Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)

The International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) is comprised of hematologists, hematopathologists, and laboratory scientists and its main goal is to provide a forum for scientific exchange and collaboration. During its first general meeting in April 2006, the IWG-MRT established uniform treatment response criteria for chronic idiopathic myelofibrosis (CIMF); also known as agnogenic myeloid metaplasia (AMM), myelofibrosis with myeloid metaplasia (MMM), and many other names in the hematologic literature. This document summarizes the proceedings from the second meeting of the IWG-MRT, in November 2006, where the group discussed and agreed to standardize the nomenclature referring to CIMF: (i) the term primary myelofibrosis (PMF) was chosen over several other designations including CIMF, AMM, and MMM, (ii) myelofibrosis that develops in the setting of either polycythemia vera (PV) or essential thrombocythemia (ET) will be referred to as post-PV MF and post-ET MF, respectively, and (iii) "leukemic" transformation will be recognized as blast phase disease (PMF-BP, post-PV/ET MF in blast phase).     

Ovarian metastasis from colorectal cancer: prognostic value of radical oophorectomy

BACKGROUND: Ovarian metastases from primary colorectal cancer occur in 3-8% of female patients. The aim of this study was to assess the prognostic value of radical oophorectomy for ovarian metastasis from colorectal cancer. PATIENTS AND METHODS: From our series of 859 patients operated for colorectal cancer from 1982 to 2005 ten patients with isolated ovarian metastasis were retrieved. Ovarian colorectal metastasis diagnosis was confirmed by pathology revision. Overall and disease-free survival after radical oophorectomy for metastases were assessed and compared with literature data. RESULTS: The median follow-up from ovarian metastases resection was 36 months (range 194-14). Survival analysis showed that survival rate after ovarian metastasectomy was 100% at 1 year and 80% at 5 years of follow up. CONCLUSION: Our study, although limited by a relatively short follow-up and small sample size, shows that bilateral oophorectomy for ovarian metastasis from colorectal cancer has a good impact on disease-free and overall survival.     

Distinctive characteristics of diabetes mellitus after hematopoietic cell transplantation during childhood

We report on six patients who developed diabetes mellitus after hematopoietic cell transplantation (HCT). The prevalence in our cohort of long-term survivors after HCT performed below 18 yr of age was 3%. The median age at onset of diabetes was 22.4 yr (range 11.3-34.4). The median period between HCT and diabetes was 10.1 yr (range 5.6-22.1). Five out of the six patients received total irradiation therapy and five had other endocrinological abnormalities. The onset of diabetes in all patients was insidious and none had diabetic ketoacidosis. Body mass indexes at diabetes onset were within normal levels. The clinical and laboratory features that characterized our patients with diabetes after HCT make it difficult to classify them as having type-1 or type-2 diabetes. The relatively high prevalence of diabetes and its insidious onset in this group of patients, advocate clinicians to evaluate carefully even slight variations in fasting blood glucose, usually included in the routine biochemistry follow-up. These data also suggest that HbA1c and oral glucose-tolerance test should be added to the follow-up program of late complications if fasting blood glucose levels are slightly increased.     

Evaluation of Emergency Air Evacuation of Critically Ill Patients from Cruise Ships

BACKGROUND: The study objectives were to assess the ship physician's diagnostic accuracy in making the decision to air evacuate critically ill patients from cruise ships, to determine the outcome of these patients, and the overall benefit of air evacuation. METHODS: From October 1999 to May 2000, we performed a prospective study of critically ill patients coming from cruise ships in the Caribbean and transported to our institution by air ambulance. Demographics, initial diagnosis, and treatment on board were collected by the triage officer at the time of the cruise physician's first call. In route complications and flight team composition were obtained from the air ambulance monitoring log. Patients were followed-up in the hospital for complications, outcome, and final diagnosis.RESULTS: A consecutive series of 104 patients were considered for analysis. There were 65 men and 39 women (mean age: 68.7 years). Cruise physician's diagnosis was correct in more than 90% of the cases. Internal medicine and surgical conditions represented 80.8% and 19.2% of the cases respectively, falling mainly into three categories: cardiac (34.6%), neurological (20.2%), and digestive (14%). Two cardiac arrests and 1 ventricular fibrillation were successfully resuscitated and 5 of 15 myocardial infarctions received thrombolytic therapy on board. Air transfers were warranted in 96.1% of the cases and physician presence in the flight was considered appropriate in 97.6%. In route complications and mortality rate were 5.8% and 2.9% respectively, related to serious cardiac events. Among the 98 hospitalized patients, 10 patients developed new complications and 5 died. The overall mortality rate was 7.7%. CONCLUSION: The cruise industry appears off to a good start in the medical treatment of passengers needing air evacuation to a land based medical facility. There is room for improvement and adoption of American College of Emergency Physicians (ACEP) and International Council of Cruise Lines (ICCL) Health Care Guidelines are meaningful first steps. Analysis of Caribbean medical facilities and implementation of active telemedicine conferencing represent alternatives to air evacuation that need to be studied.     

