Pancreatitis in cystic fibrosis: Presentation, medical and surgical management, and the impact of modulator therapies.

Patients with Cystic Fibrosis (CF) are at increased risk of acute (AP) and chronic (CP) pancreatitis, and their complications. The extent of remaining healthy pancreatic parenchyma determines the risk of developing future episodes of pancreatitis, as well as pancreatic exocrine or endocrine insufficiency. Pancreatitis may be the presenting symptom of CF, and genetic testing is especially important in pediatrics. AP and recurrent AP are managed with intravenous fluid hydration and pain control, in addition to early refeeding and treatment of complications. With the use of modulator therapy in CF, pancreatic function may be restored to some extent. CP related pain is managed with analgesics and neuromodulators, with surgery if indicated in specific situations including TPIAT as a possible type of surgical intervention. Long-term sequelae of CP in patients with CF include exocrine pancreatic insufficiency treated with pancreatic enzyme replacement therapy, fat-soluble vitamin deficiencies and associated metabolic complications such as bone disease/osteoporosis, pancreatogenic diabetes, and less commonly, pancreatic cancer. We review the presentation and etiologies of pancreatitis in CF patients as well as the management of AP and CP primarily in children.

What is new with irritable bowel syndrome.

PURPOSE OF REVIEW: This review aims to summarize the most up-to-date criteria for diagnosis of pediatric irritable bowel syndrome (IBS) and treatment options. It also explores recent research that has been performed evaluating risk factors, pathophysiology, and treatment designed to improve quality of life for those who suffer from IBS. IBS is a common disorder in pediatrics and one of the most common causes of abdominal pain for children; thus, it can be quite debilitating for individuals to achieve a satisfactory quality of life on a routine basis. Reliable, available treatment is needed but can be challenging to find, given the variety of symptoms and triggers involved and lack of a clear understanding of how IBS develops. RECENT FINDINGS: There are multiple pharmacologic and nonpharmacologic treatment options being explored and studied globally but further, larger, and well controlled studies are needed to confirm these outcomes. SUMMARY: Because pediatric IBS is one of the most common functional disorders associated with abdominal pain and can be brought on by multiple factors, management often involves addressing these individual triggers with a multifaceted treatment plan, which could include dietary changes, probiotics, medication, or psychotherapy and should be tailored to each affected individual.

Spatiotemporal transitions in Pseudo-nitzschia species assemblages and domoic acid along the Alaska coast.

The toxic diatom genus Pseudo-nitzschia is distributed from equatorial to polar regions and is comprised of >57 species, some capable of producing the neurotoxin domoic acid (DA). In the Pacific Arctic Region spanning the Bering, Chukchi, and Beaufort seas, DA is recognized as an emerging human and ecosystem health threat, yet little is known about the composition and distribution of Pseudo-nitzschia species in these waters. This investigation characterized Pseudo-nitzschia assemblages in samples collected in 2018 during summer (August) and fall (October-November) surveys as part of the Distributed Biological Observatory and Arctic Observing Network, encompassing a broad geographic range (57.8° to 73.0°N, -138.9° to -169.9°W) and spanning temperature (-1.79 to 11.7°C) and salinity (22.9 to 32.9) gradients associated with distinct water masses. Species were identified using a genus-specific Automated Ribosomal Intergenic Spacer Analysis (ARISA). Seventeen amplicons were observed; seven corresponded to temperate, sub-polar, or polar Pseudo-nitzschia species based on parallel sequencing efforts (P. arctica, P. delicatissima, P. granii, P. obtusa, P. pungens, and two genotypes of P. seriata), and one represented Fragilariopsis oceanica. During summer, particulate DA (pDA; 4.0 to 130.0 ng L-1) was observed in the Bering Strait and Chukchi Sea where P. obtusa was prevalent. In fall, pDA (3.3 to 111.8 ng L-1) occurred along the Beaufort Sea shelf coincident with one P. seriata genotype, and south of the Bering Strait in association with the other P. seriata genotype. Taxa were correlated with latitude, longitude, temperature, salinity, pDA, and/or chlorophyll a, and each had a distinct distribution pattern. The observation of DA in association with different species, seasons, geographic regions, and water masses underscores the significant risk of Amnesic Shellfish Poisoning (ASP) and DA-poisoning in Alaska waters.

