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1.
–  Carcinoma of the pancreas remains a formidable challenge for clinicians.
–  Pancreatic cancer is the tenth most common type of new cancer, and according to estimates of the American Cancer Society, 29,000 new cases occurred in 1998.
–  The relative frequency of the disease is compounded by its lethality. Pancreatic cancer accounts for 5% and 6% of cancer deaths among men and women respectively, making it the fourth leading site of cancer deaths.
–  In 1998, an estimated 28,000 deaths from pancreatic cancer were expected.
–  The five-year relative survival rate for pancreatic cancer between 1986 and 1993, as reported by the NCI Surveillance, Epidemiology, and End Results Program, was only 4%.
–  Although this was a statistically significant increase from the 3% five-year survival rate for pancreatic cancer between 1974 and 1976 it remained the lowest survival rate among all reported sites. By comparison, survival for gastric and esophageal cancers during the more recent reporting period were 19% and 12% respectively.
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2.
Opinion statement  
–  Dysplasia is the most important marker of progression to invasive cancer in Barrett’s esophagus.
–  Intensive endoscopic surveillance with biopsy may identify invasive cancer in a patient with high-grade dysplasia (HGD).
–  Close relationship with an experienced gastrointestinal pathologist and thoracic surgeon will improve treatment decisions and patient outcomes.
–  No intervention is required in patients with low-grade dysplasia (LGD); continued surveillance is recommended.
–  Surgical resection is the currently accepted therapy for high-grade dysplasia. Endoscopic ablative therapy remains experimental.
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3.
Opinion statement  
–  Esophageal malignancies presenting with dysphagia from luminal obstruction generally are not resectable for cure, and palliative therapy is the primary focus.
–  Self-expandable metal stents (SEMS) have replaced plastic stents as a primary mode of palliation for malignant esophageal obstruction because of the relative ease of insertion, lower initial morbidity, and larger stent diameter.
–  Self-expandable metal stents are ideal for patients with midesophageal tumors.
–  A majority of patients experience relief of dysphagia with SEMS and dietary modification, but the initial cost is high and early morbidity may be significant.
–  The placement of SEMS across the gastroesophageal junction may result in free reflux that may improve with a stent containing a one-way gastric flap valve.
–  The placement of SEMS in the cervical esophagus, although more difficult, less effective, and less well tolerated, also may be successful.
–  Coated SEMS are a treatment of choice for individuals with tracheoesophageal fistula.
–  Delayed complications occur in up to 40% of patients and include stent migration, bleeding, perforation, fistula formation, and occlusion. Most complications can be managed endoscopically and additional stents may be placed for tumor overgrowth.
–  The comparison of three currently available SEMS for esophageal malignancy show no statistically significant differences with regard to ease of placement, effectiveness, complications, and mortality.
–  The use of SEMS for patients with benign disease is still considered experimental.
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4.
Opinion statement  
–  Prenatal diagnosis of hypoplastic left heart syndrome is strongly encouraged through careful, routine level II ultrasound screening, especially in highrisk families.
–  A decision for palliative care (leading to death) should be discouraged if cardiac anatomy or physiology is favorable, centers of expertise are available, sufficient resources exist, and there are no associated coexisting anomalies or genetic defects that permit little chance of meaningful survival.
–  Staged surgical palliation is preferred over cardiac transplantation as the initial therapeutic approach.
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5.
Opinion statement  
–  An oral calcium supplement (1000 mg/day) is recommended.
–  Regular exercise should be performed.
–  Ethanol intake should be moderate.
–  Protein intake should be moderate.
–  The patient’s vitamin D status should be determined and corrected with an oral supplement when deficiency is present.
–  Baseline and yearly bone density measurement should be taken.
–  Alendronate, 10 mg/d orally, or risedronate, 5 mg/d orally, should be given to patients with osteopenia.
–  Use of corticosteroids, cyclosporin, tacrolimus, and methotrexate should be limited to the short term when possible.
