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1.
Mult Scler Relat Disord ; 87: 105594, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38718748

ABSTRACT

BACKGROUND: in the early stages of Multiple Sclerosis (MS), initiating high-efficacy disease-modifying therapy (HE DMTs) may represent an optimal strategy for delaying neurological damage and long-term disease progression, especially in highly active MS patients (HAMS). Natalizumab (NAT) and Ocrelizumab (OCR) are recognized as HE DMTs with significant anti-inflammatory effects. This study investigates NEDA-3 achievement in treatment-naïve HAMS patients receiving NAT or OCR over three years. METHODS: we retrospectively enrolled treatment-naïve HAMS patients undergoing NAT or OCR, collecting demographic, clinical, and instrumental data before and after treatment initiation to compare with propensity score analysis disease activity, time to disability worsening, and NEDA-3 achievement. RESULTS: we recruited 281 HAMS patients with a mean age of 32.7 years (SD 10.33), treated with NAT (157) or OCR (124). After three years, the Kaplan-Meier probability of achieving NEDA-3 was 66.0 % (95 % CI: 57.3 % - 76.0 %) with OCR and 68.2 % (95 % CI: 59.9 % - 77.7 %) with NAT without significant differences between the two groups (p = 0.27) DISCUSSION AND CONCLUSION: starting HE DMT with monoclonal antibodies for HAMS could achieve NEDA-3 in a high percentage of patients without differences between NAT or OCR.


Subject(s)
Antibodies, Monoclonal, Humanized , Immunologic Factors , Multiple Sclerosis, Relapsing-Remitting , Natalizumab , Humans , Natalizumab/therapeutic use , Natalizumab/administration & dosage , Female , Male , Antibodies, Monoclonal, Humanized/administration & dosage , Antibodies, Monoclonal, Humanized/pharmacology , Adult , Multiple Sclerosis, Relapsing-Remitting/drug therapy , Immunologic Factors/administration & dosage , Immunologic Factors/pharmacology , Retrospective Studies , Young Adult , Disease Progression
2.
Int Arch Otorhinolaryngol ; 28(2): e332-e338, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38618604

ABSTRACT

Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction.

3.
J Neurol ; 271(5): 2182-2194, 2024 May.
Article in English | MEDLINE | ID: mdl-38366072

ABSTRACT

OBJECTIVE: Multiple sclerosis (MS) is a complex disorder in which environmental and genetic factors interact modifying disease risk and course. This multicentre, case-control study involving 18 Italian MS Centres investigated MS course by ethnicity and native-country economic status in foreign-born patients living in Italy. METHODS: We identified 457 MS patients who migrated to Italy and 893 age- and sex-matched native-born Italian patients. In our population, 1225 (93.2%) subjects were White Europeans and White Northern Americans (WENA) and 89 (6.8%) patients were from other ethnical groups (OEG); 1109 (82.1%) patients were born in a high-income (HI) Country and 241 (17.9%) in a low-middle-income (LMI) Country. Medical records and patients interviews were used to collect demographic and disease data. RESULTS: We included 1350 individuals (973 women and 377 men); mean (SD) age was 45.0 (11.7) years. At onset, 25.45% OEG patients vs 12.47% WENA (p = 0.039) had > 3 STIR spine lesions. At recruitment, the same group featured mean (SD) EDSS score of 2.85 (2.23) vs 2.64 (2.28) (p = 0.044) reached in 8.9 (9.0) vs 12.0 (9.0) years (p = 0.018) and underwent 1.10 (4.44) vs. 0.99 (0.40) annual MRI examinations (p = 0.035). At disease onset, patients from LMI countries had higher EDSS score than HI patients (2.40 (1.43) vs 1.99 (1.17); p = 0.032). DISCUSSION: Our results suggested that both ethnicity and socio-economic status of native country shape MS presentation and course and should be considered for an appropriate management of patients. To the best of our knowledge, this is the first study reporting on the impact of ethnicity in MS at an individual level and beyond an ecological population-perspective.


