Predictors of Biliary Strictures After Liver Transplantation Among Recipients of DCD (Donation After Cardiac Death) Grafts.

INTRODUCTION: Biliary strictures are a common complication among donation after cardiac death (DCD) liver transplantation (LT) recipients and may require multiple endoscopic retrograde cholangiopancreatography (ERCP) procedures. We evaluated the risk factors associated with development of biliary strictures in DCD LT recipients. METHODS: DCD LT recipients who underwent transplantation from 2012 to 2017 were divided into 2 groups: (a) those with anastomotic or non-anastomotic biliary strictures who required ERCP ("stricture group") and (b) those who did not require ERCP or had cholangiograms without evidence of biliary strictures ("non-stricture group"). Clinical data, cholangiograms and laboratory values at day 0 and day 7 after LT were compared between the two groups. RESULTS: Forty-nine of the 100 DCD LT recipients underwent ERCP. Thirty-four of these 49 LT recipients had evidence of anastomotic or non-anastomotic biliary strictures (stricture group), while the remaining 66 LT recipients comprised the non-stricture group. Donor age was significantly higher in stricture group compared to non-stricture group (49.2 ± 1.8 vs 42.8 ± 1.57 years, respectively; p = 0.01). The stricture group had a significantly higher total bilirubin at day 0 (3.5 ± 0.37 vs 2.6 ± 0.21 mg/dL; p = 0.02) and INR at day 7 (1.24 ± 0.06 vs 1.13 ± 0.01; p = 0.048) compared to the non-stricture group. Multi-variate analysis demonstrated significant association between biliary strictures and total bilirubin at day 0 of LT and age of donor. CONCLUSION: Biliary strictures occur frequently in DCD LT recipients and may be associated with older age of donor. Hyperbilirubinemia immediately after transplant and higher INR in the first 7 days after transplant may predict subsequent development of biliary strictures.

Primary sclerosing cholangitis in children versus adults: lessons for the clinic.

INTRODUCTION: Primary sclerosing cholangitis (PSC) is a rare cholestatic disorder that presents with multifocal biliary strictures. PSC has a variable course but often leads to progressive liver disease, and most patients will eventually require liver transplantation. PSC has a strong association with inflammatory bowel disease and autoimmune liver disease. Areas covered: The objective of this article is to compare and contrast the clinical features and natural history of PSC in children to adults. We performed a PubMed search of the English literature using keywords 'primary sclerosing cholangitis', 'PSC', 'children', and 'pediatric.' Expert commentary: While certain features of PSC are similar in the pediatric and adult population, there are unique features of pediatric PSC. More longitudinal studies are needed to better understand the natural history of pediatric PSC. It is conceivable that treatment for PSC that will alter the course of disease may become available in the future.

Metastatic renal cell carcinoma presenting with melena.

Renal cell carcinoma is a highly malignant neoplasm. Metastasis to the pancreas and gastrointestinal tract is rare. In this case report, we show images of metastatic renal cell carcinoma to the upper gastrointestinal tract in a patient who presented with melena.

An incidental windsock diverticulum.

Windsock diverticulum is a rare anomalous finding of a true intraluminal duodenal diverticulum. While complications of bowel obstruction, bleeding, and pancreatitis may occur, most patients are asymptomatic. Surgical or endoscopic management may be pursued when complications develop.

Is the level of cleanliness using segmental Boston bowel preparation scale associated with a higher adenoma detection rate?

