Adult Intussusception in Chronic Marijuana Users.

Intussusception is common in children, but it is rare in adults. The most common causes of adult intussusception (AI) are due to a pathological lead point with a common etiology being malignancy. Intra-luminal irritants should be considered the possible etiology of intussusception in patients without a pathological lead point. Marijuana use has increased dramatically in the United States over the last decade. With increasing public acceptance and legalization of marijuana, various adverse side effects have become more prominent. Marijuana has been shown to disrupt gastrointestinal tract motility by inhibiting cholinergic mechanisms. Here we describe four cases of AI who are chronic marijuana users. This well-referenced review gives attention to the harmful effects of marijuana, given the increasing use of marijuana and its derivatives in the United States.

Procalcitonin and C-reactive protein in the diagnosis of spontaneous bacterial peritonitis.

Background: Spontaneous bacterial peritonitis (SBP) is a serious complication of cirrhosis and is associated with high morbidity and mortality. Rapid institution of appropriate antibiotics is central to the improved patient outcome. Correctly obtaining ascites fluid for analysis has several technical and logistic limitations resulting in overuse of empiric antibiotics when patients are admitted to the hospital with suspected SBP. Procalcitonin and C-reactive protein (CRP) are non-invasive markers of infection. We conducted a study to illustrate the role of these markers in making the diagnosis of SBP in patients with cirrhosis. Methods: A total of 45 patients were enrolled in this prospective cohort study, 14 (31.1%) of which were found to have SBP. Ascitic fluid neutrophils, serum procalcitonin and CRP levels were measured prior to initiation of antibiotics and these parameters were compared between the two groups. Area under receiver operator characteristic (AUROC) curves were used to assess the diagnostic accuracy of procalcitonin and CRP in this population. We defined neutrocytic SBP group as a combination of patients who had classic SBP (positive ascitic culture and >250 neutrophils/mm3) and culture-negative neutrocytic ascites. Results: Serum procalcitonin (2.81±2.59 vs. 0.43±0.48 ng/mL; P=0.0032), serum CRP (60.30±44.48 vs. 22.2±23.28; P=0.0055) and ascitic fluid neutrophil levels (49.23±30.90 vs. 16.7±20.39; P=0.0064) were significantly higher in SBP group than non-SBP group. AUROC for procalcitonin (cut-off >2.0 ng/mL) was 0.75 (95% CI, 0.61-0.88), CRP (cut-off >3.0 mg/L) was 0.55 (95% CI, 0.43-0.68) and for procalcitonin combined with CRP was 0.76 (95% CI, 0.61-0.90) for diagnosing all-cause SBP. In a subgroup analysis of patients with neutrocytic SBP, AUROC for procalcitonin was 0.88 (95% CI, 0.74-1.00), CRP was 0.62 (95% CI, 0.45-0.79) and for procalcitonin combined with CRP was 0.93 (95% CI, 0.81-1.00). Addition of CRP to procalcitonin did not significantly change the AUROC for diagnosis of SBP. Conclusions: Serum procalcitonin could be used as an adjunctive non-invasive biomarker in diagnosing SBP with a high degree of accuracy in cirrhotic patients. Addition of CRP does not seem to significantly increase the diagnostic accuracy of procalcitonin.

Budd-Chiari Syndrome as an Initial Presentation of Non-Promyelocytic Acute Myelogenous Leukemia.

Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction, frequently due to an underlying thrombophilic disorder. Acute myeloid leukemia rarely presents as acute BCS due to hyperfibrinolysis, hyperleukocytosis, nonspecific proteolytic activity, and disseminated intravascular coagulation causing acute hepatic vein thrombosis. In patients presenting with acute BCS with acute liver failure (ALF), a high index of suspicion and exclusion of underlying malignancy is a must, as it is a contraindication for liver transplantation. We report a case of a 19-year-old Caucasian male who presented with acute BCS causing ALF as an initial presentation of acute myelogenous leukemia.

Disseminated Histoplasmosis Involving Soft Palate, Duodenum, Sigmoid Colon and Bone Marrow in a Patient With Isolated CD4+ T-Lymphocytopenia.