Subject(s)
Multiple Sclerosis , Adult , Female , Humans , Male , Middle Aged , Case-Control Studies , Ethnicity , Income , Italy/epidemiology , Italy/ethnology , Multiple Sclerosis/ethnology , White People , North American People , North America/ethnology , Europe/ethnology
4.
Int. arch. otorhinolaryngol. (Impr.) ; 28(2): 332-338, 2024. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1558034

ABSTRACT

Abstract Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction.

5.
Neurol Sci ; 45(6): 2807-2810, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38150132

ABSTRACT

INTRODUCTION: Lymphopenia is a known side effect of dimethyl fumarate (DMF), a disease-modifying therapy (DMT) for patients with multiple sclerosis (pwMS). A body mass index ≥ 30 kg/m2 has been identified as a protective factor; however, no data are available on lymphopenia in pwMS undergoing to weight loss due to bariatric surgery. METHODS: We described two pwMS with history of bariatric surgery who started DMF as DMT. RESULTS: The two pwMS experienced persistent lymphopenia during DMF-treatment, which was resolved after its discontinuation. CONCLUSIONS: Several mechanisms might modify DMF pharmacokinetic profiles after bariatric surgery and its bioavailability. Absolute lymphocyte count should be monitored in pwMS treated with DMF and history of bariatric surgery and weight loss.


Subject(s)
Bariatric Surgery , Dimethyl Fumarate , Immunosuppressive Agents , Lymphopenia , Humans , Bariatric Surgery/adverse effects , Dimethyl Fumarate/adverse effects , Immunosuppressive Agents/adverse effects , Lymphopenia/chemically induced , Multiple Sclerosis/drug therapy , Multiple Sclerosis, Relapsing-Remitting/drug therapy
6.
Mult Scler ; 29(7): 856-865, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37165941

ABSTRACT

BACKGROUND: Little is known about COVID-19 course and outcomes after a third booster dose of mRNA vaccine against SARS-CoV-2 (mRNA-Vax) in patients with multiple sclerosis (pwMS) treated with ocrelizumab (OCR) and fingolimod (FNG), which showed a weakened immune response to mRNA-vax. OBJECTIVES: The aim of this study was to evaluate COVID-19 course and outcomes in pwMS on OCR and FNG after receiving the third dose of mRNA-Vax and to compare it with pwMS on natalizumab (NTZ). METHODS: Inclusion criteria: >18 years of age, being treated with OCR/FNG/NTZ since the first mRNA-Vax dose; COVID-19 after a third booster dose of mRNA-Vax; no steroids use. RESULTS: Overall, 290 pwMS (79 NTZ, 126 OCR, and 85 FNG) from 17 Italian MS centers were included. Age, Expanded Disability Status Scale (EDSS) score, MS phenotype, disease, and treatment duration were significantly different across groups. PwMS who had COVID-19 on OCR and FNG compared with those on NTZ were slightly more symptomatic with higher hospitalization rates (11.1% vs 7.1% vs 1.3%, respectively). Regression models showed that the majority of the differences observed were not related to the disease-modifying treatments (DMTs) used. No fatal cases were observed. CONCLUSION: Our results support the effectiveness of the third booster dose of mRNA-Vax against severe forms of COVID-19 in pwMS treated with OCR and FNG.


Subject(s)
COVID-19 , Multiple Sclerosis , Humans , Multiple Sclerosis/drug therapy , COVID-19/prevention & control , COVID-19 Vaccines , SARS-CoV-2 , Natalizumab/therapeutic use , Fingolimod Hydrochloride , RNA, Messenger , mRNA Vaccines
7.
Am J Med Genet A ; 191(4): 1111-1118, 2023 04.
Article in English | MEDLINE | ID: mdl-36607831

ABSTRACT

Multisystemic smooth muscle dysfunction syndrome (MSMDS, OMIM # 613834) is a rare autosomal dominant condition caused by pathogenetic variants of ACTA2 gene that result in impaired muscle contraction. MSMDS is characterized by an increased susceptibility to aneurismal dilatations and dissections, patent ductus arteriosus, early onset coronary artery disease, congenital mydriasis, chronic interstitial lung disease, hypoperistalsis, hydrops of gall bladder, and hypotonic bladder. Here, we report an early diagnosis of a MSMDS related to ACTA2 p.Arg179His (R179H) mutation in a newborn and performed a review of the literature. An early diagnosis of MSMDS is extremely important, because of the severe involvement of cardiovascular system in the MSMDS. Multidisciplinary care and surveillance and timely management of symptoms are important to reduce the risk of complications.


