Acquired aplastic anemia

In aplastic anemia, hematopoiesis fails: Blood cell counts are extremely low, and the bone marrow appears empty. The pathophysiology of aplastic anemia is now believed to be immune-mediated, with active destruction of blood-forming cells by lymphocytes. The aberrant immune response may be triggered by environmental exposures, such as to chemicals and drugs or viral infections and, perhaps, endogenous antigens generated by genetically altered bone marrow cells. In patients with post-hepatitis aplastic anemia, antibodies to the known hepatitis viruses are absent; the unknown infectious agent may be more common in developing countries, where aplastic anemia occurs more frequently than it does in the West. The syndrome paroxysmal nocturnal hemoglobinuria (PNH) is intimately related to aplastic anemia because many patients with bone marrow failure have an increased population of abnormal cells. In PNH, an entire class of proteins is not displayed on the cell surface because of an acquired X-chromosome gene mutation. The PNH cells may have a selective advantage in resisting immune attack. In contrast, the disease myelodysplasia can be confused with aplasia and can also evolve from aplastic anemia. The occurrence of cytogenetic abnormalities in patients years after presentation implies that genomic instability is a feature of this immune-mediated disease. Aplastic anemia can be effectively treated by stem-cell transplantation or immunosuppressive therapy. Transplantation is curative but is best used for younger patients who have histocompatible sibling donors. Antithymocyte globulin and cyclosporine restore hematopoiesis in approximately two thirds of patients. However, recovery of blood cell count is often incomplete, recurrent pancytopenia requires retreatment, and some patients develop late complications (especially myelodysplasia).     

Detection of erosions in the rheumatoid hand; a comparative study of multidetector computerized tomography versus magnetic resonance scanning

OBJECTIVE: To compare the detection and scoring of erosions in patients with rheumatoid arthritis (RA) using magnetic resonance (MR) and multidetector helical computerized tomographic (CT) scanning. METHODS: Comparative CT and MR scans of the dominant wrist were obtained from 9 patients with RA and clinical examination was performed to assess disease activity. MR and CT scans were scored for erosions and MR scans for bone edema by 2 radiologists using a validated system. Radiographs of the hands and feet were also scored for erosions using the modified Sharp score. RESULTS: In 117 of 135 (87%) sites there was concordance for erosions between MR and CT scans. At the remaining 18/135 sites (13%), erosions were identified by CT but not MR in 12/135 (9%) and by MR but not CT in 6/135 (4%). Partial volume artefacts on MR images and shifts in slice position were the most common reasons for erosion mismatch between MR and CT. The mean CT bone erosion score was significantly higher than the MR erosion score when individual bony sites were examined (p = 0.024), with the greatest difference being at the metacarpal bases. The total bone erosion score also tended to be higher on CT than MR [median scores of 20 (range 0-66) and 12 (0-51), respectively; p = 0.060]. MR and CT erosion scores correlated strongly with the total Sharp score (r = 0.93, p = 0.0002 and r = 0.94, p = 0.0002, respectively) and with the Disease Activity Score (MR: r = 0.77, p = 0.02; CT: r = 0.71, p = 0.03). CONCLUSION: Most erosions were detected using both modalities, but erosion scores were higher on CT than MR scans, especially at the metacarpal bases. It is possible that small erosions in some regions are more easily detected by CT because of its ability to clearly delineate cortical bony margins.     

Two dimensional Doppler echocardiographic/M mode echocardiographic and phonocardiographic method for study of extracardiac heterograft valved conduits in the right ventricular outflow tract position

Significant concern exists over the long-term results of right ventricular outflow tract repair using heterograft valved conduits. Because these conduits and valves are difficult to image using ultrasound, a serially applicable two dimensional Doppler echocardiographic, M mode echocardiographic and phonocardiographic method for noninvasive investigation was developed and applied in 15 children. The method provides two dimensional echocardiographic imaging of valve contour and motion, as well as M mode and phonocardiographic analysis and quantitative range-gated Doppler information about the timing of flow through the conduit. Conduit diameter in two dimensional echocardiographic images correlated well with known conduit size (r = +0.96). A thickened and stenosed heterograft valve was predicted in two patients before hemodynamic investigation. This new method provides serially obtainable information to aid in the management of children and infants with a valved conduit placed for repair of congenital heart malformations and aids in planning the timing of hemodynamic follow-up studies.     

