Roifman syndrome: a description of further immunological and radiological features.

Roifman syndrome is a rare autosomal recessive inherited syndromic immunodeficiency. We wish to add to the available literature by reporting two brothers with clinical, radiological and immunological features of Roifman syndrome, confirmed on whole exome sequencing. We report an excellent response to subcutaneous immunoglobulin therapy in both brothers, reducing infection burden and hospital admissions. New radiological features are also described here which may assist in the diagnosis of other patients.

Low-dose CT imaging of the acute abdomen using model-based iterative reconstruction: a prospective study.

OBJECTIVES: Performance of a modified abdominopelvic CT protocol reconstructed using full iterative reconstruction (IR) was assessed for imaging patients presenting with acute abdominal symptoms. MATERIALS AND METHODS: Fifty-seven patients (17 male, 40 female; mean age of 56.5 ± 8 years) were prospectively studied. Low-dose (LD) and conventional-dose (CD) CTs were contemporaneously acquired between November 2015 and March 2016. The LD and CD protocols imparted radiation exposures approximating 10-20% and 80-90% those of routine abdominopelvic CT, respectively. The LD images were reconstructed with model-based iterative reconstruction (MBIR), and CD images with hybrid IR (40% adaptive statistical iterative reconstruction (ASIR)). Image quality was assessed quantitatively and qualitatively. Independent clinical interpretations were performed with a 6-week delay between reviews. RESULTS: A 74.7% mean radiation dose reduction was achieved: LD effective dose (ED) 2.38 ± 1.78 mSv (size-specific dose estimate (SSDE) 3.77 ± 1.97 mGy); CD ED 7.04 ± 4.89 mSv (SSDE 10.74 ± 5.5 mGy). LD-MBIR images had significantly lower objective and subjective image noise compared with CD-ASIR (p < 0.0001). Noise reduction for LD-MBIR studies was greater for patients with BMI < 25 kg/m2 than those with BMI ≥ 25 kg/m2 (5.36 ± 3.2 Hounsfield units (HU) vs. 4.05 ± 3.1 HU, p < 0.0001). CD-ASIR studies had significantly better contrast resolution, and diagnostic acceptability (p < 0.0001 for all). LD-MBIR studies had significantly lower streak artifact (p < 0.0001). There was no difference in sensitivity for primary findings between the low-dose and conventional protocols with the exception of one case of enteritis. CONCLUSIONS: Low-dose abdominopelvic CT performed with MBIR is a feasible radiation dose reduction strategy for imaging patients presenting with acute abdominal pain.

A comparison of embalming fluids on the structures and properties of tissue in human cadavers.

Cadaveric material has long been used to teach anatomy and more recently to train students in clinical skills. The aim of this study was to develop a systematic approach to compare the impact of four embalming solutions on the tissues of human cadavers. To this end, a formalin-based solution, Thiel, Genelyn and Imperial College London soft-preservation (ICL-SP) solution were compared. The effect of these chemicals on the properties of the tissue was assessed by measuring the range of motion (ROM) of joints and measuring the dimensions of different structures on computed tomography (CT) images before and after embalming. The mean changes in the ratio (angle to ROM) differed statistically between embalming methods (Welch Statistic 3,1.672  = 67.213, p = 0.026). Thiel embalmed cadavers showed an increase in range of motion while ICL-SP cadavers remained relatively the same. Genelyn and formalin embalmed cadavers registered a notable decrease in range of motion. Furthermore, investigation into the impact of the embalming chemicals on the dimensions of internal organs and vessels revealed that Thiel embalming technique leads to a decrease in the dimension of the cardiovascular system alone while formalin-based solutions maintain the shape of the organs and vessels investigated. Our findings suggest that the joints of cadavers' embalmed using ICL-SP technique may faithfully mimic that of unembalmed cadavers and that formalin is necessary to retain shape and size of the organs and vessels investigated in this study. Despite this, a study with larger numbers of cadavers is required to confirm these findings.

Sarcopenia and Post-Operative Morbidity and Mortality in Patients with Gastric Cancer.

