The case describes a 25-year-old Caucasian female diagnosed with Alexander's disease (AxD) as an outpatient after extensive inpatient workup. Her presenting complaints included incontinence, clumsiness, seizures, dysphagia, and dysarthria. She was also found to have pancytopenia and dysautonomia. A full neurologic and hematologic workup yielded very little results, until a thorough literature search of her presenting complaints and radiologic findings pointed to adult-onset Alexander's Disease. Alexander's disease is a rare genetic leukodystrophy with a broad variety of presentations. Despite its infrequency in adults and the difficulty in diagnosis, the prevalence of AxD has been increasing due to ease of genetic analysis and identification of key clinical and radiological findings. This case illustrates the necessity of vigilance and persistence in the face of unusual patient presentations; occasionally, the sound of hoofbeats is zebras.
http://bit.ly/2AE9z0N
Cancer Medicine by Alexandros G.Sfakianakis,Anapafseos 5 Agios Nikolaos,Crete 72100,Greece,tel :00302841026182 & 00306932607174
Τετάρτη 9 Ιανουαρίου 2019
A Novel Mutation in the Adult-Onset Alexander’s Disease GFAP Gene
Immediate Use of Continuous Positive Airway Pressure in Patients With Obstructive Sleep Apnea Following Transsphenoidal Pituitary Surgery: A Case Series
Background: Patients who undergo transsphenoidal pituitary resection have an elevated risk of obstructive sleep apnea (OSA) yet their outcomes and the safety of continuous positive airway pressure (CPAP) remains unclear. Our study objective was to determine the incidence of complications related to the use of early positive airway pressure following pituitary resection. Methods: We retrospectively identified all patients who underwent endoscopic transsphenoidal pituitary tumor resection between January 1, 2005 and March 24, 2016 at our institution, including those with diagnosed or suspected OSA. We compared characteristics and postoperative complications of OSA patients who did and did not receive CPAP postoperatively. Results: In total, 427 patients underwent transsphenoidal pituitary resection. Of these, 64 (15%) had OSA. Acromegaly was more common in patients with OSA (42% vs. 10%; P
http://bit.ly/2RkwF74
http://bit.ly/2RkwF74
Lack of consensus on the peri-operative management of patients with diabetes mellitus
No abstract availablehttp://bit.ly/2FlnWtW
Noninvasive continuous arterial pressure monitoring with Clearsight during awake carotid endarterectomy: A prospective observational study
BACKGROUND Continuous noninvasive blood pressure (CNBP) measurement using the volume-clamp method is a less invasive alternative compared with invasive intra-arterial monitoring for awake patients during carotid endarterectomy (CEA) under regional anaesthesia. OBJECTIVE We investigated the agreement of blood pressure (BP) recorded with invasive and CNBP methods during awake CEA. DESIGN A prospective observational study for assessing agreement with Bland–Altman plots, agreement-tolerability indices (ATI), concordance and interchangeability. SETTING Azienda Ospedaliera Universitaria G. Martino, Messina, a University tertiary referral centre in Italy. PATIENTS In 30 consecutive patients, we recorded continuously ipsilateral invasive and noninvasive BPs, from 3 min before carotid cross-clamping to 5 min after unclamping. MAIN OUTCOME MEASURES Primary outcome was bias, 95% limits of agreement, ATI, concordance and interchangeability for mean arterial pressure (MAP). Secondary outcomes were agreements for systolic arterial pressure and diastolic arterial pressure. Tracking of changes was assessed with four-quadrant polar plots and the trend interchangeability method. Optimal bias was defined as 5 mmHg or less. RESULTS A total of 2672 invasive and CNBP paired measurements (93% of overall data) were analysed, with a median of 92 readings per patient [IQR 76 to 100]. Mean (SD) bias for MAP, systolic arterial pressure and DAP were −6.8 (6.7), −3.0 (9.7) and −9.0 (5.4) mmHg, respectively. The ATIs were 0.88, 0.95 and 0.71, respectively, where ATI of 1.0 or less and at least 2.0 defined acceptable, marginal and unacceptable agreements. The four-quadrant plot analysis for beat-to-beat differences showed concordance rates of 97.3%, 99.98% and 96.4%, respectively. Polar plot analysis showed 95% limits of agreement of −3 to 3, −2 to 2 and −2 to 2 mmHg respectively. Trend interchangeability method showed an interchangeability rate of 95% for MAP. CONCLUSION During CEA performed under regional anaesthesia, CNBP offers a less invasive approach for BP monitoring. We found acceptable agreement for MAP defined by an ATI of 0.88 and an excellent 95% global interchangeability rate. A suboptimal bias of 7 mmHg was found with CNBP for MAP.http://bit.ly/2Fib7Bs
