Successful treatment of severe passenger lymphocyte syndrome with efgartigimod synergy.

INTRODUCTION: This case describes passenger lymphocyte syndrome (PLS) generating human platelet antigen 1a (HPA-1a) alloantibodies against the recipient's platelets after liver transplant. Given the rarity of PLS, especially in liver transplant with HPA-1a alloantibodies, disease course and management options are poorly described. METHODS: The patient had cirrhosis secondary to nonalcoholic steatohepatitis complicated by hepatocellular carcinoma, encephalopathy, and severe ascites. The model for end-stage liver disease (MELD) score was 15 at presentation. The patient developed hepatic artery thrombosis after an orthotopic liver transplant and was relisted for transplant with a MELD score of 40. The patient received a hepatitis C virus antibody positive, hepatitis C virus nucleic amplification test positive donor liver on postoperative day (POD) 7 after first transplant. On POD 7 after the second transplant, the patient developed profound thrombocytopenia refractory to platelet infusion. They were found to have serum antibody to HPA-1a based upon serum platelet alloantibody testing. The donor was later found to be negative for HPA-1a by genetic testing. However, the patient's native platelets were HPA-1a positive. The patient was diagnosed with PLS. RESULTS: The patient's treatment course included 57 units of platelets transfused, emergency splenectomy, rituximab, plasma exchange, intravenous immunoglobulin (IVIG), eltrombopag, romiplostim, and efgartigimod. DISCUSSION: The synergistic effect of efgartigimod with eltrombopag and romiplostim most likely resolved the patient's thrombocytopenia. This case represents a novel use of efgartigimod in the treatment of passenger lymphocyte syndrome following liver transplant.

Fidaxomicin treatment for Clostridioides difficile infection in patients with inflammatory bowel disease.

BACKGROUND AND AIM: Although fidaxomicin is an effective first-line treatment for Clostridioides difficile infection, it has not been well studied in patients with inflammatory bowel disease. We aimed to assess the effectiveness of fidaxomicin for the treatment of C. difficile infection in patients with inflammatory bowel disease. METHODS: This was a multicenter retrospective study of adults with inflammatory bowel disease and C. difficile infection treated with fidaxomicin with at least 3 months of follow up. The primary outcomes were treatment response, defined as resolution of C. difficile infection-attributed diarrhea and/or negative C. difficile infection stool test, and time to C. difficile infection recurrence after fidaxomicin. RESULTS: Thirty-three patients (median age 42 years; 60.6% female) were included. Most patients had ulcerative colitis (26, 78.8%), were receiving treatment with a biologic or small molecule medication (19, 57.6%), and had a prior episode of C. difficile infection (26, 78.8%, median 2 episodes, range 0-15). Fidaxomicin led to resolution of C. difficile infection in 20 (60.6%) patients, with 6/20 (30.0%) developing a recurrence at a median of 55 days. Most patients who failed to respond to fidaxomicin underwent fecal microbiota transplantation (10/13, 76.9%) with resolution. CONCLUSIONS: In this cohort of patients with inflammatory bowel disease and C. difficile infection, 60.6% responded to treatment with fidaxomicin. Of those who did not respond, fecal microbiota transplantation was an effective therapy.

Abnormal findings on abdominopelvic cross-sectional imaging in patients with microscopic colitis: a retrospective, multicenter study.

OBJECTIVES: Patients with microscopic colitis may have subtle macroscopic findings on colonoscopy such as erythema, edema, or altered vascular pattern; however, radiographic abnormalities on cross-sectional imaging have not been investigated. We aimed at identifying the abdominopelvic radiographic abnormalities in patients with microscopic colitis, as well as possible correlation with endoscopic findings and the need for extended duration of treatment. MATERIALS AND METHODS: This was a retrospective study of patients with biopsy-proven microscopic colitis at two tertiary centers between 1 January 2010 and 30 April 2020. Patients underwent computed tomography scan or magnetic resonance imaging within 30 days of a diagnostic flexible sigmoidoscopy or colonoscopy. Patients with colon ischemia and other causes of colitis were excluded. Radiographic abnormalities from imaging reports included bowel wall thickening, mucosal hyperenhancement and mesenteric fat stranding. Univariate and multivariable logistic regression models were used to identify predictors of radiographic abnormalities. RESULTS: 498 patients with microscopic colitis underwent abdominopelvic cross-sectional imaging within 30 days of flexible sigmoidoscopy/colonoscopy. Lymphocytic colitis was diagnosed in 54.6% of patients, and collagenous colitis in 45.4%. Endoscopic and radiographic abnormalities were identified in 16.1% and 12.4% of patients, respectively. Radiographic abnormalities were associated with the need for budesonide therapy (p = .029) and budesonide therapy long-term (p = .0028). Budesonide therapy long-term (p = .047) was associated with radiographic abnormalities in multivariate analysis. CONCLUSIONS: Radiographic abnormalities may be present on abdominopelvic cross-sectional imaging in a minority of patients with biopsy-proven microscopic colitis, suggesting cross-sectional imaging has low clinical value in the evaluation and treatment of this disease.