BACKGROUND: The impact of Boston bowel preparation scale (BBPS) scores on the adenoma detection rate (ADR) in each segment has not been adequately addressed. The aim of this study was to determine the association between segmental or overall ADR and serrated polyp detection rate (SDR) with segmental and total BBPS scores. METHODS: All outpatient screening colonoscopies with documented BBPS scores were retrospectively reviewed at a tertiary institution from January to December 2013. Chi-square tests and logistic regression were used to analyze the detection rates of adenomas and serrated polyps with bowel prep scores. Odds ratios were calculated using logistic regression that controlled for withdrawal time, age, body mass index, diabetes status and sex. RESULTS: We analyzed 1991 colonoscopies. The overall ADR was 37.5% (95% confidence interval [CI], 35.3-39.6). There was a significant difference in the overall ADR, and in SDR across all bowel category groups, with total BBPS scores of 8 and 9 having lower detection rates than scores of 5, 6 and 7. As the quality of bowel preparation increased, there was a statistical decrease in the ADR (odds ratio [OR] 0.79 [CI 0.66-0.94], P=0.04) of the right colon, while in the left colon, there was a statistical decrease in SDR (OR 0.78, [CI 0.65-0.92] P=0.019). CONCLUSION: Segmental ADR and SDR both decreased as prep scores increased, decreasing notably in patients with excellent prep scores of 8 and 9. A possible explanation for this unexpected discrepancy may be related to longer and better visualization of the mucosa when cleansing and suctioning is necessary.

Small Intestinal Bacterial Overgrowth: Nutritional Implications, Diagnosis, and Management.

Small intestinal bacterial overgrowth (SIBO), characterized by the presence of excessive bacteria in the small intestine, is typically described as a malabsorptive syndrome occurring in the context of gut stasis syndromes. SIBO is now considered to be a disorder associated with diverse clinical conditions without classic risk factors for SIBO and a cause of several nonspecific gastrointestinal and nongastrointestinal symptoms. Because there is currently no gold standard for diagnosing SIBO, its prevalence and role in the pathogenesis of other diseases remain uncertain; as does optimal treatment of patients with relapsing symptoms.

International Normalized Ratio Does Not Predict Gastrointestinal Bleeding After Endoscopic Retrograde Cholangiopancreatography in Patients With Cirrhosis.

BACKGROUND: Cirrhosis is often accompanied by an elevated international normalized ratio (INR) due to a decrease in pro-coagulant factors. An elevated INR in cirrhosis is often interpreted as an increased risk of bleeding. There are a paucity of data in the literature on the use of INR to predict risk of gastrointestinal bleeding (GIB) following endoscopic retrograde cholangiopancreatography (ERCP) in patients with cirrhosis. The aims of the study were to determine if there is a correlation between INR and GIB following ERCP in patients with cirrhosis, and to determine if there is a difference in frequency of post-ERCP complications in patients with and without cirrhosis. METHODS: A retrospective review of all ERCP procedures was performed at a tertiary care institution between 2012 and 2015. We identified ERCPs performed in patients with cirrhosis and compared them to a randomly selected group without liver cirrhosis. Univariate analysis was performed using Chi-square and ANOVA tests. A multivariable logistic regression model using generalized estimating equations was used to examine the association between INR and GIB. RESULTS: There were a total of 1,610 ERCPs performed from 2012 to 2015 with 129 performed in 56 patients with cirrhosis compared with 392 ERCPs performed in 310 patients without cirrhosis. There was no difference in the frequency of GIB following ERCP in both groups (P = 0.117). However, there was a difference in overall complications between both groups (P = 0.007), but no difference observed amongst Child-Turcotte-Pugh classes (P = NS). In a multivariable analysis, sphincterotomy during ERCP (odds ratio (OR) = 3.22; 95% confidence interval (CI): 1.05 - 9.94; P = 0.042) and cirrhosis (OR = 3.58; 95% CI: 1.22 - 10.47; P = 0.02) were significant for predicting GIB. Anti-coagulation (OR = 2.90; 95% CI: 0.82 - 10.23; P = 0.097) and INR were not significant in the multivariable model (OR = 2.09; 95% CI: 0.85 - 5.12; P = 0.10). CONCLUSION: There was a statistical difference in overall complications between patients with and without cirrhosis but no difference was observed amongst Child-Turcotte-Pugh classes. Overall, INR was not a significant factor in predicting risk of bleeding in patients after ERCP.

