The risks of rejection vs. infection: Ramsay Hunt syndrome, Gradenigo syndrome, and varicella meningoencephalitis in a heart transplant patient.

Background: Orthotopic heart transplant (OHT) recipients are at increased risk for varicella zoster reactivation, and severe complications may arise due to their immunosuppressive regimens. Managing immunosuppression in acute infection is difficult, and specific guideline recommendations or evidence from the literature are lacking. However, patient care must involve weighing the risk of transplant rejection with the consequences of worsening infection. Case summary: An OHT patient with a history of multiple episodes of acute rejection, latent varicella zoster virus (VZV) infection, and recent completion of anti-viral prophylaxis presented with unilateral facial droop and pain, abducens nerve palsy, crusting facial rash, and ear swelling. Imaging revealed necrotizing otitis externa, with associated otitis media, and petrous apicitis concerning for Gradenigo syndrome. A VZV-positive viral panel confirmed our suspicion for Ramsay Hunt syndrome (RHS). The patient's mentation continued to decline, and subsequent lumbar puncture also revealed VZV meningoencephalitis. The patient's mycophenolate mofetil (MMF) was suspended, with continuation of tacrolimus, and initiation of intravenous acyclovir. The patient demonstrated gradual resolution of his infection, without developing any signs of acute rejection. Discussion: Varicella zoster virus reactivation is common in OHT patients, particularly when viral prophylaxis is discontinued; however, cardiologists should be aware of the rarer manifestations that can manifest in these immunocompromised patients. This is the first documented case of simultaneous RHS, Gradenigo syndrome, and VZV meningoencephalitis in any patient, regardless of transplant status. We demonstrate that even in patients at very high risk of rejection, MMF can be safely discontinued and host immunity maintained with temporary tacrolimus monotherapy.

THE CHATTERJEE PHENOMENON: A DEMONSTRATION OF CARDIAC MEMORY IN PACED RHYTHMS.

Introduction: While T-wave inversions (TWI) are associated with various pathologies, they are rarely associated with cardiac memory, termed the Chatterjee phenomenon. Case: A 76-year-old man with sick sinus syndrome with a pacemaker presented with chest tightness and new onset TWI in his precordial leads. On admission, he tested positive for COVID-19, but remained stable and only required minimal supplemental oxygen. His troponin was only slightly elevated, and EKG showed TWI throughout his precordial leads. A previous EKG had shown normal sinus rhythm without a paced rhythm or ST wave abnormalities. Interrogation of his pacemaker revealed an AV-paced rhythm. Given his chest tightness without dynamic changes in his troponin or EKG, the symptoms were considered more likely related to his COVID-19 infection, and he was discharged home. Discussion: Aberrancies in normal cardiac conduction can result in altered electrical activation, especially for those with AV pacemakers, leading some patients to develop cardiac memory, manifesting as TWI. Conclusion: AV-paced rhythm and narrow QRS complexes with TWI localized to precordial leads without evidence of active cardiac ischaemia may suggest cardiac memory, termed the Chatterjee phenomenon, requiring no invasive interventions. LEARNING POINTS: In patients with T-wave inversions, various conditions should considered in the differential diagnosis, including left bundle branch block and sick sinus syndrome, although T-wave inversions in V1-V3 are non-specific and benign.Cardiac memory, termed the Chatterjee Phenomenon, is one of the causes of T-wave inversions which is sometimes ignored.No invasive interventions are needed for T-wave inversions with the Chatterjee phenomenon.

Clinical Characteristics and Outcomes of Gastritis Associated With Immune Checkpoint Inhibitors: Scoping Review.

