Customised acoustic therapy delivered through a web‐based platform – an innovative approach to tinnitus treatment

alexandrossfakianakis shared this article with you from Inoreader Customised acoustic therapy delivered through a web‐based platform – an innovative approach to tinnitus treatment via Clinical Otolaryngology Abstract Objectives Customised acoustic therapy aims to moderate the neural pathways implicated in the pathophysiology of tinnitus. This study aimed to assess the efficacy of customised acoustic therapy administered via a web-based treatment platform. Design Clinical trial with prospective recruitment. 58 participants underwent six weeks of customised acoustic therapy. Setting Treatment was delivered for 2 hours each day using a smartphone, tablet or computer. Treatment was integrated into usual daily activities. Participants Participants with subjective tinnitus were recruited through public and private otolaryngology clinics and electronic and print media. Main Outcomes Measured FiveQ, a novel 5 question tinnitus questionnaire, was measured at baseline and each week of treatment. Statistical analyses, including Wilcoxon, Mann-Whitney and mixed linear regression, were used to assess treatment efficacy and identify factors associated with treatment response. Results 39/58 participants (67.2%) had an improvement in symptom severity scores, 4 had no change (6.9%) and 15 had a decline from baseline (25.9%). Mean FiveQ scores improved by 22.9% from 40.8 (SD=21.4) at baseline to 31.5 (SD=21.3) following 6 weeks of treatment (p<0.001). With the exception of the slight tinnitus group, all other groups (from mild to catastrophic) demonstrated a treatment response. Participants with low frequency tinnitus (<2000 Hertz) had a significantly greater treatment response (p<0.001). Conclusion Customised acoustic therapy administered via a web-based platform demonstrated encouraging efficacy. At least mild symptoms at baseline and low frequency tinnitus were associated with a greater treatment response. Customised acoustic therapy offers accessible and efficacious tinnitus treatment, however longer term clinical studies are required to confirm the observed initial benefit is maintained. View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Neuroendokrine Tumoren im Kopf-, Halsbereich

alexandrossfakianakis shared this article with you from Inoreader Neuroendokrine Tumoren im Kopf-, Halsbereich via Laryngo-Rhino-Otologie Laryngorhinootologie DOI: 10.1055/a-1976-9641 Hintergrund Neuroendokrine Tumoren (NET) sind benigne oder maligne Neoplasien, die entwicklungsgeschichtlich der Neuralleiste oder paraganglionären Zellen des vegetativen Nervensystems entstammen. Die alte Bezeichnung Karzinoid (karzinomähnlicher Tumor) ist noch sehr verbreitet, jedoch inzwischen obsolet. Lediglich 0,5–2 % aller Neoplasien des Menschen sind neuroendokrine Tumoren, welche überwiegend gastrointestinal oder bronchopulmonal lokalisiert sind. Die Inzidenz beträgt 9.263/100.000. Jedoch ist nur 1 % der Kopf-Hals-Tumore neuroendokriner Genese. Fallzahlstarke Langzeitstudien und Leitlinien zur Diagnose und Therapie im Kopf-Hals Bereich fehlen. Ziel dieser Arbeit ist der wertende Vergleich bekannter Therapiestrategien und daraus folgender Krankheitsverläufe sowie deren Vergleich mit dem Schrifttum anhand eigener Fälle unterschiedlicher Lokalisation und Ausdehnung. Patienten und Methoden In unserer Klinik wurden zwischen 2010 und 2017 14 Patienten mit neuroendokrinen Tumoren im Kopf-Hals-Bereich behandelt. 8 Patienten davon wurden primär operativ mit adjuvanter Radiochemotherapie versorgt. 6 Patienten erhielten eine primäre Radiochemotherapie in kurativer Absicht. 1 Patient erhielt auf Grund des Tumorprogress nach der primären Radiochemotherapie eine weitere Chemotherapie mit palliativer Indikation. Ergebnisse Die Tumorlokalisationen verteilen sich auf den Larynx (n = 7), die Glandula parotis (n = 2) und auf die Nasenhaupt- und -nebenhöhlen (n=5). Von den 8 operierten Patienten konnte in sechs Fällen eine in-sano Resektion (R0) erreicht werden. Die im Kollektiv gemessene Gesamtüberlebensrate beträgt im Mittel 19±6 Monate. Von den 14 untersuchten Patienten erlitten zwei Patienten ein Rezidiv, wovon ein Patient nach 7 Monaten verstarb und ein Patient 32 Monate nach der Nachresektion Rezidiv-frei lebt. In zwei Fällen sprach das NET nicht auf die kombinierte RCT an und die Patienten verstarben jeweils nach sechs und 13 Monaten. Die Überlebenszeit ist abhängig vom T-Stadium des NET sowie vom Erreichen einer R0-Resektion. Die R0-Resektion bietet eine höhere Überlebenswahrscheinlichkeit als die primäre RCT. Schlussfolgerung Patienten mit NET des Kopf-Hals-Bereiches müssen in spezialisierten Zentren interdisziplinär behandelt werden. Zur Erfassung dieser Daten ist eine Tumordatenbank notwendig. Jedoch sind aufgrund der niedrigen Inzidenz keine Studien auf hohem Evidenzniveau zu erwarten. Nach effizienter Diagnosestellung sollte zeitliche Verzögerung unmittelbar im Rahmen einer interdisziplinären Tumorkonferenz eine individuelle auf den Patienten zugeschnittene Therapie erfolgen. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

