Undiagnosed Cirrhosis and Hepatic Encephalopathy in a National Cohort of Veterans With Dementia.

Importance: Dementia and hepatic encephalopathy (HE) are challenging to distinguish clinically. Undiagnosed cirrhosis in a patient with dementia can lead to missed opportunities to treat HE. Objective: To examine the prevalence and risk factors of undiagnosed cirrhosis and therefore possible HE in veterans with dementia. Design, Setting, and Participants: A retrospective cohort study was conducted between 2009 and 2019 using data from the Veterans Health Administration (VHA) and 2 separate validation cohorts from the Richmond Veterans Affairs Medical Center. Data analysis was conducted from May 20 to October 15, 2023. Participants included 177 422 US veterans with a diagnosis of dementia at 2 or more clinic visits, no prior diagnosis of cirrhosis, and with sufficient laboratory test results to calculate the Fibrosis-4 (FIB-4) score. Exposures: Demographic and clinical characteristics. Main Outcomes and Measures: An FIB-4 score (>2.67 suggestive of advanced fibrosis and >3.25 suggestive of cirrhosis), capped at age 65 years even for those above this cutoff who were included in the analysis. Results: Among 177 422 veterans (97.1% men; 80.7% White; mean (SD) age, 78.35 [10.97] years) 5.3% (n = 9373) had an FIB-4 score greater than 3.25 and 10.3% (n = 18 390) had an FIB-4 score greater than 2.67. In multivariable logistic regression models, FIB-4 greater than 3.25 was associated with older age (odds ratio [OR], 1.07; 95% CI, 1.06-1.09), male gender (OR, 1.43; 95% CI, 1.26-1.61), congestive heart failure (OR, 1.48; 95% CI, 1.43-1.54), viral hepatitis (OR, 1.79; 95% CI, 1.66-1.91), Alcohol Use Disorders Identification Test score (OR, 1.56; 95% CI, 1.44-1.68), and chronic kidney disease (OR, 1.11; 95% CI, 1.04-1.17), and inversely associated with White race (OR, 0.79; 95% CI, 0.73-0.85), diabetes (OR, 0.78; 95% CI, 0.73-0.84), hyperlipidemia (OR, 0.84; 95% CI, 0.79-0.89), stroke (OR, 0.85; 95% CI, 0.79-0.91), tobacco use disorder (OR, 0.78; 95% CI, 0.70-0.87), and rural residence (OR, 0.92; 95% CI, 0.87-0.97). Similar findings were associated with the FIB-4 greater than 2.67 threshold. These codes were associated with cirrhosis on local validation. A local validation cohort of patients with dementia showed a similar percentage of high FIB-4 scores (4.4%-11.2%). Conclusions and Relevance: The findings of this cohort study suggest that clinicians encountering patients with dementia should be encouraged to screen for cirrhosis using the FIB-4 score to uncover reversible factors associated with cognitive impairment, such as HE, to enhance outcomes.

QuickStroop Predicts Time to Development of Overt Hepatic Encephalopathy and Related Hospitalizations in Patients With Cirrhosis.

Cirrhosis-related neurocognitive impairment caused by covert or minimal hepatic encephalopathy (CHE) affects psychosocial function, increases risk of overt hepatic encephalopathy (OHE) development, and worsens survival.1,2 However, detection in clinical practice is challenging.2 One modality used for screening and prediction of outcomes related to cirrhosis is the EncephalApp Stroop, but it can require up to 10 minutes. Furthermore, the assessment comprises of distinct stages of difficulty, with an easier "Off" stage and a more challenging "On" stage.3 To alleviate these concerns, QuickStroop, which takes <1 minute, was developed. This uses only the first 2 runs of the Off stage of the EncephalApp Stroop, where number signs presented in red, green, or blue need to be matched quickly to their respective colors.4 A prior study showed these versions were comparable cross-sectionally to diagnose CHE.4 However, the utility of QuickStroop to predict cirrhosis-related outcomes is unclear.5-7 Our aim was to determine the ability of QuickStroop to determine time to development of OHE and OHE-related hospitalizations, all-cause hospitalizations, and death in outpatients with cirrhosis.

