Diagnostic Work-up Of Patients Research Articles

18F-FDG-PET/MRI in the diagnostic work-up of limbic encephalitis.

IntroductionLimbic encephalitis (LE) is an immune-related, sometimes paraneoplastic process of the central nervous system. Initial diagnosis and treatment are based on the clinical presentation as well as antibody profiles and MRI. This study investigated the diagnostic value of integrated 18F-FDG-PET/MRI in the diagnostic work-up of patients with LE for a cerebral and whole-body imaging concept.Material and methodsTwenty patients with suspected LE were enrolled in this prospective study. All patients underwent a dedicated PET/MRI protocol of the brain as well as the whole-body. Two neuroradiologists, one body radiologist and one nuclear medicine physician performed blinded consensus readings of each corresponding MRI and PET/MRI dataset of the brain and whole-body. Diagnostic confidence was evaluated on a Likert scale.ResultsBased on integrated PET/MRI 19 / 20 patients were found to show morphologic and / or metabolic changes indicative of LE, whereas sole MRI enabled correct identification in 16 / 20 patients. Three patients with negative MRI showed metabolic changes of the limbic system or extra-limbic regions, shifting the diagnosis from (negative) MRI to positive for LE in PET/MRI. Whole-body staging revealed suspected lesions in 2/20 patients, identified by MRI and PET, one confirmed as malignant and one false positive. Diagnostic confidence for cerebral and whole-body imaging reached higher scores for PET/MRI (cerebral: 2.7 and whole body: 4.8) compared to MRI alone (cerebral: 2.4 and whole body: 4.5).ConclusionLE diagnosis remains challenging for imaging as it shows only subtle imaging findings in most patients. Nevertheless, based on the simultaneous and combined analysis of morphologic and metabolic data, integrated PET/MRI may enable a dual platform for improved diagnostic confidence and overall detection of LE as well as whole-body imaging for exclusion of paraneoplastic LE.

Endoscopic ultrasound underestimates tumor size in patients with pancreatic head adenocarcinoma

Presenter: Joseph Reza MD | AdventHealth Orlando Background: Tumor sizing has prognostic consequences in patients with pancreatic cancer, as larger tumors tend to invade vital structures impeding resection and are associated with higher rates of metastatic lymphadenopathies. Endoscopic ultrasound (EUS) plays a crucial role in the staging of these patients, but findings are directly dependent on the experience of the operator and tumor location. In this study, we compared preoperative tumor sizing measured by EUS to size on pathological specimens in patients that underwent curative-intended resections. Methods: A retrospective review of patients with biopsy proven adenocarcinoma of the head of the pancreas that underwent resection at a tertiary center was performed. Basic demographics were recorded. Maximal tumor dimensions determined by EUS were compared to those found on final pathological reports. Analysis was performed with Pearson correlation test using SAS studio. Results: A total of 97 patients underwent a pancreaticoduodenectomy during the study period. Patients that underwent neoadjuvant therapy for borderline resectable or locally advanced cancer were excluded from the analysis. From the remaining group, 56 patients underwent upfront surgery for resectable disease. Most patients were males, whites, with an average age at diagnosis of 69 years. The total number of harvested lymph nodes was 15 with a positivity rate of 82%; 77% of patients had R0 resections. The average maximal dimension of the tumor calculated by EUS was 2.45 cm, compared to 3.11 cm seen on pathological reports (Pearson correlation coefficient 0.45400, p=0.0004). Conclusion: Endoscopic ultrasonography is commonly used in the diagnostic workup of patients with pancreatic cancer. Based on our results, determination of tumor sizing based on EUS has the potential to under-stage patients with pancreatic cancer.

LncRNA TUG1 Expression in NSCLC and Its Clinical Significance.

This study was aimed at exploring the expression of lncRNA TUG1 in non-small cell lung cancer (NSCLC) and analyzing the correlations between TUG1 expression and an NSCLC patient's clinical and pathological parameters and prognosis. This study included 132 NSCLC patients who were admitted between January 2012 and May 2013 in our hospital. Expression levels of TUG1 expression in the resected cancer tissue and normal adjacent tissue (NAT) were assessed using the ISH and RT-qPCR assays to analyze the correlations between TUG1 expression in NSCLC tissue and an NSCLC patient's clinical and pathological parameters and prognosis. Compared to NAT, NSCLC tissue has a lower expression level of TUG1. The TUG1 expression further decreases as NSCLC progressed to a later stage, indicating a statistically significant difference (p < 0.01 or p < 0.001). High TUG1 expression is not strongly associated with age, gender, smoking history, or the degree of differentiation of NSCLC (p > 0.05) but exhibits close correlations with tumor size, TNM stage, and lymph node metastasis (p < 0.05). Patients with a higher level of TUG1 expression have a higher survival rate and a longer survival time than those with lower TUG1 expression. The inter-group differences were statistically significant (p < 0.05). Considering TUG1 presence in the development and progression of NSCLC, hopefully, it can be used as a tumor marker in the diagnostic workup of patients with NSCLC.

