Citations

By the current WHO classification, benign notochordal cell tumor (BNCT) and chordoma comprise the entire spectrum of notochordal-derived tumors. They have defined radiologic and histologic criteria, and differ considerably in management and clinical outcome. Chordomas are malignant tumors; they show progressive, destructive growth and have the capacity for metastasis. In contrast, BNCT are benign and show limited intraosseous growth. Patients with BNCT can be managed with serial imaging or conservative excision, whereas patients with spinal/sacral chordomas typically undergo radical en bloc resection often with adjuvant therapy and significant morbidity. As such, the distinction between BNCT and chordoma is critically important. We have seen 4 unusual notochordal tumors with radiologic and/or histologic features that defy classification as either BNCT or chordoma. Cases occurred in 4 adults (53 to 83 y), and involved the lumbar spine (N=2) and sacrum (N=2). Three cases had subtle radiologic features of cortical permeation with minimal soft tissue extension. All 4 cases had the characteristic histologic features of BNCT; however, 2 cases also had focal myxoid change. Three patients were followed with serial imaging (follow-up range, 26 to 120 mo); 2 showed no disease progression and 1 had a 10-year cumulative interval growth of 3.7 mm. One patient underwent sacrectomy. The tumor was examined in toto and had the characteristic histologic features of BNCT, with the exception of minimal soft tissue extension. On the basis of these observations, we propose a provisional designation of atypical notochordal cell tumors (ANCT) be used for the subset of notochordal-derived tumors that fail to fulfill current diagnostic criteria for either BNCT or chordoma. We would argue that designating these atypical notochordal tumors as chordoma precipitates potentially overly aggressive surgical management. Patients with ANCT may be better managed by close observation and serial imaging. Additional studies with more cases and longer clinical follow-up should clarify the relationship of ANCT to BNCT and chordoma.

Abstract Postoperative residual neuromuscular block has been recognized as a potential problem for decades, and it remains so today. Traditional pharmacologic antagonists (anticholinesterases) are ineffective in reversing profound and deep levels of neuromuscular block; at the opposite end of the recovery curve close to full recovery, anticholinesterases may induce paradoxical muscle weakness. The new selective relaxant-binding agent sugammadex can reverse any depth of block from aminosteroid (but not benzylisoquinolinium) relaxants; however, the effective dose to be administered should be chosen based on objective monitoring of the depth of neuromuscular block. To guide appropriate perioperative management, neuromuscular function assessment with a peripheral nerve stimulator is mandatory. Although in many settings, subjective (visual and tactile) evaluation of muscle responses is used, such evaluation has had limited success in preventing the occurrence of residual paralysis. Clinical evaluations of return of muscle strength (head lift and grip strength) or respiratory parameters (tidal volume and vital capacity) are equally insensitive at detecting neuromuscular weakness. Objective measurement (a train-of-four ratio greater than 0.90) is the only method to determine appropriate timing of tracheal extubation and ensure normal muscle function and patient safety.

AbstractObjectiveThe aim of this study was to examine the relative contributions of body mass index (BMI) and pubertal measures for risk and age of onset of pediatric MS.MethodsCase–control study of 254 (63% female) MS cases (onset<18 years of age) and 420 (49% female) controls conducted at 14 U.S. Pediatric MS Centers. Sex‐ and age‐stratified BMI percentiles were calculated using CDC growth charts from height and weight measured at enrollment for controls, and within 1 year of onset for MS cases. Sex‐stratified associations between MS risk and age at symptom onset with both BMI and pubertal factors were estimated controlling for race and ethnicity.ResultsOnly 11% of girls and 15% of boys were prepubertal (Tanner stage I) at MS onset. 80% of girls had onset of MS after menarche. BMI percentiles were higher in MS cases versus controls (girls: P < 0.001; boys: P = 0.018). BMI was associated with odds of MS in multivariate models in postpubertal girls (OR = 1.60, 95% confidence interval [CI]: 1.12, 2.27, P = 0.009) and boys (OR = 1.43, 95% CI: 1.08, 1.88, P = 0.011). In girls with MS onset after menarche, higher BMI was associated with younger age at first symptoms (P = 0.031). Younger menarche was associated with stronger effects of BMI through mediation and interaction analysis. In pubertal/postpubertal boys, 89% of whom were obese/overweight, earlier sexual maturity was associated with earlier onset of MS (P < 0.001).InterpretationHigher BMI in early adolescence is a risk factor for MS in girls and boys. Earlier age at sexual maturity contributes to earlier age at MS onset, particularly in association with obesity.