Development of a prediction model for postoperative pneumonia: A multicentre prospective observational study
BACKGROUND Postoperative pneumonia is associated with increased morbidity, mortality and costs. Prediction models of pneumonia that are currently available are based on retrospectively collected data and administrative coding systems. OBJECTIVE To identify independent variables associated with the occurrence of postoperative pneumonia. DESIGN A prospective observational study of a multicentre cohort (Prospective Evaluation of a RIsk Score for postoperative pulmonary COmPlications in Europe database). SETTING Sixty-three hospitals in Europe. PATIENTS Patients undergoing surgery under general and/or regional anaesthesia during a 7-day recruitment period. MAIN OUTCOME MEASURE The primary outcome was postoperative pneumonia. Definition: the need for treatment with antibiotics for a respiratory infection and at least one of the following criteria: new or changed sputum; new or changed lung opacities on a clinically indicated chest radiograph; temperature more than 38.3 °C; leucocyte count more than 12 000 μl−1. RESULTS Postoperative pneumonia occurred in 120 out of 5094 patients (2.4%). Eighty-two of the 120 (68.3%) patients with pneumonia required ICU admission, compared with 399 of the 4974 (8.0%) without pneumonia (P http://bit.ly/2FmgCOU
Six-minute walk test in pre-operative evaluation of patients for upper abdominal surgery
No abstract availablehttp://bit.ly/2FhZ9rt
Intra-operative tachycardia is not associated with a composite of myocardial injury and mortality after noncardiac surgery: A retrospective cohort analysis
BACKGROUND Myocardial injury after noncardiac surgery (MINS) is a major contributor to peri-operative morbidity and mortality with a reported incidence of about 8%. Tachycardia increases myocardial oxygen demand, and decreases oxygen supply, and is therefore a potential cause of MINS. OBJECTIVE We tested the hypothesis that there is an association between intra-operative area above a heart rate (HR) of 90 bpm and a composite of MINS and in-hospital all-cause mortality. DESIGN Retrospective analyses. SETTING Major tertiary care hospital, Cleveland, USA. PATIENTS Adults having elective or nonelective noncardiac surgery and scheduled troponin monitoring during the first 3 postoperative days between 2010 and 2015. MAIN OUTCOME MEASURES All-or-none composite of myocardial injury (MINS), defined by a peak postoperative generation 4 troponin T concentration at least 0.03 ng ml−1, and in-hospital all-cause mortality. RESULTS Among 2652 eligible patients, 123 (4.6%) experienced MINS within 7 days after surgery and 6 (0.2%) died before discharge. Intra-operative area above HR more than 90 bpm was not associated with the all-or-none composite of MINS and in-hospital mortality, with an estimated odds ratio (95% confidence interval) of 0.99 (0.97 to 1.01) per 1 h bpm increase in area above HR more than 90 bpm. Secondary outcomes were also unrelated to the composite, with estimated odds ratios (98.3% confidence interval) of 0.99 (0.98 to 1.00) for area above HR more than 80, 0.98 (0.92 to 1.04) for area above HR more than 100 bpm, and 0.96 (0.88 to 1.05) for maximum HR. CONCLUSION There was no apparent association between various measures of tachycardia and a composite of MINS and death, a result that contradicts previously reported associations between other measures of intra-operative tachycardia and MINS/mortality.http://bit.ly/2FjGr21
Postoperative pneumonia: Can this important complication be predicted and anticipated?