PURPOSE: Surgical resection for gastric adenocarcinoma is associated with significant post-operative morbidity and mortality. The aim of this study was to assess the prognostic significance of sarcopenia in patients undergoing resection for gastric adenocarcinoma with respect to post-operative morbidity and survival. MATERIALS AND METHODS: A retrospective analysis was conducted on a cohort of consecutive patients who underwent surgical resection for gastric adenocarcinoma between 2008 and 2014. Patient demographics, radiological parameters, and pathological data were collected. OsiriX software (Pixmeo) was used to measure skeletal muscle area, which was normalized for height to calculate skeletal muscle index. RESULTS: A total of 56 patients (41 male, 15 female; mean age, 68.4 ± 11.9 years) met the inclusion criteria. Of these, 36% (20 of 56) of the patients were sarcopenic pre-operatively. Both sarcopenic and non-sarcopenic patient groups were equally matched with the exception of weight and body mass index (P=0.036 and 0.001, respectively). Sarcopenia was associated with a decreased overall survival (log-rank P=0.003) and was an adverse prognostic predictor of overall survival in multivariate analysis (hazard ratio, 10.915; P=0.001). Sarcopenia was a predictor of serious in-hospital complications in multivariate analysis (odds ratio, 3.508; P=0.042). CONCLUSIONS: In patients undergoing curative resection for gastric cancer, there was a statistically significant association between sarcopenia and both decreased overall survival and serious post-operative complications. The measurement and reporting of skeletal muscle index on pre-operative computed tomography should be considered.

Tissue and Bronchoalveolar Lavage Biomarkers in Idiopathic Pulmonary Fibrosis Patients on Pirfenidone.

BACKGROUND: Pirfenidone is a novel anti-fibrotic agent in idiopathic pulmonary fibrosis with proven clinical benefit. Better human tissue models to demonstrate the immunomodulatory and anti-fibrotic effect of pirfenidone are required. OBJECTIVES: The purpose of the study was to use transbronchial lung cryobiopsy (TBLC), a novel technique which provides substantial tissue samples, and a large panel of biomarkers to temporally assess disease activity and response to pirfenidone therapy. METHODS: Thirteen patients with confirmed idiopathic pulmonary fibrosis (IPF) underwent full physiological and radiological assessment at diagnosis and after 6-month pirfenidone therapy. They underwent assessment for a wide range of potential serum and bronchoalveolar lavage biomarkers of disease activity. Finally, they underwent TBLC before and after treatment. Tissue samples were assessed for numbers of fibroblast foci, for Ki-67, a marker of tissue proliferation and caspase-3, a marker of tissue apoptosis. RESULTS: All patients completed treatment and investigations without significant incident. There was no significant fall in number of fibroblast foci per unit tissue volume after treatment (pre-treatment: 0.14/mm2 vs. post-treatment 0.08/mm2, p = 0.1). Likewise, there was no significant change in other markers of tissue proliferation, Ki-67 or Caspase-3 with pirfenidone treatment. We found an increase in three bronchoalveolar lavage angiogenesis cytokines, Placental Growth Factor, Vascular Endothelial Growth Factor-A, and basic Fibroblast Growth Factor, two anti-inflammatory cytokines Interleukin-10 and Interleukin-4 and Surfactant Protein-D. CONCLUSIONS: TBLC offers a unique opportunity to potentially assess the course of disease activity and response to novel anti-fibrotic activity in IPF.

Determination of a suitable low-dose abdominopelvic CT protocol using model-based iterative reconstruction through cadaveric study.

