Clinicopathological Spectrum of Mitral Valve Myxoma.

Cardiac myxomas are the most common primary tumors of the heart. Although these tumors have been reported in all cardiac chambers, myxomas arising from heart valves are extremely rare. Here, the details are described of a patient with mitral valve myxoma, and a review is provided of 99 cases reported in the literature. Mitral valve myxomas most often occur in middle-aged patients, with a slight female predominance. Most of the tumors arise in an isolated fashion on the atrial side of the anterior mitral valve leaflet. Embolic events were the most frequent manifestation, followed by obstructive symptoms. Unlike atrial wall myxomas, these tumors have a lower incidence of constitutional manifestations. Tumor resection with valve preservation is the usual method of surgical treatment in isolated tumors, while most patients with multiple valve myxomas underwent valve replacement. Mitral valve myxomas should be considered in the differential diagnosis of mitral valve tumors, with an awareness of a relatively high risk of embolic events. This location should not raise suspicion for a heritable myxoma syndrome. Prompt surgical resection is warranted to reduce complications.

[Update on chronic pancreatitis: review article]. / Una actualización de pancreatitis crónica: artículo de revisión.

Chronic pancreatitis is a progressive fibro-inflammatory disease of the pancreas characterized by irreversible fibrosis of the gland with eventual failure of exocrine and endocrine functions and hallmark features of abdominal pain, malabsorption, malnutrition, diabetes mellitus and pancreatic calcifications. In many patients this disease results from a complex mix of environmental (eg, alcohol, cigarettes, and occupational chemicals), genetic factors and a few patients with hereditary or autoimmune disease. The management includes medical, endoscopic and surgical approaches with the need for interaction between various specialties, calling for a concerted multidisciplinary approach. This review provides the reader with a comprehensive overview of the studies summarizing the epidemiology, etiology, physiopatology, clinical manifestation, diagnosis and treatments of the disease.

Hepatotoxicity by herbs: a practical review of a neglected disease.

Herbs are commonly used worldwide for the treatment of various diseases, constituting a multi-billion dollar market. Unfortunately, hepatotoxicity induced by herbs is also common. The true incidence and prevalence are not known. There is need for more strict regulations andexperimental and pre-clinical studies regarding its efficacy and safety. There is no gold standard for the diagnosis of herbs-induced liver injury (HILI) and it constitutes a diagnostic challenge for the clinician, whereestablishing causality could be cumbersome. Clinical presentation varies from asymptomatic cases with mildly abnormal liver tests to fulminant liver failure requiring liver transplantation. In this review, we will discuss the epidemiology, clinical manifestations, challenges and diagnostic approach of HILI and will also present some exemplary cases from the University of Miami, Division of Hepatology.

Type 1 autoimmune pancreatitis: case scenario and review of the disease.

Autoimmune pancreatitis (AIP) is an uncommon disease that represents a diagnostic challenge unless it is considered as a cause of acute pancreatitis, pancreatic exocrine insufficiency and a pancreatic mass. This entity is under diagnosed and successful medical therapy is available. In this paper, we will describe a case of a 59 year-old, Hispanic woman diagnosed with autoimmune pancreatitis, a disease previously believed to affect typically older men. We will review the definition, types, clinical manifestations, radiological features, serology, histopathological findings, treatment strategies and diagnostic criteria of autoimmune pancreatitis.

[Acute respiratory distress syndrome due to Clostridium difficile colitis: a case report and review of the literature]. / Síndrome de distress respiratorio agudo debido a colitis por Clostridium difficile: reporte de caso y revisión de la literatura.

Clostridium difficile is a major cause of antibiotic-associated diarrhea. We report a patient with complicated Clostridium difficile infection (CDI) who developed rapidly progressive acute respiratory distress syndrome (ARDS), for which CDI was the only identifiable source. CDI should be considered in the differential diagnosis for anyone with diarrhea who presents especially in high-risk groups such as the elderly, hospitalized patients, or those who have had a history of CDI.

