mTOR Inhibitors and Life-Threatening Conditions in Tuberous Sclerosis Complex

Tuberous sclerosis complex (TSC) is a multisystem disease associated with an overall reduction in life expectancy due to the possible occurrence of different life-threatening conditions. Subjects affected by TSC are in fact at risk of hydrocephalus secondary to the growth of subependymal giant cell astrocytomas (SEGA), or of sudden unexpected death in epilepsy. Other non-neurological life-threatening conditions include abdominal bleeding due to renal angiomyolipomas (AML) rupture, renal insufficiency due to progressive parenchymal destruction by multiple cysts, pulmonary complications due to lymphangioleiomyomatosis (LAM), and cardiac failure or arrhythmias secondary to rhabdomyomas.
Source: Seminars in Pediatric Neurology - Category: Neurology Authors: Source Type: research

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The objectives of this video are to describe common anatomic and pathologic challenges for achieving hemostasis, to outline the strategies for laparoscopic dissection to prevent bleeding and to deconstruct the process of regaining hemostasis when brisk bleeding occurs.
Source: American Journal of Obstetrics and Gynecology - Category: OBGYN Authors: Tags: Video Presentation Source Type: research
Vagus nerve stimulation for refractory epilepsy may induce laryngeal side effects such as dysphonia and dysphagia. Careful tuning of the stimulation parameters and collaboration between epileptologists and otolaryngologists can help significantly reduce side effects. AbstractVagus nerve stimulation for refractory epilepsy may induce laryngeal side effects such as dysphonia and dysphagia. Careful tuning of the stimulation parameters and collaboration between epileptologists and otolaryngologists can help significantly reduce side effects.
Source: Clinical Case Reports - Category: General Medicine Authors: Tags: CASE REPORT Source Type: research
We report a series of patients who underwent simultaneous tilt table testing with EEG. Diagnostic benefits are not exclusively the verification of EEG correlates secondary to clinical and cardiovascular changes during syncope, but also confirmation of nonphysiological events, including psychogenic nonepileptic seizures and psychogenic pseudosyncope. Fifty tilt table studies were combined with EEG over a six-year period. Seven studies (14%) captured episodes of behavioral unresponsiveness with normal EEG and EKG patterns, diagnostic of psychogenic pseudosyncope. Whereas psychogenic nonepileptic events are well established i...
Source: The Neurodiagnostic Journal - Category: Neurology Tags: Neurodiagn J Source Type: research
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Source: European Journal of Emergency Medicine - Category: Emergency Medicine Tags: Perspective Articles Source Type: research
AbstractMinesapride (drug code: DSP ‐6952) is a potential gastrointestinal prokinetic agent with high selectivity for 5‐hydroxytryptamine 4 (5‐HT4) receptor that acts as a partial agonist. Although 5 ‐HT4 receptor agonists are expected to show efficacy in patients with irritable bowel syndrome with constipation, only tegaserod is available for female patients, with limitations, in the United States. Previously, another 5 ‐HT4 receptor agonist, cisapride, was widely used for the treatment of upper gastrointestinal diseases, but was withdrawn from the market because of arrhythmia with QT prolongation. Chemically, b...
Source: Clinical Pharmacology in Drug Development - Category: Drugs & Pharmacology Authors: Tags: Original Manuscript Source Type: research
ConclusionsFetuses with structurally normal hearts and sustained SVT can be effectively managed with transplacental drug therapy with minimal risk of intrauterine fetal demise. Treatment requires multiple anti ‐arrhythmic agents in over half of cases. Later gestational age at fetal diagnosis and the presence of depressed fetal ventricular function, but not hydrops, predict postnatal arrhythmia burden.This article is protected by copyright. All rights reserved.
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Authors: Akiyama T, Saigusa D, Hyodo Y, Umeda K, Saijo R, Koshiba S, Kobayashi K Abstract To characterize metabolic profiles within the central nervous system in epilepsy, we performed gas chromatography-tandem mass spectrometry (GC-MS/MS)-based metabolome analysis of the cerebrospinal fluid (CSF) in pediatric patients with and without epilepsy. The CSF samples obtained from 64 patients were analyzed by GC-MS/MS. Multivariate analyses were performed for two age groups, 0-5 years of age and 6-17 years of age, to elucidate the effects of epilepsy and antiepileptic drugs on the metabolites. In patients aged 0-5 years ...
Source: Acta Medica Okayama - Category: General Medicine Tags: Acta Med Okayama Source Type: research
AbstractPlatelets crucially facilitate wound healing but can become depleted in traumatic injury or chronic wounds. Previously, our group developed injectable platelet ‐like particles (PLPs) comprised of highly deformable, ultralow crosslinked pNIPAm microgels (ULCs) coupled to fibrin binding antibodies to treat post‐trauma bleeding. PLP fibrin‐binding facilitates homing to sites of injury, promotes clot formation, and, due to high particle deformability, in duces clot retraction. Clot retraction augments healing by increasing clot stability, enhancing clot stiffness, and promoting cell migration into the wound bed. ...
Source: Journal of Biomedical Materials Research Part B: Applied Biomaterials - Category: Materials Science Authors: Tags: ORIGINAL RESEARCH REPORT Source Type: research
AbstractBackgroundThe optimal mitral prosthesis in young patients is unclear. This systematic review and meta ‐analysis were performed to compare outcomes between bileaflet mechanical mitral valve replacement (mMVR) and bioprosthesis mitral valve replacement (bioMVR) for MVR patients aged less than 70 years.MethodsWe searched MEDLINE and EMBASE databases from inception to July 2018 for studies comparing surgical outcomes of mMVR vs bioMVR.ResultsThere were 14 observational studies with 20  219 patients (n = 14 658 mMVR and n = 5561 bioMVR). Patients receiving an mMVR were younger...
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
Publication date: June 2020Source: Interdisciplinary Neurosurgery, Volume 20Author(s): Takeshi Ogura, Kazuma Kowata, Mayu Nakajima, Hiroaki Neki, Shigehiro Oomori
Source: Interdisciplinary Neurosurgery - Category: Neurosurgery Source Type: research
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