INTRODUCTION: Cadaveric studies provide a means of safely assessing new technologies and optimizing scanning prior to clinical validation. Reducing radiation exposure in a clinical setting can entail incremental dose reductions to avoid missing important clinical findings. The use of cadavers allows assessment of the impact of more substantial dose reductions on image quality. Our aim was to identify a suitable low-dose abdominopelvic CT protocol for subsequent clinical validation. METHODS: Five human cadavers were scanned at one conventional dose and three low-dose settings. All scans were reconstructed using three different reconstruction algorithms: filtered back projection (FBP), hybrid iterative reconstruction (60% FBP and 40% adaptive statistical iterative reconstruction (ASIR40)), and model-based iterative reconstruction (MBIR). Two readers rated the image quality both quantitatively and qualitatively. RESULTS: Model-based iterative reconstruction images had significantly better objective image noise and higher qualitative scores compared with both FBP and ASIR40 images at all dose levels. The greatest absolute noise reduction, between MBIR and FBP, of 34.3 HU (equating to a 68% reduction) was at the lowest dose level. MBIR reduced image noise and improved image quality even in CT images acquired with a mean radiation dose reduction of 62% compared with conventional dose studies reconstructed with ASIR40, with lower levels of objective image noise, superior diagnostic acceptability and contrast resolution, and comparable subjective image noise and streak artefact scores. CONCLUSION: This cadaveric study demonstrates that MBIR reduces image noise and improves image quality in abdominopelvic CT images acquired with dose reductions of up to 62%.

C-reactive protein and radiographic findings of lower respiratory tract infection in infants.

AIM: To evaluate the association between C-reactive protein (CRP) and radiological evidence of lower respiratory tract infection (LRTI) in infants. METHODS: All patients aged less than 4 years who presented with suspected lower respiratory tract infection, who received a peri-presentation chest radiograph and CRP blood measurement over an 18-mo period were included in the study. Age, gender, source of referral, CRP, white cell count, neutrophil count along with the patients' symptoms and radiologist's report were recorded. RESULTS: Three hundred and eleven patients met the inclusion criteria. Abnormal chest radiographs were more common in patients with elevated CRP levels (P < 0.01). Radiologic signs of LRTI were identified in 73.7% of chest radiographs when a patient had a CRP level between 50-99 mg/L. CRP levels were a better predictor of positive chest radiograph findings for those aged greater than I year compared to those 1 year or less. CONCLUSION: CRP may be used in patients with suspected LRTI diagnosis to select those who are likely to have positive findings on chest radiograph, thus reducing unnecessary chest radiographs.

CT of the head for acute stroke: Diagnostic performance of a tablet computer prior to intravenous thrombolysis.

INTRODUCTION: The authors propose that tablet computers could benefit patients with acute stroke in the remote care setting, where time to and accuracy of CT interpretation greatly influences patient outcome. METHODS: One hundred and fifty consecutive patients who presented to the Emergency Department of a tertiary referral neurosciences centre within a time window suitable for intravenous thrombolytic therapy were included. Images were wirelessly transmitted to a tablet computer (iPad 3rd Generation, model = A1430, Apple, Cupertino, CA) and were reviewed by radiologists with three levels of experience for signs of intracranial haemorrhage, large vessel occlusion and parenchymal infarction. Reference standard interpretation was performed by two neuroradiologists using a diagnostic monochrome display. RESULTS: Consensus neuroradiologist review on the tablet display found and correctly classified all of the 23 cases of intracranial haemorrhage including 21 cases of parenchymal haematoma, two cases of petechial haemorrhage and one patient with an acute subdural haematoma. Less experienced readers missed cases of petechial and subdural haematomas. There was excellent agreement between the tablet and diagnostic monochrome display in cases with no infarct or extensive parenchymal infarction. CONCLUSIONS: Tablet computers can be used to facilitate rapid preliminary CT interpretation in patients with acute stroke in the remote setting.

Accurate tissue characterization in low-dose CT imaging with pure iterative reconstruction.