Idiopathic CD4 T-lymphocytopenia (ICL) is a rare entity that is associated with decreased immunity which predisposes affected individuals to opportunistic infections and malignancies. Autoimmune conditions are common in patients with ICL and they are considered part of its clinical spectrum as well. Treatment of ICL includes treatment of opportunistic infections and prophylaxis against them. Some cases are self-limited while others require long-term monitoring. We present a case of a 60-year-old man who was diagnosed with disseminated histoplasmosis involving soft palate, duodenum, colon and bone marrow in the setting of idiopathic CD4 T-lymphocytopenia.

Clinical and Genetic Risk Factors of Recurrent Nonalcoholic Fatty Liver Disease After Liver Transplantation.

INTRODUCTION: Nonalcoholic fatty liver disease (NAFLD) has been increasingly reported among recipients of liver transplantation (LT). We aimed to identify clinical and genetic risk factors responsible for the development of early recurrent NAFLD in nonalcoholic steatohepatitis transplant recipients. METHODS: Forty-six total single nucleotide polymorphisms with known association with NAFLD were tested among both recipient and donor liver samples in 66 LT recipients with nonalcoholic steatohepatitis to characterize influences on NAFLD recurrence at ∼1 year post-LT (median interval from LT to biopsy: 377 days). RESULTS: Recurrent NAFLD was identified in 43 (65.2%) patients, 20 (30.3%) with mild recurrence, and 23 (34.8%) with moderate to severe NAFLD. On adjusted analysis, change in the body mass index (BMI) (ΔBMI) was significantly associated with NAFLD recurrence, whereas post-LT diabetes mellitus was associated with increased severity of NAFLD recurrence. ADIPOR1 rs10920533 in the recipient was associated with increased risk of moderate to severe NAFLD recurrence, whereas the minor allele of SOD2 rs4880 in the recipient was associated with reduced risk. Similar reduced risk was noted in the presence of donor SOD2 rs4880 and HSD17B13 rs6834314 polymorphism. DISCUSSION: Increased BMI post-LT is strongly associated with NAFLD recurrence, whereas post-LT diabetes mellitus was associated with increased severity of NAFLD recurrence. Both donor and recipient SOD2 rs4880 and donor HSD17B13 rs6834314 single nucleotide polymorphisms may be associated with reduced risk of early NAFLD recurrence, whereas presence of the minor allele form of ADIPOR1 rs10920533 in the recipient is associated with increased severity NAFLD recurrence.

Recurrent Cholangiocarcinoma Presenting as Sister Mary Joseph Nodule After Liver Transplantation.

Hilar cholangiocarcinoma, also known as Klatskin tumor, is the most common type of cholangiocarcinoma. It usually has a lymphatic spread and is rarely associated with an umbilical nodule, also known as Sister Mary Joseph nodule. We report a case of a 53-year-old Caucasian man with hilar cholangiocarcinoma. The patient had an inoperable tumor and was referred to our center for liver transplantation. Post liver transplantation, the patient presented with a recurrence of the carcinoma in the umbilical region. The patient was found to have Sister Mary Joseph nodule. It carries a poor prognosis, and our patient succumbed to the illness in four months. Cholangiocarcinoma carries a poor prognosis. Surgical resection and liver transplantation with neoadjuvant chemoradiation are the preferred treatment strategies. Association of cholangiocarcinoma with umbilical metastasis is rare, and our patient had an even rarer presentation in the form of recurrence with umbilical nodule post-liver transplantation. We want to increase the awareness of the rare presentation, association, and recurrence of hilar cholangiocarcinoma in the form of umbilical nodule post-liver transplantation.

Gastric schwannoma.

Gastric Schwannomas are rare benign slow-growing tumors and warrant treatment/resection only when symptomatic. Watchful waiting is recommended for incidental or asymptomatic schwannomas.

Hyperammonemic encephalopathy associated with 5-fluorouracil in a patient with previous orthotopic liver transplantation.