–  Estrogen replacement therapy is recommended in postmenopausal women unless contraindications exist.
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6.
Opinion statement  
–  Disorders of augmented visceral perception include functional (or nonulcer) dyspepsia and irritable bowel syndrome (IBS).
–  Enhancement of luminal perception can result from alterations in normal elastic gut wall properties or exaggerated responsiveness of visceral sensory nerve pathways.
–  Standard therapies for functional dyspepsia are effective in subsets of patients and may act in part by compensating for gastric hypersensitivity (as with acid-suppressing drugs) or by enhancing gastric compliance (as with some motor-stimulating agents).
–  Likewise, conventional treatments of patients with IBS reduce visceral perception via effects on sensory nerve function (fiber supplements) or luminal wall properties (antispasmodic drugs).
–  Antidepressants are increasingly used in patients with functional dyspepsia or IBS. These drugs have several purported mechanisms, including 1) luminal relaxation, 2) blunting of visceral hypersensitivity, and 3) modulation of central nervous system pain processing pathways.
–  The efficacy of available and investigational agents that act as visceral analgesics or luminal relaxants is an area of intense pharmaceutical research.
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7.
Opinion statement  The first goal of therapy is the control of gastric acid hypersecretion using PPIs or high-dose H2R antagonists.
–  The diagnosis of Multiple Endocrine Neoplasia (MEN I) should be established early in the disease.
–  Localization of gastrinoma tumor should be performed using a combination of endoscopic ultrasonography (EUS), somatostatin receptor scintigraphy (SRS), and computerized tomography (CT), or Magnetic Resonance Imaging (MRI).
–  Surgical resection in sporadic ZES should be performed to attempt cure of tumor.
–  Surgery, hormonal, chemotherapy, embolization therapy or therapeutic OctreoScan should be considered in patients with metastatic tumor.
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8.
Opinion statement  
–  Primary biliary cirrhosis (PBC) is one of the most common chronic cholestatic liver diseases affecting the adult population.
–  The clinical presentation of PBC can be diverse, ranging from the presymptomatic individual to the patient with advanced liver disease. The initial evaluation to establish the diagnosis, and the appropriate followup, are very important in the lifelong management of these patients.
–  The primary medical treatment in PBC should focus on reducing the rate of disease progression. To this extent, ursodeoxycholic acid has been extensively evaluated and proven to improve liver biochemistries and survival in patients with PBC.
–  The secondary medical management in PBC should address the treatment of complications of chronic cholestasis, hepatic cirrhosis, and failure.
–  Liver transplantation remains the only established therapeutic approach in treating patients with end-stage PBC and its associated complications.
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9.
Opinion statement  
–  There is currently no effective treatment for patients with nonulcer dyspepsia.
–  Helicobacter pylori eradication has no beneficial effect on dyspeptic symptoms.
–  Proton pump inhibitors are superior to placebo in the subset of patients with epigastric pain as the predominant symptom. H2 Receptor antagonists have no effect.
–  Patients with dysmotility-like dyspepsia should be treated first with prokinetics. Unfortunately, cisapride no longer can be used to treat patients with functional dyspepsia because of reports of serious cardiovascular side effects and subsequent withdraw from the US market. Therefore, metoclopramide (or domperidone, if available) should be given. Treatment with motilides has no use in the relief of symptoms, even in patients with delayed gastric emptying.
–  If the initial therapy has no effect after 4 weeks, switch treatment (eg, from proton pump inhibitor to metoclopramide or vice versa).
–  If both of these pharmacologic therapies fail, consider treatment with an antidepressant (or with buspirone, an anxiolytic agent) or psychotherapy.
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10.
–  Colorectal cancer (CRC) is a complication in both patients with longstanding ulcerative colitis and those with Crohn’s disease. As with sporadic CRC, surgical therapy (with adjuvant chemotherapy in advanced disease) is the only effective treatment.