Subject(s)
Ductus Arteriosus, Patent , Eye Diseases, Hereditary , Mydriasis , Pupil Disorders , Infant, Newborn , Humans , Ductus Arteriosus, Patent/genetics , Mydriasis/diagnosis , Mydriasis/genetics , Mutation , Eye Diseases, Hereditary/genetics , Actins/genetics
8.
J Am Chem Soc ; 144(50): 22838-22843, 2022 12 21.
Article in English | MEDLINE | ID: mdl-36508588

ABSTRACT

The symmetry of a crystal's morphology usually reflects the symmetry of the crystallographic packing. For single crystals, the space and point groups allow only a limited number of mathematical descriptions of the morphology (forms), all of which are convex polyhedrons. In contrast, concave polyhedrons are a hallmark of twinning and polycrystallinity and are typically inconsistent with single crystallinity. Here we report a new type of structure: a concave polyhedron shape single crystal having a multidomain appearance and a rare space group (P622). Despite these unusual structural features, the hexagonal symmetry is revealed at the morphological levels.


Subject(s)
Metal-Organic Frameworks , Crystallography
9.
Neurol Ther ; 11(3): 1193-1208, 2022 Sep.
Article in English | MEDLINE | ID: mdl-35653061

ABSTRACT

INTRODUCTION: Cladribine administration has been approved for the treatment of relapsing-remitting multiple sclerosis (MS) in 2017; thus, data on cladribine in a real-world setting are still emerging. METHODS: We report on cladribine effectiveness, safety profile, and treatment response predictors in 243 patients with MS followed at eight tertiary MS centers. Study outcomes were: (1) No Evidence of Disease Activity-3 (NEDA-3) status and its components (absence of clinical relapses, MRI activity, and sustained disability worsening); (2) development of grade III/IV lymphopenia. The relationship between baseline features and the selected outcomes was tested via multivariate logistic models. RESULTS: Of the 243 subjects included in the study (66.5% female, age 34.2 ± 10 years, disease duration 6.6 ± 9.6 years), 64% showed NEDA-3 at median follow-up (22 months). Patients with higher number of previous treatments had lower probability to retain NEDA-3 [odds ratio (OR) 0.64, 95% confidence interval (CI) 0.41-0.98, p = 0.04] and were more prone to experience clinical relapses (OR 1.6, 95% CI 1-2.6, p = 0.04). The presence of active lesions at baseline was associated with follow-up magnetic resonance imaging (MRI) activity (OR 1.92, 95% CI 1.04-3.55, p = 0.04). Patients with higher rate of relapses in the year prior to cladribine start were at higher risk of developing sustained disability worsening (OR 2.95% CI 1-4.2, p = 0.04). Lymphopenia grade III/IV over the follow-up was associated with baseline lymphocyte count (OR 0.998, 95% CI 0.997-0.999, p = 0.01). CONCLUSION: In this large cohort, we confirm previous data about cladribine effectiveness on disease activity and disability worsening and provide information on response predictors that might inform therapeutic choices.

10.
Chemistry ; 28(54): e202201108, 2022 Sep 27.
Article in English | MEDLINE | ID: mdl-35735237

ABSTRACT

Formation of highly interpenetrated frameworks is demonstrated. An interesting observation is the presence of very large adamantane-shaped cages in a single network, making these crystals new entries in the collection of diamondoid-type metal-organic frameworks (MOFs). The frameworks were constructed by assembling tetrahedral pyridine ligands and copper dichloride. Currently, the networks' degree of interpenetration is among the highest reported and increases when the size of the ligand is increased. Highly interpenetrated frameworks typically have low surface contact areas. In contrast, in our systems, the voids take up to 63 % of the unit cell volume. The MOFs have chiral features but are formed from achiral components. The chirality is manifested by the coordination chemistry around the metal center, the structure of the helicoidal channels, and the motifs of the individual networks. Channels of both handednesses are present within the unit cells. This phenomenon shapes the walls of the channels, which are composed of 10, 16, or 32 chains correlated with the degree of interpenetration 10-, 16-, and 32-fold, respectively. By changing the distance between the center of the ligand and the coordination moieties, we succeeded in tuning the diameter of the channels. Relatively large channels were formed, having diameters up to 31.0 Å×14.8 Å.