P319 Evidence for abnormal deformational reserve in hypertrophic cardiomyopathy on exercise: is it a game-changer?

Abstract Funding Acknowledgements None Hypertrophic obstructive cardiomyopathy (HOCM) is characterized by muscle hypertrophy and fibrosis, interfering with force generation and relaxation. Abnormal ventricular (LV) myocardial deformation have been demonstrated in patients with HOCM at rest, but there is lack of data regarding the deformational mechanics in exercise. AIMS: We wanted to assess the adaptability of LV deformational behaviour to physical exercise in HCM patients as compared to healthy control subjects. METHODS 24 obstructive HOCM (age 51.2 ± 14.2yrs; 16 men, LVOT-obstruction 56 ± 19mmHg at rest or on Valsalva maneuver) and 32 control subjects (50.9 ± 6.8 yrs, 19 men from the MAGYAR-PATH Registry) underwent supine bicycle stress echocardiography (ESE) with measurements of 2D- and Doppler, 2D-Speckle Tracking Tracking Imaging and 3D-Full Volume Analysis. Beyond conventional LV/RV functional measurements; peak longitudinal (LS), circumferential (CS) Strain values; peak Twist and Torsion angles; post-systolic shortening index (PSS) and diastolic phase indices (Untwisting time - UTT and rate - UTR) calculated; the UTR/Twist ratio was given as "coupling index". The LV/RV-EF calculated by 3D-Full Volume Analysis off-line by TomTec Arena™ software both at rest and on submaximal ESE. RESULTS The HOCM group had lower resting LS (-14.6 ± 4.5 vs 18.4 ± 2.6%, p &amp;lt; 001) but higher CS (-32.9 ± 5.1 vs 28.8 ± 2.3%, p &amp;lt; 0.001) and Twist angles (9.9 ± 2.6 vs 6.1 ± 2.2º, p &amp;lt; 0.01) than control subjects. Exercise induced an increase in all strains in control subjects, but much less in HOCM (LS: -21.4 ± 3.5 vs 15.1 ± 3.0% and CS: -33.9 ± 3.6 vs 34.1 ± 4.2% in HOCM, p &amp;lt; 0.02 in controls, NS in HOCM); the increase of Twist angle was minimal in HOCM (Δ1.2 ± 1.2 vs Δ3.6 ± 2.3º in controls, p &amp;lt; 0.01). The PSS was more pronounced on ESE in HOCM than in controls (46.6 ± 12 vs 21.2 ± 9.6% in controls, p &amp;lt; 0.001). Peak UTR was slower (118 ± 2.1 vs 133.1 ± 14.1 º/s) during ESE and occured later (141 ± 19 vs 121 ± 9.1% of systolic time, p &amp;lt; 0.02) in HOCM than in controls. There was significant relationship between the Twist and UTR in control subjects (β=-0.0807, p &amp;lt; 0.001), but not in HOCM (β=-0.0046, p = 0.05). The UTR/Twist ratio diminished only in HOCM but not in controls (-8.0 ± 0.6 vs -13.1 ± 2.5 1/s, p &amp;lt; 0.02). CONCLUSIONS The HOCM patients had significantly impaired strain-adaptability; developed post-systolic shortening and no LV Torsional reserve was found on exercise. Also, I detected decreased and delayed UTR indices in the HOCM group. These findings support evidence for reduced systolic-diastolic coupling efficiency, assessed by Twist-Untwist mechanics in HOCM patients, which can contribute to the development of exercise-related symptoms and the dynamic LVOT-obstruction. This unique pattern of deformational behaviour to exercise can help in the differential diagnostic workup in patients with LV hypertrophy of unknown aetiology and also would hold additional value in the risk stratification process for patients with HCM-phenotypes.

Incidental findings during computed tomographic angiography diagnostic work-up in patients with arteriogenic erectile dysfunction.