5-Fluorouracil (5-FU)-based chemotherapy is the most common regimen used to treat metastatic or advanced colon cancer. Neurotoxicity is a rare but serious adverse effect of 5-FU-based therapy. We report an unusual case of 5-FU-induced hyperammonemic encephalopathy in a patient with recurrent colon cancer that metastasized to the liver and lung. Two days after receiving the third dose of FOLFOX, he presented with altered mental status, agitation, abdominal pain, nausea, and vomiting. His total ammonia level was 434 µmol/L, for which he was treated with lactulose retention enema and lactulose via nasogastric tube. Over the next 24 hours, his condition improved significantly.

Granulomatous Myositis Associated with Extremely Elevated Anti-striated Muscle Antibodies in the Absence of Myasthenia Gravis.

Granulomatous myositis is a rare disease that predominantly results in proximal muscle weakness in the upper and/or lower extremities. As it can resemble other inflammatory myopathies, it is important to obtain a muscle biopsy to make the underlying diagnosis. We report the first case of granulomatous myositis associated with extremely elevated anti-striated muscle antibodies in a 69-year-old Caucasian woman. Granulomatous myositis has been associated with various autoimmune, infectious, rheumatologic, vasculitis, and oncologic disorders, and several antibodies have previously been reported to be associated with it. However, to the best of our knowledge, this is the first report where extremely elevated anti-striated muscle antibodies were found to be associated with granulomatous myositis in the absence of myasthenia gravis. The treatment of granulomatous myositis revolves around the use of corticosteroids, steroid-sparing immunosuppressive agents, and newer biologics.

Terry's nails.

Terry's nails can be manifested in systemic diseases like cirrhosis, congestive heart failure, diabetes mellitus, renal failure, and other conditions which emphasizes the importance of physical examination in every clinical encounter.

Intrahepatic Cholangiocarcinoma Associated with High Procalcitonin, Hypercalcemia, Polycythemia and Leukocytosis.

Intrahepatic cholangiocarcinomas or bile duct cancers comprise approximately 10-20% of all cholangiocarcinomas and may present with right upper quadrant pain, weight loss, liver enzyme abnormalities or they may be completely asymptomatic and be picked incidentally on routine abdominal imaging. Typically, hepatocellular carcinomas have been associated with various paraneoplastic syndromes such as hypercalcemia, erythrocytosis, hypoglycemia, diarrhea and skin changes though paraneoplastic syndromes in the setting of cholangiocarcinoma do occur as well. Cholangiocarcinomas are usually associated with dermal paraneoplastic syndromes (Sweet syndrome, porphyria cutanea tarda, acanthosis nigricans, necrotic migratory erythema, erythema multiforme, bullous pemphigoid), hypercalcemia, leukocytosis and limbic encephalitis. We present a case of an 80-year-old man with intrahepatic cholangiocarcinoma associated with high procalcitonin levels in the absence of infection, paraneoplastic syndromes of hypercalcemia, polycythemia and leukocytosis in the same individual. This constellation of symptoms, to the best of our knowledge, has not been previously reported in the scientific literature.

Magnitude of Nonalcoholic Fatty Liver Disease: Western Perspective.

The incidence of nonalcoholic fatty liver disease (NAFLD) is continuing to rise worldwide, and it is estimated that this disquieting trend will continue for another 10-15 years before prevalence begins to decrease. NAFLD is the hepatic manifestation of metabolic syndrome. As obesity, diabetes, and other lifestyle-related diseases continue to rise, the spectrum of NAFLD, e.g., nonalcoholic steatohepatitis, liver fibrosis, liver cirrhosis, liver-related morbidity, and mortality, will increase in parallel. Its widespread prevalence and associated economic burden have drawn significant attention, and a multitude of pharmaceutical companies are participating in active research trying to find a "cure". Unfortunately, as of now, no targeted treatment exists to treat this condition, and therefore, emphasis has been on its prevention. The current review focuses on the epidemiology, clinical characteristics, risk factors, and clinical outcomes of NAFLD in Western countries. It is important to understand the magnitude of NAFLD and its risk factors in Western countries where the prevalence of NAFLD has now reached epidemic proportions to identify the best strategy to prevent and possibly control this epidemic.

