Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis.

While typically considered a pulmonary disease, cystic fibrosis patients develop significant nutritional complications and comorbidities, especially those who are pancreatic insufficient. Clinicians must have a high suspicion for cystic fibrosis among patients with clinical symptoms of pancreatic insufficiency, and pancreatic enzymatic replacement therapy (PERT) must be urgently initiated. PERT presents a myriad of considerations for patients and their supporting dieticians and clinicians, including types of administration, therapy failures, and complications.

Acute Pancreatitis in a Previously Exocrine Pancreatic Insufficient Cystic Fibrosis Patient Who Had Improved Pancreatic Function After Being Treated With Lumacaftor/Ivacaftor.

Exocrine pancreatic insufficiency (EPI) is a common complication of cystic fibrosis (CF). While previously considered to be irreversible, recent reported cases document improved pancreatic function in CF patients with mild mutations after ivacaftor treatment alone. We report a 12-year-old female with homozygous F508del CF and EPI who developed acute pancreatitis after 3 years on lumacaftor/ivacaftor and subsequently had improved pancreatic function. As CF therapies advance, some EPI CF patients with more severe CF transmembrane conductance regulator mutations may see improved pancreatic function and subsequently develop pancreatitis.

WD Repeat Domain 1 (WDR1) Deficiency Presenting as a Cause of Infantile Inflammatory Bowel Disease.

Infantile and very early onset inflammatory bowel disease (VEOIBD) are a rare phenomenon wherein patients develop intestinal inflammation with typical IBD symptoms before ages 2 and 6, respectively. In recent years, there has been an increasing number of monogenetic immunological disorders identified that lead a child to develop VEOIBD. We present a case of an infant boy who presented with hematochezia and thrombocytopenia in the first week of life and developed IBD by the age of 1 month. Additional clues to his diagnosis included lymphopenia and nuclear herniation observed in his neutrophils. Compound heterozygous damaging variants were identified in WD Repeat Domain 1 (WDR1) by whole-exome sequencing (WES) and represents a novel cause of VEOIBD. Our patient's IBD and immunologic phenotype was successfully treated by hematopoietic stem cell transplant (HSCT).

The Use of a Computerized Provider Order Entry Alert to Decrease Rates of Clostridium difficile Testing in Young Pediatric Patients.

BACKGROUND Infants and young children are frequently colonized with C. difficile but rarely have symptomatic disease. However, C. difficile testing remains prevalent in this age group. OBJECTIVE To design a computerized provider order entry (CPOE) alert to decrease testing for C. difficile in young children and infants. DESIGN An interventional age-targeted before-after trial with comparison group SETTING Monroe Carell Jr. Children's Hospital at Vanderbilt University, Nashville, Tennessee. PATIENTS All children seen in the inpatient or emergency room settings from July 2012 through July 2013 (pre-CPOE alert) and September 2013 through September 2014 (post-CPOE alert) INTERVENTION In August of 2013, we implemented a CPOE alert advising against testing in infants and young children based on the American Academy of Pediatrics recommendations with an optional override. We further offered healthcare providers educational seminars regarding recommended C. difficile testing. RESULTS The average monthly testing rate significantly decreased after the CPOE alert for children 0-11 months old (11.5 pre-alert vs 0 post-alert per 10,000 patient days; P<.001) and 12-35 months old (61.6 pre-alert vs 30.1 post-alert per 10,000 patients days; P<.001), but not for those children ≥36 months old (50.9 pre-alert vs 46.4 post-alert per 10,000 patient days; P=.3) who were not targeted with a CPOE alert. There were no complications in those children who testing positive for C. difficile. CONCLUSIONS The average monthly testing rate for C. difficile for children <35 months old decreased without complication after the use of a CPOE alert in those who tested positive for C. difficile. Infect Control Hosp Epidemiol 2017;38:542-546.

Artificial Sweetened Beverages and Pediatric Obesity: The Controversy Continues.

The pediatric obesity epidemic has gathered public and political interest recently. People often choose "diet" or artificial sweetened beverages (ASB) to combat this epidemic, but the obesity incidence continues to rise. First, I review the pediatric studies on the effect of ASB consumption with subsequent food intake. Next, I present pediatric studies of chronic ASB consumption and weight change. Some epidemiologic pediatric studies have supported an association between artificial sweetener use and increased BMI but cannot prove causation. Randomized control trials have provided some evidence of weight loss with ASB ingestion among children, but study limitations may minimize these conclusions. Finally, I summarize the possible mechanisms that may drive potential effects of artificial sweeteners.