To analyse the incidental findings during computed tomographic angiography (CTA) diagnostic work-up in patients with arteriogenic erectile dysfunction (ED). The medical records of all patients with suspected arteriogenic ED were entered into a database. Risk factors and underlying comorbidities were also collected. Pathological CTA findings were extracted from the CT readings and entered into the database. Incidental findings on CTA were classified as those requiring immediate medical treatment, requiring deferred medical treatment or of no clinical importance. A total of 200 patients underwent CTA for suspected arteriogenic ED. Mean patient age was 59.6 &plusmn; 11.7 years. Of these, 181 patients (90.5%) had obstructions of erection-related arteries. In 168 patients (84.0%), CTA showed multiple incidental pathological findings. Eighty-five of 200 patients (42.5%) exhibited incidental findings requiring immediate further medical workup and/or treatment: coronary artery calcification was diagnosed in 75/200 (37.5%), aorto-iliac aneurysms in 8/200 (4%) of patients and incidentally detected embolism in 1/200 patient. Pancreatic and liver tumours were less frequent (incidence 1.5% and 1%, respectively). Incidental findings requiring deferred medical workup and/or treatment were detected in 175/200 patients (87.5%). The findings with the highest prevalence were liver steatosis followed by colon diverticulosis and prostate hyperplasia. Findings of little to no clinical importance were reported in 117 (58.5%) patients. These included uncomplicated renal cysts, spinal degeneration and renal vascular anomalies. Almost every second patient presenting with ED had an incidental finding which required immediate treatment. Incidental findings not directly related to ED were common among patients undergoing CTA scans for suspected arterial obstructions. Coronary artery calcification was the leading finding requiring further medical workup and/or treatment. Thus, the benefit of CTA investigations extends beyond the anatomic description of arterial obstructions of erection-related arteries.

Cardiovascular Diseases And Psychiatric Disorders During The Diagnostic Workup Of Suspected Hematological Malignancy.

BackgroundLittle attention has been given to the risk of cardiovascular and psychiatric comorbidities during the clinical evaluation of a suspected hematological malignancy.MethodsBased on Skåne Healthcare Register, we performed a population-based cohort study of 1,527,449 individuals residing during 2005–2014 in Skåne, Sweden. We calculated the incidence rate ratios (IRRs) of cardiovascular diseases or psychiatric disorders during the diagnostic workup of 5495 patients with hematological malignancy and 18,906 individuals that underwent a bone marrow aspiration or biopsy or lymph node biopsy without receiving a diagnosis of any malignancy (“biopsied individuals”), compared to individuals without such experience (i.e., reference).ResultsThere was a higher rate of cardiovascular diseases during the diagnostic workup of patients with hematological malignancy (overall IRR, 3.3; 95% CI, 2.9 to 3.8; greatest IRR for embolism and thrombosis, 8.1; 95% CI, 5.2 to 12.8) and biopsied individuals (overall IRR, 4.9; 95% CI, 4.6 to 5.3; greatest IRR for stroke, 37.5; 95% CI, 34.1 to 41.2), compared to reference. Similarly, there was a higher rate of psychiatric disorders during the diagnostic workup of patients with hematological malignancy (IRR, 2.1; 95% CI, 1.5 to 2.8) and biopsied individuals (IRR, 3.1; 95% CI, 2.9 to 3.4). The rate increases were greater around the time of diagnosis or biopsy, compared to thereafter, for both outcomes.ConclusionThere were higher rates of cardiovascular diseases and psychiatric disorders during the diagnostic workup of a suspected hematological malignancy, regardless of the final diagnosis.

Quantification and discriminative power of 18F-FE-PE2I PET in patients with Parkinson's disease.

