Radiologic Imaging Studies Research Articles

Feasibility of measuring memory response to increasing dexmedetomidine sedation in children

BackgroundThe memory effect of dexmedetomidine has not been prospectively evaluated in children. We evaluated the feasibility of measuring memory and sedation responses in children during dexmedetomidine sedation for non-painful radiological imaging studies. Secondarily, we quantified changes in memory in relation to the onset of sedation. MethodsA 10 min bolus of dexmedetomidine (2 mcg kg−1) was given to children as they named simple line drawings every five s. The absence of sedation was identified as any verbal response, regardless of correctness. After recovery, recognition memory was tested with correct Yes/No recognitions (50% novel pictures) and was matched to sedation responses during the bolus period (subsequent memory paradigm). ResultsOf 64 accruals, 30 children (mean [SD]6.1 (1.2) yr, eight male) received dexmedetomidine and completed all study tasks. Individual responses were able to be modelled successfully in the 30 children completing all the study tasks, demonstrating feasibility of this approach. Children had 50% probability of verbal response at five min 40 s after infusion start, whereas 50% probability of subsequent recognition memory occurred sooner at four min five s. ConclusionsQuantifying memory and sedation effects during dexmedetomidine infusion in verbal children was possible and demonstrated that memory function was present until shortly before verbal unresponsiveness occurred. This is the first study to investigate the effect of dexmedetomidine on memory in children. Clinical trial registrationNCT 02354378.

Management of Incidentally Diagnosed Splenic Masses

The aim of this study is to consider the management of incidentally diagnosed splenic masses. Patients who underwent splenectomy between September 2005 and February 2015 were analyzed retrospectively. Patients with the history of lymphoproliferative disease were excluded. Radiologic evaluation was done in all patients. Preoperative tru-cut biopsies were performed on three patients. The patients were grouped according to the nature of the splenic mass such as cystic, benign, and malign lesions. The groups were compared according to age, gender, tumor size, and the number of lesions. There were 13 male and 12 female patients with the median age of 52 years (range 15–84 years). The final pathology revealed cyst in 7 patients (28.0%), benign lesion in 7 patients (28.0%), and malign lesion in 11 patients (44%). The results of tru-cut biopsies were similar with postoperative pathologies. Solitary lesions were detected in 15 patients, and there were multiple lesions in 10 cases. There was not a significant difference between the tumor sizes of malign vs. benign lesions (p = 0.42). Malignancy was a higher trend in multiple lesions (p = 0.06). Advanced radiologic imaging studies and more frequent and reliable use of percutaneous diagnostic procedures may limit the number of unnecessary splenectomies especially for benign lesions.

Usefulness of Blood Cultures and Radiologic Imaging Studies in the Management of Patients with Community-Acquired Acute Pyelonephritis.

BackgroundThe objective of this study was to examine the usefulness of blood cultures and radiologic imaging studies for developing therapeutic strategies in community-acquired acute pyelonephritis (CA-APN) patients.Materials and MethodsWe prospectively collected the clinical data of CA-APN patients who visited 11 hospitals from March 2010 to February 2011.ResultsPositive urine and blood cultures were obtained in 69.3% (568/820) and 42.7% (277/648), respectively, of a total of 827 CA-APN patients. Blood culture identified the urinary pathogen in 60 of 645 (9.3%) patients for whom both urine and blood cultures were performed; the organisms isolated from urine were inconsistent with those from blood in 11 and only blood cultures were positive in 49 patients. Final clinical failure was more common in the bacteremic patients than the non-bacteremic ones (8.0% vs. 2.7%, P = 0.003), as was hospital mortality (3.6% vs. 0.3%, P = 0.003). Likewise, durations of hospitalization and fever were significantly longer. Bacteremia was independent risk factor for mortality (OR 9.290, 1.145-75.392, P = 0.037). With regard to radiologic studies, the detection rate of APN was 84.4% (445/527) by abdominal computed tomography and 40% (72/180) by abdominal ultrasonography. Eighty-one of 683 patients (11.9%) were found to have renal abscess, perinephric abscess, urolithiasis, hydronephorosis/hydroureter or emphysematous cystitis, which could potentially impact on clinical management. Patients with Pitt score ≥ 1, flank pain or azotemia were significantly more likely to have such structural abnormalities.ConclusionBlood cultures are clinically useful for diagnosis of CA-APN, and bacteremia is predictive factor for hospital mortality. Early radiologic imaging studies should be considered for CA-APN patients with Pitt scores ≥1, flank pain or azotemia.

