http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/17-4-reading-leung a video presentation of this article http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/17-4-interview-leung the interview with the author
Background. Available treatments for Wilson disease (WD) prevent longterm complications of copper accumulation. Current anti-copper agents include zinc salts, penicillamine, and trientine. Patients with WD may switch between the agents for a number of reasons. Due to the different mechanisms of action between the copper chelators and zinc salts, transitioning could require a period of overlap and increased monitoring. There are no large studies that investigate the best transition strategies between agents. In this article, we review the treatments for WD and how to monitor for treatment efficacy. Case Summary. The patient had been diagnosed with WD for over 20 years prior to establishing care in our Hepatology Clinic. During his initial course, he was transitioned from penicillamine to zinc due to evidence suggesting penicillamine had greater adverse effects in the long term. Later, he was switched to trientine. His liver enzymes and 24-hour urine copper were monitored. During these years, he intermittently had some financial hardship, requiring him to be on penicillamine rather than trientine. He also had developed acute kidney injury. Overall, his liver disease remained under control and he never had signs of decompensated cirrhosis, but had fluctuations of liver enzymes over the years. Conclusion. Anti-copper treatment for WD has to be tailored to medication side effects profile, patient’s chronic and emerging comorbidities, as well as costs. Transitioning regimens is often challenging, and it requires closer monitoring, with no predictors of response.
Rationale for hepatic artery infusion (HAI) chemotherapyThere are approximately 150,000 new cases of colorectal carcinoma diagnosed annually in the United States (1). Approximately 25% of patients present with metastatic disease at the time of diagnosis (2) and over 50% will develop metastasis to the liver at some point in their lifetime (3). Five-year overall survival (OS) in metastatic colorectal cancer (CRC) confined to the liver is approximately 20%, although complete resection can increase 5-year survival to over 50% in selected series (4-7).Liver resection provides the only chance for cure in patients with colorectal liver metastases (CRLM), however, only 15-20% of patients with CRC metastases confined to the liver are deemed resection candidates at presentation. Most patients with CRC die from metastatic disease, and two-thirds of CRC deaths are due to liver metastases (8).For patients with initially unresectable CRLM, regional treatment of metastatic disease has been a topic of considerable interest given the underwhelming response rates to systemic chemotherapy alone with median survival of roughly 20 months (9). First line systemic chemotherapy for metastatic CRC includes a fluoropyrimidine combined with other agents in various schedules (10). Despite the advances of modern combination regimens [e.g., 5-FU/ leucovorin/oxaliplatin (FOLFOX), 5-FU/leucovorin/ irinotecan (FOLFIRI) and 5-FU/leucovorin/oxaliplatin/ irinotecan (FOLFOXIRI) and targeted therapies including anti-epidermal growth factor receptor (EGFR) for RAS wild-type tumors, and anti-vascular endothelial growth
Background We provide detailed analysis and outcomes in patients post‐kidney transplant (KT) developing ascites, which has never been categorically reported. Methods Ascites was identified by ICD9/10 codes and detailed chart review in patients post‐KT from 01/2004‐06/2019. The incidence of patient death and graft loss were determined per 100‐person‐years, and the incidence rate ratio was obtained. Results Of 3329 patients receiving KT, 83 (2.5%) patients had new‐onset ascites, of whom 58% were male, 21% blacks, and 29% whites. Seventy‐five percentage were on hemodialysis. Patients were maintained primarily on tacrolimus and mycophenolate for immunosuppression. Only 14% of patients with ascites had the appropriate diagnostic workup. There was a trend toward an increased mortality in patients with ascites (incidence rate ratio, IRR [95% CI]: 1.8 [0.92, 3.19], p = .06), and a significantly higher incidence of graft loss (IRR: 5.62 [3.97, 7.76], p < .001), compared with non‐ascites patients. When classified by ascites severity, determined by imaging, moderate/severe ascites patients had the worst clinical outcomes, with a mortality of 32% and graft failure in 57%, compared with 9% and 10%, respectively, in those without ascites. Conclusion In this large cohort employing stepwise analysis of ascites post‐KT, worse outcomes were noted, dictating the need for optimized management to improve clinical outcomes.