Among immune-related adverse events associated with immune checkpoint inhibitors, immune-mediated gastritis (IMG) has been rarely described in the literature and has not yet been well characterized. This scoping review aimed to characterize IMG in terms of precipitating agents, clinical presentations, and prognosis. After the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including "gastritis," "immune checkpoint inhibitor," and "immune-related adverse event" from their inception to December 28, 2021. Twenty-two articles, including 5 observational studies and 17 case reports and case series, were included. Nivolumab, pembrolizumab, and combination therapy with those and cytotoxic T-lymphocyte-associated antigen-4 inhibitor (ipilimumab) were commonly used in those with IMG. 59.8% had epigastric pain, and 50% had erosive gastritis. 87.5% had Common Terminology Criteria for Adverse Events (CTCAE) grade 3 gastritis, and 91.2% received corticosteroids. Recurrence was noted in 16.7%, and only 1 expiration was noted. 4.3% had positive helicobacter pylori and cytomegalovirus from the gastric specimen. Similar to immune-related colitis, patients with IMG may have a favorable prognosis with a better response to immune checkpoint inhibitors if treated appropriately. The diagnosis of IMG is made by exclusion, and a thorough workup is necessary to rule out concurrent helicobacter pylori and cytomegalovirus involvement. Further studies are critical for a better understanding of this complication.

Clinicopathological characteristics of gastric IgG4-related disease: Systematic scoping review.

BACKGROUND AND AIM: Gastric IgG4-related disease (IgG4-RD) can mimic malignancy, submucosal tumors (SMT), and ulcers, leading to over-triage and unnecessary medical interventions such as gastrectomy. The variability in the clinicopathological presentation of IgG4-related disease is not yet well defined, posing a diagnostic challenge. METHODS: Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including "gastritis," "stomach," "gastrointestinal stromal tumor," and "IgG4-RD" from their inception to December 28, 2021. RESULTS: Thirty-nine articles, including 2 observational studies and 42 cases, were included in the systematic review. While bottom-heavy lymphoplasmacytic mucosal infiltration is a characteristic finding of gastric IgG4-RD, it was only present in less than half of the patients in the observational studies. Patients with gastric IgG4-RD were more likely to be diagnosed with gastrointestinal stromal tumor (GIST), gastric cancer, or peptic ulcer disease and their clinical course involved resection (51.3%) or even gastrectomy. Diagnosis of gastric IgG4-RD was most frequently made by post-operative pathological analysis. CONCLUSION: This systematic review summarizes the current understanding of the characteristics of gastric IgG4-RD. Increased awareness of gastric IgG4-RD as a differential diagnosis of gastric SMT or ulcers among clinicians is crucial in order to reduce unnecessary high-risk, invasive interventions.

Survival characteristics of fibrolamellar hepatocellular carcinoma: A Surveillance, Epidemiology, and End Results database study.

BACKGROUND: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and distinct type of hepatocellular carcinoma that frequently presents in an advanced stage in younger patients with no underlying liver disease. Currently, there is a limited understanding of factors that impact outcomes in FL-HCC. AIM: To characterize the survival of FL-HCC by age, race, and surgical intervention. METHODS: This is a retrospective study of The Surveillance, Epidemiology, and End Results database. We identified patients with FL-HCC between 2000-2018 by using an ICD-O-3 site code C22.0 and a histology code 8171/3: Hepatocellular carcinoma, fibrolamellar. In addition, demographics, tumor characteristics, types of surgical procedure, stages, and survival data were obtained. We conducted three separate survival analyses by age groups; ≤ 19, 20-59, and ≥ 60-year-old, and race; White, Black, Hispanic, Asian and Pacific islanders (API), and surgical types; Wedge resection or segmental resection, lobectomy, extended lobectomy (lobectomy + locoregional therapy or resection of the other lobe), and transplant. The Chi-Square test analyzed categorical variables, and continuous variables were examined using the Mann-Whitney U test. The Kaplan-Meier survival curve was used to compare survival. Multivariate analysis was done with Cox regression analysis. RESULTS: We identified 225 FL-HCC patients with a mean age of 36.9. Overall median survival was 34 (95%CI: 27-41) mo. Patients ≤ 19-years-old had more advanced disease with positive lymph nodes status. However, they received more surgical interventions such as a wedge, segmental resection, lobectomy, extended lobectomy, and transplant. Survival for ≤ 19 was 85 (95%CI: 37-137) mo, age 20-59 was 29 (95%CI: 18-41) mo, and age ≥ 60 years was 12 (95%CI: 7-31) mo (P < 0.001). There were no differences in stage, lymph node status, metastasis status, and surgical treatment among races. The median survival were; Whites had 39 (95%CI: 29-63), Blacks 26 (95%CI: 5-92), Hispanics 31 (95%CI: 11-54), and APIs 28 (95%CI: 5-39) mo (P = 0.28). Of 225 patients, 111 FL-HCC patients had surgical procedures. Median survivals for a wedge or segmental resection was 112 (95%CI: 78-NA), lobectomy was 92 (95%CI: 57-NA), extended lobectomy was 54 (95%CI: 23-NA), and a transplant was 63 (95%CI: 20-NA) mo (P < 0.001). The median survival was better in patients who had surgical treatments regardless of lymph nodes or metastasis status (P < 0.001). CONCLUSION: FL-HCC occurs in a primarily younger population, but survival can be prolonged despite the aggressive disease. There were no racial differences in the survival of FL-HCC; however, Asians with FL-HCC tended to be older than in other races. Surgical treatment provided better survival even in those patients with nodal disease or metastases. Although future studies are needed to explore other therapies for FL-HCC, surgical options should be considered in all cases of FL-HCC unless contraindicated.