INTRODUCTION: We assess the ability of low-dose hybrid iterative reconstruction (IR) and 'pure' model-based IR (MBIR) images to maintain accurate Hounsfield unit (HU)-determined tissue characterization. METHODS: Standard-protocol (SP) and low-dose modified-protocol (MP) CTs were contemporaneously acquired in 34 Crohn's disease patients referred for CT. SP image reconstruction was via the manufacturer's recommendations (60% FBP, filtered back projection; 40% ASiR, Adaptive Statistical iterative Reconstruction; SP-ASiR40). MP data sets underwent four reconstructions (100% FBP; 40% ASiR; 70% ASiR; MBIR). Three observers measured tissue volumes using HU thresholds for fat, soft tissue and bone/contrast on each data set. Analysis was via SPSS. RESULTS: Inter-observer agreement was strong for 1530 datapoints (rs > 0.9). MP-MBIR tissue volume measurement was superior to other MP reconstructions and closely correlated with the reference SP-ASiR40 images for all tissue types. MP-MBIR superiority was most marked for fat volume calculation - close SP-ASiR40 and MP-MBIR Bland-Altman plot correlation was seen with the lowest average difference (336 cm3 ) when compared with other MP reconstructions. CONCLUSIONS: Hounsfield unit-determined tissue volume calculations from MP-MBIR images resulted in values comparable to SP-ASiR40 calculations and values that are superior to MP-ASiR images. Accuracy of estimation of volume of tissues (e.g. fat) using segmentation software on low-dose CT images appears optimal when reconstructed with pure IR.

Effects of oral contrast on dose in abdominopelvic computed tomography with pure iterative reconstruction.

AIM: To assess the effect of neutral (NC) and positive (PC) oral contrast use on patient dose in low-dose abdominal computed tomography (CT). METHODS: Low-dose clinically indicated CTs were performed on 79 Crohn's patients (35 = PC, 1 L 2% gastrografin; 44 = NC, 1.5 L polyethylene glycol). Scanner settings for both acquisitions were identical apart from 25 s difference in intravenous contrast timing. Body mass index (BMI), scan-ranges, dose-length product and size-specific dose estimated were recorded. Data was reconstructed with pure model-based iterative reconstruction. Image quality was objectively and subjectively analysed. Data analysis was performed with Statistical Package for Social Scientists. RESULTS: Higher doses were seen in neutral contrast CTs (107.60 ± 78.7 mGy.cm, 2.47 ± 1.21 mGy vs 85.65 ± 58.2 mGy.cm, 2.18 ± 0.96 mGy). The difference was significant in 2 of 4 BMI groups and in those that had both NC and PC investigations. Image-quality assessment yielded 6952 datapoints. NC image quality was significantly superior (P < 0.001) (objective noise, objective signal to noise ratio, subjective spatial resolution, subjective contrast resolution, diagnostic acceptability) at all levels. NC bowel distension was significantly (P < 0.001) superior. CONCLUSION: The use of polyethylene glycol as a neutral OC agent leads to higher radiation doses than standard positive contrast studies, in low dose abdominal CT imaging. This is possibly related to the osmotic effect of the agent resulting in larger intraluminal fluid volumes and resultant increased overall beam attenuation.

Low-Dose Carotid Computed Tomography Angiography Using Pure Iterative Reconstruction.

UNLABELLED: The aim of this study was to assess if a low-dose carotid computed tomography angiography (CTA) performed with pure iterative reconstruction (IR) is comparable to a conventional dose CTA protocol. METHODS: Twenty patients were included. Radiation dose was divided into a low-dose acquisition reconstructed with pure IR and a conventional dose acquisition reconstructed with 40% hybrid IR. Dose, image noise, contrast resolution, spatial resolution, and carotid artery stenosis were measured. RESULTS: Mean effective dose was significantly lower for low-dose than conventional dose studies (1.84 versus 3.71 mSv; P < 0.001). Subjective image noise, contrast resolution, and spatial resolution were significantly higher for the low-dose studies. There was excellent agreement for stenosis grading accuracy between low- and conventional dose studies (Cohen κ = 0.806). CONCLUSIONS: A low-dose carotid CTA protocol reconstructed with pure IR is comparable to a conventional dose CTA protocol in terms of image quality and diagnostic accuracy while enabling a dose reduction of 49.6%.

Computed tomography dose optimisation in cystic fibrosis: A review.

