Clinical and radiological correlates of activities of daily living in cerebellar atrophy caused by PMM2 mutations (PMM2-CDG).

We aimed to identify clinical, molecular and radiological correlates of activities of daily living (ADL) in patients with cerebellar atrophy caused by PMM2 mutations (PMM2-CDG), the most frequent congenital disorder of glycosylation. Twenty-six PMM2-CDG patients (12 males; mean age 13 ± 11.1 years) underwent a standardized assessment to measure ADL, ataxia (brief ataxia rating scale, BARS) and phenotype severity (Nijmegen CDG rating scale, NCRS). MRI biometry of the cerebellum and the brainstem were performed in 23 patients (11 males; aged 5 months-18 years) and 19 control subjects with equal gender and age distributions. The average total ADL score was 15.3 ± 8.5 (range 3-32 out of 36 indicating severe functional disability), representing variable functional outcome in PMM2-CDG patients. Total ADL scores were significantly correlated with NCRS (r2 = 0.55, p < 0.001) and BARS scores (r2 = 0.764; p < 0.001). Severe intellectual disability, peripheral neuropathy, and severe PMM2 variants were all significantly associated with worse functional outcome. Higher ADL scores were significantly associated with decreased diameters of cerebellar vermis (r2 = 0.347; p = 0.004), hemispheres (r2 = 0.436; p = 0.005), and brainstem, particularly the mid-pons (r2 = 0.64; p < 0.001) representing the major radiological predictor of functional disability score in multivariate regression analysis. We show that cerebellar syndrome severity, cognitive level, peripheral neuropathy, and genotype correlate with ADL used to quantify disease-related deficits in PMM2-CDG. Brainstem involvement should be regarded among functional outcome predictors in patients with cerebellar atrophy caused by PMM2-CDG.

Long-Range Chiral Induction by a Fully Noncovalent Approach in Supramolecular Porphyrin-Calixarene Assemblies.

The hierarchical assembly, in aqueous solution, of a new multi-metalloporphyrin/calixarene aggregate has been accomplished. In this supramolecular system transfer of chirality, from the outermost components to the central porphyrin reporter, takes place as a result of favorable and fully noncovalent long-range electronic communication.

Photodegradation of Antibiotics by Noncovalent Porphyrin-Functionalized TiO2 in Water for the Bacterial Antibiotic Resistance Risk Management.

Antibiotics represent essential drugs to contrast the insurgence of bacterial infections in humans and animals. Their extensive use in livestock farming, including aquaculture, has improved production performances and food safety. However, their overuse can implicate a risk of water pollution and related antimicrobial resistance. Consequently, innovative strategies for successfully removing antibiotic contaminants have to be advanced to protect human health. Among them, photodegradation TiO2-driven under solar irradiation appears not only as a promising method, but also a sustainable pathway. Hence, we evaluated several composite TiO2 powders with H2TCPP, CuTCPP, ZnTCPP, and SnT4 porphyrin for this scope in order to explore the effect of porphyrins sensitization on titanium dioxide. The synthesis was realized through a fully non-covalent functionalization in water at room conditions. The efficacy of obtained composite materials was also tested in photodegrading oxolinic acid and oxytetracycline in aqueous solution at micromolar concentrations. Under simulated solar irradiation, TiO2 functionalized with CuTCPP has shown encouraging results in the removal of oxytetracycline from water, by opening the way as new approaches to struggle against antibiotic's pollution and, finally, to represent a new valuable tool of public health.

Nanoassemblies Based on Supramolecular Complexes of Nonionic Amphiphilic Cyclodextrin and Sorafenib as Effective Weapons to Kill Human HCC Cells.