Autoimmunity in people with cystic fibrosis.

Cystic fibrosis (CF) clinicians may see patients who have difficult-to-manage symptoms that do not have a clear CF-related etiology, such as unusual gastrointestinal (GI) complaints, vasculitis, or arthritis. Alterations in immunity, inflammation and intraluminal dysbiosis create a milieu that may lead to autoimmunity, and the CF transmembrane regulator protein may have a direct role as well. While autoantibodies and other autoimmune markers may develop, these may or may not lead to organ involvement, therefore they are helpful but not sufficient to establish an autoimmune diagnosis. Autoimmune involvement of the GI tract is the best-established association. Next steps to understand autoimmunity in CF should include a more in-depth assessment of the community perspective on its impact. In addition, bringing together specialists in various fields including, but not limited to, pulmonology, gastroenterology, immunology, and rheumatology, would lead to cross-dissemination and help define the path forward in basic science and clinical practice.

The use of DXA for early detection of pediatric cystic fibrosis-related bone disease.

BACKGROUND: Cystic fibrosis (CF)-related bone disease (CFBD) is seen in adults and can be associated with respiratory illness and malnutrition. There is limited and conflicting data regarding CFBD in pediatric CF. With longer life expectancy and promotion of disease prevention, pediatric CFBD demands further investigation. METHODS: Our center initiated a quality improvement (QI) project from April 2016 to December 2018 to improve CFBD screening in patients 8 years or older, per current CF Foundation (CFF) guidelines. Our team formulated a dual-energy X-ray absorptiometry (DXA) scan algorithm based upon degree of bone mineral density (BMD); shared CFBD guideline recommendations in our quarterly newsletter; and ordered scans for eligible patients at weekly review meetings. We reviewed DXA results from 141 patients after institutional review board approval and gathered data including comorbidities, genetics, anthropometric measures, medication exposure, and relevant serum studies. RESULTS: Fifty-three percent of our patients had normal BMD (n = 75). Seventeen patients (12%) had a Z score ≤ -2. Patients with lower BMD also had lower mean forced expiratory volume (FEV1 ) percent predicted (FEV1 %) (p < 0.001) as well as lower body mass index % (p = 0.001). Patients with lower BMD were overall older at time of DXA (p = 0.016). During study duration, 13 patients who had abnormal DXA results underwent repeat DXAs after physical therapy; 11 of the 13 showed improvement in DXA results. CONCLUSIONS: A DXA scan is a useful screening tool and can be used to identify pediatric patients who could benefit from further therapy and interventions to preserve adequate bone health and avoid further loss. QI initiatives can lead to improved screening and diagnosis and earlier intervention such as physical therapy. Further studies are needed to better understand the utility of physical therapy in children with CF.

Adnexal Masses: Diagnosis and Management.

Pelvic masses occur in up to 20% of women throughout their lifetime. These masses represent a spectrum of gynecologic and nongynecologic conditions. Adnexal masses-found in the fallopian tubes, ovaries, and surrounding areas-are mostly benign. Evaluation includes assessment for symptoms that may suggest malignancy, such as abdominal pain, abdominal bloating, and early satiety. A family history of ovarian, breast, or certain heritable syndromes increases the risk of malignancy. For women of reproductive age, ectopic pregnancies must be considered; a beta human chorionic gonadotropin level should be obtained. Transvaginal ultrasonography is the imaging test of choice for evaluating adnexal masses for size and complexity. Adnexal cysts that are greater than 10 cm, contain solid components, or have high color flow on Doppler ultrasonography are high risk for malignancy. Further imaging, if warranted, should be completed with computed tomography or magnetic resonance imaging, particularly if there is concern for disease outside the ovary. Multimodal assessment tools that use ultrasonography and biomarkers, such as the risk of malignancy index, are useful in the diagnosis and exclusion of malignant causes. Asymptomatic masses that are determined to be benign may be observed and managed expectantly. In symptomatic or emergent cases, such as ectopic pregnancy or ovarian torsion, a gynecologist should be consulted. In any adnexal mass with high risk for malignancy, a consultation with gynecologic oncology is indicated.