Pulmonary actinomycosis mimicking lung cancer on positron emission tomography.

Pulmonary actinomycosis frequently mimics lung malignancy on radiologic imaging studies. Positron emission tomography-computed tomography (PET-CT) is a useful diagnostic modality for differentiating lung malignancy from benign diseases. However, few studies evaluated PET-CT findings of pulmonary actinomycosis. Therefore, it is unclear whether PET-CT is helpful to distinguish lung malignancy from benign lung disease when pulmonary actinomycosis is clinically suspected. We investigated PET-CT findings in 11 patients with pathologically confirmed pulmonary actinomycosis. The median maximal standardized uptake value (SUV) on PET-CT of pulmonary actinomycosis was increased to 5.5 (interquartile range, 4.2–8.8), which was higher than the threshold value of 2.5 indicating malignancy. Pulmonary actinomycosis without central necrosis demonstrated higher maximal SUV of 7.5 (4.9–12.2) compared to 4.8 (3.2–5.6) of ones with central necrosis. PET-CT might be not helpful in differentiating lung malignancy from benign lesions when pulmonary actinomycosis is clinically suspected.

An Unusual Case of Lumbar Facet Arthropathy presenting with Pain Abdomen

Introduction: Facet joint arthropathy refers to a degenerative disease that affects the joints of the vertebrae. Lumbar facet joints (zygapophyseal joint) were first suggested in the medical literature as a source of low back pain (LBP) and lower extremity pain in 1911. These joints have been implicated as the cause of pain in 15 to 45% of patients with chronic LBP. The majority of published clinical investigations report no correlation between the clinical symptoms of LBP and degenerative spinal changes observed on radiologic imaging studies, including radiographs, magnetic resonance imaging (MRI), computed tomography (CT), single photon emission CT, and radionuclide bone scanning. Low back pain from the facet joints often radiates down into the buttocks and down the back of the upper leg. Pain is rarely present in the front of the leg or below the knee. Our case report is about a 24-year-old female patient who presented with left-sided paramedian LBP with severe lower abdominal pain, who underwent several investigations for her abdominal pain and was treated for abdominal pain without any significant relief. Careful history and clinical examination revealed lumbar facet joint tenderness involving left L4–L5 and L5–S1 facet joint. Diagnostic block followed by radiofrequency (RF) ablation of medial branch supplying the corresponding facet joint was performed with near-complete pain relief.

Total Kidney Volume as a Biomarker of Disease Progression in Autosomal Dominant Polycystic Kidney Disease.

Purpose of review:Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the formation of kidney cysts and kidney enlargement, which progresses to kidney failure by the fifth to seventh decade of life in a majority of patients. Disease progression is evaluated primarily through serum creatinine and estimated glomerular filtration rate (eGFR) measurements; however, it is known that serum creatinine and eGFR values typically do not change until the fourth or fifth decade of life. Until recently, therapy only existed to target complications of ADPKD. As therapeutic agents continue to be investigated for use in ADPKD, a suitable biomarker of disease progression in place of serum creatinine is needed.Sources of information:This review summarizes recent research regarding the use of total kidney volume as a biomarker in ADPKD, as presented at the Canadian Society of Nephrology symposium held in April 2015.Findings:Measurement of patients’ total kidney volume made using ultrasound (US) or magnetic resonance imaging (MRI) has been shown by the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) study to be directly correlated with both increases in cyst volume and change in glomerular filtration rate (GFR). Additional studies have shown total kidney volume to have an association with complications of ADPKD as well.Limitations:Areas for further study continue to exist in comparison of methods of measuring total kidney volume.Implications:We believe that the evidence suggests that total kidney volume may be an appropriate surrogate marker for ADPKD disease progression.

