Availability and utilization of endoscopic retrograde cholangiopancreatography at children's hospitals.

OBJECTIVES: No study has explored whether availability of endoscopic retrograde cholangiopancreatography (ERCP) is adequate and equitable across US children's hospitals. We hypothesized that ERCP availability and utilization differs by geography and patient factors. METHODS: Healthcare encounter data from 2009 to 2019 on children with pancreatic and biliary diseases from the Pediatric Health Information System were analyzed. ERCP availability was defined as treatment at a hospital that performed pediatric ERCP during the year of service. RESULTS: From 2009 to 2019, 37,946 children (88,420 encounters) had a potential pancreatic or biliary indication for ERCP; 7066 ERCPs were performed. The commonest pancreatic diagnoses leading to ERCP were chronic (47.2%) and acute pancreatitis (43.2%); biliary diagnoses were calculus (68.3%) and obstruction (14.8%). No ERCP was available for 25.0% of pancreatic encounters and 8.1% of biliary encounters. In multivariable analysis, children with public insurance, rural residence, or of Black race were less likely to have pancreatic ERCP availability; those with rural residence or Asian race were less likely to have biliary ERCP availability. Black children or those with public insurance were less likely to undergo pancreatic ERCP where available. Among encounters for calculus or obstruction, those of Black race or admitted to hospitals in the West were less likely to undergo ERCP when available. CONCLUSIONS: One-in-four children with pancreatic disorders and one-in-12 with biliary disorders may have limited access to ERCP. We identified racial and geographic disparities in availability and utilization of ERCP. Further studies are needed to understand these differences to ensure equitable care.

Comparison of the demographic characteristics of pediatric and adult colorectal cancer patients: a national inpatient sample based analysis.

BACKGROUND: Colorectal cancer in children is rare, but characterized by late presentation, unfavorable histology and poor prognosis. Risk factors for colorectal cancer in children overlap with those for adults with greater influence of hereditary syndromes. The epidemiology of colon cancer in children is poorly understood; the aim of this study was to characterize and compare the demographics and relevant clinical characteristics of pediatric and adult colon cancer, using a national inpatient sample. METHODS: The AHRQ online resource HCUPnet/KID database was queried for children, under the age of 18 admitted with ICD 9 CM diagnoses relating to colorectal cancer, at the time of discharge. For comparison, the corresponding diagnoses in adult patients were queried for each successive year. Patient demographics including residential type and median income by zip-code, tumor localization; if recorded, and mean hospital charges were all accrued and analyzed. RESULTS: Inpatient admissions for pediatric colorectal cancer were more likely male (54%), in the 15-17 years age bracket (57%). They were significantly more likely from Southwestern regions of the Unites States, and were significantly more likely from residential zip-codes identified as at or below the lowest income quartile than adult CRC patients or pediatric patients as a whole. Hospital charges have more than quadrupled over the time period studied (1997-2012). CONCLUSIONS: Pediatric colorectal cancer is the most common primary gastrointestinal malignancy in children. Better understanding associated risk factors including those associated with gender, geographic region and social economic status may contribute to future prevention or early detection strategies.

Familial adenomatous polyposis in pediatrics: natural history, emerging surveillance and management protocols, chemopreventive strategies, and areas of ongoing debate.

Familial adenomatous polyposis (FAP) is a hereditary condition with a near 100 % lifetime risk of colorectal cancer without prophylactic colectomy. Most patients with FAP have a mutation in the adenomatous polyposis coli gene on chromosome 5q22. This condition frequently presents in children with polyps developing most frequently in the second decade of life and surveillance colonoscopy is required starting at age ten. Polyps are found not only in the colon, but in the stomach and duodenum. Knowledge of the natural history of FAP is important as there are several extra-colonic sequelae which also require surveillance. In infants and toddlers, there is an increased risk of hepatoblastoma, while in teenagers and adults duodenal carcinomas, desmoid tumors, thyroid cancer and medulloblastoma are more common in FAP than in the general population. Current chemopreventive strategies include several medications and natural products, although to this point there is no consensus on the most efficacious and safe agent. Genetic counseling is an important part of the diagnostic process for FAP. Appropriate use and interpretation of genetic testing is best accomplished with genetic counselor involvement as many families also have concerns regarding future insurability or discrimination when faced with genetic testing.

Economic Assessment of Supercritical CO2 Extraction of Waxes as Part of a Maize Stover Biorefinery.