Reduced representation sequencing accurately quantifies relative abundance and reveals population-level variation in Pseudo-nitzschia spp.

Certain species within the genus Pseudo-nitzschia are able to produce the neurotoxin domoic acid (DA), which can cause illness in humans, mass-mortality of marine animals, and closure of commercial and recreational shellfisheries during toxic events. Understanding and forecasting blooms of these harmful species is a primary management goal. However, accurately predicting the onset and severity of bloom events remains difficult, in part because the underlying drivers of bloom formation have not been fully resolved. Furthermore, Pseudo-nitzschia species often co-occur, and recent work suggests that the genetic composition of a Pseudo-nitzschia bloom may be a better predictor of toxicity than prevailing environmental conditions. We developed a novel next-generation sequencing assay using restriction site-associated DNA (2b-RAD) genotyping and applied it to mock Pseudo-nitzschia communities generated by mixing cultures of different species in known abundances. On average, 94% of the variance in observed species abundance was explained by the expected abundance. In addition, the false positive rate was low (0.45% on average) and unrelated to read depth, and false negatives were never observed. Application of this method to environmental DNA samples collected during natural Pseudo-nitzschia spp. bloom events in Southern California revealed that increases in DA were associated with increases in the relative abundance of P. australis. Although the absolute correlation across time-points was weak, an independent species fingerprinting assay (Automated Ribosomal Intergenic Spacer Analysis) supported this and identified other potentially toxic species. Finally, we assessed population-level genomic variation by mining SNPs from the environmental 2bRAD dataset. Consistent shifts in allele frequencies in P. pungens and P. subpacifica were detected between high and low DA years, suggesting that different intraspecific variants may be associated with prevailing environmental conditions or the presence of DA. Taken together, this method presents a potentially cost-effective and high-throughput approach for studies aiming to evaluate both population and species dynamics in mixed samples.

COPD inhaler therapy: A path to success.

Keys to therapeutic success include choosing the right device and drug regimen, providing rigorous patient education, and reducing environmental exposures.

Acute Pancreatitis: Rapid Evidence Review.

Acute pancreatitis is the most common gastrointestinal-related reason for hospitalization in the United States. It is diagnosed based on the revised Atlanta classification, with the presence of at least two of three criteria (upper abdominal pain, serum amylase or lipase level greater than three times the upper limit of normal, or characteristic findings on imaging studies). Although computed tomography and other imaging studies can be useful to assess severity or if the diagnosis is uncertain, imaging is not required to diagnose acute pancreatitis. Based on limited studies, several scoring systems have comparable effectiveness for predicting disease severity. The presence of systemic inflammatory response syndrome on day 1 of hospital admission is highly sensitive in predicting severe disease. Treatment of acute pancreatitis involves goal-directed fluid resuscitation, analgesics, and oral feedings as tolerated on admission. If oral feedings are not tolerated, nasogastric or nasojejunal feedings are preferred over parenteral nutrition. Cholecystectomy is recommended during the initial admission for patients with mild acute biliary pancreatitis. Medical management is usually sufficient for necrotizing pancreatitis; however, if surgical intervention is needed, a minimally invasive approach is advised over direct endoscopic or open surgical debridement (necrosectomy) because of lower complication rates.

Slicing the Pie: Interpreting harmful algal blooms one pie chart at a time.

The Earth's oceans are home to a diverse array of life, from large marine mammals to microscopic organisms. Among the most important are the marine phytoplankton, which comprise the basis of marine food webs, and also produce a large percentage of the Earth's oxygen through photosynthesis. Although the vast majority of phytoplankton are essential to ocean health, several dozen species produce potent toxins, and can form what are called Harmful Algal Blooms (HABs). This activity focuses on the importance of HABs, as well as the types of data scientists collect to understand blooms. In the classroom exercises, students will calculate the proportional abundance (% contribution) of five HAB species present in water samples, and use these data to create pie graphs to depict species composition. Students will then compare these results with levels of HAB toxins in water samples collected over the same time period. Thought questions challenge students to develop hypotheses regarding how changes in the HAB community may relate to observed trends in toxin concentrations. This activity was successfully taught to visually impaired students who were able to complete the pie charts and answer the thought questions.