enzyme replacement therapy followed by stem cell transplantation in children with Gaucher disease type 1 and 3

alexandrossfakianakis shared this article with you from Inoreader Comprehensive and long‐term outcomes of enzyme replacement therapy followed by stem cell transplantation in children with Gaucher disease type 1 and 3 via Pediatric Blood & Cancer Abstract Background Gaucher disease (GD) is a lysosomal storage disorder, characterized by hepatosplenomegaly, pancytopenia, bone diseases, with or without neurological symptoms. Plasma glucosylsphingosine (lyso-Gb1), a highly sensitive and specific biomarker for GD, has been used for diagnosis and monitoring the response to treatment. Enzyme replacement therapy (ERT) is an effective treatment for the non-neurologic symptoms of GD. Neuronopathic GD (type 2 and 3) accounts for 60%–70% of the Asian affected population. Methods We explored combination therapy of ERT followed by hematopoietic stem cell transplantation (HSCT) and its long-term outcomes in patients with GD type 3 (GD3). Results Four patients with GD3 and one with GD type 1 (GD1) underwent HSCT. The types of donor were one matched-related, one matched-unrelated, and three haploidentical. The age at disease onset was 6–18 months and the age at HSCT was 3.8–15 years in the patients with GD3. The latest age at follow-up was 8–22 years, with a post-HSCT duration of 3–14 years. All patients had successful HSCT. Chronic graft-versus-host disease occurred in one patient. The enzyme activities were normalized at 2 weeks post HSCT. Lyso-Gb1 concentrations became lower than the pathological value. All of the patients are still alive and physically independent. Most of them (4/5) returned to school. None of the patients with GD3 had seizures or additional neurological symptoms after HSCT, but showed varying degrees of cognitive impairment. Conclusions ERT followed by HSCT could be considered as an alternative treatment for patients with GD3 who have a high risk of fatal neurological progression. View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Dendritic spine loss in epileptogenic Type II focal cortical dysplasia: Role of enhanced classical complement pathway activation

alexandrossfakianakis shared this article with you from Inoreader Dendritic spine loss in epileptogenic Type II focal cortical dysplasia: Role of enhanced classical complement pathway activation via Brain Pathology Abstract Dendritic spines are the postsynaptic sites for most excitatory glutamatergic synapses. We previously demonstrated a severe spine loss and synaptic reorganization in human neocortices presenting Type II focal cortical dysplasia (FCD), a developmental malformation and frequent cause of drug-resistant focal epilepsy. We extend the findings, investigating the potential role of complement components C1q and C3 in synaptic pruning imbalance. Data from Type II FCD were compared with those obtained in focal epilepsies with different etiologies. Neocortical tissues were collected from 20 subjects, mainly adults with a mean age at surgery of 31 years, admitted to epilepsy surgery with a neuropathological diagnosis of: cryptogenic, temporal lobe epilepsy with hippocampal sclerosis, and Type IIa/b FCD. Dendritic spine density quantitation, evaluated in a previous paper using Golgi impregnation, was available in a subgroup. Immunohistochemistry, in situ hybridization, electron microscopy, a nd organotypic cultures were utilized to study complement/microglial activation patterns. FCD Type II samples presenting dendritic spine loss were characterized by an activation of the classical complement pathway and microglial reactivity. In the same samples, a close relationship between microglial cells and dendritic segments/synapses was found. These features were consistently observed in Type IIb FCD and in 1 of 3 Type IIa cases. In other patient groups and in perilesional areas outside the dysplasia, not presenting spine loss, these features were not observed. In vitro treatment with complement proteins of organotypic slices of cortical tissue with no sign of FCD induced a reduction in dendritic spine density. These data suggest that dysregulation of the complement system plays a role in microglia-mediated spine loss. This mechanism, known to be involved in the removal of redundant synapses during development, is likely reactivated in Type II FCD, particularly in Type IIb; loc al treatment with anticomplement drugs could in principle modify the course of disease in these patients. View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Epstein–Barr virus‐positive diffuse large B‐cell lymphoma onset as familial hemophagocytic lymphohistiocytosis in an infant

alexandrossfakianakis shared this article with you from Inoreader Epstein–Barr virus‐positive diffuse large B‐cell lymphoma onset as familial hemophagocytic lymphohistiocytosis in an infant via Pediatric Blood & Cancer View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Imaging of pediatric calvarial and skull base tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper

alexandrossfakianakis shared this article with you from Inoreader Imaging of pediatric calvarial and skull base tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper via Pediatric Blood & Cancer Abstract A standardized imaging protocol for pediatric oncology patients is essential for accurate and efficient imaging, while simultaneously promoting collaborative understanding of pathologies and radiologic assessment of treatment response. The objective of this article is to provide standardized pediatric imaging guidelines and parameters for evaluation of tumors of the pediatric orbit, calvarium, skull base, and temporal bone. This article was drafted based on current scientific literature as well as consensus opinions of imaging experts in collaboration with the Children's Oncology Group Diagnostic Imaging Committee, Society of Pediatric Radiology Oncology Committee, and American Society of Pediatric Neuroradiology. View on Web

Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.