Systemic capillary leak syndrome: recognition prevents morbidity and mortality.

Idiopathic systemic capillary leak syndrome (SCLS) is extremely rare but carries a high morbidity and mortality. The diagnosis is made clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis. We report a case of SCLS to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin in this condition.

Clinical efficacy and safety of the factor VIII/von Willebrand factor concentrate BIOSTATE in patients with von Willebrand's disease: a prospective multi-centre study.

von Willebrand's disease (VWD) is an inherited bleeding disorder characterized by deficient levels of or dysfunctional von Willebrand factor (VWF). This phase II/III open-label, multicentre study evaluated the efficacy and safety of BIOSTATE, a high purity plasma-derived double-virus inactivated FVIII/VWF concentrate, when used in non-surgical bleeds, surgical procedures and prophylactic therapy in VWD patients for whom desmopressin treatment was deemed ineffective, inadequate or contraindicated. Twenty three patients (7 type 1, 9 type 2 and 7 type 3; 12 male, 11 female), who received FVIII/VWF concentrate as part of their VWD management, were recruited prospectively between December 2004 and May 2007 from eight centres in Australia and New Zealand. BIOSTATE dosing was based on pre-treatment FVIII:C and/or VWF:RCo plasma levels and a predetermined dosing guide. Haemostatic efficacy of BIOSTATE was rated as excellent or good for all major and minor surgery events, long-term prophylaxis, and for four of the six assessable non-surgical bleeding events. Blood transfusions were required by two major surgery patients as well as one patient with a non-surgical bleed. The median overall exposure to BIOSTATE across all groups was 8 days, greater in the prophylactic group (range 53-197) compared with major surgery (3-24), minor surgery (1-8) and non-surgical bleeds (1-10). BIOSTATE was shown to be efficacious and well tolerated when treating patients with VWD. This study also provides important insights into dosing regimens with BIOSTATE and the role of monitoring therapy with FVIII:C and VWF:RCo.

Clinical role of flow cytometry in redefining bone marrow involvement in diffuse large B-cell lymphoma (DLBCL) - a new perspective.

AIMS: The clinical role of flow cytometry in staging bone marrow in diffuse large B-cell lymphoma (DLBCL), especially its impact on outcome, remains uncertain. The aim was to determine the contribution of flow cytometry to conventional staging, and to study the impact of this revised staging on survival. METHODS AND RESULTS: One hundred and thirteen cases of DLBCL diagnosed at The Canberra Hospital from 1996 to 2005 were identified. Blinded analysis of bone marrow (BM) morphology and flow cytometric data showed involvement on morphology (M) in 25 (22.1%) cases, on flow cytometry (F) in 21 (18.6%) cases and overall (M + F) in 32 cases (28.3%); discordance was noted in 16 cases (16.1%). Cases with and without marrow involvement on conventional staging alone (M) had no significant difference in survival (P = NS). However, when BM involvement was defined as positivity on morphology and/or flow cytometry (M + F), the median survival of patients with involvement was significantly worse than patients without involvement (P = 0.026). CONCLUSIONS: Flow cytometry-positive cases should be included with those positive on morphology in a summative model to define BM involvement in DLBCL, as it may have a potential impact on predicting outcome.

Health-related quality of life in chronic coagulation disorders.

Measurement of health-related quality of life (HR-QoL) is used in patients with haemophilia as a way of assessing the effectiveness of health care, especially as cure is not possible. We report the first such study on patients with chronic coagulation disorders in Australia, using The RAND 36-item Health Survey 1.0 (SF-36), a standardized validated questionnaire combined with a semistructured interview. The mean scores for the eight domains of the SF-36 ranged from 52.5 +/- 42.1 for physical role to 80.0 +/- 20.0 for social functioning. Comparison with normative data obtained from the Australian Bureau of Statistics (ABS) demonstrated a reduction in all domains in this population with statistically significant reductions in general health, physical role limitation and vitality. Comparison with other studies indicates that the HR-QoL of patients with haemophilia and von Willebrand's disorder in Australia is comparable with other Western countries, reflecting the overall similar quality of care available to these patients. This study also provides a good cross-sectional view of the psychosocial factors of life in patients in Australia and recognizes the positive family support available to this population, while raising relevant shortcomings in schools and at the workplace that need to be studied further in a controlled manner.

A method for the determination of flux in elementary modes, and its application to Lactobacillus rhamnosus.

In this article we address the question of how, given information about the reaction fluxes of a system, flux values can be assigned to the elementary modes of that system. Having described a method by which this may be accomplished, we first illustrate its application to a hypothetical, in silico system, and then apply it to fermentation data from Lactobacillus rhamnosus. This reveals substantial changes in the flux values assigned to elementary modes, and thus to the internal metabolism, as the fermentation progresses. This is information that could not, to our knowledge, be obtained by existing methods. The relationship between our technique and the well-known method of Metabolic Flux Analysis is also discussed.

Partial deletion of chromosome 1 in a case of acute myelocytic leukemia.

