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Δευτέρα 30 Νοεμβρίου 2020

Investigation of Vestibular Function in Adult Patients with Gitelman Syndrome:

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Investigation of Vestibular Function in Adult Patients with Gitelman Syndrome: Results of an Observational Study.

J Clin Med. 2020 Nov 23;9(11):

Authors: Alexandru M, Courbebaisse M, Le Pajolec C, Ménage A, Papon JF, Vargas-Poussou R, Nevoux J, Blanchard A

Abstract
Gitelman syndrome (GS) is a rare salt-losing tubulopathy caused by an inactivating mutation in the SLC12A3 gene, encoding the thiazide-sensitive sodium chloride cotransporter (NCC). Patients with GS frequently complain of vertigo, usually attributed to hypovolemia. Because NCC is also located in the endolymphatic sac, we hypothesized that patients with GS might have vestibular dysfunction. Between April 2013 and September 2016, 20 (22%) out of 90 patients followed at the reference center complained of vertigo in the absence of orthostatic hypotension. Sixteen of them were referred to an otology department for investigation of vestibular function. The vertigo was of short duration and triggered in half of them by head rotation. Seven patients (44%) had a vestibular syndrome. Vestibular syndrome was defined: (1) clinically, as nystagmus triggered by the head shaking test (n = 5); and/or (2) paraclinically, as an abnormal video head impulse test (n = 0), abnormal kinetic test (n = 4) and/or abnormal bithermal caloric test (n = 3). Five patients had associated auditory signs (tinnitus, aural fullness or hearing loss). In conclusion, we found a high frequency of vestibular disorder in GS patients suffering from vertigo, suggesting a role of NCC in the inner ear. Referent physicians of these patients should be aware of this extrarenal manifestation that requires specific investigations and treatment.

PMID: 33238651 [PubMed]

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Pupillometry Assessment of Speech Recognition and Listening Experience

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Pupillometry Assessment of Speech Recognition and Listening Experience in Adult Cochlear Implant Patients.

Front Neurosci. 2020;14:556675

Authors: Russo FY, Hoen M, Karoui C, Demarcy T, Ardoint M, Tuset MP, De Seta D, Sterkers O, Lahlou G, Mosnier I

Abstract
Objective: The aim of the present study was to investigate the pupillary response to word identification in cochlear implant (CI) patients. Authors hypothesized that when task difficulty (i.e., addition of background noise) increased, pupil dilation markers such as the peak dilation or the latency of the peak dilation would increase in CI users, as already observed in normal-hearing and hearing-impaired subjects.
Methods: Pupillometric measures in 10 CI patients were combined to standard speech recognition scores used to evaluate CI outcomes, namely, speech audiometry in quiet and in noise at +10 dB signal-to-noise ratio (SNR). The main outcome measures of pupillometry were mean pupil dilation, maximal pupil dilation, dilation latency, and mean dilation during return to baseline or retention interval. Subjective hearing quality was evaluated by means of one self-reported fatigue questionnaire, and the Speech, Spatial, and Qualities (SSQ) of Hearing scale.
Results: All pupil dilation data were transformed to percent change in event-related pupil dilation (ERPD, %). Analyses show that the peak amplitudes for both mean pupil dilation and maximal pupil dilation were higher during the speech-in-noise test. Mean peak dilation was measured at 3.47 ± 2.29% noise vs. 2.19 ± 2.46 in quiet and maximal peak value was detected at 9.17 ± 3.25% in noise vs. 8.72 ± 2.93% in quiet. Concerning the questionnaires, the mean pupil dilation during the retention interval was significantly correlated with the spatial subscale score of the SSQ Hearing scale [r(8) = -0.84, p = 0.0023], and with the global score [r(8) = -0.78, p = 0.0018].
Conclusion: The analysis of pupillometric traces, obtained during speech audiometry in quiet and in noise in CI users, provided interesting information about the different processes engaged in this task. Pupillometric measures could be indicative of listening difficulty, phoneme intelligibility, and were correlated with general hearing experience as evaluated by the SSQ of Hearing scale. These preliminary results show that pupillometry constitutes a promising tool to improve objective quantification of CI performance in clinical settings.

PMID: 33240035 [PubMed]

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CT-guided iodine-125 seed implantation assisted by 3D non-coplanar template for retroperitoneal recurrent carcinoma.