Dopamine transporter (DAT) imaging is an important adjunct in the diagnostic workup of patients with Parkinsonism. 18F-FE-PE2I is a suitable PET radioligand for DAT quantification and imaging with good pharmacokinetics. The aim of this study was to determine a clinical optimal simplified reference tissue-based image acquisition protocol and to compare the discriminatory value and effect size for 18F-FE-PE2I to that for 123I-FP-CIT scan currently used in clinical practice. Nine patients with early Parkinson's disease (PD, 64.3 ± 6.8 years, 3M), who had previously undergone a 123I-FP-CIT scan as part of their diagnostic workup, and 34 healthy volunteers (HV, 47.7 ± 16.8 years, 13M) underwent a 60-min dynamic 18F-FE-PE2I PET-MR scan on a GE Signa 3T PET-MR. Based on dynamic data and MR-based VOI delineation, BPND, semi-quantitative uptake ratio and SUVR[t1-t2] images were calculated using either occipital cortex or cerebellum as reference region. For start-and-end time of the SUVR interval, three time frames [t1-t2] were investigated: [15-40] min, [40-60] min, and [50-60] min postinjection. Data for putamen (PUT) and caudate nucleus-putamen ratio (CPR) were compared in terms of quantification bias versus BPND and discriminative power. Using occipital cortex as reference region resulted in smaller bias of SUVR with respect to BPND + 1 and higher correlation between SUVR and BPND + 1 compared with using cerebellum, irrespective of SUVR [t1-t2] interval. Smallest bias was observed with the [15-40]-min time window, in accordance with previous literature. The correlation between BPND + 1 and SUVR was slightly better for the late time windows. Discriminant analysis between PD and HV using both PUT and CPR SUVRs showed an accuracy of ≥ 90%, for both reference regions and all studied time windows. Semi-quantitative 123I-FP-CIT and 18F-FE-PE2I values and relative decrease in the striatum for patients were highly correlated, with a higher effect size for 18F-FE-PE2I for PUT and CPR SUVR. 18F-FE-PE2I is a suitable radioligand for in vivo DAT imaging with high discriminative power between early PD and healthy controls. Whereas a [15-40]-min window has lowest bias with respect to BPND, a [50-60]-min time window at pseudoequilibrium can be advocated in terms of clinical feasibility with optimal discriminative power. The occipital cortex may be slightly preferable as reference region because of the higher time stability, stronger correlation of SUVR with BPND + 1, and lower bias. Moreover, the data suggest that the diagnostic accuracy of a 10-min static 18F-FE-PE2I scan is non-inferior compared with 123I-FP-CIT scan used in standard clinical practice.

Interstitial Lung Disease in Rheumatoid Arthritis Remains a Challenge for Clinicians.

Interstitial lung disease (ILD) is a serious complication of rheumatoid arthritis (RA) contributing to significantly increased morbidity and mortality. Other respiratory complications, such as chronic obstructive pulmonary disease and bronchiectasis, are frequent in RA. Infections and drug toxicity are important differential diagnoses and should be considered in the diagnostic work-up of patients with RA presenting with respiratory symptoms. This review provides an overview of the epidemiology and pathogenesis of RA-ILD, the radiological and histopathological characteristics of the disease as well as the current and future treatment options. Currently, there is no available evidence-based therapy for RA-ILD, and immunosuppressants are the mainstay of therapy. Ongoing studies are exploring the role of antifibrotic therapy in patients with progressive fibrotic ILD, which may lead to a new treatment approach for subgroups of patients with RA-ILD.

Diagnostic accuracy of 99mTc-antigranulocyte SPECT/CT in patients with osteomyelitis and orthopaedic device-related infections: A retrospective analysis

ObjectivesConventional imaging techniques are routinely used in the diagnostic work-up of patients with suspected osteomyelitis or orthopaedic implant-associated infections. Hybrid nuclear medicine imaging techniques are a suitable alternative to routine imaging modalities as they provide anatomical and functional information within one procedure. Our study investigated the performance of anti-granulocyte SPECT/CT using 99mTc-labelled monoclonal antibodies in the diagnosis of osteomyelitis and orthopaedic implant-associated infections. MethodsIn this retrospective analysis, we included patients with 99mTc-antigranulocyte SPECT/CT acquired in the context of a suspected bone and joint infection. All patients underwent routine diagnostics and/or had a clinical follow-up of at least 12months. Results26 episodes were included. Fifteen exams were performed for suspected osteomyelitis, and 11 for suspected orthopaedic implant-associated infection. SPECT/CT was ordered most often if standard diagnostic tests or conventional imaging modalities remained inconclusive. The overall sensitivity and specificity for the diagnosis of an infection were 77.8% and 94.1%, respectively. The positive predictive value was 87.5% and the negative predictive value 88.9%. Diagnostic accuracy was 88.5%. Conclusions99mTc-antigranulocyte SPECT/CT imaging has a high accuracy in the diagnosis of osteomyelitis and orthopaedic implant-associated infections and is a suitable non-invasive diagnostic tool if standard diagnostic examinations are inconclusive or not applicable.

Diagnosis and management of malignant peripheral nerve sheath tumors: Current practice and future perspectives.