No abstract availablehttp://bit.ly/2FiaRCu
Relevance of peripheral cholinesterase activity on postoperative delirium in adult surgical patients (CESARO): A prospective observational cohort study
BACKGROUND The cholinergic system is considered to play a key role in the development of postoperative delirium (POD), which is a common complication after surgery. OBJECTIVES To determine whether peri-operative acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) activities are associated with the development of POD in in-hospital surgical patients, and raise hypotheses on cholinergic regulatory mechanisms in POD. DESIGN A prospective multicentre observational study by the Peripheral Cholinesterase-activity on Neurocognitive Dysfunctions in Surgical Patients (CESARO) study group. SETTING Nine German hospitals. PATIENTS Patients of at least 18 years of age scheduled for inpatient elective surgery for a variety of surgical procedures. A total of 650 patients (mean age 61.5 years, 52.8% male) were included. METHODS Clinical variables, and peripheral AChE and BuChE activities, were assessed throughout the peri-operative period using bedside point-of-care measurements (one pre-operative and two postoperative measurements). POD screening was conducted postoperatively for at least 24 h and up to the third postoperative day using a validated screening tool (nursing delirium screening scale). RESULTS In all, 179 patients (27.5%) developed POD within the early postoperative phase. There was a lower BuChE activity in patients with delirium compared with patients without delirium pre-operatively (Cohen's r = 0.07, P = 0.091), on postoperative day 1 (Cohen's r = 0.12, P = 0.003) and on postoperative day 2 (Cohen's r = 0.12, P = 0.002). In contrast, there was a significantly higher AChE activity in patients with delirium compared with patients without delirium pre-operatively (Cohen's r = 0.10, P = 0.012), on postoperative day 1 (Cohen's r = 0.11, P = 0.004) and on postoperative day 2 (Cohen's r = 0.13, P = 0.002). After adjusting for covariates in multiple logistic regression, a significant association between both BuChE and AChE activities and POD was not found. However, in the multivariable analysis using the Generalized Estimating Equation, cholinesterase activities showed that a decrease of BuChE activity by 100 U L−1 increased the risk of a delirium by approximately 2.1% (95% CI 1.6 to 2.8%) and for each 1 U g−1 of haemoglobin increase in AChE activity, there was a 1.4% (95% CI 0.6 to 2.2%) increased risk of POD. CONCLUSION Peri-operative peripheral cholinesterase activities may be related to the development of POD, but the clinical implications remain unclear. Further studies, in homogeneous patient groups with a strict protocol for measurement time points, are needed to investigate the relationship between cholinesterase activities and POD. TRIAL REGISTRATION www.clinicaltrials.gov. Identifier NCT01964274.http://bit.ly/2FmpKmy
Goal-directed therapy during transthoracic oesophageal resection does not improve outcome: Randomised controlled trial
BACKGROUND Goal-directed therapy (GDT) is expected to be of highest benefit in high-risk surgery. Therefore, GDT is recommended during oesophageal resection, which carries a high risk of postoperative complications. OBJECTIVES The aim of this study was to confirm the hypothesis that GDT during oesophageal resection improves outcome compared with standard care. DESIGN A randomised controlled study. SETTING Two Swedish university hospitals, between October 2011 and October 2015. PATIENTS Sixty-four patients scheduled for elective transthoracic oesophageal resection were randomised. Exclusion criteria included colonic interposition and significant aortic or mitral valve insufficiency. INTERVENTION A three-step GDT protocol included stroke volume optimisation using colloid boluses as assessed by pulse-contour analysis, dobutamine infusion if cardiac index was below 2.5 l min−1 m−2 and norepinephrine infusion if mean arterial blood pressure was below 65 mmHg. MAIN OUTCOME MEASURE The incidence of complications per patient at 5 and 30 days postoperatively as assessed using a predefined list. RESULTS Fifty-nine patients were available for analysis. Patients in the intervention group received more colloid fluid (2190 ± 875 vs. 1596 ± 759 ml, P http://bit.ly/2Fhax73
Orthostatic intolerance following hip arthroplasty: incidence, risk factors and effect on length of stay: A prospective cohort study
