Sleep apnea-hypopnea quantification by cardiovascular data analysis.

Sleep disorders are a major risk factor for cardiovascular diseases. Sleep apnea is the most common sleep disturbance and its detection relies on a polysomnography, i.e.

Supraspinal gene transfer by intrathecal adeno-associated virus serotype 5.

We report the pattern of transgene expression across brain regions after intrathecal delivery of adeno-associated virus serotype 5 (AAV5). Labeling in hindbrain appeared to be primarily neuronal, and was detected in sensory nuclei of medulla, pontine nuclei, and all layers of cerebellar cortex. Expression in midbrain was minimal, and generally limited to isolated neurons and astrocytes in the cerebral peduncles.

Robust fetal ECG extraction and detection from abdominal leads.

The fetal ECG derived from abdominal leads provides an alternative to standard means of fetal monitoring. Furthermore, it permits long-term and ambulant recordings, which expands the range diagnostic possibilities for evaluating the fetal health state. However, due to the temporal and spectral overlap of maternal and fetal signals, the usage of abdominal leads imposes the need for elaborated signal processing routines.

Biodistribution of adeno-associated virus serotype 9 (AAV9) vector after intrathecal and intravenous delivery in mouse.

Adeno-associated virus serotype 9 (AAV9)-mediated gene transfer has been reported in central nervous system (CNS) and peripheral tissues. The current study compared the pattern of expression of Green Fluorescent Protein (GFP) across the mouse CNS and selected peripheral tissues after intrathecal (i.t.

Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches.

Thrombotic microangiopathy (TMA) is a rare but severe disorder characterized by endothelial cell activation and thrombus formation. It manifests with the triad of hemolytic anemia, thrombocytopenia, and organ failure. Prompt diagnosis and treatment initiation are crucial for long-term outcome.

Cushing's syndrome in a morbidly obese patient undergoing evaluation before bariatric surgery.

Objective: Cushing's syndrome (CS) is extremely rare in morbidly obese patients. To date, no occurrences in obese patients with BMI above 60 kg/m2 have been reported in the literature.

Case Report: This case report describes a patient who was admitted to the ward of the Clinical Division of Endocrinology and Metabolism of the Medical University of Vienna in preparation for bariatric surgery.

An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome.

Background: In 2009, the European Paediatric Study Group for Haemolytic Uraemic Syndrome (HUS) published a clinical practice guideline for the investigation and initial therapy of diarrhea-negative HUS (now more widely referred to as atypical HUS, aHUS). The therapeutic component of the guideline (comprising early, high-volume plasmapheresis) was derived from anecdotal evidence and expert consensus, and the authors committed to auditing outcome.

Methods: Questionnaires were distributed to pediatric nephrologists across Europe, North America, and the Middle East, who were asked to complete one questionnaire per patient episode of aHUS between July 1, 2009 and December 31, 2010.

Treatment of enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome (eHUS).

Treatment of enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome (eHUS) still mostly relies on supportive intensive care regimens. Antibiotic treatment, as administered to eHUS patients during the 2011 O104:H4 outbreak, may reduce the shedding period, but this may apply only to this particular strain. In any case, there is no evidence for a beneficial use in the diarrheal phase and earlier warnings that antibiotic therapy at this stage may actually increase the likelihood of HUS remain unrefuted.

Repeat treatment of acute hereditary angioedema attacks with open-label icatibant in the FAST-1 trial.

Hereditary angioedema (HAE) is characterized by potentially life-threatening recurrent episodes of oedema. The open-label extension (OLE) phase of the For Angioedema Subcutaneous Treatment (FAST)-1 trial (NCT00097695) evaluated the efficacy and safety of repeated icatibant exposure in adults with multiple HAE attacks. Following completion of the randomized, controlled phase, patients could receive open-label icatibant (30 mg subcutaneously) for subsequent attacks.

A consensus parameter for the evaluation and management of angioedema in the emergency department.

Despite its relatively common occurrence and life-threatening potential, the management of angioedema in the emergency department (ED) is lacking in terms of a structured approach. It is paramount to distinguish the different etiologies of angioedema from one another and more specifically differentiate histaminergic-mediated angioedema from bradykinin-mediated angioedema, especially in lieu of the more novel treatments that have recently become available for bradykinin-mediated angioedema. With this background in mind, this consensus parameter for the evaluation and management of angioedema attempts to provide a working framework for emergency physicians (EPs) in approaching the patient with angioedema in terms of diagnosis and management in the ED.

