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1.
Opinion statement  
–  Crohn’s disease and ulcerative colitis remain medically incurable conditions with potentially significant morbidity. The treatment of children with these conditions therefore should seek to reduce or eliminate symptoms, optimize nutritional status, promote normal growth and development, prevent complications, and minimize the potential psychologic effects of chronic illness. Treatment strategies must seek to both induce and maintain clinical remission. For all but the most mildly affected children with Crohn’s disease, a combination of nutritional and pharmacologic approaches is optimal. For those with ulcerative colitis, anti-inflammatory medication is necessary.
–  Moderate to severe Crohn’s disease acutely responds best to potent immunomodulatory therapy, eg, corticosteroids and infliximab. Either agent must be coupled with 6-mercaptopurine or azathioprine to maintain long-term remission and to minimize toxicity. Particular attention must be paid to limit the growth suppression and other toxic effects of corticosteroids. Elemental or semielemental enteral nutrition also can induce remission effectively, but relapse is common after primary nutritional therapy is discontinued, mandating concomitant pharmacologic therapy with either 6-mercaptopurine or azathioprine. The availability of 6-mercaptopurine/azathioprine metabolite testing allows optimization of immunomodulatory therapy, detection of noncompliance, and avoidance of potentially dangerous toxicity.
–  Mild ulcerative colitis acutely responds to treatment with a 5-aminosalicylate medication. Long-term remission frequently can be maintained with the same medication. Moderate to severe disease activity requires potent immunomodulatory therapy if colectomy is to be avoided.
–  Surgery is a potential cure for patients with ulcerative colitis, although the development of pouchitis after ileal pouch anal anastomosis is common and frequently requires long-term medical management. Surgery provides only palliative relief of complications in those with Crohn’s disease.
–  Emerging therapies, especially evolving biologic and probiotic agents, offer hope for better treatments in the years ahead.
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2.
–  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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3.
Opinion statement  Acute lower gastrointestinal bleeding is an uncommon but potentially life-threatening complication of Crohn’s disease. Because of the relative infrequency of severe bleeding due to CD, reports of larger numbers of cases are few, and the medical literature provides insufficient information to direct management. Nevertheless, certain clear guidelines can be offered once other causes for GI blood loss have been excluded and the diagnosis of a Crohn’s-related bleed is likely. While the initial episode may abate spontaneously, the primary treatment is determined by the severity and persistence of bleeding as well as the risk for recurrence.
–  Localization of the site of bleeding is essential whether endoscopic therapy, surgery, or medical management is attempted.
–  Endoscopic evaluation is preferable, if feasible, as it will provide a broader assessment of extent of disease as well as having a reasonable likelihood of identifying the site of bleeding with possible therapeutic intervention.
–  An angiogram can be a useful alternative, but therapeutic intervention with embolization should be avoided because of the risk for intestinal infarction. Surgery remains the standard of care for persistent severe acute bleeds.
–  If the acute bleeding ceases, an interval to attempt pharmacologic therapy may be available, permitting treatment of the underlying Crohn’s disease. Newer therapies such as infliximab, which may produce relatively rapid mucosal healing, may be beneficial; while they offer hopeful treatment alternatives, they have not been demonstrated to be of use for these cases.
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4.
Opinion statement  
–  6-Mercaptopurine and its prodrug counterpart, azathioprine, have proven efficacy in the induction and maintenance of remission, fistula closure, and steroid sparing in patients with Crohn’s disease. Long-term follow-up has demonstrated the safety of the purine analogues, with no increased risk of malignancy. For patients with Crohn’s disease intolerant or unresponsive to azathioprine or 6-mercaptopurine, methotrexate has emerged as an effective alternative.
–  In patients with severe ulcerative colitis, intravenous cyclosporine is highly efficacious in the short term, and with the addition of azathioprine or 6-mercaptopurine to oral cyclosporine, long-term remission rates of 60% to 70% can be achieved. Azathioprine or 6-mercaptopurine therapy is effective in patients with steroid-dependent or steroid-refractory colitis and is valuable in maintaining remission. Neither methotrexate nor cyclosporine has been shown to be effective for maintenance therapy in patients with ulcerative colitis.
–  Current data are insufficient to recommend routine use of genetic or enzymatic testing of thiopurine methyltransferase or measurements of blood 6-thioguanine metabolites to guide 6-mercaptopurine or azathioprine dosing.
