Value of upper gastrointestinal endoscopy for gastric cancer surveillance in patients with Lynch syndrome

In our study, we evaluated the effectiveness of upper gastrointestinal (GI) endoscopy as an instrument for early gastric cancer (GC) detection in Lynch syndrome (LS) patients by analyzing data from the registry of the German Consortium for Familial Intestinal Cancer. In a prospective, multicenter cohort study, 1128 out of 2009 registered individuals with confirmed LS underwent 5176 upper GI endoscopies. Compliance was good since 77.6% of upper GI endoscopies were completed within the recommended interval of 1 to 3 years. Forty‐nine GC events were observed in 47 patients. MLH1 (n = 21) and MSH2 (n = 24) mutations were the most prevalent. GCs in patients undergoing regular surveillance were diagnosed significantly more often in an early‐stage disease (UICC I) than GCs detected through symptoms (83% vs 25%; P = .0231). Thirty‐two (68%) patients had a negative family history of GC. The median age at diagnosis was 51 years (range 28‐66). Of all GC patients, 13 were diagnosed at an age younger than 45. Our study supports the recommendation of regular upper GI endoscopy surveillance for LS patients beginning no later than at the age of 30.     

Academic Proficiency in Children After Early Congenital Heart Disease Surgery

Children with early surgery for congenital heart disease (CHD) are known to have impaired neurodevelopment; their performance on school-age achievement tests and their need for special education remains largely unexplored. The study aimed to determine predictors of academic achievement at school age and placement in special education services among early CHD surgery survivors. Children with CHD surgery at <1 year of age from January 1, 1998 to December 31, 2003, at the Arkansas Children’s Hospital were identified. Out-of-state births and infants with known genetic and/or neurologic conditions were excluded. Infants were matched to an Arkansas Department of Education database containing standardized assessments at early school age and special-education codes. Predictors for achieving proficiency in literacy and mathematics and the receipt of special education were determined. Two hundred fifty-six children who attended Arkansas public schools and who had surgery as infants were included; 77.7 % had either school-age achievement-test scores or special-education codes of mental retardation or multiple disabilities. Scores on achievement tests for these children were 7–13 % lower than those of Arkansas students (p < 0.01). They had an eightfold increase in receipt of special education due to multiple disabilities [odds ratio (OR) 10.66, 95 % confidence interval (CI) 4.23–22.35] or mental retardation (OR 4.96, 95 % CI 2.6–8.64). Surgery after the neonatal period was associated with decreased literacy proficiency, and cardiopulmonary bypass during the first surgery was associated with decreased mathematics proficiency. Children who had early CHD surgery were less proficient on standardized school assessments, and many received special education. This is concerning because achievement-test scores at school age are “real-world” predictors of long-term outcomes.     

Mice lacking ghrelin receptors resist the development of diet-induced obesity

Ghrelin is the endogenous ligand for the growth hormone secretagogue receptor (GHSR; ghrelin receptor). Since its discovery, accumulating evidence has suggested that ghrelin may play a role in signaling and reversing states of energy insufficiency. For example, ghrelin levels rise following food deprivation, and ghrelin administration stimulates feeding and increases body weight and adiposity. However, recent loss-of-function studies have raised questions regarding the physiological significance of ghrelin in regulating these processes. Here, we present results of a study using a novel GHSR-null mouse model, in which ghrelin administration fails to acutely stimulate food intake or activate arcuate nucleus neurons. We show that when fed a high-fat diet, both female and male GHSR-null mice eat less food, store less of their consumed calories, preferentially utilize fat as an energy substrate, and accumulate less body weight and adiposity than control mice. Similar effects on body weight and adiposity were also observed in female, but not male, GHSR-null mice fed standard chow. GHSR deletion also affected locomotor activity and levels of glycemia. These findings support the hypothesis that ghrelin-responsive pathways are an important component of coordinated body weight control. Moreover, our data suggest that ghrelin signaling is required for development of the full phenotype of diet-induced obesity.     

PillCam ESO in esophageal studies: improved diagnostic yield of 14 frames per second (fps) compared with 4 fps

BACKGROUND AND STUDY AIM: Capsule endoscopy, using the PillCam ESO and sending images at a rate of 4 frames per second (fps), has a high sensitivity and specificity in diagnosing gastroesophageal reflux disease (GERD) lesions. We tested a new device which produces images at a rate of 14 fps. The diagnostic performance and esophageal visualization of these two devices were compared. PATIENTS AND METHODS: 42 patients with GERD symptoms and eight patients with a history of Barrett's esophagus had an esophagogastroduodenoscopy (EGD). All patients underwent capsule endoscopy of the esophagus within 1 hour prior to EGD. The first 25 patients had a capsule endoscopy examination with the 4-fps device. The following 25 patients underwent capsule endoscopy under identical conditions but using the 14-fps device. The reader of the capsule endoscopy study was blinded to the EGD findings. A diagnosis of GERD or Barrett's esophagus was established with EGD. The findings at capsule endoscopy were compared with the EGD findings. We also examined how frequently the esophagus in its entirety was visualized by these two devices. RESULTS: The 4-fps device diagnosed 16/19 cases of esophageal erosions or ulcers (sensitivity 84 %) and 6/8 cases of Barrett's esophagus (sensitivity 75 %). The 14-fps capsule diagnosed 16/16 cases of esophageal ulcers or erosions and 7/7 cases of Barrett's esophagus (sensitivity 100 %). The total diagnostic miss rate in the 4-fps group was 5/27 (18 %) whereas the diagnostic miss rate in the 14-fps group was 0/23 (0 %) P < 0.02). The upper esophageal sphincter (UES) was clearly identified in 6/25 patients (24 %) in the 4-fps group and in 20/25 patients (80 %) in the 14-fps group ( P < 0.01). The entire esophagus was well visualized in 3/25 patients (12 %) by the 4-fps device and in 19/25 (76 %) by the 14-fps device ( P < 0.01). The superiority of the 14-fps PillCam ESO capsule is consistent with the data obtained from fluoroscopic studies of swallowed PillCam capsules, showing that capsule speed may reach 20 cm/s. For the 14-fps PillCam this means one image transmitted per 3-cm segment at maximal capsule speed, therefore still allowing for full visualization of the entire esophagus. CONCLUSIONS: Capsule endoscopy using the 14-fps PillCam ESO showed a greater sensitivity than that of the 4-fps device for identifying GERD. The 14-fps PillCam ESO was statistically superior to the 4-fps device in visualizing the opening of the UES and the entirety of the esophagus.     

"Hemicrania Continua": A Clinical Review

Hemicrania continua (HC) is a headache entity completely responsive to indomethacin. Since 1984, 18 cases have been described, 15 females and 3 males, i.e. a F:M ratio of 5.0. The finding of a female preponderance, like that in chronic paroxysmal hemicrania, is a new observation. HC is, in general, a unilateral headache in the sense that it sets in on one side and subsequently sticks to this side. In two cases, both sides might possibly be involved, when the pain was at its maximum. In another (somewhat dubious) case the headache was bilateral. The pain was continuous from the beginning in 8 of 18 cases (early stage ratio continuous: non-continuous = 0.8). Over time, the headache developed a continuous character in 16 of the 18 cases, producing a "continuous: non-continuous ratio" of 8:1. The intensity of pain generally was moderate and was not reported as excruciatingly severe by any patient. The autonomic involvement from a clinical point of view, was clearly less pronounced than that of other unilateral headaches, such as cluster headache and chronic paroxysmal hemicrania.