Prolonged acid reflux monitoring using acid exposure time and DeMeester score: two days are not enough.

BACKGROUND: Wireless pH monitoring allows for a definitive GERD diagnosis, which is essential for optimal medical or surgical management of the patient. However, there is no guideline recommendation on whether prolonged pH testing (72 or 96 h) provides additional benefit when compared to the standard 48-h testing. We aimed to assess whether prolonged pH monitoring diagnoses more patients with GERD, as well as compare the DeMeester score to acid exposure time as diagnostic criteria for GERD. METHODS: This was a retrospective analysis of consecutive adult patients who underwent wireless esophageal pH monitoring between August 2018 and July 2021. The primary outcome was the additional diagnoses of GERD (predominant acid exposure pattern) in patients who underwent 48-h versus 96-h pH monitoring. Secondary outcomes included comparison of the DeMeester score to acid exposure time and internal agreement between the first and second 48-h blocks of a prolonged 96-h pH study. RESULTS: When comparing 48-h versus 96-h pH testing, the prolonged monitoring group was more likely to have a predominant reflux pattern and thus be diagnosed with definitive GERD by elevated DeMeester score (58.8% vs. 40.8%, p = 0.003) or acid exposure time > 6% (44.7% vs. 32.4%, p = 0.039). For patients who underwent prolonged testing, the results of monitoring beyond 48 h led to a clinically meaningful change in study interpretation in 24.8% of patients. The study data from Days 3 to 4 yielded only a 56.6% agreement with the first 2 days. CONCLUSIONS: In patients undergoing extended pH monitoring, almost half were found to have an abnormal pH study after a normal study on Day 1. An additional 25% of patients had a change in study interpretation by extending the study beyond 48 h. Our findings suggest only 48 h of pH monitoring will miss a diagnosis of GERD in a clinically important number of patients.

What's in Your Drink? Poster Educates Families About Sugar Content and Fatty Liver Disease.

OBJECTIVE: Drinking sugar-sweetened beverages is a risk factor for developing childhood obesity and nonalcoholic fatty liver disease (NAFLD). This study investigated the impact of an educational poster in pediatric offices on family's knowledge of sugar content in beverages and assessed awareness of NAFLD. DESIGN: Preclinic visit surveys asked patients' caregivers about the sugar content in beverages and awareness of NAFLD. Postclinic visit surveys assessed improvement in knowledge of sugar content and willingness to change dietary habits. SETTING: Outpatient visits in a single center in Houston between September and November 2019. PARTICIPANTS: One hundred and forty-nine caregivers were surveyed, and patients' median age was 5.5 years (range, 0-18 years) with 57% males. INTERVENTION: Educational posters displayed the sugar content of common beverages in each clinic room. MAIN OUTCOME MEASURES: Outcomes measured included pre-post clinic visit change and predictors of change in (1) knowledge of sugar content in beverages and (2) intent to change beverage consumption. Baseline awareness of NAFLD and associated predictors were also assessed. ANALYSIS: Logistic regression identified factors associated with an intended change in beverage consumption, change in survey score, and NAFLD awareness. RESULTS: Increased knowledge of sugar content with median scores of 25% preclinic to 50% postclinic (P < 0.001). Eighty-eight percent of caregivers were very/moderately likely to provide their children fewer sugar-sweetened beverages. Sixty percent of caregivers were aware of NAFLD, but only 32.8% were concerned. CONCLUSIONS AND IMPLICATIONS: Posters in clinics increased awareness of the sugar content in beverages, and most caregivers reported intent to decrease children's sugary beverage consumption.

Evaluating the Revised American Society for Gastrointestinal Endoscopy Guidelines for Common Bile Duct Stone Diagnosis.