Intravenous Access in Gastrointestinal Hemorrhage: A Multidisciplinary Quality Improvement Initiative Led by Emergency Department Nurses and Internal Medicine Physicians.

BACKGROUND: The management of acute gastrointestinal hemorrhage (GIH) is focused on early resuscitation through 2 large-bore intravenous (2LBIV) catheters, although adherence to this recommendation is low. LOCAL PROBLEM: Of 100 patients hospitalized with GIH in 2017, only 14 received 2LBIV access. The goal of this study was to improve this measure. METHODS: A multidisciplinary team used the DMAIC (define, measure, analyze, improve, and control) framework to perform a quality improvement initiative. INTERVENTIONS: The team used quality tools including a stakeholder survey, swimlane diagram, and fishbone diagram. The first intervention involved education of the hospitalists directing admissions, and the second intervention involved education of emergency department (ED) physicians and nurses regarding the importance of 2LBIV placement. RESULTS: Following the second intervention, there was a substantial increase in 2LBIV placement to 37 of 86 (43%). CONCLUSIONS: Carefully directed education of ED physicians and nurses with monthly feedback was effective in improving appropriate intravenous placement in patients with GIH.

An Argument for Vitamin D, A, and Zinc Monitoring in Cirrhosis.

Malnutrition is prevalent in cirrhosis. Vitamin and mineral deficiencies, including vitamin D, vitamin A, and zinc, are common and have been shown to correlate with survival. Our aim was to review the mechanisms of vitamin D, vitamin A, and zinc deficiencies in cirrhosis and the clinical assessment of affected patients, their outcomes based on the current literature, and management. This is a narrative review including the relevant literature for cirrhosis and vitamin D, vitamin A, and zinc deficiencies. Vitamin D deficiency has important effects in cirrhosis, regardless of the cause of chronic liver disease.These effects include associations with fibrosis and outcomes such as infections, hepatocellular carcinoma, and mortality. Vitamin A deficiency is associated with liver disease progression to cirrhosis and clinical decompensation, including occurrence of ascites or hepatic encephalopathy. Zinc deficiency can lead to hepatic encephalopathy and impaired immune function. Such deficiencies correlate with patient survival and disease severity. Caution should be applied when replacing vitamin D, vitamin A, and zinc to avoid toxicity. Identification and appropriate treatment of vitamin and mineral deficiencies in cirrhosis may reduce specific nutritional and cirrhosis-related adverse events. Routine monitoring of vitamin A, vitamin D and zinc levels in cirrhosis should be considered.

Implementation of a New Guideline and Educational Sessions to Reduce Low-Value Continuous Pulse Oximetry Among Hospitalized Patients.

OBJECTIVES: The use of continuous pulse oximetry (CPOX) is ubiquitous among hospitalized patients, despite limited evidence that it improves clinical outcomes. The objective of this study was to reduce the use of CPOX among hospitalized patients in the nonintensive care unit and nonprogressive care unit settings. METHODS: This interventional trial included the creation a new local guideline for CPOX use and subsequent staff education. CPOX use, patient acuity, hospital length of stay, and code blue events were measured before and after the intervention. RESULTS: Postintervention there was a clinically significant and sustained decrease in CPOX use of 18% over 1 year. There were no significant changes postintervention in hospital length of stay or number of code blue events. CONCLUSIONS: Development of a guideline for CPOX use and staff education successfully led to a decrease in CPOX use, without an increase in hospital length of stay or code blue events.

Presence of esophageal contractility after achalasia treatment is associated with improved esophageal emptying.