Evaluation of the Role of Radiological Imaging and Bone Marrow Biopsy in Staging of Cutaneous B-Cell Lymphoma

Background: Primary cutaneous B-cell lymphoma (PCBCL) refers to B-lymphocyte derived lymphoma that develops in the skin without any extracutaneous involvement at the time of diagnosis. Primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous large B-cell lymphoma (PCLBCL) are the three major entities of PCBCL under the World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas. Current guidelines recommend obtaining staging Positron emission tomography/computed tomography (PET/CT) scan or CT scan for all PCBCL, and bone marrow biopsy for at least PCLBCL-leg type variant. However, evidence supporting these recommendations, especially radiological imaging, is lacking.Methods: Data including demographics, white blood cell (WBC) count at diagnosis, lactate dehydrogenase (LDH) at diagnosis, and results of staging CT-scan, PET/CT-scan, single-photon emission computed tomography scan (SPECT-scan), and bone marrow biopsy were collected through chart review on all patients seen at Roswell Park Cancer Institute between 2001-2016 who presented with a skin lesion and had a biopsy diagnostic of B-cell lymphoma. Patients without any radiological imaging at diagnosis and those diagnosed of diffuse large B-cell lymphoma (DLBCL), marginal zone lymphoma (MZL), or follicular lymphoma (FL) prior to cutaneous manifestation were excluded.Results: 67 patients met criteria for this study of whom 97% were Caucasian and 60% were male. Cutaneous biopsies noted follicle center cell histology (16 patients; 24%), marginal zone histology (32 patients; 48%), or large B-cell histology (19 patients; 29%). Staging CT-scan, functional imaging (PET/CT-scan or SPECT-scan), and bone marrow biopsy were performed for 59 (88%), 48 (72%), and 36 (54%) patients respectively (distribution across B-cell lymphomas shown in Figure 1). Radiological imaging studies were over-interpreted in 13 patients. Radiological imaging upstaged diagnosis in 13 patients (8 DLBCL, 3 MZL, 2 FL) while bone marrow biopsy alone upstaged diagnosis in only 1 patient (DLBCL). Together, work-up upstaged diagnosis in patients with cutaneous high-grade lymphoma (large B-cell lymphoma) significantly more than it did for cutaneous low-grade lymphoma (follicle center cell and marginal zone lymphoma) histology (47% vs. 10%; p=0.0018). Presence of B-symptoms correlated with systemic disease (0 patients with PCBCL vs. 4 patients with systemic disease; p=0.0013). However, age (p=0.059), gender (p=0.5418), WBC (p=0.6676), and LDH (p=0.1736) had no correlation with systemic disease.Conclusion: Our single center retrospective analysis showed that staging work-up including radiological imaging (CT-scan or functional imaging like PET/CT-scan) and bone marrow biopsy upstaged the diagnosis in a small minority (10%) of low-grade cutaneous B-cell lymphomas. However, nearly half (47%) of those with cutaneous large B-cell lymphoma histology were found to have systemic disease upon staging. Given the aggressive disease course of large B-cell lymphomas, staging through radiological imaging and bone marrow biopsy should be pursued as currently recommended. However, for low-grade B-cell lymphomas, where even observation is a reasonable management option in selected stage IV patients, staging radiological imaging and bone marrow biopsies could be avoided unless dictated by clinical judgment. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Metastatic neuroendocrine tumor with initial presentation of orbital apex syndrome

Abstract The possible etiologies of orbital apex syndrome range from inflammatory, infectious, neoplastic, iatrogenic/traumatic, to vascular processes. In patients without obvious infection or systemic cancer history, judicious use of corticosteroids is a reasonable strategy. We describe a 64-year-old man who presented with orbital apex syndrome and had progressed to total visual loss in three days after admission. Radiological imaging and pathological studies were consistent with a neuroendocrine tumor with multiple metastases. We recommend that a biopsy-proven specimen is warranted in patient with orbital apex syndrome even without a cancer history.

Clinical Outcomes of Endoscopic Ultrasonography-Guided Pancreatic Cyst Ablation.

