Outcomes of a 3-Year Quality Improvement Study to Improve Advance Care Planning in Patients With Decompensated Cirrhosis.

INTRODUCTION: To report outcomes of a 3-year quality improvement pilot study to improve advance directive (AD) completion. METHODS: The pilot consisted of champions, education, electronic health record templates, and workflow changes. We assessed changes, predictors, and effects of AD completion. RESULTS: The pilot led to greater (8.3%-36%) and earlier AD completion, particularly among those divorced, with alcohol-associated liver disease, and with higher Model of End-Stage Liver Disease-Sodium score. Decedents whose AD specified nonaggressive goals experienced lower hospital lengths of stay. DISCUSSION: Advance care planning initiatives are feasible and may reduce health care utilization among decedents requesting less aggressive care.

Safety of Endoscopy in Heritable Connective Tissue Disorders.

INTRODUCTION: Little is known about the gastrointestinal manifestations or safety of endoscopy among patients with heritable connective tissue disorders such as Marfan syndrome or Ehlers-Danlos syndrome (EDS). METHODS: We conducted an electronic cross-sectional survey nested within preexisting registries of patients with heritable connective tissue disorders and examined self-reported rates of endoscopic complications. RESULTS: The rate of endoscopy-related perforation was 9.4% (95% confidence interval 2.0%-25.0%) among individuals with vascular EDS, <1% in classical and hypermobility-type EDS, and zero in Marfan syndrome (P < 0.001). Spontaneous intestinal perforation was also significantly higher in the vascular EDS group. DISCUSSION: Clinicians should consider noninvasive screening methods for patients with vascular EDS.

Prevalence of Functional GI Diseases and Pelvic Floor Symptoms in Marfan Syndrome and Ehlers-Danlos Syndrome: A National Cohort Study.

BACKGROUND AND AIMS: Prior studies have shown a high prevalence of gastrointestinal (GI) symptoms, diagnoses of functional GI diseases (FGIDs), and pelvic floor symptoms associated with Ehlers-Danlos syndrome (EDS). It is unclear if Marfan syndrome (MFS), another common hereditary noninflammatory connective tissue disorder, is also associated these symptoms. This study evaluates the prevalence of and compares FGIDs and pelvic floor symptoms in a national cohort of EDS and MFS patients. METHODS: A questionnaire was sent to members of local and national MFS and EDS societies. The questionnaire evaluated the presence of GI and pelvic floor symptoms and diagnoses. The presence of FGIDs was confirmed using Rome III criteria. Quality of life was evaluated and scored with the CDC quality of life. KEY RESULTS: Overall, 3934 patients completed the questionnaire, from which 1804 reported that they had some form of EDS and 600 had MFS. In total, 93% of patients with EDS complained of GI symptoms and qualified for at least one FGID compared with 69.8% of patients with MFS. When comparing EDS prevalence of upper and lower GI symptoms as well as FGIDs, subjects with EDS reported significantly higher prevalence of Rome III FGIDs as compared with those with MFS. Irritable bowel syndrome (57.8% vs. 27.0%, P<0.001), functional dyspepsia (FD) (55.4% vs. 25.0%, P<0.001), postprandial distress (49.6% vs. 21.7%, P<0.001), heartburn (33.1% vs. 16.8%, P<0.001), dysphagia (28.5% vs. 18.3%, P<0.001), aerophagia (24.7% vs. 12.3%, P<0.001), and nausea (24.7% vs. 7.2%, P<0.001) were all significantly greater in the EDS population compared with MFS population. The prevalence of FGIDs was similar across subtypes of EDS. In general, participants with EDS were more likely to have nearly all pelvic floor symptoms as compared with participants with MFS. CONCLUSIONS: The prevalence of FGIDs and pelvic floor symptoms in EDS is higher than that found in MFS. The prevalence of FGIDs were similar across EDS subtypes. This study supports the mounting evidence for FGIDs in those with connective tissue diseases, but more specifically, in EDS.

Findings of Hepatic Severe Acute Respiratory Syndrome Coronavirus-2 Infection.

BACKGROUND & AIMS: Liver injury due to coronavirus disease 2019 (COVID-19) is being increasingly recognized. Abnormal liver chemistry tests of varying severities occur in a majority of patients. However, there is a dearth of accompanying liver histologic studies in these patients. METHODS: The current report details the clinical courses of 2 patients having severe COVID-19 hepatitis. Liver biopsies were analyzed under light microscopy, portions of liver tissue were hybridized with a target probe to the severe acute respiratory syndrome coronavirus-2 S gene, and small sections from formalin-fixed paraffin-embedded liver tissue were processed for electron microscopy. RESULTS: The liver histology of both cases showed a mixed inflammatory infiltrate with prominent bile duct damage, endotheliitis, and many apoptotic bodies. In situ hybridization and electron microscopy suggest the intrahepatic presence of severe acute respiratory syndrome coronavirus-2, the findings of which may indicate the possibility of direct cell injury. CONCLUSIONS: On the basis of the abundant apoptosis and severe cholangiocyte injury, these histopathologic changes suggest a direct cytopathic injury. Furthermore, some of the histopathologic changes may resemble acute cellular rejection occurring after liver transplantation. These 2 cases demonstrate that severe COVID-19 hepatitis can occur even in the absence of significant involvement of other organs.

