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Berber Kapitein K 1Intensive Care Unit, Erasmus MC-Sophia Children's Hospital 2Cardiothoracic Surgery, Erasmus MC 3Anesthesiology, Erasmus - - 2014
Cardiopulmonary bypass (CPB) induces a systemic inflammatory response syndrome (SIRS) by factors such as contact of the blood with the foreign surface of the extracorporeal circuit, hypothermia, reduction of pulmonary blood flow during CPB and endotoxaemia. SIRS is maintained in the post-operative phase, co-occurring with a counter anti-inflammatory response syndrome ...
Bennett Jeremy M JM Department of Cardiothoracic Anesthesiology, Vanderbilt University Medical Center, Nashville, TN 37232, USA. Electronic address: - - 2014
To propose a set of recommendations for the perioperative management of patients with Eisenmenger syndrome and similar physiology, based on 20 years of experience at a single institution. Retrospective study of institutional outcomes of Eisenmenger syndrome patients and patients with balanced or fixed right-to-left intracardiac shunts with pulmonary hypertension undergoing ...
Ganigara Anuradha A Department of Anesthesiology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, - - 2014
Frontometaphyseal dysplasia (FMD), also called Gorlin-Cohen syndrome, is a rare hereditary X-linked dominant craniotubular bone disorder. The presentation describes the airway management of a 2-year-old child suffering from FMD with significant retrognathia, posted for major long bone corrective osteotomy. Induction with a combination of dexmedetomidine and ketamine preceded a successful ...
Kim Yongsuk Y Department of Anesthesiology and Pain Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, - - 2014
Patients with Pierre Robin syndrome are characterized by micrognathia, retrognathia, glossoptosis, and respiratory obstruction and are prone to have a difficult-to-intubate airway. The McGrath® MAC video laryngoscope provides a better view of the glottis than a Macintosh laryngoscope, but it is not easy to insert an endotracheal tube through the ...
Yeoh Tze Yeng TY Department of Anesthesiology, Mayo Clinic, Rochester, MN; Department of Anaesthesia, National University Hospital, National University Health System, Republic of - - 2014
LEOPARD syndrome is a rare congenital disease that can manifest with cardiac anomalies, multiple lentigines, ocular hypertelorism, growth retardation, and deafness. The purpose of this case series was to review the most prominent comorbidities associated with LEOPARD syndrome, and describe perioperative outcomes in a series of patients undergoing anesthesia. Retrospective ...
Pineiro Mildred Lopez ML 1Geisel School of Medicine at Dartmouth, Lebanon, NH, - - 2014
Williams syndrome is a relatively rare genetic disorder caused by the hemizygous microdeletion of a region in chromosome 7q11.23. Individuals with Williams syndrome typically present with a highly social, overfriendly, and empathic personality. Comorbid medical and neuropsychiatric disorders are common. Reports of effective pharmacological treatment of associated neuropsychiatric disorders are ...
Gross Anne F - - 2013
Although anesthetics have been used for more than a century, their mechanisms of action remain poorly understood. Given that a number of intraoperative and postoperative neuropsychiatric syndromes have been linked to the use of anesthetics, practitioners should familiarize themselves with these conditions. Basic concepts about anesthesia are reviewed and neuropsychiatric ...
Mtaweh Haifa - - 2013
Propofol infusion syndrome is a recognized complication of prolonged propofol use in the pediatric population, but little is reported on other metabolic effects of propofol, especially in children with mitochondrial disorders. We report on a child with metabolic encephalopathy, lactic acidosis, and stroke-like syndrome who received a single dose of ...
Meggiolaro Marco - - 2013
Brugada syndrome is an electrical cardiac disease predisposing to ventricular arrhythmias in which typical electrocardiographic (ECG) features consist of nonischemic repolarization abnormalities in the right precordial leads V1-V3. The appearance of a Brugada-ECG pattern is increasingly observed in critically ill patients and is traditionally attributed to the effect of body ...
Bhaskar Pradeep - - 2013
The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil ...
Kim Young-Lok - - 2013
The I-gel™ is a single-use supraglottic airway device introduced in 2007 which features a non-inflatable cuff and allows passage of a tracheal tube owing to its large diameter and short length of the airway tube. In this case, the authors experienced a difficult airway management on a 4-year-old boy with ...