Cardio-respiratory coordination increases during sleep apnea.

Cardiovascular diseases are the main source of morbidity and mortality in the United States with costs of more than $170 billion. Repetitive respiratory disorders during sleep are assumed to be a major cause of these diseases. Therefore, the understanding of the cardio-respiratory regulation during these events is of high public interest.

Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.

Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified.

The VGF-derived peptide TLQP-21 contributes to inflammatory and nerve injury-induced hypersensitivity.

VGF (nonacronymic) is a granin-like protein that is packaged and proteolytically processed within the regulated secretory pathway. VGF and peptides derived from its processing have been implicated in neuroplasticity associated with learning, memory, depression, and chronic pain. In sensory neurons, VGF is rapidly increased following peripheral nerve injury and inflammation.

US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency.

Background: The treatment of hereditary angioedema (HAE) has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of these patients requires a comprehensive management plan. Although several consensus papers have been published concerning the diagnosis and treatment of HAE, guidelines for a comprehensive management plan have not been developed.

Hereditary angioedema with normal C1-INH (HAE type III).

Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH), also known as HAE type III, is a familial condition only clinically recognized within the past three decades. Similar to HAE from C1-INH deficiency (HAE types I and II), affected individuals experience unpredictable angioedema episodes of the skin, gastrointestinal tract, and airway. Unique clinical features of HAE with normal C1-INH include the predominance of affected women, frequent exacerbation by estrogen, and a prominence of angioedema that involves the face and oropharynx.

Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial.

Background: Hereditary angioedema (HAE), caused by C1 inhibitor (C1INH) deficiency or dysfunction, is characterized by recurrent attacks of tissue swelling affecting multiple anatomic locations. Recombinant human C1INH (rhC1INH) has been shown effective for acute treatment of HAE attacks.

Objective: To evaluate the efficacy and safety of rhC1INH (50 IU/kg to maximum 4,200 IU/treatment) vs placebo in a larger HAE population.

Biomechanical comparison of prophylactic medial malleolar fixation in total ankle arthroplasty.

The purpose of this study was to compare the biomechanical properties of two common forms of prophylactic fixation of the medial malleolus during total ankle arthroplasty (TAA). Ten matched pairs of cadaveric lower extremities were fixed with a single cannulated cortical screw or a one-third tubular plate after preparation for TAA. Ten unmatched single cadaver lower extremities were used as the control.

Nanofiltered C1 esterase inhibitor for treatment of laryngeal attacks in patients with hereditary angioedema.

Background: Laryngeal edema is a life-threatening manifestation of hereditary angioedema (HAE), an autosomal-dominant disorder caused by quantitative or functional C1 esterase inhibitor (C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis against angioedema attacks in the United States and for treatment, preprocedure prevention, and routine prevention of HAE in Europe. The objective of this analysis was to evaluate the effectiveness and tolerability of C1 INH-nf when used for the treatment of laryngeal attacks.

Creating a comprehensive treatment plan for hereditary angioedema.

Management of hereditary angioedema (HAE) has changed dramatically in recent years because of the availability of several HAE-specific medications. This paradigm shift in therapy provides the opportunity to dramatically improve the care of HAE patients, but has increased the complexity of treatment. Patient education and communication with health care providers is crucial.

Synchronisation and coupling analysis: applied cardiovascular physics in sleep medicine.

Sleep is a physiological process with an internal program of a number of well defined sleep stages and intermediate wakefulness periods. The sleep stages modulate the autonomous nervous system and thereby the sleep stages are accompanied by different regulation regimes for the cardiovascular and respiratory system. The differences in regulation can be distinguished by new techniques of cardiovascular physics.

Enhanced activity of Akt in Teff cells from children with lupus nephritis is associated with reduced induction of tumor necrosis factor receptor-associated factor 6 and increased OX40 expression.

Objective: The breakdown of peripheral tolerance mechanisms is central to the pathogenesis of systemic lupus erythematosus (SLE). Although true Treg cells in patients with SLE exhibit intact suppressive activity, Teff cells are resistant to suppression. The underlying mechanisms are incompletely understood.