Cystic fibrosis (CF) is the most common autosomal recessive disease of the Caucasian population worldwide, with respiratory disease remaining the most relevant source of morbidity and mortality. Computed tomography (CT) is frequently used for monitoring disease complications and progression. Over the last fifteen years there has been a six-fold increase in the use of CT, which has lead to a growing concern in relation to cumulative radiation exposure. The challenge to the medical profession is to identify dose reduction strategies that meet acceptable image quality, but fulfil the requirements of a diagnostic quality CT. Dose-optimisation, particularly in CT, is essential as it reduces the chances of patients receiving cumulative radiation doses in excess of 100 mSv, a dose deemed significant by the United Nations Scientific Committee on the Effects of Atomic Radiation. This review article explores the current trends in imaging in CF with particular emphasis on new developments in dose optimisation.

Cumulative radiation exposure from diagnostic imaging in intensive care unit patients.

AIM: To quantify cumulative effective dose of intensive care unit (ICU) patients attributable to diagnostic imaging. METHODS: This was a prospective, interdisciplinary study conducted in the ICU of a large tertiary referral and level 1 trauma center. Demographic and clinical data including age, gender, date of ICU admission, primary reason for ICU admission, APACHE II score, length of stay, number of days intubated, date of death or discharge, and re-admission data was collected on all patients admitted over a 1-year period. The overall radiation exposure was quantified by the cumulative effective radiation dose (CED) in millisieverts (mSv) and calculated using reference effective doses published by the United Kingdom National Radiation Protection Board. Pediatric patients were selected for subgroup-analysis. RESULTS: A total of 2737 studies were performed in 421 patients. The total CED was 1704 mSv with a median CED of 1.5 mSv (IQR 0.04-6.6 mSv). Total CED in pediatric patients was 74.6 mSv with a median CED of 0.07 mSv (IQR 0.01-4.7 mSv). Chest radiography was the most commonly performed examination accounting for 83% of all studies but only 2.7% of total CED. Computed tomography (CT) accounted for 16% of all studies performed and contributed 97% of total CED. Trauma patients received a statistically significant higher dose [median CED 7.7 mSv (IQR 3.5-13.8 mSv)] than medical [median CED 1.4 mSv (IQR 0.05-5.4 mSv)] and surgical [median CED 1.6 mSv (IQR 0.04-7.5 mSv)] patients. Length of stay in ICU [OR = 1.12 (95%CI: 1.079-1.157)] was identified as an independent predictor of receiving a CED greater than 15 mSv. CONCLUSION: Trauma patients and patients with extended ICU admission times are at increased risk of higher CEDs. CED should be minimized where feasible, especially in young patients.

Pure Iterative Reconstruction Improves Image Quality in Computed Tomography of the Abdomen and Pelvis Acquired at Substantially Reduced Radiation Doses in Patients With Active Crohn Disease.

UNLABELLED: We assessed diagnostic accuracy and image quality of modified protocol (MP) computed tomography (CT) of the abdomen and pelvis reconstructed using pure iterative reconstruction (IR) in patients with Crohn disease (CD). METHODS: Thirty-four consecutive patients with CD were referred with suspected extramural complications. Two contemporaneous CT datasets were acquired in all patients: standard protocol (SP) and MP. The MP and SP protocols were designed to impart radiation exposures of 10% to 20% and 80% to 90% of routine abdominopelvic CT, respectively. The MP images were reconstructed with model-based IR (MBIR) and adaptive statistical IR (ASIR). RESULTS: The MP-CT and SP-CT dose length product were 88 (58) mGy.cm (1.27 [0.87] mSv) and 303 [204] mGy.cm (4.8 [2.99] mSv), respectively (P < 0.001). Median diagnostic acceptability, spatial resolution, and contrast resolution were significantly higher and subjective noise scores were significantly lower on SP-ASIR 40 compared with all MP datasets. There was perfect clinical agreement between MP-MBIR and SP-ASIR 40 images for detection of extramural complications. CONCLUSIONS: Modified protocol CT using pure IR is feasible for assessment of active CD.

Computed tomography and patient risk: Facts, perceptions and uncertainties.

