Isolated unilateral absence of a pulmonary artery: a case report and review of the literature

OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. METHODS: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. RESULTS: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. CONCLUSION: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes

Low agreement between cardiologists diagnosing left ventricular hypertrophy in children with end-stage renal disease

Background: Monitoring of the appearance of left ventricular hypertrophy (LVH) by echocardiography is currently recommended for in the management of children with End-stage renal disease (ESRD). In order to investigate the validity of this method in ESRD children, we assessed the intra- and inter-observer reproducibility of the diagnosis LVH. Methods. Echocardiographic measurements in 92 children (0-18 years) with ESRD, made by original analysists, were reassessed offline, twice, by 3 independent observers. Smallest detectable changes (SDC) were calculated for continuous measurements of diastolic interventricular septum (IVSd), Left ventricle posterior wall thickness (LVPWd), Left ventricle end-diastolic diameter (LVEDd), and Left ventricle mass index (LVMI). Cohen's kappa was calculated to assess the reproducibility of LVH defined in two different ways. LVHWT was defined as Z-value of IVSd and/or LVPWd>2 and LVHMI was defined as LVMI> 103 g/m 2 for boys and >84 g/m2 for girls. Results: The intra-observer SDCs ranged from 1.6 to 1.7 mm, 2.0 to 2.6 mm and 17.7 to 30.5 g/m2 for IVSd, LVPWd and LVMI, respectively. The inter-observer SDCs were 2.6 mm, 2.9 mm and 24.6 g/m2 for IVSd, LVPWd and LVMI, respectively. Depending on the observer, the prevalence of LVHWT and LVHMI ranged from 2 to 30% and from 8 to 25%, respectively. Kappas ranged from 0.4 to 1.0 and from 0.1 to 0.5, for intra-and inter- observer reproducibility, respectively. Conclusions: Changes in diastolic wall thickness of less than 1.6 mm or LVMI less than 17.7 g/m2 cannot be distinguished from measurement error in individual children, even when measured by the same observer. This limits the use of echocardiography to detect changes in wall thickness in children with ESRD in routine practice

The 'reach' of Digital Language Archives: towards criteria for evaluation

Over the last decade, and with the help of digital media and technologies, archives (with the focus here on archives for endangered and minority languages) have extended their focus from preservation to also becoming facilities for dissemination. Their innovations have largely been on ‘discovery’: firstly by encouraging digitisation and inclusion of analogue and obscure materials, and by partnership with funding institutions to support the creation of new, ‘born digital’ language resources; and secondly through online provision of language resources via web catalogues driven by standardised metadata and in some cases providing enhanced discovery through web portals aggregating the holdings of multiple archives. These advances have increased the visibility, relevance and authority of archives for language-related disciplines and for language-speaker communities. This paper considers a broader set of parameters describing the ‘reach’ of archives, where ‘reach’ includes (a) archives’ understanding of their key audiences in order to provide appropriate services for them, e.g. identifying a range of relevant audiences, their languages of access, their varied technological and information literacies, interface design and usability; (b) discovery, drawing on the understandings of audiences in order to help them browse, navigate, search, identify and select their items of interest; (c) delivery, i.e. making available selected resources according to users’ preferences whether by download, view-in-browser, through apps or other means; (d) access management such that resource delivery follows depositors’ and communities’ preferences, and where users have ways of applying for and negotiating for access; (e) information accessibility, where the actual desired content is accessible to users, whether in terms of contextualisation or appropriate complexity, language, or modality; and finally (f) feedback channels, where users can utilise the archive to provide feedback to depositors or to enhance deposits with user-generated content. Through considering how a number of archives are providing such services, we can see their transition from repositories of memory to facilities for fostering participation and understanding