Sorafenib (Sor), an effective chemiotherapeutic drug utilized against hepatocellular carcinoma (HCC), robustly interacts with nonionic amphiphilic cyclodextrin (aCD, SC6OH), forming, in aqueous solution, supramolecular complexes that behave as building blocks of highly water-dispersible colloidal nanoassemblies. SC6OH/Sor complex has been characterized by complementary spectroscopic techniques, such as UV-vis, steady-state fluorescence and anisotropy, resonance light scattering and (1)H NMR. The spectroscopic evidences and experiments carried out in the presence of an adamantane derivative, which competes with drug for CD cavity, agree with the entrapment of Sor in aCD, pointing out the role of the aCD cavity in the interaction between drug and amphiphile. Nanoassemblies based on SC6OH/Sor display size of ∼200 nm, negative zeta-potential (ζ = -11 mV), and both maximum loading capacity (LC ∼ 17%) and entrapment efficiency (EE ∼ 100%). Kinetic release profiles show a slower release of Sor from nanoassemblies with respect to the free drug. SC6OH/Sor nanoassemblies have very low hemolytic activity and high efficiency in vitro in decreasing cell growth and viability of HCC cell lines, such as HepG2, Hep3B, and PLC/PRF/5, opening promising chances to their in vivo applications.

Sky is no limit for ureteroscopy: extending the indications and special circumstances.

PURPOSE: To critically review and synthesize data of ureteroscopy (URS) in different circumstances that all urologists may encounter during everyday clinical practice, such as pregnancy, obesity, bleeding diathesis, renal stones larger than 2 cm, calyceal diverticula, and kidney malformations. METHODS: According to PRISMA guidelines, a systematic literature review was performed to identify articles published between 1990 and December 2013 that reported different indications and special circumstances for URS. Articles were separated into the following categories: pregnancy, obesity, bleeding diathesis, stones larger than 2 cm in diameter, calyceal diverticula, and kidney malformations. We used a narrative synthesis for the analyses of the studies, including a description of the characteristics and main outcomes reported in the articles. RESULTS: Records identified through database searching were 1396; at the end of study selection, articles included were 57. The majority of these are retrospective studies and involve small cohorts of patients. There does not exist a consensus about important parameters in ureterorenoscopy like stone size, stone free status and complication rate. CONCLUSION: Ureteroscopy is effective and reliable tool capable of treating the majority of stones even in the most complicated clinical scenarios and will have more fundamental roles in endourology. The lack of definitive conclusions is due to the great heterogeneity in collecting study's results; multicentric randomized trials that define in advance the parameters to be studied should be encouraged.

Is retrograde intrarenal surgery for the treatment of renal stones with diameters exceeding 2 cm still a hazard?

INTRODUCTION: Major kidney stones have traditionally been treated with percutaneous nephrolithotomy. However, retrograde intrarenal surgery (RIRS), which until a few years ago was considered inappropriate for this purpose, is becoming a viable, attractive alternative. The aim of the current study was to assess the efficacy and safety of RIRS combined with holmium laser lithotripsy for the treatment of stones > 2 cm in diameter in a large series of patients, reporting complications according to the Clavien-Dindo classification. MATERIALS AND METHODS: By retrospective analysis, we identified a total of 162 patients who were affected by stones greater than 2 cm in diameter and who had undergone RIRS. We reviewed demographic and stone characteristics, intraoperative and postoperative outcomes, and complications. RESULTS: The mean stone size was 2.7 cm +/- 0.6 cm. The primary, secondary, and tertiary stone-free rates were 66%, 80.9%, and 87.7%, respectively. The mean number of procedures per patient was 1.48. The complication rates according to the Clavien-Dindo classification were Clavien I in 20.4% of patients, Clavien II in 0%, Clavien III in 4.9%, Clavien IV in 0.6%, and Clavien V in 0%. CONCLUSIONS: As an alternative to standard procedures for the treatment of renal calculi greater than 2 cm in diameter, RIRS is safe and effective, with a low complication rate.

Pneumatic lithotripsy versus holmium:YAG laser lithotripsy for the treatment of single ureteral stones: a prospective, single-blinded study.