Enterococcus casseliflavus, Streptococcus equinus and Klebsiella oxytoca septicaemia associated with perinephric haematoma in a post-liver transplant patient with allograft cirrhosis.

Liver transplant recipients are immunocompromised by the virtue of being on immunosuppressive agents which put them at risk of having infections from unusual and even multiple concomitant pathogens. We present a case of a 39-year-old man who developed septicaemia with Enterococcus casseliflavus, Streptococcus equinus and Klebsiella oxytoca in the setting of perinephric haematoma which resulted following a kidney biopsy performed to evaluate his nephrotic range proteinuria. E. casseliflavus has been known to cause infections in patients with liver disease/cirrhosis; however, simultaneous infection with S. equinus and K. oxytoca along with E. casseliflavus has never been reported earlier in post-transplant state.

Liver Transplantation for Nonalcoholic Steatohepatitis: Pathophysiology of Recurrence and Clinical Challenges.

Nonalcoholic steatohepatitis is the fastest-growing indication for the liver transplant and a leading cause of hepatocellular carcinoma among patients listed for liver transplantation in the USA. Post-transplant nonalcoholic hepatic steatosis and steatohepatitis are frequent complications of liver transplantation. Nonalcoholic steatohepatitis poses a significant challenge in both pre- and post-transplant period due to its association with metabolic syndrome, coronary artery disease, chronic kidney disease, and obstructive sleep apnea. While optimal therapy is not yet available in the post-liver transplant setting, lifestyle interventions continue to remain as the mainstay of therapy for post-transplant nonalcoholic steatohepatitis. Early recognition with protocol biopsies and noninvasive modalities, along with modification of known risk factors, are the most effective methods to curtail the progression of nonalcoholic steatohepatitis in the absence of FDA-approved pharmacologic therapy.

Transplantation of kidneys from hepatitis C-infected donors to hepatitis C-negative recipients: Single center experience.

Our aim was to evaluate the safety of transplanting kidneys from HCV-infected donors in HCV-uninfected recipients. Data collected from 53 recipients in a single center, observational study included donor and recipient characteristics, liver and kidney graft function, new infections and de novo donor-specific antibodies and renal histology. Treatment with a direct-acting antiviral regimen was initiated when HCV RNA was detected. The mean ± SD age of recipients was 53 ± 11 years, 34% were female, 19% and 79% of recipients were white and African American, respectively. The median and interquartile range (IQR) time between transplant and treatment initiation was 76 (IQR: 68-88) days. All 53 recipients became viremic (genotype: 1a [N = 34], 1b [N = 1], 2 [N = 3], and 3 [N = 15]). The majority (81%) of recipients did not experience clinically significant increases (>3 times higher than upper limit of the normal value) in aminotransferase levels and their HCV RNA levels were in the 5 to 6 log range. One patient developed fibrosing cholestatic hepatitis with complete resolution. All recipients completed antiviral treatment and 100% were HCV RNA-negative and achieved 12-week sustained virologic response. The estimated GFRs at end of treatment and 12-week posttreatment were 67 ± 21 mL/min/1.73 m2 and 67 ± 17 mL/min/1.73 m2 , respectively. Four recipients developed acute rejection. Kidney transplantation from HCV-infected donors to HCV-negative recipients should be considered in all eligible patients.

Metoclopramide-induced Serotonin Syndrome.

Serotonin syndrome is a clinical diagnosis characterized by a constellation of autonomic and neurological physical examination findings due to the use of one or more serotonergic agents. Due to high morbidity and mortality associated with this condition, high index of suspicion is required in making this diagnosis. Treatment is aimed at discontinuation of the offending agent and supportive care. We present a case of a 28-year-old woman who presented with acetaminophen toxicity, however developed iatrogenic serotonin syndrome due to use of scheduled intravenous metoclopramide. Metoclopramide, by itself, very rarely causes serotonin syndrome and typically results in this condition when used in combination with other pro-serotonergic agents.