11.
J Colloid Interface Sci ; 623: 723-734, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35605451

ABSTRACT

HYPOTHESIS: Self-assembling molecular structures responding to light stimulus are appealing for applications as sensing and drug delivery. Supramolecular nanotubes have a relevant potential in nanotechnology as they can be used to encapsulate different loads like drugs, biological macromolecules, and nanomaterials. In addition, they are suitable elements for novel supracolloidal materials. Structural responses of supramolecular nanotubes to non-invasive stimuli are very much desired to enable controlled release of the encapsulated guests and to provide these recently developed new materials with an external trigger. Here, we describe the formation of well-defined, single wall tubules that interconvert into twisted ribbons upon UV-light exposure in aqueous environment. The structures are provided by self-assembly of an azobenzene substituted cholic acid, a biological surfactant belonging to the family of bile acids. The azobenzene group allows for the light responsiveness of the molecular packing. Concurrently the steroidal moieties assure both chiral features and extensive hydrophobic interactions for time and temperature resistant aggregates. EXPERIMENTS: The molecular packing interconversion was followed by circular dichroism. Microscopy, small angle X-ray scattering and light scattering measurements demonstrated the drastic morphological variation upon irradiation. A model of the molecular arrangement within the tubular walls was suggested based on the circular dichroism spectra simulation. FINDINGS: Innovatively, the molecular design reported in our work allows for encoding in the same light responsive system multiple desirable features (e.g. bio-origin, temperature resistance and chirality of the aggregates). Such combination of properties, never reported before for a single molecule, might be relevant for the realization of robust, stimuli-responsive bio-vectors.


Subject(s)
Nanostructures , Nanotubes , Circular Dichroism , Hydrophobic and Hydrophilic Interactions , Nanostructures/chemistry , Nanotechnology , Nanotubes/chemistry
12.
Angew Chem Int Ed Engl ; 61(34): e202205238, 2022 Aug 22.
Article in English | MEDLINE | ID: mdl-35594390

ABSTRACT

We show that metal-organic frameworks, based on tetrahedral pyridyl ligands, can be used as a morphological and structural template to form a series of isostructural crystals having different metal ions and properties. An iterative crystal-to-crystal conversion has been demonstrated by consecutive cation exchanges. The primary manganese-based crystals are characterized by an uncommon space group (P622). The packing includes chiral channels that can mediate the cation exchange, as indicated by energy-dispersive X-ray spectroscopy on microtome-sectioned crystals. The observed cation exchange is in excellent agreement with the Irving-Williams series (MnZn) associated with the relative stability of the resulting coordination nodes. Furthermore, we demonstrate how the metal cation controls the optical and magnetic properties. The crystals maintain their morphology, allowing a quantitative comparison of their properties at both the ensemble and single-crystal level.