BACKGROUND Early postoperative mobilisation is important for enhanced recovery, but can be hindered by orthostatic intolerance, characterised by dizziness, nausea, vomiting, feeling of heat, blurred vision and ultimately syncope. Although the incidence of orthostatic intolerance following total hip arthroplasty has been identified, few studies have yet investigated potential risk factors for developing orthostatic intolerance after hip arthroplasty. OBJECTIVES The aim of this study was to assess the incidence of orthostatic intolerance on the first postoperative day after total hip arthroplasty, potential predisposing risk factors for developing orthostatic intolerance and its effect on length of stay. DESIGN A prospective observational study. SETTING Tertiary hospital, Auckland, New Zealand, May to September 2015. PATIENTS One hundred and seventeen consecutive patients undergoing unilateral total hip arthroplasty. Patients were excluded if they had revision surgery. MAIN OUTCOME MEASURES Incidence of orthostatic intolerance during mobilisation on the first postoperative day. Significant peri-operative risk factors for developing orthostatic intolerance were identified using logistic regression. Length of stay was compared between orthostatic intolerant and orthostatic tolerant patients using the Mann–Whitney U-test. RESULTS On the first postoperative day, 22% of patients failed mobilisation due to orthostatic intolerance. Factors independently associated with orthostatic intolerance were female sex; OR (95% CI), 3.11 (1.01 to 9.57), postoperative use of gabapentin; OR 3.55 (1.24 to 10.15) and high peak pain levels (≥5/10) during mobilisation; OR 4.05 (1.30 to 12.61). Overall, 78% of patients were correctly identified. The model was more accurate at predicting those who would not get orthostatic intolerance (89% correct), compared with those who did have orthostatic intolerance (39% correct). Length of stay was longer in patients with orthostatic intolerance (P = 0.019). CONCLUSION Orthostatic intolerance is common after total hip arthroplasty. Optimising pain control prior to mobilisation and limiting gabapentin use may modify the risk of developing postoperative orthostatic intolerance. Although personalised recovery pathways appear attractive, at present, the ability to predict at-risk individuals is still limited.http://bit.ly/2FmpJPw
Peri-operative endothelial dysfunction in patients undergoing minor abdominal surgery: An observational study
BACKGROUND Myocardial injury after noncardiac surgery is common and associated with major adverse cardiac events. Surgery induces acute endothelial dysfunction, which might be central in the pathophysiology of myocardial injury; however, the relationship between surgical stress and endothelial function remains incompletely understood. OBJECTIVES This study aimed to assess the acute peri-operative changes in endothelial function after minor elective abdominal surgery. DESIGN A prospective, observational, single-centre study. SETTING A university hospital from February 2016 to January 2017. PATIENTS Sixty patients undergoing elective minor abdominal surgery. MAIN OUTCOME MEASURES The change in endothelial function, expressed as the reactive hyperaemia index (RHI), was assessed by non-invasive digital pulse tonometry. RHI, biomarkers of nitric oxide bioavailability and oxidative stress were assessed prior to and 4 h after surgery. RESULTS RHI decreased significantly from 1.93 [95% confidence interval (95% CI 1.78 to 2.09)] before surgery to 1.76 (95% CI 1.64 to 1.90), P = 0.03, after surgery. The nitric oxide production, L-arginine/asymmetric dimethylarginine, decreased significantly from a ratio of 213.39 (95% CI 188.76 to 241.2) to a ratio of 193.3 (95% CI 171.82 to 217.54), P = 0.03. Plasma biopterins increased significantly after surgery, while the ratio between tetrahydrobiopterin and dihydrobiopterin was unchanged. Total ascorbic acid decreased significantly after surgery (P http://bit.ly/2FllVOq
Anaesthesiology residents have no significant effect on anaesthesia or procedure times
No abstract availablehttp://bit.ly/2Fhayb7
Monitoring haemodynamic response to fluid-challenge in ICU: comparison of pressure recording analytical method and oesophageal Doppler: A prospective observational study
BACKGROUND The ability of the pressure recording analytical method (PRAM) in tracking change in cardiac output (ΔCO) after a fluid challenge in ICU needs to be evaluated with the most contemporary comparison methods recommended by experts. OBJECTIVE Our objective was to report the trending ability of PRAM in tracking ΔCO after a fluid challenge in ICU and to compare this with oesophageal Doppler monitoring (ODM). DESIGN Prospective, observational study. SETTING Hôpital Lariboisière and Hôpital Européen George Pompidou, Paris, France, from April 2016 to December 2017. PATIENTS Critically ill patients admitted to ICU with monitoring of CO monitored by ODM and invasive arterial pressure. INTERVENTION ΔCO after fluid challenge was simultaneously registered with ODM and PRAM connected to the arterial line. MAIN OUTCOME MEASURE Polar statistics (mean angular