–  Identifying risk factors for CRC in inflammatory bowel disease (IBD) should allow patients to receive appropriate medical, endoscopic, and surgical care to minimize CRC morbidity and mortality.
–  Total proctocolectomy remains the most effective form of cancer prophylaxis in IBD patients, but because of the impact of this approach and the low absolute risk of cancer, clinicians seldom recommend it.
–  Colonoscopic surveillance with systematic biopsies is used to detect mucosal dysplasia and thus identify those patients at greatest risk for developing CRC. Patients with dysplasia other than that in readily excised polyps should be referred for surgery. Although fraught with limitations, surveillance is the best method currently available for reducing CRC mortality and morbidity short of prophylactic colectomy. It will have to remain the standard of practice until better diagnostic tests are available. Surveillance should be offered and performed in the same manner for patients with Crohn’s disease and ulcerative colitis.
–  Chemoprevention may prove effective in the future, but currently used agents have only a modest benefit, if any.
–  Adenocarcinoma of the small intestine occurs at an increased rate in patients with Crohn’s disease of the bowel with longstanding small bowel involvement, but there are no current methods of early detection. Treatment is based on disease identified from evaluation of symptoms or incidental finding.
–  Some extraintestinal cancers have been noted to occur at increased rates in series from referral centers but not in population-based studies.
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11.
Opinion statement  
–  Physicians must aggressively treat heart failure in the early stages to prevent disease progression and improve survival. Early treatment implies early diagnosis of left ventricular (LV) dysfunction, before the onset of symptoms. Patients with risk factors for the development of heart failure, especially coronary disease or hypertension, should undergo echocardiography to evaluate LV function. Patients with LV systolic dysfunction should be further evaluated to determine the type of cardiac dysfunction, uncover correctable etiologic factors, determine prognosis, and guide treatment.
–  Angiotensin-converting enzyme (ACE) inhibitors and beta-adrenergic blocking drugs improve survival and are integral to the treatment plan. Physicians should prescribe an ACE inhibitor as initial therapy for all patients with LV systolic dysfunction unless there are specific contraindications. The combination of hydralazine and isosorbide dinitrate is an acceptable alternative therapy for patients who cannot take ACE inhibitors.
–  Diuretics should be used if there are signs or symptoms of volume overload.
–  Beta-adrenergic blocking drugs should be added to therapy in stable patients with mild to moderate heart failure after optimal treatment with ACE inhibitors, diuretics, or other vasodilators.
–  Digoxin should be used routinely in patients with severe heart failure and should be added to therapy in patients with mild to moderate heart failure who remain symptomatic despite optimal doses of ACE inhibitors and diuretics.
–  Spironolactone should be added, but electrolytes should be closely monitored.
–  Warfarin anticoagulation should be considered in patients with a left ventricular ejection fraction (LVEF) of 35% or less.
–  Until survival data exist, angiotensin receptor blockers (ARBs) should not be used as initial therapy or as sole therapy but can be used for ACE-intolerant patients or can be added to standard heart failure therapy.
–  Outpatient use of intravenous inotropic therapy should be avoided.
–  Patients with severe heart failure should have peak oxygen consumption measured to quantify functional impairment, determine prognosis, and identify the need for advanced heart failure therapy.
–  Patients who remain symptomatic while receiving optimal standard therapy should be referred early to a specialized heart failure center.
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12.
Opinion statement  
–  The treatment of sarcoid cardiomyopathy can be considered in part the treatment of the systemic disorder, and in part cardiac involvement, the manifestations of which may differ greatly. Therapy for the systemic disease is corticosteroid. Therapy for cardiac involvement includes prednisone, but because treatment must ameliorate or abolish many differing manifestations, therapy differs among patients.
–  Asymptomatic patients (the majority) who are free from serious manifestations of the disease do not require pharmacologic or interventional treatment.
–  Patients with dilated cardiomyopathy require treatment for congestive heart failure.
–  High-grade atrioventricular conduction delay usually necessitates a permanent electronic pacemaker.
