Case-Control Cohort Study of Patients' Perceptions of Disability in Mastocytosis

BACKGROUND: Indolent forms of mastocytosis account for more than 90% of all cases, but the types and type and severity of symptoms and their impact on the quality of life have not been well studied. We therefore performed a case-control cohort study to examine self-reported disability and impact of symptoms on the quality of life in patients with mastocytosis. METHODOLOGY/PRINCIPAL FINDINGS: In 2004, 363 mastocytosis patients and 90 controls in France were asked to rate to their overall disability (OPA score) and the severity of 38 individual symptoms. The latter was used to calculate a composite score (AFIRMM score). Of the 363 respondents, 262 were part of an ongoing pathophysiological study so that the following data were available: World Health Organization classification, standard measures of physical and psychological disability, existence of the D816V KIT mutation, and serum tryptase level. The mean OPA and AFIRMM scores and the standard measures of disability indicated that most mastocytosis patients suffer from disabilities due to the disease. Surprisingly, the patient's measurable and perceived disabilities did not differ according to disease classification or presence or absence of the D816V KIT mutation or an elevated (> or = 20 ng/mL) serum tryptase level. Also, 32 of the 38 AFIRMM symptoms were more common in patients than controls, but there were not substantial differences according to disease classification, presence of the D816V mutation, or the serum tryptase level. CONCLUSIONS: On the basis of these results and for the purposes of treatment, we propose that mastocytosis be first classified as aggressive or indolent and that indolent mastocytosis then be categorized according to the severity of patients' perceived symptoms and their impact on the quality of life. In addition, it appears that mastocytosis patients suffer from more symptoms and greater disability than previously thought, that mastocytosis may therefore be under-diagnosed, and that the symptoms of the indolent forms of mastocytosis might be due more to systemic release of mediators than mast cell burden

Pregnancy outcomes and cytomegalovirus DNAaemia in HIV infected pregnant women with CMV

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Rate , correlates and outcomes of repeat pregnancy in HIV-infected women

Objectives: The aim of the study was to assess the rate, determinants, and outcomes of repeat pregnancies in women with HIV infection. Methods: Data from a national study of pregnant women with HIV infection were used. Main outcomes were preterm delivery, low birth weight, CD4 cell count and HIV plasma viral load. Results: The rate of repeat pregnancy among 3007 women was 16.2%. Women with a repeat pregnancy were on average younger than those with a single pregnancy (median age 30 vs. 33 years, respectively), more recently diagnosed with HIV infection (median time since diagnosis 25 vs. 51 months, respectively), and more frequently of foreign origin [odds ratio (OR) 1.36; 95% confidence interval (CI) 1.10–1.68], diagnosed with HIV infection in the current pregnancy (OR: 1.69; 95% CI: 1.35–2.11), and at their first pregnancy (OR: 1.33; 95% CI: 1.06–1.66). In women with sequential pregnancies, compared with the first pregnancy, several outcomes showed a significant improvement in the second pregnancy, with a higher rate of antiretroviral treatment at conception (39.0 vs. 65.4%, respectively), better median maternal weight at the start of pregnancy (60 vs. 61 kg, respectively), a higher rate of end-of-pregnancy undetectable HIV RNA (60.7 vs. 71.6%, respectively), a higher median birth weight (2815 vs. 2885 g, respectively), lower rates of preterm delivery (23.0 vs. 17.7%, respectively) and of low birth weight (23.4 vs. 15.4%, respectively), and a higher median CD4 cell count (+47 cells/μL), with almost no clinical progression to Centers for Disease Control and Prevention stage C (CDC-C) HIV disease (0.3%). The second pregnancy was significantly more likely to end in voluntary termination than the first pregnancy (11.4 vs. 6.1%, respectively). Conclusions: Younger and foreign women were more likely to have a repeat pregnancy; in women with sequential pregnancies, the second pregnancy was characterized by a significant improvement in several outcomes, suggesting that women with HIV infection who desire multiple children may proceed safely and confidently with subsequent pregnancies

Good prenatal detection rate of major birth defects in HIV-infected pregnant women in Italy

What's already known about this topic? Exposure to antiretroviral treatment in pregnancy does not seem to increase the risk of birth defects, but there is no information on the rate of prenatal detection of such defects. What does this study adds? We provide for the first time, in a national case series, information about prenatal detection rate in women with HIV (51.6% for any major defect, 66.7% for chromosomal abnormalities, and 85% for severe structural defect

Consequences of presentation with advanced HIV disease in pregnancy : data from a national study in Italy