A Rare Case of Esophageal Leukoplakia in Achalasia.

Esophageal leukoplakia refers to a clinical finding of a white patch on the mucous membrane surface that cannot be scraped off. It has been associated with alcohol and tobacco use and chronic acid reflux. An association with squamous cell dysplasia and carcinoma has been reported with potential for malignant transformation warranting endoscopic intervention or surveillance, but no guidelines exist. We present a case of a 77-year-old female with a history of longstanding achalasia requiring multiple Botox injections. After presenting with weight loss, esophageal dysphagia, and acid reflux the patient underwent an esophagogastroduodenoscopy (EGD) showing a 20 mm white plaque in the middle third of the esophagus and histopathology consistent with esophageal leukoplakia. After repeated Botox injection and treatment with PPI and H2 blocker, no findings of esophageal leukoplakia were noted on repeat EGD. With this case, we aim to increase awareness of this rare disease pathology, especially in the setting of underlying achalasia. This case also raises the question if maximum anti-reflux therapy could have a potential benefit in avoiding the recurrence of esophageal leukoplakia.

Enlarging Liver Mass: Inflammatory Pseudotumor in a Patient With Polymyalgia Rheumatica.

Inflammatory pseudotumors of the liver are rare, non-neoplastic liver tumors. Due to the nonspecific clinical presentation, imaging features, and histopathological findings, they can mimic malignant tumors requiring invasive diagnostics. We present a case of a 61-year-old female patient with a history of type 2 diabetes mellitus, hypothyroidism, hyperlipidemia, and polymyalgia rheumatica who had initially presented with abdominal pain for 3 weeks. Further workup showed normal liver chemistries and tumor markers: AFP and CA 19-9. Magnetic resonance imaging (MRI) of the abdomen showed a segment 6 lesion measuring 4.1 × 4.0 × 3.7 cm. A liver biopsy then confirmed the diagnosis of an inflammatory pseudotumor of the liver with negative IgG4. On follow-up imaging, a rapid growth of this liver lesion was noted. Laparoscopy was done but did not show any distinct liver lesion. Follow-up imaging confirmed a decrease in the size of the mass. Interestingly, the patient had been on a higher dose of steroids for her polymyalgia rheumatic leading up to the follow-up imaging. This is the first case of an inflammatory pseudotumor of the liver in a patient with polymyalgia rheumatica. With this case, we would like to increase the awareness for inflammatory pseudotumors of the liver as a differential diagnosis of liver lesions in patients with underlying autoimmune disorders.

Gastrointestinal Hemorrhage From Esophageal Dissecans Superficialis After the Initiation of a Tyrosine Kinase Inhibitor.

Esophagitis dissecans superficialis (EDS) is described as the peeling of squamous mucosa of the esophagus with regurgitation of esophageal casts. It is a rare endoscopic finding associated with chemical irritants, autoimmune disorders, or medications. Most patients are asymptomatic but clinical manifestations can include dysphagia, heartburn, bleeding, or vomiting. In this report, we present a case of a 70-year-old man with a previous history of small cell lung and hepatocellular carcinoma for which he had undergone chemoradiation and immunotherapy. He had presented with upper gastrointestinal hemorrhage manifested as coffee ground emesis. Endoscopic findings were consistent with EDS. No recurrence of his gastrointestinal hemorrhage was observed after acid-suppressive therapy. With our case report, we aim to increase awareness for EDS as a differential diagnosis for gastrointestinal hemorrhage.

Meckel's diverticulum in an adult with iron deficiency.

Meckel's diverticulum is a true diverticulum caused by the incomplete obliteration of the vitelline duct during gestation. It is the most common congenital malformation of the gastrointestinal tract. Meckel's diverticulum is a rare presentation in adults, especially because symptomatic patients present in early childhood. We present a 57-year-old woman with symptomatic iron deficiency after recent nonsteroidal anti-inflammatory use. After normal upper and lower endoscopy, a capsule endoscopy showed an oozing polyp in the distal ileum. The small bowel was surgically resected, and histopathology confirmed Meckel's diverticulum as the cause of her small bowel bleeding. Rare causes such as Meckel's diverticulum should be kept in mind in the differential diagnosis for patients with suspected small bowel bleeding.