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The accuracy and safety of CT-guided iodine-125 seed implantation assisted by 3D non-coplanar template for retroperitoneal recurrent carcinoma.

World J Surg Oncol. 2020 Nov 25;18(1):307

Authors: Jiang W, Jiang P, Wei S, Jiang Y, Ji Z, Sun H, Fan J, Li W, Shao Y, Wang J

Abstract
PURPOSE: To investigate the accuracy, dosimetric parameters, and safety of 3D-printing non-coplanar template (3D-PNCT)-assisted CT guidance for radioactive iodine-125 (125I) seed implantation brachytherapy (RSI-BT) for retroperitoneal recurrent carcinomas METHODS AND MATERIALS: We enrolled 15 patients with 17 retroperitoneal recurrent carcinomas after external beam radiotherapy (EBRT). All patients received CT-guided 125I RSI-BT assisted by 3D-PNCT successfully. We compared the original needle insertion position, angular, and the needle tip distance deviations of preoperative plan with that of intraoperative in brachytherapy treatment planning system (B-TPS). The dosimetric parameters of RSI-BT were evaluated on preoperative plan, intraoperative real-time plan, and postoperative plan, including D90, D100 (the dose to 90% and 100% of the target volume), V100, V150, and V200 (the volume receives 100%, 150%, and 200% of the prescribed doses). The quality assurance of RSI-BT eval uated on conformal index (CI), external index (EI), and homogeneity index (HI) of the targets were compared among preoperative plan, intraoperative real-time plan, and postoperative plan. The perioperation complications and RSI-BT-related toxicity were assessed.
RESULTS: The median follow-up was 8.2 months (range 1-18.5 months). One patient was lost to follow-up after RSI-BT. Fourteen patients were assessed for response rate and toxicity. The mean entrance point distance deviation for all 165 needles was 4.50 ± 4.10 mm (range, 0-30). The mean angular deviation was 2.70 ± 3.00° (range, 0-20). The needle tip distance deviation was 6.90 ± 6.00 mm (range, - 30-28). D90 for preoperative plan, intraoperative plan, and postoperative plan were 140.55 ± 23.93, 124.25 ± 28.04, and 128.98 ± 22.75, respectively. There was significant difference between D90 of preoperative plan with that of intraoperative plan (p = 0.036). Four lesions reached CR, six lesions reached PR, three lesions were SD, and three lesions were PD. Four patients with moderate pain became mild, and two with mild pain relieved completely after RSI-BT. The other parameters showed no differences among preoperative plan, intraoperative plan, and postoperative plan. The perioperative complications were observed in four patients, including three patients of grade 1 and one patient of grade 2. No ≥ grade 3 side effects were observed.
CONCLUSION: CT-guided 125I RSI-BT assisted by 3D-PNCT was a safe, accurate, and feasible strategy for recurrent carcinomas located in the retroperitoneal regions.

PMID: 33239023 [PubMed - in process]

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FDA Approval Selpercatinib for the Treatment of Lung and Thyroid cancers with RET Gene Mutations or Fusions.

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FDA Approval Summary: Selpercatinib for the Treatment of Lung and Thyroid cancers with RET Gene Mutations or Fusions.

Clin Cancer Res. 2020 Nov 25;:

Authors: Bradford D, Larkins E, Mushti SL, Rodriguez L, Skinner AM, Helms WS, Price LSL, Fourie Zirkelbach J, Li Y, Liu J, Charlab R, Reyes Turcu F, Liang D, Ghosh S, Roscoe D, Philip R, Zack-Taylor A, Tang S, Kluetz PG, Beaver JA, Pazdur R, Theoret MR, Singh H

Abstract
On May 8, 2020, the Food and Drug Administration granted accelerated approval to selpercatinib for 1) adult patients with metastatic RET fusion-positive non-small cell lung cancer (NSCLC), 2) adult and pediatric patients ≥12 years of age with advanced or metastatic RET-mutant medullary thyroid cancer (MTC) who require systemic therapy, and 3) adult and pediatric patients ≥12 years of age with advanced or metastatic RET fusion-positive thyroid cancer who require systemic therapy and who are radioactive iodine-refractory (if radioactive iodine is appropriate). Approval was granted based on the clinically important effects on overall response rate (ORR) with prolonged duration of responses observed in a multicenter, open-label, multi-cohort clinical trial (LIBRETTO-001, NCT03157128) in patients whose tumors had RET alterations. ORRs within the approved patient populations ranged from 64% (95% CI: 54, 73) in prior platinum treated RET fusion-positive NSCLC to 100% (95% CI: 63 , 100) in systemic therapy naïve RET fusion-positive thyroid cancer, with the majority of responders across indications demonstrating responses of at least 6 months. The product label includes warnings and precautions for hepatotoxicity, hypertension, QT interval prolongation, hemorrhagic events, hypersensitivity, risk of impaired wound healing, and embryo-fetal toxicity. This is the first approval of a drug specifically for patients with RET alterations globally.