Volquind Daniel D Title in Anesthesiology (TSA); Professor of the Universidade de Caxias do Sul; Anesthesiologist of the CAN-Clínica de Anestesiologia de Caxias do Sul, RS, Brazil. Electronic address: - - 2013
Guillain-Barre syndrome during pregnancy is considered a rare neurological complication, and there is no consensus in literature for anesthetic management for cesarean section in such patients. The objective of this paper is to report the case of a pregnant woman with Guillain-Barre syndrome undergoing cesarean section. Female patient, 22-year old, ...
Pothineni Naga Venkata Krishna Chand - - 2013
Propofol is one of the most commonly used sedating agents in critical care units worldwide. It is generally well tolerated and preferred for its pharmacokinetic profile. Here, we describe a rare and devastating adverse effect of propofol, the propofol-related infusion syndrome.
Yamaguchi Satoshi - - 2013
Hajdu-Cheney syndrome is an extremely rare disorder characterized by progressive skeletal acro-osteolysis, which results in extremity fractures and scoliosis often requiring surgical treatment from childhood. A unique facial structure and deformity of the cervical spine is associated with a difficult airway. We report here a 10-year-old girl with Hajdu-Cheney syndrome ...
Sayin Refah - - 2013
Guillain-Barre Syndrome (GBS) is the most common disease resulting in acute diffuse flaccid paralysis. It is an autoimmune disease that can occur at any age. The clinical course is characterized by weakness in the arms and legs, areflexia and the progression of muscle weakness from the lower limbs to the ...
Hwang Woo Sub WS Departments of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, - - 2013
Propofol is used for treating refractory status epilepticus, which has high rate of mortality. Propofol infusion syndrome is a rare but often fatal syndrome, characterized by lactic acidosis, lipidemia, and cardiac failure, associated with propofol infusion over prolonged periods of time. We investigated the clinical factors that characterize propofol infusion ...
Deters Darlene - - 2013
This article presents a case study of a young man with a history of epilepsy and the use of a propofol infusion to control his symptoms. Propofol-related infusion syndrome is presented.
Maranhão Marcius Vinícius M - - 2013
Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic ...
Hechadi Jawad - - 2013
A 43-year-old man with Marfan syndrome presented with recurrent aortic root dilatation and aortic regurgitation at ten years after a remodeling (Yacoub) procedure. Herein is described a reoperative valve-sparing procedure employing the reimplantation technique (David procedure) used in the treatment of this patient.
Maranhão Marcius Vinícius M MV TSA, Professor of Pharmacology, Institute of Biological Sciences, Universidade de Pernambuco; Co-responsible for CET, Hospital da Restauração, Hospital Getúlio Vargas and Hospital Universitário Oswaldo Cruz; Anesthesiologist, Hospital Universitário Oswaldo Cruz; Member of Education and Training/Sociedade Brasileira de Anestesiologia (SBA), Recife, PE, Brazil. Electronic address: - - 2013
Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic ...
Chaudhuri Souvik - - 2013
Morquio-Brailsford syndrome is a type of mucopolysaccharidoses. It is a rare disease with features of short stature, atlantoaxial instability with risk of cord damage, odontoid hypoplasia, pectus carinatum, spine deformities, hepatomegaly, and restrictive lung disease. Neck movements during intubation are associated with the risk of quadriparesis due to cervical instability. ...
Ferrando Carlos - - 2013
CONTEXT: Acute respiratory distress syndrome is characterised by activation of the inflammatory cascade. The only treatment that reduces the mortality rate associated with this syndrome is lung protective ventilation, which requires sedation of patients. Sedation in critical care units is usually induced intravenously, although there is reason to believe that ...
Ji Fuhai - - 2013
Hypernatremia and polyuria are the main symptoms of diabetes insipidus. Polyuria is characterized by a 24-h urine volume in excess of 40-50 ml/kg in adults. Dexmedetomidine, a highly selective, short-acting intravenous alpha-2 agonist, is used as a component of anesthesia, and has been suspected to induce polyuric syndrome. We report a ...
Kim Young Mi - - 2013
We report on an anesthetic experience with a 74-year-old female with Isaacs' syndrome, who underwent elective surgery for open rotator cuff repair. Isaacs' syndrome is a rare peripheral motor neuron disorder with clinical manifestations such as involuntary muscle twitching, cramps, mild weakness and increased sweating. To avoid prolonged neuromuscular blockade, ...
Lim Kyung-Jee - - 2013
Tapia's syndrome is the palsy of the 10th and 12th cranial nerves, resulting in ipsilateral paralysis of the vocal cord and tongue. It is a rare complication which is related to the anesthetic airway management and positioning of the patient's head during the surgery. We describe a patient with a ...