Orbital Myositis in a Patient With Ileal Crohn's Disease in Remission on Vedolizumab.

Orbital myositis (OM) is an extremely rare ocular extraintestinal manifestation of inflammatory bowel disease. Acute or chronic inflammation of one or more extraocular muscles leads to symptoms related to the mass effect including orbital pain, swelling, ophthalmoplegia, proptosis, and diplopia. Although the use of steroids typically leads to rapid resolution of symptoms, recurrence is common, necessitating long-term steroid-sparing therapies. Given the rare presentation of OM, its pathogenesis and optimal therapy are not well established. We present a young woman with Crohn's disease in remission on vedolizumab who developed OM, and we discuss our management approach.

A patient-like swine model of gastrointestinal fibrotic strictures for advancing therapeutics.

Gastrointestinal (GI) strictures are difficult to treat in a variety of disease processes. Currently, there are no Food and Drug Administration (FDA) approved drugs for fibrosis in the GI tract. One of the limitations to developing anti-fibrotic drugs has been the lack of a reproducible, relatively inexpensive, large animal model of fibrosis-driven luminal stricture. This study aimed to evaluate the feasibility of creating a model of luminal GI tract strictures. Argon plasma coagulation (APC) was applied circumferentially in porcine esophagi in vivo. Follow-up endoscopy (EGD) was performed at day 14 after the APC procedure. We noted high grade, benign esophageal strictures (n = 8). All 8 strictures resembled luminal GI fibrotic strictures in humans. These strictures were characterized, and then successfully dilated. A repeat EGD was performed at day 28 after the APC procedure and found evidence of recurrent, high grade, fibrotic, strictures at all 8 locations in all pigs. Pigs were sacrificed and gross and histologic analyses performed. Histologic examination showed extensive fibrosis, with significant collagen deposition in the lamina propria and submucosa, as well as extensive inflammatory infiltrates within the strictures. In conclusion, we report a porcine model of luminal GI fibrotic stricture that has the potential to assist with developing novel anti-fibrotic therapies as well as endoscopic techniques to address recurring fibrotic strictures in humans.

Management of Asymptomatic Terminal Ileitis.

Ileal intubation is often performed during screening colonoscopies. This had led to the recognition of mild ileitis in many asymptomatic patients. The natural history and clinical significance of this finding are not well established, and there are no guidelines on whether these patients merit further work-up or an interval surveillance colonoscopy. This conundrum was presented and discussed on @MondayNightIBD. In this article, we review the specific literature on the topic and make reference to the informed opinions of the convo participants. We propose an #IBDAlgorithm for management of asymptomatic ileitis.

#MondayNightIBD: Management of Chronic #Pouchitis.

We present a #MondayNightIBD case of chronic pouchitis and discuss key diagnostic and therapeutic challenges commonly encountered in clinical practice. We make reference not only to the limited published literature but also to the informed opinions of the #MondayNightIBD discussion participants, identifying gaps where management guidelines and research are needed. A #MondayNightIBD Algorithm for Pouchitis Management is proposed.

Pancreatic Pseudocysts and Parenchymal Necrosis in Patients With Autoimmune Pancreatitis: A Systematic Review.

