J. Ted Gerstle

Plain abdominal radiography is the current standard imaging modality for evaluation of necrotizing enterocolitis (NEC). Sonography is still not routinely used for diagnosis and follow-up, as it is not widely recognized that it can provide information that is not provided by plain abdominal radiography and that may affect the management of NEC. Like plain abdominal radiography, sonography can depict intramural gas, portal venous gas, and free intraperitoneal gas. However, the major advantages of abdominal sonography over plain abdominal radiography are that it can depict intraabdominal fluid, bowel wall thickness, and bowel wall perfusion. Sonography may depict changes consistent with NEC when the plain abdominal radiographic findings are nonspecific and inconclusive. Thinning of the bowel wall and lack of perfusion at sonography are highly suggestive of nonviable bowel and may be seen before visualization of pneumoperitoneum at plain abdominal radiography. The mortality rate is higher after perforation; thus, earlier detection of severely ischemic or necrotic bowel loops, before perforation occurs, could potentially improve the morbidity and mortality in NEC. The information provided by sonography allows a more complete understanding of the state of the bowel in patients with NEC and may thus make management decisions easier and potentially change outcome.

BACKGROUND: Pain is one of the most common and distressing symptoms reported by adolescents with cancer. Despite advancements in pain assessment and management research, pain due to cancer and/or its treatments continues to be poorly managed. Our research group has developed a native iPhone application (app) called Pain Squad to tackle the problem of poorly managed pain in the adolescent with cancer group. The app functions as an electronic pain diary and is unique in its ability to collect data on pain intensity, duration, location, and the impact pain has on an adolescent's life (ie, relationships, school work, sleep, mood). It also evaluates medications and other physical and psychological pain management strategies used. Users are prompted twice daily at configurable times to complete 20 questions characterizing their pain and the app transmits results to a database for aggregate reporting through a Web interface. Each diary entry represents a pain case filed by an adolescent with cancer and a reward system (ie, moving up through law-enforcement team ranks, built-in videotaped acknowledgements from fictitious officers) encourages consistent use of the diary. OBJECTIVE: Our objective was to design, develop, and test the usability, feasibility, compliance, and satisfaction of a game-based smartphone pain assessment tool for adolescents with cancer. METHODS: We used both low- and high-fidelity qualitative usability testing with qualitative semi-structured, audio-taped interviews and iterative cycles to design and refine the iPhone based Pain Squad app. Qualitative thematic analysis of interviews using constant comparative methodology captured emergent themes related to app usability. Content validity was assessed using question importance-rating surveys completed by participants. Compliance and satisfaction data were collected following a 2-week feasibility trial where users were alarmed to record their pain twice daily on the app. RESULTS: Thematic analysis of usability interviews showed the app to be appealing overall to adolescents. Analyses of both low- and high-fidelity testing resulted in minor revisions to the app to refine the theme and improve its usability. Adolescents resoundingly endorsed the game-based nature of the app and its virtual reward system. The importance of app pain diary questions was established by content validity analysis. Compliance with the app, assessed during feasibility testing, was high (mean 81%, SD 22%) and adolescents from this phase of the study found the app likeable, easy to use, and not bothersome to complete. CONCLUSIONS: A multifaceted usability approach demonstrated how the Pain Squad app could be made more appealing to children and adolescents with cancer. The game-based nature and built-in reward system of the app was appealing to adolescents and may have resulted in the high compliance rates and satisfaction ratings observed during clinical feasibility testing.

Neuroblastoma is a heterogeneous pediatric tumor thought to arise from the embryonic neural crest. Identification of the cell responsible for propagating neuroblastomas is essential to understanding this often recurrent, rapidly progressing disease. We have isolated and characterized putative tumor-initiating cells from 16 tumors and bone marrow metastases from patients in all neuroblastoma risk groups. Dissociated cells from tumors or bone marrow grew as spheres in conditions used to culture neural crest stem cells, were capable of self-renewal, and exhibited chromosomal aberrations typical of neuroblastoma. Primary spheres from all tumor risk groups differentiated under neurogenic conditions to form neurons. Tumor spheres from low-risk tumors frequently formed large neuronal networks, whereas those from high-risk tumors rarely did. As few as 10 passaged tumor sphere cells from aggressive neuroblastoma injected orthotopically into severe combined immunodeficient/Beige mice formed large neuroblastoma tumors that metastasized to liver, spleen, contralateral adrenal and kidney, and lung. Furthermore, highly tumorigenic tumor spheres were isolated from the bone marrow of patients in clinical remission, suggesting that this population of cells may predict clinical behavior and serve as a biomarker for minimal residual disease in high-risk patients. Our data indicate that high-risk neuroblastoma contains a cell with cancer stem cell properties that is enriched in tumor-initiating capacity. These cells may serve as a model system to identify the molecular determinants of neuroblastoma and to develop new therapeutic strategies for this tumor.

BACKGROUND AND OBJECTIVE: The increasing use of sonography has resulted in an increase in the proportion of children with gallstones who are asymptomatic at the time of diagnosis. In adults, the literature supports expectant management of clinically silent gallstones. The evidence for this management approach in children is limited to a number of small series. Our objective was to review the risk factors, complications, and outcomes of gallstones at our institution, particularly in those patients who are asymptomatic at the time of initial diagnosis. MATERIALS AND METHODS: We reviewed 382 cases of gallstones in children. These patients were diagnosed with sonography. Data on age at diagnosis, presentation, sonographic findings, risk factors, complications, surgery, and follow-up were collected. A chi2 test was used to compare the complication rates between symptomatic and asymptomatic groups. Descriptive statistics were used to analyze the sample. RESULTS: At diagnosis, 50.5% of children were asymptomatic; these patients were diagnosed at a mean age of 8.23 years. Compared with symptomatic patients, they were less likely to have a hemolytic anemia but more likely to have other risk factors, including cardiac surgery, leukemia and lymphoma, short bowel syndrome, or exposure to total parenteral nutrition or cephalosporins. These patients had a lower rate of complications than the symptomatic patients (4.6% vs 28.2% of symptomatic, P < 0.0001) and only 3.1% developed symptoms that necessitated surgery (vs 59.0% of symptomatic). Of the 58 (15.1%) diagnosed in infancy, 47 (81.0%) were asymptomatic. The infant group also had low rates of complications (8.6%) and cholecystectomy (1.7%). In cases with sonographic follow-up, resolution of gallstones was demonstrated in 16.5% of asymptomatic patients and in 34.1% of infants. CONCLUSIONS: The data suggest that clinically silent gallstones in children and infants are associated with low rates of complications and can be managed conservatively, unless complications occur. Patients with sickle cell disease, spherocytosis, and elliptocytosis had high complication rates and required surgery more often.