An unusual cause of hypoglycemia in a middle-aged female after bariatric surgery

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is a disorder characterized by postprandial hypoglycemia and islet cell hypertrophy. It is an uncommon complication of weight-loss surgery. However, with the rising incidence of gastric bypass surgeries, it is important to be able to recognize the clinical picture of NIPHS and not to incorrectly ascribe the symptoms to late dumping syndrome

The red hearing: swollen ear in a patient with ulcerative colitis

Relapsing polychondritis is a rare connective tissue disease of unknown etiology characterized by recurrent inflammation, degeneration and deformity of auricular cartilage. The autoimmune inflammation may also affect cartilage at other sites including nose, larynx, trachea and bronchi. Here, we present a case of relapsing polychondritis in a patient with ulcerative colitis. We also review the presentation, diagnosis and management of this condition

HIF-VEGF pathways are critical for chronic otitis media in Junbo and Jeff mouse mutants.

Otitis media with effusion (OME) is the commonest cause of hearing loss in children, yet the underlying genetic pathways and mechanisms involved are incompletely understood. Ventilation of the middle ear with tympanostomy tubes is the commonest surgical procedure in children and the best treatment for chronic OME, but the mechanism by which they work remains uncertain. As hypoxia is a common feature of inflamed microenvironments, moderation of hypoxia may be a significant contributory mechanism. We have investigated the occurrence of hypoxia and hypoxia-inducible factor (HIF) mediated responses in Junbo and Jeff mouse mutant models, which develop spontaneous chronic otitis media. We found that Jeff and Junbo mice labeled in vivo with pimonidazole showed cellular hypoxia in inflammatory cells in the bulla lumen, and in Junbo the middle ear mucosa was also hypoxic. The bulla fluid inflammatory cell numbers were greater and the upregulation of inflammatory gene networks were more pronounced in Junbo than Jeff. Hif-1A gene expression was elevated in bulla fluid inflammatory cells, and there was upregulation of its target genes including Vegfa in Junbo and Jeff. We therefore investigated the effects in Junbo of small-molecule inhibitors of VEGFR signaling (PTK787, SU-11248, and BAY 43-9006) and destabilizing HIF by inhibiting its chaperone HSP90 with 17-DMAG. We found that both classes of inhibitor significantly reduced hearing loss and the occurrence of bulla fluid and that VEGFR inhibitors moderated angiogenesis and lymphangiogenesis in the inflamed middle ear mucosa. The effectiveness of HSP90 and VEGFR signaling inhibitors in suppressing OM in the Junbo model implicates HIF-mediated VEGF as playing a pivotal role in OM pathogenesis. Our analysis of the Junbo and Jeff mutants highlights the role of hypoxia and HIF-mediated pathways, and we conclude that targeting molecules in HIF-VEGF signaling pathways has therapeutic potential in the treatment of chronic OM

A Mouse Model for Osseous Heteroplasia

GNAS/Gnas encodes Gsa that is mainly biallelically expressed but shows imprinted expression in some tissues. In Albright Hereditary Osteodystrophy (AHO) heterozygous loss of function mutations of GNAS can result in ectopic ossification that tends to be superficial and attributable to haploinsufficiency of biallelically expressed Gsa. Oed-Sml is a point missense mutation in exon 6 of the orthologous mouse locus Gnas. We report here both the late onset ossification and occurrence of benign cutaneous fibroepithelial polyps in Oed-Sml. These phenotypes are seen on both maternal and paternal inheritance of the mutant allele and are therefore due to an effect on biallelically expressed Gsa. The ossification is confined to subcutaneous tissues and so resembles the ossification observed with AHO. Our mouse model is the first with both subcutaneous ossification and fibroepithelial polyps related to Gsa deficiency. It is also the first mouse model described with a clinically relevant phenotype associated with a point mutation in Gsa and may be useful in investigations of the mechanisms of heterotopic bone formation. Together with earlier results, our findings indicate that Gsa signalling pathways play a vital role in repressing ectopic bone formation

Prevalence of Pulmonary Embolism Among Patients with Acute Exacerbations of Chronic Obstructive Pulmonary: Data from Nationwide Inpatient Sample