bias, radial limits of agreement and polar concordance rate) and clinical concordance evaluation (error grid and clinical concordance rate). Predictors of bias were determined. RESULTS Sixty-eight fluid challenge were administered in 49 patients. At the time of fluid challenge, almost all were mechanically ventilated (99%), with 85% receiving norepinephrine. Admission diagnosis was septic shock in 70% of patients. Patients had a Sequential Organ Failure Assessment score of 10 [7 to 12] and a median Simplified Acute Physiology Score II of 61 [49 to 69]. Relative ΔCO bias was 7.8° (6.3°) with radial limits of agreement of ±41.7°, polar concordance rate 80% and clinical concordance rate 74%. ΔCO bias was associated with baseline bias (P = 0.007). Baseline bias was associated with radial location of the arterial line (P = 0.03). CONCLUSION When compared with ODM, PRAM has insufficient performance to track ΔCO induced by fluid challenge in ICU patients. Baseline bias is an independent predictor of trending bias. TRIAL REGISTRATION IRB 00010254-2016-033.http://bit.ly/2Flbz0Z
Early-Onset Neonatal Meningitis Caused by an Unusual Pathogen‐Moraxella catarrhalis
Introduction. Moraxella catarrhalis are part of the normal flora of the human respiratory tract and are known to have low pathogenic potential. The organism is rarely reported in the literature as the causative agent of meningitis. We report the first case of early-onset neonatal meningitis associated with M. catarrhalis from Nepal. Case Report. A 3-day-old baby with fever and yellowish discolouration of the body since 48 hrs was admitted to the neonatal ward. The baby developed 3 episodes of seizures in the form of uprolling of eyes on the first day of admission during phototherapy course for raised serum bilirubin. Sepsis screen was positive, and meningitis was confirmed as the cerebrospinal fluid culture grew M. catarrhalis. Cranial ultrasound scan was normal. The baby received a 21-day course of intravenous cefotaxime and amikacin. Recovery has been uneventful to date. Conclusion. Neonatal meningitis is a life-threatening infection. This case report presents an uncommon aetiology of neonatal meningitis which can be misidentified in the diagnostic bacteriology laboratory in resource constraints area like ours.
http://bit.ly/2CZJs6c
A nail in the appendix, accidental discovery on an asymptomatic patient
Abstract
Inadvertent ingestion of foreign bodies is a common condition within clinical practice. It rarely produces any symptoms. The diagnosis is difficult since most patients do not recall having swallowed any object. Needles, pins, keys, nails and bones are among the most commonly ingested foreign bodies. Severe complications are uncommon, but if present they can put patients' lives at risk. Although extremely rare, the ingested foreign body may end lodging in the appendix, posing a challenge for the clinical team. Once the exact location of the object is confirmed, the extraction of the foreign object must be performed to avoid complications. The present report describes a case of a young adult patient, who presented to the emergency room after a routine medical examination. A 30 mm metallic nail was discovered in the tip of the appendix. After a failed endoscopic approach an appendectomy was performed, and the patient underwent a complete recovery.http://bit.ly/2Qza9Sp
Spontaneous regression as a ‘burned-out’ non-seminomatous testicular germ cell tumor: a case report and literature review
Abstract
Testicular germ cell tumors (TGCTs) are the most frequent type of cancer in young adults. An exceptional event is the spontaneous regression (SR) of the primary tumor. Herein, we describe a burned-out non-seminomatous TGCT case and relevant literature review. A 34-year-old male presenting with low back pain was found to have a retroperitoneal mass upon urotomography. During workup, a heterogeneous testicular mass was evident, and its biopsy showed findings that support the diagnosis of spontaneous tumoral regression. The patient underwent unilateral orchiectomy and a chemotherapy protocol was later initiated, with 85% regression of the retroperitoneal metastatic mass. No progression of the primary tumor has been found. The etiology of SR across different cancer types appears to be associated with the host's immune response and an angiogenic disturbance of the tumor microenvironment. The burned-out phenomenon is a rare event that needs further research into its molecular sequencing.http://bit.ly/2VCinxb
Complete duplication of left and right external iliac vein: two case reports and review of the literature
Abstract