Gastric Splenosis Mimicking Gastrointestinal Stromal Tumor.

Translocation of splenic tissue in patients after traumatic spleen injury or splenectomy is called splenosis. Gastric splenosis is a rare presentation that can be mistaken for gastrointestinal stromal tumor (GIST). Patients are usually asymptomatic and do not require surgical intervention. In this report, we present a case of a 68-year-old male patient with a previous history of surgical splenectomy after traumatic splenic rupture, who underwent routine upper endoscopy for the evaluation of dysphagia. An endoscopic exam of the stomach revealed an incidental finding of a submucosal gastric nodule. On endoscopic ultrasound exam, the lesion was found to be suggestive of GIST originating from layer 4. A core biopsy was obtained from the nodule, which was consistent with gastric splenosis. The differentiation of gastric splenosis from other gastric lesions such as GIST is important since asymptomatic patients with gastric splenosis do not need to undergo surveillance or surgical resection. It should be suspected especially in patients with a history of splenectomy or splenic rupture. Endoscopic ultrasound (EUS)-guided core biopsy can help confirm the diagnosis and differentiate the condition from GIST.

Duodenal bulb obstruction caused by a gallstone (Bouveret syndrome) successfully treated with endoscopic measures.

Bouveret syndrome is the rarest variant of gallstone ileus, with a high morbidity and mortality rate as well as life-threatening complications. We present a case of an 86-year-old woman who presented with abdominal pain and was diagnosed with acute cholecystitis. After laparoscopic cholecystectomy, her symptoms did not improve. She then underwent endoscopic retrograde cholangiopancreatography and was found to have a stone causing duodenal bulb obstruction. The stone was removed using a snare, and her symptoms improved greatly. This rare case of Bouveret syndrome with no apparent fistula was successfully treated using endoscopic measures.

Bilateral pulmonary artery thrombectomy with saddle embolism and COVID-19 infection.

Thrombotic complications such as venous thromboembolism, ischemic stroke, and myocardial infarction have emerged as causes of significant morbidity and mortality in patients infected with COVID-19. We present a 32-year-old man who developed a large saddle pulmonary embolus secondary to COVID-19 infection and underwent successful bilateral percutaneous pulmonary artery mechanical thrombectomy.

Pneumococcal Meningitis Complicated by Subarachnoid Hemorrhage and Tonsillar Herniation.

Bacterial meningitis is a fatal infectious disease with an annual incidence of four to six cases per 100,000 adults. The most common pathogens associated with this condition are Streptococcus pneumoniae, Neisseria meningitides, and Hemophilus influenzae. Mortality rates range between 10 and 40% despite the availability of highly effective antibiotic therapy, and severe neurological damage affects 30-52% of survivors. The causes of death in patients with pneumococcal meningitis are multifactorial and involve both neurological complications such as cerebral edema, hydrocephalus, infarction, and septic sinus or venous thrombosis and systemic complications such as septic shock, disseminated intravascular coagulation, and acute respiratory distress syndrome. We present an unfortunate case of a 42-year-old woman with asplenia and sickle cell disease, admitted for pneumococcal meningitis, who developed diffuse cerebral edema leading to tonsillar herniation and aneurysmal subarachnoid hemorrhage (SAH) with a fatal outcome. To the best of our knowledge, this is the only case ever reported of meningitis complicated by both SAH and brain herniation.

Real-World Effectiveness of Obeticholic Acid in Patients with Primary Biliary Cholangitis.