OBJECTIVE: The aim of this prospective single-blinded study was to analyze the stone-free (SF) rates between pneumatic lithotripsy (PL) and laser lithotripsy (LL) for the treatment of single and primary ureteral stones and to evaluate potentially predictive factors of a SF status. MATERIAL AND METHODS: From January 2010 to January 2011, 133 consecutive patients with single and primary ureteral stones were prospectively enrolled. Uni- and multivariate logistic regression were performed to estimate predictive factors of a SF status. RESULTS: The SF rate in the PL group was 80.7 and 86.1% in the LL group (p = 0.002). Success rates with regard of stone position were not significantly different between groups. At univariate logistic regression, middle ureteral stone (OR 3.33, p = 0.04), distal ureteral stone (OR 4.4, p = 0.02), LL (OR 3.05, p = 0.04) and Hounsfield units (HUs) (OR 1.07, p = 0.03) were significantly predictive factors of a SF status. At a multivariate logistic regression, middle ureteral stone (OR 5.58, p = 0.01), distal ureteral stone (OR 7.87, p < 0.01), LL (OR 2.4, p = 0.02) and HUs ≥1,200 (OR 1.15, p = 0.02) were significantly associated with a SF status. CONCLUSIONS: LL significantly influences the SF status after ureteroscopy, allowing a higher SF rate when compared to PL. HUs may significantly influence this success rate.

Contrast-enhanced ultrasound in renal cystic lesions: an update.

This narrative review aims to describe the current status of contrast-enhanced ultrasound (CEUS) in characterizing renal cystic lesion. The imaging techniques usually performed for their evaluation are ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) with different criteria of application based on the individual case and the purpose of the examination. Generally, US, as a non-ionizing examination, is the first imaging modality performed and therefore the one that incidentally detects cystic lesions. CT is the most performed imaging modality for cystic lesion assessment before MRI evaluation. It provides better characterization and management and has been introduced into the Bosniak classification. In this context, CEUS is making its way for its characteristics and represents the emerging technique in this field. With these premises, the authors analyze the role of CEUS in the evaluation of renal cysts, starting with an explanation of the technique, describe its main advantages and limitations, and end with a discussion of its application in the Bosniak classification and management, following the current major guidelines.

18 F-FET PET/CT in Calcifying Pseudoneoplasm of the Neuraxis : First Case.

ABSTRACT: A 66-year-old man has been treated in a psychiatric department for 4-5 years for a depressive syndrome, which is associated with poor motor initiative, confusional state, and dysosmia. Dynamic 18 F-FET PET/CT showed only faint uptake of radiotracer just above the background on the left frontal calcific lesion. The time-activity curve of the neoplasms showed a descending pattern. After a left fronto-orbitary minicraniotomy surgery, histology examination concluded for a rare calcifying pseudoneoplasm of the neuraxis (CAPNON). To our knowledge, no data are available on the metabolic behavior of CAPNON in 18 F-FET PET/CT. This case highlighted that a faint uptake and descending pattern on dynamic 18 F-FET PET/CT may be helpful in suspected CAPNON before surgery.

The Role of Intraoperative Ultrasonography Associated with Clampless Technique in Three-Dimensional Retroperitoneoscopic Laparoscopic Enucleation of Completely Endophytic Renal Tumors.

Objectives: To evaluate perioperative and functional outcomes of clampless laparoscopic tumor enucleation for completely endophytic renal tumors with the guide of intraoperative ultrasonography. Methods: We analyzed patients with clinically completely endophytic tumors, renal tumors, who underwent clampless three-dimensional (3D) retroperitoneoscopic laparoscopic tumor enucleation between January 2012 and January 2021. Patients with exophytic tumors were excluded. Intraoperative ultrasonography was used to map out the mass in all surgeries. Results: Overall, 57 patients underwent clampless 3D retroperitoneoscopic laparoscopic tumor enucleation. Mean surgical time was 131 minutes, and mean estimated blood loss was 202 mL. Mean hospital stay was 4.7 days. Major and minor postoperative complications occurred, respectively, in 3 and 10 cases. Only a patient had a positive surgical margin. One-year renal function did not differ from baseline. Conclusion: Our study showed that clampless laparoscopic enucleation guided by laparoscopic ultrasonography ensured satisfactory outcomes for completely intrarenal tumors, with excellent renal function preservation 1 year after surgery.

Clampless Laparoscopic Tumor Enucleation for Exophytic Masses Greater Than 4 cm: Is Renorrhaphy Necessary?