The femoral vein is named as external iliac vein as it progresses in the cranial direction. In the current case report, a rare variation of the left external iliac vein that was found incidentally during pelvic lymph node dissection in a 36-year-old woman is presented. We also found the same variation at the right side in a 55-year-old woman during laparoscopic pelvic lymph node dissection. Duplication of left external iliac vein throughout its entire length was detected during the operation of the patient with diagnosis of malignant ovarian serous tumor. The other patient with duplication of the right external iliac vein was diagnosed as stage IA, grade 3 endometrial cancer. These rare variations of external iliac veins are of great importance in gynecologic oncologic surgery and other operations requiring pelvic lymph node dissection. Here, we presented and discussed the importance of this variation with the photographs taken during the operation.http://bit.ly/2QzHCME
Madelung’s disease: long-term follow-up
Abstract
Madelung's disease (MD) is a rare lipid metabolic disorder of adipose tissue overgrowth, which has been reported to be related to alcohol abuse. Although it does not affect survival itself, alcoholism and metabolic disorders associated with MD can be life-threatening. Although surgical procedures and classifications have been reported, long-term follow-up has rarely been reported. Here, we report a 61-year-old Japanese man with MD who has been followed-up for 12 years. Lipectomy was performed three times over the first 3 years and a total of 4 kg of adipose tissue was excised. Relapse has not been observed. He was diagnosed with multiple hepatocellular carcinomas (MHC) and placed on dialysis due to hepatorenal syndrome caused by alcoholism. Moreover, one of the MHC ruptured resulting in hemorrhagic shock. The cause of death in MD is not fat proliferation but comorbidities. Long-term observation and multidisciplinary systemic management are necessary for MD patients.http://bit.ly/2VDhELX
Primary hydatid cyst localized in soft tissue during pregnancy
Abstract
Hydatid cyst in pregnancy is a rare condition. Moreover, it is very rare that it is in the soft tissue other than the liver. In this case, there are no data in the literature on treatment options, but only experience in case presentations. We present a patient who had severe pain during pregnancy due to isolated soft tissue hydatid cyst disease in this case report.http://bit.ly/2QzHvRe
Literature review and case series of haemorrhagic cholecystitis
Abstract
A diagnosis of haemorrhagic cholecystitis is difficult to make as it is rare and mimics other common disorders. We present three patients who presented with haemorrhagic cholecystitis, two of whom were on anti-coagulation at presentation. All 3 patients were treated conservatively, 2 with percutaneous cholecystostomy drainage and 1 patient with intravenous antibiotics. There are few guidelines on the management of such a condition.http://bit.ly/2VDhFiZ
An anomalous band originating from the fabella causing semimembranosus impingement presenting as knee pain: a case report
The presentation of patients with knee injuries which can have a significant impact on their ability to work and perform activities of daily living is constantly rising. The posterolateral corner of the knee h...
http://bit.ly/2RjvxAm
The haemodynamic instability score: Development and internal validation of a new rating method of intra-operative haemodynamic instability
BACKGROUND There is no consensus on how to define haemodynamic instability during general anaesthesia. Patients are often classified as stable or unstable based solely on blood pressure thresholds, disregarding the degree of instability. Vasoactive agents and volume therapy can directly influence classification but are usually not considered. OBJECTIVE To develop and validate a scoring tool to quantify the overall degree of haemodynamic instability. DESIGN Retrospective observational study. SETTING University hospital. PATIENTS The development cohort consisted of 50 patients undergoing high-risk surgery with a control group of 50 undergoing video-assisted thoracoscopic surgery. In the validation cohort, there were 153 high-risk surgery patients and 78 controls. INTERVENTION None. MAIN OUTCOME MEASURES The haemodynamic instability score (HI-score) was calculated as a weighted continuous measure ranging from 0 to 160 points, intended to reflect deviations of blood pressure and heart rate from predefined thresholds, and infusion rates of vasoactive agents and fluids. Thresholds were first determined in a development cohort and subsequently tested in a validation cohort. Results are presented as median [interquartile range]. RESULTS In the validation cohort the HI-score was 59 [37 to 96] in the high-risk surgery group compared with 44 [24 to 58] in the control group (P
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http://bit.ly/2H1uwIH
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