Audit of Early Mortality among Patients Admitted to the General Medical Ward at a District Hospital in Botswana.

BACKGROUND: Mortality among adult general medical admissions has been reported to be high across sub-Saharan Africa, yet there is a paucity of literature on causes of general medical inpatient mortality and quality-related factors that may contribute to the high incidence of deaths. Based on a prior study at our hospital as well as our clinical experience, death early in the hospitalization is common among patients admitted to the adult medical wards. OBJECTIVE: Quantify early inpatient mortality and identify factors contributing to early in-hospital mortality of medical patients in a resource-limited hospital setting in Botswana. METHODS: Twenty-seven cases of patients who died within 48 hours of admission to the general medical wards at Scottish Livingstone Hospital in Molepolole, Botswana from December 1, 2015-April 25, 2016 were retrospectively reviewed through a modified root cause analysis. FINDINGS: Early in-hospital mortality was most frequently attributed to septic shock, identified in 20 (74%) of 27 cases. The most common care management problems were delay in administration of antibiotics (15, 56%), inappropriate fluid management (15, 56%), and deficient coordination of care (15, 56%). The most common contributing factors were inadequate provider knowledge and skills in 25 cases (93%), high complexity of presenting condition in 20 (74%), and inadequate communication between team members in 18 (67%). CONCLUSIONS: Poor patient outcomes in low-and middle-income countries like Botswana are often attributed to resource limitations. Our findings suggest that while early in-hospital mortality in such settings is associated with severe presenting conditions like septic shock, primary contributors to lack of better outcomes may be healthcare-provider and system-factors rather than lack of diagnostic and therapeutic resources. Low-cost interventions to improve knowledge, skills and communication through a focus on provider education and process improvement may provide the key to reducing early in-hospital mortality and improving hospitalization outcomes in this setting.

Management of small bowel polyps: A literature review.

Despite the small bowel comprising 90% of the mucosal surface area of the gastrointestinal tract, it is a rare site for neoplasia and only accounts for a little over 3% of the tumors that arise in the digestive tract. Benign small bowel lesions include lipomas, lymphangiomas, leiomyomas, neurofibromas, nodular lymphoid hyperplasia and adenomas, many of which are precursors to malignant lesions. Several polyposis syndromes are associated with small bowel polyps as well, including familial adenomatous polyposis syndrome, lynch syndrome, Peutz-Jeghers syndrome, Cowden syndrome and juvenile polyposis syndrome. Our aim was to review non-malignant small bowel polyps and discuss the prevalence, typical location, clinical presentation, diagnosis, endoscopic and histologic description and lastly management of each of these lesions.

Decrease in airway mucous gene expression caused by treatment with anti-tumor necrosis factor alpha in a murine model of allergic asthma.

BACKGROUND: Mucous hypersecretion increases asthma morbidity and mortality. Tumor necrosis factor a (TNF-a) levels are elevated in bronchoalveolar fluid, sputum, and monocyte membranes in some patients with asthma. Anti-TNF-a decreased asthma exacerbations and improved forced expiratory volume in 1 second in these patients. Whether anti-TNF-a reduces mucous cell metaplasia or hyperplasia has not been evaluated. OBJECTIVE: To investigate the role of anti-TNF-alpha in mucous hypersecretion. METHODS: BALB/c mice sensitized intraperitoneally and challenged intratracheally with ovalbumin were treated with 250 microg of anti-TNF-alpha before ovalbumin sensitization and challenge or before only ovalbumin challenge. Control groups were sham treated. The tumor necrosis factor receptor (TNFR) mice (TNFR-/- and TNFR+/+) were identically sensitized and challenged. Seventy-two hours after the final challenge, the airway pressure time index (APTI), which measures airway hyperresponsiveness, was recorded. Mucous cell metaplasia was accessed by quantitative polymerase chain reaction for MUC-5AC (the epithelial cell mucous-inducing gene) and the percentage of periodic acid-Schiff (PAS) staining of bronchial epithelial cells. A human airway cell line (constitutively expressing MUC-5AC) was pretreated with a NF-kappaB inhibitor before TNF-alpha culture. RESULTS: The mean (SE) fold change of MUC-5AC expression (compared with naive controls), the percentage of PAS-positive bronchiole epithelial cells, and the APTI decreased in BALB/c mice treated with anti-TNF-alpha before sensitization and challenge (4.9 [1.14], P = .007; 28.9% [6.8%], P < .001; and 545.8 [104.5] cm H2O/s, P < .001, respectively) and before challenge alone (9.3 [1.8], P = .03; 43.6% [10.7%], P = .009; and 896.8 [81.23] cm H2O/s, P = .06, respectively) compared with sham-treated mice (20.9 [3.9], 82.4% [1.8%], and 1,055 [30.6] cm H20/s, respectively). MUC-5AC expression decreased in ovalbumin sensitized or challenged TNFR-/- (2.41 [0.4]) compared with ovalbumin sensitized or challenged TNFR+/+ mice (18.4 [2.5], P < .001). TNF-alpha-induced MUC-5AC expression in human airway culture significantly decreased with pretreatment of a NF-kappaB inhibitor. CONCLUSIONS: Anti-TNF-alpha treatment reduces airway mucous cell metaplasia in a mouse model of asthma, which may in part underlie its beneficial effect as asthma therapy.