Thrombin generation, ProC(®)Global, prothrombin time and activated partial thromboplastin time in thawed plasma stored for seven days and after methylene blue/light pathogen inactivation.

Background: Methylene blue pathogen inactivation and storage of thawed plasma both lead to changes in the activity of several clotting factors. We investigated how this translates into a global loss of thrombin generation potential and alterations in the protein C pathway.

Materials And Methods: Fifty apheresis plasma samples were thawed and each divided into three subunits.

Rigid eye shields: a critical gap in the individual first aid kit.

From 5% to 22% of all U.S. Department of Defense combat casualties between 2001 and 2010 suffered some form of ocular trauma.

Analysis of recovered tourniquets from casualties of Operation Enduring Freedom and Operation New Dawn.

Background: Tourniquet use recently became common in war, but knowledge gaps remain regarding analysis of recovered devices. The purpose of this study was to analyze tourniquets to identify opportunities for improved training.

Methods: We analyzed tourniquets recovered from deceased service members serving in support of recent combat operations by a team at Dover Air Force Base from 2010 to 2012.

Storage of thawed plasma for a liquid plasma bank: impact of temperature and methylene blue pathogen inactivation.

Background: Rapid transfusion of fresh-frozen plasma (FFP) is desired for treating coagulopathies, but thawing and issuing of FFP takes more than 40 minutes. Liquid storage of plasma is a potential solution but uncertainties exist regarding clotting factor stability. We assessed different storage conditions of thawed FFP and plasma treated by methylene blue plus light (MB/light) for pathogen inactivation.

Are serum TSH levels associated with oxidized low-density lipoprotein? Results from the Study of Health in Pomerania.

Objective: Oxidized LDL (oxLDL) is involved in the pathogenesis of atherosclerosis. Thus, it is important to investigate putative risk factors for increased oxLDL. Evidence suggests that, compared to euthyroid individuals, LDL-cholesterol (LDL-C) levels are lower in individuals with overt hyperthyroidism.

A genome-wide association study of metabolic traits in human urine.

We present a genome-wide association study of metabolic traits in human urine, designed to investigate the detoxification capacity of the human body. Using NMR spectroscopy, we tested for associations between 59 metabolites in urine from 862 male participants in the population-based SHIP study. We replicated the results using 1,039 additional samples of the same study, including a 5-year follow-up, and 992 samples from the independent KORA study.

Nesprin-1 and -2 are involved in the pathogenesis of Emery Dreifuss muscular dystrophy and are critical for nuclear envelope integrity.

Emery-Dreifuss muscular dystrophy (EDMD) is a heterogeneous late-onset disease involving skeletal muscle wasting and heart defects caused, in a minority of cases, by mutations in either of two genes encoding the inner nuclear membrane (INM) proteins, emerin and lamins A/C. Nesprin-1 and -2 are multi-isomeric, spectrin-repeat proteins that bind both emerin and lamins A/C and form a network in muscle linking the nucleoskeleton to the INM, the outer nuclear membrane, membraneous organelles, the sarcomere and the actin cytoskeleton. Thus, disruptions in nesprin/lamin/emerin interactions might play a role in the muscle-specific pathogenesis of EDMD.

p.S143F mutation in lamin A/C: a new phenotype combining myopathy and progeria.

We report a young girl with a phenotype combining early-onset myopathy and a progeria. She had myopathy and marked axial weakness during the first year of life; progeroid features, including growth failure, sclerodermatous skin changes, and osteolytic lesions, developed later. We identified the underlying cause to be a hitherto unreported de novo missense mutation in the LMNA gene (S143F) encoding the nuclear envelope proteins lamins A and C.

Basal level glucose-6-phosphatase gene transcription requires binding sites for Sp family proteins within the gene promoter.

The significance of two regions (SpA: -19 to -11 and SpB: -63 to -55) within the human glucose-6-phosphatase (G6Pase) gene promoter for gene expression was examined. The mutation of SpA and SpB together, but not alone, decreased G6Pase promoter activity. Electromobility shift assays showed that SpA and SpB were able to bind the transcription factors Sp1 and Sp3.

Identification of a cAMP response element within the glucose- 6-phosphatase hydrolytic subunit gene promoter which is involved in the transcriptional regulation by cAMP and glucocorticoids in H4IIE hepatoma cells.

