The epidemiology of primary hepatic malignancies in U.S. children is poorly characterized. We analyzed the incidence, mortality, and characteristics of primary hepatic malignancies in U.S. residents less than 20 years of age. Fatal primary hepatic malignancies in persons less than 20 years of age, between 1979 and 1996, were identified using the multiple-cause-ofdeath database (National Center for Health Statistics). Histologically confirmed primary hepatic malignancies occurring between 1973 and 1997 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. Between 1979 and 1996, 918 primary hepatic malignancy deaths (average, 0.7/1,000,000/year) were reported nationally among persons less than 20 years of age; rates were higher among Asians and among foreignborn children. Between 1973 and 1997, 271 primary hepatic malignancy cases were reported to SEER among persons less than 20 years of age, of which 184 (67%) and 83 (31%) were hepatoblastoma and hepatocellular carcinoma, respectively. Among children less than 5 years of age, hepatoblastoma accounted for 91% of primary hepatic malignancy cases, whereas among those 15 to 19 years of age, hepatocellular carcinoma accounted for 87% of cases. Five-year survival for hepatoblastoma was 52%, compared with 18% for hepatocellular carcinoma. In the SEER sites, between 1973 and 1977 and 1993 and 1997, hepatoblastoma rates increased (0.6 to 1.2/1,000,000, respectively), while hepatocellular carcinoma rates decreased (0.45 to 0.29/1,000,000, respectively). In conclusion, histologically confirmed hepatocellular carcinoma was reported in children less than 5 years of age, also, where hepatoblastoma is the predominant primary hepatic malignancy. Hepatocellular carcinoma has worse survival rates than hepatoblastoma, and its incidence has not increased. Better maintenance of databases may provide information about associated factors behind this unexpected occurrence. (HEPATOLOGY 2003;38:560-566.) P rimary hepatic malignancies are rare in children. 1

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Pediatric patients with chronic upper gastrointestinal symptoms may have underlying orthostatic intolerance. In patients with upper gastrointestinal symptoms and orthostatic intolerance, treatment of orthostatic intolerance may result in resolution of gastrointestinal symptoms.

G-J feeding tubes are associated with the frequent need for tube maintenance and replacement and may not be the most feasible clinical option in providing long-term (>1 month) enteral access in children intolerant to gastrostomy tube feeds. Future studies are needed to develop innovative percutaneous jejunostomy tube placement techniques that facilitate long-term enteral access.

Leukodystrophies are a broad class of genetic disorders that result in disruption or destruction of central myelination. Although the mechanisms underlying these disorders are heterogeneous, there are many common symptoms that affect patients irrespective of the genetic diagnosis. The comfort and quality of life of these children is a primary goal that can complement efforts directed at curative therapies. Contained within this report is a systems-based approach to management of complications that result from leukodystrophies. We discuss the initial evaluation, identification of common medical issues, and management options to establish a comprehensive, standardized care approach. We will also address clinical topics relevant to select leukodystrophies, such as gallbladder pathology and adrenal insufficiency. The recommendations within this review rely on existing studies and consensus opinions and underscore the need for future research on evidence-based outcomes to better treat the manifestations of this unique set of genetic disorders.

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Background: Neurogastroenterology and motility (NGM) disorders are common and have a high health care burden. Although pediatric gastroenterology fellows are expected to obtain comprehensive training in the diagnosis and management of NGM disorders, there is ongoing concern for unmet training needs and lack of exposure in treating patients who suffer from NGM problems. Study Design: We conducted a cross-section survey of trainees listed as pediatric gastroenterology fellows in North American training programs in 2018 via direct e-mail and the pediatric gastroenterology listserv. Eighty-one pediatric gastroenterology fellows responded to the anonymous survey. Results: A total of 53.1% of the fellows reported interest in NGM, however, 75.1% of the fellows believed they had not been adequately trained in NGM during their fellowship. Eighty percent of fellows with two weeks or less of dedicated motility training reported that they received inadequate NGM training, compared to 46.2% fellows who received one or more months of dedicated motility training (p=0.0148). The majority of fellows reported not being comfortable in performing gastrointestinal (GI) motility studies. The majority of fellows also reported not being comfortable in interpreting GI motility studies. Conclusion: While most pediatric gastroenterology fellows expressed interest in NGM, the lack of exposure and dedicated training in motility during fellowship were identified as barriers to pursuing motility-focused careers. Furthermore, most fellows reported limited comfort with performing and/or interpreting motility studies. Changes are needed to encourage fellows to develop their interest and expertise in NGM.