13.
J Clin Med ; 11(8)2022 Apr 07.
Article in English | MEDLINE | ID: mdl-35456175

ABSTRACT

Pivotal trials showed the effectiveness of the monoclonal antibody ocrelizumab in relapsing and progressive multiple sclerosis (MS). However, data on everyday practice in MS patients and markers of treatment effectiveness are scarce. We aimed to collect real-world data from ocrelizumab-treated MS patients, relapsing-remitting (RR) and progressive MS patients (PMS), including active secondary progressive MS (aSPMS) and primary progressive MS (PPMS) patients, and to explore potential prognostic factors of clinical outcome. Patients were enrolled at MS centres in the Campania region, Italy. We collected clinic-demographic features retrospectively one year before ocrelizumab start (T−1), at ocrelizumab start (T0), and after one year from ocrelizumab start (T1). We explored possible clinical markers of treatment effectiveness in those patients receiving ocrelizumab treatment for at least one year using multilevel-mixed models. We included a total of 383 MS patients (89 RRMS and 294 PMS; 205 females, mean age: 45.8 ± 11.2, disease duration: 12.7 ± 11.6 years). Patients had a mean follow-up of 12.4 ± 8.2 months, and 217 patients completed one-year ocrelizumab treatment. Overall, EDSS increased from T−1 to T0 (coeff. = 0.30, 95% coefficient interval [CI] = 0.19−0.41, p < 0.001) without a further change between T0 and T1 (p = 0.61). RRMS patients did not show an EDSS change between T−1 and T0 nor between T0 and T1. Conversely, PMS patients showed EDSS increase from T−1 to T0 (coeff. = 0.34, 95% CI = 0.22−0.45, p < 0.001) without a further change between T0 and T1 (p = 0.21). PMS patients with a time from conversion shorter than 2 years showed increased EDSS from T−1 to T0 (coeff. = 0.63, 95% CI = 0.18−1.08, p = 0.006) without a further change between T0 and T1 (p = 0.94), whereas PMS patients with a time from conversion longer than 2 years showed increased EDSS from T0 to T1 (coeff. = 0.30, 95% CI = 0.11−0.49, p = 0.002). Naïve patients showed an EDSS decrease between T0 and T1 (coeff. = −0.30, 95% CI = −0.50−−0.09, p = 0.004). In conclusion, our study highlighted that early ocrelizumab treatment is effective in modifying the disability accrual in MS patients.

14.
Int. arch. otorhinolaryngol. (Impr.) ; 25(3): 374-378, Jul.-Sept. 2021. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1340010

ABSTRACT

Abstract Introduction The transmastoid approach is the most recommended technique to Bonebridge surgery, while in patients with bad anatomy or in the canal wall down technique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options. Objective To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes. Methods Seven patients submitted to the IMFA were included. All patients presented conductive and mixed hearing loss with bone thresholds of the audiogram > 40 dB. The audiological test was conducted pre- and postoperatively. Results A total of 5 males and 2 females, aged 13,8 years old (range 6-25 years old) were studied. The average follow-up was of 20 months (12 to 32 months). All patients presented aural atresia, except one with severe osseous-fibrous dysplasia of the temporal bone. Two patients showed bilateral compromise, three patients had associated Goldenhar and Treacher Collins syndrome. On the preoperative audiograms, air conduction (AC) thresholds showed a PTA4 (0.5, 1, 2 and 4 kHz) of 66.7 dB (standard deviation [SD] = ± 7.8), while the bone conduction thresholds reached an average of 11.2 dB (SD = ± 6.9). The postoperative thresholds did not change, and additional sensorineural damage was not observed before activation. Four weeks after surgery, all the patients were fitted with the external processor. The postoperative audiological aided exam showed AC PTA 4 thresholds of 18.9 dB (SD = ± 5.9). Conclusion The IMFA allows the nearest position of the microphone to the external auditory canal. The technique is a suitable option to the 3 classical approaches with similar rate of audiological results. More investigation is needed to determine the benefit of the novel approach compared with the others.

15.
Int Arch Otorhinolaryngol ; 25(3): e374-e378, 2021 Jul.
Article in English | MEDLINE | ID: mdl-34377170

ABSTRACT

Introduction The transmastoid approach is the most recommended technique to Bonebridge surgery, while in patients with bad anatomy or in the canal wall down technique, retrosigmoid or Middle Fossa Approaches are the alternative surgical options. Objective To describe a novel alternative approach called inverted middle fossa approach (IMFA) and its technique and audiological outcomes. Methods Seven patients submitted to the IMFA were included. All patients presented conductive and mixed hearing loss with bone thresholds of the audiogram > 40 dB. The audiological test was conducted pre- and postoperatively. Results A total of 5 males and 2 females, aged 13,8 years old (range 6-25 years old) were studied. The average follow-up was of 20 months (12 to 32 months). All patients presented aural atresia, except one with severe osseous-fibrous dysplasia of the temporal bone. Two patients showed bilateral compromise, three patients had associated Goldenhar and Treacher Collins syndrome. On the preoperative audiograms, air conduction (AC) thresholds showed a PTA4 (0.5, 1, 2 and 4 kHz) of 66.7 dB (standard deviation [SD] = ± 7.8), while the bone conduction thresholds reached an average of 11.2 dB (SD = ± 6.9). The postoperative thresholds did not change, and additional sensorineural damage was not observed before activation. Four weeks after surgery, all the patients were fitted with the external processor. The postoperative audiological aided exam showed AC PTA 4 thresholds of 18.9 dB (SD = ± 5.9). Conclusion The IMFA allows the nearest position of the microphone to the external auditory canal. The technique is a suitable option to the 3 classical approaches with similar rate of audiological results. More investigation is needed to determine the benefit of the novel approach compared with the others.