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5.
Opinion statement  
–  Crohn’s disease of the small intestine is a chronic relapsing disease that requires all the knowledge and ingenuity of the gastroenterologist to successfully treat the disease and the patient.
–  For mild to moderate disease, the first line of therapy is to utilize maximum doses of mesalamine to achieve a remission.
–  If the patient relapses, the maximum dose of mesalamine required to achieve remission should be continued.
–  If the disease relapses despite maximum mesalamine, antibiotics should be tried (before prescribing corticosteroids) using ciprofloxacin 500 mg b.i.d., alone or in combination with metronidazole 250 mg q.i.d. for 2 to 3 weeks. If successful, antibiotics can be slowly tapered off.
–  If antibiotics are unsuccessful, one may try elemental diet for 2 weeks before starting corticosteroids.
–  For moderate to severe Crohn’s disease, begin 40 mg of prednisone. After 2 weeks, taper slowly and start 6-MP or azathioprine, which can be used for several years.
–  If the disease recurs on 6-MP or azathioprine, or prior to 6-MP or azathioprine having a chance to be effective, give an IV infusion of Infliximab, which can be repeated at 4 to 8 weeks after the initial infusion.
–  If the patient continues to be unwell, surgery should be contemplated.
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6.
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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7.
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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8.
Opinion statement  
–  Extraintestinal complications of inflammatory bowel disease (IBD) are often secondary to the underlying disease. Therefore, the first priority is to get active IBD into remission with medications, since surgery for IBD is not indicated for the treatment of extraintestinal complications.
–  Symptoms of extraintestinal complications usually can be treated with simple agents; the treatment of patients with refractory symptoms and the use of more complex drug regimens should be done in cooperation with specialists on affected organ systems.
–  Careful consideration of prescribed drugs is necessary because they may negatively influence the course of IBD.
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9.
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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10.
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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11.
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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12.
Protein-losing enteropathy and gastropathy   总被引:1,自引:0,他引:1  
Opinion statement  
–  The diagnosis of protein-losing enteropathy (PLE) should be considered in all patients with hypoalbuminemia and edema without other known causes, and established by plasma alpha1-antitrypsin (a1-AT) clearance or nuclear studies.
–  The therapy for PLE should focus principally on the treatment of the underlying disease after it has been identified. Therapeutic goals should include improvement of hypoalbuminemia, edema, and lymphopenia.
–  The existing primary literature for therapy of PLE syndromes consists mainly of case reports and expert opinions, subject to substantial reporting bias and unknown rates of spontaneous remission; the rarity of and the diversity among this set of diseases make future large randomized trials unlikely. Therapeutic choices, therefore, must involve clinical acumen, empiricism, and understanding of the pathophysiology of the underlying disease process, and must be tailored to each individual patient’s syndrome.
–  Dietary interventions including hypolipidic, high-protein regimens, supplemented by medium-chain triglycerides (MCTs), are extremely useful, particularly in protein loss due to increased lymphatic pressure.
–  Corticosteroids can be very useful in certain cases of PLE (though not without substantial long-term toxicity) when clinical serologic or histologic markers of inflammatory disease are present. Octreotide is a well tolerated drug that has been demonstrated to improve PLE in some patients, and is worth consideration.
–  Octreotide is a well tolerated drug that has been demonstrated to improve PLE in some patients, and is worth consideration.
–  Surgery finds its best role in treating gastrointestinal protein loss from neoplasia, inflammatory bowel disease, and hypertrophic gastritis. Most other PLEs are distributed too widely for surgical intervention.
–  Protein-losing gastropathy (PLG) behaves somewhat differently from the general group of PLE, marked by excellent responses to elimination of Helicobacter pylori, antisecretory therapy, and surgical resection.
–  Protein-losing enteropathy stemming from cardiovascular disease is best treated by medical or surgical cardiovascular interventions; however, some patients may respond to mucosa-directed therapy.
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13.
Opinion statement  
–  Patients with chronic functional abdominal pain (CFAP) present particularly challenging management issues for primary care physicians and gastroenterologists.
–  A major objective in the care of patients with CFAP is to develop an understanding of this condition as a biopsychosocial disorder where symptoms can be attributed to “brain-gut” dysfunction rather than to a missed structurally based condition.
–  The foundation of any therapeutic strategy is the patient-physician partnership, probably the hardest element of treatment for most physicians.