–  Life-threatening arrhythmia usually requires implantation of an automatic implantable cardiac defibrillator (AICD). Antiarrhythmic drugs may also be needed.
–  Cardiac tamponade should be treated by drainage of pericardial fluid.
–  Pericardiectomy is usually the appropriate treatment for patients who develop significant constrictive pericarditis.
–  Calcium channel blockers may be helpful for severe diastolic dysfunction that occurs in those with restrictive cardiomyopathy.
–  Therapy should be given to those few patients who manifest hypertrophic cardiomyopathy to relieve left ventricular outflow obstruction.
–  Cardiac transplantation for intractable heart failure or arrhythmia may be needed.
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13.
Opinion statement  
–  Adenocarcinomas of the small intestine are rare tumors accounting for about 1% of all gastrointesinal neoplasms.
–  These cancers have a poor prognosis, perhaps because of the nonspecific symptoms and delay in diagnosis.
–  They can arise in the setting of Crohn’s disease, celiac sprue, and inherited colon cancer syndromes like familial adenomatous polyposis (FAP).
–  As most of these lesions occur in the duodenum or proximal jejunum, upper endoscopy and push enteroscopy are reasonable initial diagnostic tests. Enteroclysis and intraoperative enteroscopy may be complementary to these procedures.
–  Surgical cures may be possible in patients with tumors detected at an early and resectable stage. Endoscopic therapy is useful for cure of premalignant lesions and palliation of unresectable disease.
–  Chemotherapy and radiotherapy have limited impact in the management of these tumors, because they are often of advanced stage at the time of presentation.
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14.
Opinion statement  
–  Functional dyspepsia is a heterogeneous disorder in which distinct pathophysiological abnormalities are present in subgroups of patients. Accommodation of the proximal stomach to a meal is impaired in 40% of patients with functional dyspepsia. This is associated with symptoms of early satiety and weight loss.
–  The presence of early satiety as a relevant or severe symptom is a good predictor of impaired accommodation.
–  Gastric barostat or proximal gastric ultrasound may confirm the presence of impaired accommodation after a meal. Sophisticated analysis of scintigraphic gastric emptying images or a simple caloric drinking test are under investigation in the diagnosis of impaired accommodation.
–  It seems logical to have patients eat more frequent, smaller sized meals. Cisapride is the only well-evaluated form of pharmacological treatment for this condition, and has been withdrawn from the US market (see Important Note under Treatment, below). Small or preliminary studies suggest some benefit from buspirone or selective serotonin reuptake inhibitors (SSRIs).
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15.
Opinion Statement  
–  Early diagnosis permits preventive therapy to preempt development of organ damage.
–  In all diagnosed patients, both symptomatic and asymptomatic, pharmacologic therapy is lifelong, and maintenance treatment to prevent copper toxicity is mandatory.
–  Patients with either fulminant hepatic failure or hepatic insufficiency unresponsive to medical therapy should be considered for orthotopic liver transplantation, which effectively cures Wilson’s disease.
–  Prognosis is excellent for compliant patients receiving pharmacologic therapy.
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16.
Opinion Statement  
–  Progress has been made in the adjuvant treatment of colorectal carcinoma.
–  The improvement in survival with the use of adjuvant 5-FU and leucovorin in patients with stage III colon carcinoma has been readily established. However, a survival benefit in stage II patients treated with adjuvant therapy remains unproven. Further evaluation using additional/new prognostic factors may identify a high-risk stage II group that would benefit from adjuvant treatment.
–  Adjuvant chemoradiation has become standard therapy for stage II and III patients with rectal carcinoma. Investigations using preoperative combined-modality therapy are being explored to assess sphincter preservation rates and to evaluate any impact on survival. Radiosensitizing chemotherapeutic agents need to be evaluated in this patient population.
–  Recent advances in metastatic disease have occurred. Frontline therapy remains 5-FU and leucovorin. CPT-11 has demonstrated responses in 5-FU relapsed and refractory patients and is the new standard therapy in these patients. New data recently available also show a survival advantage in patients treated with CPT-11 versus supportive care in 5-FU and leucovorin failures.