Objective: To demonstrate the safety of clampless 3D laparoscopic tumor enucleation (cLTE) for exophytic T1b kidney masses, avoiding suture to achieve hemostasis. Methods: Between January 2010 and January 2021, 241 consecutive patients with an exophytic renal tumor underwent sutureless cLTE. Patients with predominantly endophytic growth or tumors 4 mm closer to the collecting system less were excluded. In all cases, an attempt was made to accomplish surgery without suturing kidney parenchyma. Data were obtained from a retrospective review of history and physical examinations, operative and pathology reports, anesthesia records during the hospital stay, and follow-up visits. Results: Among 241 patients who underwent cLTE, 148 had cT1a and 93 had cT1b renal tumor. The median tumor size was 32 mm, and the median R.E.N.A.L. (radius exophytic/endophytic nearness anterior/posterior location) score was 6. Renorrhaphy was necessary in 5 cases. The median operative time (OT) was 100 minutes, and the estimated blood loss (EBL) was 150 mL. The median 24-hour decrease in hemoglobin was 1.8 g/dL. The median length of stay was 4 days. Nineteen patients had postoperative complications: 3 cases had Clavien-Dindo (CD) 3a or more, 6 had CD2, and 10 had CD1. Comparing the T1a and T1b groups, except for the median OT and the EBL, no significant differences were observed in all the other variables analyzed. In both groups, renal function was preserved after 1 year from surgery. Conclusion: Our experience showed that sutureless cLTE is safe and feasible for T1b tumors leading to radical oncological outcomes and preserving renal function.

KLVFF oligopeptide-decorated amphiphilic cyclodextrin nanomagnets for selective amyloid beta recognition and fishing.

Recognition and capture of amyloid beta (Aß) is a challenging task for the early diagnosis of neurodegenerative disorders, such as Alzheimer's disease. Here, we report a novel KLVFF-modified nanomagnet based on magnetic nanoparticles (MNP) covered with a non-ionic amphiphilic ß-cyclodextrin (SC16OH) and decorated with KLVFF oligopeptide for the self-recognition of the homologous amino-acids sequence of Aß to collect Aß (1-42) peptide from aqueous samples. MNP@SC16OH and MNP@SC16OH/Ada-Pep nanoassemblies were fully characterized by complementary techniques both as solid powders and in aqueous dispersions. Single domain MNP@SC16OH/Ada-Pep nanomagnets of 20-40 nm were observed by TEM analysis. DLS and ζ-potential measurements revealed that MNP@SC16OH nanoassemblies owned in aqueous dispersion a hydrodynamic radius of about 150 nm, which was unaffected by Ada-Pep decoration, while the negative ζ-potential of MNP@SC16OH (-40 mV) became less negative (-30 mV) in MNP@SC16OH/Ada-Pep, confirming the exposition of positively charged KLVFF on nanomagnets surface. The ability of MNP@SC16OH/Ada-Pep to recruit Aß (1-42) in aqueous solution was evaluated by MALDI-TOF and compared with the ineffectiveness of undecorated MNP@SC16OH and VFLKF scrambled peptide-decorated nanoassemblies (MNP@SC16OH/Ada-scPep), pointing out the selectivity of KLVFF-decorated nanohybrid towards Aß (1-42). Finally, the property of nanomagnets to extract Aß in conditioned medium of cells over-producing Aß peptides was investigated as proof of concept of effectiveness of these nanomaterials as potential diagnostic tools.

Effects of Argon-Based and Nitrogen-Based Modified Atmosphere Packaging Technology on the Quality of Pomegranate (Punica granatum L. cv. Wonderful) Arils.