Right Hepatic Lobe Resection and Thrombocytopenia

Abstract: Thrombocytopenia has previously been reported after right lobe resection for organ donation. The mechanism(s) of low platelets after right hepatectomy is unclear and several hypotheses have been proposed including a decrease in thrombopoietin, and hepatic insufficiency resulting in relative portal hypertension following hepatic resection. However, there has previously not been any comparison between patients who undergo hepatic resection for neoplasia vs. for living organ donation. We compared platelet values in the postoperative period of patients who underwent right hepatectomy for living donation (n = 93) to those who underwent hepatectomy for neoplasia (n = 21). There was no significant difference in platelet values between the two groups at one month (291.2 ( 100 vs. 285.73 ( 159, p = NS), three months (223.8 ( 61 vs. 185.27 ( 80, p = NS) and at 12 months (212 ( 44 vs. 191 ( 60, p = NS). We conclude that thrombocytopenia is not uncommon following hepatic lobe resection, and is unaffected by the indication for hepatectomy.

Right hepatic lobe resection and thrombocytopenia.

 Thrombocytopenia has previously been reported after right lobe resection for organ donation. The mechanism(s) of low platelets after right hepatectomy is unclear and several hypotheses have been proposed including a decrease in thrombopoietin, and hepatic insufficiency resulting in relative portal hypertension following hepatic resection. However, there has previously not been any comparison between patients who undergo hepatic resection for neoplasia vs. for living organ donation. We compared platelet values in the postoperative period of patients who underwent right hepatectomy for living donation (n = 93) to those who underwent hepatectomy for neoplasia (n = 21). There was no significant difference in platelet values between the two groups at one month (291.2 ± 100 vs. 285.73 ± 159, p = NS), three months (223.8 ± 61 vs. 185.27 ± 80, p = NS) and at 12 months (212 ± 44 vs. 191 ± 60, p = NS). We conclude that thrombocytopenia is not uncommon following hepatic lobe resection, and is unaffected by the indication for hepatectomy.

A Mysterious DRESS Case: Autoimmune Enteropathy Associated with DRESS Syndrome.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a rare but potentially life-threatening cutaneous hypersensitivity reaction characterized by extensive mucocutaneous eruption, fever, hematologic abnormalities, and extensive organ involvement. Here, we present a case of a young woman with DRESS syndrome following exposure to vancomycin with renal, cutaneous, and gastrointestinal involvement. To the best of our knowledge, this is the first case description in the literature of DRESS of the gastrointestinal tract with autoimmune enteropathy.

An Unusual Suspect: Lymphoepithelial Cyst of the Pancreas.

Lymphoepithelial cysts (LECs) of the pancreas are benign, rare pancreatic cysts that are found predominantly in men. These cysts can present as a diagnostic conundrum given their rarity and difficulty of distinguishing these cysts from those with malignant potential. We present an incidental case of a LEC in a middle-aged man.

Pseudocirrhosis: A Case Series and Literature Review.

Pseudocirrhosis describes morphological changes of the liver that closely mimic cirrhosis, without the typical histopathological changes seen in cirrhosis. It most commonly occurs in patients with metastatic breast cancer, although it has been reported in other malignancies as well. Like in cirrhosis, portal hypertension is often seen in patients with pseudocirrhosis. Pseudocirrhosis is a rare but important complication of metastatic cancer. In this case series and literature review, we describe 6 patients with hormone-receptor-positive metastatic breast cancer. We report the significant morbidity associated with pseudocirrhosis in the course of treatment in patients with metastatic breast cancer.

Primary thyroid lymphoma presenting as dermatomyositis: a first case and review of the literature.