PMID: 33239432 [PubMed - as supplied by publisher]

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Gender differences in estimating I-131 thyroid uptake from Tc-99m thyroid uptake for benign thyroid disease.

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Gender differences in estimating I-131 thyroid uptake from Tc-99m thyroid uptake for benign thyroid disease.

Br J Radiol. 2020 Nov 26;:20200700

Authors: Al-Jabri A, Cooke J, Cournane S, Healy ML

Abstract
OBJECTIVE: For radioactive Iodine-131 (131I) treatments of thyroid diseases, increased efficacy has been reported for personalized dosimetry treatments. The measurement of Iodine-131 thyroid uptake (131IU) is required in these cases. This study aims to investigate whether 99mTc thyroid uptake (99mTcU) may be used in place of 131IU for implementing personalised treatments.
METHODS: A retrospective study of 152 benign thyroid disease 131I treatments was carried out during 2012-2020; 117 treatments were for female patients while 35 were for male patients diagnosed with either Graves' disease, multinodular goitre or toxic nodules.
RESULTS: A statistically significant correlation was found between 131IU and 99mTcU data, with the data more correlated for male than female patients (r = 0.71 vs 0.38, p-value < 0.001). Patient age and time difference between the two respective uptake measurements significantly influenced the uptake correlation in females but not for the male cohort, although there was no significant difference between the parameters across gender. Thyroid diagnosis and hormone levels showed a significant correlation with uptakes in both genders. Estimating 131IU based on 99mTcU was shown to be predictive for male but not in female patients (R2 = 91% vs 16%).
CONCLUSION: Estimating 131IU based on 99mTcU is not recommended for females at our centre. Males reported good correlation, but a larger sample would be needed for validation.
ADVANCES IN KNOWLEDGE: The initial findings showed a significant gender difference in benign thyroid uptake parameters at our centre, highlighting the potential need for gender consideration when planning 131IU patient management and when reporting studies results.

PMID: 33242250 [PubMed - as supplied by publisher]

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Peri-intraventricular hemorrhage: the inhibitory effect of auditory efferent pathway

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Abstract: Purpose: to determine the functioning of the efferent auditory system in premature newborns with intraventricular hemorrhage. Method: the sample consisted of 44 newborns, divided into two groups. The study group was composed of 22 premature newborns with intraventricular hemorrhage/and the control group was composed of 22 newborns without intraventricular hemorrhage, matched to the study group for gestational age, correct gestational age and sex. The groups were submitted to the evaluation of the inhibitory effect of auditory efferent in otoacoustic emissions (equipment ILOv6-Otodynamics Ltda®) and auditory evoked potential with and without contralateral noise (equipment SmartEP-Intelligent Hearing Systems®). Results: newborns with intraventricular hemorrhage exhibited a higher occurrence of central hearing alteration as well as a lesser occurrence of the inhibitory effect of auditory efferent in otoacoustic emissions and auditory evoked potential compared to th e newborns without this condition. An association was found between central hearing alteration and a lower occurrence of inhibitory effect. Agreement was found between the inhibitory effect test on otoacoustic emissions and latency of the auditory evoked potential. Conclusion: premature newborns with intraventricular hemorrhage have a greater occurrence of functional abnormality of the afferent auditory system, which can be effectively identified through an evaluation of the inhibitory effect of auditory efferent in otoacoustic emissions evoked by a transient stimulus and latency parameter in the brainstem auditory evoked potential.
Resumo: Objetivo: verificar o funcionamento do sistema auditivo eferente em recém-nascidos pré-termo com hemorragia peri-intraventricular. Método: a amostra foi constituída por 44 recém-nascidos, distribuídos em dois grupos. O grupo estudo foi composto por 22 recém-nascidos pré-termo com hemorragia peri-intraventricular, e o grupo contro le por 22 sem hemorragia peri-intraventricular, pareados por idade gestacional, idade gestacional corrigida e sexo. Os grupos foram submetidos à avaliação do efeito inibitório da via auditiva eferente nas emissões otoacústicas (equipamento ILOv6-Otodynamics Ltda ® ) e no potencial evocado auditivo de tronco encefálico, sem e com ruído contralateral (equipamento Smart-EP-Intelligent Hearing Systems ® ). Resultados: recém-nascidos com hemorragia peri-intraventricular apresentaram maior ocorrência de alteração central, além de menor ocorrência do efeito inibitório da via auditiva eferente nas emissões otoacústicas e no potencial evocado auditivo. Houve associação entre alteração auditiva central e menor ocorrência do efeito inibitório. Observou-se, também, concordância entre o efeito inibitório da via auditiva eferente nas emissões otoacústicas e nas latências do potencial evocado auditivo. Conclusão: recém-nascidos pré-termo com hemorragia peri- intraventricular apresentaram maior ocorrência de alteração funcional do sistema auditivo eferente, que pôde ser identificada de forma efetiva por meio da avaliação do efeito inibitório da via eferente nas emissões otoacústicas evocadas por estímulo transiente e no parâmetro latência do potencial evocado auditivo de tronco encefálico.
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Postural balance in type 2 diabetics with vertigo, dizziness and/or unsteadiness