BACKGROUND/AIMS: The American Society for Gastrointestinal Endoscopy (ASGE) revised its guidelines for risk stratification of patients with suspected choledocholithiasis. This study aimed to assess the diagnostic performance of the revision and to compare it to the previous guidelines. METHODS: We conducted a retrospective cohort study of 267 patients with suspected choledocholithiasis. We identified high-risk patients according to the original and revised guidelines and examined the diagnostic accuracy of both guidelines. We measured the association between individual criteria and choledocholithiasis. RESULTS: Under the original guidelines, 165 (62%) patients met the criteria for high risk, of whom 79% had confirmed choledocholithiasis. The categorization had a sensitivity and specificity of 68% and 55%, respectively, for the detection of choledocholithiasis. Under the revised guidelines, 86 (32%) patients met the criteria for high risk, of whom 83% had choledocholithiasis. The revised categorization had a lower sensitivity and higher specificity of 37% and 80%, respectively. The positive predictive value of the high-risk categorization increased with the revision, reflecting a potential decrease in diagnostic endoscopic retrograde cholangiopancreatograpies (ERCPs). Stone visualized on imaging had the greatest specificity for choledocholithiasis. Gallstone pancreatitis was not associated with the risk for choledocholithiasis. CONCLUSION: The 2019 revision of the ASGE guidelines decreases the utilization of ERCP as a diagnostic modality and offers an improved risk stratification tool.

Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.

OBJECTIVES: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). BACKGROUND: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown. METHODS: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death. RESULTS: A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001). CONCLUSIONS: In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs.

Molecular mechanisms of functional natural killer deficiency in patients with partial DiGeorge syndrome.

BACKGROUND: DiGeorge syndrome affects more than 3.5 million persons worldwide. Partial DiGeorge syndrome (pDGS), which is characterized by a number of gene deletions in chromosome 22, including the chicken tumor virus number 10 regulator of kinase (Crk)-like (CrkL) gene, is one of the most common genetic disorders in human subjects. To date, the role of natural killer (NK) cells in patients with pDGS remains unclear. OBJECTIVE: We sought to define the effect of pDGS-related Crk haploinsufficiency on NK cell activation and cytotoxic immunological synapse (IS) structure and function. METHODS: Inducible CrkL-silenced NK cells were used to recapitulate the pDGS, CrkL-haploinsufficient phenotype. Findings were validated by using NK cells from patients with actual pDGS. Ultimately, deficits in the function of NK cells from patients with pDGS were restored by lentiviral transduction of CrkL. RESULTS: Silencing of CrkL expression inhibits NK cell function. Specifically, pDGS haploinsufficiency of CrkL inhibits accumulation of activating receptors, polarization of cytolytic machinery and key signaling molecules, and activation of ß2-integrin at the IS. Reintroduction of CrkL protein restores NK cell cytotoxicity. CONCLUSION: CrkL haploinsufficiency causes functional NK deficits in patients with pDGS by disrupting both ß2-integrin activation and activating receptor accumulation at the IS. Our results suggest that NK cell IS quality can directly affect immune status, providing a potential target for diagnosis and therapeutic manipulation in patients with pDGS and in other patients with functional NK cell deficiencies.

Impacts of roads and hunting on central African rainforest mammals.

Road expansion and associated increases in bunting pressure are a rapidly growing threat to African tropical wildlife. In the rainforests of southern Gabon, we compared abundances of larger (>1 kg) mammal species at varying distances from forest roads and between hunted and unhunted treatments (comparing a 130-km2 oil concession that was almost entirely protected from bunting with nearby areas outside the concession that had moderate hunting pressure). At each of 12 study sites that were evenly divided between hunted and unhunted areas, we established standardized 1-km transects at five distances (50, 300, 600, 900, and 1200 m) from an unpaved road, and then repeatedly surveyed mammals during the 2004 dry and wet seasons. Hunting had the greatest impact on duikers (Cephalophus spp.), forest buffalo (Syncerus caffer nanus), and red river hogs (Potamochoerus porcus), which declined in abundance outside the oil concession, and lesser effects on lowland gorillas (Gorilla gorilla gorilla) and carnivores. Roads depressed abundances of duikers, sitatungas (Tragelaphus spekei gratus), and forest elephants (Loxondonta africana cyclotis), with avoidance of roads being stronger outside than inside the concession. Five monkey species showed little response to roads or hunting, whereas some rodents and pangolins increased in abundance outside the concession, possibly in response to greater forest disturbance. Our findings suggest that even moderate hunting pressure can markedly alter the structure of mammal communities in central Africa. Roads had the greatest impacts on large and small ungulates, with the magnitude of road avoidance increasing with local hunting pressure.