A 55-year-old woman presented with a 3-week history of weakness. On examination, she had a heliotrope rash, V-neck and shawl signs. There was symmetric proximal muscle weakness, and 2/5 strength in upper and lower extremities. Laboratory values showed CK 5836, CRP 14.9, erythrocyte sedimentation rate 49, lactate dehydrogenase 633, negative antinuclear antibodies and anti-Jo1 antibodies. Muscle biopsy and immunohistochemistry findings were consistent with dermatomyositis. Treatment with high-dose steroids, cyclophosphamide and intravenous immunoglobulin (IVIG) was started, without improvement of symptoms. She was found to have cold agglutinins with a cold screen titre of 1:256,256. Work up for malignancy ensued. Positron emission tomography-computed tomography (PET-CT) showed increased uptake in the thyroid. Fine-needle thyroid biopsy with flow cytometry studies revealed atypical lymphocytes consistent with diffuse large B-cell lymphoma. The patient underwent R-CHOP therapy, with remarkable improvement of her symptoms. Dermatomyositis associated with primary thyroid lymphoma has not been previously reported. This case reinforces the importance of cancer screening in dermatomyositis patients.

Olmesartan-Induced Enteropathy.

Olmesartan-induced enteropathy mimics celiac disease clinically and pathologically. As in celiac disease, the pathologic findings are villous atrophy and increased intraepithelial lymphocytes. Clinical presentation of olmesartan-induced enteropathy includes diarrhea, weight loss, and nausea. In contrast to celiac disease, tissue transglutaminase is not elevated and there is no response to a gluten-free diet. Including this entity in the differential diagnosis of sprue-like enteropathy is critical for its early diagnosis since replacing olmesartan with an alternative antihypertensive drug can simplify the diagnostic workup and provide both clinical and histologic improvement.

Case Report: Acute Cerebellar Thrombosis in an Adult Patient with IgM Nephropathy.

IgM nephropathy is a relatively rare cause of idiopathic nephrotic syndrome.1 It was initially described by van de Putte,2 then by Cohen and Bhasin in 1978, as a distinctive feature of mesangial proliferative glomerulonephritis.2 It is typically characterized by diffuse IgM deposits on the glomeruli and diffuse mesangial hypercellularity. Little is known about the pathogenesis and treatment of this disease.1,3 We describe a patient who presented with nonspecific symptoms of epigastric pain, nausea, and early satiety. Abdominal imaging and endoscopies were unremarkable. She was found to have significant proteinuria (6.4 g/24 hours), hyperlipidemia, and edema consistent with a diagnosis of nephrotic syndrome. Kidney biopsy was performed and confirmed an IgM nephropathy. Less than 2 weeks after her diagnosis of IgM nephropathy, she presented with an acute cerebellar stroke. Thrombophilia is a well-known complication of nephrotic syndrome, but a review of the literature failed to show an association between IgM nephropathy and acute central nervous system thrombosis.

Acute Hepatitis E in the US Today Occurs in Diverse Patient Populations: Case Reports.

Hepatitis E has long been thought of as an infection confined to the developing world. However, there has been an increased incidence of locally acquired cases in developed countries especially in transplant patients. Our first case is a 56-year-old Caucasian female post-heart transplant patient who presented with diarrhea and abdominal pain. She was found to be acutely infected with hepatitis E and progressed to stage 3 liver fibrosis. Our second patient was an otherwise healthy 76-year-old Vietnamese female who presented with abdominal pain, jaundice and fatigue. She was diagnosed with acute hepatitis E complicated by acute renal failure. There have only been a few reported cases of acute hepatitis E complicated by renal failure.

Gastrointestinal motility problems in critical care: a clinical perspective.

Advances in surgery, anesthesia and intensive care have led to a dramatic increase in the number of patients who spend time in our intensive care units (ICU). Gastrointestinal (GI) motility disorders are common complications in the intensive care setting and are predictors of increased mortality and length of the stay in the ICU. Several risk factors for developing GI motility problems in the ICU setting have been identified and include sepsis, being on mechanical ventilation and the use of vasopressors, opioids or anticholinergic medications. Our focus is on the most common clinical manifestations of GI motor dysfunction in the ICU patient: gastroesophageal reflux, gastroparesis, ileus and acute pseudo-obstruction of the colon.