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Early childhood growth status has been used to predict long-term clinical outcomes in Cystic Fibrosis (CF) patients. Adulthood CF outcomes based on early weight-for-length (WFL) measurements, using either World Health Organization (WHO) or Centers for Disease Control (CDC) scales, have not been compared.Cystic Fibrosis Foundation registry patients were studied (n=3014). Participants were categorized at age two years as WFL50th percentile on both WHO and CDC scales, ≥50th percentile on WHO but not CDC, or ≥50th percentile on both. Pulmonary function and overall survival were assessed at age 18years.Stepwise gains in pulmonary function and lung transplant-free survival were noted across the three increasing WFL categories.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.

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.

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 International Study Group of Pediatric Pancreatitis: In Search for a Cure (INSPPIRE), 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, was 2.3 per person per year, costing an estimated average $38,755 per person per year. Including outpatient medications, estimated total mean cost was $40,589 per person per year. Subjects using surgical procedures or endoscopic retrograde cholangiopancreatography incurred mean annual costs of $42,951 per person and $12,035 per person, respectively. Estimated annual costs of pancreatic enzyme replacement therapy, diabetic medications, and pain medications were $4114, $1761, 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 per 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 health care utilization and costs. Interventions that reduce the need for hospitalization could lower costs for these children and their families.

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. We conducted a review of the pediatric pancreatitis literature with respect to the following risk factors: hyperlipidemia, hypercalcemia, chronic renal failure, smoking exposure, alcohol, and medications. Areas of additional research were identified. 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 bystander status may be implicated. Other pancreatitis risk factors must be sought in all cases. 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.

Cystic fibrosis (CF) is the most common life-shortening inherited disease in the USA, affecting approximately 30,000 children and adults. This autosomal recessive disorder is caused by mutations in the gene encoding the cystic fibrosis transmembrane regulator (CFTR) (Riordan et al., Science 245:1066–1073, 1989). CFTR is an ion channel that is present in multiple organs throughout the body. Mutations in this gene are divided into five major categories. Class I, II, and III mutations generally result in more severe pulmonary disease and pancreatic insufficiency; CF patients with these mutations generally have symptomatic disease in infancy or childhood. Class IV and V mutations typically result in milder disease, which is often diagnosed later in life (O’Sullivan and Freedman, Lancet 373:1891–1904, 2009). Neonatal screening catches most patients in the USA with diagnosis confirmed by sweat or genetic testing. Diagnosis in the neonatal period leads to opportunities to optimize nutrition earlier in life.

ObjectivesTo systematically review risks and summarize reported complication rates associated with the performance of endoscopic retrograde cholangiopancreatography (ERCP) in children during the past 2 decades.Study designA systematic literature search of MEDLINE, Embase, and Web of Science from January 1995 to January 2016 was conducted for observational studies published in English. Studies reporting ERCP complications in patients

Approximately 60% of newborns develop jaundice in the first few days after birth.1 Although in most cases jaundice is harmless, the need to monitor bilirubin levels with repeated visits and blood draws and occasionally to readmit infants to the hospital for phototherapy may cause parental anxiety and adversely affect the parent–infant relationship.2,3 Mothers, including those with previous experience with neonatal jaundice, may feel at fault for having caused the jaundice, worry when total serum bilirubin (TSB) levels increase, and have difficulty bonding with their infants.4 Green and Solnit5 first coined the term “vulnerable child syndrome” in 1964 to refer to a pattern of parental overprotection and excessive medical concerns that followed children's recovery from life-threatening illness. Subsequent work has found that children may be perceived as vulnerable after less serious illnesses or events, even as minor as a febrile illness that led to a negative lumbar puncture,6 sickle cell trait,6,7 or a false-positive result on newborn screening.8–10 Indeed, Shonkoff,11 commenting on Green and Solnit's classic article 34 years later, wrote, “The most important contribution of this report is the extent to which it underscores the critical importance of what physicians say (and don't say) to parents. . . . Anything that a pediatrician conveys to a mother or father, whether it is thought through clearly or not, can have enormous impact on them and their children. Moreover, the effect may not only be immediate, but it often can be long-lasting.” Previous studies have raised concern that neonatal jaundice may be a cause of the vulnerable child syndrome. Kemper et al2,3 found that mothers of infants with jaundice (TSB ≥12 mg/dL) were less likely than mothers of control infants to leave their infants with another person and more likely to bring them for well-child, sick, and emergency department (ED) visits. Such disproportionate use of health care resources by children who are perceived as vulnerable has been reported by others as well6,12–15 and is 1 of the hallmarks of the vulnerable child syndrome.16 The studies of Kemper et al2,3 included only 85 infants who had jaundice and were born in 1987 and 1988. Our access to an integrated health care delivery system has allowed us to analyze data for a larger and more recent cohort of infants who were born in a setting in which considerable variation in the use of phototherapy has been documented.17 Although this study based on electronically available data cannot assess parental perceptions of vulnerability directly, our very large sample size allows us to quantify precisely and objectively 1 important manifestation of perceived vulnerability: increased use of outpatient services for sick- and well-child care. Thus, the main goal of this study was to determine, by using a more recent cohort, whether either neonatal hyperbilirubinemia or its treatment with inpatient phototherapy is associated with an increase in subsequent outpatient visit rates.

Though independent findings suggest roles for the allergic arm of the immune system and myelin-reactive antibodies in MS, myelin-reactive IgE has not been investigated. We have developed a radioimmunoassay that measures reactive IgE, IgG and IgA against short (5-6-mers) myelin protein-derived peptides bearing little to no sequence identity with other human proteins, and which might therefore be targets of a CNS-specific autoimmune attack. Here we show that, irrespective of clinical subtype, MS patients' sera are characterized by a higher frequency of measurable IgE against the peptides. Moreover, in controls with measurable IgE reactive against test peptides, IgG or IgA reactive with the same peptide epitopes is almost always present in vastly greater quantities, whereas in MS subjects peptide-reactive IgA or IgG is often undetectable. The sensitivity of the full assay, when considering overall positive a serum sample that has detectable autoreactive IgE without other competing Igs, is 69% (S.E.: 5%), with a specificity of 87% (S.E.: 9%). We speculate that IgE reactive against CNS target antigens may have both diagnostic and pathogenic significance, particularly if other peptide-specific, potentially blocking Igs are absent.