One of the most common malignancies affecting adults with the neurofibromatosis type 1 (NF1) cancer predisposition syndrome is the malignant peripheral nerve sheath tumor (MPNST), a highly aggressive sarcoma that typically develops from benign plexiform neurofibromas. Approximately 8–13% of individuals with NF1 will develop MPNST during young adulthood. There are few therapeutic options, and the vast majority of people with these cancers will die within 5 years of diagnosis. Despite efforts to understand the pathogenesis of these aggressive tumors, the overall prognosis remains dismal. This manuscript will review the current understanding of the cellular and molecular progression of MPNST, diagnostic workup of patients with these tumors, current treatment paradigms, and investigational treatment options. Additionally, we highlight novel areas of preclinical research, which may lead to future clinical trials. In summary, MPNST remains a diagnostic and therapeutic challenge, and future work is needed to develop novel and rational combinational therapy for these tumors.

Ultrasound-based techniques for the diagnosis of liver steatosis.

Non-alcoholic fatty liver disease (NAFLD) is the leading cause of diffuse liver disease. An accurate estimate of the fat in the liver is important in the diagnostic work-up of patients with NAFLD because the degree of liver steatosis is linked to the metabolic syndrome and the cardiovascular risk. Ultrasound (US) B-mode imaging allows to subjectively estimate the fatty infiltration in the liver; however, it has a low performance for the detection of mild steatosis. Quantitative US is based on the analysis of the radiofrequency echoes detected by an US system, and it allows to calculate a backscatter coefficient or an attenuation coefficient or the sound speed. The estimation of the backscatter coefficient is rather cumbersome and requires the use of a phantom for addressing all sources of variability. Controlled attenuation parameter (CAP) available on the FibroScan® system (Echosens, France) measures the attenuation of the US beam. CAP is accurate in grading fatty infiltration-even though there is an overlap between consecutive grade of liver steatosis-and the values are not influenced by liver fibrosis. Several US manufacturers are developing or have already developed software for quantifying the attenuation of the US beam. Preliminary results show that proprietary technologies implemented in US systems seem more accurate than CAP for grading liver steatosis. Another available method for quantifying liver steatosis is based on the computation of the sound speed and the initial results appear promising.

Mild Zellweger syndrome due to functionally confirmed novel PEX1 variants

Zellweger spectrum disorders (ZSD) constitute a group of rare autosomal recessive disorders characterized by a defect in peroxisome biogenesis due to mutations in one of 13 PEX genes. The broad clinical heterogeneity especially in late-onset presenting patients and a mild phenotype complicates and delays the diagnostic process. Here, we report a case of mild ZSD, due to novel PEX1 variants. The patient presented with an early hearing loss, bilateral cataracts, and leukodystrophy on magnetic resonance (MR) images. Normal results of serum very-long-chain fatty acids (VLCFA) and phytanic acid were found. Molecular diagnostics were performed to uncover the etiology of the clinical phenotype. Using whole exome sequencing, there have been found two variants in the PEX1 gene—c.3450T>A (p.Cys1150*) and c.1769T>C (p.Leu590Pro). VLCFA measurement in skin fibroblasts and C26:0-lysoPC in dried blood spot therefore was performed. Both results were in line with the diagnosis of ZSD. To conclude, normal results of routine serum VLCFA and branched-chain fatty acid measurement do not exclude mild forms of ZSD. The investigation of C26:0-lysoPC should be included in the diagnostic work-up in patients with cataract, hearing loss, and leukodystrophy on MR images suspected to suffer from ZSD.

P6170Incremental value of on-site computed tomography-derived fractional flow reserve for the diagnosis and management strategy of obstructive coronary artery disease in the randomized CRESCENT trials