Endoscopic ultrasonography-guided ethanol ablation therapy for pancreatic cystic lesions is a minimally invasive treatment but still is an experimental therapy. The aims were to investigate the safety and efficacy of endoscopic ultrasonography-guided ethanol ablation therapy. The inclusion criteria were the following: clinically indeterminate pancreatic cystic lesions in radiologic imaging studies, 2 to 5 cm unilocular or oligolocular cysts without communication to main pancreatic duct, and patients with high-risk operation. There were 91 study patients with median follow-up of 40 months. The response rate was as follows: complete resolution, 41 (45%); partial resolution, 37; and persistent cysts, 13. Pancreatic cystic lesions were categorized based on cystic fluid analysis: 9 intraductal papillary mucinous neoplasms (IPMNs), 12 mucinous cystic neoplasms, 33 serous cystic neoplasms, and 28 uncategorized cysts. The success rate was significantly different according to cystic fluid analysis (serous cystic neoplasm, 58%; mucinous cystic neoplasm, 50%; IPMN, 11%; uncategorized cysts, 39%; P < 0.0001). There were 3 patients with mild pancreatitis after the treatment. Endoscopic ultrasonography-guided ethanol ablation therapy seems to be a safe treatment modality. However, it was only effective in 11% of IPMNs. Therefore, the clinical application should be very limited for certain patients who could not tolerate the surgical treatment.

Brachial Plexus Surgery: Clinical Analysis of Ten Cases

Background and Aim: Brachial plexus surgery is a complex surgery and multiple surgical techniques were advanced. The concept that patients who underwent surgery will obtain full recovery is still far from the truth. In this study, we show our results after usage some surgical techniques, aiming to describe them. Methods: Through case series study, the team operated 10 cases, including infants and adults (Seven males and three females) between January 2013 and June 2015. Detailed history, physical examinations, radiological imaging studies and electrophysiological studies were performed to confirm the nature and severity of the injury. Exclusion criteria, including individuals who spontaneously recovered after 4 months or those came after more than 12 months of injury. Neurolysis, nerve grafting and nerve transfer were done according to certain protocol performed for each case. Results:History of road traffic accident was reported in five cases, obstetric brachial plexus palsy in three cases, gunshot wound in two cases. They were diagnosed as upper brachial plexus injury (4 cases), lower brachial plexus injury (2 cases), and complete flail limb (4 cases). Four cases were submitted into neurolysis, three into nerve grafting, and three into the nerve transfer.Four cases showing functional motor outcome improvement (2 cases, Neurolysis and 2 cases, nerve transfer) but the other six cases do not show any improvement until now (3 nerve grafting, 2 neurolysis and 1 nerve transfer).Assessment of the motor power was done using the Medical Research Council scale. One case was complicated immediately post-operatively with pan plexopathy of the same limb of surgery and was improved one week after. Conclusion: Brachial plexus surgery renders satisfactory results for restoring the upper limb functions. The results shown that neurolysis and nerve transfer give better results than nerve grafting.

Predicted Mutation Strength of Nontruncating PKD1 Mutations Aids Genotype-Phenotype Correlations in Autosomal Dominant Polycystic Kidney Disease.

Autosomal dominant polycystic kidney disease (ADPKD) often results in ESRD but with a highly variable course. Mutations to PKD1 or PKD2 cause ADPKD; both loci have high levels of allelic heterogeneity. We evaluated genotype-phenotype correlations in 1119 patients (945 families) from the HALT Progression of PKD Study and the Consortium of Radiologic Imaging Study of PKD Study. The population was defined as: 77.7% PKD1, 14.7% PKD2, and 7.6% with no mutation detected (NMD). Phenotypic end points were sex, eGFR, height-adjusted total kidney volume (htTKV), and liver cyst volume. Analysis of the eGFR and htTKV measures showed that the PKD1 group had more severe disease than the PKD2 group, whereas the NMD group had a PKD2-like phenotype. In both the PKD1 and PKD2 populations, men had more severe renal disease, but women had larger liver cyst volumes. Compared with nontruncating PKD1 mutations, truncating PKD1 mutations associated with lower eGFR, but the mutation groups were not differentiated by htTKV. PKD1 nontruncating mutations were evaluated for conservation and chemical change and subdivided into strong (mutation strength group 2 [MSG2]) and weak (MSG3) mutation groups. Analysis of eGFR and htTKV measures showed that patients with MSG3 but not MSG2 mutations had significantly milder disease than patients with truncating cases (MSG1), an association especially evident in extreme decile populations. Overall, we have quantified the contribution of genic and PKD1 allelic effects and sex to the ADPKD phenotype. Intrafamilial correlation analysis showed that other factors shared by families influence htTKV, with these additional genetic/environmental factors significantly affecting the ADPKD phenotype.