16.
J Colloid Interface Sci ; 604: 415-428, 2021 Dec 15.
Article in English | MEDLINE | ID: mdl-34271493

ABSTRACT

HYPOTHESIS: Sodium deoxycholate is a natural bile salt produced by animals and fulfilling important physiological processes. It is also used as dispersive surfactant and building block for self-assembled architectures in biology and material science. Although long debated, the study of its self-assembly in water is hereto incomplete and the models of the known aggregates are still controversial. This background suggests a complex scenario likely missing of additional mesophases. EXPERIMENTS: Electron and optical microscopy techniques were crossed with SAXS data for the research. FINDINGS: Novel rod, sponge, vesicle, lamellae, nanotube phases and reversible transitions among them arise at conditions (concentration, pH, temperature, ionic strength, ionic composition) fitting the physiological working environment of sodium deoxycholate. These findings enlarge the perspective towards different directions. The integration of the previous literature with this work removes any interpretative contradiction since all the structures cover the entire spectrum of phases expected for surfactants, thus being explained according to the Israelachvili's scheme. It is not trivial that a single molecule can show such a high structural variability. This fact highlights a very versatile system. Probably it is not a coincidence that it occurs in a multitasking biomolecule. These results furnish fundamental knowledge to clarify the bile salts' role in vivo.


Subject(s)
Deoxycholic Acid , Surface-Active Agents , Scattering, Small Angle , Water , X-Ray Diffraction
17.
Audiol Neurootol ; 26(6): 435-444, 2021.
Article in English | MEDLINE | ID: mdl-33831862

ABSTRACT

BACKGROUND: The ADHEAR™ system (MED-EL, Innsbruck, Austria) is a nonsurgical bone conduction device (BCD) to treat conductive hearing loss (CHL) and single-sided deafness. In contrast to the nonsurgical alternatives on headbands or spectacle frames, the audio processor of ADHEAR is placed retroauricularly on an adhesive adapter. The published evidence on the performance of this system is limited to studies with a trial period of 2-8 weeks. OBJECTIVE: This study assesses audiological and subjective outcomes over a period of 12 months, on patients with congenital aural atresia (CAA) using the ADHEAR hearing system. METHOD: Fifteen children (mean age: 9.4 ± 4 years; range: 5-16 years) diagnosed with CAA (7 uni/8 bilateral) were included in this prospective, observational, repeated-measures study. Each subject used ADHEAR for 1 year, and the performance was evaluated after 1, 6, and 12 months. Free-field audiometry and speech discrimination tests were performed, and hearing-, general health- and device-specific questionnaires were used. RESULTS: The unaided sound field threshold improved from an average PTA4 of 63.6 ± 3.4 dB HL to an aided average PTA4 of 29.3 ± 3.0 dB HL after 1 month of device use. The word recognition score (WRS) improved from an average of 27.9 ± 15.9% unaided to an aided average WRS of 91.3 ± 4.4% (p = 0.0003) after 1 month, 92.0 ± 4.1% (p = 0.0002) after 6 months, and 92.7 ± 5.3% (p < 0.0001) after 12 months using the ADHEAR system compared to the unaided condition for all 3 time points. The improvements in the speech in noise at 1, 6, and 12 months were as well consistent over time. The average improvement at the signal to noise ratio (SNR) of +5 dB was 58% and 53% at the SNR of +0 dB. No complications were reported, and all patients continued to use the ADHEAR after the study end. The questionnaire results revealed high user satisfaction and an average wearing time of 12 h per day. CONCLUSION: This 12-month trial of the nonsurgical adhesive BCD in CAA patients showed sufficient and reliable audiological and subjective outcomes, long wearing time, and high acceptance. The ADHEAR can be considered a suitable option to treat children with CAA for the given indication, without the drawbacks of nonsurgical devices that use pressure for retention of the audio processor or the costs and possible complications involved with a surgical alternative.