Abstract Background Coronary computed tomography angiography (CCTA) accurately rules out coronary artery disease (CAD), but has a limited ability to predict hemodynamically significant CAD. Implementing on-site computed tomography-derived fractional flow reserve (CT-FFR) could improve the clinical value and efficiency of cardiac CT in the diagnostic work-up of patients with stable angina. Purpose To determine the impact of on-site CT-FFR on diagnostic effectiveness, management strategy and downstream invasive coronary angiography (ICA) in patients with suspected CAD. Methods 196 patients (59.1±9.6 years, 47% women) with suspected CAD underwent a CCTA in the CRESCENT I and II trials. On-site CT-FFR analysis was performed in all patients with at least one ≥50% stenosis on CCTA (N=53). We assessed the effect of adding CT-FFR analysis to CCTA in terms of 1) diagnostic effectiveness, i.e. the number of additional tests required to determine the final diagnosis; 2) reclassification of the initial management strategy; 3) ICA efficiency, i.e. ICA rate without ≥50% CAD. Results CT-FFR was calculated in 42/53 (79%) of the eligible patients as it could not be calculated in patients with suspected coronary total occlusion (N=7), severe coronary calcification (N=2), severe CT artefacts (N=1) or missing CT images (N=1). CT-FFR ≤0.80 was present in 27/196 (14%) patients, including 8/196 (4%) patients with high-risk ischemia (CT-FFR ≤0.80 in all three vessels, left main or proximal left anterior descending coronary artery). The final diagnosis was achieved with CT-FFR in an additional 30/196 (15%) patients compared to CT alone (p&lt;0.0001), and rendered 42/56 (75%) of additional tests unnecessary (p&lt;0.0001). The initial management strategy was reclassified in 30/196 patients (15%, p&lt;0.0001); 24/196 (12%) patients were reclassified to optimal medical therapy and 6/196 (3%) patients were reclassified directly to ICA including 4/8 (50%) patients with high-risk CAD on ICA. CT-FFR would result in 6/32 (19%, p=0.012) ICA cancellations in which none of the patients had high-risk CAD. The rate of ICA without ≥50% stenosis would decrease from 22% (7/32) to 11% (3/27) (p=0.012). Conclusion Implementation of CT-FFR has the potential for improved diagnostic effectiveness. Functional reclassification of CAD provides more efficient ICA referral in patients with suspected CAD compared to CTA alone. Acknowledgement/Funding Dutch Heart Foundation [NHS 2014T061 and NHS 2013T071]

The pattern of urine cytology among patients with clinical diagnosis of bladder tumor in a tertiary hospital northwest Nigeria

Background: Urine cytology is a simple, safe, non-invasive and cheap investigation that is used as adjunct to cystoscopy in the diagnosis of bladder cancer. Its low sensitivity is a major limitation against its use as a sole diagnostic test for bladder cancer. The objective of this study was to determine the pattern of urine cytology seen in patients with clinical diagnosis of bladder tumour in our practice.Methods: This is a retrospective study of patients with clinical diagnosis of bladder tumour that had urine cytology in our centre. The age and gender of the patients, number of urine cytology per patient per year and cytologic diagnosis were analysed using the SPSS 20.Results: During the period under review, a total of 512 urine cytology was done for patients with clinical diagnosis of bladder tumour. The age range of the patients was 6 to 90 years with modal age of 60 years. 457 (89.3%) were males while 54 (10.5%) were females and 1 (0.2%) was unspecified. Male to female ratio was 8.5:1. The highest number of urine cytology was done in 2013 with 64 (12.5%) while the least number was 1 (0.2%) recorded in 2001 and 2003. Only 68 (13.3%) specimens were reported to be malignant while 245 (47.9%) were reported as negative representing the most common cytological diagnosis in the study.Conclusions: Although urine cytology is useful in the diagnostic workup of patients with bladder mass, it is unlikely it would supplant cystoscopy and biopsy in the diagnosis of bladder cancer.

Diagnostic Accuracy of Computed Tomography to Exclude Pheochromocytoma: A Systematic Review, Meta-analysis, and Cost Analysis

ObjectivesTo assess the diagnostic accuracy of unenhanced computed tomography (CT) attenuation values to exclude a pheochromocytoma in the diagnostic work-up of patients with an adrenal incidentaloma and to model the associated difference in diagnostic costs. MethodsThe MEDLINE and Embase databases were searched from indexing to September 27, 2018, and studies reporting the proportion of pheochromocytomas on either side of the 10–Hounsfield unit (HU) threshold on unenhanced CT were included. The pooled proportion of pheochromocytomas with an attenuation value greater than 10 HU was determined, as were the modeled financial costs of the current and alternative diagnostic approaches. ResultsOf 2957 studies identified, 31 were included (N=1167 pheochromocytomas). Overall risk of bias was low. Heterogeneity was not observed between studies (Q=11.5, P=.99, I2=0%). The pooled proportion of patients with attenuation values greater than 10 HU was 0.990 (95% CI, 0.984-0.995). The modeled financial costs using the new diagnostic approach were €55 (∼$63) lower per patient. ConclusionPheochromocytomas can be reliably ruled out in the case of an adrenal lesion with an unenhanced CT attenuation value of 10 HU or less. Therefore, determination of metanephrine levels can be restricted to adrenal tumors with an unenhanced CT attenuation value greater than 10 HU. Implementing this novel diagnostic strategy is cost-saving.

