Objective To estimate selected direct medical care costs of children with chronic pancreatitis (CP) and acute recurrent pancreatitis (ARP). Methods We performed a cross-sectional study of data from INSPPIRE (International Study Group of Pediatric Pancreatitis: In search for a cuRE), a multinational registry of children with ARP or CP. We determined health care utilization and estimated costs of hospitalizations, surgical and endoscopic procedures, and medications in our study population. Health care utilization data were obtained from all subjects enrolled in the study, and costs were calculated using national United States costs. Results We included 224 subjects (median age 12.7 years), 42% of whom had CP. Mean number of hospitalizations, including for surgery and endoscopic retrograde cholangiopancreatography (ERCP), was 2.3/person/year, costing an estimated average $38,755/person/year. Including outpatient medications, estimated total mean cost was $40,589/person/year. Subjects utilizing surgical procedures or ERCP incurred mean annual costs of $42,951/person and $12,035/person, respectively. Estimated annual costs of pancreatic enzyme replacement therapy, diabetic medications, and pain medications were $4,114, $1,761, and $614 per person, respectively. In an exploratory analysis, patients with the following characteristics appear to accrue higher costs than those without them: more frequent ARP attacks/year, reported constant or episodic pain, family history of pancreatic cancer, and use of pain medication. Conclusions ARP and CP are uncommon childhood conditions. The severe burden of disease associated with these conditions and their chronicity results in high healthcare utilization and costs. Interventions that reduce the need for hospitalization could lower costs for these children and their families.

Objectives To systematically review risks and summarize reported complication rates associated with performance of ERCP in children over the past two decades. Study design A systematic literature search of MEDLINE, Embase, and Web of Science from Jan 1995 to Jan 2016 was conducted for observational studies published in English. Studies reporting ERCP complications in patients <21 years without history of liver transplant or cholecystectomy were included. A summary estimate of the proportion of children who experienced complications following ERCP was derived using a random effects meta-analysis. Results Thirty-two studies involving 2612 children and 3566 procedures were included. Subjects’ ages ranged from 3 days to 21 years. Procedures were performed for biliary (54%), pancreatic (38%), and other (8%) indications. 56% of ERCPs were interventional. The pooled complication rate was 6% (95% CI: 4%– 8%). Procedural complications included post-ERCP pancreatitis (166, 4.7%), bleeding (22, 0.6%) and infections (27, 0.8%). The pooled estimate of post-ERCP pancreatitis was 3% (95%CI 0.02–0.05), and other complications were 1% (95%CI: 0.02–0.05). In neonatal cholestasis subgroup the pooled complication rate was 3% (95% CI: 0.01–0.07). Adult and pediatric gastroenterologists and surgeons performed the ERCPs. Available data limited the ability to report differences between pediatric-trained and other endoscopists. Conclusions Complications associated with pediatric ERCP range widely in severity and are reported inconsistently. Our review suggests 6% of pediatric ERCPs have complications. Further studies using systematic and standardized methodologies are needed to determine the frequency and risk factors for ERCP related complications.

Despite a high prevalence of pancreatic endocrine and exocrine insufficiency in cystic fibrosis (CF), pancreas transplantation is rarely reported. United Network for Organ Sharing (UNOS) data were used to examine utilization of pancreas transplant and posttransplant outcomes in CF patients. Between 1987–2014, CF patients (N = 4600) underwent 17 liver–pancreas, three lung–pancreas, one liver–lung pancreas, four kidney–pancreas, and three pancreas-only transplants. Of the 303 CF patients who received liver transplantation, 20% had CF-related diabetes (CFRD) before transplantation, and nine of those received a liver–pancreas transplant. Of 4241 CF patients who underwent lung transplantation, 33% had CFRD before transplantation, and three of those received a pancreas transplant. Of 49 CF patients who received a liver–lung transplant, 57% had CFRD before transplantation and one received a pancreas transplant. Posttransplantation diabetes developed in 7% of CF pancreas transplant recipients versus 24% of CF liver and 29% of CF lung recipients. UNOS has no data on pancreas exocrine insufficiency. Two-year post-transplantation survival was 88% after liver–pancreas transplant, 33% after lung–pancreas transplant, and 100% after pancreas–kidney and pancreas-only transplants. Diabetes is common pretransplantation and posttransplantation in CF solid organ transplant recipients, but pancreas transplantation remains rare. Further consideration of pancreas transplant in CF patients undergoing other solid organ transplant may be warranted.