Dementia assessment in primary care: results from a study in three managed care systems

BACKGROUND: Prior research has found that dementia is often undiagnosed in primary care, but there has been limited research on whether physicians respond to symptoms, behaviors, or other events that may be indicators of dementia. METHODS: A cross-sectional cohort study design was used to screen 553 patients aged 75 years or older for dementia in 3 managed health care systems in Portland, Oregon. For participants determined to be cognitively impaired, their medical charts were reviewed to determine if they had experienced adverse events, had been clinically evaluated for possible dementia, had received a diagnosis of dementia, or had been offered treatment. RESULTS: Nearly 43% of participants were identified as cognitively impaired: 29.7% were classified as mildly cognitively impaired (MI) and 13.7% as moderately to severely cognitively impaired (MSI). Eighteen percent of the MI group and 34.8% of the MSI group had evidence in their medical chart of having been clinically evaluated for dementia. None of the MI group and only 4.3% of the MSI group had been offered a cholinesterase inhibitor. Nearly two thirds (61.6%) of the MI and three fourths (75.4%) of the MSI participants had experienced 1 or more adverse events. Of those who had experienced adverse events, less than one quarter (23.7%) in the MI group and less than one half (44.2%) in the MSI group had received a clinical evaluation for dementia. CONCLUSIONS: These findings suggest the need for greater attention by primary care physicians to the cognitive functioning of older patients, especially patients who experience adverse events that may be indicators of dementia.     

Liver Fibrosis is Associated with Ischemic Stroke Risk in Women but not Men: The REGARDS Study

BackgroundNonalcoholic fatty liver disease is inconsistently associated with ischemic stroke, with one study suggesting an association in women and not men. The relative importance of liver fibrosis, as opposed to fatty liver, for cardiovascular risk is increasingly appreciated. We hypothesized that advanced liver fibrosis is associated with incident ischemic stroke risk, and especially in women.MethodsWe performed a case-cohort study in the REasons for Geographic and Racial Differences in Stroke cohort. Black and white individuals aged 45 and older were recruited between 2003 and 2007 and followed for ischemic stroke. The Fibrosis-4 (FIB-4) score and Nonalcoholic Fatty Liver Disease Fibrosis Score (NFS) were calculated using baseline data for stroke cases and a cohort random sample; advanced liver fibrosis was classified using validated cutoffs. Cox proportional hazards models were used to estimate hazard ratios (HR) of stroke after adjusting for potential confounders. Sex differences were assessed.ResultsThere were 572 incident ischemic strokes (285 in women) over 5.4 (SD, 2.2) years. Advanced liver fibrosis was not significantly associated with ischemic stroke overall using the FIB-4 (HR 1.44; 95% CI 0.49–4.28) or NFS (HR 1.76; 95% CI 0.67–4.61). However, liver fibrosis was associated with stroke in women (HR 3.51; 95% CI 1.00–12.34) but not men (HR 0.70, 95% CI 0.16–3.16) (P = 0.098 for interaction) when using FIB-4. A similar but non-significant sex difference was seen for NFS.ConclusionAdvanced liver fibrosis may be associated with a higher risk of ischemic stroke in women but not men.     

Barriers to physical activity during the COVID-19 pandemic in adults: a cross-sectional study

Sport Sciences for Health - Social isolation due to the coronavirus disease 2019 (COVID-19) pandemic has reduced physical activity levels in both men and women. The identification of barriers to...