Diagnostic imaging for acute abdominal pain in an Emergency Department in Italy

Imaging plays a key role in the diagnostic work-up of patients with non-traumatic acute abdominal pain (AAP) in emergency department (ED). We aimed to evaluate the use and diagnostic performance of imaging techniques in adult patients with AAP in an ED in Italy. Patients with non-traumatic AAP admitted at the ED of S. Orsola-Malpighi University Hospital of Bologna (Italy) from the 1st to the 30th November 2016 were included. Demographic and clinical data, data on radiological procedures and discharge diagnosis were collected. Sensitivity and specificity for detecting acute diseases were assessed using the discharge diagnosis from the ED as reference standard. Of the 578 patients (female 52.8%, mean age 51.3years) admitted to the ED for AAP, 433 (74.9%) underwent abdominal imaging. The most frequent techniques used were abdominal plain radiography and ultrasonography (US), performed in 38.4% and 37.9% of patients, respectively, followed by computed tomography (CT) in 28% of patients. Plain radiography yielded a sensitivity of 28% and specificity of 91.1%; the sensitivity increased to 79.4% in patients with small bowel obstruction. Ultrasonography's sensitivity and specificity were 61.8% and 98.4%, respectively; the sensitivity of US reached 85.2% and 90% in patients with acute cholecystitis/biliary colic and urolithiasis, respectively. The sensitivity and specificity of CT were 87.8% and 92.9%, respectively. Plain radiography is still overused in the diagnostic work-up of AAP in ED in Italy, despite its unsatisfactory sensitivity. Ultrasonography and CT has a higher sensitivity and should be used as first-level imaging in most patients.

Malnutrition-related hyperammonemic encephalopathy presenting with burst suppression: a case report

BackgroundHyperammonemia is a common cause of metabolic encephalopathy, mainly related to hepatic cirrhosis. Numerous nonhepatic etiologies exist but they are infrequent and not well known, thus, leading to misdiagnosis and inadequate care. Electroencephalography has a proven diagnostic and prognostic role in comatose patients. Burst suppression is a preterminal pattern found in deep coma states and is rarely associated with metabolic causes.Case presentationWe report the case of an 81-year-old Caucasian man presenting with rapidly progressive somnolence and mutism. Soon after his arrival in our hospital, he developed profound coma. A comprehensive diagnostic workup was unremarkable except for admission electroencephalography showing diffuse slowing of cerebral activity with an intermittent pattern of burst suppression. He was admitted to our intensive care unit for supportive care where malnutrition-related hyperammonemia was diagnosed. His clinical course was spontaneously favorable and follow-up electroencephalography demonstrated normal cerebral activity.ConclusionsNonhepatic hyperammonemia is a rare and potentially reversible cause of encephalopathy. Ammonia level measurement should be part of the diagnostic workup in patients with unexplained coma, particularly in the setting of nutritional deficiencies or nutritional supply. Detection of diffuse and nonspecific mild to moderate slowing of cerebral activity (theta-delta ranges) on electroencephalography is common. In contrast, to the best of our knowledge, burst suppression has never been described in association with hyperammonemia.

Small fiber neuropathy in coeliac disease and gluten sensitivity

ABSTRACTObjectives: The commonest types of peripheral neuropathy in the context of Coeliac Disease (CD) and gluten sensitivity (GS) are length-dependent symmetrical sensorimotor neuropathies and sensory ganglionopathies. In patients with such neuropathy, (gluten neuropathy), peripheral neuropathic pain is prevalent suggesting involvement of small fibers. The purpose of this report was to describe the clinical characteristics of patients with CD or GS and pure small fiber neuropathy (SFN).Methods: We reviewed the records of all patients that had been referred to the Gluten-Related Neurological Disorders clinic who had clinical and neurophysiological evidence of SFN. All patients had serological evidence of gluten sensitivity (GS) prior to commencing GFD. All patients were offered a duodenum biopsy. Patients with comorbidities that could cause SFN were excluded.Results: We identified 13 patients (9 males) with SFN and gluten sensitivity. Of 11 patients who underwent duodenal biopsy 10 had evidence of enteropathy (CD). Mean age at onset of pain was 53.5 ± 11.4 years (range 34–72) and mean age of CD/GS diagnosis was 50.8 ± 10.4 years (range 34–68). In 8 patients (61.5%) pain was the presenting feature.Neurophysiological assessment suggested a length-dependent small fiber neuropathy in 11 patients, whereas in 2, a non-length dependent pattern was identifying suggesting that the predominant pathology lies in the dorsal root ganglia.Conclusion: SFN can be a presenting feature of CD and GS and, therefore, screening for CD and GS should be included in the diagnostic workup of patients with idiopathic SFN.