Since its introduction in the 1970s, computed tomography (CT) has revolutionized diagnostic decision-making. One of the major concerns associated with the widespread use of CT is the associated increased radiation exposure incurred by patients. The link between ionizing radiation and the subsequent development of neoplasia has been largely based on extrapolating data from studies of survivors of the atomic bombs dropped in Japan in 1945 and on assessments of the increased relative risk of neoplasia in those occupationally exposed to radiation within the nuclear industry. However, the association between exposure to low-dose radiation from diagnostic imaging examinations and oncogenesis remains unclear. With improved technology, significant advances have already been achieved with regards to radiation dose reduction. There are several dose optimization strategies available that may be readily employed including omitting unnecessary images at the ends of acquired series, minimizing the number of phases acquired, and the use of automated exposure control as opposed to fixed tube current techniques. In addition, new image reconstruction techniques that reduce radiation dose have been developed in recent years with promising results. These techniques use iterative reconstruction algorithms to attain diagnostic quality images with reduced image noise at lower radiation doses.

Comparison of MRI and high-resolution transvaginal sonography for the local staging of cervical cancer.

PURPOSE: The aim of this prospective study was to compare the diagnostic accuracy of transvaginal sonography (TVS) with that of MRI in the local staging of cervical cancer. METHODS: All consecutive patients diagnosed with invasive carcinoma of the cervix over a 3-year period underwent MRI of the pelvis and TVS according to a standardized protocol. RESULTS: In total, 46 patients were recruited to the study. We found a strong correlation between MRI and TVS in the assessment of tumor volume in both early-stage and advanced-stage disease (p < 0.0001). Both MRI and TVS had a sensitivity of 80%, a specificity of 50%, and a diagnostic accuracy of 63.6% for the detection of stromal invasion in early-stage disease. For the detection of parametrial invasion, we found sensitivity rates of 40% for MRI and 86% for TVS; specificity rates of 78.8% for MRI and 20% for TVS; and diagnostic accuracy rates of 89% for MRI and 78.7% for TVS. A matched-sample analysis revealed that there was no statistically significant difference between MRI and TVS in the assessment of stromal or parametrial invasion (p = 0.06). CONCLUSIONS: TVS performed by a dedicated gynecologic radiologist is a feasible and economic imaging modality with a diagnostic accuracy comparable to that of MRI. It may be used as an adjunct to MRI for the local staging of invasive cervical cancer or to allow for rapid and confident triage of patients into operative and nonoperative categories for management in the gynecologic outpatient setting.

Efficacy and Safety of Rituximab in Connective Tissue Disease related Interstitial Lung Disease.

BACKGROUND: Pulmonary complications of connective tissue disease are being identified more frequently with the advent of more sophisticated radiological investigations. Limited previous studies have suggested Rituximab (RTX), a chimeric monoclonal antibody with activity against CD-20, may benefit connective tissue disease patients with pulmonary complications. We performed a retrospective analysis of the efficacy and safety of RTX in patients attending a tertiary referral centre. METHODS: Ten patients treated with RTX for pulmonary complications of CTD in our institution were identified. Baseline demographics, pre- and post-treatment investigations and adverse events were documented with an average follow up time-frame of 12.3 months (range: 3 - 27). Statistical analysis was performed using the Wilcoxan Signed-Rank test in SPSS. RESULTS: There was a statistically significant improvement in pulmonary function, with a mean increase of 19% in DLCO (median DLCO (ml/min/mmHg) pre-treatment vs. post-treatment: 13.94 vs. 19.34, p=0.028) and a mean increase of 13% in FVC (median FVC (L) pre-treatment vs. post-treatment: 3.47 vs.3.6, p=0.28). For patients with pulmonary fibrosis (n=7), CT severity was improved on post-treatment scan, though this did not reach statistical significance. There was a reduction in the number of nodules seen on the follow-up scans of two patients without fibrosis. No patient had a severe adverse reaction to RTX. CONCLUSIONS: Treatment with RTX resulted in an objective, measurable improvement in pulmonary function and/or radiological severity for the majority of patients included in the series. This was statistically significant despite the small numbers included. These results indicate a positive response to RTX with few complications of treatment.

