Clinical Characteristics and Long-term Outcomes of Children With Fibrosing Pancreatitis.

OBJECTIVES: Fibrosing pancreatitis (FP) shares clinical features with autoimmune pancreatitis (AIP), although both entities have not been definitely linked. This study aimed to assess the presence of AIP criteria in an historic FP patient cohort and investigate the clinical features, management, and long-term outcomes of pediatric FP (P-FP). METHODS: Clinical data of 14 P-FP patients from Toronto and 42 P-FP cases from a literature review were collected and compared to pediatric AIP (P-AIP). Toronto P-FP patients were recontacted to assess their current health status using a brief questionnaire. RESULTS: Jaundice and abdominal pain were the symptoms at presentation in 44 of 56 (79%) and 50 of 56 (89%) P-FP patients, respectively. Common findings on cross sectional imaging were an enlarged pancreas head with narrowing of the distal common bile duct (51/54, 94%). Histopathology mainly showed gland fibrosis (39/39, 100%). Three of twelve (25%) P-FP patients had elevated IgG4 in serum. None of the patients were treated with corticosteroids, but some underwent surgical or endoscopic intervention. Toronto patients were followed for a median of 13.6 years (interquartile range: 2.9-22.8). Complications during follow-up included exocrine pancreatic insufficiency (3/14, 21%) and pancreatic gland atrophy (5/13, 38%); but none of the patients had disease relapse or developed diabetes type 3c. Five (5/14, 36%) patients developed other immune-mediated diseases over time. CONCLUSIONS: Clinical features of patients with P-FP resembled those recently described in a subgroup of P-AIP presenting with jaundice. Long-term outcome of these patients is generally good, with or without invasive interventions. As some patients may develop exocrine pancreatic insufficiency and/or other immune-mediated diseases, ongoing clinical monitoring is recommended.

Outcomes of multi-gestational pregnancies affected by esophageal atresia - tracheoesophageal fistula.

BACKGROUND: Contemporary outcomes of infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) from multi-gestational pregnancies compared to those of singleton pregnancies have not been reported. METHODS: A single-center retrospective review of EA/TEF patients born from 1999 to 2013 was performed. Patient demographics, gestational age (GA), birth weight, associated anomalies, requirement for gastrostomy tube and mortality were reviewed. RESULTS: Singleton EA/TEF patients outnumbered those from multi-gestational pregnancies nearly 10:1 (214 vs 22 patients). EA/TEF patients from multi-gestational pregnancies were more likely to be premature (77% vs. 32%), have lower birth weight (mean 1766 g vs. 2695 g), have associated duodenal atresia (18% vs. 6%) and require gastrostomy tube (41% vs. 33%) for feeding challenges compared to EA/TEF singletons. Mortality was also significantly greater for multi-gestational EA/TEF patients compared to singleton EA/TEF patients (18% vs. 6%). CONCLUSION: EA/TEF infants from multi-gestational pregnancies have greater clinical complexity and mortality than singleton EA/TEF patients. Parents of EA/TEF multi-gestational infants should be appropriately counseled and supported.

Pulmonary function and nutritional morbidity in children and adolescents with congenital diaphragmatic hernia.

BACKGROUND: Malnutrition is common among congenital diaphragmatic hernia (CDH) survivors and may result from elevated respiratory effort. We evaluated body mass index (BMI), measured resting energy expenditure (mREE) and pulmonary function test (PFT) results in children and adolescents with CDH to determine if there is a correlation. METHODS: With ethics approval (REB# 1000035323), anthropometrics, indirect calorimetry (IC) results and PFTs were collected from patients 5-17years of age during CDH clinic visits between 2000 and 2016. Malnutrition was defined as BMI z-scores <-2.0; mREE (as percent predicted REE) was measured using IC; z-scores for forced expiratory volume in 1s (FEV1) and forced vital capacity (FVC) were normal if <-1.64. STATISTICS: GraphPad Prism 6, San Diego, CA. RESULTS & DISCUSSION: Of 118 patients who attended clinic, 33 had reproducible PFTs, anthropometrics and IC results. Mean BMI z-score was -0.89±1.47 and 24% of patients were malnourished; mean FVC z-score (-1.32±1.39) was within normal range, whereas mean z-scores for FEV1 (-2.21±1.68) and FEV1/FVC ratio (-1.78±0.73) were below normal. A correlation was noted between BMI and PFTs (FEV1 r=0.70, P<0.0001; FVC r=0.74 P<0.0001). Mean mREE was 112%±12% of expected and 67% of patients were hypermetabolic (mREE<110% predicted). IC results did not correlate with z-scores for either FEV1 (r=0.10, P=0.57); or FVC (r=0.28, P=0.12). CONCLUSIONS: These preliminary results suggest that a correlation is present between BMI and lung function in CDH children and adolescents, whereas lung function does not seem to correlate with mREE. LEVEL OF EVIDENCE: II.

Long-term nutritional morbidity for congenital diaphragmatic hernia survivors: Failure to thrive extends well into childhood and adolescence.

BACKGROUND: Failure to thrive (FTT) is well documented among congenital diaphragmatic hernia (CDH) survivors ≤3years of age, but its etiology, severity, and persistence beyond this age require further elucidation. METHODS: We conducted a single-center, retrospective study assessing anthropometrics, measured energy expenditure, and feeding tube (FT) use of 5-17 year olds in our multidisciplinary CDH clinic since January 2001. We stratified clinic visits based on age A: 5.0-6.9, B: 7.0-9.9, C: 10.0-14.9, and D: 15-17.9years. RESULTS: One hundred sixteen patients with 376 outpatient visits were reviewed. Anthropometric z-scores were below zero and did not vary across age cohorts. FTT and growth stunting each occurred in 14% of clinic visits. FTs inserted during infancy occurred in 25% of patients, and 60% remained by age 7years. In cohort A, those with FTs were lighter and shorter than those without (p<0.05) but had similar BMIs. FTT incidence was higher in the FT group (p=0.020), but FTs were present in only 30% of those with FTT. Indirect calorimetry revealed increased energy expenditure in 58% of patients. CONCLUSIONS: Failure to thrive continues in long-term CDH survivors, FTs may not improve incidence of FTT. Increased energy expenditure may play a role.