Update on the Role of Imaging in Staging of Common Pediatric Abdominal Tumors.

Neuroblastoma, Wilms tumor, and hepatoblastoma are the most common pediatric abdominal malignancies. Management of these diseases is a multidisciplinary process that continues to evolve based on the results of international collaborative trials and advances in understanding of tumor biology. Each of these tumors has unique characteristics and behavior which are reflected in their respective staging systems. It is important for clinicians involved in the care of children with abdominal malignancies to be familiar with current staging guidelines and imaging recommendations. This article reviews the current role of imaging in the management of these common pediatric abdominal malignancies, with emphasis on initial staging.

A trial of the AASPIRE healthcare toolkit with Australian adults on the autism spectrum.

BACKGROUND: Autistic adults experience barriers to accessing health care, such as service provider communication not meeting their needs, healthcare facilities causing sensory discomfort and feeling fear or anxiety regarding their healthcare visit. The Academic Autism Spectrum Partnership in Research and Education (AASPIRE) developed and trialled an online healthcare toolkit to reduce such barriers and improve healthcare interactions between autistic adults and their primary care providers in the United States. This preliminary study aimed to explore experiences of autistic adults using the AASPIRE Healthcare Toolkit in Australia. METHODS: Semi-structured interviews were conducted with six autistic adults about their experiences and perceptions of utilising the toolkit in an Australian healthcare setting. RESULTS: Participants identified that the toolkit facilitated their interactions with health professionals by providing structure to appointments, supplementing new knowledge and increasing individual confidence. They also offered suggestions to tailor the toolkit for use in Australia. CONCLUSIONS: Future research should seek to explore the experiences of autistic adults using a version of the toolkit adapted for Australian use, as well as exploring the views of health professionals utilising it.

Partially intraosseous schwannoma of the distal humerus with increased enhancement after biopsy: Radiologic-pathologic correlation.

Intraosseous schwannomas are rare benign tumors that most often occur at the mandible or sacrum. We present an unusual case of a bilobed schwannoma of the distal humerus with both intraosseous and extraosseous components. The extraosseous component was non-enhancing on initial MRI and enhanced on a subsequent MRI obtained after biopsy. We hypothesize that this change was attributable to decreased intra-tumoral pressure secondary to biopsy-related disruption of the tumor capsule.

Pharmacokinetics and Pharmacodynamics of Ropeginterferon Alfa-2b in Healthy Japanese and Caucasian Subjects After Single Subcutaneous Administration.

BACKGROUND AND OBJECTIVES: Ropeginterferon alfa-2b is a novel monopegylated recombinant interferon alfa-2b for the treatment of patients with polycythemia vera. The objectives of this study were to evaluate the pharmacokinetics, pharmacodynamics, safety, and tolerability of ropeginterferon alfa-2b in healthy Japanese subjects compared with Caucasian subjects. METHODS: In this multicenter, parallel-group phase I study, a cohort consisting of six Japanese and six Caucasian subjects was designated to receive a single subcutaneous dose of ropeginterferon alfa-2b (100, 200, 300, and 450 µg). Pharmacokinetic and pharmacodynamic parameters, and immunogenicity were evaluated. Safety was assessed throughout the study. RESULTS: Cohort 4 (450-µg dose) was not initiated because the primary objective of this study was achieved based on the three completed cohorts. A total of 36 enrolled subjects (18 Japanese and 18 Caucasian) in three cohorts were included in the safety, pharmacokinetic, and pharmacodynamic analysis sets. Ropeginterferon alfa-2b exposure in terms of the area under the serum concentration-time curve (AUC) from time zero extrapolated to infinity and the AUC from time zero to the time of the last quantifiable concentration was approximately 1.7-fold and two-fold higher in Japanese subjects than in Caucasian subjects, respectively. Across the same dose range, the maximum serum concentration was approximately 1.25-fold higher in Japanese subjects than in Caucasian subjects. The time to reach the median maximum serum concentration was similar between ethnicities (approximately 96-111 h). The terminal half-life was 48-57 h in Japanese subjects and 31-75 h in Caucasian subjects. The slope of the relationship between dose and drug exposure was greater than 1 in both ethnicities. The dose-dependent induction of beta-2 microglobulin and neopterin expression was observed in both ethnicities, and the two groups showed similar pharmacodynamic parameters. At the end of the study, 22.2% of Japanese subjects and 11.1% of Caucasian subjects developed anti-ropeginterferon alfa-2b-binding antibodies. The neutralizing capacity of these antibodies was not tested. Ropeginterferon alfa-2b up to 300 µg was safe and well tolerated, with no unexpected safety findings based on previous experiences with ropeginterferon alfa-2b and other forms of interferon. CONCLUSIONS: Ropeginterferon alfa-2b exposure was higher in Japanese subjects than in Caucasian subjects. The increase in ropeginterferon alfa-2b exposure was greater than the dose proportion in the dose range of 100-300 µg. Ropeginterferon alfa-2b was safe and well tolerated. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT03546465, registered on 6 June, 2018.

Primary Septic Arthritis Among Children 6 to 48 Months of Age: Implications for PCR Acquisition and Empiric Antimicrobial Selection.