Biomarkers to identify ILD and predict lung function decline in scleroderma lung disease or idiopathic pulmonary fibrosis.

BACKGROUND: SSc-ILD and IPF demonstrate significant morbidity and mortality. Predicting disease progression is challenging in both diseases. OBJECTIVES: We sought a serum biomarker that could identify patients with SSc-ILD or IPF and prospectively predict short-term decline in lung function in these patients. METHODS: 10 healthy controls, 5 SSc w/o ILD, 6 SSc-ILD and 13 IPF patients underwent venesection. An array of cytokines including KL-6, SP-D and MMP7 were measured. PFTs were obtained at baseline and six months. Cytokine measurements were correlated with PFTs. RESULTS: KL-6 in IPF patients (633 ng/ml, IQR 492-1675) was significantly elevated compared to controls (198 ng/ml, IQR 52-360, p<0.01) and SSc w/o ILD patients (192 ng/ml, IQR 0-524, p<0.05); KL-6 in SSc-ILD patients (836 ng/ml, IQR 431-1303) was significantly higher than in controls (p<0.05). SP-D was significantly higher in IPF patients (542 ng/ml, IQR 305-577) compared to controls (137 ng/ml, IQR 97-284, p<0.01) or to SSc w/o ILD patients (169 ng/ml, IQR 137-219, p<0.05). In comparison with controls (0.0 ng/ml, IQR 0.0-0.6), MMP7 was significantly higher in both IPF patients (2.85 ng/ml, IQR 1.5-3.6, p<0.05) and SSc-ILD patients (5.41 ng/ml, IQR 2.6-7.2, p<0.001). Using a cut-off level of 459ng/ml for KL-6 and of 1.28 ng/ml for MMP7, 18 out of 19 patients with ILD had a serum value of either KL-6 or MMP7 above these thresholds. For all ILD patients, baseline serum SP-D correlated with ΔFVC %pred over six months (r=-0.63, p=0.005, 95% CI -0.85 to -0.24). CONCLUSIONS: Combining KL-6 with MMP7 may be a useful screening tool for patients at risk of ILD. SP-D may predict short-term decline in lung function.

Haematuria: an imaging guide.

This paper discusses the current status of imaging in the investigation of patients with haematuria. The physician must rationalize imaging so that serious causes such as malignancy are promptly diagnosed while at the same time not exposing patients to unnecessary investigations. There is currently no universal agreement about the optimal imaging work up of haematuria. The choice of modality to image the urinary tract will depend on individual patient factors such as age, the presence of risk factors for malignancy, renal function, a history of calculus disease and pregnancy, and other factors, such as local policy and practice, cost effectiveness and availability of resources. The role of all modalities, including conventional radiography, intravenous urography/excretory urography, ultrasonography, retrograde pyelography, multidetector computed tomography urography (MDCTU), and magnetic resonance urography, is discussed. This paper highlights the pivotal role of MDCTU in the imaging of the patient with haematuria and discusses issues specific to this modality including protocol design, imaging of the urothelium, and radiation dose. Examination protocols should be tailored to the patient while all the while optimizing radiation dose.

Castleman disease: an unexpected cause of a solitary pleural mass.

Castleman disease (CD) is a rare benign lymphoproliferative disorder, the etiology of which is unclear. Clinically it may manifest as localized disease (unicentric) or disseminated disease (multicentric). CD occurs in the thorax in 70% of cases, abdomen and pelvis in 15%, and in the neck in 10-15% of cases. We present a case of a pleural mass located posteriorly in a paraspinal location, which was discovered incidentally in a 50-year-old man and was subsequently resected followed by an unexpected diagnosis of Castleman disease on histological examination. In this report, we review the clinical and histological findings in a rare presentation of Castleman disease and discuss the findings in this case as part of an overall review of the typical radiological findings seen in Castleman disease.