Multiple myeloma, version 1.2013.

These NCCN Guidelines Insights highlight the important updates/changes specific to the management of relapsed or progressive disease in the 2013 version of the NCCN Clinical Practice Guidelines in Oncology for Multiple Myeloma. These changes include the addition of new regimens as options for salvage therapy and strategies to mitigate the adverse effects and risks associated with newer regimens for the treatment of multiple myeloma.

Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma, version 2.2013.

These NCCN Guidelines Insights highlight the important updates/changes specific to the management of Waldenström's Macroglobulinemia/Lymphoplasmacytic Lymphoma. These include the addition of regimens containing novel agents as primary and salvage therapy options, inclusion of the updated summary of response categories and criteria from the sixth international workshop on Waldenström's Macroglobulinemia, and a section on management of peripheral neuropathy in the accompanying discussion.

De novo mutations in ATP1A3 cause alternating hemiplegia of childhood.

Alternating hemiplegia of childhood (AHC) is a rare, severe neurodevelopmental syndrome characterized by recurrent hemiplegic episodes and distinct neurological manifestations. AHC is usually a sporadic disorder and has unknown etiology. We used exome sequencing of seven patients with AHC and their unaffected parents to identify de novo nonsynonymous mutations in ATP1A3 in all seven individuals.

Brief intensive therapy for older adults with newly diagnosed Burkitt or atypical Burkitt lymphoma/leukemia.

Older patients with Burkitt lymphoma/leukemia (BL) have inferior outcomes. Because cyclophosphamide is highly active in BL and can be dose-escalated without stem-cell rescue, we designed a short, cyclophosphamide-intensive regimen without anthracyclines for patients aged ≥ 30 with untreated, non-HIV-associated BL/atypical BL. Two cycles involving cyclophosphamide 1500 mg/m(2), vincristine, rituximab, prednisone, methotrexate 3 g/m(2), and intrathecal cytarabine were delivered 2 weeks apart, followed by intensification with high-dose cyclophosphamide (50 mg/kg/day for 4 days) and rituximab.

Genetic analysis of ancestry, admixture and selection in Bolivian and Totonac populations of the New World.

Background: Populations of the Americas were founded by early migrants from Asia, and some have experienced recent genetic admixture. To better characterize the native and non-native ancestry components in populations from the Americas, we analyzed 815,377 autosomal SNPs, mitochondrial hypervariable segments I and II, and 36 Y-chromosome STRs from 24 Mesoamerican Totonacs and 23 South American Bolivians.

Results And Conclusions: We analyzed common genomic regions from native Bolivian and Totonac populations to identify 324 highly predictive Native American ancestry informative markers (AIMs).

The physician trustee. Balancing clinical perspective and conflicts of interest.

In addition to his or her clinical perspective, a physician on the board brings the risk of conflicts of interest.

Correlations between physician-perceived functional status, patient-perceived health status, and cardiopulmonary exercise results in hypertrophic cardiomyopathy.

Background: Dyspnea and the resulting functional impairment are a common complication in hypertrophic cardiomyopathy (HCM). The relationship between physician-perceived functional status, patient-perceived health status, and objective exercise test results has not been evaluated in this condition.

Purpose: To evaluate the correlation between the Kansas City Cardiomyopathy Questionnaire (KCCQ) and (1) physician's perceived (NYHA class) and (2) objective measurement (cardiopulmonary exercise test) of functional capacity in patients with HCM.

Metabolic insight into mechanisms of high-altitude adaptation in Tibetans.

Recent studies have identified genes involved in high-altitude adaptation in Tibetans. Genetic variants/haplotypes within regions containing three of these genes (EPAS1, EGLN1, and PPARA) are associated with relatively decreased hemoglobin levels observed in Tibetans at high altitude, providing corroborative evidence for genetic adaptation to this extreme environment. The mechanisms that afford adaptation to high-altitude hypoxia, however, remain unclear.

Expanding the spectrum of monoclonal light chain deposition disease in muscle.

Introduction: The diagnosis of amyloid myopathy is delayed when monoclonal gammopathies are not detected on initial testing and muscle biopsies are nondiagnostic, and the EMG and symptoms can mimic an inflammatory myopathy.

Methods: Case report of a patient presenting with severe progressive muscle weakness of unclear etiology despite an extensive workup including two nondiagnostic muscle biopsies.

Results: Directed by MRI, a third biopsy revealed amyloid angiopathy and noncongophilic kappa light chain deposition in scattered subsarcolemmal rings and perimysial regions.

Under the microscope: public boards face unique governance challenges, yet find ways to thrive.

Boards of publicly funded hospitals learn how to navigate unique governance challenges.

Epidemiology and outcomes of Clostridium difficile infections in hematopoietic stem cell transplant recipients.

Background. Clostridium difficile is the leading cause of infectious diarrhea among hospitalized patients and is a major concern for patients undergoing hematopoietic stem cell transplantation (HSCT). Risk factors and the natural history of C.

Location, location, location.

Rural hospitals rethink their strategies for recruiting physicians, including offering unique incentives.

Where are the specialists?

It's not just primary care physicians who are in short supply. Hospitals are using a variety of creative strategies to recruit certain hard-to-find specialists.

Lenalidomide-induced immunomodulation in multiple myeloma: impact on vaccines and antitumor responses.

Purpose: To show that the immunomodulatory drug lenalidomide can be used in patients with relapsed multiple myeloma to augment vaccine responses.

Experimental Design: Early phase clinical trial of patients with multiple myeloma who received at least one prior therapy. Patients were treated with single-agent lenalidomide and randomized to receive two vaccinations with pneumococcal 7-valent conjugate vaccine (PCV) on different schedules.

Commanding safety: how the VA drives systemwide error prevention.

Using presurgery briefings and root cause analysis, Veterans Affairs hospitals make impressive safety gains.

Does your patient really understand?

Research shows that more than 80 percent of patients have poor health literacy. Cutting the jargon, asking direct questions and simplifying signage are among the ways hospitals are working to improve communication--and care.

Cascade catalysis for the homogeneous hydrogenation of CO2 to methanol.

This communication demonstrates the homogeneous hydrogenation of CO(2) to CH(3)OH via cascade catalysis. Three different homogeneous catalysts, (PMe(3))(4)Ru(Cl)(OAc), Sc(OTf)(3), and (PNN)Ru(CO)(H), operate in sequence to promote this transformation.

A comprehensive map of mobile element insertion polymorphisms in humans.

As a consequence of the accumulation of insertion events over evolutionary time, mobile elements now comprise nearly half of the human genome. The Alu, L1, and SVA mobile element families are still duplicating, generating variation between individual genomes. Mobile element insertions (MEI) have been identified as causes for genetic diseases, including hemophilia, neurofibromatosis, and various cancers.

Crohn's disease and genetic hitchhiking at IBD5.

Inflammatory bowel disease 5 (IBD5) is a 250 kb haplotype on chromosome 5 that is associated with an increased risk of Crohn's disease in Europeans. The OCTN1 gene is centrally located on IBD5 and encodes a transporter of the antioxidant ergothioneine (ET). The 503F variant of OCTN1 is strongly associated with IBD5 and is a gain-of-function mutation that increases absorption of ET.

New strategies for psych care.

Growing numbers of patients and shrinking budgets have inspired providers to develop creative solutions, such as offering integrated treatment and fast-tracking ED patients.