INTRODUCTION: Primary septic arthritis requires unique evaluation and treatment considerations for children in the 6- to 48-month age range because of the spectrum of identified pathogens and high rate of negative cultures. The purpose of this study is to evaluate primary septic arthritis in this age group in order to differentiate children with infection caused by Kingella kingae from those with other confirmed pathogens and those with no identified pathogen. METHODS: Preschool children who underwent multidisciplinary evaluation and treatment for septic arthritis between 2009 and 2019 were retrospectively studied. Three cohorts were established for comparison of clinical and laboratory features of primary septic arthritis: (1) confirmed K. kingae, (2) confirmed other pathogen, and (3) presumed (without identified pathogen). RESULTS: Among 139 children with septic arthritis, 40 (29%) were confirmed K. kingae, 29 (21%) other pathogen, and 70 (50%) presumed. Children with Kingella and those with presumed septic arthritis had significantly lower initial C-reactive protein (4.8 and 4.5 vs. 9.3 mg/dL) and fewer febrile hospital days (0.2 and 0.4 vs. 1.3 d) than children with other confirmed pathogens. Children with other pathogens had higher rates of bacteremia (38% vs. 0%) and positive joint fluid cultures (86% vs. 15%) than that of children with Kingella. The rate of polymerase chain reaction (PCR) acquisition was 38 of 40 (95.0%) Kingella cases, 18 of 29 (62.1%) other pathogen cases, and 33 of 70 (47.1%) presumed cases. CONCLUSIONS: K. kingae was the most commonly identified pathogen among 6-month to 4-year-old children. The Kingella and other identified pathogens in this study serve to guide empiric antimicrobial recommendations for this age range. Because of similarities between children with septic arthritis because of K. kingae and those with no identified pathogen, it is likely that an unrecognized burden of Kingella resides in culture negative cases, particularly if no PCR is sent. Systematic evaluation, including PCR acquisition, and a high index of suspicion for K. kingae are recommended to thoroughly evaluate septic arthritis in preschool children. LEVEL OF EVIDENCE: Level III-Retrospective cohort comparison.

Endobronchial Paraganglioma: AIRP Best Cases in Radiologic-Pathologic Correlation.

Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).

Pediatric Biliary Interventions in the Native Liver.

Biliary disease in infants and children frequently presents diagnostic and therapeutic challenges. Pediatric interventional radiologists are often involved in the multidisciplinary teams who care for these patients. This article reviews several notable causes of biliary disease in children who have not undergone liver transplantation, describes the role of percutaneous interventional procedures in managing these conditions, and details applicable biliary interventional techniques.

Effect of prognostic classification on temsirolimus efficacy and safety in patients with relapsed or refractory mantle cell lymphoma: a retrospective analysis.

BACKGROUND: Temsirolimus, a selective inhibitor of the mammalian target of rapamycin, has demonstrated clinical benefit versus investigator's choice (INV) of therapy in patients with relapsed/refractory mantle cell lymphoma (MCL). METHODS: This post hoc study retrospectively assigned simplified Mantle Cell Lymphoma International Prognostic Index (MIPI) scores (ie, secondary MIPI) based on parameters at the time of randomization in patients with MCL (N = 162) who received temsirolimus 175 mg once weekly for 3 weeks followed by once-weekly 75 mg or 25 mg or the INV of active therapy. Outcomes were analyzed according to the low-, intermediate- or high-risk category. RESULTS: Patient distribution by MIPI risk category was 31%, 39%, and 30% in the low-, intermediate-, and high-risk groups, respectively. Among patients in all categories, objective response rate (complete response + partial response) was higher in patients in the temsirolimus 175/75-mg group versus the INV group, respectively: 42% versus 0% (low-risk); 33% versus 5% (intermediate-risk); 10% versus 0% (high-risk). Median progression-free survival was significantly longer with temsirolimus 175/75 mg versus INV, respectively, in patients with intermediate (4.3 vs 1.9 months; P = 0.035) or high (4.5 vs 1.6 months; P = 0.0025) risk, and a trend toward improvement was observed in patients with low risk (5.3 vs 2.6 months; P = 0.091). Improvement in median overall survival was observed with temsirolimus 175/75 mg versus INV in low-risk patients (18.0 vs 10.5 months, respectively; P = 0.069). CONCLUSIONS: This analysis suggests that, compared with INV, temsirolimus demonstrated benefit in all MIPI risk categories in patients with MCL. In all treatment groups, patients with high secondary MIPI scores at baseline faced a dismal prognosis. TRIAL REGISTRATION: ClinicalTrials.gov NCT00117598.

Difficult OptEase filter retrievals after prolonged indwelling times.