Patients with primary biliary cholangitis (PBC) with incomplete response to ursodeoxycholic acid are at risk of disease progression and need additional therapy. Obeticholic acid (OCA) was approved in Canada in May 2017, but its effectiveness in a real-world setting has not been described. We sought to describe our experience with OCA in a Canadian cohort. OCA-naive patients treated at two Canadian centers were included. Clinical and biochemical data were collected at OCA initiation and during follow-up. Primary outcomes were changes in serum alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and total bilirubin (TB) over the duration of therapy. Secondary outcomes were changes in alanine aminotransferase (ALT), aspartate aminotransferase (AST), immunoglobulin M (IgM), platelets, and albumin; and achievement of the primary endpoint of the original phase 3 study that led to OCA approval (A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis [POISE]), dose reductions, discontinuations, and tolerability. Repeated-measures models were used to assess changes in biochemistry over time. Sixty-four patients were included; 4 carried a diagnosis of overlap with autoimmune hepatitis. Mean age was 54.6 years, median ALP was 250 U/L, TB was 13 µmol/L, platelet count was 225 × 109/L, and 24% had liver stiffness measurements ≥16.9 kPa. There was a significant reduction in mean ALP of 55 U/L (P < 0.001), GGT of 138 U/L (P < 0.001), ALT of 11.9 U/L (P < 0.001), AST of 5.7 U/L (P < 0.05), and IgM of 0.70 g/L (P < 0.001) over 12 months; TB remained stable (P = 0.98). Forty-four patients met POISE-inclusion criteria, 39% (n = 17) of whom had 12-month biochemical measurements. In this subset, 18% (n = 3/17) met the 12-month POISE primary endpoint, but considering follow-up to 19 months, 43% achieved this target (n = 9/21). Pruritus was the most commonly reported complaint. Conclusion: Use of OCA was associated with improvement in biochemical surrogates of outcome in PBC in a real-world setting.

A Unique Interplay of Multiple Predisposing Factors Culminating in a Catastrophic QT Prolongation.

Implantable cardioverter-defibrillators (ICDs) are used in patients without a reversible cause for long QT syndrome (LQTS) and secondary prevention in patients with LQTS-associated sudden cardiac arrest. We present a female patient with multiple reversible factors for QT prolongation, including the use of antidepressants, antidiarrheals, antiemetics, and antihistamines; chronic malabsorption from bariatric surgery; probable Gitelman syndrome and urinary losses of electrolytes, causing QT prolongation which leads to polymorphic ventricular tachycardia and a successfully resuscitated cardiac arrest. Our patient also had history suggestive of probable congenital LQTS with multiple childhood syncopal episodes and a history of seizures in first-degree relatives, further justifying the placement of an ICD. Also, this case gives us an opportunity to delve into the risks of catastrophic QT prolongation in the morbidly obese population undergoing bariatric surgery.

Acellular Dermal Matrix (Permacol®) for Heterologous Immediate Breast Reconstruction after Skin-Sparing Mastectomy in Patients with Breast Cancer: A Single-Institution Experience and a Review of the Literature.

OBJECTIVE: Skin-sparing mastectomy (SSM) with immediate heterologous reconstruction is a safe oncological option in surgical therapy of early breast cancer. Permacol® is an acellular dermal matrix (ADM) placed between the implant and the skin to improve lower pole projection and implant coverage. The aim of our study was to evaluate the outcome with a focus on patient satisfaction after 6 months and to analyze physical changes of ADM. METHODS: 10 patients who underwent SSM with Permacol® were analyzed retrospectively. All patients were followed using a satisfaction questionnaire and an ultrasound evaluation of the tissue thickness of the pectoralis muscle and the Permacol®. RESULTS: No intraoperative complications were observed. One patient required removal of the implant for necrosis after 3 months. Half of the patients underwent secondary corrective surgery. A statistically significant thinning of the pectoralis muscle was observed, compared to the thickening of the Permacol®. A majority of the patients were satisfied with the operation, and we found a correlation between lower body mass index and patient satisfaction. CONCLUSION: In our small case series Permacol®-assisted immediate reconstruction is shown to be an option for selected cases. Physical changes of Permacol® result in a symmetrical coverage of the implant, which may improve cosmetic outcome.