Current approaches and perspectives in the medical treatment of diabetic retinopathy

Diabetic retinopathy is a leading cause of visual loss in industrialized countries. Its classification includes preclinical, nonproliferative (mild, moderate, and severe or preproliferative diabetic retinopathy) and proliferative stages (low risk, high risk, and advanced). Diabetic maculopathy (exudative, edematous, or ischemic) may be associated with either nonproliferative or proliferative retinopathy. Prevention requires the tightest possible control of both blood glucose and blood pressure. Laser photocoagulation remains the only procedure recommended for severe nonproliferative or proliferative retinopathy and maculopathy. Since it reduces legal blindness by more than 90% in proliferative retinopathy and prevents severe sight loss in diabetic maculopathy, photocoagulation is probably one of the most effective forms of treatment known today. Less destructive approaches are desirable, however, and those currently under phase 3 trial include blockade of angiotensin receptors, the beta-isoform of protein kinase C, and growth hormone secretion by long-acting analogues of somatostatin. Evidence from past randomized controlled studies does not support a role for inhibitors of platelet aggregation, aldose reductase, and advanced glycosylation end products in the prevention/treatment of retinopathy. Future approaches might include the use of thiamine and its analogues in the primary and secondary prevention of early retinopathy and blockers of vascular endothelial growth factor/vascular permeability factor in more advanced stages.     

Neural basis of generation of conclusions in elementary deduction

In everyday life, people untrained in formal logic draw simple deductive inferences from linguistic material (i.e., elementary propositional deductions). Presently, we have limited information on the brain areas implicated when such conclusions are drawn. We used event-related fMRI to identify these brain areas. A set of multiple and independent criteria was derived from the two main theories in the field of reasoning to maximize the reliability of detection of areas in which activity is specifically associated with deductive inferences. Two left lateralized clusters of areas, one in frontal cortex (Brodmann Area 44 and 6) and one in parietal lobe (BA 40), satisfied all criteria; activation was present at the moment of inference, it was shared by both conditional ("if-based") and disjunctive ("or-based") inferences but was greater for disjunctive clauses. Identification of the reasoning network was corroborated by the observation that activity in these areas was greater the longer the reasoning time. Taken together with results from preceding studies, our findings suggest possible theoretically relevant dissociations between elementary propositional deductions and other types of deductive reasoning.     

Efficacy and Safety of Ramipril in Hypertensive Single-Kidney Patients

The effects of medium-term antihypertensive treatment with the ACE inhibitor ramipril were studied on 10 hypertensive single-kidney patients in a double-blind study versus placebo. Patients with renovascular hypertension were excluded. Compared to placebo, ramipril induces a significant reduction of arterial blood pressure (p < 0.02 for systolic, p < 0.01 for diastolic, and p < 0.05 for mean blood pressure), renal vascular resistance (p < 0.005), and microalbuminuria (p < 0.005), but a significant rise of effective renal plasma flow (p < 0.01) and no significant variation of the glomerular filtration rate. The reduction of microalbuminuria was not related to arterial blood pressure variation. Our study shows that ramipril, in appropriately selected-kidney patients, is effective and safe in reducing arterial blood pressure, bringing about an improvement of renal function and reducing microalbuminuria, which is frequently observed in this condition.