Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease affecting multiple body systems with wide variability in presentation. In 2013, Pediatric Neurology published articles outlining updated diagnostic criteria and recommendations for surveillance and management of disease manifestations. Advances in knowledge and approvals of new therapies necessitated a revision of those criteria and recommendations. Methods: Chairs and working group cochairs from the 2012 International TSC Consensus Group were invited to meet face-to-face over two days at the 2018 World TSC Conference on July 25 and 26 in Dallas, TX, USA. Before the meeting, working group cochairs worked with group members via e-mail and telephone to (1) review TSC literature since the 2013 publication, (2) confirm or amend prior recommendations, and (3) provide new recommendations as required. Results: Only two changes were made to clinical diagnostic criteria reported in 2013: "multiple cortical tubers and/or radial migration lines" replaced the more general term "cortical dysplasias," and sclerotic bone lesions were reinstated as a minor criterion. Genetic diagnostic criteria were reaffirmed, including highlighting recent findings that some individuals with TSC are genetically mosaic for variants in TSC1 or TSC2. Changes to surveillance and management criteria largely reflected increased emphasis on early screening for electroencephalographic abnormalities, enhanced surveillance and management of TSCassociated neuropsychiatric disorders, and new medication approvals. Conclusions: Updated TSC diagnostic criteria and surveillance and management recommendations presented here should provide an improved framework for optimal care of those living with TSC and their families.

Abstract. Background: Patient satisfaction is an indicator of the quality of care provided by emergency department (ED) personnel. It is this perception of satisfaction that becomes the basis for future ED choice or the recommendation of a specific ED to other potential patients. Objective: To perform an evidence-based literature review to: 1) characterize measures of ''patient satisfaction''; 2) identify factors that have been associated with overall ED patient satisfaction; 3) critique the methods used to assess patient satisfaction in the literature; and 4) address how this information can be beneficial to those reading the satisfaction literature or designing a satisfaction survey instrument. Methods: The MEDLINE database was searched for studies addressing ED patient satisfaction, from January 1976 through July 1999, using MeSH terms and a text word search. Bibliographies of manuscripts also were searched for additional relevant articles and each clinical study was used as a search criterion in Science Citation Index, from the date of publication through July 1999. Results: Multiple measures have been used to evaluate overall patient satisfaction. Sixteen studies were found associating ED patient satisfaction with service and patient factors. Most studies are observational and of these, most are cross-sectional. Hence, causeand-effect determination of factors responsible for patient satisfaction cannot be resolved using the current literature. Conclusions: Despite considerable methodologic variability, key themes (e.g., association of satisfaction with patient information, provider-patient interpersonal factors, and perceived waiting time) emerge from review of the ED patient satisfaction literature. To standardize future investigations, clinicians and investigators should use a common definition for the state of overall patient satisfaction, e.g., when the patient's own expectations for treatment and care are met (or exceeded). This common definition should be incorporated into the instrument used to measure overall ED patient satisfaction.

The placenta is often overlooked in the routine evaluation of a normal gestation, receiving attention only when an abnormality is detected. Although uncommon, abnormalities of the placenta are important to recognize owing to the potential for maternal and fetal morbidity and mortality. Pathologic conditions of the placenta include placental causes of hemorrhage, gestational trophoblastic disease, retained products of conception, nontrophoblastic placental tumors, metastases, and cystic lesions. Sonography remains the imaging modality of choice for evaluation of the placenta. Magnetic resonance (MR) imaging can be of added diagnostic value when further characterization is required, particularly in the setting of invasive placental processes such as placenta accreta and gestational trophoblastic disease. Computed tomography (CT) has a limited role in the evaluation of placental disease owing to limited tissue characterization, compared with that of MR imaging, and the radiation risk to the fetus; this risk often outweighs the benefit. The primary role for CT is in the evaluation of trauma and gestational trophoblastic disease, for which it allows characterization of the primary lesion and distant metastases.

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Neuroblastoma is a common malignancy observed in infants and young children. It has a varied prognosis, ranging from spontaneous regression to aggressive metastatic tumors with fatal outcomes despite multimodality therapy. Patients are divided into risk groups on the basis of age, stage, and biologic tumor factors. Multiple clinical and imaging tests are needed for accurate patient assessment. Iodine 123 ((123)I) metaiodobenzylguanidine (MIBG) is the first-line functional imaging agent used in neuroblastoma imaging. MIBG uptake is seen in 90% of neuroblastomas, identifying both the primary tumor and sites of metastatic disease. The addition of single photon emission computed tomography (SPECT) and SPECT/computed tomography to (123)I-MIBG planar images can improve identification and characterization of sites of uptake. During scan interpretation, use of MIBG semiquantitative scoring systems improves description of disease extent and distribution and may be helpful in defining prognosis. Therapeutic use of MIBG labeled with iodine 131 ((131)I) is being investigated as part of research trials, both as a single agent and in conjunction with other therapies. (131)I-MIBG therapy has been studied in patients with newly diagnosed neuroblastoma and those with relapsed disease. Development and implementation of an institutional (131)I-MIBG therapy research program requires extensive preparation with a focus on radiation protection.

Purpose To prospectively assess agreement and repeatability of magnetic resonance (MR) elastography liver stiffness measurements across imager manufacturers, field strengths, and pulse sequences. Materials and Methods This prospective cross-sectional study was approved by the institutional review board; informed consent was obtained from all subjects. On the basis of an a priori power calculation, 24 volunteer adult subjects underwent MR elastography with four MR imaging systems (two vendors) and multiple pulse sequences (two-dimensional [2D] gradient-echo [GRE] imaging, 2D spin-echo [SE] echo-planar imaging, and three-dimensional [3D] SE echo-planar imaging). Each sequence was performed twice in each patient with each imaging system. Intraclass correlation coefficients (ICCs) were used to assess agreement and repeatability. P < .05 was considered indicative of a statistically significant difference. Results Pairwise ICCs were 0.67-0.82 and 0.62-0.83 for agreement between pulse sequences across manufacturers (n = 4) and field strengths (n = 5), respectively. ICCs were 0.45-0.90 for pairwise agreement between sequences while fixing manufacturer and field strength (n = 8). Test-retest repeatability across the various manufacturer, field strength, and pulse sequence combinations (n = 10) was excellent (ICCs, 0.77-0.94). The overall ICC for all manufacturer, field strength, and sequence combinations (n = 10) was 0.68 (95% confidence interval [CI]: 0.55, 0.82). ICC according to field strength was 0.78 (95% CI: 0.67, 0.88) at 1.5 T (n = 5) and 0.64 (95% CI: 0.49, 0.78) at 3.0 T (n = 5). ICCs according to vendor were 0.83 (95% CI: 0.73, 0.91) (n = 4) and 0.65 (95% CI: 0.51, 0.79) (n = 6). Average patient level variance was 0.042 kPa, with a coefficient of variation of 10.7%. Conclusion MR elastography is a reliable method for assessing liver stiffness, with small amounts of variability between imager manufacturers, field strengths, and pulse sequences. RSNA, 2016.

MRI-estimated PDFF has high diagnostic accuracy to both classify and predict histological steatosis grade and change in histological steatosis grade in children with NAFLD. (Hepatology 2018;67:858-872).

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