Procalcitonin to Predict Severity of Acute Cholangitis and Need for Urgent Biliary Decompression: Systematic Scoping Review.

Serum procalcitonin (PCT) has been reported as a potential biomarker to predict the severity of acute cholangitis (AC) or the need for urgent biliary decompression. This study aimed to identify and summarize the existing research about serum PCT and the severity of AC, and to find gaps towards which future studies can be targeted. Following the PRISMA extension for scoping reviews, MEDLINE, EMBASE, and Google Scholar were searched for all peer-reviewed articles with relevant keywords including "cholangitis" and "procalcitonin" from their inception to 13 July 2021. We identified six studies. All the studies employed a case-control design and aimed to evaluate the usefulness of serum PCT to predict the severity of AC with key identified outcomes. While the potential cut-off values of serum PCT for severe AC ranged from 1.8-3.1 ng/mL, studies used different severity criteria and the definition of urgent biliary decompression. No studies proposed cut-off PCT values for the need for urgent biliary decompression. This scoping review identified the current level of evidence regarding the usefulness of serum PCT in assessing the severity of AC. Further clinical research is warranted with a focus on standardized outcome measures employing prospective or experimental designs.

Impact of the World Inflammatory Bowel Disease Day and Crohn's and Colitis Awareness Week on Population Interest Between 2016 and 2020: Google Trends Analysis.

Background: More than 6 million people are affected by inflammatory bowel disease (IBD) globally. The World IBD Day (WID, May 19) and Crohn's and Colitis Awareness Week (CCAW, December 1-7) occur yearly as national health observances to raise public awareness of IBD, but their effects are unclear. Objective: The aim of this study was to analyze the relationship between WID or CCAW and the public health awareness on IBD represented by the Google search engine query data. Methods: This study evaluates the impact of WID and CCAW on the public awareness of IBD in the United States and worldwide from 2016 to 2020 by using the relative search volume of "IBD," "ulcerative colitis," and "Crohn's disease" in Google Trends. To identify significant time points of trend changes (joinpoints), we performed joinpoint regression analysis. Results: No joinpoints were noted around the time of WID or CCAW during the study period in the search results of the United States. Worldwide, joinpoints were noted around WID in 2020 with the search for "IBD" and around CCAW in 2017 and 2019 with the search for "ulcerative colitis." However, the extents of trend changes were modest without statistically significant increases. Conclusions: These results posed a question that WID and CCAW might not have worked as expected to raise public awareness of IBD. Additional studies are needed to precisely estimate the impact of health observances to raise the awareness of IBD.