Ready-to-eat pomegranate arils are considered a "functional food" for their health benefits and have desirable sensory characteristics, which have caused an increasing interest by the consumers for this product. The preparation process of ready-to-eat fruit products can cause severe injuries and worsen their quality and shelf life significantly. Modified atmosphere packaging (MAP) has been used broadly in the last years to maintain the quality of processed fruits and showed optimal results, in spite of the possible problems caused by the depletion of O2 and corresponding accumulation of CO2 in the package. This study was conducted to evaluate the effects of different MAP treatments, based on nitrogen or alternatively on a noble gas, argon, in combination with refrigerated storage (0, 4, 8, 12, and 16 days at 4 ± 1 °C and 90 ± 5% RH) on the qualitative parameters of pomegranate arils with the aim to prolong their post-harvest life maintaining the original quality. The argon-based MAP treatment (MAPAr) was the one that provided the best results, assuring a limited loss of weight and juice content. The use of noble gas allowed to maintain a high sugar/acid ratio until 16 days from packaging. Sensory analysis on all MAP treated arils and, on the juice, obtained from them were carried out, and judges showed a preference for MAPAr treated arils and juice until day 12 from packaging.

Effect of Different Modified Atmosphere Packaging on the Quality of Mulberry Fruit (Morus alba L. cv Kokuso 21).

The control of temperature and gas composition is essential to maintain the fresh flavor and quality of perishable fruits like mulberry. This study presented a modified atmosphere experiment (MAP) for fresh fruit showing the potential benefits of innovative gas mixing with argon. The effects of MAP were studied on the physicochemical and qualitative attributes of mulberry preserved at 4 ± 1°C and 90 ± 5% R.H. Fresh mulberries were packaged with different gas combinations: MAP1 (4%O2+6%CO2+90%N2), MAP2 (10%O2+5%CO2+85%Ar), CTR1 (20.9%O2+0.04%CO2), and CTR2 (10%O2+5%CO2+85%N2). Changes in quality parameters were evaluated after 0, 4, 8, and 12 days of storage. Mulberries packaged with MAP had a lower weight loss than CTR samples which lost more than 80% of their initial weight. Furthermore, the results showed that the argon treatment was the best in keeping the fruit juice content, preserving its structure. Despite not showing great differences with MAP1 treatment, Ar allowed to maintain high TSS up to 8 storage days, slowed CO2 production. The sensory profile of mulberry fruit was not significantly affected by storage in modified atmospheres, and the production of potential unpleasant odors in MAP2 could not be perceived. The results of this study confirm that this innovative approach, using MAP technology, has a potential use in maintaining mulberry fruit quality for a longer time.

Laparoscopic single port radical prostatectomy in the 2020: Why not? Our experience.

OBJECTIVES: To analyze the feasibility, safety and advantages of Laparo-Endoscopic Single-site Surgery radical prostatectomy (LESS-RP) based on our personal experience. PATIENTS AND METHODS: Details of 520 patients were retrospectively analyzed, from 2009 to 2019. Extraperitoneal approach, with only two accesses (2.5 cm and 5 mm respectively) was used to perform radical prostatectomy. Perioperative characteristics and postoperative oncologic and functional outcomes are reported. RESULTS: The mean age was 66.6 ± 5.6 years. Mean PSA level was 9 ± 3.5 ng/ml. According to D'Amico classification, the percentage of patients with low-, intermediate-, and high-risk disease cases were 116 (22.4%), 275 (52.8%), and 129 (24.8%) respectively. Mean operative time was 156 ± 43 min. Mean estimated blood loss was 214 ± 93 ml. Positive surgical margins (PSMs) were detected in 110 (21.2%) patients. PSM rates in pT2 and pT3 stages were 20.1% and 22.9%, respectively. The overall complication rate was 9.2%, based on the modified Clavien classification. The 12 months continence and potency rates were 90.9% and 49.1%, respectively. The biochemical recurrence rate was 6.8%, at the median follow-up time of 26.7 months (IQR 12-32). CONCLUSIONS: Our analyses show that LESS-RP is a safe procedure, if performed by surgeons with adequate experience and skills. Unlike the classic laparoscopic prostatectomy, this technique allows better aesthetic and psychological results, reduced postoperative pain, and a faster return to normal daily activity with the same functional and oncological results.

FLAIRectomy in Supramarginal Resection of Glioblastoma Correlates With Clinical Outcome and Survival Analysis: A Prospective, Single Institution, Case Series.