BACKGROUND AND AIMS: Some achalasia patients exhibit esophageal contractile activity on follow-up after treatment, yet its importance remains unclear. We aimed to identify factors associated with presence of contractility after treatment and to assess its impact on timed barium esophagram (TBE) and clinical outcomes. METHODS: Patients with type I or II achalasia on baseline high-resolution manometry (HRM) who completed HRM, TBE, and functional lumen imaging probe (FLIP) after treatment were retrospectively identified. Contractility was defined on post-treatment HRM as presence of at least 1 supine swallow with DCI ≥100 mmHg s cm. KEY RESULTS: One hundred twenty-two patients were included (mean age 48 ± 17 years, 50% female). At follow-up evaluation after treatment (54% peroral endoscopic myotomy, 24% pneumatic dilation, 22% laparoscopic Heller myotomy), 61 (50%) patients had contractility on HRM. Patients with contractility (compared to those without) more frequently had type II achalasia (84% vs 57%, p = 0.001) and a post-treatment normal EGJ opening classification on FLIP (69% vs 49%; p < 0.001). In the subgroup of patients with post-treatment integrated relaxation pressure <15 mmHg and normal EGJ opening on FLIP (n = 53), those with contractility had a lower median column height on TBE at 1 min (4 vs 7 cm, p = 0.002) and 5 min (0 vs 5 cm, p = 0.001). In patients with "abnormal" EGJ metrics, patients with contractility showed lower symptom scores (median Eckardt score 2 vs 3, p = 0.03). CONCLUSIONS & INFERENCES: Occurring more frequently in type II achalasia, and if adequate EGJ opening is achieved after treatment, esophageal contractility may contribute to improved esophageal emptying and improved symptoms in non-spastic achalasia. Preservation of esophageal body muscle could improve outcomes in these patients.

Effect of esophageal body recoil on clinical outcomes in non-spastic achalasia.

BACKGROUND: Despite the established efficacy of achalasia treatments on symptomatic outcomes, there are limited data evaluating the treatment effect on esophageal dilatation. This study aimed to assess the effect achalasia treatment on esophageal dilatation and the effect of esophageal width reduction ("recoil") on clinical outcomes. METHODS: Patients with type I or type II achalasia that completed high-resolution manometry (HRM), functional lumen imaging probe (FLIP), and timed barium esophagram (TBE) pre and post treatment were included. Esophageal width was measured using TBE. Focused subgroup analysis was performed on patients with normal posttreatment EGJ opening on FLIP. Good clinical outcomes were defined as barium column height of <5 cm at 5 min and Eckardt Score ≤3. KEY RESULTS: Sixty-nine patients (41% type I and 59% type II) were included. Esophageal width decreased from pre to post treatment mean (SD) 4.2 (1.3) cm-2.8 (1.2) cm; p < 0.01. In the normal post treatment EGJ opening subgroup, esophageal width was less in patients with good TBE outcome compared to poor outcome mean (SD) 2.2 (0.7) cm versus 3.2 (1.4) cm (p < 0.01), but did not differ in good versus poor symptomatic outcome groups. Esophageal width recoil >25% posttreatment was associated with a greater rate of good TBE outcome (71% vs. 50%, p = 0.04) and good symptomatic outcome (88% vs. 50%; p = 0.04). CONCLUSIONS AND INFERENCES: Esophageal recoil was associated with good achalasia treatment outcome in patients without posttreatment EGJ obstruction. This suggests that mechanical properties of the esophageal wall, likely associated with tissue remodeling, play a role in clinical outcomes following achalasia treatment.

The impact of primary peristalsis, contractile reserve, and secondary peristalsis on esophageal clearance measured by timed barium esophagogram.

BACKGROUND: Primary and secondary peristalsis facilitate esophageal bolus transport; however, their relative impact for bolus clearance remains unclear. We aimed to compare primary peristalsis and contractile reserve on high-resolution manometry (HRM) and secondary peristalsis on functional lumen imaging probe (FLIP) Panometry with emptying on timed barium esophagogram (TBE) and incorporate findings into a comprehensive model of esophageal function. METHODS: Adult patients who completed HRM with multiple rapid swallows (MRS), FLIP, and TBE for esophageal motility evaluation and without abnormal esophagogastric junction outflow/opening or spasm were included. An abnormal TBE was defined as a 1-min column height >5 cm. Primary peristalsis and contractile reserve after MRS were combined into an HRM-MRS model. Secondary peristalsis was combined with primary peristalsis assessment to describe a complementary neuromyogenic model. KEY RESULTS: Of 89 included patients, differences in rates of abnormal TBEs were observed with primary peristalsis classification (normal: 14.3%; ineffective esophageal motility: 20.0%; absent peristalsis: 54.5%; p = 0.009), contractile reserve (present: 12.5%; absent: 29.3%; p = 0.05), and secondary peristalsis (normal: 9.7%; borderline: 17.6%; impaired/disordered: 28.6%; absent contractile response: 50%; p = 0.039). Logistic regression analysis (akaike information criteria, area under the receiver operating curve) demonstrated that the neuromyogenic model (80.8, 0.83) had a stronger relationship predicting abnormal TBE compared to primary peristalsis (81.5, 0.82), contractile reserve (86.8, 0.75), or secondary peristalsis (89.0, 0.78). CONCLUSIONS AND INFERENCES: Primary peristalsis, contractile reserve, and secondary peristalsis were associated with abnormal esophageal retention as measured by TBE. Added benefit was observed when applying comprehensive models to incorporate primary and secondary peristalsis supporting their complementary application.