Among 469 women with a diagnosis of HIV in pregnancy, 74 (15.8%) presented with less than 200 CD4 cells per cubic millimeter. The only variable significantly associated with this occurrence was African origin (odds ratio: 2.22, 95% confidence intervals: 1.32 to 3.75, P = 0.003). Four women with low CD4 (5.6%), compared with none with higher CD4 counts, had severe AIDS-defining conditions (P < 0.001) during pregnancy or soon after delivery, and one transmitted HIV to the newborn. Early preterm delivery (<32 weeks) was significantly more frequent with low CD4 (6.2% vs. 1.4%, P = 0.015). An earlier access to HIV testing, particularly among immigrants of African origin, can prevent severe HIV-related morbidity

Managing Neovascular Age-Related Macular Degeneration in Clinical Practice: Systematic Review, Meta-Analysis, and Meta-Regression

The use of anti-vascular endothelial growth factor (VEGF) agents has profoundly changed the prognosis of neovascular age-related macular degeneration (nAMD). As clinical experiences have accumulated, it has become mandatory to summarize data to give information that can be useful in everyday practice. We conducted a systematic review to identify randomized controlled trials (RCTs) and observational studies that reported 12-month changes in best-corrected visual acuity (BCVA) in patients with nAMD on anti-VEGF monotherapy. Data were analyzed in a random-effects meta-analysis with BCVA change as the primary outcome. Meta-regression was conducted to evaluate the impact of multiple covariates. Four hundred and twelve heterogeneous study populations (109,666 eyes) were included. Anti-VEGFs induced an overall improvement of +5.37 ETDRS letters at 12 months. Meta-regression showed that mean BCVA change was statistically greater for RCTs (p = 0.0032) in comparison with observational studies. Populations following a proactive regimen had better outcomes than those following a reactive treatment regimen. Mean BCVA change was greater in younger populations, with lower baseline BCVA and treated with a higher number of injections (p < 0.001). Our results confirm that anti-VEGFs may produce a significant functional improvement at 12 months in patients with nAMD

Improved dynamic response of magnetic feedback in RFX-mod and DIII-D

The wall of any magnetic fusion device is characterized by the presence of several 3D structures, such as portholes for diagnostics and for heating and current drive systems, coil feeds and other features. Time-varying magnetic fields induce eddy currents in the wall, whose pattern is modified by these structures, giving rise to magnetic field errors that can be amplified or shielded by the plasma. Two examples will be given on how the dynamic response of a 3D wall to external magnetic fields can be identified and used to optimize magnetic feedback. In the RFX-mod reversed-field pinch, a dynamic decoupler algorithm has been developed, which allows for the production of pure radial magnetic field harmonics inside the wall, reducing the harmonic distortion due to the 3D wall structures. This is applied here to the problem of producing helical boundary conditions to control helical RFP equilibria. In the DIII-D tokamak, a frequency-dependent scheme for the compensation of the magnetic sensors from spurious n = 1 fields due to the coupling with the feedback and axisymmetric coils has been recently implemented in real time and tested with plasma. The possible relevance of these 3D effects for high performance scenarios is discussed

Does pre-operative breast MRI have an impact on surgical outcomes in high-grade DCIS?

OBJECTIVES: High-grade DCIS (HG DCIS) is associated with upgrade to invasive disease but few studies evaluate the role of MRI in this subset of DCIS. This study compared surgical outcomes of females with HG DCIS on biopsy who had pre-operative MRI with those that proceeded directly to surgery. METHODS: This single-centre retrospective, observational study identified patients with pure HG DCIS on pre-operative biopsy from the pathology database. Surgical outcomes, clinicopathological and radiological features were obtained for all patients. RESULTS: From August 2015 to February 2020, 217 patients had HG DCIS on biopsy. Pre-operative MRI was performed in 40 (MRI group) and not in 88 (No MRI group) patients. Initial mastectomy was performed in 25/40 (63%) women in the MRI group and 20/88 (23%) women in the no MRI group (p < 0.0001). No difference was observed in re-operation rate between the two groups, 15% in MRI group vs 22% in No MRI group (p = 0.4749). Mean tumour size on histology was larger in mastectomy cases in the MRI group (73.4 mm, range 6-140 mm), than the total MRI group, (58.3 mm, range 0-140 mm) or no MRI group (30.7 mm, range 0-130 mm) (p < 0.0001). CONCLUSIONS: Pre-operative MRI in HG DCIS is associated with higher mastectomy rates, possibly due to patient selection for MRI, as tumours on final histology were significantly larger. Fewer re-operations were observed in the MRI group although this was not significant. ADVANCES IN KNOWLEDGE: Breast MRI performed pre-operatively in HG DCIS is associated with higher mastectomy rates and fewer re-operation rates