Automated Segmentation of Kidneys from MR Images in Patients with Autosomal Dominant Polycystic Kidney Disease.

Our study developed a fully automated method for segmentation and volumetric measurements of kidneys from magnetic resonance images in patients with autosomal dominant polycystic kidney disease and assessed the performance of the automated method with the reference manual segmentation method. Study patients were selected from the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease. At the enrollment of the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Study in 2000, patients with autosomal dominant polycystic kidney disease were between 15 and 46 years of age with relatively preserved GFRs. Our fully automated segmentation method was on the basis of a spatial prior probability map of the location of kidneys in abdominal magnetic resonance images and regional mapping with total variation regularization and propagated shape constraints that were formulated into a level set framework. T2-weighted magnetic resonance image sets of 120 kidneys were selected from 60 patients with autosomal dominant polycystic kidney disease and divided into the training and test datasets. The performance of the automated method in reference to the manual method was assessed by means of two metrics: Dice similarity coefficient and intraclass correlation coefficient of segmented kidney volume. The training and test sets were swapped for crossvalidation and reanalyzed. Successful segmentation of kidneys was performed with the automated method in all test patients. The segmented kidney volumes ranged from 177.2 to 2634 ml (mean, 885.4±569.7 ml). The mean Dice similarity coefficient ±SD between the automated and manual methods was 0.88±0.08. The mean correlation coefficient between the two segmentation methods for the segmented volume measurements was 0.97 (P<0.001 for each crossvalidation set). The results from the crossvalidation sets were highly comparable. We have developed a fully automated method for segmentation of kidneys from abdominal magnetic resonance images in patients with autosomal dominant polycystic kidney disease with varying kidney volumes. The performance of the automated method was in good agreement with that of manual method.

Randomized Controlled Trial of Electronic Care Plan Alerts and Resource Utilization by High Frequency Emergency Department Users with Opioid Use Disorder.

IntroductionThere is a paucity of literature supporting the use of electronic alerts for patients with high frequency emergency department (ED) use. We sought to measure changes in opioid prescribing and administration practices, total charges and other resource utilization using electronic alerts to notify providers of an opioid-use care plan for high frequency ED patients.MethodsThis was a randomized, non-blinded, two-group parallel design study of patients who had 1) opioid use disorder and 2) high frequency ED use. Three affiliated hospitals with identical electronic health records participated. Patients were randomized into “Care Plan” versus “Usual Care groups”. Between the years before and after randomization, we compared as primary outcomes the following: 1) opioids (morphine mg equivalents) prescribed to patients upon discharge and administered to ED and inpatients; 2) total medical charges, and the numbers of; 3) ED visits, 4) ED visits with advanced radiologic imaging (computed tomography [CT] or magnetic resonance imaging [MRI]) studies, and 5) inpatient admissions.ResultsA total of 40 patients were enrolled. For ED and inpatients in the “Usual Care” group, the proportion of morphine mg equivalents received in the post-period compared with the pre-period was 15.7%, while in the “Care Plan” group the proportion received in the post-period compared with the pre-period was 4.5% (ratio=0.29, 95% CI [0.07–1.12]; p=0.07). For discharged patients in the “Usual Care” group, the proportion of morphine mg equivalents prescribed in the post-period compared with the pre-period was 25.7% while in the “Care Plan” group, the proportion prescribed in the post-period compared to the pre-period was 2.9%. The “Care Plan” group showed an 89% greater proportional change over the periods compared with the “Usual Care” group (ratio=0.11, 95% CI [0.01–0.092]; p=0.04). Care plans did not change the total charges, or, the numbers of ED visits, ED visits with CT or MRI or inpatient admissions.ConclusionElectronic care plans were associated with an incremental decrease in opioids (in morphine mg equivalents) prescribed to patients with opioid use disorder and high frequency ED use.