The expression of a luciferase reporter gene under the control of the human glucose 6-phosphatase gene promoter was stimulated by both dexamethasone and dibutyryl cAMP in H4IIE hepatoma cells. A cis-active element located between nucleotides -161 and -152 in the glucose 6-phosphatase gene promoter was identified and found to be necessary for both basal reporter-gene expression and induction of expression by both dibutyryl cAMP and dexamethasone. Nucleotides -161 to -152 were functionally replaced by the consensus sequence for a cAMP response element.

Glucose induces glucose 6-phosphatase hydrolytic subunit gene transcription in an insulinoma cell line (INS-1).

Primer extension analysis and RNase protection assays revealed the identity of glucose 6-phosphatase gene transcripts in both the insulinoma cell line INS-1 and hepatic cells. In transient transfection assays of INS-1 cells, using constructs between the human glucose 6-phosphatase gene promoter and a luciferase reporter gene, the reporter gene activity was induced by dexamethasone and dibutyryl cAMP. Furthermore, the promoter was regulated by the glucose concentration in the medium.

The art of unproven remedies.

Dealing with unproven treatments involves more than analyzing the available scientific data. The patient-physician relationship--the foundation of medical practice--is challenged and threatened by unproven treatments. To understand the problem, we need to understand ourselves as well as the patient and the disease.

Unproven arthritis remedies. How to approach the problem.

The traditional medical model (ie, history taking, physical examination, diagnosis, and treatment) is not sufficient in the management of chronic diseases such as arthritis. The complex social interactions that lead patients toward and away from traditional medical treatment must be recognized and addressed. Physicians need to acknowledge the likelihood that their patients may use unproven arthritis remedies and to make an effort to reduce the problem.

DR antigens in systemic sclerosis: lack of clinical correlations.

The information available in the literature is controversial as to the association between HLA-DR and systemic sclerosis (SS). We studied 44 Caucasians with SS and found DR1 (P = 0.025; RR = 2.

A controlled trial of antihypertensive therapy in systemic sclerosis (scleroderma).

Antihypertensive treatment may be life saving in scleroderma renal crisis. Patients surviving such crises frequently have had dramatic improvement in the dermal manifestations of their scleroderma. To investigate the potential role of antihypertensive treatment in nonhypertensive patients we randomly assigned 28 patients with systemic sclerosis into drug (14) and placebo (14) groups, using blocked randomisation , and followed them up in a prospective, double-blind clinical trial for 24 months.

Patients, physicians, and unproven remedies.

Chronic arthritis management is complicated by patient administered unproven treatments. Disease, patient, and physician characteristics all contribute to this problem. Physicians must understand the complex cultural, sociological and psychological variables which encourage the use of unproven treatments.

A multicenter study of outcome in systemic lupus erythematosus. I. Entry variables as predictors of prognosis.

A retrospective study of factors influencing survival in 1,103 patients with systemic lupus erythematosus (SLE) was carried out at 9 university centers diverse in geographic, socioeconomic, and racial characteristics. The mortality and disease characteristics of the patients at study entry varied widely among centers. The survival rates from the time patients with a diagnosis of SLE were first evaluated at the participating center was 90% at 1 year, 77% at 5 years, and 71% at 10 years.

A multicenter study of outcome in systemic lupus erythematosus. II. Causes of death.

Causes of death were examined for 1,103 systemic lupus erythematosus patients who were followed from 1965 to 1978 at 9 centers that participated in the Lupus Survival Study Group. A total of 222 patients (20%) died. Lupus-related organ system involvement (mainly active nephritis) and infection were the most frequent primary causes of death.

The use of unorthodox therapies and marginal practitioners.

This study examines the extent of utilization of unorthodox therapies and marginal practitioners among patients with one broad category of chronic disease, rheumatic disorders. Almost all respondents (94%) had tried some unorthodox remedy or practitioner, and several had used 13 different unorthodox remedies or practitioners. There was no relationship between age, sex, race, geographic location and magnitude of usage of treatments.

Nonsteroidal anti-inflammatory agents in rheumatoid arthritis and ankylosing spondylitis.

The relative effectiveness of six nonsteroidal anti-inflammatory agents was studied in 33 patients with rheumatoid arthritis and 32 patients with ankylosing spondylitis in a double-blind, randomized, prospective study employing a six-way multiple crossover design with six-week trials of each agent. In ankylosing spondylitis, naproxen, indomethacin, and fenoprofen calcium were the most effective agents. In rheumatoid arthritis, relatively little mean difference between drugs was found.

Scleroderma. Current understanding of pathogenesis and management.

Scleroderma is no longer the diagnostic or therapeutic dilemma it previously was. New diagnostic techniques facilitate earlier diagnosis, and many symptoms are amenable to treatment.