BACKGROUND: Extent of tumor resection (EOTR) in glioblastoma surgery plays an important role in improving survival. OBJECTIVE: To analyze the efficacy, safety and reliability of fluid-attenuated inversion-recovery (FLAIR) magnetic resonance (MR) images used to guide glioblastoma resection (FLAIRectomy) and to volumetrically measure postoperative EOTR, which was correlated with clinical outcome and survival. METHODS: A total of 68 glioblastoma patients (29 males, mean age 65.8) were prospectively enrolled. Hyperintense areas on FLAIR images, surrounding gadolinium-enhancing tissue on T1-weighted MR images, were screened for signal changes suggesting tumor infiltration and evaluated for supramaximal resection. The surgical protocol included 5-aminolevulinic acid (5-ALA) fluorescence, neuromonitoring, and intraoperative imaging tools. 5-ALA fluorescence intensity was analyzed and matched with the different sites on navigated MR, both on postcontrast T1-weighted and FLAIR images. Volumetric evaluation of EOTR on T1-weighted and FLAIR sequences was compared. RESULTS: FLAIR MR volumetric evaluation documented larger tumor volume than that assessed on contrast-enhancing T1 MR (72.6 vs 54.9 cc); residual tumor was seen in 43 patients; postcontrast T1 MR volumetric analysis showed complete resection in 64 cases. O6-methylguanine-DNA methyltransferase promoter was methylated in 8/68 (11.7%) cases; wild type Isocytrate Dehydrogenase-1 (IDH-1) was found in 66/68 patients. Progression free survival and overall survival (PFS and OS) were 17.43 and 25.11 mo, respectively. Multiple regression analysis showed a significant correlation between EOTR based on FLAIR, PFS (R2 = 0.46), and OS (R2 = 0.68). CONCLUSION: EOTR based on FLAIR and 5-ALA fluorescence is feasible. Safety of resection relies on the use of neuromonitoring and intraoperative multimodal imaging tools. FLAIR-based EOTR appears to be a stronger survival predictor compared to gadolinium-enhancing, T1-based resection.

Effect of Three Different Aloe vera Gel-Based Edible Coatings on the Quality of Fresh-Cut "Hayward" Kiwifruits.

In recent years, the market for minimally processed fruit has increased. Fresh-cut fruits are characterized by a short shelf life due to the processing phases that accelerate the ripening courses. The aim of this work is to analyze the effect on the quality of fresh-cut Hayward kiwis of three different edible coatings based on (1) Aloe vera gel, (2) Aloe vera gel + hydroxypropyl methylcellulose and (3) Aloe vera gel + lemon essential oil. Fruit firmness, weight loss, color, soluble solids content, titratable acidity, microbial load and sensory analysis were evaluated as fresh after 2, 4, 7 and 10 days. Aloe vera gel and Aloe vera gel + lemon essential oil maintained the best values, as they acted as a barrier to gas exchange and further reduced the microbial load. These results were confirmed by sensory analysis: Aloe vera gel + hydroxypropyl methylcellulose does not alter the natural taste of kiwi slices, Aloe vera gel + lemon essential oil gives the characteristic taste of lemon essential oil and Aloe vera gel gives an herbaceous taste. The Aloe vera gel, in combination with these additives, maintains the ability to preserve the quality of fresh-cut kiwifruit.

Laparoscopic radical nephrectomy with inferior vena cava thrombectomy: A combined retroperitoneal and transperitoneal approach.

Renal cell carcinoma with inferior cava thrombus indicates biologically aggressive cancer, so the complete surgical resection remains standard of care with best long-term outcomes. Laparoscopic radical nephrectomy with vena cava thrombectomy represents nowadays a mini-invasive surgical alternative to the classic open nephrectomy. We present the case of a patient with incidental diagnosis of a right renal mass with level II inferior cava thrombus completely managed with both retroperitoneal and transperitoneal approaches. The use of a double access was planned to ensure a safe and complete vascular control. In our opinion, optimal patient selection, extensive laparoscopic experience, and knowledge of both transperitoneal and retroperitoneal approaches are critical elements for the safe application of this technique, although this procedure remains challenging and technically demanding.