Laparoscopic Surgery for Meckel’s Diverticulum Presenting as Small Bowel Obstruction: A Case Report

Meckel’s diverticulum is not commonly encountered surgical entity and presents unique challenges for a pediatric surgeon, as it is prone to varied complications. A 14-year-old boy was admitted with us with a 48-hour history of lower abdominal pain and multiple episodes of vomiting. Radiological imaging studies revealed a high-grade partial small bowel obstruction. A fleeting conservative management was tried. The diagnostic laparoscopy revealed a small bowel obstruction secondary to a Meckel’s diverticulum. The diverticulum was resected using an endovascular GIA stapler. The patient was discharged on postoperative day four, tolerating a regular diet. Laparoscopy is a useful diagnostic and therapeutic means for a patient with a small bowel obstruction due to an uncertain etiology.

The risk of shorter fasting time for pediatric deep sedation.

Background:Current guidelines adopted by the American Academy of Pediatrics calls for prolonged fasting times before performing pediatric procedural sedation and analgesia (PSA). PSA is increasingly provided to children outside of the operating theater by sedation trained pediatric providers and does not require airway manipulation. We investigated the safety of a shorter fasting time compared to a longer and guideline compliant fasting time. We tried to identify the association between fasting time and sedation-related complications.Methods:This is a prospective observational study that included children 2 months to 18 years of age and had an American Society of Anesthesiologists physical status classification of I or II, who underwent deep sedation for elective procedures, performed by pediatric critical care providers. Procedures included radiologic imaging studies, electroencephalograms, auditory brainstem response, echocardiograms, Botox injections, and other minor surgical procedures. Subjects were divided into two groups depending on the length of their fasting time (4–6 h and >6 h). Complication rates were calculated and compared between the three groups.Results:In the studied group of 2487 subjects, 1007 (40.5%) had fasting time of 4–6 h and the remaining 1480 (59.5%) subjects had fasted for >6 h. There were no statistically significant differences in any of the studied complications between the two groups.Conclusions:This study found no difference in complication rate in regard to the fasting time among our subjects cohort, which included only healthy children receiving elective procedures performed by sedation trained pediatric critical care providers. This suggests that using shorter fasting time may be safe for procedures performed outside of the operating theater that does not involve high-risk patients or airway manipulation.

Reliability of Total Renal Volume Computation in Polycystic Kidney Disease From Magnetic Resonance Imaging

Total renal volume (TRV) is an important quantitative indicator of the progression of autosomal dominant polycystic kidney disease (ADPKD). The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease proposes a method for TRV computation based on manual tracing and geometric modeling. Alternative approaches for TRV computation are represented by the application of advanced image processing techniques. In this study, we aimed to compare TRV estimates derived from these two different approaches. The nearly automated technique for the analysis of magnetic resonance (MR) images was tested on 30 ADPKD patients. TRV was computed from both axial (KVax) and coronal (KVcor) acquisitions and compared to measurements based on geometric modeling (KVap) by linear regression and Bland-Altman analysis. In addition, to assess reproducibility, intraobserver and interobserver variabilities were computed. Linear regression analysis between KVax and KVcor resulted in an excellent correlation (KVax = 1KVcor - 0.78; r(2) = 0.997). Bland-Altman analysis showed a negligible bias and narrow limits of agreement (bias: -11.7 mL; SD: 54.3 mL). Similar results were obtained by comparison of volumes obtained applying the nearly automated method and the one based on geometric modeling (y = 0.98x + 75.9; r(2) = 0.99; bias: -53.7 mL; SD: 108.1 mL). Importantly, geometric modeling does not provide reliable TRV estimates in huge kidney affected by regional deformation. Intraobserver and interobserver variability resulted in very small percentage error <2%. The results of this study provide the feasibility of using a nearly automated approach for accurate and fast evaluation of TRV also in markedly enlarged ADPKD kidneys including exophytic cysts.

Geometry-independent assessment of renal volume in polycystic kidney disease from magnetic resonance imaging.

Total renal volume (TRV) is an important quantitative indicator of the progression of autosomal dominant polycystic kidney disease (ADPKD). The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease proposes a method for TRV computation based on manual tracing and geometric modeling. We developed a fast and nearly-automated technique for kidney segmentation and automatically compute TRV. In this study we aim to compare TRV estimates derived from these two different approaches. The highly-automated technique for the analysis of MR images was tested on 30 ADPKD patients. TRV was computed from both axial and coronal acquisitions, and compared to measurements based on geometric modeling by linear regression and Bland Altman analysis. In addition, to assess reproducibility, intra-observer and inter-observer variabilities were computed. The results of this study provide the feasibility of using a nearly-automated approach for accurate and fast evaluation of TRV also in markedly enlarged ADPKD kidneys.