Acute myelocytic leukemia (AML) is a malignant disease characterized by the proliferation of immature myelocytic precursor cells causing the disruption of normal bone marrow function. Many chromosomal aberrations have been described in AML including translocations, inversions, deletions, and additions. Here we describe a novel deletion of chromosome 1, del(1)(p34p36) in a case of AML, French-American-British classification M1, in a previously healthy 33-year-old male. This isolated cytogenetic abnormality occurred in 33% of the myeloblasts examined at diagnosis. Subsequent cytogenetic analyses conducted on marrow following induction and consolidation therapy demonstrated a normal male karyotype in all cells examined. The patient remains in clinical and hematological remission 22 months following diagnosis. The presence of 1p abnormalities in AML and other malignancies is reviewed, as are candidate tumor suppressor genes in the 1p34 approximately p36 region. The implications of chromosome 1p abnormalities on clinical outcome are also discussed.

An image reconstruction algorithm for three-dimensional electrical impedance tomography.

Electrical impedance tomography (EIT) has been studied by many authors and in most of this work it has been considered to be a two-dimensional problem. Many groups are now turning their attention to the full three-dimensional case in which the computational demands become much greater. It is interesting to look for ways to reduce this demand and in this paper we describe an implementation of an algorithm that is able to achieve this by precomputing many of the quantities needed in the image reconstruction. The algorithm is based on a method called NOSER introduced some years ago by Cheney et al. [3]. In this paper we have significantly extended the method by introducing a more realistic electrode model into the analysis. We have given explicit formulae for the quantities involved so that the reader can reproduce our results.

A one step image reconstruction algorithm for electrical impedance tomography in three dimensions.

The move from two to three dimensions in the study of electrical impedance tomography (EIT) has generated a great increase in computational demands. It is therefore interesting to investigate ways in which this demand can be reduced, and in this paper we have presented some results of one such approach. The NOSER algorithm was introduced some years ago and we have extended it to include more realistic electrode models. The main feature of the method is that by starting from a uniform conductivity distribution many quantities can be pre-calculated.

Some boundary problems in electrical impedance tomography.

Accurate mathematical modelling is important in the development of iterative image reconstruction algorithms for electrical impedance tomography (EIT). In such schemes the forward problem of calculating the electric potential from Neumann boundary data is solved many times. One aspect of this problem which has received some attention is the mathematical modelling of the electrodes used in the technique. In this paper we describe an integral equation formulation of a boundary value problem associated with this tissue and we indicate some of the ways in which this formulation can be used to obtain numerical and analytic results.

Singularities of mixed boundary value problems in electrical impedance tomography.

The importance of accurate mathematical modelling in the development of image reconstruction algorithms for electrical impedance tomography (EIT) has been discussed in a number of recent papers. It is particularly important in iterative reconstruction schemes where the forward problem of calculating the electric potential from Neumann boundary data is solved many times. One area which needs to be considered it the mathematical modelling of the electrodes used in the technique. In this paper we discuss one of the more sophisticated models which has been proposed and present the results of a number of numerical and analytic calculations which we have made as a contribution to the understanding of this question.

Extramedullary hemopoiesis arising in the gut mimicking carcinoma of the cecum.

A patient initially treated for iron deficiency anemia was later found to have myelofibrosis. She developed increasing splenomegaly and then a mass in the right iliac fossa for which a right hemicolectomy was performed. Histopathology demonstrated extramedullary hemopoiesis in the spleen and cecum. The patient subsequently developed a protein-losing enteropathy and she deteriorated and died 14 mths later. Autopsy was not performed.

An adaptive current tomography using voltage sources.

This paper describes the development of an adaptive electric current tomography system which contains a novel front-end analog architecture. Programmable voltage sources have been used to deliver currents into the study object and to avoid the difficulties of obtaining high quality current sources. Through inverting an admittance matrix, the system is capable of achieving a desired current drive pattern by applying a computed voltage pattern. The tomograph, operating at 9.6 kHz, comprises 32 driving electrodes and 32 voltage measurement electrodes. The study of system noise performance shows high SNR in the data acquisition which is enhanced by a digital demodulation scheme. In vitro reconstruction images have been obtained with the data collected by the tomograph.

Optimal experiments in electrical impedance tomography.

Electrical impedance tomography (EIT) is a noninvasive imaging technique which aims to image the impedance within a body from electrical measurements made on the surface. The reconstruction of impedance images is a ill-posed problem which is both extremely sensitive to noise and highly computationally intensive. The authors define an experimental measurement in EIT and calculate optimal experiments which maximize the distinguishability between the region to be imaged and a best-estimate conductivity distribution. These optimal experiments can be derived from measurements made on the boundary. The analysis clarifies the properties of different voltage measurement schemes. A reconstruction algorithm based on the use of optimal experiments is derived. It is shown to be many times faster than standard Newton-based reconstruction algorithms, and results from synthetic data indicate that the images that it produces are comparable.

A hybrid phantom for electrical impedance tomography.

One of the design considerations for electrical impedance tomography phantoms is that they must be easy to model accurately. This paper describes a phantom with this property. Experimental results from its evaluation and testing are given.

Data errors and reconstruction algorithms in electrical impedance tomography.

In electrical impedance tomography the reconstruction problem is a non-linear inverse problem and can only be solved by iterative methods. This paper describes two such algorithms, one based on the regularised Newton's method of Levenburg and Marquardt, and a second modified version of this algorithm which uses optimal current drive patterns. The second algorithm is shown to give superior reconstruction in a simulation study. Some effects of errors in the knowledge of boundary shape and electrode position are also discussed.