Patients with cirrhosis are at high risk for sepsis and sepsis-related mortality. Aggressive treatment aimed at avoidance of hypoperfusion and prompt identification and treatment of the causative organism can improve patients’ survival. Fluid administration is the first-line treatment to improve perfusion to vital organs; however, care should be taken to assess true intravascular volume status. In patients with adequate intravascular volume, vasopressors are then added to support blood pressure. Complications of cirrhosis often worsen in the setting of sepsis. Portosystemic encephalopathy, pulmonary complications, renal complications, adrenal insufficiency, malnutrition, and insufficient glucose control all must be considered and treated to support a patient with cirrhosis through sepsis. The quality of care that these patients receive ultimately influences their survival.

The most common causes of chronic cholestatic liver disease are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both disease processes are characterized by a destruction of intrahepatic and/or extrahepatic biliary ducts. The etiology is not entirely clear; however, there is an underlying autoimmune component contributing to both disease processes. Although PBC and PSC are often diagnosed and managed in the outpatient setting, in some instances, a patient may have jaundice, fatigue, and pruritus requiring evaluation and determination of the cholestatic cause. Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms. Liver transplant has the potential for improving quality of life, although disease recurrence is a risk.

Key Points The expression level of ROR1 on CLL cells varies between patients. High-level CLL-cell expression of ROR1 associates with more aggressive disease.

In this article, we present the case of a 52-year-old male with a history of parotid carcinosarcoma with initial diagnosis being 18 months prior. Initial treatment included a combination of gamma knife surgery coupled with high dosage chemotherapy and X-ray radiation therapy. At the time of follow-up, the patient presented with no complaints and had a nearly normal physical exam with the exception of some facial nerve weakness on the same side as the initial surgery. Despite being asymptomatic, the patient had a significant progression of disease that was manifested with intracranial lesions, multiple pathologic fractures, and a dramatic increase in overall tumor burden. Ultimately, the patient decided to pursue comfort measures only and succumbed to the disease peacefully soon thereafter.

Acute liver failure, also known as fulminant hepatic failure, is a rare life-threatening disease that has a high mortality rate and affects many organ systems. Causes of acute liver failure vary—it can be attributed to drugs, viruses, and other uncommon sources. Complications of liver failure can include encephalopathy, cerebral edema, sepsis, renal failure, gastrointestinal bleeding, and respiratory failure. Fortunately, with advances in critical care medicine and emergent liver transplant, mortality rates have decreased in the past decade. This article reviews acute liver failure, its manifestations in different organ systems, and its treatment.

The F sex factor plasmid of Escherichia coli contains a pair of genes, ccdA and ccdB, whose protein gene products are involved in an unusual feature of plasmid maintenance. The CcdB protein is a cytotoxin that becomes activated when the F plasmid is lost, thereby killing the F- segregant cells. In F+ cells, the CcdA protein protects against the lethal effects of CcdB. In the present study we show that ccdA and ccdB expressions are negatively autoregulated at the level of transcription. Genetic studies showed that repression required at least ccdB; ccdA alone was without effect, and ccdB alone was not examined because it is lethal. Ccd-operator complexes were purified and contained a mixture of both CcdA and CcdB proteins; however, we could not conclude from our results whether CcdA was necessary for DNA binding or autorepression. By using restriction fragments of the promoter-operator region, we obtained results indicating that at least two DNA-binding sites existed for the Ccd protein(s). Subsequent footprinting of the binding sites showed protection over about a 113-base-pair region encompassing the putative promoter-operator and the beginning of the ccdA gene.