Therapeutic Area Data Standards for Autosomal Dominant Polycystic Kidney Disease: A Report From the Polycystic Kidney Disease Outcomes Consortium (PKDOC)

Data standards provide a structure for consistent understanding and exchange of data and enable the integration of data across studies for integrated analysis. There is no data standard applicable to kidney disease. We describe the process for development of the first-ever Clinical Data Interchange Standards Consortium (CDISC) data standard for autosomal dominant polycystic kidney disease (ADPKD) by the Polycystic Kidney Disease Outcomes Consortium (PKDOC). Definition of common data elements and creation of ADPKD-specific data standards from case report forms used in long-term ADPKD registries, an observational cohort (Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease [CRISP] 1 and 2), and a randomized clinical trial (Halt Progression of Polycystic Kidney Disease [HALT-PKD]) are described in detail. This data standard underwent extensive review, including a global public comment period, and is now available online as the first PKD-specific data standard (www.cdisc.org/therapeutic). Submission of clinical trial data that use standard data structures and terminology will be required for new electronic submissions to the US Food and Drug Administration for all disease areas by the end of 2016. This data standard will allow for the mapping and pooling of available data into a common data set in addition to providing a foundation for future studies, data sharing, and long-term registries in ADPKD. This data set will also be used to support the regulatory qualification of total kidney volume as a prognostic biomarker for use in clinical trials. The availability of consensus data standards for ADPKD has the potential to facilitate clinical trial initiation and increase sharing and aggregation of data across observational studies and among completed clinical trials, thereby improving our understanding of disease progression and treatment.

A Comprehensive Review of Noninvasive Liver Fibrosis Tests in Pediatric Nonalcoholic Fatty Liver Disease

Nonalcoholic fatty liver disease (NAFLD) and its spectrum ranging from simple steatosis to nonalcoholic steatohepatitis (NASH) and fibrosis have been increasing in the pediatric population. The presence and severity of fibrosis in patients with NAFLD are important prognostic factors for the risk of disease progression to cirrhosis. The gold standard for staging liver fibrosis is a liver biopsy. However, given the risks of this procedure, especially in the pediatric population, the development of noninvasive markers to diagnose and monitor progression of NAFLD is desirable. This paper will review recently developed noninvasive methods for diagnosing liver fibrosis in children with NAFLD. These include simple fibrosis scores, advanced biochemical markers, and radiologic imaging studies. Simple fibrosis scores use readily available laboratory tests; available one include AST/ALT ratio, AST to platelet ratio index (APRI), fibrosis (FIB)-4 index, NAFLD fibrosis score (NFS), pediatric NAFLD fibrosis index (PNFI), and pediatric NALFD fibrosis score (PNFS). Advanced biochemical markers include biomarkers of hepatocyte cell death such as cytokeratin 18 fragment levels, and markers of extracellular matrix turnover such as the Enhanced Liver Fibrosis (ELF) test and hyaluronic acid. Radiologic imaging studies estimate liver stiffness as a surrogate for liver fibrosis; these include transient elastography (TE), magnetic resonance elastography (MRE), and acoustic radiation force impulse imaging (ARFI).

Jejunal web masquerading as intestinal malrotation in a neonate with bilious vomiting: A case report

Abstract Neonatal bilious vomiting is considered as a pathological sign and warrant urgent medical attention. Upper gastro-intestinal contrast study can be notoriously difficult to diagnose incomplete intestinal rotation, especially when there is partial hold up of the contrast at the duodenum. We report a case of a full term neonate with incomplete jejunal obstruction due to a fenestrated jejunal web, presenting with bilious vomiting and masquerading as intestinal malrotation in the contrast study. We discuss the recent literature and the potential diagnostic difficulty in the diagnosis of jejunal web. In view of the low specificity of radiological imaging study of jejunal web, clinicians should be aware that congenital gastro-intestinal webs are a possible cause of partial gastro-intestinal obstruction in infants and children.