To date limited work has focused on assessing the economic viability of scCO2 extraction to obtain waxes as part of a biorefinery. This work estimates the economic costs for wax extraction from maize stover. The cost of manufacture (COM) for maize stover wax extraction was found to be € 88.89 per kg of wax, with the fixed capital investment (FCI) and utility costs (CUT) contributing significantly to the COM. However, this value is based solely on scCO2 extraction of waxes and does not take into account the downstream processing of the biomass following extraction. The cost of extracting wax from maize stover can be reduced by utilizing pelletized leaves and combusting the residual biomass to generate electricity. This would lead to an overall cost of € 10.87 per kg of wax (based on 27% combustion efficiency for electricity generation) and €4.56 per kg of wax (based on 43% combustion efficiency for electricity generation). A sensitivity analysis study showed that utility costs (cost of electricity) had the greatest effect on the COM.

Present Standard in Pediatric Gastroenterology Fellowship Training in the Interpretation of Capsule Endoscopy.

Consensuses on fellowship training in wireless capsule endoscopy (WCE) interpretation have been published for adult gastroenterology (GI) but not in pediatric GI training. A questionnaire has been sent to 64 pediatric and 45 adult GI fellowship programs to compare their present training approach. Adult GI programs reported having a formal GI capsule endoscopy module in 38% and required to attend hands-on course in 27% as compared with 4% and 8% in pediatric programs, respectively. A more formalized approach to WCE training may be required for credentialing pediatric trainees to be aligned with expectations in adult GI programs.

Burden of celiac disease in the Mediterranean area.

AIM: To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of morbidity, mortality and health cost. METHODS: For statistics regarding the population of each country in the Mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available confidence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic profiles amongst Mediterranean countries. RESULTS: In the next 10 years, the Mediterranean area will have about half a billion inhabitants, of which 120 million will be children. The projected number of CD diagnoses in 2020 is 5 million cases (1 million celiac children), with a relative increase of 11% compared to 2010. Based on the 2010 rate, there will be about 550,000 symptomatic adults and about 240,000 sick children: 85% of the symptomatic patients will suffer from gastrointestinal complaints, 40% are likely to have anemia, 30% will likely have osteopenia, 20% of children will have short stature, and 10% will have abnormal liver enzymes. The estimated standardized medical costs for symptomatic celiac patients during the delay between symptom onset and diagnosis (mean 6 years for adults, 2 years for children) will be about €4 billion (€387 million for children) over the next 10 years. A delay in diagnosis is expected to increase mortality: about 600,000 celiac patients will die in the next 10 years, with an excess of 44.4% vs age- and sex-matched controls. CONCLUSION: In the near future, the burden of CD will increase tremendously. Few Mediterranean countries are able to face this expanding epidemic alone.

A web-based assessment of pediatrics resident medical knowledge in childhood hereditary gastrointestinal cancer predisposing syndromes.

BACKGROUND: Pediatricians need to competently care for children with hereditary gastrointestinal cancer-predisposing syndromes. Pediatrics resident education on this subject has hithertofore never been studied. METHODS: Forty-five US pediatrics/internal medicine-pediatrics program directors allowed their residents to participate in an anonymous questionnaire. The survey-questionnaire was administered as a hyperlinked interactive Web page through e-mail to all consenting residents. RESULTS: Thirty-eight sites including 290 of 1327 residents (21.5%) and 33 internal medicine-pediatrics participated in the study. Knowledge on polyposis syndromes varied by syndrome (14% to 84% correct) but not by year of training. Internal medicine-pediatrics residents were more likely to inquire on family history including polyposis, early colorectal cancer, than pediatrics residents. CONCLUSIONS: This study suggests that familiarity with cancer syndromes does not accrue during resident training in pediatrics. The observations suggest that greater emphasis on resident education on these syndromes may improve outcomes in this vulnerable group.

Hereditary colorectal cancer syndromes: molecular genetics, genetic counseling, diagnosis and management.

Hereditary forms of colorectal cancer, as is the case with virtually all forms of hereditary cancer, show extensive phenotypic and genotypic heterogeneity, a phenomenon discussed throughout this special issue of Familial Cancer. Clearly, the family physician, oncology specialist, genetic counselor, and cancer geneticist must know fully the complexity of hereditary cancer syndromes, their differential diagnosis, in order to establish a diagnosis, direct highly-targeted surveillance and management, and then be able to communicate effectively with the molecular geneticist so that an at-risk patient's DNA can be tested in accord with the syndrome of concern. Thus, a family with features of the Lynch syndrome will merit microsatellite instability testing, consideration for immunohistochemistry evaluation, and mismatch repair gene testing, while, in contrast, a patient with FAP will require APC testing. However, other germline mutations, yet to be identified, may be important should testing for these mutations prove to be absent and, therein, unrewarding to the patient. Nevertheless, our position is that if the patient's family history is consistent with one of these syndromes, but a mutation is not found in the family, we still recommend the same surveillance and management strategies for patients from families with an established cancer-causing germline mutation. Our purpose in this paper is to provide a concise coverage of the major hereditary colorectal cancer syndromes, to discuss genetic counseling, molecular genetic evaluation, highly targeted surveillance and management, so that cancer control can be maximized for these high hereditary cancer risk patients.