Echocardiography Clues to Suspect Transthyretin Cardiac Amyloidosis Diagnosed by Tc-Pyrophosphate SPECT Imaging

Introduction Transthyretin (TTR) amyloidosis is a debilitating systemic disease often associated with symptomatic cardiac involvement. Cardiac amyloidosis remains underappreciated as its diagnosis has been challenging. Recently, Technetium99-m pyrophosphate (Tc-PYP) single-photon emission computed tomography (SPECT) imaging has emerged as a reliable modality to diagnose patients with TTR cardiac amyloidosis. However, there are no clear guidelines regarding which patients should be referred for Tc-PYP imaging. As electrocardiogram (ECG) and transthoracic echocardiography (TTE) are universally utilized in the diagnostic workup in patients with cardiac symptoms, we sought to evaluate the predictive value of longitudinal strain assessment and strain pattern by TTE in the diagnosis of TTR cardiac amyloidosis. Methods and Results In this single-center, retrospective study, patients who underwent Tc-PYP imaging between June 2016 and October 2018 were reviewed. ECG and TTE variables assessed are shown in Table 1. Relative apical longitudinal strain was calculated from left ventricular longitudinal strain (LV LS) values. Of 39 consecutive Tc-PYP scans, 24 positive scans and 12 negative scans were identified; 3 “equivocal” scans were excluded. No ECG parameters were predictive of “Tc-PYP positive” scans. LV mass index (p=0.003) demonstrated a significant difference between positive and negative Tc-PYP groups. Significantly, “Tc-PYP positive” patients had a higher relative apical sparing of LV LS (p=0.0004), and notably, no Tc-PYP negative patient had a ratio >1.0. Patients with “Tc-PYP positive” scans were significantly more likely to exhibit relative apical sparing of LV LS compared to other standard parameters assessed by electrocardiogram or echocardiography. Conclusions LV LS measurement should be routinely included as part of a standard echocardiographic protocol to better identify patients at risk. A patient with relative apical sparing of LV LS ratio >1.0 should be referred for Tc-PYP imaging to evaluate for TTR cardiac amyloidosis.

Exorotated radiographic views have additional diagnostic value in detecting an osseous impediment in patients with posterior ankle impingement

ObjectivesA standard lateral radiograph is the first step in the diagnostic workup in patients with posterior ankle pain. Because of overprojection by other structures at suboptimal radiographic projection angle, often an os trigonum is not discovered or erroneously be mistaken for a hypertrophic posterior talar process. The aim of this study was to identify the projection angles at which a radiograph is optimal for detecting bony impediments in patients suffering from posterior ankle impingement.MethodsUsing ankle CT scans of patients with posterior ankle impingement, digitally reconstructed radiographs (DRRs) simulating 13 different radiographic projection angles were generated. The ankle CT scans served as a reference for the detection of an os trigonum and hypertrophic posterior talar process. Members of the Ankleplatform Study Group were invited to assess the DRRs, for presence or absence of an os trigonum or hypertrophic posterior talar process. Diagnostic accuracy and interobserver reliability were estimated for each projection angle. In addition, the diagnostic accuracy of the standard lateral view in combination with the rotated views was calculated.ResultsHigh sensitivity for detecting an os trigonum was found for +15° (90.3%), +20° (81.7%) and +25° (89.7%) degrees of exorotation. Specificity in this range of projection angles was between 89.6% and 97.8%. Regarding the presence of a hypertrophic posterior talar process, increased sensitivity was found for +15° (65.7%), +20° (61.0%), +25° (60.7%), +30° (56.3%) and +35° (54.5%). Specificity ranged from 78.0% to 94.7%. The combination of the standard lateral view in combination with exorotated views showed higher sensitivity. For detecting an os trigonum, a negative predictive value of 94.6% (+15°), 94.1% (+20°) and 96.1% (+25°) was found.ConclusionThis study underlines the additional diagnostic value of exorotated views instead of, or in addition to the standard lateral view in detecting an osseous impediment. We recommend to use the 25° exorotated view in combination with the routine standard lateral ankle view in the workup of patients with posterior ankle pain.Level of evidenceLevel III