysis and potential use for energy storage

Industrial waste salt (IWS) treatment is a significant technology for current environmental protection and resource utilization, and the complex organic pollutants in waste salts seriously hamper the development of cleaner production. In this work, the kinetic mechanism of the organic pollutant removal by pyrolysis is investigated, and the energy storage performance of the recovered products is studied, which provides a new idea for the coupling of waste salt resource treatment and energy storage utilization. Experimental results and pyrolysis kinetic analysis show that high temperature pyrolysis can deeply remove the organic pollutants in IWS, and the total organic carbon (TOC) removal rate can reach more than 99%. The high temperature pyrolysis of waste salt is mainly divided into two stages, and the pyrolysis reaction mainly occurs in the high temperature section (270°C–330 °C) with lower activation energy. The obtained salt containing carbon material exhibits high light absorptance of about 82%, enhanced thermal conductivity of 0.39 W/(m·K) and specific heat of 1.49 J/(g·K), which can be employed as the low-cost and high-performance photothermal absorption and thermal storage material.

Systematic laboratory investigations concerning the reduction of uncertainties related to the modeling of the material behavior of crushed salt

Abstract. The planned use of crushed salt as the backfill and sealing material in the context of the final disposal of radioactive waste in rock salt is justified and favored by the realization that the material properties of loosely deposited crushed salt will be transformed into the material properties of in situ rock salt in the long term by the convergence of the viscous rock salt mass. In other words, the convergence-induced compaction of crushed salt ultimately results in a nearly pore-free material, with a tightness and creep capacity corresponding to that of the ambient rock salt mass. The computational prognosis of the load-bearing and sealing behavior required for the long-term safety assessment of underground repositories requires a sufficiently proven physical modeling of the, in general, three-dimensional load bearing and permeability behavior of crushed salt. This requirement can only be met if the influencing variables on the load bearing and permeability behavior are sufficiently quantified and represented by mathematical–physical functions on the basis of suitable laboratory and field investigations. In order to eliminate or reduce the currently (still) existing scientific deficits and uncertainties concerning the general three-dimensional load bearing and permeability behavior of crushed salt, a systematized laboratory program was developed within the framework of the joint project KOMPASS (compaction of crushed salt for the safe containment), with the aim of ensuring an isolated recording of individual processes and influencing factors on the compaction behavior. Based on this, triaxial long-term compaction tests, TUC-V2 and TUC-V4, with a test duration of 750 and 180 d, respectively, were carried out within the framework of the joint project KOMPASS and of the follow-up project KOMPASS II. Both tests, but especially TUC-V2 with its 2-year duration, represent a sequence of several loading phases for separate analysis of the effects of individual influencing factors on the compaction behavior of crushed salt. By integrating several loading phases in one long-term test each, it was possible, on the one hand, to eliminate the scattering of the material behavior that would have been expected if several individual tests had been carried out as an alternative and, on the other hand, to provide metrological evidence of the range of validity of the compaction behavior as a function of individual influencing factors for a comparatively wide range of different porosities in each case. The computational reanalysis of the long-term tests opens up the possibility of validating and strengthening the currently available constitutive model approaches with regard to their ability to influence several parameters on the compaction behavior, while eliminating the scatter band of the material behavior. The improvement of the existing constitutive model approaches up to the sufficient reproduction of the measurement results equals an increase in the validation status or the credibility of the constitutive models or, vice versa, a reduction in the uncertainties remaining due to (not yet) validated model assumptions. Within the framework of the KOMPASS II joint project, a benchmark was carried out against the background outlined above for the comparative analysis of the constitutive models currently available to the project partners for depicting the compaction behavior of crushed salt with regard to their ability to computationally represent the measurement results generated within the TUC-V2 experiment. Specifically, the focus is on constitutive model functions that control the viscous compaction and shear deformation behavior and, within these functions, the influencing factors of mean stress, deviatoric stress, and temperature, which were recorded by laboratory tests for medium ranges of porosities from ≈ 16 % to very low porosities of ≈ 2.5 %. Within the framework of the continuation of the investigations planned under the acronym MEASURES (Multi-scale Experimental and numerical Analysis of crushed Salt material Used as engineered backfill for a nuclear waste REpository in rock Salt), it is intended to continue the benchmark study with a more detailed investigation of the influence of the deviatoric stress, which includes considering the data from TUC-V4. It is also intended to supplement the laboratory data base with further experiments by (a) investigations on the compaction behavior in the range of high-to-medium porosities and (b) investigations on the influence of the moisture content on the compaction capability (to be performed with KOMPASS reference material).

Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Study of a Cohort of Patients from a Single Italian Center.

Testicular adrenal rest tumors (TARTs) are a common complication in male patients with congenital adrenal hyperplasia (CAH). The aim of our cross-sectional cohort study is to estimate the frequency of TARTs with the correlation of genotype and disease control on tumor development. Thirty-five male patients, aged 14-26 years, were included in the study, all followed by the same center of pediatric endocrinology in Bologna. We studied genotypes, hormonal profiles at different time intervals and testicular ultrasound. A logistic regression model with multivariant analysis was developed for the statistical analysis. TARTs were detected in 31.4% of the cases, 90.9% of them had a classic form with salt wasting, while 9.1% had a non-classic form. Additionally, a significant correlation between the incidence of TARTs and severity of genotype was detected. Patients with TARTs had markedly worse metabolic control on average (p = 0.027), reflected by high ACTH, 17OH progesterone, and overall delta4-androstenedione. In conclusion, a screening tool is mandatory, especially (but not exclusively) in patients with the most severe forms of CAH and poor endocrine control of the disease.