INTRODUCTION: Non-alcoholic fatty liver disease (NAFLD) has emerged as the most common cause of chronic liver disease. NAFLD affects ∼1/3rd of the US population, with ∼70% prevalence in diabetics of whom 4-5% have advanced fibrosis. Despite its prevalence, NAFLD is under-recognized by physicians. Defining at-risk NAFLD patients is a cost-effective strategy. There is limited data on the referral process of NAFLD patients to help incorporate changes within health systems. In this study we queried referral trends for NAFLD, with specific focus on diabetes patients and non-invasive fibrosis score. METHODS: This is a retrospective analysis of referrals to gastroenterology at a tertiary care referral center for a large part of Northern California, for fatty liver disease between Jan 1, 2017 – December 31, 2018. Demographics, laboratory data, imaging (Fibroscan, MR elastography), comorbidities and liver biopsy, if available, were recorded. Well-validated FIB-4 score was calculated. RESULTS: Of 435,739 active patients at the center, 31,934 (7%) were diabetics. In 2 years, 188 referrals were made for NAFLD of whom 23% had diabetes, 67% did not have diabetes, 8% were unknown. Primary care providers made 86% referrals; there were no referrals from endocrinology. Approximately half of referrals (46%) had elevated ALT. Among them, 40% were referred for chief complaint of abnormal labs. As noted in the Table 1, FIB-4 >2.67 was seen in 14% of diabetics and 12% non-diabetics, while FIB-4< 1.3 was 39% and 57% of diabetics and non-diabetics. Nearly half of the referred patients underwent fibroscan, MRE or liver biopsy with the tests primarily in those with higher FIB-4 (66% of those with FIB-4≥1.3). Based on biopsy and/or imaging, 45% had stage 0-1 fibrosis while 24% and 6% were stages 2-3 or 4, respectively. Of those with FIB-4< 1.3, 84% had fibrosis 0-1 on further imaging/biopsy, while in those with FIB-4 1.3- 2.67 or >2.67 had stage 2-4 fibrosis in 29% and 54%, respectively. CONCLUSION: In the span of two years, only a handful of diabetic patients with NAFLD have been linked to care, with a notable lack of referrals from endocrinology. Majority of the referrals were for those with low FIB-4 scores, potentially missing a large percentage of advanced fibrosis patients. Our data shows a significant need for systems change and provider education to link-to-care patients at high risk of advanced NAFLD, with FIB-4 as a starting point.
Objective-To study and compare the eVects of mental and physical stress on long QT syndrome (LQTS) patients. Design-Case-control study. Main outcome measures-QT intervals were measured from lead V3. Serum potassium and plasma catecholamine concentrations were also monitored. Patients-16 patients with type 1 LQTS (LQT1), 14 with type 2 LQTS (LQT2), both groups asymptomatic, and 14 healthy control subjects. Interventions-Three types of mental stress tests and a submaximal exercise stress test. Results-Heart rate responses to mental stress and exercise were similar in all groups. During mental stress, the mean QT interval shortened to a similar extent in controls (-29 ms), LQT1 patients (-34 ms), and LQT2 patients (-30 ms). During exercise, the corresponding QT adaptation to exercise stress was more pronounced (p < 0.01) in healthy controls (-47 ms) than in LQT1 (-38 ms) or LQT2 patients (-38 ms). During exercise changes in serum potassium concentrations were correlated to changes in QT intervals in controls, but not in LQTS patients. LQT1 and LQT2 patients did not diVer in serum potassium, catecholamine or heart rate responses to mental or physical stress. Conclusions-QT adaptation to mental and exercise stress in healthy people and in patients with LQTS is diVerent. In healthy people QT adaptation is more sensitive to physical than to mental stress while no such diverging pattern was seen in asymptomatic LQTS patients.
INTRODUCTION: Percutaneous radiofrequency ablation (RFA) is now a common minimally invasive procedure for the maintenance of sinus rhythm in drug-resistant atrial fibrillation. We present a rare case of acute delayed gastric emptying after an RFA procedure. CASE DESCRIPTION/METHODS: 57-year-old male with a history significant only for paroxysmal atrial fibrillation, who presented for a planned RFA procedure. He has no other medical or surgical history. The patient tolerated the procedure well and was discharged the same day. That evening, 1-2 hours after he ate dinner, he experienced severe nausea, vomiting, and abdominal pain. He was hemodynamically stable on presentation to the emergency department with labs significant only for a mild leukocytosis. Computed tomography showed a severely dilated stomach and proximal duodenum, with a transition point identified at the third portion of the duodenum. The patient was managed conservatively as a case of proximal small bowel obstruction. As he had mild improvement in symptoms after five days, the decision was made for an exploratory laparotomy. An intra-operative push enteroscopy demonstrated large amounts of retained food in the stomach, but no gastroduodenal outlet obstruction. A diagnosis of acute gastroparesis syndrome was made, and surgical exploration was not done. The patient was discharged, managed conservatively, and on follow-up 4 weeks later his symptoms had significantly improved. DISCUSSION: Acute gastroparesis syndrome is a rare complication of RFA for atrial fibrillation, that occurs due to thermal injury to the peri-esophageal vagal nerves during ablation. Symptoms of nausea, bloating, vomiting, gastric pain occur within 72 hours after the procedure. Most patients fully recover within 1-2 months after the procedure with conservative management. However, a few reported cases with persistent symptoms required eventual esophagojejunal anastomosis or intra-pyloric botulinum injection. Due to wide variation in vagal plexus anatomy around the lower esophagus, and lack of visualization of the nervous system, nerve injury is hard to avoid. Higher BMI was noted to be a protective. Minimizing RF ablative power around the esophagus and/or esophageal temperature monitoring may be a strategy to lower the risk. As RFA ablation for atrial fibrillation becomes more common, gastroenterologists should be aware of this complication.
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