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Abstract: Purpose: To evaluate the postural balance of type 2 diabetics with vertigo, dizziness, and/or unsteadiness. Methods: limit of stability, pressure center displacement area, and sway velocity of 20 patients with type 2 diabetes were compared with 22 controls using the Balance Rehabilitation Unit (BRUTM, Medicaa) posturography. Results: Compared to the control group, patients with type 2 diabetes showed a significantly lower limit of stability and a significantly higher-pressure center displacement area on a firm surface with eyes open, eyes closed, and horizontal vestibular-visual interaction; and higher sway velocity on a firm surface with eyes open and with eyes closed. Conclusion: type 2 diabetics with vertigo, dizziness, and/or imbalance compromised postural balance related to visual stimuli and vestibular-visual interaction and moderate impairment in the quality of life.
Resumo: Objetivo: avaliar o equilíbrio postural em diabéticos tipo 2 com vertige m, tontura e/ou instabilidade. Método: área do limite de estabilidade, área de deslocamento do centro de pressão e velocidade de oscilação de 20 pacientes com diabetes mellitus tipo 2 com vertigem, tontura e/ou instabilidade foram comparados a um grupo controle com 22 indivíduos hígidos à posturografia do Balance Rehabilitation Unit (BRUTM). Resultados: a área do limite de estabilidade dos diabéticos tipo 2 foi significantemente menor do que a do grupo controle. Os diabéticos tipo 2 apresentaram valores significantemente maiores quanto à área de deslocamento do centro de pressão, em superfície firme, de olhos abertos, olhos fechados e com interação visuovestibular na direção horizontal; e quanto à velocidade de oscilação em superfície firme, de olhos abertos e olhos fechados. Conclusão: diabéticos tipo 2 com vertigem, tontura e/ou instabilidade apresentam comprometimento do equilíbrio postural relacionado com estímulos visuais e de interação visuo vestibular e prejuízo moderado na qualidade de vida.
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Targeting NRF2 to suppress ferroptosis in brain injury.

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Targeting NRF2 to suppress ferroptosis in brain injury.

Histol Histopathol. 2020 Nov 26;:18286

Authors: Song S, Gao Y, Sheng Y, Rui T, Luo C

Abstract
Brain injury is accompanied by serious iron metabolism disorder and oxidative stress. As a novel form of regulated cell death (RCD) depending on lipid peroxidation caused by iron overload, ferroptosis (FPT) further aggravates brain injury, which is different from apoptosis, autophagy and other traditional cell death in terms of biochemistry, morphology and genetics. Noteworthy, transcriptional regulator NRF2 plays a key role in the cell antioxidant system, and many genes related to FPT are under the control of NRF2, including genes for iron regulation, thiol-dependent antioxidant system, enzymatic detoxification of RCS and carbonyls, NADPH regeneration and ROS sources from mitochondria or extra-mitochondria, which place NRF2 in the key position of regulating the ferroptotic death. Importantly, NRF2 can reduce iron load and resist FPT. In the future, it is expected to open up a new way to treat brain injury by targeting NRF2 to alleviate FPT in brain.