–  All treatment programs should be based on a multi-component strategy, with the patient as a full partner. Realistic expectations should be agreed upon for “care” rather than “cure.” The patient’s preferences should be a major factor in the decision as to whether to recommend pharmacologic or psychological treatment, or a combination of the two.
–  Antidepressant drugs are increasingly being used in CFAP, and well-designed studies are emerging to confirm their clinical effectiveness.
–  Patients’ understanding of the indications for and effects of antidepressants must be reframed in order to address barriers and achieve adherence to therapy.
–  The clinical effectiveness of antidepressants can often only be assessed after 3 to 6 weeks of therapy. The side effects, which appear early, are usually transient and often disappear within 1 to 2 weeks.
–  There is no conclusive evidence that one antidepressant drug is more effective than others, although most studies have tested tricyclic agents. The most important factors in the choice of antidepressant medication are side-effect profile, physician familiarity and comfort with the drug, and cost.
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14.
Opinion statement  
–  There is no definitive therapy for this disorder.
–  Heliobacter pylori eradication therapy is only useful in areas with a high prevalence of H. pylori-related ulcer disease
–  Proton pump inhibitors are effective in short-term treatment for dyspepsia with a predominant symptom of epigastric pain
–  Prokinetic agents may be useful in some patients, particularly those with dysmotility-like dyspepsia, but serious side effects limit their usefulness.
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15.
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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16.
Opinion statement  
–  Results of recent clinical trials allow an evidence-based approach to ventricular arrhythmias (VAs).
–  The implantable cardioverter-defibrillator (ICD) has clearly established its role in the secondary prevention of VA and should be considered first-line therapy in patients surviving episodes of potentially lethal VAs. It has also been clearly shown that in these patients, antiarrhythmic drug selection by means of serial Holter recording or electrophysiologic study does not improve survival.
–  Antiarrhythmic drug therapy (including amiodarone) as primary prevention in high-risk patients (eg, those who have experienced a myocardial infarction or who have heart failure) has thus far not reduced the mortality rate.
–  In contrast, use of the ICD as a primary preventative strategy has reduced the mortality rate in patients after myocardial infarction who have reduced left ventric-ular function, nonsustained ventricular tachycardia, and inducible ventricular tachycardia during electrophysiologic study. Thus, patients fitting this clinical pro-file are best served by implantation of an ICD.
–  Monomorphic ventricular tachycardia occurs rarely in patients without heart disease. These arrhythmias are best treated with catheter ablation therapy, a treatment with a high rate of success and a low rate of complications.
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17.
Opinion statement  
–  Many options are available to diagnose and treat patients with the Budd-Chiari syndrome who present with either thrombotic or non-thrombotic occlusion of the major hepatic veins and or vena cava.
–  The goal of therapy is to alleviate venous obstruction and to preserve hepatic function.
–  Low-sodium diets, diuretics, and therapeutic paracentesis are generally ineffective, except for the rare patient who presents with volume overload and incomplete hepatic venous occlusion.
–  Anticoagulants and thrombolytics may be appropriate for selected patients with acute thrombotic venous obstruction.
–  Percutaneous transluminal angioplasty (PTA) of hepatic venous stenoses or caval webs with or without placement of intraluminal stents yield excellent short-term results, but additional studies are warranted to assess long-term efficacy.
–  Transjugular intrahepatic portosystemic shunts (TIPS) may be effective for patients with subacute or chronic disease and ascites refractory to sodium restriction and diuretics. Intrahepatic stents may also serve as a bridge to transplantation for selected patients presenting with fulminant hepatic failure consequent to hepatic venous occlusion. Additional studies will be necessary to assess the role of TIPS in the armamentarium of therapies for patients with the Budd-Chiari syndrome.
–  Decompressive shunts, reconstruction of the vena cava and hepatic venous ostia, transatrial membranotomy, and dorsocranial resection of the liver with hepatoatrial anastomosis are appropriate options for patients with acute or subacute disease who are not candidates for, or fail less invasive therapies. The majority of patients benefit with improvement in liver function tests, ascites, and liver histology; however, hepatic function may deteriorate in patients with marginal reserve.
–  Liver transplantation is reserved for patients with Budd-Chiari syndrome who present with fulminant hepatic failure or end-stage liver disease with portal hypertensive complications. Transplantation is also appropriate for patients who deteriorate after failed attempts at surgical shunting.
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18.
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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19.
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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20.
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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