Abstract Background Patients suffering from acute exacerbation of chronic obstructive pulmonary disease (AECOPD) are thought to be at a higher risk of developing venous thromboembolism due to various reasons such as smoking, immobilization and a transient procoagulant state. However, clinical diagnosis of acute pulmonary embolism (PE) in patients with AECOPD is often difficult due to the similarity in the presenting symptoms of the two conditions. Literature regarding the true prevalence of PE among patients with AECOPD and the role of routine screening for PE in these patients with imaging is controversial. Although some studies have suggested prevalence rates to be as high as 20-25%, thus justifying a routine CT pulmonary angiography (CTPA) to evaluate for PE in these patients, other studies have refuted such findings. Methods We used the 2011 Nationwide Inpatient Sample database to identify patients aged ≥18 years admitted with AECOPD (International Classification of Diseases, 9th Revision, Clinical-Modification [ICD-9-CM] code 491.21). Patients with AECOPD with co-existing PE were identified using the ICD-9-CM codes 415.1x and 673.2x. Prevalence of PE in patients with AECOPD was calculated. Similarly, in-hospital mortality, length of stay and mean hospital charge was derived for patient with AECOPD, with and without PE. Statistical analysis was performed using Stata 13.1 (STATA Corp, College Station, TX), which accounted for the complex survey design and clustering of the database. Results A total of 1,187,808 admissions with AECOPD were identified, of which 1.18% (n=13,988) patients were found to have co-existent PE. On Univariate analyses, no differences were seen in the demographic characteristics (mean age, sex, race, primary payer, region, bed-size, teaching status) of AECOPD patients with and without PE. However, diagnosis of concurrent PE in patients with AECOPD was associated with higher in-hospital mortality (10.6% vs. 3.81%, p&lt;0.001), mean length of stay (9.38 vs. 5.92 days, p&lt;0.001) and mean total hospital charges ($74,234 vs. 40,424, p&lt;0.001). Conclusion In this study of large national database, we found the prevalence of PE in patients admitted for AECOPD to be much lower than reported in literature, suggesting that routine imaging to rule out PE is unlikely to be cost effective. Furthermore, routine screening of AECOPD patients for PE with CTPA might actually result in more untoward effects such as incidental pulmonary nodules and PEs with further unnecessary testing and treatment. Disclosures No relevant conflicts of interest to declare. </jats:sec

Effectiveness of Clinical Decision Support for Hypercoagulable Evaluations in One Hospital

Introduction: Venous Thromboembolism (VTE) is a potentially life-threatening disorder that may be provoked by venous stasis, endothelial injury or hypercoagulable states. Workups for hypercoagulable states are frequently undertaken, but tests performed are often either not indicated (if performed in older patients, provoked VTE) or uninterpretable ( if functional tests are ordered while patient anticoagulated or with active thrombus). Clinical decision support at point of order entry has the potential to prevent this unnecessary testing. As part of a larger QI project to improve care of VTE patients, we studied the impact of clinical decision support embedded in order sets, measuring the rates of appropriate hypercoagulable evaluations as a primary outcome. Intervention: This study was undertaken from February 2013 - May 2015, and the effects of intervention were subsequently measured until May 2017. Hypercoagulable workups were defined as appropriate when ordered in patients who are \u3c 45 years of age with unprovoked thrombus for all tests (gene and functional tests); and patients without active thrombi or anticoagulants for functional tests. A new order set was developed on the hospital health information system for VTE admissions. The order set provided evidenced-based guidance for ordering work-up. The order set was rolled out in April 2015, after extensive education of health care providers. Outcome: Over the 2 years after the addition of the clinical decision support intervention, there was a reduction of the total number of hypercoagulable state work-ups from 21% pre-intervention to 15.7% post intervention, the average number of inappropriate tests reduced from 33 to 14 per month - a 57% reduction. Cost savings realized per month from the intervention was approximately $4,500 (pre-intervention:$9,050.73, post-intervention $4,569.98; P - 0.0004) and totaled about$117,000 over 26 months post intervention. Conclusion: Clinical decision support interventions may result in a sustained decrease in unnecessary hypercoagulable workups