OBJECTIVE: To investigate the prevalence and natural history of pancreatic pseudocysts (PCs) and parenchymal necrosis in autoimmune pancreatitis (AIP). METHOD: A search using PubMed, Embase, Scopus, and Cochrane was performed. Search terms were AIP, PC, acute fluid collection, and pancreatic necrosis. RESULTS: Fifteen studies with 17 patients were included. In 8 of 17 patients, PC was noted concurrently with the AIP diagnosis, whereas in the other half, PC appeared months or years after. In 10 of 17 cases, PC appeared as solitary. The location was variable. Pseudocysts were small (<3 cm) in 4 cases and large (>3 cm) in 13 cases. A normal pancreatic duct was observed in 6 of 17 cases, whereas 9 of 17 had pancreatic duct stenosis. Steroids were given to 4 of 4 small and 10 of 13 large PC. All small PC resolved with steroids, whereas only 4 of 10 large PC treated had some response. Most (9/13) of large PC underwent endoscopic or surgical procedures. None of the 17 cases developed necrosis. CONCLUSIONS: Pseudocysts in AIP are rare. Pancreatic pseudocyst can present in variable number, size, and location. Small PC resolved with steroids. Large PC had poor response to steroids requiring invasive interventions. Necrosis in AIP has not been reported.

Henoch-Schönlein Purpura with Adalimumab Therapy for Ulcerative Colitis: A Case Report and Review of the Literature.

Tumor necrosis factor-α (TNFα) inhibitor therapy has signified an important milestone in the fight against many rheumatological disorders and inflammatory bowel disease (IBD). Cutaneous adverse events caused by this class of medications are well known but relatively uncommon. Most reactions are mild and rarely warrant treatment withdrawal. Henoch-Schönlein purpura (HSP) is a disease with cutaneous vasculitis, arthritis, and gastrointestinal and renal involvement that is usually seen in children, though the worst complications are typically seen in adults. We present a case of HSP complicating adalimumab treatment in a patient with ulcerative colitis who had achieved endoscopic remission. We review similar cases reported in the literature and discuss the consequences of these autoimmune diseases.

Extracellular superoxide dismutase ameliorates skeletal muscle abnormalities, cachexia, and exercise intolerance in mice with congestive heart failure.

BACKGROUND: Congestive heart failure (CHF) is a leading cause of morbidity and mortality, and oxidative stress has been implicated in the pathogenesis of cachexia (muscle wasting) and the hallmark symptom, exercise intolerance. We have previously shown that a nitric oxide-dependent antioxidant defense renders oxidative skeletal muscle resistant to catabolic wasting. Here, we aimed to identify and determine the functional role of nitric oxide-inducible antioxidant enzyme(s) in protection against cardiac cachexia and exercise intolerance in CHF. METHODS AND RESULTS: We demonstrated that systemic administration of endogenous nitric oxide donor S-nitrosoglutathione in mice blocked the reduction of extracellular superoxide dismutase (EcSOD) protein expression, as well as the induction of MAFbx/Atrogin-1 mRNA expression and muscle atrophy induced by glucocorticoid. We further showed that endogenous EcSOD, expressed primarily by type IId/x and IIa myofibers and enriched at endothelial cells, is induced by exercise training. Muscle-specific overexpression of EcSOD by somatic gene transfer or transgenesis (muscle creatine kinase [MCK]-EcSOD) in mice significantly attenuated muscle atrophy. Importantly, when crossbred into a mouse genetic model of CHF (α-myosin heavy chain-calsequestrin), MCK-EcSOD transgenic mice had significant attenuation of cachexia with preserved whole body muscle strength and endurance capacity in the absence of reduced HF. Enhanced EcSOD expression significantly ameliorated CHF-induced oxidative stress, MAFbx/Atrogin-1 mRNA expression, loss of mitochondria, and vascular rarefaction in skeletal muscle. CONCLUSIONS: EcSOD plays an important antioxidant defense function in skeletal muscle against cardiac cachexia and exercise intolerance in CHF.

Leiomyosarcoma of the splenic vein.

Leiomyosarcomas arising from the wall of blood vessels are rare and aggressive neoplasm. We report a case of a previously healthy 66-year-old woman who presented with intermittent abdominal pain, progressive constipation, and weight loss. Abdominal computed tomography showed a 12 cm solid heterogeneous tumor in the tail of the pancreas. The patient subsequently underwent surgical resection of the pancreatic mass. Surprisingly, histological and immunohistochemical analyses revealed leiomyosarcoma arising from the smooth muscle of the splenic vein. After surgery, she received adjuvant chemotherapy. One year later, there was no evidence of local recurrence. In this paper, we discuss the available information about leiomyosarcomas of splenic vein and its management.