Choi Eun Kyeong - - 2013
The case of a 33-day-old boy with Pierre Robin syndrome using a Cook® airway exchange catheter in laryngeal mask airway-guided fiberoptic intubation is presented. After induction with sevoflurane, classical reusable laryngeal mask airway (LMA) #1 was inserted and ultrathin fiberoptic bronchoscope (FOB) was passed through. A Cook® airway exchange catheter ...
Davis Jennifer J - - 2013
A patient who manifested signs of serotonin syndrome during an intravenous anesthetic with remifentanil and propofol is presented. The patient displayed lower extremity clonus, nystagmus, and diaphoresis. At the time of surgery, the patient was being treated with fluoxetine (a selective serotonin reuptake inhibitor). A presumptive diagnosis of serotonin syndrome ...
Karaman Yucel - - 2013
SUMMARY: Following a propofol anesthetic, a 5-year-old girl with lower extremity spasticity seized and developed hypertriglyceridemia, hyperkalemia, and metabolic acidosis. A presumed diagnosis of propofol infusion syndrome (PRIS) was made, but further investigation revealed neonatal adrenoleukodystrophy. PRIS should be considered with this constellation of symptoms, but other neurometabolic disorders must ...
Martorano Pietro Paolo PP Head of Neuroanesthesia Unit, Ospedali Riuniti, Via Conca 71 - 60126 Ancona, - - 2013
Brugada syndrome (BrS) is one of the most common causes of sudden death in young people. It usually presents with life-threatening arrhythmias in subjects without remarkable medical history. The need for surgical treatment may unmask BrS in otherwise asymptomatic patients. The best anaesthesiological treatment in such cases is matter of ...
Bradley Beverly D - - 2012
OBJECTIVE:: Our aim is to better characterize the impact of sedation and its interruption on continuously monitored heart rate variability and respiratory rate variability in critically ill patients. We aim to explore whether sedation reduces heart rate variability and respiratory rate variability in critically ill patients and whether the extent ...
Agrawal Nikhil N Trauma Fellow, State Trauma Unit, Royal Perth Hospital, Wellington Street, Perth, Western - - 2013
Propofol, an intravenously administered, centrally acting sedative/hypnotic, is a popular medication for anesthesia and sedation due to rapid onset, controllability and short recovery time. Prolonged propofol infusions, (>48 h) with elevated doses (>67 mcg/kg/min) may result in a rare but fatal condition known as the Propofol Related Infusion Syndrome (PRIS). This is ...
Yin Lina - - 2012
CYP11B1 inhibition is a promising therapy for Cushing's syndrome. Starting from etomidate, references I and II, the title compounds were designed and synthesized. Cyclopropyl analogue 4 was identified as a CYP11B1 inhibitor more potent (IC(50) = 2.2 nM) than leads and more selective (SF = 11) than I and metyrapone. ...
Annen Eva E Department of Anesthesia and Intensive Care Medicine, University Hospital Basel, - - 2012
We present the case of a 7-year old boy with traumatic brain injury who received propofol during 38 h. Thirty-six hours after cessation of propofol infusion asystole occurred. After immediate mechanical and medical resuscitation, unreactive dilated pupils were observed. The following computed tomography scan revealed a generalized brain edema with ...
Gurumurthy T - - 2012
Eisenmenger's syndrome is a cyanotic congenital heart disease that includes pulmonary hypertension with reversed or bidirectional shunt associated with septal defects or patent ductus arteriosus. The decreased systemic vascular resistance associated with pregnancy increases the degree of right to left shunting, thereby carrying substantial risk to both the mother and ...
Valente T - - 2011
Nontraumatic acute thoracic aortic syndromes (AAS) describe a spectrum of life-threatening aortic pathologies with significant implications on diagnosis, therapy and management. In this context, multidetector computed tomography (MDCT) is the gold standard due to its intrinsic diagnostic value; its performance approaches 100% sensitivity and specificity, and it is accepted as ...
Hopps E - - 2012
Metabolic syndrome is commonly accompanied by an elevated cardiovascular risk with high morbidity and mortality. The alterations of the arterial vasculature begin with endothelial dysfunction and lead to micro- and macrovascular complications. The remodeling of the endothelial basal membrane, that promotes erosion and thrombosis, has a multifactorial pathogenesis that includes ...
Wheeler Donna - - 2011
Postpolio syndrome is a disorder related to the recurrence of neuromuscular symptoms in survivors of paralytic poliomyelitis. A comprehensive understanding of the pathophysiology is necessary for the anesthesia provider to develop a safe anesthetic plan. This case report discusses the anesthetic challenges and considerations in patients with postpolio syndrome, focusing ...