Objectives Pancreatitis in children can result from metabolic and toxic risk factors, but the evidence linking these factors is sparse. We review the evidence for association or causality of these risk factors in pancreatitis, discuss management strategies and their rationale. Methods We conducted a review of the pediatric pancreatitis literature with respect to the following risk factors: (a) hyperlipidemia, (b) hypercalcemia, (c) chronic renal failure, (d) smoking exposure, (e) alcohol, and (f) medications. Areas of additional research were identified. Results Hypertriglyceridemia of 1000 mg/dl or greater poses an absolute risk for pancreatitis; persistent elevations of calcium are predisposing. Further research is necessary to determine whether end stage renal disease leads to increased pancreatitis in children similar to adults. It is unknown whether cigarette smoking exposure, which clearly increases risk in adults, also increases risk in children. The role of alcohol in pediatric pancreatitis, whether direct or modifying, needs to be elucidated. The evidence supporting most cases of medication-induced pancreatitis is poor. Drug structure, improper handling of drug by host, and by-stander status may be implicated. Other pancreatitis risk factors must be sought in all cases. Conclusions The quality of evidence supporting causative role of various toxic and metabolic factors in pediatric pancreatitis is variable. Careful phenotyping is essential, including search for other etiologic risk factors. Directed therapy includes correction/ removal of any agent identified, and general supportive measures. Further research is necessary to improve our understanding of these pancreatitis risk factors in children.

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Children with CF who achieve higher WFL at age two years have improved pulmonary and survival outcomes into adulthood. CF providers should continue to utilize current early growth recommendations, with goal WFL ≥50th percentile on CDC growth charts before age two.

Surgical management of children with short bowel syndrome (SBS) changed with the introduction of the serial transverse enteroplasty procedure (STEP). We conducted a systematic review and meta-analysis using MEDLINE and SCOPUS to determine if children with SBS had improved enteral tolerance following STEP. Studies were included if information about a child's pre- and post-STEP enteral tolerance was provided. A random effects meta-analysis provided a summary estimate of the proportion of children with enteral tolerance increase following STEP. From 766 abstracts, seven case series involving 86 children were included. Mean percent tolerance of enteral nutrition improved from 35.1 to 69.5. Sixteen children had no enteral improvement following STEP. A summary estimate showed that 87 % (95 % CI 77-95 %) of children who underwent STEP had an increase in enteral tolerance. Compilation of the literature supports the belief that SBS subjects' enteral tolerance improves following STEP. Enteral nutritional tolerance is a measure of efficacy of STEP and should be presented as a primary or secondary outcome. By standardizing data collection on children undergoing STEP procedure, better determination of nutritional benefit from STEP can be ascertained.

Objective The objective of this study was to determine whether either hyperbilirubinemia or inpatient phototherapy is associated with increased subsequent outpatient visit rates, a possible effect of the “vulnerable child syndrome.” Methods We compared 3 groups of otherwise well term and late-preterm infants who were born between 1995 and 2004 in Northern California Kaiser hospitals: group 1 never had a documented total serum bilirubin (TSB) level ≥12 mg/dL (n = 128 417); group 2 had a TSB level ≥17 and <23 mg/dL as outpatients between 48 hours and 7 days of age and did not receive inpatient phototherapy (n = 6777); and group 3 met criteria for group 2 but did receive inpatient phototherapy (n = 1765). We compared outpatient visit rates from 15 to 364 days of age adjusting for other predictors of visit rates by using Poisson and linear regression. Results The mean total number of visits between 15 and 364 days was 9.83. Compared with group 1, adjusted total first-year visit rates were slightly increased in group 2 (adjusted incidence rate ratio: 1.04 [95% confidence interval: 1.02–1.05]) and group 3 (incidence rate ratio: 1.07 [95% confidence interval: 1.05–1.10]). The increases in visit rates were greatest for visits from 15 to 59 days of age, for specialty visits, and for unspecified diagnoses. These rates correspond to adjusted increases in total first-year visits (compared with group 1) of 0.36 visits in group 2 and 0.73 visits in group 3. Conclusions Neonatal jaundice and inpatient phototherapy are associated with only small increases in first-year outpatient visit rates, consistent with mild or infrequent contribution to the vulnerable child syndrome in this population.