PURPOSE: The OptEase vena cave filter (Cordis, Piscataway, NJ) is commercially available as a retrievable or permanent filter with short recommended indwelling time, presumably due to extensive contact of the filter side struts with the inferior vena cava wall and subsequent neointimal hyperplasia leading to incorporation. Our purpose was to evaluate OptEase filter retrievals with a long indwelling time period that required unconventional retrieval techniques. MATERIALS AND METHODS: We retrospectively reviewed patients who underwent OptEase filter retrieval with long undwelling times requiring additional maneuvers for retrieval. Techniques used included rigid endobronchial forceps dissection and wire-through-loop snare. Each patient underwent postretrieval venogram to evaluate for possible complications. In addition, patients had clinical follow-up 2 weeks after the retrieval procedure. RESULTS: There were three patients (2 women, 1 man; average age 64 years) who underwent OptEase filter retrieval. The mean indwelling time was 6.4 months. The indwelling filters were successfully retrieved. There were no complications. Postprocedural follow-up showed no clinical pathology. CONCLUSION: Unconventional techniques aided in the retrieval of OptEase filters with long indwelling times.

Percutaneous drainage of abdominal and pelvic abscesses in children.

It has only been in the last several decades that abscesses within deep compartments, particularly within the abdomen and pelvis, have become safely accessible with imaging guidance. Since that time, percutaneous abscess drainage has become the standard of care in children. We review the clinical features, diagnosis, and image-guided management of abdominal and pelvic abscesses in children.

Role of interventional radiology in the emergent management of acute upper gastrointestinal bleeding.

Approximately 100,000 cases of upper gastrointestinal bleeding (UGIB) require inpatient admission annually in the United States. When medical management and endoscopic therapy are inadequate, endovascular intervention can be lifesaving. These emergent situations highlight the importance of immediate competence of the interventional radiologist in the preangiographic evaluation as well as the endovascular treatment of UGIB. We describe a case of UGIB managed with endovascular embolization and detail the angiographic techniques used. The case description is followed by a detailed discussion of the treatment approach to UGIB, with attention to both nonvariceal and variceal algorithms.

Acute lower gastrointestinal bleeding.

The annual incidence of lower gastrointestinal bleeding (LGIB) is ∼20.5 in 100,000 in the general Western population and results in 1 to 2% of hospital emergencies. When medical management and endoscopic therapy are inadequate in cases of acute LGIB, endovascular intervention can be lifesaving. In these emergent situations it is important for the interventional radiologist to be well versed in the multidisciplinary preangiographic work-up, the angiographic presentations of LGIB, and the endovascular therapeutic options. We describe a case of LGIB managed with endovascular embolization and detail the angiographic techniques used, followed by a detailed discussion of the various treatment approaches to LGIB.

Assessment of retromesenteric position of the third portion of the duodenum: an US feasibility study in 33 newborns.

BACKGROUND: US can be used to assess bowel and does not require ionizing radiation or the administration of contrast material. Prior studies of the duodenum with US are limited. OBJECTIVE: This study assesses the success rate of US demonstration of the third portion of the duodenum (D3) between the superior mesenteric artery (SMA) and the aorta in newborns to exclude malrotation based on embryologic and anatomic principles. MATERIAL AND METHODS: Thirty-three newborns underwent US studies. The structures between the SMA and the aorta, including D3, were evaluated in axial and longitudinal planes. The length of time to acquire diagnostic images was recorded. RESULTS: In both the axial and longitudinal planes, D3 was seen between the SMA and the aorta in all 33 infants, including some with abundant bowel gas. The mean length of time to acquire diagnostic images was 34 s. CONCLUSION: Bedside US successfully illustrated the retromesenteric position of D3 in all 33 infants. Overlying gas-filled bowel was effectively effaced by graded compression. The short study duration indicates the practicality of the method. Further studies in broader patient populations and in correlation with other imaging and/or surgical findings is required to validate our technique.

Luminal bacterial flora determines physiological expression of intestinal epithelial cytoprotective heat shock proteins 25 and 72.

Heat shock proteins (HSP) 25 and 72 are expressed normally by surface colonocytes but not by small intestinal enterocytes. We hypothesized that luminal commensal microflora maintain the observed colonocyte HSP expression. The ability of the small intestine to respond to bacteria and their products and modulate HSPs has not been determined. The effects of luminal bacterial flora in surgically created midjejunal self-filling (SFL) vs. self-emptying (SEL) small-bowel blind loops on epithelial HSP expression were studied. HSP25 and HSP72 expression were assessed by immunoblot and immunohistochemistry. SFL were chronically colonized, whereas SEL contained levels of bacteria normal for the proximal small intestine. SFL creation significantly increased HSP25 and HSP72 expression relative to corresponding sections from SEL. Metronidazole treatment, which primarily affects anaerobic bacteria as well as a diet lacking fermentable fiber, significantly decreased SFL HSP expression. Small bowel incubation with butyrate ex vivo induced a sustained and significant upregulation of HSP25 and altered HSP72 expression, confirming the role of short-chain fatty acids. To determine whether HSPs induction altered responses to an injury, effects of the oxidant, monochloramine, on epithelial resistance and short-circuit current (I(sc)) responses to carbachol and glucose were compared. Increased SFL HSP expression was associated with protection against oxidant-induced decreases in transmural resistance and I(sc) responses to glucose, but not secretory responses to carbachol. In conclusion, luminal microflora and their metabolic byproducts direct expression of HSPs in gut epithelial cells, an effect that contributes to preservation of epithelial cell viability under conditions of stress.