Komen Niels - - 2011
Abstract Purpose: To present a case of penetrating aortic ulcer with extraordinary etiology. Case Report: A 57-year-old man was admitted with acute retrosternal and interscapular pain. He was a demolition worker and often used a pneumatic drill to which he pressed his chest as he drilled. Clinical examination showed previously ...
Morgan Gareth - - 2011
Long segment narrowing of the thoracoabdominal aorta or so-called middle aortic syndrome, is an uncommon lesion presenting variably with physical signs of coarctation of the aorta, hypertension, renal insufficiency and or mesenteric ischemia. The etiologies are multiple, but Takayasu's arteritis is a leading cause. Variable involvement of diverse systemic arterial ...
Shah Tanmay H TH Department of Anesthesiology, Magee Womens Hospital, University of Pittsburgh Medical Center, Pittsburgh, PA, - - 2011
Patients with Klippel-Feil Syndrome (KFS) have congenital fusion of their cervical vertebrae due to a failure in the normal segmentation of the cervical vertebrae during the early weeks of gestation and also have myriad of other associated anomalies. Because of limited neck mobility, airway management in these patients can be ...
Anagnostakou Vania - - 2011
Acute aortic syndrome encompasses classic aortic dissection, intramural haematoma, penetrating aortic ulcer, and iatrogenic or traumatic transection of the aorta. These acute aortic pathologies present with characteristic 'aortic pain', and are managed by several operative and endovascular techniques.
Baptista Rui - - 2011
Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and hypoxia induced by the upright position and relieved by the supine position. Several factors related to atrial anatomy can facilitate shunting through an atrial septal defect; in many cases, the syndrome is associated with patent foramen ovale and right-to-left shunt, and ...
McKinley Sharon - - 2011
Objectives: To report time from the onset of symptoms to hospital presentation in Australian and New Zealand patients with subsequently confirmed acute coronary syndrome, and to identify factors associated with prehospital delay time in these patients. Methods: Patients with coronary artery disease enrolled in a randomized clinical trial testing an ...
Aziz Fahad - - 2011
Acute aortic dissection is a rare clinical entity that mainly affects patients older than 50 years. It is unusual in younger patients and its presence has been traditionally associated with trauma, Marfan syndrome, bicuspid aortic valve and pregnancy. We present here, a case of a 30 year old pregnant female ...
Kolovou Genovefa D - - 2011
At the present time, there is no widely agreed definition of postprandial lipaemia (PPL). This lack of a shared definition limits the identification and treatment of patients with exaggerated PPL as well as the evaluation of potential therapeutic agents. PPL is a complex syndrome characterized by non-fasting hypertriglyceridaemia that is ...
Bankoski Andrea - - 2011
This study examined the association between objectively measured sedentary activity and metabolic syndrome among older adults. Data were from 1,367 men and women, aged ≥ 60 years who participated in the 2003-2006 National Health and Nutrition Examination Survey (NHANES). Sedentary time during waking hours was measured by an accelerometer (<100 ...
Kates Wendy R - - 2011
Velocardiofacial syndrome (VCFS; 22q11.2 deletion syndrome) represents one of the highest known risk factors for schizophrenia. Insofar as up to 30% of individuals with this genetic disorder develop schizophrenia, VCFS constitutes a unique, etiologically homogeneous model for understanding the pathogenesis of schizophrenia. Using a longitudinal, case-control design, anatomic magnetic resonance ...
Guo Gao - - 2011
Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and dissection are the major determinants of premature death in untreated patients. In previous work, we showed that extracts of aortic tissues from the mgR mouse model of Marfan syndrome ...
Barbosa-Leiker Celestina - - 2011
Without verification of longitudinal measurement invariance, researchers cannot be certain whether observed change in the metabolic syndrome reflects true change or changes in assessment or structure of the construct over time. This research tested longitudinal measurement invariance of a 1-factor model of the metabolic syndrome during the course of 6 ...
Artunc Ferruh - - 2010
HISTORY AND ADMISSION FINDINGS: A 79-year-old female patient was referred with acute renal failure requiring haemodialysis and haemoptysis. INVESTIGATIONS: Kidney biopsy showed extracapillary proliferative glomerulonephritis with crescents in 7 from 15 glomeruli and sclerosis in the remaining. A computed tomography scan of the chest showed evidence of alveolar haemorrhage. Serologic ...
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