A primary cardiac osteosarcoma: Case report and review of the literature.

Primary cardiac osteosarcoma is a rare and aggressive neoplasm that can be difficult to diagnose. We report a case of a previously healthy 49-year-old woman who presented with dyspnea, atrial flutter, and heart failure. A mass was visualized in her left atrium by echocardiography and cardiac computed tomography, and the diagnosis of cardiac myxoma was raised. The patient subsequently underwent surgical resection of the mass and atrial reconstruction. Surprisingly, histological and immunohistological analyses revealed the mass to be an osteosarcoma. The patient received chemotherapy and radiotherapy. Eight months later, she has shown evidence of local recurrence. We briefly discuss primary osteosarcomas in the cardiac cavity and their management. .

Una actualización de pancreatitis crónica: artículo de revisión / Update on chronic pancreatitis: review article

La pancreatitis crónica es una enfermedad fibro-inflamatoria progresiva del páncreas caracterizada por la fibrosis irreversible de la glándula con el eventual fallo de las funciones exocrinas y endocrinas. Las características distintivas de la enfermedad son el dolor abdominal, la malabsorción, la desnutrición, la diabetes mellitus y las calcificaciones pancreáticas. En muchos pacientes el origen de esta enfermedad se debe a una compleja mezcla de factores ambientales (por ejemplo, alcohol, cigarrillos y productos químicos en el trabajo), factores genéticos y en algunos casos origen hereditario o autoinmune. El manejo incluye enfoques médico, endoscópico y quirúrgico con la necesidad de la interacción entre diversas especialidades para dar un enfoque multidisciplinario coordinado. Esta revisión ofrece una visión general de los estudios recientes resumiendo la epidemiología, etiología, fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento de la enfermedad.

Chronic pancreatitis is a progressive fibro-inflammatory disease of the pancreas characterized by irreversible fibrosis of the gland with eventual failure of exocrine and endocrine functions and hallmark features of abdominal pain, malabsorption, malnutrition, diabetes mellitus and pancreatic calcifications. In many patients this disease results from a complex mix of environmental (eg, alcohol, cigarettes, and occupational chemicals), genetic factors and a few patients with hereditary or autoimmune disease. The management includes medical, endoscopic and surgical approaches with the need for interaction between various specialties, calling for a concerted multidisciplinary approach. This review provides the reader with a comprehensive overview of the studies summarizing the epidemiology, etiology, physiopatology, clinical manifestation, diagnosis and treatments of the disease.

Acute respiratory distress syndrome due to Clostridium difficile colitis: a case report and review of the literature / Síndrome de distress respiratorio agudo debido a colitis por Clostridium difficile: reporte de caso y revisión de la literatura

Clostridium difficile is a major cause of antibiotic-associated diarrhea. We report a patient with complicated Clostridium difficile infection (CDI) who developed rapidly progressive acute respiratory distress syndrome (ARDS), for which CDI was the only identifiable source. CDI should be considered in the differential diagnosis for anyone with diarrhea who presents ARDS, especially in high-risk groups such as the elderly, hospitalized patients, or those who have had a history of CDI.

El Clostridium difficile es una de las causas más frecuentes de diarreas asociadas a antibióticos. Reportamos un paciente con infección por Clostridium difficile complicada (CDI) que desarrolló rápida y progresivamente un síndrome de distress respiratorio agudo (ARDS), del cual el CDI fue la fuente única identificable. El CDI debe considerarse en el diagnóstico diferencial de cualquier persona con diarrea que presenta ARDS, especialmente en los grupos de alto riesgo como los ancianos, pacientes hospitalizados o aquellos que han tenido historia precia de DCI.