–  New agents such as UFT and oxaliplatin have demonstrated activity in colorectal carcinomas and in the future these agents will likely aid in the treatment of this disease.
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17.
Opinion statement  
–  Fulminant ulcerative colitis necessitates immediate hospitalization.
–  Supportive therapy such as aggressive rehydration, restriction of oral intake, and consideration of parenteral nutrition should be initiated.
–  High-dose intravenous steroids should be started in almost all cases.
–  Antibiotics and cyclosporine should be considered, especially in disease refractory to steroid therapy.
–  Indications for surgery should always be kept in mind, and early involvement of the surgical team is always encouraged.
–  Avoidance of life-threatening complications such as toxic megacolon, hemorrhage, and perforation is the goal of any treatment for fulminant ulcerative colitis.
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18.
Opinion statement  
–  Coagulopathy in patients with liver disease results from impairments in the clotting and fibrinolytic systems, as well as from reduced number and function of platelets.
–  Parenteral vitamin K replacement corrects coagulopathy related to biliary obstruction, bacterial overgrowth, or malnutrition. Vitamin K is less effective for coagulopathy caused by severe parenchymal liver injury.
–  Transfusion of fresh frozen plasma is the hallmark of treatment of significant coagulopathy in patients with liver disease and active bleeding.
–  Transfusion of fresh frozen plasma also reverses moderate to severe coagulopathy of cirrhosis prior to invasive procedures.
–  Cryoprecipitate is useful for severe coagulopathy with hypofibrinogenemia, especially when avoidance of volume overload is desired.
–  Exchange plasmapheresis is useful in selected patients with coagulopathy due to liver disease, in whom fresh frozen plasma fails to correct coagulopathy or in patients who have coexistent severe fluid overload.
–  Platelet transfusions, pooled or single donor, are useful in thrombocytopenic patients prior to performing invasive procedures or in the presence of significant bleeding, especially when the platelet count is below 50,000/mL.
–  The use of recombinant factor VIIa and thrombopoietin therapy for correction of coagulopathy and thrombocytopenia, respectively, in patients with cirrhosis, is currently under investigation.
–  Therapy with prothrombin complex concentrates, 1-deamino-8-D-arginine vasopressin and antithrombin III concentrates for the management of coagulopathy caused by liver disease can be hazardous and the use of these products is considered investigational at the present time.
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19.
Opinion statement  
–  The percutaneous approach-specifically, ultrasound-guided percutaneous catheter drainage-associated with intravenous administration of antibiotics is the therapy of choice in patients with single or multiple pyogenic liver abscesses.
–  The initial empiric antibiotic therapy should be modified based on results of blood and pus cultures.
–  Surgery should be reserved for patients in whom antibiotic therapy and percutaneous drainage is unsuccessful.
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20.
Opinion statement  
–  Venous malformations of blue rubber bleb nevus syndrome (BRBNS) may involve any area of the gastrointestinal tract.
–  Gastrointestinal blood loss and anemia brings these patients to the attention of gastroenterologists.
–  Effective treatment of these malformations throughout the gastrointestinal tract requires aggressive management to ultimately decrease blood loss and restore the patient’s hemoglobin to a near-normal level.
–  Treatment of patients with BRBNS includes supportive measures, endoscopic ablation, and surgery.
–  Supportive therapy consists of proton pump inhibitors and octreotide to decrease blood loss, iron replacement, and blood transfusions.
–  The effective management of patients with anemia demands aggressive treatment of venous malformations in the small bowel. This requires a collaboration between the surgeon and the therapeutic endoscopist, ie, laparotomy and excision of larger lesions with surgically assisted enteroscopy and thermal ablation of smaller lesions via enterotomy.
–  There is no effective systemic therapy for treatment of the vascular malformations in patients with BRBNS.
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