PMID: 33242213 [PubMed - as supplied by publisher]

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Facial nerve stimulation post-cochlear implantation.

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Evaluation of computed tomography parameters in patients with facial nerve stimulation post-cochlear implantation.

Eur Arch Otorhinolaryngol. 2020 Nov 26;:

Authors: Aljazeeri IA, Khurayzi T, Al-Amro M, Alzhrani F, Alsanosi A

Abstract
PURPOSE: To compare the preoperative computed tomography (CT) parameters, including the thickness and density of the bone separating the upper basal turn of the cochlea (UBTC) and the labyrinthine segment of the facial nerve (LSFN), in patients with and without facial nerve stimulation (FNS) in post-cochlear implants (CI).
METHODS: A retrospective case review of 1700 CI recipients in a tertiary referral center between January 2010 and January 2020 was performed; out of the 35 recipients who were found to have FNS, 29 were included in the study. The control group comprised the same number of randomly selected patients. CT parameters of the patients were measured independently by three fellowship-trained neuro-otologists blinded to the postoperative status of the patients. Thickness in axial and coronal views and density of the bone separating the UBTC and the LSFN were measured.
RESULT: There was satisfactory agreement between the readings of the three reviewers. The distances (in mm) between the UBTC and LSFN obtained from the coronal (0.43 ± 0.24 vs. 0.63 ± 0.2) and axial (0.42 ± 0.25 vs. 0.6 ± 0.18) views were statistically lower in the FNS group (p = 0.001 and 0.005, respectively). The density (in HU) of the bony partition was also statistically lower in the FNS group (1038 ± 821 vs. 1409 ± 519; p = 0.029).
CONCLUSION: Patients who experienced FNS postoperatively had significantly lower distance and bone density between the UBTC and the LSFN. This finding can help surgeons in preoperative planning in an attempt to decrease the occurrence of FNS.

PMID: 33242112 [PubMed - as supplied by publisher]

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Spatial Segmentation for Laryngeal High-Speed Videoendoscopy in Connected Speech

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This study proposes a new computational framework for automated spatial segmentation of the vocal fold edges in high-speed videoendoscopy (HSV) data during connected speech. This spatio-temporal analytic representation of the vocal folds enables the HSV-based measurement of the glottal area waveform and other vibratory characteristics in the context of running speech.
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Association of hepatitis B genotypes with clinical profile of patients with chronic hepatitis B.

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Association of hepatitis B genotypes with clinical profile of patients with chronic hepatitis B.

Int J Mol Epidemiol Genet. 2020;11(2):26-30

Authors: Baclig MO, Reyes KG, Liles VR, Gopez-Cervantes J

Abstract
Hepatitis B virus (HBV) infection is a common cause of chronic liver disease and is responsible for HBV-related deaths due to cirrhosis and HCC. It is well recognized that viral genotypes play an important role on the outcome of HBV infection. Ten HBV genotypes have been identified and the prevalence varies geographically. A hospital-based cross-sectional study was conducted to determine the association of HBV genotypes with the clinical profile of CHB patients. PCR-RFLP was performed to identify HBV genotypes. In this study, majority (70%) of patients were males; with ages between 22 to 67 years with a mean of 42.5 years. The ALT ranged from 23 to 111 U/L (mean 72.5 U/L). HBV DNA levels varied from less than 6 to more than 110,000,000 IU/ml. Forty-seven percent of the patients had chronic active hepatitis at the time of diagnosis. Of these, 36% were HBeAg positive while 64% were HBeAg negative. Inactive HBsAg carrier was found in 53% of cases. No significant association was established between HBV genotypes and fibrosis. PCR-RFLP analysis showed that 57%, 10%, and 13% of the samples belonged to HBV/A, HBV/B, and HBV/C, respectively and the remaining 20% had non-detectable HBV genotype. HBV/D to HBV/J were not observed in this study. Taken together, the patient's clinical profile such as sex, ALT levels, HBeAg status, HBV DNA levels and liver histology were not found to be significantly associated with HBV genotypes. A large-scale longitudinal study examining multiple HBV strains are needed to determine significant correlation of clinical profile.

PMID: 33240460 [PubMed]

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