Number of bottles of esophageal biopsies in the evaluation of eosinophilic esophagitis and clinical outcomes.

BACKGROUND: The number of bottles of esophageal biopsies needed for the evaluation of eosinophilic esophagitis (EoE) is unclear, despite cost differences. AIMS: Assess the clinical outcomes between patients with one and two bottles of esophageal biopsies for the assessment of EoE. METHODS: Retrospective study of adults who underwent esophagogastroduodenoscopy (EGD) for esophageal symptoms between January 2015 and June 2021 and findings of ≥15 eosinophils per high power field (eos/hpf). Patients with one bottle (1 bottle-EoE) had biopsies from the entire or proximal esophagus. Patients with two bottles had biopsies separated from the distal and proximal esophagus and were separated into those with ≥ 15 eos/hpf in both bottles (2 bottle Dif-EoE), or the distal bottle alone (2 bottle Lim-EoE). The primary outcomes were endoscopic findings at follow-up EGD as assessed by the Eosinophilic Esophagitis Endoscopic Reference Score (EREFS) and the presence of ≥15 eos/hpf. RESULTS: Of 85 patients with esophageal eosinophilia who met inclusion criteria, 49 had 2 bottle Dif-EoE, 18 had 2 bottle Lim-EoE, and 18 had 1 bottle-EoE. At median follow-up of 3.3-5.6 months, more patients with 1 bottle EoE had dysphagia (p = 0.029), however there were no differences in the EREFS (p = 0.14) or presence of ≥15 eos/hpf (p = 0.39). More patients with 2 bottle Dif-EoE were treated with topical steroids (16.3% vs. 0% vs. 0%, p = 0.039) and diet (20.4% vs. 0% vs. 5.6%, p = 0.05). CONCLUSION: Endoscopic and histologic outcomes were similar in patients who had one and two bottles for esophageal biopsies in the evaluation of EoE.

A survey of lifestyle habits, physician counseling, and direct-to consumer genetic testing in patients with hereditary hemochromatosis.

INTRODUCTION AND OBJECTIVES: The goal of this study was to assess lifestyle habits and physician counseling of patients with hereditary hemochromatosis (HH), and determine the prevalence of direct-to-consumer (DTC) genetic testing. MATERIALS AND METHODS: A 52-question survey was created to collect information on lifestyle habits and physician counseling among patients with HH, and the use of DTC genetic testing of patients referred to a clinic for evaluation of HH. A multivariate logistic regression model was applied to identify predictors of DTC genetic testing use. RESULTS: The survey was e-mailed to 379 patients, of which 101 responded (26.6%). Among patients with HH, 37% reported alcohol use more than once weekly and 50% reported red meat consumption. The use of a vitamin C supplement was reported by 38.9% of participants. Among patients with living children and siblings, physicians failed to recommend HH screening 15.3% and 21.2% of the time respectively. Thirty-one patients reported DTC genetic testing, of which 46.7% (14/31) reported their DTC genetic test screened for HH. Six (19%) of those patients were prompted to see a specialist in HH based on the results. CONCLUSIONS: Among patients with HH, lifestyle habits that may impact iron stores are common, but not all receive appropriate counseling. Direct-to-consumer genetic testing is common, and physicians should be aware of its limitations when patients seek further evaluation for HH based on their test results.

An Unusual Cause of Large Bowel Obstruction in a Patient With Ulcerative Colitis.

Endometriosis is a rare cause of large bowel obstruction and has been infrequently reported in patients with inflammatory bowel disease. We present an unusual case of a young woman with ulcerative colitis, who presented with a large bowel obstruction with colonic stricture and peripancreatic mass concerning for malignancy. The evaluation revealed endometriosis, and her large bowel obstruction was successfully managed with leuprolide and colonic stenting.

Gastric outlet obstruction: A red flag, potentially manageable.

Gastric outlet obstruction is a common condition in which mechanical obstruction in the distal stomach, pylorus, or duodenum causes nausea, vomiting, abdominal pain, and early satiety. This article reviews the changing etiology of this disorder and advances in its treatment.

Acute pancreatitis-induced takotsubo cardiomyopathy and cardiogenic shock treated with a percutaneous left ventricular assist device.

A 63-year-old man was admitted for severe acute pancreatitis. On day 3 of hospitalisation, he developed shortness of breath and acute pulmonary oedema. Echocardiogram revealed global hypokinesis with a left ventricular ejection fraction of 20%, and he was diagnosed with takotsubo cardiomyopathy. He developed cardiogenic shock which was treated successfully with a percutaneous left ventricular assist device. His left ventricular ejection fraction improved by hospital follow-up 3 weeks later.