Subject(s)
Hearing Aids , Speech Perception , Adolescent , Bone Conduction , Child , Child, Preschool , Follow-Up Studies , Hearing Loss, Conductive/therapy , Humans , Prospective Studies , Treatment Outcome
18.
Neuroradiology ; 63(8): 1383-1388, 2021 Aug.
Article in English | MEDLINE | ID: mdl-33760956

ABSTRACT

Cortical subarachnoid hemorrhage is an infrequent subtype of non-aneurysmal subarachnoid hemorrhage, rarely reported in watershed territories (wSAH) after carotid stenting. It has never been reported after treatment of middle cerebral artery stenosis (MCAS) that is increasingly used in selected patients, as rescue treatment of failed mechanical thrombectomy, due to recent advancements in endovascular interventions. We present a series of patients with MCAS that developed a wSAH after stenting.


Subject(s)
Brain Ischemia , Carotid Stenosis , Endovascular Procedures , Ischemic Stroke , Stroke , Subarachnoid Hemorrhage , Humans , Middle Cerebral Artery/diagnostic imaging , Retrospective Studies , Stents , Subarachnoid Hemorrhage/diagnostic imaging , Subarachnoid Hemorrhage/therapy , Treatment Outcome
19.
Nat Commun ; 12(1): 957, 2021 02 11.
Article in English | MEDLINE | ID: mdl-33574249

ABSTRACT

The coexistence of single-crystallinity with a multidomain morphology is a paradoxical phenomenon occurring in biomineralization. Translating such feature to synthetic materials is a highly challenging process in crystal engineering. We demonstrate the formation of metallo-organic single-crystals with a unique appearance: six-connected half-rods forming a hexagonal-like tube. These uniform objects are formed from unstable, monodomain crystals. The monodomain crystals dissolve from the inner regions, while material is anisotropically added to their shell, resulting in hollow, single-crystals. Regardless of the different morphologies and growth mechanism, the crystallographic structures of the mono- and multidomain crystals are nearly identical. The chiral crystals are formed from achiral components, and belong to a rare space group (P622). Sonication of the solvents generating radical species is essential for forming the multidomain single-crystals. This process reduces the concentration of the active metal salt. Our approach offers opportunities to generate a new class of crystals.

20.
Int J Mol Sci ; 22(4)2021 Feb 10.
Article in English | MEDLINE | ID: mdl-33579036

ABSTRACT

Bile acids (BAs) are facial amphiphiles synthesized in the body of all vertebrates. They undergo the enterohepatic circulation: they are produced in the liver, stored in the gallbladder, released in the intestine, taken into the bloodstream and lastly re-absorbed in the liver. During this pathway, BAs are modified in their molecular structure by the action of enzymes and bacteria. Such transformations allow them to acquire the chemical-physical properties needed for fulling several activities including metabolic regulation, antimicrobial functions and solubilization of lipids in digestion. The versatility of BAs in the physiological functions has inspired their use in many bio-applications, making them important tools for active molecule delivery, metabolic disease treatments and emulsification processes in food and drug industries. Moreover, moving over the borders of the biological field, BAs have been largely investigated as building blocks for the construction of supramolecular aggregates having peculiar structural, mechanical, chemical and optical properties. The review starts with a biological analysis of the BAs functions before progressively switching to a general overview of BAs in pharmacology and medicine applications. Lastly the focus moves to the BAs use in material science.


Subject(s)
Bile Acids and Salts/metabolism , Animals , Bile Acids and Salts/analysis , Bile Acids and Salts/chemical synthesis , Cholesterol/metabolism , Gallbladder/metabolism , Humans , Intestinal Mucosa/metabolism , Lipid Metabolism , Liver/metabolism
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