The BILAG2004-Pregnancy index is reliable for assessment of disease activity in pregnant SLE patients

Objective. To assess the inter-rater reliability of the BILAG2004-Pregnancy index for assessment of SLE disease activity in pregnancy. Methods. Pregnant SLE patients were recruited from four centres and assessed separately by two raters/physicians in routine clinical practice. Disease activity was determined using the BILAG2004-Pregnancy index. Reliability was assessed using level of agreement, κ-statistics and analysis of disagreement. Major disagreement was defined as a score difference of A and C/D/E or B and D/E between the two raters, and minor disagreement was a score difference of A and B or B and C between raters. Results. A total of 30 patients (63.3% Caucasian, 13.3% Afro-Caribbean, 16.7% South Asian) were recruited. The majority of patients had low-level disease activity according to the local rater's assessment, and there was no grade A activity, with grade B activity present in the following systems: mucocutaneous (nine patients), musculoskeletal (two patients), cardiorespiratory (one patient) and renal (one patient). The distribution of disease activity was similar to the external rater's assessment. Good levels of agreement (>70%) were achieved in all systems. κ-statistics were not appropriate for use in the gastrointestinal, ophthalmic, constitutional and neuropsychiatric systems, as there was minimal variation between patients but good levels of agreement otherwise. There were three major disagreements (0.1 per patient, all differences between B and D/E) and five minor disagreements (0.17 per patient). Conclusion. The BILAG2004-Pregnancy index is reliable for assessment of disease activity in pregnant SLE patients.     

Cardiac risks and management of complications in pregnant women with congenital heart disease

There are a growing number of women with congenital heart disease reaching adulthood and contemplating and/or undergoing pregnancy. However, pregnancy imposes hemodynamic stress on the heart and this can result in maternal, fetal and neonatal complications. Most women with congenital heart disease do well during pregnancy, but some women with high-risk cardiac lesions will not tolerate the hemodynamic changes of pregnancy. Physicians must be aware of the potential risks for the mother both during and after pregnancy, the risks to the fetus and neonate, and the risks and benefits of medications and procedures used during pregnancy. For women with complex cardiac conditions, management during pregnancy benefits from multidisciplinary care involving cardiologists with expertise in pregnancy, obstetricians with expertise in maternal fetal medicine, neonatologists and obstetric anesthetists, among others. This review will focus on the cardiac risks faced by women with congenital heart disease; particularly those at high risk, and on management strategies to mitigate risk and address cardiac complications.     

Pelvic organ prolapse surgery in Western Australia: a population-based analysis of trends and peri-operative complications

Introduction and hypothesis

We previously described a declining rate of surgery in the treatment of pelvic organ prolapse (POP) in Western Australia. This paper builds on previous work by examining temporal trends and the post-operative risk of in-hospital complications, following first time incident prolapse surgery in a population-based cohort of women.

Methods

We investigated rates of prolapse surgery between 1988 and 2005 according to age group and concomitant procedure type for 34,509 women whose data were extracted from the WA Data Linkage System. We investigated changes over time in the demographic characteristics of women undergoing surgery and whether the presence of selected concomitant procedures increased the risk of in-hospital complications.

Results

During the study period, 34,509 women underwent an incident surgery for POP. Concomitant hysterectomy was performed in more than half of all surgeries (52.4 %) and a concomitant urinary incontinence (UI) surgery was noted in 25.8 %. 10.9 % of patients experienced a complication of interest, with the highest percentage of complications recorded in women who underwent multi-concomitant surgery. After controlling for age, comorbidity and time period we found that concomitant UI surgery increases in-hospital complications (OR 1.61 95 % CI 1.42–1.83) only in women who have a repair procedure (colporrhaphy and/or enterocele repair). There was no significant effect of concomitant procedures in women who underwent a combined repair and apical prolapse procedure.

Conclusions

Surgery to treat prolapse is common, has low mortality and concomitant surgery only increases complications when combined with simpler prolapse surgery.     

Clinical impairment in premanifest and early Huntington's disease is associated with regionally specific atrophy

TRACK‐HD is a multicentre longitudinal observational study investigating the use of clinical assessments and 3‐Tesla magnetic resonance imaging as potential biomarkers for future therapeutic trials in Huntington's disease (HD). The cross‐sectional data from this large well‐characterized dataset provide the opportunity to improve our knowledge of how the underlying neuropathology of HD may contribute to the clinical manifestations of the disease across the spectrum of premanifest (PreHD) and early HD. Two hundred and thirty nine gene‐positive subjects (120 PreHD and 119 early HD) from the TRACK‐HD study were included. Using voxel‐based morphometry (VBM), grey and white matter volumes were correlated with performance in four domains: quantitative motor (tongue force, metronome tapping, and gait); oculomotor [anti‐saccade error rate (ASE)]; cognition (negative emotion recognition, spot the change and the University of Pennsylvania smell identification test) and neuropsychiatric measures (apathy, affect and irritability). After adjusting for estimated disease severity, regionally specific associations between structural loss and task performance were found (familywise error corrected, P < 0.05); impairment in tongue force, metronome tapping and ASE were all associated with striatal loss. Additionally, tongue force deficits and ASE were associated with volume reduction in the occipital lobe. Impaired recognition of negative emotions was associated with volumetric reductions in the precuneus and cuneus. Our study reveals specific associations between atrophy and decline in a range of clinical modalities, demonstrating the utility of VBM correlation analysis for investigating these relationships in HD. Hum Brain Mapp, 2013. © 2011 Wiley Periodicals, Inc.